[Federal Register Volume 87, Number 124 (Wednesday, June 29, 2022)]
[Proposed Rules]
[Pages 38838-38867]
From the Federal Register Online via the Government Publishing Office [www.gpo.gov]
[FR Doc No: 2022-12980]



[[Page 38837]]

Vol. 87

Wednesday,

No. 124

June 29, 2022

Part II





Social Security Administration





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20 CFR Part 404





Revised Medical Criteria for Evaluating Cardiovascular Disorders; 
Proposed Rule

Federal Register / Vol. 87, No. 124 / Wednesday, June 29, 2022 / 
Proposed Rules

[[Page 38838]]


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SOCIAL SECURITY ADMINISTRATION

20 CFR Part 404

[Docket No. SSA-2019-0013]
RIN 0960-AI43


Revised Medical Criteria for Evaluating Cardiovascular Disorders

AGENCY: Social Security Administration.

ACTION: Notice of proposed rulemaking (NPRM).

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SUMMARY: We propose to revise some of the criteria in the Listing of 
Impairments (listings) that we use to evaluate claims involving 
cardiovascular disorders in adults and children under titles II and XVI 
of the Social Security Act (Act). The proposed revisions reflect 
advances in medical knowledge, our adjudicative experience, and 
comments we received from experts and the public in response to an 
advance notice of proposed rulemaking (ANPRM), and at an outreach 
policy conference.

DATES: To ensure that your comments are considered, we must receive 
them by no later than August 29, 2022.

ADDRESSES: You may submit comments by any one of three methods--
internet, fax, or mail. Do not submit the same comments multiple times 
or by more than one method. Regardless of which method you choose, 
please state that your comments refer to Docket No. SSA-2019-0013, so 
that we may associate your comments with the correct regulation.
    Caution: You should be careful to include in your comments only 
information that you wish to make publicly available. We strongly urge 
you not to include in your comments any personal information, such as 
Social Security numbers or medical information.
    1. Internet: We strongly recommend that you submit your comments 
via the internet. Please visit the Federal eRulemaking portal at http://www.regulations.gov. Use the search function to find docket number 
SSA-2019-0013. The system will issue a tracking number to confirm your 
submission. You will not be able to view your comment immediately 
because we must post each comment manually. It may take up to a week 
for your comment to be viewable.
    2. Fax: Fax comments to (410) 966-2830.
    3. Mail: Address your comments to the Office of Regulations and 
Reports Clearance, Social Security Administration, 3100 West High Rise, 
6401 Security Boulevard, Baltimore, Maryland 21235-6401.
    Comments are available for public viewing on the Federal 
eRulemaking portal at http://www.regulations.gov or in person, during 
regular business hours, by arranging with the contact person identified 
below.

FOR FURTHER INFORMATION CONTACT: Michael J. Goldstein, Office of 
Disability Policy, Social Security Administration, 6401 Security 
Boulevard, Baltimore, Maryland 21235-6401, (410) 965-1020. For 
information on eligibility or filing for benefits, call our national 
toll-free number, 1-800-772-1213, or TTY 1-800-325-0778, or visit our 
internet site, Social Security Online, at http://www.socialsecurity.gov.

SUPPLEMENTARY INFORMATION: 

Background

    For adults, the listings describe, for each of the major body 
systems, impairments that we consider to be severe enough to prevent an 
individual from doing any gainful activity regardless of his or her 
age, education, or work experience.\1\ For children, the listings 
describe impairments we consider severe enough to cause marked and 
severe functional limitations.\2\ We use the listings at step 3 of the 
sequential evaluation process to identify claims in which the 
individual is clearly disabled under our rules.\3\ However, we do not 
deny any claim solely because a person's medical impairment(s) does not 
satisfy the criteria of a listing.
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    \1\ 20 CFR 404.1525(a) and 20 CFR 416.925(a).
    \2\ 20 CFR 416.925(a).
    \3\ 20 CFR 404.1520, 20 CFR 416.920, and 20 CFR 416.924.
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Why are we proposing to revise the listings for cardiovascular 
disorders?

    We last published final rules that comprehensively revised the 
cardiovascular disorders listings on January 13, 2006, and the rules 
became effective on April 13, 2006.\4\ We are now proposing targeted 
revisions to the cardiovascular disorders listings, as previously 
mentioned, to reflect advances in medical knowledge, our adjudicative 
experience, and comments we received from experts and the public in 
response to an ANPRM, and at an outreach policy conference.
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    \4\ 71 FR 2312 (2006).
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How did we develop this proposed rule?

    In developing this proposed rule:
    <bullet> We published an ANPRM for cardiovascular disorders in the 
Federal Register on April 16, 2008.\5\ We invited the public to send us 
written comments and suggestions about whether and how we should revise 
the cardiovascular disorders listings. We received five comments on the 
ANPRM. The commenters made several suggestions that we incorporated 
into the proposals, such as consideration for people with a single 
ventricle; clarifying in proposed 4.00D(4)(c)(ii) (How do we evaluate 
CHF using 4.02?) and 4.02B1 (Chronic heart failure) that when we 
evaluate a person's ability to perform activities of daily living, we 
will also consider the person's ability to perform them effectively; 
\6\ and providing more examples of skin examination findings that might 
accompany venous insufficiency. Two commenters asked us to base our 
proposals on the American College of Cardiology/American Heart 
Association (ACC/AHA) clinical practice guidelines and one suggested we 
review and incorporate the American Society of Echocardiography (ASE) 
guidelines. As a result, we tasked a committee of medical experts with 
reviewing and analyzing the ACC/AHA and ASE guidelines to inform the 
recommendations in the Institute of Medicine (IOM) \7\ report titled, 
``Cardiovascular Disability: Updating the Social Security Listings.'' 
\8\ This report, which is discussed in more detail below, informed some 
of the proposed changes in this NPRM.
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    \5\ 73 FR 20564 (2008).
    \6\ In current listing 4.02B1 (Chronic heart failure), we 
require persistent symptoms of heart failure ``which very seriously 
limit the person's ability to independently initiate, sustain or 
complete activities of daily living.'' Consistent with the 
commenter's suggestion and how we assess functional limitations in 
the adult mental disorders listings (12.00), in proposed 4.02B1, we 
require ``a very serious limitation in the ability to perform 
activities of daily living independently, appropriately, 
effectively, and on a sustained basis.''
    \7\ On April 28, 2015, the membership of the National Academy of 
Sciences voted to change the name of the IOM to the National Academy 
of Medicine. At that time, reports and studies of the IOM continued 
as activities of the Health and Medicine Division, a program unit 
operating under the direction of the National Academies of Sciences, 
Engineering, and Medicine.
    \8\ Institute of Medicine (IOM). (2010). Cardiovascular 
Disability: Updating the Social Security Listings. Washington, DC: 
The National Academies Press.
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    <bullet> On September 24 and 25, 2008, we hosted a policy 
conference titled ``Cardiovascular Disorders in the Disability 
Programs'' in Baltimore, Maryland. At this conference, we received 
public comments and suggestions from physicians and advocacy groups for 
updating and revising our criteria for evaluating cardiovascular 
disorders. Physicians and advocacy groups specifically discussed the 
evaluation of chronic heart failure, ischemic heart disease, peripheral 
artery disease, and chronic

[[Page 38839]]

venous insufficiency. Participants made several suggestions that we 
researched and incorporated into the proposals, such as distinguishing 
cardiovascular disorders from pulmonary disorders by using biomarkers 
such as B-type natriuretic peptide (BNP).
    <bullet> In 2009, we commissioned a study by an ad hoc committee of 
medical experts appointed by the Institute of Medicine (IOM). The 
committee: (1) conducted a comprehensive review of the relevant 
research literature and current professional practice guidelines 
developed jointly by the ACC/AHA; (2) assessed the current criteria in 
light of current research knowledge and evidence-based medical 
practice; and (3) produced a report with specific recommendations for 
revision of the criteria based on evidence and professional judgment. 
The committee provided its recommendations in a 2010 report titled, 
``Cardiovascular Disability: Updating the Social Security Listings.'' 
\9\ We recently sought guidance from our cardiologists and other 
medical experts, reviewed disability claims involving cardiovascular 
disorders, and reviewed current research to ensure the IOM 
recommendations are still relevant.
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    \9\ IOM. (2010).
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    Recommendations we received from the IOM report, responses to the 
ANPRM, and the ``Cardiovascular Disorders in the Disability Programs'' 
policy conference informed the proposed changes in this NPRM. As with 
the IOM report, we have conducted independent medical research, 
consulted with agency cardiologists, and reviewed disability claims 
involving cardiovascular disorders to ensure the accuracy and relevance 
of these stated resources. In developing this proposed rule, we also 
considered information from several other sources, including:
    <bullet> Medical experts in cardiology from SSA's Office of Medical 
Assistance \10\ who assist in the development and evaluation of policy 
and whom we regularly consulted with in drafting these proposals;
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    \10\ SSA's Office of Medical Assistance (OMA) provides medical 
and analytical support to ensure accurate and consistent disability 
policy and procedure application. In addition to a full complement 
of subject matter experts who are permanent SSA staff, OMA contracts 
with medical and psychological consultants to provide medical 
expertise in the development and evaluation of policy.
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    <bullet> Advocacy groups for people with cardiovascular disorders 
and individuals with cardiovascular disorders and their families who 
submitted comments on the ANPRM or participated in the 2008 
``Cardiovascular Disorders in the Disability Program'' policy 
conference;
    <bullet> Individuals who make and review disability determinations 
and decisions for us in State agencies; in our Office of Hearings 
Operations; and in our Office of Analytics, Review, and Oversight; and
    <bullet> The published sources of medical literature and research 
we list in the references section at the end of this preamble.

What revisions are we proposing for cardiovascular disorders?

    We propose to:
    <bullet> Change the name of the body system from ``Cardiovascular 
System'' to ``Cardiovascular Disorders'' to be consistent with the 
nomenclature of all body systems in our listings;
    <bullet> Reorganize and revise the introductory text (section 4.00 
for adults and 104.00 for children) to provide guidance for using the 
revised criteria in the listings;
    <bullet> Revise the adult and childhood listings for chronic heart 
failure (4.02 and 104.02), recurrent arrhythmias (4.05 and 104.05), 
symptomatic congenital heart disease (4.06) and congenital heart 
disease (104.06), and heart transplant (4.09 and 104.09);
    <bullet> Revise the adult listings for ischemic heart disease (IHD) 
(4.04), chronic venous insufficiency (4.11), and peripheral arterial 
disease (4.12);
    <bullet> Add adult listings for aortic valvular disease (4.07) and 
cardiomyopathy (4.08);
    <bullet> Add adult and childhood listings for cardiac allograft 
vasculopathy (4.16 and 104.16);
    <bullet> Remove childhood listing for rheumatic heart disease 
(104.13) and reserve listing number 104.13; and
    <bullet> Make minor editorial revisions, including changes to 
conform to revised rules for evaluating medical evidence,\11\ to the 
introductory text and to the listings for clarity.
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    \11\ 82 FR 5844 (2017).
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Proposed Changes to the Adult Cardiovascular Disorders Introductory 
Text

    The following table shows the heading of the current and proposed 
sections of the adult introductory text for cardiovascular disorders:

                         Introductory Text 4.00
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------------------------------------------------------------------------
Current Sections of the Adult            Proposed Sections of the Adult
 Introductory Text for Cardiovascular     Introductory Text for
 System.                                  Cardiovascular Disorders.
4.00 Cardiovascular System.............  4.00 Cardiovascular Disorders.
A. General.............................  A. How do we define
                                          cardiovascular disorders and
                                          cardiovascular terms?
B. Documenting Cardiovascular            B. What documentation do we
 Impairment.                              need to evaluate
                                          cardiovascular disorders?
C. Using Cardiovascular Test Results...  C. How do we use cardiovascular
                                          test results?
D. Evaluating Chronic Heart Failure....  D. How do we evaluate chronic
                                          heart failure?
E. Evaluating Ischemic Heart Disease...  E. How do we evaluate ischemic
                                          heart disease?
F. Evaluating Arrhythmias..............  F. How do we evaluate
                                          arrhythmias?
G. Evaluating Peripheral Vascular        G. How do we evaluate
 Disease.                                 peripheral vascular disease?
H. Evaluating Other Cardiovascular       H. How do we evaluate
 Impairments.                             congenital heart disease?
I. Other Evaluation Issues.............  I. How do we evaluate other
                                          cardiovascular disorders?
                                         J. How do we evaluate issues
                                          that affect the cardiovascular
                                          system?
                                         K. How do we evaluate
                                          cardiovascular disorders that
                                          do not meet one of these
                                          listings?
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[[Page 38840]]

Proposed 4.00--Introductory Text to the Adult Cardiovascular Disorders 
Listings

    The following is a detailed description of the primary changes we 
are proposing to the introductory text. In addition to the changes we 
describe below, we are proposing minor changes to the introductory text 
to clarify how we use the proposed listings to evaluate cardiovascular 
disorders, changes to be consistent with current medical terminology, 
the language we use in other body system listings, and the revised 
rules for evaluating medical evidence.\12\ We repeat much of the 
introductory text of proposed 4.00 in the introductory text of proposed 
104.00 (the introductory text to the childhood cardiovascular disorders 
listings), making distinctions where needed. This is necessary because 
the same basic criteria for evaluating cardiovascular disorders apply 
to both adults and children.
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    \12\ Id.
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Proposed 4.00A--How do we define cardiovascular disorders and 
cardiovascular terms?
    To improve clarity and promote consistent understanding of the 
terms we use in these listings, we propose:
    <bullet> In 4.00A3b (Persistent), to clarify that ``exceptions'' 
means brief periods when the required finding(s) is greatly reduced or 
gone. These periods are so brief or inconsequential, the required 
finding(s) remains a factor in the person's condition;
    <bullet> In 4.00A3c (Recurrent), to clarify in our definition of 
``recurrent'' that the term ``improvement of sufficient duration'' 
means the finding is greatly reduced (for example, treatment reduced a 
grade 3 chronic venous insufficiency (CVI) skin ulcer to a grade 1 CVI 
skin ulcer) or not present for long enough that the required finding(s) 
is no longer a factor in the person's condition.
    <bullet> In 4.00A3f (Uncontrolled) we would remove the definition 
for the term ``uncontrolled'' because we propose to eliminate the term 
as a descriptor for recurrent episodes of cardiac syncope. The 
definition of ``uncontrolled'' was redundant after describing 
reoccurring episodes of cardiac syncope despite treatment.
Proposed 4.00C--How do we use cardiovascular test results?
    We propose:
    <bullet> In 4.00C8d(iv) (When will we not purchase an exercise test 
or wait before we purchase an exercise test?), to include the procedure 
percutaneous coronary intervention (PCI) to be consistent with medical 
advancements that indicate a PCI is a nonsurgical procedure to improve 
blood flow to the heart.\13\
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    \13\ IOM. (2010), 109.
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    <bullet> In 4.00C15 (How do we evaluate cardiac catheterization 
evidence?), to remove 4.00C15b and 4.00C15c and create a new 4.00C15b 
to simplify our explanation of cardiac catheterization reports and to 
include information about fractional flow reserve (FFR), which we use 
in the proposed 4.04D1 (Ischemic heart disease).
Proposed 4.00D--How do we evaluate chronic heart failure?
    We propose:
    <bullet> In 4.00D1a (What is chronic heart failure (CHF)?), to 
provide a more descriptive definition of ``ejection fraction'' for 
clarity;
    <bullet> In 4.00D1b (What is chronic heart failure (CHF)?), to 
explain that high blood levels of the proteins B-type natriuretic 
peptide (BNP) and N-terminal pro-BNP (NT-pro-BNP) may help identify 
chronic heart failure as the cause of a person's symptoms (for example, 
shortness of breath);Sec.  <SUP>14 15 16 17</SUP>
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    \14\ Cohen-Solal, A., Laribi, S., Ishihara, S., Vergaro, G., 
Baudet, M., Logeart, D., . . . Seronde, M.-F. (2015). Prognostic 
markers of acute decompensated heart failure: The emerging roles of 
cardiac biomarkers and prognostic scores. Archives of Cardiovascular 
Disease, 108(1), 64-74. doi:10.1016/j.acvd.2014.10.002.
    \15\ Desai, A.S. (2013). Are serial BNP measurements useful in 
heart failure management? Serial natriuretic peptide measurements 
are not useful in heart failure management: The art of medicine 
remains long. Circulation, 127(4), 509-516. doi:10.1161/
CIRCULATIONAHA.112.120493.
    \16\ Patterson, C.C., Blankenberg, S., Ben-Shlomo, Y., Heslop, 
L., Bayer, A., Lowe, G., . . . Yarnell, J. (2015). Which biomarkers 
are predictive specifically for cardiovascular or for non-
cardiovascular mortality in men? Evidence from the Caerphilly 
Prospective Study (CaPS). International Journal of Cardiology, 201, 
113-118. doi:10.1016/j.ijcard.2015.07.106.
    \17\ Uszko-Lencer, N.H., Frankenstein, L., Spruit, M.A., Maeder, 
M.T., Gutmann, M., Muzzarelli, S., . . . Brunner-La Rocca, H.-P. 
(2017). Predicting hospitalization and mortality in patients with 
heart failure: The BARDICHE-index. International Journal of 
Cardiology, 227, 901-907. doi:10.1016/j.ijcard.2016.11.122.
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    <bullet> In 4.00D2a(i) (What evidence of CHF do we need?) and 
4.00D2a(iii) (What evidence of CHF do we need?), to explain left atrial 
volume index (LAVi) and how it is calculated. LAVi is a new measurement 
used in criterion A2 of proposed listing 4.02 (Chronic heart failure) 
to provide a more precise representation of increased left atrial 
pressure;
    <bullet> In 4.00D4c (How do we evaluate CHF using 4.02? ),to 
describe in more detail the two-part process we use in criteria B1 of 
proposed listing 4.02 (Chronic heart failure) to evaluate chronic heart 
failure if a person cannot perform an exercise tolerance test (ETT); 
\18\ and
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    \18\ We define an exercise tolerance test at 4.00C3b (What are 
exercise tests and what are they used for?) as ``a sign-or symptom-
limited test in which you exercise while connected to an ECG until 
you develop a sign or symptom that indicates that you have exercised 
as much as is considered safe for you.'' This is an existing 
definition coming from existing regulation.
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    <bullet> Add 4.00D4e (How do we evaluate CHF treated with a 
mechanical circulatory support device?) to explain mechanical 
circulatory support devices (MCSD) and clarify how we would evaluate 
individuals treated for heart failure with a MCSD.\19\ We propose to 
evaluate MCSDs under proposed 4.02D1 (Chronic heart failure )to account 
for cardiac bridge treatment which we currently evaluate under listing 
4.09 (Heart transplantation).
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    \19\ Ciarka, A., Edwards, L., Nilsson, J., Stehlik, J., & Lund, 
L.H. (2017). Trends in the use of mechanical circulatory support as 
a bridge to heart transplantation across different age groups. 
International Journal of Cardiology, 231, 225-227. doi:10.1016/
j.ijcard.2016.10.049.
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Proposed 4.00E--How do we evaluate ischemic heart disease?
    We propose:
    <bullet> In 4.00E9b (How do we evaluate IHD using 4.04?), to 
consolidate and revise the guidance that is currently in 4.00E9b, c, d, 
and e for ease of reference;
    <bullet> In 4.00E9b (How do we evaluate IHD using 4.04?), to 
explain that we will use an interpretation of electrocardiogram (ECG) 
findings by an acceptable medical source (AMS) \20\ if the 
interpretation concludes that the ECG findings are positive for IHD. 
Interpreting ECG results requires a systematic review and analysis of 
several components, including relevant clinical details and raw data. 
Relying on an interpretation by an AMS is consistent with our rules for 
establishing a medically determinable impairment (MDI) and will ensure 
the accuracy of adjudication for claims involving IHD because 
interpretation of ECG findings requires medical judgment; \21\
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    \20\ 20 CFR 404.1502(a) and 416.902(a).
    \21\ 20 CFR 404.1521 and 416.921.
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    <bullet> In 4.00E9c (How do we evaluate IHD using 4.04?), to 
clarify that revascularizations that result from unplanned 
hospitalizations must be an emergency and unplanned; \22\
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    \22\ As we explain in 4.00E9c, revascularization means 
angioplasty (with or without stent placement) or bypass surgery.
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    <bullet> To redesignate current 4.00E9f, g, and h (How do we 
evaluate IHD using 4.04?) as proposed new sections 4.00E9c, d, and f, 
respectively; and
    <bullet> In 4.00E9e ( How do we evaluate IHD using 4.04?), to add a 
definition for

[[Page 38841]]

the term ``fractional flow reserve (FFR),'' which is an objective 
measure of flow access across an obstruction that we use in criterion 
D1 of proposed listing 4.04 (Ischemic heart disease).\23\
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    \23\ Fearon, W.F. (2014). Percutaneous coronary intervention 
should be guided by fractional flow reserve measurement. 
Circulation, 129(18), 1860-1870. doi:10.1161/
CIRCULATIONAHA.113.004300.
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Proposed 4.00G--How do we evaluate peripheral vascular disease?
    In 4.00G6 (Are there any other studies that are helpful in 
evaluating PAD?), as IOM advised, we propose to provide more 
information about imaging and other tests used to diagnose peripheral 
arterial disease (PAD) to clearly convey our intent of the listing, 
which is to tie PAD to mobility.\24\
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    \24\ IOM. (2010), 151.
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Proposed 4.00H--How do we evaluate congenital heart disease?
    We propose:
    <bullet> To redesignate and rename some paragraphs for ease of 
reference;
    <bullet> In 4.00H (How do we evaluate congenital heart disease?), 
to add a definition for the term ``single ventricle,'' and include more 
detailed guidance on how we evaluate congenital heart disease as 
recommended by the IOM; and
    <bullet> To add 4.00H1 (What is congenital heart disease?), 4.00H2 
(What is Eisenmenger syndrome?), 4.00H3 (What is single ventricle?), 
and 4.004H4 (How do we evaluate conditions associated with congenital 
heart disease?).
Proposed 4.00I--How do we evaluate other cardiovascular disorders?
    We propose:
    <bullet> To rename and rearrange the content of 4.00I (How do we 
evaluate other cardiovascular disorders?), including redesignating some 
paragraphs, for ease of reference;
    <bullet> In 4.00I2 (What is cardiomyopathy and how will we evaluate 
it?), to explain how we would evaluate cardiomyopathy under proposed 
new 4.08 (Cardiomyopathy);
    <bullet> In 4.00I3 (How do we evaluate valvular heart disease?), to 
explain that we would evaluate valvular heart disease under the 
proposed new 4.07 (Aortic valvular disease);
    <bullet> In 4.00I4 (What do we consider when we evaluate heart 
transplant recipients?), to explain that we would evaluate cardiac 
allograft vasculopathy under proposed new 4.16 (Cardiac allograft 
vasculopathy); and
    <bullet> To add 4.00I5 (What is cardiac allograft vasculopathy and 
how do we evaluate it?), to explain proposed new 4.16 (Cardiac 
allograft vasculopathy).
Proposed 4.00J--How do we evaluate issues that affect the 
cardiovascular system?
    We propose:
    <bullet> To redesignate and rename some paragraphs for ease of 
reference;
    <bullet> In 4.00J1 (How do we consider the effects of obesity when 
we evaluate your cardiovascular disorder?), to simplify and refocus our 
discussion of how we consider the effects of obesity more specifically 
on cardiovascular disorders;
    <bullet> To add 4.00J3 (How do we consider hospitalizations?) to 
explain how we would evaluate hospitalizations for repeated 
exacerbations and complications of cardiovascular disorders under 
proposed 4.02B3 (Chronic heart failure), 4.04E (Ischemic heart 
disease), 4.06E (Congenital heart disease), and 4.08D (Cardiomyopathy).
    Proposed 4.00K--How do we evaluate cardiovascular disorders that do 
not meet one of these listings?
    <bullet> We propose to rename and redesignate 4.00I3 (How do we 
evaluate impairments that do not meet one of the cardiovascular 
listings?) as 4.00K and redesignate 4.00I3a and 4.00I3b as 4.00K1 and 
4.00K2 for ease of reference.

Proposed Changes to the Adult Cardiovascular Disorders Listings

    The following table shows the heading of the current and proposed 
sections of the adult listings for cardiovascular disorders:

                 Adult Cardiovascular Disorders Listings
------------------------------------------------------------------------
                Current                              Proposed
------------------------------------------------------------------------
4.02 Chronic heart failure.............  4.02 Chronic heart failure.
4.04 Ischemic heart disease............  4.03 [Reserved].
4.05 Recurrent arrhythmias.............  4.04 Ischemic heart disease.
4.06 Symptomatic congenital heart        4.05 Recurrent arrhythmias.
 disease.
4.09 Heart transplant..................  4.06 Congenital heart disease.
4.10 Aneurysm of aorta or major          4.07 Aortic valvular disease.
 branches.
4.11 Chronic venous insufficiency......  4.08 Cardiomyopathy.
4.12 Peripheral arterial disease.......  4.09 Heart transplantation.
                                         4.10 Dissecting aneurysm of the
                                          aorta or major branches.
                                         4.11 Chronic venous
                                          insufficiency.
                                         4.12 Peripheral arterial
                                          disease.
                                         4.13 [Reserved].
                                         4.14 [Reserved].
                                         4.15 [Reserved].
                                         4.16 Cardiac allograft
                                          vasculopathy.
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[[Page 38842]]

    The following table shows our proposed changes to the adult 
cardiovascular disorders listings criteria that involve changes to 
healthcare utilization and condition/episode requirements, the 
rationale for each change, and supporting resource. Following this 
table, we discuss all of our proposed changes to the adult 
cardiovascular disorders listings in more detail.

    Adult Cardiovascular Disorders Listings Criteria--Changes in Healthcare Utilization and Condition/Episode
                                                  Requirements
----------------------------------------------------------------------------------------------------------------
                                       Proposed listing
     Current listing criterion            criterion                 Rationale                  Resources
----------------------------------------------------------------------------------------------------------------
                                       Listing 4.02 Chronic heart failure
----------------------------------------------------------------------------------------------------------------
4.02 A1--A. Medically documented    A. Medically           Proposed criterion 4.02A1   IOM. (2010), 88, 89.
 presence of one of the following:   documented presence    requires an increased
1. Systolic failure (see             of one of the          left ventricular end
 4.00D1a(i)), with left              following:             diastolic dimension
 ventricular end diastolic          1. Systolic failure     (LVEDD) equal to or
 dimensions greater than 6.0 cm or   documented by          greater than 7.0
 ejection fraction of 30 percent     appropriate            centimeters (cm) instead
 or less during a period of          medically acceptable   of the current criterion
 stability (not during an episode    imaging during a       of an LVEDD greater than
 of acute heart failure); or.        period of stability    6.0 cm. We followed the
                                     (not during an         IOM's recommendation in
                                     episode of             determining that an
                                     exacerbation of        increased LVEDD of
                                     heart failure), with   greater than 6.0 cm but
                                     left ventricular end   less than 7.0 cm
                                     diastolic dimension    indicates only a
                                     equal to or greater    moderately enlarged
                                     than 7.0 cm; or        heart, and an increased
                                     ejection fraction of   LVEDD of at least 7.0 cm
                                     30 percent or less     more clearly establishes
                                     during a period of     an enlarged heart with
                                     stability (not         signs and symptoms
                                     during an episode of   indicating listing-level
                                     acute heart failure).  heart failure.
4.02B2--Three or more separate      B.3. Exacerbations or  We propose to remove        IOM. (2010), 89, 91.
 episodes of acute congestive        complications of       current 4.02B2 ``three or
 heart failure within a              chronic heart          more separate episodes of
 consecutive 12-month period (see    failure (see           acute congestive heart
 4.00A3e), with evidence of fluid    4.00D1b) requiring     failure'' because we
 retention (see 4.00D2b(ii)) from    three                  would evaluate these
 clinical and imaging assessments    hospitalizations       episodes under proposed
 at the time of the episodes,        within a consecutive   4.02B3. As recommended by
 requiring acute extended            12-month period (see   the IOM, proposed 4.02B3
 physician intervention such as      4.00A3e) and at        would evaluate
 hospitalization or emergency room   least 30 days apart.   exacerbations or
 treatment for 12 hours or more,     Each hospitalization   complications of CHF,
 separated by periods of             must last at least     requiring three
 stabilization (see 4.00D4c);        48 hours, including    hospitalizations within a
                                     hours in a hospital    consecutive 12-month
                                     emergency department   period and at least 30
                                     immediately before     days apart. An impairment
                                     the hospitalization    resulting in
                                     (see 4.00J3).          exacerbations or
                                                            complications that
                                                            require this many
                                                            hospitalizations in 12
                                                            months is a very severe
                                                            impairment. We would
                                                            require these
                                                            hospitalizations to be at
                                                            least 30 days apart to
                                                            ensure we are evaluating
                                                            separate episodes of
                                                            exacerbations or
                                                            complications.
No current listing criteria.......  C. Heart failure with  The IOM recommended a       IOM. (2010), 84, 89.
                                     left ventricular       criterion for chronic      Desai, R.V., Guichard,
                                     ejection fraction of   heart failure with an EF    J.L., Mujib, M., Ahmed,
                                     20 percent or less     on a sustained basis of     M.I., Feller, M.A.,
                                     while on a regimen     20 percent or less. An EF   Fonarow, G.C., . . .
                                     of prescribed          of only 20 percent means    Ahmed, A. (2013).
                                     therapy, on two        the heart's pumping         Reduced right
                                     evaluations at least   action is less than a       ventricular ejection
                                     90 days apart within   third of normal, and        fraction and increased
                                     a consecutive 12-      critically affects a        mortality in chronic
                                     month period (see      person's ability to         systolic heart failure
                                     4.00A3e) during a      perform gainful activity.   patients receiving beta-
                                     period of stability                                blockers: Insights from
                                     (not during an                                     the BEST trial.
                                     episode of                                         International Journal of
                                     exacerbation of                                    Cardiology, 163(1), 61-
                                     heart failure);                                    67. doi:10.1016/
                                                                                        j.ijcard.2011.05.051.
                                                                                       Runge, M.S., Patterson,
                                                                                        C., Stouffer, G.A., &
                                                                                        Netter, F.H. (2010).
                                                                                        Netter's Cardiology (2nd
                                                                                        ed.). Philadelphia, PA:
                                                                                        Saunders Elsevier.

[[Page 38843]]

 
No current listing criteria.......  D. One of the          Implanted MCSDs help the    Malotte, K., Saguros, A.,
                                     following while        heart pump blood and may    & Groninger, H. (2018).
                                     hospitalized, at       be used as a ``bridge''     Continuous cardiac
                                     home, or both:         while a person waits for    inotropes in patients
                                    1. Mechanical           a heart transplant. We      with end-stage heart
                                     circulatory support    currently use our medical   failure: An evolving
                                     device except          equivalence rules to find   experience. Journal of
                                     extracorporeal         someone with heart          Pain Symptom Management,
                                     membrane oxygenation   failure and an implanted    55(1), 159-163.
                                     (ECMO) (see            MCSD disabled under         doi:10.1016/
                                     4.00D4e). Consider     listing 4.09 (Heart         j.jpainsymman.2017.09.02
                                     under a disability     transplantation). Adding    6.
                                     for 1 year from the    this new criterion will    Bistola, V., Arfaras-
                                     date of                ensure that people with     Melainis, A.,
                                     implantation; after    heart failure treated       Polyzogopoulou, E.,
                                     that, evaluate any     with MCSD are               Ikonomidis, I., &
                                     residual               consistently identified.    Parissis, J. (2019).
                                     impairment(s) under   People who require           Inotropes in acute heart
                                     the criteria for the   continuous intravenous      failure: From guidelines
                                     affected body system.  administration of           to practical use:
                                    2. Continuous           inotropic medication (for   Therapeutic options and
                                     intravenous            example, milrinone) have    clinical practice.
                                     administration of      very serious heart          Cardiac Failure Review,
                                     inotropic medication   failure, and the length     5(3), 133-139.
                                     (for example,          of this treatment can be    doi:10.15420/
                                     milrinone) for at      an accurate predictor of    cfr.2019.11.2.
                                     least 30 consecutive   impairment severity.
                                     days. Consider under   Accordingly, proposed
                                     a disability for 1     4.02D2 would find people
                                     year from the date     disabled if they require
                                     of initiation of the   continuous intravenous
                                     treatment; after       inotropic medications for
                                     that, evaluate any     30 or more consecutive
                                     residual               days.
                                     impairment(s) under
                                     the criteria for the
                                     affected body system.
----------------------------------------------------------------------------------------------------------------
                                       Listing 4.04 Ischemic heart disease
----------------------------------------------------------------------------------------------------------------
4.04B--Three separate ischemic      4.04C--Documentation   We would evaluate           Hua, M., Gong, M.N.,
 episodes, each requiring            of three separate      unplanned                   Brady, J., & Wunsch, H.
 revascularization or not amenable   ischemic episodes      hospitalizations under      (2015). Early and late
 to revascularization (see           (see 4.00E9c)          this section to ensure we   unplanned
 4.00E9f), within a consecutive 12-  requiring unplanned    are only evaluating         rehospitalizations for
 month period (see 4.00A3e).         hospitalization        urgent ischemic episodes.   survivors of critical
                                     (inpatient or          Individuals who have        illness. Critical Care
                                     observation status)    ischemic episodes that      Medicine, 43(2), 430-
                                     within a consecutive   result in unplanned         438. doi:10.1097/
                                     12-month period (see   hospitalizations may need   CCM.0000000000000717.
                                     4.00A3e).              intensive care, can have
                                                            long hospital stays, may
                                                            require multiple
                                                            procedures, and can be at
                                                            high risk for post-
                                                            discharge morbidity and
                                                            mortality.
No current criterion for repeated   4.04E--Exacerbations   An impairment resulting in  Hua, M., Gong, M.N.,
 exacerbations or complications      or complications of    exacerbations or            Brady, J., & Wunsch, H.
 ischemic heart disease, 4.04B       ischemic heart         complications that          (2015). Early and late
 (above) only considers episodes     disease (see 4.00E2-   require three or more       unplanned
 requiring revascularization or      4.00E7) requiring      hospitalizations in 12      rehospitalizations for
 that are not amenable to            three                  months is a very severe     survivors of critical
 revascularization.                  hospitalizations       impairment. We would        illness. Critical Care
                                     within a consecutive   require these               Medicine, 43(2), 430-
                                     12-month period (see   hospitalizations to be at   438. doi:10.1097/
                                     4.00A3e) and at        least 30 days apart to      CCM.0000000000000717.
                                     least 30 days apart.   ensure we are evaluating
                                     Each hospitalization   separate episodes of
                                     must last at least     exacerbations or
                                     48 hours, including    complications of ischemic
                                     hours in a hospital    heart disease.
                                     emergency department
                                     immediately before
                                     the hospitalization
                                     (see 4.00E9c).

[[Page 38844]]

 
                                      Listing 4.06 Congenital heart disease
----------------------------------------------------------------------------------------------------------------
4.06A--A. Cyanosis at rest, and:    A. Chronic hypoxemia,  We propose to revise        Stout, K.K., Daniels,
1. Hematocrit of 55 percent or       and 1, 2, or 3:        current 4.06A to require    C.J., Aboulhosn, J.A.,
 greater; or.                       1. Hematocrit of 55     ``hypoxemia'' rather than   Bozkurt, B., Broberg,
2. Arterial O2 saturation of less    percent or greater     ``cyanosis or               C.S., Colman, J.M., . .
 than 90 percent in room air or      on two evaluations     acyanosis''. Cyanosis is    . Van Hare, G.F. (2019).
 resting arterial PO2 of 60 Torr     at least 90 days       a more subjective           2018 AHA/ACC Guideline
 or less.                            apart within a         assessment subject to       for the management of
                                     consecutive 12-month   misinterpretation due to    adults with congenital
                                     period (see            by many factors,            heart disease: A report
                                     4.00A3e); or.          including skin              of the American College
                                    2. Arterial blood gas   complexion. Thus, the       of Cardiology/American
                                     test measurement       term ``hypoxemia''          Heart Association Task
                                     obtained at rest       relates more to the         Force on Clinical
                                     while breathing room   laboratory and pulse        Practice Guidelines.
                                     air, as described in   oximetry findings than      Journal of the American
                                     either a or b:.        the term ``cyanosis.        College of Cardiology,
                                    a. SaO2 (arterial      We would require two         73(12), e81-e192.
                                     oxygen saturation)     hematocrit measurements     doi:10.1016/
                                     less than or equal     instead of the current      j.jacc.2018.08.1029.
                                     to 89 percent; or.     listing's single           Stack, S.W, . . . &
                                    b. PO2 or PaO2          measurement. Two            Berger, S.A. (2009). The
                                     (partial pressure of   measurements, at least 90   effects of high
                                     oxygen) less than or   days apart within a         hematocrit arterial
                                     equal to 60 mmHg;.     consecutive 12-month        flow--A phenomenological
                                    3. SpO2 (percentage     period will help ensure     study of health risk
                                     of oxygen saturation   the person's hematocrit     implications. Chemical
                                     of blood hemoglobin)   level is associated with    Engineering Science,
                                     measured by pulse      chronic hypoxemia and not   64(22), 4701-4706.
                                     oximetry either at     the result of a             doi:10/1016/
                                     rest, during a 6-      reversible condition.       j.ces.2009.07.017.
                                     minute walk test      Proposed 4.06A3 would       IOM. (2010), 178.
                                     (6MWT), or after a     require three SpO2         Oster, M.E, . . . &
                                     6MWT, while            measurements 30 days        Kochilas, L.K. (2016).
                                     breathing room air,    apart within a              Screening for critical
                                     less than or equal     consecutive 12-month        congenital heart
                                     to 87 percent on       period showing hypoxemia.   disease. Clinics in
                                     three evaluations at   This will document that     Perinatology, 43(1), 73-
                                     least 30 days apart    the condition is chronic    80. doi:10.1016/
                                     within a consecutive   and persistent, and the     j.clp.2015.11.005.
                                     12-month period (see   measurements are not       Mechem, C.C. (2014).
                                     4.00A3e).              related to a reversible     Pulse oximetry. In P.E.
                                                            condition or an             Parsons (Ed.), UpToDate
                                                            inaccurate reading.         (Jan. 2014). Retrieved
                                                           We would add a criterion     from https://
                                                            for SpO2 (percentage of     www.uptodate.com/
                                                            oxygen saturation of        contents/pulse-oximetry.
                                                            blood hemoglobin)
                                                            measured by pulse
                                                            oximetry, including
                                                            measurements taken while
                                                            the person is at rest or
                                                            while doing a six-minute
                                                            walk test (6MWT). Pulse
                                                            oximetry measurements are
                                                            a non-invasive
                                                            alternative to invasive
                                                            testing. A person's
                                                            medical evidence often
                                                            provides SpO2 findings,
                                                            and SpO2 measured by
                                                            pulse oximetry reflects
                                                            an advance in medical
                                                            technology that provides
                                                            another way to establish
                                                            listing-level severity.
No current criteria...............  4.06E--Exacerbations   An impairment resulting in  Hua, M., Gong, M.N.,
                                     or complications of    exacerbations or            Brady, J., & Wunsch, H.
                                     congenital heart       complications that          (2015). Early and late
                                     disease (see 4.00J3)   require three or more       unplanned
                                     requiring three        hospitalizations in 12      rehospitalizations for
                                     hospitalizations       months is a very severe     survivors of critical
                                     within a consecutive   impairment. We would        illness. Critical Care
                                     12-month period (see   require these               Medicine, 43(2), 430-
                                     4.00A3e) and at        hospitalizations to be at   438. doi:10.1097/
                                     least 30 days apart.   least 30 days apart to      CCM.0000000000000717.
                                     Each hospitalization   ensure we are evaluating
                                     must last at least     separate episodes of
                                     48 hours, including    exacerbations or
                                     hours in a hospital    complications.
                                     emergency department
                                     immediately before
                                     the hospitalization
                                     (see 4.00J3).
----------------------------------------------------------------------------------------------------------------
                                           Listing 4.08 Cardiomyopathy
----------------------------------------------------------------------------------------------------------------
No current listing................  4.08D--D.              Consistent with IOM         IOM. (2010), 80.
                                     Exacerbations or       recommendations, we        In addition, SSA has
                                     complications of       created this new            designated
                                     cardiomyopathy         cardiomyopathy listing to   endomyocardial fibrosis
                                     requiring three        specifically address        and cardiac amyloidosis
                                     hospitalizations       hypertrophic                AL type as Compassionate
                                     within a consecutive   cardiomyopathy,             Allowance (CAL)
                                     12-month period (see   endomyocardial fibrosis,    conditions. See
                                     4.00A3e) and at        and cardiac amyloidosis     Compassionate Allowances
                                     least 30 days apart.   AL type, which are more     Website Home Page
                                     Each hospitalization   serious types of            (ssa.gov).
                                     must last at least     cardiomyopathy.
                                     48 hours, including
                                     hours in a hospital
                                     emergency department
                                     immediately before
                                     the hospitalization
                                     (see 4.00J3).

[[Page 38845]]

 
                                    Listing 4.11 Chronic venous insufficiency
----------------------------------------------------------------------------------------------------------------
4.11--Chronic venous insufficiency  4.11--Chronic venous   As recommended by IOM, we   IOM. (2010), 161.
 of a lower extremity with           insufficiency (see     would require
 incompetency or obstruction of      4.00G) of a lower      confirmation of CVI by
 the deep venous system and one of   extremity with         duplex ultrasound or
 the following:                      reflux or              other appropriate
                                     obstruction of the     diagnostic technique. The
                                     venous system          medical community
                                     documented by duplex   considers the use of
                                     ultrasound or other    duplex ultrasound to be
                                     appropriate            the best method for
                                     diagnostic             detecting reflux or
                                     technique, with A or   obstruction.
                                     B:
4.11A--A. Extensive brawny edema    A. Extensive trophic   We would adopt IOM          IOM. (2010), 157-161.
 (see 4.00G3) involving at least     changes of skin (for   recommendations and
 two-thirds of the leg between the   example,               broaden the listing
 ankle and knee or the distal one-   hyperpigmentation,     criteria we apply to
 third of the lower extremity        lipodermatosclerosis   trophic changes of the
 between the ankle and hip.          , brawny edema)        skin. For example, in
                                     involving at least     addition to brawny edema,
                                     two-thirds of the      trophic changes evaluated
                                     leg below the knee,    under the proposed
                                     on two evaluations     listing would include
                                     at least 90 days       hyperpigmentation and
                                     apart within a         lipodermatosclerosis
                                     consecutive 12-month  We would revise the
                                     period (see            current requirement that
                                     4.00A3e), with both    these skin changes
                                     1 and 2:               involve ``at least two-
                                                            thirds of the leg between
                                                            the ankle and knee or the
                                                            distal one-third of the
                                                            lower extremity between
                                                            the ankle and hip.''
                                                            Instead, we would require
                                                            extensive skin changes
                                                            involving at least two-
                                                            thirds of the leg below
                                                            the knee, to make the
                                                            requirement simpler to
                                                            understand and apply.
                                                            This revision is
                                                            consistent with IOM's
                                                            recommendation to require
                                                            skin changes below the
                                                            knee.
                                                           We would require the skin
                                                            changes under proposed
                                                            4.11A to be consistent
                                                            with CVI, and we would
                                                            document the skin changes
                                                            over a period of at least
                                                            90 days to ensure they
                                                            are chronic.
4.11B--Superficial varicosities,    4.11B--Two or more     This requirement is more    IOM. (2010), 161.
 stasis dermatitis, and either       episodes of            conclusive than the
 recurrent ulceration or             ulceration that has    current requirement of 3
 persistent ulceration that has      not healed following   months of unsuccessful
 not healed following at least 3     at least 6 months of   prescribed treatment as
 months of prescribed treatment.     prescribed treatment.  it demonstrates the
                                                            condition has persisted
                                                            despite treatment for a
                                                            longer period of time.
                                                            The CVI must be
                                                            unresponsive to
                                                            compression therapy,
                                                            because this therapy
                                                            usually enables people to
                                                            return to a good level of
                                                            functioning.
----------------------------------------------------------------------------------------------------------------

Proposed Listing 4.02--Chronic Heart Failure

    We propose to revise the listing criteria for chronic heart failure 
(CHF) in 4.02A and 4.02B and add new listing criteria 4.02C and 4.02D. 
Proposed listing-level severity for CHF would be met when the person's 
CHF satisfies the criteria in 4.02A and 4.02B. Listing-level severity 
for CHF would also be met when the person's CHF satisfies either 
proposed 4.02C or 4.02D.
    Proposed criterion 4.02A1 requires an increased left ventricular 
end diastolic dimension (LVEDD) equal to or greater than 7.0 
centimeters (cm) instead of the current criterion of an LVEDD greater 
than 6.0 cm, because an LVEDD less than 5.6 cm is normal. We followed 
the IOM's recommendation in determining that an increased LVEDD of 
greater than 6.0 cm but less than 7.0 cm indicates only a moderately 
enlarged heart, and an increased LVEDD of at least 7.0 cm more clearly 
establishes an enlarged heart with signs and symptoms indicating 
listing-level heart failure and is comparable to an ejection fraction 
(EF) of 30 percent or less.\25\
---------------------------------------------------------------------------

    \25\ IOM. (2010), 88, 89.
---------------------------------------------------------------------------

    In proposed 4.02A2, we would consider an elevated left atrial 
volume index (LAVi) measurement. An LAVi measurement provides a precise 
representation of increased left atrial pressure, making it a more 
accurate indicator of heart failure than considering left atrium size 
alone.\26\
---------------------------------------------------------------------------

    \26\ Cacciapuoti, Fu., Scognamiglio, A., Paoli, V.D., Romano, 
C., & Cacciapuoti, Fe. (2012). Left atrial volume index as indicator 
of left ventricular diastolic dysfunction: Comparation between left 
atrial volume index and tissue myocardial performance index. Journal 
of Cardiovascular Ultrasound, 20(1), 25-29. doi:10.4250/
jcu.2012.20.1.25.
---------------------------------------------------------------------------

    To establish listing-level severity for CHF, in addition to 
satisfying the criteria in proposed 4.02A, a person's CHF must satisfy 
the criteria in proposed 4.02B. The criteria in proposed 4.02B are 
similar to the criteria in current 4.02B1 and 4.02B3, respectively, 
with some important changes. For proposed 4.02B1a, we can use a 
conclusion by a medical source that an exercise tolerance test (ETT) 
presents a significant risk to a person; for example, the person's 
cardiologist stating that an ETT would cause cardiac instability or 
injury. For proposed 4.02B1b, we would require findings showing that a 
person is very seriously limited in his or her ability to perform an 
ETT, similar to current 4.02B3. Consistent with the IOM's 
recommendations, proposed 4.02B2 would also include findings showing 
the inability to perform on an ETT at 15 milliliters/kilograms/minute 
(ml/kg/min) peak VO<INF>2</INF> (oxygen consumption).\27\ Peak 
VO<INF>2</INF> at this level is comparable to the requirement in 
current 4.02B3 for an inability to perform on an ETT at a workload 
equivalent to 5 metabolic equivalents (METs) of task.
---------------------------------------------------------------------------

    \27\ IOM. (2010), 85, 93.
---------------------------------------------------------------------------

    We propose to remove current 4.02B2 ``three or more separate 
episodes of acute congestive heart failure'' because we would evaluate 
these episodes under proposed 4.02B3. As recommended by the IOM, 
proposed 4.02B3 would evaluate exacerbations or complications

[[Page 38846]]

of CHF, requiring three hospitalizations within a consecutive 12-month 
period and at least 30 days apart.\28\
---------------------------------------------------------------------------

    \28\ IOM. (2010), 89, 91.
---------------------------------------------------------------------------

    Additionally, proposed 4.02B2 would not include the requirement in 
current 4.02B3b of frequent premature ventricular contractions (PVCs). 
Frequent PVCs do not necessarily reflect an inability to perform an 
ETT.<SUP>29 30 31</SUP> The proposed listing also would no longer 
include the criterion in current 4.02B3d requiring signs attributable 
to inadequate cerebral perfusion, such as ataxic gait or mental 
confusion. Such manifestations rarely occur during an ETT, even if the 
person has very serious CHF.\32\
---------------------------------------------------------------------------

    \29\ Cha, Y.-M., Lee, G.K., Klarich, K.W., & Grogan, M. (2012). 
Premature ventricular contraction-induced cardiomyopathy: A 
treatable condition. Circulation: Arrhythmia and Electrophysiology, 
5(1), 229-236. doi:10.1161/CIRCEP.111.963348.
    \30\ Dukes, J.W., Dewland, T.A., Vittinghoff, E., Mandyam, M.C., 
Heckbert, S.R., Siscovick, D.S., . . . Marcus, G.M. (2015). 
Ventricular ectopy as a predictor of heart failure and death. 
Journal of the American College of Cardiology, 66(2), 101-109. 
doi:10.1016/j.jacc.2015.04.062.
    \31\ IOM. (2010), 87.
    \32\ IOM. (2010), 87, 88.
---------------------------------------------------------------------------

    The IOM recommended a criterion for chronic heart failure in people 
who are stable and receiving treatment but have an EF on a sustained 
basis of 20 percent or less. An EF of only 20 percent means the heart's 
pumping action is less than a third of normal, and critically affects a 
person's ability to perform gainful activity.<SUP>33 34 35</SUP> Most 
individuals with disease this advanced have greater risk of mortality 
and major functional limitations, such as shortness of breath or 
fatigue, even during mild exertion.\36\ We propose 4.02C consistent 
with the IOM's recommendation. We would require at least two EF 
measurements equal to or less than 20 percent at least 90 days apart 
within a consecutive 12-month period to document chronic disease and to 
exclude heart failure resulting from reversible causes.
---------------------------------------------------------------------------

    \33\ Desai, R.V., Guichard, J.L., Mujib, M., Ahmed, M.I., 
Feller, M.A., Fonarow, G.C., . . . Ahmed, A. (2013). Reduced right 
ventricular ejection fraction and increased mortality in chronic 
systolic heart failure patients receiving beta-blockers: Insights 
from the BEST trial. International Journal of Cardiology, 163(1), 
61-67. doi:10.1016/j.ijcard.2011.05.051.
    \34\ IOM. (2010), 84, 89.
    \35\ Runge, M.S., Patterson, C., Stouffer, G.A., & Netter, F.H. 
(2010). Netter's Cardiology (2nd ed.). Philadelphia, PA: Saunders 
Elsevier.
    \36\ IOM. (2010), 78, 81, 82, 94.
---------------------------------------------------------------------------

    Under proposed 4.02D1, we would include a new criterion for heart 
failure treated with a mechanical circulatory support device (MCSD). 
Implanted MCSDs, such as a left ventricle assistive device (LVAD) or a 
right ventricle assistive device (RVAD), help the heart pump blood and 
may be used as a ``bridge'' while a person waits for a heart 
transplant. We currently use our medical equivalence rules \37\ to find 
someone with heart failure and an implanted MCSD disabled under listing 
4.09 (Heart transplantation). Adding this new criterion will ensure 
that people with heart failure treated with MCSD are consistently 
identified.
---------------------------------------------------------------------------

    \37\ 20 CFR 404.1526 and 416.926.
---------------------------------------------------------------------------

    People who require continuous intravenous administration of 
inotropic medication (for example, milrinone) have very serious heart 
failure, and the length of this treatment can be an accurate predictor 
of impairment severity.<SUP>38 39</SUP> Accordingly, proposed 4.02D2 
would find people disabled if they require continuous intravenous 
inotropic medications for 30 or more consecutive days.
---------------------------------------------------------------------------

    \38\ Malotte, K., Saguros, A., & Groninger, H. (2018). 
Continuous cardiac inotropes in patients with end-stage heart 
failure: An evolving experience. Journal of Pain Symptom Management, 
55(1), 159-163. doi:10.1016/j.jpainsymman.2017.09.026.
    \39\ Bistola, V., Arfaras-Melainis, A., Polyzogopoulou, E., 
Ikonomidis, I., & Parissis, J. (2019). Inotropes in acute heart 
failure: From guidelines to practical use: Therapeutic options and 
clinical practice. Cardiac Failure Review, 5(3), 133-139. 
doi:10.15420/cfr.2019.11.2.
---------------------------------------------------------------------------

Proposed Listing 4.04--Ischemic Heart Disease

    As noted earlier in this preamble, we propose to use reports from 
AMSs under proposed 4.04A to determine whether ECG findings are 
positive for IHD. We would also rely on such reports to determine 
whether systolic blood pressure measurements during ETTs are positive 
for IHD. Based on our program experience and consultation with agency 
medical experts, we expect that relying on these reports from AMSs will 
ensure the accuracy of disability claims adjudication.
    We would replace current 4.04A4 with proposed 4.04A. Proposed 4.04B 
adds the requirement to consider imaging results derived from 
pharmacologic stress testing. The new proposed requirement will provide 
more specific findings than the current criterion, which generally 
requires only ``documented ischemia.'' \40\ Because of these changes, 
we would redesignate current 4.04B as proposed 4.04C and revise the 
introductory text accordingly.
---------------------------------------------------------------------------

    \40\ IOM. (2010), 120.
---------------------------------------------------------------------------

    Proposed 4.04C (current 4.04B) would require three separate 
ischemic episodes that result in unplanned hospitalizations within a 
consecutive 12-month period (see 4.00A3e (What do the following terms 
or phrases mean in these listings?), including episodes requiring 
unplanned revascularization or treatment for myocardial infarction 
(heart attack), unstable angina, or an irregular heartbeat. We would 
evaluate unplanned hospitalizations under this section to ensure we are 
only evaluating urgent ischemic episodes. Individuals who have ischemic 
episodes that result in unplanned hospitalizations may need intensive 
care, can have long hospital stays, may require multiple procedures, 
and can be at high risk for post-discharge morbidity and 
mortality.<SUP>41 42 43 44</SUP> Many also have serious co-occurring 
medical conditions (for example, heart failure, chronic kidney disease, 
and chronic obstructive pulmonary disease).<SUP>45 46 47</SUP>
---------------------------------------------------------------------------

    \41\ Hua, M., Gong, M.N., Brady, J., & Wunsch, H. (2015). Early 
and late unplanned rehospitalizations for survivors of critical 
illness. Critical Care Medicine, 43(2), 430-438. doi:10.1097/
CCM.0000000000000717.
    \42\ Kim, Y., Gani, F., Canner, J.K., Margonis G.A., Makary, 
M.A., Schneider, E.B., & Pawlik, T.M. (2016). Hospital readmission 
after multiple major operative procedures among patients with 
employer provided health insurance. Surgery, 160(1), 178-190. 
doi:10.1016/j.surg.2016.01.025.
    \43\ Reynolds, K., Butler, M.G., Kimes, T.M., Rosales, A.G., 
Chan, W., & Nichols, G.A. (2015). Relation of acute heart failure 
hospital length of stay to subsequent readmission and all-cause 
mortality. American Journal of Cardiology, 116(3), 400-405. 
doi:10.1016/j.amjcard.2015.04.052.
    \44\ Yu, P.-J., Cassiere, H.A., Fishbein, J., Esposito, R.A., & 
Hartman, A.R. (2016). Outcomes of patients with prolonged intensive 
care unit length of stay after cardiac surgery. Journal of 
Cardiothoracic and Vascular Anesthesia, 30(6), 1550-1554. 
doi:10.1053/j.jvca.2016.03.145.
    \45\ Frigola-Capell, E., Comin-Colet, J., Davins-Miralles, J., 
Gich-Saladich, I., Wensing, M., & Verd[uacute]-Rotellar, J.M. 
(2012). Trends and predictors of hospitalization, readmissions and 
length of stay in ambulatory patients with heart failure. Revista 
Clinica Espanola, 213(1), 1-7. doi:10.1016/j.rce.2012.10.006.
    \46\ Nombela-Franco, L., del Trigo, M., Morrison-Polo, G., 
Veiga, G., Jimenez-Quevedo, P., Altisent, O.A.-J., . . . 
Rod[eacute]s-Cabau, J. (2015). Incidence, causes, and predictors of 
early (<30 days) and late unplanned hospital readmissions after 
transcatheter aortic valve replacement. Journal of the American 
College of Cardiology: Cardiovascular Interventions, 8(13), 1748-
1757. doi:10.1016/j.jcin.2015.07.022.
    \47\ Versteeg, H., Hoogwegt, M.T., Hansen, T.B., Pedersen, S.S., 
Zwisler, A.-D., & Thygesen, L.C. (2013). Depression, not anxiety, is 
independently associated with 5-year hospitalizations and mortality 
in patients with ischemic heart disease. Journal of Psychosomatic 
Research, 75(6), 518-525. doi:10.1016/j.jpsychores.2013.10.005.
---------------------------------------------------------------------------

    We would redesignate current 4.04C as proposed 4.04D. We would 
reorganize the criteria in current 4.04C and follow IOM's 
recommendation to add new criteria to evaluate the severity of a 
person's IHD regardless of whether he or she has had a timely ETT or 
pharmacologic stress test or whether such tests are contraindicated. 
ETTs and pharmacologic stress test are commonly performed for 
diagnostic and prognostic purposes, and applicable to determine

[[Page 38847]]

functional capacity in persons with IHD.\48\
---------------------------------------------------------------------------

    \48\ IOM. (2010), 70.
---------------------------------------------------------------------------

    We would create proposed new 4.04D1 based upon blood flow in the 
coronary arteries expressed as fractional flow reserve (FFR). Updated 
medical science has shown FFR is a more objective and medically updated 
measure of the severity of stenosis.\49\ A clinician may measure FFR in 
a stenotic (obstructed) artery to determine whether it requires 
revascularization. A normal (patent) artery has an FFR equal to 1.0. If 
a stenotic artery has an FFR equal to or less than 0.80, and the artery 
is amenable to revascularization, the clinician will use 
revascularization to restore the vessel, because the obstructed artery 
is causing significant ischemia. Accordingly, an FFR measurement less 
than or equal to 0.80 in the proximal or mid segment of an artery that 
is not amenable to revascularization is consistent with the 
requirements of proposed 4.04D1.<SUP>50 51 52</SUP>
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    \49\ Shlofmitz, E., and Jeremias, A. (2017). FFR in 2017: 
Current Status in PCI Management--American College of Cardiology. 
Available online at: https://www.acc.org/latest-in-cardiology/articles/2017/05/25/08/34/ffr-in-2017-current-status-in-pci-management (accessed September 17, 2021).
    \50\ Fearon, W.F. (2014). Percutaneous coronary intervention 
should be guided by fractional flow reserve measurement. 
Circulation, 129(18), 1860-1870. doi:10.1161/
CIRCULATIONAHA.113.004300.
    \51\ Fearon, W.F., De Bruyne, B., & Pijlis, N.H. (2016). 
Fractional flow reserve in acute coronary syndromes. Journal of the 
American College of Cardiology, 68(11), 1192-1194. doi:10.1016/
j.jacc.2016.07.713.
    \52\ Tebaldi, M., Biscaglia, S., Pecoraro, A., Fineschi, M., & 
Campo, G. (2016). Fractional flow reserve implementation in daily 
clinical practice: A European survey. International Journal of 
Cardiology, 207, 206-207. doi:10.1016/j.ijcard.2016.01.097.
---------------------------------------------------------------------------

    In proposed 4.04D2 we would evaluate IHD by taking into 
consideration a person's history of coronary artery bypass graft 
surgery. In proposed 4.04D3, we would evaluate IHD by taking into 
consideration that a person has a decreased EF (i.e., EF of less than 
50 percent while medically stable and on a regimen of prescribed 
treatment).
    In both proposed 4.04D2 and 4.04D3, we would require the person to 
have symptoms of myocardial ischemia, as described in current 4.00E3 
through 4.00E7 and be on a regimen of prescribed treatment. Proposed 
4.04D2 and 4.04D3 would include the criteria in current 4.04C1a, b, and 
d with minor editorial changes. Proposed 4.04D2 includes criteria in 
current 4.04C1e. We would not include the criteria in current 4.04C1c 
in the proposed listings 4.04D2 and 4.04D3 because based on our program 
experience, we determined that these criteria do not consistently 
correlate with listing-level severity.
    We would remove current 4.04C2 with its requirement that a person's 
IHD result in very serious limitations in his or her ability to 
independently initiate, sustain, or complete activities of daily 
living. The criteria in proposed listings 4.04D1, 4.04D2, and 4.04D3 
alone provide a description of listing-level IHD.
    We are proposing new 4.04E to evaluate exacerbations or 
complications of IHD requiring three hospitalizations within a 
consecutive 12-month period and at least 30 days apart. These 
hospitalizations may be planned or unplanned. The hospitalizations 
required under 4.04E differ from those required in proposed 4.04C, 
which requires that hospitalizations be unplanned.

Proposed Listing 4.05--Recurrent Arrhythmias

    We propose to reorganize the basic structure and presentation of 
current 4.05 (Recurrent arrhythmias) to improve its clarity and ease of 
reference. We would breakdown the current criteria into two parts: 
recurrent episodes of syncope (or near syncope) and findings and 
documentation by a medically acceptable test, to demonstrate that both 
parts must be satisfied to document listing-level severity. We would 
also remove ``uncontrolled'' as a descriptor for recurrent episodes of 
cardiac syncope because, inherently, these episodes are uncontrolled if 
they recur while a person is on a regimen of prescribed treatment. 
Because we would remove ``uncontrolled'' as a descriptor in the 
listing, we would also remove the definition for this term from current 
4.00A3 in the introductory text. Symptoms associated with arrhythmia 
include: anxiety, chest pain, fatigue, sweating, near-syncope, and 
fainting (syncope). Syncope and near-syncope are two of the more 
serious symptoms; individuals with arrhythmias and recurrent episodes 
of syncope have a higher risk of mortality and sudden cardiac death. 
Furthermore, syncope and near-syncope are more quantifiable and 
objective than other symptoms like anxiety and fatigue. For these 
reasons, recurrent episodes of syncope and near syncope continue to be 
an appropriate indicator of listing-level severity for individuals with 
recurrent arrhythmias.\53\
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    \53\ Koene, R.J., Adkisson, W.O., & Benditt, D.G. (2017). 
Syncope and the risk of sudden cardiac death: Evaluation, 
management, and prevention. Journal of arrhythmia, 33(6), 533-544. 
https://doi.org/10.1016/j.joa.2017.07.005.
---------------------------------------------------------------------------

Proposed Listing 4.06--Congenital Heart Disease

    For the reason discussed below, we propose to remove the 
parenthetical reference to ``cyanotic or acyanotic'' congenital heart 
disease from the heading in current 4.06 in order to focus on 
hypoxemia.
    Accordingly, we propose to revise current 4.06A to require 
``hypoxemia'' rather than ``cyanosis or acyanosis.'' ``Hypoxemia'' 
reflects abnormalities in the blood, such as an increased hematocrit 
level or a low blood oxygen level, which are detected through 
laboratory analysis or pulse oximetry. On the other hand, the term 
``cyanosis'' refers to skin discoloration observed during a physical 
examination. Cyanosis is a more subjective assessment subject to 
misinterpretation due to by many factors, including skin complexion. 
Thus, the term ``hypoxemia'' relates more to the laboratory and pulse 
oximetry findings than the term ``cyanosis.'' \54\
---------------------------------------------------------------------------

    \54\ Stout, K.K. (2019).
---------------------------------------------------------------------------

    To establish listing-level severity for individuals with congenital 
heart disease, IOM recommended documentation of chronic and persistent 
hypoxemia. Therefore to demonstrate the chronic and persistent nature, 
in proposed 4.06A1, we would require two hematocrit measurements 
instead of the current listing's single measurement. Two measurements, 
at least 90 days apart within a consecutive 12-month period will help 
ensure the person's hematocrit level is associated with chronic 
hypoxemia and not the result of a reversible condition, such as 
dehydration.\55\ The proposed requirement that the two measurements be 
90 days apart is consistent with the time period requirement used in 
our other body system listings, and consistent with instructions 
providers receive for scheduling patients \56\ and established check-up 
intervals for adults with congenital heart disease.\57\

[[Page 38848]]

Furthermore, requiring two measurements at least 90 days apart is 
consistent with the current (and proposed) childhood congenital heart 
disease criterion (104.06A1) and will assist with documenting duration 
and establishing that the persistent nature of the person's 
condition.\58\
---------------------------------------------------------------------------

    \55\ Stack, S.W. . . ., & Berger, S.A. (2009). The effects of 
high hematocrit arterial flow--A phenomenological study of health 
risk implications. Chemical Engineering Science, 64(22), 4701-4706. 
doi:10/1016/j.ces.2009.07.017.
    \56\ Bavafa, H., Savin, S., & Terwiesch, C. (2019). Redesigning 
Primary Care Delivery: Customized Office Revisit Intervals and E-
Visits. https://dx.doi.org/10.21con39/ssrn.2363685.
    Paper referenced by Bavafa: Schectman, G., G. Barnas, P. Laud, 
L. Cantwell, M. Horton, E.J. Zarling. 2005. Prolonging the return 
visit interval in primary care. The American Journal of Medicine, 
118(4) 393-39.
    \57\ According to the University of Washington's Adult 
Congenital Heart Disease Clinic's Information for Patients and 
Families (2016), most people with congenital heart disease require 
regular check-ups with their cardiologist ``at intervals ranging 
from every several months to every several years.'' Accessed May 4, 
2022, from HeartInstitute_AdultCongenitalHeartDiseaseClinic.pdf 
(uwmedicine.org).
    People with listing-level congenital heart disease are expected 
to require more frequent checkups than those who are asymptomatic or 
have less severe disease.
    \58\ See 20 CFR 404.1505(a) and 416.905(a). The law defines 
disability as the inability to do any substantial gainful activity 
by reason of any medically determinable physical or mental 
impairment which can be expected to result in death or which has 
lasted or can be expected to last for a continuous period of not 
less than 12 months.
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    We would include the medical abbreviation 
``S<INF>a</INF>O<INF>2</INF>'' in 4.06A2. This abbreviation frequently 
appears in the medical evidence to indicate arterial oxygen 
(O<INF>2</INF>) saturation determined by arterial blood gas testing. We 
would also include the medical abbreviation 
``P<INF>a</INF>O<INF>2</INF>'' (partial pressure of O<INF>2</INF>), 
because medical reports may use it interchangeably with the 
abbreviation ``PO<INF>2</INF>'' that we use in current 4.06A for 
arterial partial pressure of oxygen. Additionally, we would express 
P<INF>a</INF>O<INF>2</INF> and PO<INF>2</INF> in millimeters of mercury 
(mmHg) instead of Torr units to make the listing consistent with 
current medical practice and terminology.\59\
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    \59\ Castro D, Patil SM, Keenaghan M. Arterial Blood Gas. 2021 
Jan 27. In: StatPearls [internet]. Treasure Island (FL): StatPearls 
Publishing; 2021 Jan-. PMID: 30725604.
---------------------------------------------------------------------------

    In proposed 4.06A3, we would add a criterion for 
S<INF>p</INF>O<INF>2</INF> (percentage of oxygen saturation of blood 
hemoglobin) measured by pulse oximetry, including measurements taken 
while the person is at rest or while doing a six-minute walk test 
(6MWT). Pulse oximetry measurements are a non-invasive alternative to 
invasive testing. A person's medical evidence often provides 
S<INF>p</INF>O<INF>2</INF> findings, and S<INF>p</INF>O<INF>2</INF> 
measured by pulse oximetry reflects an advance in medical technology 
that provides another way to establish listing-level 
severity.<SUP>60 61 62</SUP>
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    \60\ IOM. (2010), 178.
    \61\ Oster, M. E. . . ., & Kochilas, L.K. (2016). Screening for 
critical congenital heart disease. Clinics in Perinatology, 43(1), 
73-80. doi:10.1016/j.clp.2015.11.005.
    \62\ Mechem, C.C. (2014). Pulse oximetry. In P.E. Parsons (Ed.), 
UpToDate (Jan. 2014). Retrieved from https://www.uptodate.com/contents/pulse-oximetry.
---------------------------------------------------------------------------

    Proposed 4.06A3 would require three S<INF>p</INF>O<INF>2</INF> 
measurements 30 days apart within a consecutive 12-month period showing 
hypoxemia. We explain in the introductory text of proposed 4.00H4c that 
these measurements must be documented by a medical source using methods 
consistent with the prevailing state of medical knowledge and clinical 
practice, and also must be consistent with the other evidence in the 
person's case record. We would require an S<INF>p</INF>O<INF>2</INF> of 
87 percent or less because this finding is comparable in severity to an 
S<INF>a</INF>O<INF>2</INF> of less than 90 percent in current 
4.06A2.\63\ By requiring several measurements at least 30 days apart, 
we ensure that the required findings span a period of at least 90 days. 
Similar to the requirement for repeated hematocrit measurements under 
4.06A1, this will document that the condition is chronic and 
persistent, and the measurements are not related to a reversible 
condition or an inaccurate reading.
---------------------------------------------------------------------------

    \63\ IOM. (2010), 178.
---------------------------------------------------------------------------

    In proposed 4.06B, we would include an additional option of taking 
an S<INF>a</INF>O<INF>2</INF> measurement for determining the level of 
hypoxemia during exertion. This change would provide an additional way 
of evaluating hypoxemia. Similarly, we would include an oxygen uptake 
measurement as another option.
    We are proposing several changes to current 4.06C. We would use the 
term ``pulmonary hypertension'' to describe the impairment instead of 
the term ``pulmonary vascular obstructive disease.'' ``Pulmonary 
hypertension'' is the term more commonly used by clinicians and, 
therefore, the most likely to appear in the medical evidence.\64\ We 
are also proposing to delete ``secondary'' from the listing's heading, 
because pulmonary hypertension can be disabling regardless of whether 
it is a ``primary'' or ``secondary'' condition.
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    \64\ A review of the website for the Journal of the American 
Medical Association (JAMA), a peer-reviewed medical journal 
published 48 times a year by the American Medical Association, found 
that the exact term ``pulmonary hypertension'' came back with more 
than 800 results. A search for the exact term ``pulmonary vascular 
obstructive disease'' came back with zero results. The search was 
conducted on September 8, 2021.
---------------------------------------------------------------------------

    We would include medical findings in proposed 4.06C that are 
expressed in millimeters of mercury (mmHg). Findings of pulmonary 
artery pressure are expressed in mmHg more often than they are 
expressed as a percentage of ``systemic arterial systolic pressure,'' 
as in current 4.06C. Pulmonary hypertension may be reported in the 
medical evidence as either pulmonary artery pressure or mean pulmonary 
artery pressure, so we would include both types of findings in the 
proposed listing.
    We would add a new criterion--proposed 4.06D--to evaluate adults 
with ``single ventricle,'' which is also known as ``single ventricle 
physiology'' or ``functional single ventricle.'' Children born with 
single ventricle have a severe, medically determinable impairment (MDI) 
that will usually need to be corrected by staged surgery called 
``Fontan procedures.'' \65\ These procedures enable an increasing 
percentage of affected children to survive into adulthood. As adults, 
they have significantly reduced functional capacities that steadily 
decline. We would find adults disabled under proposed 4.06D if 
objective medical evidence shows the person has single ventricle, 
regardless of whether or not they had Fontan or other surgical 
procedures.<SUP>66 67</SUP> We provide information in the introductory 
text in proposed 4.00H3 (What is single ventricle?) about single 
ventricle and these surgical procedures.
---------------------------------------------------------------------------

    \65\ People with single ventricle will generally undergo staged 
reconstructive ``Fontan procedures,'' ultimately resulting in a 
``Fontan circulation.'' Fontan circulation describes the state in 
which virtually all systemic venous return-blood passively flows 
directly into the pulmonary arteries via surgical or catheter-placed 
shunts, without the blood passing through a ventricle.
    \66\ Cohen, S., & Marelli, A. (2016). Evolving heart 
transplantation across the lifespan: A growing population of adults 
with congenital heart disease. Archives of Cardiovascular Disease, 
109(10), 511-513. doi:10.1016/j.acvd.2016.05.001.
    \67\ IOM. (2010), 169, 178.
---------------------------------------------------------------------------

Proposed Listing 4.07--Aortic Valvular Disease

    We currently evaluate aortic valvular disease under other 
cardiovascular disorders listings, which include requirements for ETT 
or repeated hospitalization. According to the IOM report, due to the 
risk associated with exercise testing for individuals with symptomatic 
aortic stenosis, ETT is not advised.\68\ Furthermore, very serious 
symptomatic aortic stenosis is ``universally fatal'' if left untreated 
and there are few effective, long-term medical therapies for 
individuals with this level of disease.\69\ Therefore, we followed IOM 
recommendations to provide evaluation criteria for aortic valvular 
disease and propose to add new listing 4.07 to evaluate aortic valvular 
disease. The medical community considers an aortic valve area equal to 
or less than 1.0 cm\2\ indicative of advanced stenotic disease 
associated with significant dyspnea, fatigue, angina, and other serious
---------------------------------------------------------------------------

    \68\ IOM. (2010), 195.
    \69\ IOM. (2010), 194, 195.

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[[Page 38849]]

symptoms.<SUP>70 71 72 73</SUP> Proposed 4.07 would require appropriate 
testing that documents the aortic valve area.
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    \70\ Berthelot-Richer, M., Pibarot, P., Capoulade, R., Dumesnil, 
J.G., Dahou, A., Thebault, C., . . . Clavel, M.-A. (2016). 
Discordant grading of aortic stenosis severity: Echocardiographic 
predictors of survival benefit associated with aortic valve 
replacement. Journal of the American College of Cardiology: 
Cardiovascular Imaging, 9(7), 797-805. doi:10.1016/
j.jcmg.2015.09.026.
    \71\ IOM. (2010), 195.
    \72\ Nombela-Franco, L. (2015).
    \73\ Ziberszac, R., Gabriel, H., Schemper, M., Laufer, G., 
Maurer, G., & Rosenhek, R. (2017). Asymptomatic severe aortic 
stenosis in the elderly. Journal of the American College of 
Cardiology: Cardiovascular Imaging, 10(1), 43-50. doi:10.1016/
j.jcmg.2016.05.015.
---------------------------------------------------------------------------

Proposed Listing 4.08--Cardiomyopathy

    Consistent with IOM recommendations, we would add 4.08 to evaluate 
cardiomyopathies, such as hypertrophic cardiomyopathy (HCM). We 
currently evaluate cardiomyopathy under 4.02 (Chronic heart failure), 
4.04 (Ischemic heart disease), 4.05 (Recurrent arrhythmias), or 11.04 
(Vascular insult to the brain), depending on its effects. Depending on 
the underlying cause of the person's cardiomyopathy or its effects, we 
may continue to evaluate cardiomyopathy under 4.02, 4.04, 4.05, and 
11.04. We created this new cardiomyopathy listing to specifically 
address HCM, endomyocardial fibrosis, and cardiac amyloidosis AL type, 
which are more serious types of cardiomyopathy.\74\
---------------------------------------------------------------------------

    \74\ SSA has designated endomyocardial fibrosis and cardiac 
amyloidosis AL type as Compassionate Allowance (CAL) conditions. See 
Compassionate Allowances website Home Page (ssa.gov).
---------------------------------------------------------------------------

    HCM with severe left ventricular or septal wall thickness can cause 
serious problems, including chest pain, dyspnea, syncope, and 
arrhythmias.\75\ As recommended by IOM, we would evaluate HCM under 
proposed 4.08A by requiring the heart to have a left ventricular or 
septal wall thickness equal to or greater than 20 millimeters. Proposed 
4.08 also requires the individual to be seriously limited in the 
ability to perform an ETT, or a medical source has concluded that the 
performance of an ETT would present a significant risk.
---------------------------------------------------------------------------

    \75\ IOM. (2010), 80.
---------------------------------------------------------------------------

    Proposed 4.08B would evaluate endomyocardial fibrosis, a form of 
cardiomyopathy with a generally poor prognosis despite treatment. Under 
proposed 4.08B, we would require endomyocardial fibrosis resulting in a 
loss of heart chamber volume, atrial dilatation, and mitral or 
tricuspid valve regurgitation.
    Proposed 4.08C would evaluate cardiac amyloidosis AL (light-chain) 
type, another form of cardiomyopathy with a poor prognosis. We would 
need objective medical evidence, such as biopsy findings, 
echocardiogram, cardiac MRI, and PET scan to establish listing-level 
severity.
    Proposed 4.08D would evaluate exacerbations or complications of 
cardiomyopathy, requiring three hospitalizations within a consecutive 
12-month period and at least 30 days apart.

Proposed Listing 4.09--Heart Transplantation

    We are proposing editorial changes in the heading and text of 
current 4.09, which would not be substantive but would clarify the 
guidance. We have changed from ``1 year following surgery'' to ``1 year 
from the date of the transplant'' consistent with transplantation 
listings in other body systems such as 6.04 (Chronic kidney disease) 
and 7.17 (Hematological disorders treated by bone marrow or stem cell 
transplantation).

Proposed Listing 4.10--Dissecting Aneurysm of the Aorta or Major 
Branches

    We propose to revise the heading for listing 4.10 to specify that 
we evaluate only ``dissecting'' aneurysms under the listing consistent 
with IOM recommendations.\76\
---------------------------------------------------------------------------

    \76\ IOM. (2010), 217.
---------------------------------------------------------------------------

Proposed Listing 4.11--Chronic Venous Insufficiency

    We propose to revise the heading in current listing 4.11 by 
replacing the outdated term ``incompetency'' with the term ``reflux''--
the term the medical community currently uses to describe decrease 
blood flow and pooling of blood in the veins.\77\ We would delete the 
word ``deep'' in the heading so that the listing covers reflux or 
obstruction associated with superficial and perforating veins. Reflux 
or obstruction in these veins may result in the required level of 
CVI.\78\ Additionally, as recommended by IOM, we would require 
confirmation of CVI by duplex ultrasound or other appropriate 
diagnostic technique. The medical community considers the use of duplex 
ultrasound to be the best method for detecting reflux or 
obstruction.\79\
---------------------------------------------------------------------------

    \77\ IOM. (2010), 160.
    \78\ IOM. (2010), 161.
    \79\ Id.
---------------------------------------------------------------------------

    In proposed 4.11A, we would adopt IOM recommendations and broaden 
the listing criteria we apply to trophic changes (changes resulting 
from interruption of nerve supply) of the skin. For example, in 
addition to brawny edema, trophic changes evaluated under the proposed 
listing would include hyperpigmentation and lipodermatosclerosis.
    We would revise the current requirement that these skin changes 
involve ``at least two-thirds of the leg between the ankle and knee or 
the distal one-third of the lower extremity between the ankle and 
hip.'' Instead, we would require extensive skin changes involving at 
least two-thirds of the leg below the knee, to make the requirement 
simpler to understand and apply. This revision is consistent with IOM's 
recommendation to require skin changes below the knee.\80\
---------------------------------------------------------------------------

    \80\ IOM. (2010), 157-161.
---------------------------------------------------------------------------

    We would require the skin changes under proposed 4.11A to be 
consistent with CVI, and we would document the skin changes over a 
period of at least 90 days to ensure they are chronic. Additionally, 
the CVI must be unresponsive to compression therapy, because this 
therapy usually enables people to return to a good level of 
functioning.\81\
---------------------------------------------------------------------------

    \81\ IOM. (2010), 159.
---------------------------------------------------------------------------

    In proposed 4.11B, we would remove findings in the current listing 
that no longer demonstrate required severity. For example, we would 
remove superficial varicosities, which indicate venous disease but not 
necessarily CVI. We would follow IOM's recommendation and also remove 
stasis dermatitis, because it is ``a generic term referring to the 
trophic changes,'' and it is unreliable because it may be a sign of 
other unrelated conditions including aging.\82\
---------------------------------------------------------------------------

    \82\ IOM. (2010), 161.
---------------------------------------------------------------------------

    Proposed 4.11B would require recurrent or persistent skin 
ulceration that has not healed after 6 or more months of prescribed 
treatment. In regard to documenting duration and severity of CVI, this 
requirement is more conclusive than the current requirement of 3 months 
of unsuccessful prescribed treatment as it demonstrates the condition 
has persisted despite treatment for a longer period of time.\83\
---------------------------------------------------------------------------

    \83\ IOM. (2010), 161.
---------------------------------------------------------------------------

Proposed Listing 4.12--Peripheral Arterial Disease

    We propose to revise the heading of the current listing to evaluate 
peripheral arterial disease (PAD) while the person is on a regimen of 
prescribed treatment. PAD often improves with angioplasty, supervised 
physical rehabilitation, and other prescribed therapies.\84\
---------------------------------------------------------------------------

    \84\ Poredo[scaron] P, Jezovnik M, Kalodiki E. Medical 
management of patients with peripheral arterial disease. Int Angiol. 
2015 Feb;34(1):75-93. Epub 2014 Jun 11. PMID: 24916346.

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[[Page 38850]]

    We would add leg pain in the heading as a serious and potentially 
debilitating symptom of PAD. People who have PAD with intermittent leg 
pain may be impaired to a similar extent as a person with PAD with 
intermittent claudication.\85\
---------------------------------------------------------------------------

    \85\ IOM. (2010), 151.
---------------------------------------------------------------------------

    Consistent with IOM recommendations, we would require a person's 
intermittent leg pain or claudication to interfere with his or her 
mobility. This proposed change clarifies our original intent in the 
listing, which is to tie PAD to functioning. Finally, we would replace 
the term ``appropriate medically acceptable imaging'' in the listing 
heading with ``appropriate test(s)'' (4.00G6--Are there any other 
studies that are helpful in evaluating PAD?) to acknowledge that non-
imaging procedures such as physical examination and blood tests may 
also help detect PAD.\86\
---------------------------------------------------------------------------

    \86\ Id.
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Proposed Listing 4.16--Cardiac Allograft Vasculopathy

    We propose to add new listing 4.16 to evaluate a person who 
received a heart transplant (allograft) and subsequently developed 
cardiac allograft vasculopathy (CAV). Currently, we evaluate CAV 
through medical equivalence to listing 4.09 (Heart transplant). CAV 
results in stenosis of the heart's blood vessels that may progress 
quickly and cause significant heart dysfunction. CAV with moderate 
stenosis, as defined in the medical literature,\87\ may also result in 
a listing-level impairment, depending on the extent and seriousness of 
dysfunction. To establish the required level of CAV, we would require a 
cardiac index (cardiac output) of less than 2 liters/minute/meter 
square (L/min/m\2\), an ejection fraction equal to or less than 45 
percent, right atrial pressure greater than 12 mmHg, or pulmonary 
capillary wedge pressure greater than 15 mmHg. Individuals who have any 
of these findings have a poor prognosis and are limited in their 
activities and ability to work.<SUP>88 89 90 91</SUP>
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    \87\ The International Society of Heart and Lung 
Transplantation's grading classification defines mild cardiac 
allograft vasculopathy (CAV<INF>1</INF>) as having left main artery 
stenosis of less than 50 percent, primary vessel stenosis greater 
than 70 percent (including the right coronary artery), or any branch 
stenosis greater than 70 percent (see Mehra, M.R., Crespo-Leiro, 
M.G., Dipchand, A., Ensminger, S.M., Hiemann, N.E., Kobashigawa, 
J.A., . . . Uber, P.A. (2010). International Society of Heart and 
Lung Transplantation working formulation of a standardized 
nomenclature for cardiac allograft vasculopathy--2010. Journal of 
Heart and Lung Transplantation, 29(7), 717-727. doi:10.1016/
j.healun.2010.05.017).
    \88\ Kindel, S.J., & Pahl, E. (2011). Cardiac allograft 
vasculopathy in children--treatment challenges. Progress in 
Pediatric Cardiology, 32(1), 37-42. doi:10.1016/
j.ppedcard.2011.06.008.
    \89\ Kobashingawa, J.A. (2015). The changing face of first-year 
intravascular ultrasonography in heart transplantation. Journal of 
the American College of Cardiology: Heart Failure, 3(12), 954-955. 
doi:10.1016/j.jchf.2015.09.004.
    \90\ Mehra, M.R. (2010).
    \91\ Okada, K., Kitahara, H., Yang, H., Tanaka, S., Kobayashi, 
Y., Kimura, T., . . . Fearon, W.F. (2015). Paradoxical vessel 
remodeling of the proximal segment of the left anterior descending 
artery predicts long-term mortality after heart transplantation. 
Journal of the American College of Cardiology: Heart Failure, 3(12), 
945-952. doi:10.1016/j.jchf.2015.07.013.
---------------------------------------------------------------------------

Other Proposed Changes

    As mentioned, we are proposing new criteria to evaluate 
exacerbations or complications of several categories of cardiovascular 
disorders. These new criteria include proposed 4.02B3 for evaluating 
chronic heart failure, proposed 4.04E for evaluating ischemic heart 
disease, proposed 4.06E for evaluating congenital heart disease, and 
proposed 4.08D for evaluating cardiomyopathies. Consistent with IOM 
recommendations, we are proposing these new criteria for evaluating 
chronic heart failure and cardiomyopathies.\92\ In addition, we are 
proposing these new criteria for evaluating ischemic heart disease 
(4.04) and congenital heart disease (4.06). Our adjudicative experience 
shows that these cardiovascular disorders are prone to exacerbations 
and serious complications. These proposed criteria would require 
exacerbations or complications causing a person to be hospitalized 
three or more times within a consecutive 12-month period.\93\ An 
impairment resulting in exacerbations or complications that require 
this many hospitalizations in 12 months will prevent a person from 
engaging in any gainful activity.<SUP>94 95 96 97 98 99 100</SUP> We 
would require these hospitalizations to be at least 30 days apart and 
to last at least 48 hours, including hours in a hospital emergency 
department immediately before the hospitalization, to ensure we are 
evaluating separate listing-level episodes of exacerbations or 
complications. Our proposal to require that each hospitalization last 
at least 48 hours is generally consistent with data showing that the 
average length of hospital stays for serious cardiac conditions like 
primary heart failure and adult congenital heart disease is at least 48 
hours.<SUP>101 102</SUP>
---------------------------------------------------------------------------

    \92\ IOM. (2010), 89, 172.
    \93\ Id.
    \94\ Hua, M. (2015).
    \95\ IOM. (2010), 30, 90, 196.
    \96\ Kim, Y. (2016).
    \97\ Kyriakou, M., & Kiff, P.F. (2016). Prognosis of the 
comorbid heart failure and anemia: A systematic review and meta-
analysis. Clinical Trials and Regulatory Science in Cardiology, 16, 
12-21. doi:10.1016/j.ctrsc.2016.01.008.
    \98\ Reynolds, K. (2015).
    \99\ Versteeg, H. (2013).
    \100\ Yu, P.-J. (2016).
    \101\ Jackson SL, Tong X, King RJ, et al. National Burden of 
Heart Failure Events in the United States, 2006 to 2014. 
Circulation. Heart Failure. 2018 Dec;11(12):e004873. DOI: 10.1161/
circheartfailure.117.004873. PMID: 30562099; PMCID: PMC6424109.
    \102\ Cedars, A., Benjamin, L., Burns, S.V., Novak, E., & Amin, 
A. (2017). Clinical predictors of length of stay in adults with 
congenital heart disease. Heart (British Cardiac Society), 103(16), 
1258-1263. https://doi.org/10.1136/heartjnl-2016-310841.
---------------------------------------------------------------------------

    Another revision we are proposing would affect chronic venous 
insufficiency evaluated under 4.11. Under our proposed changes, we 
would follow IOM's recommendations and require documentation that 
certain manifestations of chronic venous insufficiency (for example, 
trophic changes of the skin) occurred at least twice within a 
consecutive 12-month period, instead of only once under the current 
listings.\103\ This change is based on the IOM recommendation that two 
occurrences per year more accurately and consistently demonstrates 
listing-level severity. We would also require documentation that these 
manifestations occurred at least 90 days apart. These requirements 
ensure we are appropriately documenting the chronicity and persistence 
of these conditions and evaluating people who have very serious chronic 
conditions.
---------------------------------------------------------------------------

    \103\ IOM. (2010), 161.
---------------------------------------------------------------------------

Proposed Changes to the Childhood Cardiovascular Disorders Introductory 
Text

Proposed 104.00--Introductory Text to the Childhood Cardiovascular 
Disorders Listings

    We repeat much of the introductory text of proposed 4.00 in the 
introductory text of proposed 104.00, because the same basic criteria 
for evaluating cardiovascular disorders apply to both adults and 
children. Because we have already described these proposed criteria 
above, the following discussion describes only those criteria that are 
unique to children or that require further explanation in how they will 
be applied to children.
    The following table shows the heading of the current and proposed 
sections of the childhood introductory text for cardiovascular 
disorders:

[[Page 38851]]



------------------------------------------------------------------------
                                             Proposed sections of the
   Current sections of the childhood     childhood introductory text and
   introductory text and listings for      listings for cardiovascular
         cardiovascular system                      disorders
------------------------------------------------------------------------
104.00 Cardiovascular System...........  104.00 Cardiovascular
                                          Disorders.
A. General.............................  A. How do we define
                                          cardiovascular disorder and
                                          cardiovascular terms?
B. Documenting Cardiovascular            B. What documentation do we
 Impairment.                              need to evaluate
                                          cardiovascular disorders?
C. Evaluating Chronic Heart Failure....  C. How do we evaluate chronic
                                          heart failure?
D. Evaluating Congenital Heart Disease.  D. How do we evaluate
                                          congenital heart disease?
E. Evaluating Arrhythmias..............  E. How do we evaluate
                                          arrhythmias?
F. Evaluating Other Cardiovascular       F. How do we evaluate other
 Impairments.                             cardiovascular disorders?
G. Other Evaluation Issues.............  G. How do we evaluate issues
                                          that affect the cardiovascular
                                          system?
                                         H. How do we evaluate
                                          cardiovascular disorders that
                                          do not meet one of these
                                          listings?
------------------------------------------------------------------------

Proposed 104.00C--How do we evaluate chronic heart failure?

    We are proposing changes in current 104.00C consistent with changes 
we are proposing in the adult listings for chronic heart failure. We 
would extensively revise current 104.00C2 by removing specific findings 
for documenting cardiomegaly. These findings are often not provided in 
a child's case record and, therefore, have presented difficulty in 
adjudication. Proposed 104.00C2a would describe the types of imaging 
provided in a child's case record for documenting cardiomegaly. We 
explain at 104.00C2b(iii) that signs of congestion need not be found on 
all examinations because congestion may be controlled by prescribed 
treatment or may not be present at the time of evaluation. We have 
added 104.00C4 to explain how we propose to evaluate chronic heart 
failure treated with a mechanical circulatory support device under 
proposed 104.02D (Chronic heart failure).

Proposed 104.00D--How do we evaluate congenital heart disease?

    We plan to significantly expand the information in current 104.00D. 
In proposed 104.00D2 (How do we evaluate conditions associated with 
congenital heart disease?), we would explain how we evaluate conditions 
associated with congenital heart disease. Proposed 104.00D2 includes 
additional means of measuring oxygen saturation in 104.06 (Congenital 
heart disease), because these measurements are readily found in the 
medical evidence. We are proposing new 104.00D3 (What is Eisenmenger 
syndrome?) to explain Eisenmenger syndrome in children, and we propose 
new 104.00D4 (What is a single ventricle?) to include a definition for 
the term ``single ventricle.''

Proposed 104.00F--How do we evaluate other cardiovascular disorders?

    We propose revisions to 104.00F6 (How will we evaluate chronic 
rheumatic fever or rheumatic heart disease?) consistent with the 
removal of current listing 104.13, rheumatic heart disease. These 
revisions would explain that we evaluate rheumatic heart disease under 
104.02 (Chronic heart failure) or 104.05 (Recurrent arrhythmias). We 
propose adding 104.00F11 (What is cardiac allograft vasculopathy and 
how do we evaluate it?) consistent with proposed 104.16 (Cardiac 
allograft vasculopathy).

Proposed 104.00G--How do we evaluate issues that affect the 
cardiovascular system?

    We propose revisions to 104.00G consistent with those proposed to 
the adult listings. We propose to revise 104.00G1 (How do we consider 
the effects of obesity when we evaluate your cardiovascular disorder?) 
to simplify and refocus our discussion of how we consider the effects 
of obesity more specifically on cardiovascular disorders. We propose 
adding 104.00G3 (How do we consider hospitalizations?), consistent with 
new 104.02E (Chronic heart failure) and 104.06E (Congenital heart 
disease). We propose to redesignate current 104.00G3 (How do we 
evaluate impairments that do not meet one of the cardiovascular 
listings?) as 104.00H (How do we evaluate cardiovascular disorders that 
do not meet one of these listings?).

Proposed Changes to the Childhood Cardiovascular Disorders Listings

    We are proposing some changes to the childhood listings that 
correspond with changes we are proposing to the adult listings. Other 
changes are specific to how we evaluate cardiovascular disorders in 
children. The reasons provided above for changing or removing current 
criteria for adults also apply to the criteria for children. Because we 
have already described these proposed criteria above, the following 
discussion describes only those criteria that are unique to children or 
that require further explanation in how we will specifically apply them 
to children. Additionally, the numbering of the childhood listings 
would conform to that of the adult listings.
    The following table shows the heading of the current and proposed 
sections of the childhood listings for cardiovascular disorders:

               Childhood Cardiovascular Disorders Listings
------------------------------------------------------------------------
                Current                              Proposed
------------------------------------------------------------------------
104.02 Chronic heart failure...........  104.02 Chronic heart failure.
104.05 Recurrent arrhythmias...........  104.03 [Reserved].
104.06 Congenital heart disease........  104.04 [Reserved].
104.09 Heart transplant................  104.05 Recurrent arrhythmias.
104.13 Rheumatic heart disease.........  104.06 Congenital heart
                                          disease.
                                         104.07 [Reserved].
                                         104.08 [Reserved].
                                         104.09 Heart transplantation.
                                         104.10 [Reserved].
                                         104.11 [Reserved].
                                         104.12 [Reserved].

[[Page 38852]]

 
                                         104.13 [Reserved].
                                         104.14 [Reserved].
                                         104.15 [Reserved].
                                         104.16 Cardiac allograft
                                          vasculopathy.
------------------------------------------------------------------------

    The following table shows our proposed changes to the childhood 
cardiovascular disorders listings criteria that involve changes to 
healthcare utilization and condition/episode requirements, the 
rationale for each change, and supporting resource. Following this 
table, we discuss all of our proposed changes to the childhood 
cardiovascular disorders listings in more detail.

  Childhood Cardiovascular Disorders Listings Criteria--Changes in Healthcare Utilization and Condition/Episode
                                                  Requirements
----------------------------------------------------------------------------------------------------------------
                                           Proposed listing
      Current listing criterion               criterion                Rationale                Resources
----------------------------------------------------------------------------------------------------------------
                                      Listing 104.02 Chronic heart failure
----------------------------------------------------------------------------------------------------------------
104.02A Persistent tachycardia at      A. Persistent            We would clarify that    IOM. (2010), 171,173,
 rest (see Table I);                    tachycardia at rest      to satisfy 104.02A, we   176.
                                        measured at least        would require two or
                                        twice within a           more tachycardia
                                        consecutive 12-month     measurements in a
                                        period and at least 90   consecutive 12-month
                                        days apart documented    period. Our intent is
                                        by apical heart rate     to ensure that the
                                        greater than or equal    child has persistent
                                        to the value in Table    tachycardia despite
                                        I.                       treatment. We would
                                                                 also require that
                                                                 readings of
                                                                 tachycardia occur at
                                                                 least 90 days apart to
                                                                 further document
                                                                 chronic disease.
104.02B..............................  B. Persistent tachypnea  To satisfy 104.02B, we   IOM. (2010), 171,173,
Persistent tachypnea at rest (see       at rest measured at      would require two or     176.
 Table II) or markedly decreased        least twice within a     more tachypnea
 exercise tolerance (see 104.00C2b);.   consecutive12-month      measurements in a
                                        period and at least 90   consecutive 12-month
                                        days apart documented    period. Our intent is
                                        by respiratory rate      to ensure that the
                                        greater than or equal    child has persistent
                                        to the value in Table    tachypnea, despite
                                        II or markedly           treatment. We would
                                        decreased exercise       also require that
                                        tolerance (see           readings of tachypnea
                                        104.00C2b).              occur at least 90 days
                                                                 apart to further
                                                                 document chronic
                                                                 disease.
----------------------------------------------------------------------------------------------------------------
                                     Listing 104.06 Congenital heart disease
----------------------------------------------------------------------------------------------------------------
104.06...............................  104.06.................  In 104.06A2, we would    Based on SSA
A. 2. Arterial O2 saturation of less   A. 2. Arterial blood      use the measurement of   administrative data
 than 90 percent in room air, or        gas test measurement     millimeters of           from FY 2019-2021, of
 resting arterial PO2 of 60 Torr or     obtained at rest while   mercury, ``mmHg,''       all childhood claims
 less; or.                              breathing room air, as   instead of the           with a primary
3. Hypercyanotic spells, syncope,       described in either a    measurement of           impairment of
 characteristic squatting, or other     or b:.                   ``Torr'' that is used    congenital heart
 incapacitating symptoms directly      a. SaO2 (arterial         in current 104.06A2,     disease that met or
 related to documented cyanotic heart   oxygen saturation)       and we would note that   medically equaled
 disease; or.                           less than or equal to    arterial PO2 is          listing 104.06,
4. Exercise intolerance with            89 percent; or.          normally measured in     approximately .2
 increased hypoxemia on exertion..     b. PO2 or PaO2 (partial   room air.                percent cited 104.06A3
                                        pressure of oxygen)     We would remove current   or 104.06A4 criteria.
                                        less than or equal to    104.06A3 and 104.06A4,   See Table A and B in
                                        60 mmHg; or.             because Agency medical   supporting and related
                                       3. SpO2 (percentage of    experts indicated they   materials to this
                                        oxygen saturation of     are less objective and   Docket for more
                                        blood hemoglobin)        more difficult to        information.
                                        measured by pulse        document than the
                                        oximetry either at       other criteria and
                                        rest, or after           they are used
                                        activity, while          infrequently..
                                        breathing room air,     We would add another
                                        less than or equal to    criterion to 104.06A
                                        87 percent on three      by adding SpO2
                                        evaluations at least     (percentage of oxygen
                                        30 days apart within a   saturation of blood
                                        consecutive 12-month     hemoglobin), measured
                                        period (see              by pulse oximetry
                                        104.00A3e)..             equal to or less than
                                                                 87 percent. Consistent
                                                                 with the proposed
                                                                 adult listing (4.06),
                                                                 this criterion would
                                                                 become the new
                                                                 104.06A3..

[[Page 38853]]

 
No current criteria..................  E. Exacerbations or      We would add 104.06E to  IOM. (2010), 179.
                                        complications of         evaluate exacerbations
                                        congenital heart         or complications of
                                        disease (see 104.00D)    congenital heart
                                        requiring three          disease occurring at
                                        hospitalizations         least 30 days apart
                                        within a consecutive     and resulting in at
                                        12-month period (see     least three
                                        104.00A3e) and at        hospitalizations
                                        least 30 days apart.     within a consecutive
                                        Each hospitalization     12-month period. An
                                        must last at least 48    impairment resulting
                                        hours, including hours   in exacerbations or
                                        in a hospital            complications that
                                        emergency department     require this many
                                        immediately before the   hospitalizations in 12
                                        hospitalization (see     months will result in
                                        104.00G3).               marked and severe
                                                                 functional limitations
                                                                 for children. We would
                                                                 require these
                                                                 hospitalizations to be
                                                                 at least 30 days apart
                                                                 to ensure we are
                                                                 evaluating separate
                                                                 episodes of
                                                                 exacerbations or
                                                                 complications.
----------------------------------------------------------------------------------------------------------------

Proposed Listing 104.02--Chronic Heart Failure

    We would clarify that to satisfy 104.02A, we would require two or 
more tachycardia measurements in a consecutive 12-month period, and to 
satisfy 104.02B, we would require two or more tachypnea measurements in 
a consecutive 12-month period.\104\ Our intent is to ensure that the 
child has persistent tachycardia or persistent tachypnea, despite 
treatment. We would also require that readings of tachycardia or 
tachypnea occur at least 90 days apart to further document chronic 
disease.\105\
---------------------------------------------------------------------------

    \104\ IOM. (2010), 171, 176.
    \105\ IOM. (2010), 173.
---------------------------------------------------------------------------

    When we last published final rules for growth disorders and weight 
loss in children,\106\ we inadvertently removed Table I for tachycardia 
at rest and Table II for tachypnea at rest in listing 104.02. We would 
restore these tables to the proposed 104.02.
---------------------------------------------------------------------------

    \106\ 80 FR 19522 (2015).
---------------------------------------------------------------------------

    We would add new 104.02D to describe how we will evaluate chronic 
heart failure treated with a mechanical circulatory support device.
    We would add new 104.02E(), to describe how we will evaluate 
exacerbations and complications of heart failure requiring extended 
medical intervention in the hospital or emergency department, as 
explained above.

Proposed Listing 104.06--Congenital Heart Disease

    In 104.06A2, we would use the measurement of millimeters of 
mercury, ``mmHg,'' instead of the measurement of ``Torr'' that is used 
in current 104.06A2, and we would note that arterial PO<INF>2</INF> is 
normally measured in room air.
    We would add another criterion to 104.06A by adding 
S<INF>p</INF>O<INF>2</INF> (percentage of oxygen saturation of blood 
hemoglobin), measured by pulse oximetry equal to or less than 87 
percent. This criterion would become the new criterion 104.06A3. As we 
are proposing in the adult criteria, we would explain in the 
introductory text to the childhood listings that we need pulse oximetry 
measurements documented by medical sources using methods consistent 
with the prevailing state of medical knowledge and clinical practice. 
These measurements must be consistent with the other evidence in the 
case record.
    We would remove current 104.06A3 and 104.06A4, because they are 
used infrequently.\107\ Our adjudicative experience shows that children 
with impairments meeting these listings would be evaluated under 
current and proposed 104.06.
---------------------------------------------------------------------------

    \107\ Based on SSA administrative data from FY 2019-2021, of all 
childhood claims with a primary impairment of congenital heart 
disease that met or medically equaled listing 104.06, approximately 
.2 percent cited these criteria. See Table A and B in supporting and 
related materials to this Docket for more information.
---------------------------------------------------------------------------

    We would add multiple medical readings for pulmonary hypertension 
in 104.06B. We propose adding laboratory findings expressed in 
millimeters of mercury (mmHG) in 104.06B2, and we would add mean 
pulmonary artery pressure readings in 104.06B3.
    We are proposing 104.06C, similar to the adult criterion 4.06D 
(Congenital heart disease), to evaluate children born with a single 
ventricle. Adding consideration of single ventricle to listing 104.06 
enables seriously limited children to be identified earlier in the 
sequential evaluation process.
    We would add 104.06E to evaluate exacerbations or complications of 
congenital heart disease occurring at least 30 days apart and resulting 
in at least three hospitalizations within a consecutive 12-month 
period. An impairment resulting in exacerbations or complications that 
require this many hospitalizations in 12 months will result in marked 
and severe functional limitations for children.\108\ We would require 
these hospitalizations to be at least 30 days apart to ensure we are 
evaluating separate episodes of exacerbations or complications.
---------------------------------------------------------------------------

    \108\ IOM. (2010), 179.
---------------------------------------------------------------------------

Proposed Removal of Listing 104.13--Rheumatic Heart Disease

    We would remove and reserve listing 104.13 because rheumatic heart 
disease is a complication of rheumatic fever, which is rare in the 
United States due to widely available treatment with 
antibiotics.<SUP>109 110</SUP> When complications of rheumatic fever 
result in rheumatic heart disease, and these complications last for 12 
months or more, we would evaluate the complications under other 
cardiovascular listings, such as 104.02 (Chronic heart failure) or 
104.05

[[Page 38854]]

(Recurrent arrhythmias). Rheumatic heart disease will still be 
addressed in the introductory text under 104.00F6 (How will we evaluate 
chronic rheumatic fever or rheumatic heart disease?).
---------------------------------------------------------------------------

    \109\ Beaudoin, A., Edison, L., Introcaso, C.E., Goh, L., 
Marrone, J., Mejia, A. . . ., & Van Beneden, C. (2015). Acute 
rheumatic fever and rheumatic heart disease among children--America 
Samoa, 2011-2012. Morbidity and Mortality Weekly Report, 64(20), 
555-558. Retrieved from https://www.cdc.gov/mmwr/pdf/wk/mm6420.pdf.
    \110\ Yandrapalli, S., Tariq, S., Vuddanda, V.L.K., Sanaani, A., 
Solangi, Z., Anugu, V.R., . . . Aronow, W. (2017). In-hospital 
outcomes and hospitalizations for acute rheumatic heart disease: A 
United States national study. Journal of the American College of 
Cardiology, 69(11)(Suppl.), 1742. doi:10.1016/S0735-1097(17)35131-8.
---------------------------------------------------------------------------

Proposed Listing 104.16--Cardiac Allograft Vasculopathy

    We propose to add listing 104.16 (Cardiac allograft vasculopathy) 
to evaluate a child who received a heart transplant and developed 
cardiac allograft vasculopathy (CAV). CAV may develop after heart 
transplantation and progress to a very serious condition with 
significant functional effects. The medical literature indicates that 
CAV is a leading cause of graft failure and mortality in pediatric 
heart transplant recipients.\111\ To establish listing-level CAV for 
children, we would require only CAV documented by appropriate medically 
acceptable imaging, because pediatric CAV alone is disabling enough to 
result in marked and severe functional limitations for children
---------------------------------------------------------------------------

    \111\ Kindel, S.J. (2011).
---------------------------------------------------------------------------

Specific Questions for the Public

    While the public is welcome to comment on any aspect of this 
proposed rule, we are also seeking input on the following topics:
    <bullet> Should any of the proposed listings for cardiovascular 
disorders be combined into one listing, or divided into multiple 
listings, to enable our adjudicators to more easily identify adults or 
children with impairments that are of listing-level severity? If you 
believe our listing categories create unnecessary administrative 
barriers for impairments that meet listing level severity, please tell 
us by submitting your comments and any supporting research or data.
    <bullet> Are there changes in the medical terminology related to 
cardiovascular disorders that we should consider incorporating or 
clarifying in future revisions to the cardiovascular disorders 
listings? If you believe we should consider updating the medical 
terminology we use in our cardiovascular disorders listings, please 
tell us by submitting your comments and any supporting research or 
data.
    <bullet> Do the frequencies and durations of exacerbations of 
cardiovascular disorders in this proposed rule adequately represent 
listing level severity for cardiovascular disorders? Are there other 
treatments and evidence we should consider when assessing listing-level 
severity including additional objective medical tests, for any of the 
proposed cardiovascular disorders listings? We encourage you to cite 
relevant research or data to support your comments.
    <bullet> Are the proposed functional criteria for cardiovascular 
disorders sufficient for assessing listing level severity? Please 
provide specific suggestions along with supporting research and data 
for different criteria you would like SSA to consider.
    <bullet> Did we not include any valuable information that should be 
included in the introductory text of the cardiovascular disorders 
listings? This text is intended to ease administrative burden for 
adjudicators, claimants, claimant representatives, and the public. 
Please submit specific comments, along with supporting research or 
data, about additional information to include in the introductory text.
    <bullet> In proposed 4.02A1 (Chronic heart failure), we require 
systolic failure documented by appropriate medically acceptable imaging 
during a period of stability (not during an episode of exacerbation of 
heart failure), with left ventricular end diastolic dimension equal to 
or greater than 7.0 cm; or ejection fraction of 30 percent or less. If 
you believe we should require more than one evaluation to document the 
duration of an individual's chronic heart failure, please tell us by 
submitting your comments and any supporting research or data.
    <bullet> In proposed 4.02A2 (Chronic heart failure), we require 
diastolic failure documented by appropriate medically acceptable 
imaging during a period of stability (not during an episode of 
exacerbation of heart failure), with left ventricular posterior wall 
plus septal thickness totaling 2.5 cm or greater, with an enlarged left 
atrium greater than or equal to 4.5 cm, OR left atrial volume index 
(LAVi) greater than or equal to 40 ml, BSA/m2 (milliliters to body 
surface area in squared meters). If you believe we should consider 
other measurements of chronic heart failure, please tell us by 
submitting your comments and any supporting research or data.
    <bullet> In proposed 104.02A (Chronic heart failure), we require 
persistent tachycardia at rest measured twice within a consecutive 12-
month period and at least 90 days apart documented by apical heart rate 
greater than or equal to the value in Table I. In proposed 104.02B, we 
require persistent tachypnea measured at least twice within a 
consecutive 12-month period and at least 90 days apart documented by 
respiratory rate greater than or equal to the value in Table II or 
markedly decreased exercise tolerance. If you believe our proposed 
requirement for at least two measurements of apical heart rate and 
respiratory rate under this listing is inconsistent with current 
medical practice or standards of care (i.e., medical providers do not 
routinely repeat these measurements), please tell us by submitting your 
comments and any supporting research or data.
    <bullet> In proposed 4.06A1 (Congenital heart disease), we require 
two measurements of hematocrit at least 90 days apart within a 
consecutive 12-month period instead of the current requirement for one 
measurement. If you would like to propose a different time frame during 
which these measures should occur, please submit comments and any 
supporting research or data.
    <bullet> Are there alternatives to pulse oximetry testing that are 
reliable, non-invasive, and commonly used to measure chronic hypoxemia 
that we should consider incorporating into proposed listing criterion 
4.06A3 (Congenital heart disease) and 104.06A3 (Congenital heart 
disease)? If you believe there are tests that fit into this category, 
please tell us by submitting your comments and any supporting research 
or data.
    <bullet> At IOM's recommendation, we are proposing to add listing 
4.07 (Aortic valvular disease) to provide evaluation criteria for 
symptomatic adult individuals with aortic valvular disease.\112\ We 
currently evaluate aortic valvular disease under other cardiovascular 
disorders listings, which include requirements for exercise testing or 
repeated hospitalizations. If you disagree with proposed 4.07 (Aortic 
valvular disease), please tell us by submitting your comments and any 
supporting research or data.
---------------------------------------------------------------------------

    \112\ IOM. (2010), 195.
---------------------------------------------------------------------------

What is our authority to make rules and set procedures for determining 
whether a person is disabled under the statutory definition?

    The Act authorizes us to make rules and regulations and to 
establish necessary and appropriate procedures to implement them.\113\
---------------------------------------------------------------------------

    \113\ Sections 205(a), 702(a)(5), and 1631(d)(1) of the Social 
Security Act.
---------------------------------------------------------------------------

How long would this proposed rule be effective?

    If we publish this proposed rule as a final rule, it will remain in 
effect for five years after the date it becomes effective, unless we 
extend it, or revise and issue it again.

Rulemaking Analyses and Notices

    We will consider all comments we receive on or before the close of

[[Page 38855]]

business on the comment closing date indicated above. The comments will 
be available for examination in the rulemaking docket for this rule at 
the above address. We will file comments received after the comment 
closing date in the docket and will consider those comments to the 
extent practicable. However, we will not address untimely comments. We 
may publish a final rule at any time after close of the comment period.

Clarity of This Proposed Rule

    Executive Order 12866, as supplemented by Executive Order 13563, 
requires each agency to write all rules in plain language. In addition 
to your substantive comments on this proposed rule, we invite your 
comments on how to make them easier to understand.
    For example:
    <bullet> Would more, but shorter, sections be better?
    <bullet> Are the requirements in the rule clearly stated?
    <bullet> Have we organized the material to suit your needs?
    <bullet> Could we improve clarity by adding tables, lists, or 
diagrams?
    <bullet> What else could we do to make the rule easier to 
understand?
    <bullet> Does the rule contain technical language or jargon that is 
not clear?
    <bullet> Would a different format make the rule easier to 
understand such as using different groupings and order of sections, 
headings, or paragraphing?

When will we start to use this rule?

    We will not use this proposed rule until we evaluate public 
comments and publish a final rule in the Federal Register. All final 
rules we issue include an effective date. We will continue to use our 
current rule until that date. If we publish a final rule, we will 
include a summary of the relevant comments we received and an 
explanation of how we will apply the new rule.

Regulatory Procedures

Executive Order 12866, as Supplemented by Executive Order 13563

    We consulted with the Office of Management and Budget (OMB) and 
determined that this proposed rule meets the criteria for a significant 
regulatory action under Executive Order 12866, as supplemented by 
Executive Order 13563. Therefore, OMB reviewed the rule.
    We also determined that this proposed rule meets the plain language 
requirement of Executive Order 12866.

Executive Order 13132 (Federalism)

    We analyzed this proposed rule in accordance with the principles 
and criteria established by Executive Order 13132 and determined that 
this proposed rule will not have sufficient federalism implications to 
warrant the preparation of a federalism assessment. We also determined 
that this proposed rule will not preempt any State law or State 
regulation or affect the States' abilities to discharge traditional 
State governmental functions.

Regulatory Flexibility Act

    We certify that this proposed rule would not have a significant 
economic impact on a substantial number of small entities because it 
affects individuals only. Therefore, a regulatory flexibility analysis 
is not required under the Regulatory Flexibility Act, as amended.

Executive Order 13771

    Based upon the criteria established in Executive Order 13771 and M-
17-21 (Guidance Implementing E.O. 13771), we consider this rule a 
transfer rule with no more than de minimis costs. As such, it is exempt 
from requirements under E.O. 13771.

Anticipated Accounting Costs of This Proposed Rule

Anticipated Costs to Our Programs

    Our Office of the Chief Actuary has developed estimates of the 
effects of implementing this proposed rule, which are presented in a 
memorandum attached to this NPRM as a supplementary document. The 
memorandum indicates the estimated annual changes in Old-Age, Survivors 
and Disability Insurance (OASDI) benefit payments and Federal 
Supplemental Security Income (SSI) payments over the 10-year period of 
fiscal years (FY) 2022-2031. The memorandum also provides details about 
the case study developed for the purpose of making these estimates, as 
well as changes since the time the case study was originally developed 
and conducted, that may have impacted the case study results.
    In summary, based on the best available data, our Office of the 
Chief Actuary estimates that this proposed rule, assuming it is 
finalized and implemented for all disability decisions completed on or 
after April 1, 2023, would result in net increases of $308 million in 
scheduled OASDI benefit payments and $71 million in Federal SSI 
payments over the 10-year period of fiscal years (FY) 2022-2031.

Anticipated Administrative Costs to the Social Security Administration

    The Office of Budget, Finance, and Management estimates a net 
administrative savings of less than 15 work years and $2 million 
annually.

Paperwork Reduction Act

    This rule does not create any new or affect any existing 
collections and, therefore, does not require OMB approval under the 
Paperwork Reduction Act.

References

    We consulted the following references when we developed this 
proposed rule:

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(Catalog of Federal Domestic Assistance Program Nos. 96.001, Social 
Security--Disability Insurance; 96.002, Social Security--Retirement 
Insurance; 96.004, Social Security--Survivors Insurance; and 96.006, 
Supplemental Security Income).

List of Subjects in 20 CFR Part 404

    Administrative practice and procedure; Blind, Disability benefits; 
Old-age, survivors, and disability insurance; Reporting and 
recordkeeping requirements; Social Security.

    The Acting Commissioner of Social Security, Kilolo Kijakazi, Ph.D., 
M.S.W., having reviewed and approved this document, is delegating the 
authority to electronically sign this document to Faye I. Lipsky, who 
is the primary Federal Register Liaison for the Social Security 
Administration, for purposes of publication in the Federal Register.

Faye I. Lipsky,
Federal Register Liaison, Office of Legislation and Congressional 
Affairs, Social Security Administration.

    For the reasons set forth in the preamble, we propose to amend 
subpart P of part 404 of title 20 of the Code of Federal Regulations as 
set forth below:

PART 404--FEDERAL OLD-AGE, SURVIVORS AND DISABILITY INSURANCE 
(1950- )

0
1. The authority citation for subpart P of part 404 continues to read 
as follows:

    Authority:  Secs. 202, 205(a)-(b), and (d)-(h), 216(i), 221(a) 
and (h)-(j), 222(c), 223, 225, and 702(a)(5) of the Social Security 
Act (42 U.S.C. 402, 405(a)-(b), and (d)-(h), 416(i), 421(a) and (h)-
(j), 422(c), 423, 425, and 902(a)(5)); sec. 211(b), Pub. L. 104-193, 
110 Stat. 2105, 2189; sec. 202, Pub. L. 108-203, 118 Stat. 509 (42 
U.S.C. 902 note).

0
2. Amend appendix 1 to subpart P of part 404 by:
0
a. Revising item 5 of the introductory text before part A;
0
b. Revising the body system name for section 4.00 in the table of 
contents;
0
c. Revising the heading of 4.00A, the heading of 4.00A1, the 
introductory text of 4.00A1b, and 4.00A1b(iv), 4.00A2, and the first 
sentence of 4.00A3a; and adding two sentences to 4.00A3b, and two 
sentences to 4.00A3c; and removing 4.00A3f;
0
d. Revising the heading of 4.00B, the first sentence of 4.00B1, the 
third sentence of 4.00B2, the second and fourth sentences of 4.00B3(a), 
the second sentence of 4.00B3(b), and the first sentence of 4.00B5;
0
e. Revising the heading of 4.00C, the second sentence of 4.00C3(c), 
4.00C6(a)(i), 4.00C7(b) through (d), 4.00C8(d)(iv), 4.00C8(e), and 
4.00C9(a); removing the third sentence of 4.00C15(a), revising 
4.00C15(b), removing 4.00C15(c); and revising the first two sentences 
of 4.00C16;
0
f. Revising the heading of 4.00D; adding new fourth and fifth sentences 
to 4.00D1a; revising the second sentence of 4.00D1b; adding a third 
sentence to 4.00D1b; revising 4.00D2(a)(i) through (iii), the first 
sentence of 4.00D2(b), the second sentence of 4.00D2(b)(i), the second 
sentence of 4.00D2(b)(ii), the third sentence of 4.00D3, 4.00D4(b) and 
4.00D4(c) and the first, second, and fifth sentences of 4.00D4(d); and 
adding 4.00D4(e);
0
g. Revising the heading of 4.00E, 4.00E2b; the first sentence of 
4.00E5, 4.00E7(b)(i)-(ii); and adding 4.00E7(b)(iii);
0
h. Revising the first three sentences of 4.00E8, and4.00E9(b) through 
(f), and removing 4.00E9(g) and (h);
0
i. Revising the heading of 4.00F; adding a new sentence to 4.00F1, 
revising the first sentence of 4.00F3(a), 4.00F4(a), and the second and 
fourth sentences of 4.00F4(b);
0
j. Revising the heading of 4.00G; adding a fourth sentence to 4.00G1, 
revising 4.00G2, the first two sentences of 4.00G4(b), 4.00G6, and the 
fourth sentence of 4.00G7(b);
0
k. Redesignating 4.00H and I as 4.00I and J, respectively
0
l. Adding a new 4.00H;
0
m. Revising the heading of 4.00I; 4.00I1, removing 4.00I2, and 
redesignating 4.00I3 through 5 as 4.00I2 through 4;
0
n. Revising 4.00I2, 4.00I3, 4.00I4(a) and (d), adding a new 4.00I5, and 
revising the third sentence of 4.00I8b;
0
o. Revising the heading of 4.00J, 4.00J1, the first sentence of 4.00J2, 
redesignating 4.00J3 as 4.00K, and adding new 4.00J3;
0
p. Revising 4.00K;
0
q. Revising listings 4.01 and 4.02, adding and reserving listing 4.03, 
revising listings 4.05 and 4.06, adding listing 4.07, adding listing 
4.08, revising 4.09 through 4.12, adding and reserving listings 4.13 
through 4.15, and adding listing 4.16.
0
r. Revising the heading of 104.00A; the heading of 104.00A1; the 
introductory text of 104.00A1(b), 104.00A1(b)(iv), and 104.00A2; and 
the first sentence of 104.00A3(a), and adding two sentences to 
104.00A3(b), adding two sentences to 104.00A3(c), and removing 
104.00A3(f) and (g);
0
s. Revising the heading of 104.00B; the first sentence of 104.00B1; the 
third sentence of 104.00B2; the second and fourth sentences of 
104.00B3(a); the second sentence of 104.00B3(b); 104.00B4(a)(i); the 
first and third sentences of 104.00B5; the heading of 100.04B7; the 
second sentence of 100.04B7(a); and the first sentence of 104.00B7(b);
0
t. Revising the heading of 104.00C; the heading of 104.00C1, and the 
first sentence of 104.00C1a; adding two sentences to 104.00C1a; 
revising 104.00C1b; 104.00C2(a);.00C2(b), and the second sentence of 
104.00C2(b)(iii); and adding 104.00C4;
0
u. Revising the heading of 104.00D; 104.00D1, 104.00D1d, and 104.00D2; 
and adding 104.00D3 and 104.00D4;
0
v. Revising the heading of 104.00E; adding a new sentence to the end of 
104.00E1; revising the fourth and fifth sentences of 104.00E4(a), and 
the fourth sentence of 104.00E4(b);

[[Page 38859]]

0
w. Revising the heading of 104.00F; the last sentence of 104.00F1, the 
first sentence of 104.00F2; removing the fourth through seventh 
sentences of 104.00F3; adding a new 104.00F3a and 104.00F3b; revising 
104.00F4, 104.00F5(a), 104.00F5(d), 104.00F6, and 104.00F9b; and adding 
104.00F11;
0
x. Revising the heading of 104.00G; 104.00G1, the first sentence of 
104.00G2; redesignating 104.00G3 as 104.00H, 104.00G3(a) as 104.00H1, 
and 104.00G3(b) as 104.00H2; and adding a new 104.00G3;
0
y. Revising 104.00H; and
0
z. Revising listings 104.01,104.02; adding and reserving 104.03 and 
104.04; revising 104.05 and 104.06; adding and reserving 104.07 and 
104.08; revising 104.09; adding and reserving listings 104.10 through 
104.12; removing and reserving listing 104.13; adding and reserving 
listings 104.14 and 104.15: and adding listing 104.16.
    The additions and revision to read as follows:

Appendix 1 to Subpart P of Part 404--Listing of Impairments

* * * * *
    5. Cardiovascular Disorders (4.00 and 104.00) [DATE 5 YEARS FROM 
THE EFFECTIVE DATE OF THE FINAL RULE].
* * * * *

Part A

* * * * *
    4.00 Cardiovascular Disorders.
* * * * *

4.00 Cardiovascular Disorders

    A. How do we define cardiovascular disorders and cardiovascular 
terms?
    1. What do we mean by a cardiovascular disorder?
    a. * * *
    b. Cardiovascular disorders result from one or more of four 
consequences of heart disease:
    (i) * * *
    (ii) * * *
    (iii) * * *
    (iv) Hypoxemia due to right-to-left shunt, reduced oxygen 
concentration in the arterial blood, or pulmonary vascular disease.
    c. * * *
    2. What do we consider in evaluating cardiovascular disorders? 
The listings in this section describe cardiovascular disorders based 
on the medical and other evidence, including response to a regimen 
of prescribed treatment and functional limitations.
    3. * * *
    a. Medical consultant is a person defined in Sec. Sec.  
404.1616(a) and 416.1016(a) of this chapter. * * *
    b. * * * By ``exceptions,'' we mean brief periods when the 
required finding(s) is greatly reduced or gone. These periods are so 
brief or inconsequential, the required finding(s) remains a factor 
in the person's condition.
    c. * * * By ``improvement of sufficient duration,'' we mean the 
finding is greatly reduced or not present for long enough that the 
required finding(s) is no longer a factor in the person's condition.
* * * * *
    f. [Removed]
    B. What documentation do we need to evaluate cardiovascular 
disorders?
    1. What basic documentation do we need? We need sufficiently 
detailed reports of history, physical examinations, laboratory 
studies, and any prescribed treatment and response to allow us to 
assess the severity and duration of your cardiovascular disorder.
* * * * *
    2. Why is a longitudinal clinical record important? * * * 
Whenever there is evidence of such treatment, your longitudinal 
clinical record should include a description of the ongoing 
management and evaluation provided by your medical source(s). * * *
    3. * * *
    a. * * * In this situation, we will base our evaluation on the 
current evidence we have. * * * However, we may find you disabled 
because you have another impairment(s) that, in combination with 
your cardiovascular disorder, medically equals a listing or based on 
consideration of your residual functional capacity and age, 
education, and work experience.
    b. * * * In rare instances when there is no or insufficient 
longitudinal evidence, we may purchase a consultative examination(s) 
to help us establish the existence, severity, and duration of your 
impairment.
* * * * *
    5. Will we purchase any studies? In appropriate situations, we 
may purchase studies necessary to substantiate the existence of a 
medically determinable impairment or to document the severity of 
your impairment, generally after we have evaluated the evidence we 
already have. * * *
* * * * *
    C. How do we use cardiovascular test results?
* * * * *
    3. * * *
    c. * * * In this test, you walk on a treadmill, usually for a 
specified period of time, and the person who administers the test 
measures the effect of exercise on the flow of blood in your legs, 
usually by using ultrasound. * * *
* * * * *
    6. * * *
    a. * * *
    (i) There is a question whether your cardiovascular disorder 
meets or medically equals the severity of one of the listings, or 
there is no timely test in the evidence we have (see 4.00C9), and we 
cannot find you disabled on some other basis; or
* * * * *
    7. * * *
    a. * * *
    b. If you are under the care of a medical source (see Sec. Sec.  
404.1502 and 416.902 of this chapter) for a cardiovascular disorder, 
this source has not performed an exercise test, and there are no 
reported significant risks to testing, we will request a statement 
from that source explaining why it was not done or should not be 
done before we decide whether we will purchase the test.
    c. The MC, in accordance with the regulations and other 
instructions on consultative examinations, will generally not 
override the medical source's conclusion about the risk of exercise 
testing to you. In the rare situation in which the MC does override 
the medical source's conclusion, the MC must prepare a written 
rationale documenting the reasons for overriding the conclusion.
    d. If you do not have a medical source or we cannot obtain a 
statement from your medical source, the MC is responsible for 
assessing the risk of exercise testing based on a review of the 
records we have before purchasing an exercise test for you.
* * * * *
    8. * * *
    d. * * *
    (iv) Percutaneous transluminal coronary angioplasty (PTCA) or 
percutaneous coronary intervention (PCI) with or without stenting.
    e. If you are deconditioned after an extended period of bedrest 
or inactivity and could improve with activity, or if you are in 
acute heart failure and are expected to improve with treatment, we 
will wait an appropriate period of time until you are ready and 
there are no medical reasons that prevent us from purchasing an 
exercise test.
    9. * * *
    a. We consider exercise test results to be timely for 12 months 
after the date they are performed, provided there has been no change 
in your clinical status that may alter the severity of your 
cardiovascular disorder.
* * * * *
    15. * * *
    a. * * *
    b. Cardiac catheterization reports commonly include evaluation 
of coronary artery size and flow patterns, pressures in the left and 
right side of the heart, and evaluation of wall motion and ejection 
fraction, as well as chamber size. Also more routinely included in 
the catheterization report is fractional flow reserve (FFR), which 
is an objective measure of flow access across an obstruction. FFR 
also helps define the adequacy of collateral flow that directly 
affects function in ischemic heart disease.
    16. What details should exercise Doppler test reports contain? 
The reports of exercise Doppler tests must describe the level of 
exercise; for example, the speed and grade of the treadmill 
settings, the duration of exercise, changes in the person's 
condition during exercise, and the reasons for stopping exercise if 
the expected level of exercise was not attained. These reports must 
also provide the blood pressures at the ankle and other pertinent 
sites measured after exercise, and also provide the time required 
for the systolic blood pressure to return toward or to the pre-
exercise level. * * *
* * * * *
    D. How do we evaluate chronic heart failure?
    1. * * *
    a. * * * Ejection fraction in heart failure is a continuum 
ranging from low ejection

[[Page 38860]]

fraction due to muscle dysfunction to preserved ejection fraction 
resulting from high intracardiac pressures. We consider heart 
failure to be chronic when the condition persists or recurs over 
time despite treatment. * * *
    b. * * * If the CHF is the result of primary pulmonary 
hypertension secondary to disease of the lung, we evaluate your 
impairment under the listings in 3.00 (for example, 3.09) or 4.00, 
as appropriate. For the purposes of 4.02B3, a finding of elevated B-
type natriuretic peptide (BNP) or N-terminal pro-B-type natriuretic 
peptide (NT-pro-BNP) in the blood assists in differentiating chronic 
heart failure from non-heart failure symptoms.
    2. * * *
    a. * * *
    (i) Abnormal cardiac imaging provides objective measures of both 
left ventricular function and structural abnormality in heart 
failure. Examples of abnormal findings include increased left 
ventricular end diastolic dimension (LVEDD), decreased EF, increased 
left atrial chamber size, increased left atrial volume index (LAVi), 
increased ventricular filling pressures measured at cardiac 
catheterization, or increased left ventricular wall or septum 
thickness.
    (ii) An LVEDD equal to or greater than 7.0 cm, or an EF of 30 
percent or less during a period of stability (that is, not during an 
episode of acute heart failure) may be associated clinically with 
systolic dysfunction.
    (iii) LAVi is measured in milliliters (ml) indexed to body 
surface area (BSA) measured in squared meters (m\2\). Indexing is a 
method of standardizing measurements to different body sizes. 
Diastolic dysfunction may be clinically associated with LAVi of 40 
ml, BSA/m\2\ or greater. The imaging report will contain a 
measurement for the left atrium volume. The index is calculated by 
dividing the left atrium volume by BSA.
* * * * *
    b. Your medical history and physical examination should describe 
characteristic symptoms and signs of pulmonary or systemic 
congestion (fluid retention) or of limited cardiac output associated 
with the abnormal findings on appropriate medically acceptable 
imaging. * * *
    (i) * * * People with CHF may also experience shortness of 
breath upon lying flat (orthopnea) or episodes of shortness of 
breath that wake them from sleep (paroxysmal nocturnal dyspnea). * * 
*
    (ii) * * * However, these signs need not be found on all 
examinations because congestion may be controlled by prescribed 
treatment or may not be present at the time of evaluation.
    3. Is it safe for you to have an ETT if you have CHF? * * * ETT 
has been used safely in people with CHF. Therefore, we may purchase 
an ETT for evaluation under 4.02B2 if an MC, preferably one 
experienced in the care of patients with cardiovascular disease, 
determines that the test poses no significant risk to you. * * *
    4. * * *
    a. * * *
    b. To meet the required level of severity for this listing, your 
impairment must satisfy the requirements of the criteria in A and B 
or satisfy either C or D.
    c. In 4.02B1, we follow a two-part process to evaluate your 
impairment. Your impairment must satisfy the requirements in the 
first part of this process before we will move to the second part.
    (i) Your impairment satisfies the first part if a medical source 
has concluded that the performance of an ETT would present a 
significant risk to you. This medical source, such as a 
cardiologist, may be providing your care. If your case record does 
not include a conclusion from a medical source that an ETT would 
present a significant risk to you, an MC as defined in 4.00A3a may 
make such a conclusion if evidence in your case record supports it.
    (ii) In the second part of the process, we will evaluate 
activities of daily living (ADL). ADLs include, but are not limited 
to, such activities as doing household chores, grooming and hygiene, 
shopping at a grocery store, taking public transportation, or paying 
bills. We will assess whether you have persistent symptoms of 
chronic heart failure (for example, easy fatigue, weakness, 
shortness of breath, or chest discomfort) at rest or with activity 
that very seriously limit your ability to perform ADLs 
independently, appropriately, effectively, and on a sustained basis. 
Even if you are able to perform some ADLs, we may find your ability 
is very seriously limited and that your impairment satisfies the 
second part of the evaluation.
    d. Listing 4.02B2b requires a decrease in systolic blood 
pressure below the baseline level or below any systolic pressure 
reading recorded during exercise. We have this requirement because, 
normally, systolic blood pressure and heart rate increase gradually 
with exercise. * * * Also, some people with increased sympathetic 
responses because of deconditioning or apprehension may increase 
their systolic blood pressure and heart rate above their baseline 
level just before and early into exercise. * * *
    e. How do we evaluate CHF treated with a mechanical circulatory 
support device? We use 4.02D1 to evaluate CHF treated with an 
implanted mechanical circulatory support device (MCSD), such as a 
left ventricle assistive device (LVAD) or a right ventricle 
assistive device (RVAD). Implanted MCSDs are intended for long-term 
circulatory support in helping the heart pump blood. For the 
purposes of 4.02D1, an MCSD does not include extracorporeal membrane 
oxygenation (ECMO). Although ECMO is a form of mechanical 
circulatory support, we do not include it in 4.02D1 because ECMO is 
intended only for short-term circulatory support (maximum 30 days), 
used in a setting of imminent or actual cardiac arrest.
    E. How do we evaluate ischemic heart disease?
    1. * * *
    2. * * *
    a. * * *
    b. Instead of typical angina pectoris, some people with IHD 
experience atypical angina, anginal equivalent, variant angina, or 
silent ischemia, all of which we may evaluate using 4.04. We discuss 
the various manifestations of ischemia in 4.00E3-4.00E7.
* * * * *
    5. What is anginal equivalent? Often, people with IHD will 
complain of shortness of breath (dyspnea) on exertion without chest 
pain or discomfort. * * *
* * * * *
    7. * * *
    a. * * *
    b. * * *
    (i) People with documented past myocardial infarction or 
established angina without prior infarction who do not have chest 
pain on ETT, but have a positive test with ischemic abnormality on 
ECG, perfusion scan, or other appropriate medically acceptable 
imaging.
    (ii) People with documented past myocardial infarction or angina 
who have ST segment changes on ambulatory monitoring (Holter 
monitoring) that are similar to those that occur during episodes of 
angina. ST depression shown on the ambulatory recording should not 
be interpreted as positive for ischemia unless similar depression is 
also seen during chest pain episodes annotated in the diary that the 
person keeps while wearing the Holter monitor.
    (iii) People who have diabetes mellitus with neuropathy. People 
with diabetes mellitus can have a higher threshold for pain because 
of the neuropathy and may not feel chest pain or discomfort from 
cardiac ischemia.
* * * * *
    8. What other sources of chest discomfort are there? Chest 
discomfort of nonischemic origin may result from other 
cardiovascular disorders, such as pericarditis. Noncardiac disorders 
may also produce symptoms mimicking that of myocardial ischemia. 
These disorders include acute anxiety or panic attacks, 
gastrointestinal tract disorders, such as esophageal spasm, 
esophagitis, hiatal hernia, biliary tract disease, gastritis, peptic 
ulcer, and pancreatitis, and musculoskeletal syndromes, such as 
chest wall muscle spasm, chest wall syndrome (especially after 
coronary bypass surgery), costochondritis, and cervical or dorsal 
spine arthritis. * * *
    9. * * *
    a. * * *
    b. In 4.04A, we need evidence, such as an ECG interpretation, 
from an acceptable medical source who reviewed your ETT findings and 
found them positive for ischemia. These ETT findings may include ECG 
tracings or systolic blood pressure measurements. If your case 
record does not have such an interpretation from an acceptable 
medical source, an MC, as defined in 4.00A3a, may review your ETT 
findings and interpret them as being positive for ischemia if 
evidence in your case record supports it.
    (i) ETT findings may show the classically accepted changes in 
ECG tracings of horizontal or down sloping ST depression or of ST 
elevation. For example, ECG tracings may show horizontal or down 
sloping depression, in the absence of digitalis glycoside treatment 
or hypokalemia, of the ST segment of at least -0.10 millivolts (-1.0 
mm) in at least three consecutive complexes that are on a level 
baseline in any lead other than a VR, and depression of at least -
0.10 millivolts last for at least 1 minute of

[[Page 38861]]

recovery. Alternatively, the ECG tracings may show at least 0.10 
millivolt (1 mm) ST elevation above resting baseline in non-infarct 
leads during both exercise and 1 or more minutes of recovery.
    (ii) ETT findings may also show a decrease of 10 mmHg or more in 
systolic pressure below the baseline systolic blood pressure or the 
preceding systolic pressure measured during exercise due to left 
ventricular dysfunction, despite an increase in workload. This 
finding is the same finding required in 4.02B2b. See 4.00D4d for 
full details.
    c. In 4.04C, each ischemic episode must result in an unplanned 
hospitalization. Examples of ischemic episodes that may result in 
unplanned hospitalizations include unplanned revascularizations, 
myocardial infarctions, unstable angina, or dysrhythmias. 
Revascularization means angioplasty (with or without stent 
placement) or bypass surgery.
    (i) How do we calculate separate ischemic episodes? Reocclusion 
that occurs after a revascularization procedure but during the same 
hospitalization and that requires a second procedure during the same 
hospitalization will not be counted as another ischemic episode. If 
you are hospitalized for documented myocardial infarction and have a 
revascularization procedure during the same hospitalization, this 
event will be counted as one ischemic episode.
    (ii) How do we evaluate ischemic episodes not amenable to 
revascularizations? If your ischemic episodes are not amenable to 
revascularization, we will evaluate them using the appropriate 
listing (for example, 4.04D). Not amenable means that the 
revascularization procedure could not be done because of another 
medical impairment or because the vessel was not suitable for 
revascularization.
    d. We will use 4.04D only when you have symptoms due to 
myocardial ischemia as described in 4.00E3-4.00E7 while on a regimen 
of prescribed treatment, you are at risk for ETT (see 4.00C8), and 
we do not have a timely ETT or a timely normal drug-induced stress 
test for you. See 4.00C9 for what we mean by a timely test.
    e. In 4.04D1, the term fractional flow reserve (FFR) is a 
measurement of the pressure differences across an obstructive 
lesion, giving an estimate of the severity of stenosis. An FFR 
measurement of 1.0 indicates normal blood flow. An FFR measurement 
equal to or less than 0.80 indicates stenosis capable of producing 
serious myocardial ischemia in an artery appropriate for 
revascularization. An FFR measurement that is greater than 0.80 
indicates stenosis not likely to produce significant ischemia.
    f. In 4.04D2 and 4.04D3, the term nonbypassed means that the 
blockage is in a vessel that is potentially bypassable; that is, 
large enough to be bypassed and considered to be a cause of your 
ischemia. These vessels are usually major arteries or one of a major 
artery's major branches. A vessel that has become obstructed again 
after angioplasty or stent placement and has remained obstructed or 
is not amenable to another revascularization is considered a 
nonbypassed vessel for purposes of the listings. When you have had 
revascularization, we will not use the pre-operative findings to 
assess the current severity of your coronary artery disease under 
4.04D, although we will consider the severity and duration of your 
impairment before your surgery in making our determination or 
decision.
    F. How do we evaluate arrhythmias?
    1. What is an arrhythmia? * * * Although we use the term 
``arrhythmia'' in the listings, the term ``dysrhythmia'' may also be 
used in the medical evidence to describe this condition.
* * * * *
    3. * * *
    a. We will use 4.05 when you have arrhythmias that are not fully 
controlled by medication, an implanted pacemaker, or an implanted 
cardiac defibrillator, and you have recurrent episodes of syncope or 
near syncope. * * *
* * * * *
    4. * * *
    a. Implanted cardiac defibrillators are used to prevent sudden 
cardiac death in people who have had, or are at high risk for, 
cardiac arrest from life-threatening ventricular arrhythmias. The 
largest group at risk for sudden cardiac death consists of people 
with cardiomyopathy (ischemic or non-ischemic) and reduced 
ventricular function. However, life-threatening ventricular 
arrhythmias can also occur in people with little or no ventricular 
dysfunction. The shock from the implanted cardiac defibrillator 
rescues a person from what may have been cardiac arrest. However, as 
a consequence of the shock(s), similar to the effects of treatments 
for other cardiovascular disease, a person may experience 
psychological distress, which we may evaluate under the listings in 
12.00.
    b. * * * In some people, these functions may result in the 
termination of ventricular arrhythmias without an otherwise painful 
shock. * * * Also, exposure to strong electrical or magnetic fields, 
such as from magnetic resonance imaging, can trigger or reprogram an 
implanted cardiac defibrillator, resulting in inappropriate shocks. 
* * *
* * * * *
    G. How do we evaluate peripheral vascular disease?
    1. What is peripheral vascular disease (PVD)? * * * Neuropathy 
may mask these typical symptoms. * * *
    2. How do we assess limitations resulting from PVD? We will 
assess your limitations based on your symptoms together with 
physical findings and Doppler studies or other appropriate 
diagnostic techniques. However, if the PVD has resulted in 
amputation, we will evaluate any limitations related to the 
amputation under the listings in 1.00.
* * * * *
    4. * * *
    a. * * *
    b. Lymphedema does not meet the requirements of 4.11, although 
it may medically equal the listing. We will evaluate lymphedema by 
considering whether the underlying cause meets or medically equals 
any listing, or whether the lymphedema medically equals a 
cardiovascular disorders listing such as 4.11 or a listing in 1.00. 
* * *
    5. * * *
    6. Are there any other studies that are helpful in evaluating 
PAD? Doppler studies done using a recording ultrasonic Doppler unit 
and strain-gauge plethysmography are other useful tools for 
evaluating PAD. A recording Doppler, which prints a tracing of the 
arterial pulse wave in the femoral, popliteal, dorsalis pedis, and 
posterior tibia arteries, is an evaluation tool that compares 
waveforms in normal and compromised peripheral blood flow. 
Qualitative analysis of the pulse wave is helpful in the overall 
assessment of the severity of the occlusive disease. Tracings help 
in assessing severity if you have small vessel disease related to 
diabetes mellitus or other diseases with similar vascular changes, 
or diseases causing medial calcifications when ankle pressure is 
either normal or falsely high. When there is evidence of medial 
calcification of the ankle arteries or the ankle-brachial index is 
0.50 or greater, other appropriate tests for PAD include magnetic 
resonance angiography, computed tomography angiography, contrast 
angiography, and graded treadmill tests.
    7. * * *
    a. * * *
    b. * * * The criterion in 4.12A is met when your resting ankle/
brachial systolic blood pressure ratio is less than 0.50. * * *
* * * * *
    H. How do we evaluate congenital heart disease?
    1. What is congenital heart disease? Congenital heart disease is 
any abnormality of the heart or the major blood vessels that is 
present at birth. Congenital heart disease includes abnormal 
structure of the individual heart chambers, valves, and blood 
vessels, and abnormal relative relationship of the chambers to each 
other that alters the normal pattern of blood flow. Surgery in 
childhood is the usual treatment, and with improving surgical 
techniques and medical management, more children with congenital 
heart disease are surviving into adulthood. Rarely, a person with 
congenital heart disease may not have received the usual surgery in 
childhood, and later, as an adult, he or she is no longer a surgical 
candidate, as for example, in Eisenmenger syndrome.
    2. What is Eisenmenger syndrome? Eisenmenger syndrome refers to 
any surgically untreated congenital heart defect with intracardiac 
communication that over time leads to pulmonary hypertension, 
reversal of blood flow, and hypoxemia.
    a. Lesions in Eisenmenger syndrome, such as large septal 
defects, are characterized by elevated pulmonary pressures or a high 
pulmonary flow rate. In response, the pulmonary blood vessels 
pathologically change, leading eventually to pulmonary hypertension. 
Development of Eisenmenger syndrome represents a point at which 
pulmonary hypertension is irreversible and the cardiac lesion is 
likely inoperable.
    b. Examples of congenital heart disease that if untreated may 
cause pulmonary vascular disease leading to Eisenmenger syndrome 
include atrial septal defect (ASD), ventricular septal defect (VSD), 
and large patent ductus arteriosus (PDA).

[[Page 38862]]

    3. What is single ventricle? The term ``single ventricle'' (also 
known as single ventricle physiology or functional single ventricle) 
describes a diverse group of congenital cardiac anomalies sharing 
the common feature that only one of the two heart ventricles is 
adequately developed. At birth, one ventricle must functionally do 
the work of two, pumping blood for both the body (systemic) and the 
lungs (pulmonary). Because of this feature, the ultimate plan for 
cardiac reconstruction is similar for most of these anomalies. 
People with single ventricle will generally undergo staged 
reconstructive ``Fontan procedures,'' ultimately resulting in a 
``Fontan circulation.'' Fontan circulation describes the hemodynamic 
state in which virtually all systemic venous return-blood passively 
flows directly into the pulmonary arteries via surgical or catheter-
placed shunts, without the blood passing through a ventricle. Some 
of the anomalies described as single ventricle include the 
following:
    (a) Hypoplastic left heart syndrome;
    (b) Hypoplastic right ventricle;
    (c) Tricuspid valve atresia;
    (d) Double inlet left ventricle; and
    (e) Some variations of double outlet right ventricle.
    4. How do we evaluate conditions associated with congenital 
heart disease?
    a. We evaluate congenital heart disease that results in chronic 
heart failure with evidence of ventricular dysfunction or in 
recurrent arrhythmias under 4.02 or 4.05, respectively. Otherwise, 
we evaluate your impairment under 4.06.
    b. We evaluate pulmonary hypertension due to congenital heart 
disease under 4.06B or 4.06C. We evaluate pulmonary hypertension not 
due to congenital heart disease under the listings in 3.00 (for 
example, 3.09).
    c. We need pulse oximetry measurements documented by medical 
sources using methods consistent with the prevailing state of 
medical knowledge and clinical practice to evaluate chronic 
hypoxemia in congenital heart disease under 4.06A3. These pulse 
oximetry measurements also must be consistent with the other 
evidence in the case record.
    d. We evaluate single ventricle physiology under 4.06D and will 
consider you disabled if your medical evidence documents that you 
have any congenital heart disorder that results in single ventricle 
physiology (functional single ventricle). In addition to the above 
congenital heart disorders, examples of palliative surgical 
procedures that indicate single ventricle physiology include the 
Glenn, Fontan, and Norwood procedures.
* * * * *
    I. How do we evaluate other cardiovascular disorders?
    1. How do we evaluate hypertension? Hypertension (high blood 
pressure) over time may significantly raise the pressures in the 
heart to the point of ineffective heart muscle function known 
generally as hypertensive heart disease that we can evaluate under 
4.02. Other body systems, such as the brain, kidneys, or eyes may 
also be affected. We evaluate these impairments by reference to the 
specific body system(s) that is affected. We will also consider any 
limitations imposed by your hypertension when we assess your 
residual functional capacity.
    2. What is cardiomyopathy and how will we evaluate it? 
Cardiomyopathy is a disease of the heart muscle. The heart loses its 
ability to pump blood (heart failure), and in some instances, heart 
rhythm is disturbed, leading to irregular heartbeats (arrhythmias). 
Usually, the exact cause of the muscle damage is never found 
(idiopathic cardiomyopathy).
    a. There are various types of cardiomyopathy, which fall into 
two major categories: ischemic and nonischemic cardiomyopathy. 
Ischemic cardiomyopathy typically refers to heart muscle damage that 
results from coronary artery disease, including heart attacks. 
Nonischemic cardiomyopathy includes several types: dilated, 
hypertensive, hypertrophic, and restrictive. Cardiomyopathy includes 
hypertrophic cardiomyopathy, endomyocardial fibrosis, or cardiac 
amyloidosis AL type.
    b. We evaluate cardiomyopathy under 4.08. Depending on the 
underlying cause of the cardiomyopathy or its effects on you, we may 
also evaluate your cardiomyopathy under 4.02, 4.04, or 4.05. If your 
cardiomyopathy results in vascular insult to the brain, we may also 
evaluate it under 11.04.
    c. Under 4.08A2, we need a conclusion from a medical source that 
the performance of an exercise test would present a significant risk 
to you. If your case record does not have a conclusion from a 
medical source that an exercise test would present a significant 
risk to you, an MC defined in 4.00A3a may make such a conclusion if 
evidence in your case record supports it.
    3. How do we evaluate valvular heart disease? We evaluate aortic 
valvular disease under 4.07. We may also evaluate aortic valvular 
disease, as well as other forms of valvular disease, under 4.02, 
4.04, 4.05, 4.06, or a listing in 11.00, depending on its effects on 
you.
    4. What do we consider when we evaluate heart transplant 
recipients? a. After your heart transplant, we will consider you 
disabled under 4.09 for 1 year following the surgery because there 
is a greater likelihood of rejection of the organ and infection 
during the first year. If you develop cardiac allograft vasculopathy 
after your transplant, we will evaluate this impairment under 4.16.
    b. * * *
    c. * * *
    d. When we do a continuing disability review to determine 
whether you are still disabled, we will evaluate your residual 
impairment(s), as shown by the evidence in your case record, 
including any side effects of medication. We will consider all 
evidence indicative of cardiac dysfunction in deciding whether 
medical improvement (as defined in Sec. Sec.  404.1594 and 416.994 
of this chapter) has occurred.
    5. What is cardiac allograft vasculopathy and how do we evaluate 
it? Cardiac allograft vasculopathy (CAV) may affect a person who has 
received a heart transplant and involves thickening in the walls of 
the coronary arteries that may progress quickly into serious 
vascular stenosis and heart dysfunction. Stenosis in CAV is caused 
by a pathological process different from classic atherosclerosis and 
treatment often is only palliative. We evaluate CAV under 4.16.
* * * * *
    8. * * *
    a. * * *
    b. * * * Most people with Marfan syndrome have abnormalities 
associated with the heart and blood vessels. * * * * *
    J. How do we evaluate issues that affect the cardiovascular 
system? 1. How do we consider the effects of obesity when we 
evaluate your cardiovascular disorder? Obesity is a medically 
determinable impairment that may be associated with cardiovascular 
disorders. The additional body mass may make it harder for the chest 
and lungs to expand or may cause the heart to work harder to pump 
blood to carry oxygen to the body. The combined effects of obesity 
with a cardiovascular disorder can be greater than the effects of 
each of the impairments considered separately. We consider the 
additional and cumulative effects of obesity when we determine 
whether you have a severe cardiovascular disorder, a listing-level 
cardiovascular disorder, a combination of impairments that medically 
equals the severity of a listed impairment, and when we assess your 
residual functional capacity.
    2. How do we relate treatment to functional status? In general, 
conclusions about the severity of a cardiovascular disorder cannot 
be made on the basis of the type of treatment rendered or 
anticipated. * * *
    3. How do we consider hospitalizations? When we evaluate 
hospitalizations for chronic heart failure (4.02B3), ischemic heart 
disease (4.04E), congenital heart disease (4.06E), and 
cardiomyopathy (4.08D), the hospitalizations do not all have to be 
for the same cardiovascular disorder(s). They may be for three 
different exacerbations or complications resulting from your 
cardiovascular disorder. The hospitalizations must be at least 30 
days apart, and each one must last at least 48 hours, including 
hours in a hospital emergency department immediately before the 
hospitalization.
    K. How do we evaluate cardiovascular disorders that do not meet 
one of these listings?
    1. These listings are only examples of common cardiovascular 
disorders that we consider severe enough to prevent you from doing 
any gainful activity. If your impairment(s) does not meet the 
criteria of any of these listings, we must also consider whether you 
have an impairment(s) that satisfies the criteria of a listing in 
another body system.
    2. If you have a severe medically determinable impairment(s) 
that does not meet a listing, we will determine whether your 
impairment(s) medically equals a listing. See Sec. Sec.  404.1526 
and 416.926 of this chapter. If your impairment(s) does not meet or 
medically equal a listing, you may or may not have the residual 
functional capacity to engage in substantial gainful activity. We 
will proceed to the fourth step and, if necessary, the fifth step of 
the sequential evaluation process in Sec. Sec.  404.1520 and 416.920 
of this chapter. We will use the rules in Sec. Sec.  404.1594 or 
416.994 of this chapter, as appropriate,

[[Page 38863]]

when we decide whether you continue to be disabled.

4.01 Category of Impairments, Cardiovascular Disorders

    4.02 Chronic heart failure (see 4.00D) while on a regimen of 
prescribed treatment, with symptoms and signs described in 4.00D2. 
The required level of severity for this impairment is met when the 
requirements are satisfied by A and B; or C alone; or D alone.
    A. Medically documented presence of one of the following:
    1. Systolic failure documented by appropriate medically 
acceptable imaging during a period of stability (not during an 
episode of exacerbation of heart failure), with left ventricular end 
diastolic dimension equal to or greater than 7.0 cm; or ejection 
fraction of 30 percent or less during a period of stability (not 
during an episode of acute heart failure); OR
    2. Diastolic failure documented by appropriate medically 
acceptable imaging during a period of stability (not during an 
episode of exacerbation of heart failure), with left ventricular 
posterior wall plus septal thickness totaling 2.5 cm or greater, 
with an enlarged left atrium greater than or equal to 4.5 cm, OR 
left atrial volume index (LAVi) greater than or equal to 40 ml, BSA/
m2 (milliliters to body surface area in squared meters).
    AND
    B. Resulting in one of the following:
    1. Recurrent (see 4.00A3c) symptoms of heart failure, resulting 
in both a and b:
    a. A medical source (see 4.00D4c(i)) has concluded that the 
performance of an exercise test would present a significant risk to 
the person; and
    b. Very serious limitation in the ability to perform activities 
of daily living independently, appropriately, effectively, and on a 
sustained basis; or
    2. Inability to perform on an exercise tolerance test at a 
workload equivalent to 5 METs or less if using a standard treadmill 
(or bicycle) test without gas exchange, or at 15 ml/kg/min peak VO2 
(oxygen consumption) on a cardiopulmonary exercise test, due to 
either a or b:
    a. Dyspnea, fatigue, palpitations, or chest discomfort; or
    b. Decrease of 10 mmHg or more in systolic pressure below the 
baseline systolic blood pressure or the preceding systolic pressure 
measured during exercise (see 4.00D4d) due to left ventricular 
dysfunction, despite an increase in workload; or
    3. Exacerbations or complications of chronic heart failure (see 
4.00D1b) requiring three hospitalizations within a consecutive 12-
month period (see 4.00A3e) and at least 30 days apart. Each 
hospitalization must last at least 48 hours, including hours in a 
hospital emergency department immediately before the hospitalization 
(see 4.00J3);
    OR
    C. Heart failure with left ventricular ejection fraction of 20 
percent or less while on a regimen of prescribed therapy, on two 
evaluations at least 90 days apart within a consecutive 12-month 
period (see 4.00A3e) during a period of stability (not during an 
episode of exacerbation of heart failure);
    OR
    D. One of the following while hospitalized, at home, or both:
    1. Mechanical circulatory support device except extracorporeal 
membrane oxygenation (ECMO) (see 4.00D4e). Consider under a 
disability for 1 year from the date of implantation; after that, 
evaluate any residual impairment(s) under the criteria for the 
affected body system.
    2. Continuous intravenous administration of inotropic medication 
(for example, milrinone) for at least 30 consecutive days. Consider 
under a disability for 1 year from the date of initiation of the 
treatment; after that, evaluate any residual impairment(s) under the 
criteria for the affected body system.
    4.03 [Reserved]
    4.04 Ischemic heart disease (see 4.00E), with symptoms due to 
myocardial ischemia, while on a regimen of prescribed treatment (see 
4.00B3 if there is no regimen of prescribed treatment), with A, B, 
C, D, or E:
    A. Inability to perform on an exercise tolerance test at a 
workload equivalent to 5 METs or less with findings interpreted by 
an acceptable medical source as positive for ischemia (see 4.00E9b).
    OR
    B. Ischemic response with exercise or pharmacological (drug-
induced) stress testing (see 4.00C14) on medically appropriate 
imaging, with either 1 or 2:
    1. At least two reversible or fixed regional myocardial 
perfusion defects and either a or b:
    a. Transient ischemic dilatation; or
    b. Resting left ventricular ejection fraction of less than 50 
percent; or
    2. At least two reversible or fixed regional wall motion 
abnormalities and either a or b:
    a. Decrease in left ventricular ejection fraction during 
testing; or
    b. Resting left ventricular ejection fraction of less than 50 
percent.
    OR
    C. Documentation of three separate ischemic episodes (see 
4.00E9c) requiring unplanned hospitalization (inpatient or 
observation status) within a consecutive 12-month period (see 
4.00A3e).
    OR
    D. Coronary artery disease, documented by coronary angiography 
(obtained independently of Social Security disability evaluation) 
with 1, 2, or 3:
    1. Fractional flow reserve (see 4.00E9e) measurement of less 
than or equal to 0.80 of a proximal segment or mid segment coronary 
artery not amenable to revascularization (see 4.00E9c(ii)).
    2. History of coronary artery bypass graft surgery with 
manifestations of ischemia, as described in 4.00E3-4.00E7, while on 
a regimen of prescribed treatment (see 4.00B3 if there is no regimen 
of prescribed treatment) with a, b, c, or d:
    a. 50 percent or more stenosis of a nonbypassed left main 
coronary artery; or
    b. 70 percent or more stenosis in the proximal segment or mid 
segment of another nonbypassed coronary artery; or
    c. 50 percent or more stenosis in the proximal segment or mid 
segment of at least two nonbypassed coronary arteries; or
    d. 70 percent or more stenosis of a bypass graft vessel.
    3. Resting left ventricular ejection fraction of less than 50 
percent while medically stable (see 4.00B4) with manifestations of 
ischemia, as described in 4.00E3-4.00E7, while on a regimen of 
prescribed treatment (see 4.00B3 if there is no regimen of 
prescribed treatment) with a, b, or c:
    a. 50 percent or more stenosis of a nonbypassed left main 
coronary artery; or
    b. 70 percent stenosis in the proximal segment or mid segment of 
another nonbypassed coronary artery; or
    c. 50 percent or more stenosis in the proximal segment or mid 
segment of at least two nonbypassed coronary arteries.
    OR
    E. Exacerbations or complications of ischemic heart disease (see 
4.00E2-4.00E7) requiring three hospitalizations within a consecutive 
12-month period (see 4.00A3e) and at least 30 days apart. Each 
hospitalization must last at least 48 hours, including hours in a 
hospital emergency department immediately before the hospitalization 
(see 4.00J3).
    4.05 Recurrent arrhythmias (see 4.00F), not related to 
reversible causes such as electrolyte abnormalities or digitalis 
glycoside or antiarrhythmic drug toxicity, while on a regimen of 
prescribed treatment (see 4.00B3 if there is no prescribed 
treatment), demonstrated by both A and B:
    A. Coincident with recurrent (see 4.00A3c) episodes of cardiac 
syncope or near syncope (see 4.00F3b).
    AND
    B. Documented by either 1 or 2:
    1. Resting or ambulatory (Holter) electrocardiography; or
    2. Other appropriate medically acceptable testing.
    4.06 Congenital heart disease (see 4.00H), documented by 
appropriate medically acceptable imaging (see 4.00A3d) or cardiac 
catheterization, with A, B, C, D, or E:
    A. Chronic hypoxemia, and 1, 2, or 3:
    1. Hematocrit of 55 percent or greater on two evaluations at 
least 90 days apart within a consecutive 12-month period (see 
4.00A3e); or
    2. Arterial blood gas test measurement obtained at rest while 
breathing room air, as described in either a or b:
    a. S<INF>a</INF>O<INF>2</INF> (arterial oxygen saturation) less 
than or equal to 89 percent; or
    b. PO<INF>2</INF> or P<INF>a</INF>O<INF>2</INF> (partial 
pressure of oxygen) less than or equal to 60 mmHg;
    3. S<INF>p</INF>O<INF>2</INF> (percentage of oxygen saturation 
of blood hemoglobin) measured by pulse oximetry either at rest, 
during a 6-minute walk test (6MWT), or after a 6MWT, while breathing 
room air, less than or equal to 87 percent on three evaluations at 
least 30 days apart within a consecutive 12-month period (see 
4.00A3e).
    OR
    B. Intermittent right-to-left shunting (for example, Eisenmenger 
syndrome; see 4.00H2) during cardiopulmonary exercise testing while 
breathing room air, resulting in oxygen desaturation on exertion at 
a workload equivalent to 5 METs or less, or peak VO<INF>2</INF> 
(oxygen uptake) of 15.0 ml/kg/min or less, and arterial blood gas 
test measurement, with either 1 or 2:

[[Page 38864]]

    1. S<INF>a</INF>O<INF>2</INF> less than or equal to 89 percent; 
or
    2. PO<INF>2</INF> or P<INF>a</INF>O<INF>2</INF> less than or 
equal to 60 mmHg.
    OR
    C. Pulmonary hypertension documented by cardiac catheterization 
while medically stable, as described in 1, 2, or 3:
    1. Pulmonary arterial systolic pressure elevated to at least 70 
percent of the systemic arterial systolic pressure; or
    2. Pulmonary arterial systolic pressure equal to or greater than 
70 mmHg; or
    3. Mean pulmonary artery pressure equal to or greater than 
40mmHg.
    OR
    D. Single ventricle (with or without Fontan procedures) (see 
4.00H4).
    OR
    E. Exacerbations or complications of congenital heart disease 
(see 4.00J3) requiring three hospitalizations within a consecutive 
12-month period (see 4.00A3e) and at least 30 days apart. Each 
hospitalization must last at least 48 hours, including hours in a 
hospital emergency department immediately before the hospitalization 
(see 4.00J3).
    4.07 Aortic valvular disease (see 4.00I3), with symptoms due to 
stenosis, determined by appropriate test or tests showing an aortic 
valve area of less than 1.0 cm\2\.
    4.08 Cardiomyopathy (see 4.00I2) while on a regimen of 
prescribed treatment, with A, B, C, or D:
    A. Hypertrophic cardiomyopathy documented by appropriate 
medically acceptable imaging, with left ventricular or septal wall 
thickness equal to or greater than 20 mm in the absence of other 
causes of left ventricular hypertrophy (for example, hypertension or 
aortic valvular disease) and either 1 or 2:
    1. Inability to perform on an exercise tolerance test at a 
workload equivalent to 5 METs or less if using a standard treadmill 
(or bicycle) test without gas exchange, or at 15 ml/kg/min peak 
VO<INF>2</INF> (oxygen consumption) on a cardiopulmonary exercise 
test; or
    2. A medical source (see 4.00I2c) has concluded that the 
performance of an exercise tolerance test would present a 
significant risk to the person.
    OR
    B. Endomyocardial fibrosis documented by appropriate medically 
acceptable imaging, with 1, 2, and 3:
    1. Loss of chamber volume due to fibrosis of the endocardium of 
at least one ventricle; and
    2. Right or left atrial dilatation (chamber enlargement); and
    3. Regurgitant (backward) blood flow through the mitral or 
tricuspid valve.
    OR
    C. Cardiac amyloidosis AL (light-chain) type documented by 
biopsy.
    OR
    D. Exacerbations or complications of cardiomyopathy requiring 
three hospitalizations within a consecutive 12-month period (see 
4.00A3e) and at least 30 days apart. Each hospitalization must last 
at least 48 hours, including hours in a hospital emergency 
department immediately before the hospitalization (see 4.00J3).
    4.09 Heart transplantation (see 4.00I4). Consider under a 
disability for 1 year from the date of the transplant; after that, 
evaluate the residual impairment(s).
    4.10 Dissecting aneurysm of the aorta or major branches (see 
4.00I6), due to any cause (for example, atherosclerosis, cystic 
medical necrosis Marfan syndrome, or trauma), demonstrated by 
appropriate medically acceptable imaging, with dissection not 
controlled by prescribed treatment.
    4.11 Chronic venous insufficiency (see 4.00G) of a lower 
extremity with reflux or obstruction of the venous system documented 
by duplex ultrasound or other appropriate diagnostic technique, with 
A or B:
    A. Extensive trophic changes of skin (for example, 
hyperpigmentation, lipodermatosclerosis, brawny edema) involving at 
least two-thirds of the leg below the knee, on two evaluations at 
least 90 days apart within a consecutive 12-month period (see 
4.00A3e), with both 1 and 2:
    1. Consistent with chronic venous insufficiency; and
    2. Unresponsive to compression therapy.
    OR
    B. Two or more episodes of ulceration that has not healed 
following at least 6 months of prescribed treatment.
    4.12 Peripheral arterial disease (see 4.00G7) while on a regimen 
of prescribed treatment resulting in intermittent claudication or 
leg pain that interferes with mobility (see 4.00G1), with A, B, C, 
or D, as determined by an appropriate test(s) (see 4.00G5-4.00G6):
    A. Resting ankle/brachial systolic blood pressure ratio of less 
than 0.50 (see 4.00G7a).
    OR
    B. Decrease in systolic blood pressure at the ankle on exercise 
test (see 4.00G7a) of 50 percent or more of the pre-exercise level 
and requiring 10 minutes or more to return to pre-exercise level.
    OR
    C. Resting toe systolic pressure of less than 30 mmHg (see 
4.00G7c and 4.00G8).
    OR
    D. Resting toe/brachial systolic blood pressure ratio of less 
than 0.40 (see 4.00G7c).
    4.13-4.15 [Reserved]
    4.16 Cardiac allograft vasculopathy (see 4.00I5), documented by 
appropriate medically acceptable imaging (for example, intravascular 
ultrasonography or coronary angiography) (see 4.00A3d), with A, B, 
C, or D:
    A. Cardiac index (CI) or cardiac output (CO) less than 2 l/min/
m\2\.
    OR
    B. Left ventricular ejection fraction equal to or less than 45 
percent.
    OR
    C. Right atrial pressure (RAP) greater than 12 mmHg.
    OR
    D. Pulmonary capillary wedge pressure (PCWP) greater than 15 
mmHg.
* * * * *
    5. Amend part B of appendix 1 to subpart P of part 404 by 
revising the body system name for section 104.00 in the table of 
contents to read as follows:
* * * * *

Part B

* * * * *
    104.00 Cardiovascular Disorders
* * * * *

104.00 Cardiovascular Disorders

    A. How do we define cardiovascular disorders and cardiovascular 
terms?
    1. What do we mean by a cardiovascular disorder?
    a. * * *
    b. Cardiovascular disorders result from one or more of four 
consequences of heart disease: * * *
    (iv) Hypoxemia due to right-to-left shunt, reduced oxygen 
concentration in the arterial blood, or pulmonary vascular disease.
* * * * *
    2. What do we consider in evaluating cardiovascular disorders? 
The listings in this section describe cardiovascular disorders based 
on the medical and other evidence, including response to a regimen 
of prescribed treatment and functional limitations.
    3. What do the following terms or phrases mean in these 
listings?
    a. Medical consultant is a person defined in Sec.  416.1016(a) 
of this chapter. * * *
    b. * * * By ``exceptions,'' we mean brief periods when the 
required finding(s) is greatly reduced or gone. These periods are so 
brief or inconsequential, the required finding(s) remains a factor 
in the person's condition.
    c. * * * By ``improvement of sufficient duration,'' we mean the 
finding is greatly reduced or not present for long enough that the 
required finding(s) is no longer a factor in the person's condition.
* * * * *
    B. What documentation do we need to evaluate cardiovascular 
disorders?
    1. What basic documentation do we need? We need sufficiently 
detailed reports of history, physical examinations, laboratory 
studies, and any prescribed treatment and response to allow us to 
assess the severity and duration of your cardiovascular disorder.
* * * * *
    2. Why is a longitudinal clinical record important? * * * 
Whenever there is evidence of such treatment, your longitudinal 
clinical record should include a description of the ongoing 
management and evaluation provided by your medical source(s). * * *
    3. What if you have not received ongoing medical treatment?
    a. * * * In this situation, we will base our evaluation on the 
current evidence we have. * * * However, we may find you disabled 
because you have another impairment(s) that, in combination with 
your cardiovascular disorder, medically equals a listing or 
functionally equals the listings.
    b. * * * In rare instances when there is no or insufficient 
longitudinal evidence, we may purchase a consultative examination(s) 
to help us establish the existence, severity, and duration of your 
impairment.
    4. When will we wait before we ask for more evidence?
    a. * * *
    (i) If you have had a recent acute event; for example, acute 
heart failure.
* * * * *

[[Page 38865]]

    5. Will we purchase any studies? In appropriate situations, we 
may purchase studies necessary to substantiate the existence of a 
medically determinable impairment or to document the severity of 
your impairment, generally after we have evaluated the evidence we 
already have. * * * We will follow sections 4.00C6, 4.00C7, 4.00C8 
in part A, and 104.00B7, when we decide whether to purchase exercise 
testing. * * *
* * * * *
    7. Will we use exercise tolerance tests (ETT) for evaluating 
children with cardiovascular disorders?
    a. * * * An ETT may be of value in the assessment of some 
arrhythmias, as indicated in 104.05B2. ETTs may also be used in the 
assessment of the severity of chronic heart failure and in the 
assessment of recovery of function following cardiac surgery or 
other treatment.
    b. We will purchase an ETT only if we cannot make a 
determination or decision based on the evidence we have and an MC, 
preferably one with experience in the care of children with 
cardiovascular disorders, has determined that an ETT is needed to 
evaluate your impairment. * * *
    c. For full details on ETT requirements and usage, see 4.00C3 in 
part A.
    C. How do we evaluate chronic heart failure?
    1. What is chronic heart failure (CHF)?
    a. Heart failure is the inability of the heart to pump enough 
oxygenated blood to body tissues. * * * Ejection fraction in heart 
failure is a continuum ranging from low ejection fraction due to 
muscle dysfunction to preserved ejection fraction resulting from 
high intracardiac pressures. We consider heart failure to be chronic 
when the condition persists or recurs over time despite treatment.
    b. CHF is considered in these listings as a single category 
whether due to atherosclerosis (narrowing of the arteries), 
cardiomyopathy, hypertension, congenital, or other heart disease. If 
the CHF is the result of primary pulmonary hypertension secondary to 
disease of the lung, we will evaluate your impairment under the 
listings in 3.00 (for example, 3.09) or 4.00, as appropriate.
    2. What evidence of CHF do we need?
    a. Cardiomegaly or ventricular dysfunction must be present and 
demonstrated by appropriate medically acceptable imaging, such as 
chest x-ray, echocardiography (M-Mode, 2-dimensional, and Doppler), 
radionuclide studies, or cardiac catheterization. Other findings on 
appropriate medically acceptable imaging may include increased 
pulmonary vascular markings, pleural effusion, and pulmonary edema.
    b. Your medical history and physical examination should describe 
characteristic symptoms and signs of pulmonary or systemic 
congestion (fluid retention) or of limited cardiac output associated 
with the abnormal findings on appropriate medically acceptable 
imaging. When an acute episode of heart failure is triggered by a 
remediable factor, such as an arrhythmia, dietary sodium overload, 
or high altitude, cardiac function may be restored and a chronic 
impairment may not be present.
    (i) * * *
    (ii) * * *
    (iii) * * * However, these signs need not be found on all 
examinations because congestion may be controlled by prescribed 
treatment or may not be present at the time of evaluation.
* * * * *
    4. How do we evaluate CHF treated with a mechanical circulatory 
support device? We use 104.02D to evaluate CHF treated with an 
implanted mechanical circulatory support device (MCSD), such as a 
left ventricle assistive device (LVAD) or a right ventricle 
assistive device (RVAD). Implanted MCSDs are intended for long-term 
circulatory support in helping the heart pump blood. For the 
purposes of 104.02D, an MCSD does not include extracorporeal 
membrane oxygenation (ECMO). Although ECMO is a form of mechanical 
circulatory support, we do not include it in 104.02D because ECMO is 
intended only for short-term circulatory support (maximum 30 days), 
used in a setting of imminent or actual cardiac arrest.
    D. How do we evaluate congenital heart disease?
    1. What is congenital heart disease? Congenital heart disease is 
any abnormality of the heart or the major blood vessels that is 
present at birth. Congenital heart disease includes abnormal 
structure of the individual heart chambers, valves, and blood 
vessels, and abnormal relative relationship of the chambers to each 
other that alters the normal pattern of blood flow. Surgery is the 
usual treatment, and with improving surgical techniques and medical 
management, more children with congenital heart disease are 
surviving into adulthood. Examples of congenital heart disease 
include:
    a. * * *
    b. * * *
    c. * * *
    d. Major abnormalities of ventricular development, including 
hypoplastic left heart syndrome or tricuspid atresia with 
hypoplastic right ventricle.
    2. How do we evaluate conditions associated with congenital 
heart disease?
    a. We will evaluate congenital heart disease that results in 
chronic heart failure with evidence of ventricular dysfunction or in 
recurrent arrhythmias under 104.02 or 104.05, respectively. 
Otherwise, we will evaluate your impairment under 104.06.
    b. We need pulse oximetry measurements documented by medical 
sources using methods consistent with the prevailing state of 
medical knowledge and clinical practice to evaluate chronic 
hypoxemia in congenital heart disease under 104.06A3. These pulse 
oximetry measurements also must be consistent with the other 
evidence in the case record.
    c. 104.06D, life-threatening congenital heart disease does not 
include single ventricle; we evaluate single ventricle physiology 
separately under 104.06C. When we evaluate life-threatening 
congenital heart disease under 104.06D, we consider whether it 
responds to surgical treatment and, therefore, may not meet the 12-
month duration requirement. Examples of impairments that in most 
instances will require life-saving surgery or a combination of 
surgery and other major interventional procedures (for example, 
multiple ``balloon'' catheter procedures) before age 1 include, but 
are not limited to, the following:
    (i) Critical aortic stenosis with neonatal heart failure,
    (ii) Critical coarctation of the aorta, with associated 
anomalies,
    (iii) Complete atrioventricular canal defects,
    (iv) Transposition of the great arteries,
    (v) Tetralogy of Fallot, and
    (vi) Multiple ventricular septal defects.
    3. What is Eisenmenger syndrome? Eisenmenger syndrome refers to 
any surgically untreated congenital heart defect with intracardiac 
communication that over time leads to pulmonary hypertension, 
reversal of blood flow, and hypoxemia.
    a. Lesions in Eisenmenger syndrome, such as large septal 
defects, are characterized by elevated pulmonary pressures or a high 
pulmonary flow rate. In response, the pulmonary blood vessels 
pathologically change, leading eventually to pulmonary hypertension. 
Development of Eisenmenger syndrome represents a point at which 
pulmonary hypertension is irreversible and the cardiac lesion is 
likely inoperable.
    b. Examples of congenital heart disease that if untreated may 
cause pulmonary vascular disease leading to Eisenmenger syndrome 
include atrial septal defect (ASD), ventricular septal defect (VSD), 
and large patent ductus arteriosus (PDA). We evaluate Eisenmenger 
syndrome under 104.06A or 104.06B.
    4. What is single ventricle? The term ``single ventricle'' (also 
known as single ventricle physiology or functional single ventricle) 
describes a diverse group of congenital cardiac anomalies sharing 
the common feature that only one of the two heart ventricles is 
adequately developed. At birth, one ventricle must functionally do 
the work of two, pumping blood for both the body (systemic) and the 
lungs (pulmonary). Because of this feature, the ultimate plan for 
cardiac reconstruction is similar for most of these anomalies. 
People with single ventricle will generally undergo staged 
reconstructive ``Fontan procedures,'' ultimately resulting in a 
``Fontan circulation.'' Fontan circulation describes the hemodynamic 
state in which virtually all systemic venous return-blood passively 
flows directly into the pulmonary arteries via surgical or catheter-
placed shunts, without (the blood) passing through a ventricle. Some 
of the anomalies described as single ventricle include the 
following:
    (i) Hypoplastic left heart syndrome;
    (ii) Hypoplastic right ventricle;
    (iii) Tricuspid valve atresia;
    (iv) Double inlet left ventricle; and
    (v) Some variations of double outlet right ventricle.
    E. How do we evaluate arrhythmias?
    1. What is an arrhythmia? * * * Although we use the term 
``arrhythmia'' in the listings, the term ``dysrhythmia'' may also be 
used in the medical evidence to describe this condition.
* * * * *
    4. What will we consider when you have an implanted cardiac 
defibrillator and you do

[[Page 38866]]

not have arrhythmias that meet the requirements of 104.05?
    a. * * * The shock from the implanted cardiac defibrillator 
rescues a child from what may have been cardiac arrest. However, as 
a consequence of the shock(s), similar to the effects of treatments 
for other cardiovascular disease, a child may experience 
psychological distress, which we may evaluate under the listings in 
112.00.
    b. * * * Also, exposure to strong electrical or magnetic fields, 
such as from magnetic resonance imaging, can trigger or reprogram an 
implanted cardiac defibrillator, resulting in inappropriate shocks. 
* * *
* * * * *
    F. How do we evaluate other cardiovascular disorders?
    1. What is ischemic heart disease (IHD) and how will we evaluate 
it in children? * * * If you have IHD, we will evaluate it under 
4.04 in part A.
    2. How will we evaluate hypertension? Hypertension (high blood 
pressure) generally causes disability in children through its 
effects on other body systems, such as the brain, kidneys, or eyes, 
and we will evaluate these impairments by reference to the specific 
body system(s) that is affected. * * *
    3. What is cardiomyopathy and how will we evaluate it?
    a. There are various types of cardiomyopathy, which fall into 
two major categories: ischemic and nonischemic cardiomyopathy. 
Ischemic cardiomyopathy typically refers to heart muscle damage that 
results from coronary artery disease, including heart attacks. 
Nonischemic cardiomyopathy includes several types: dilated, 
hypertensive, hypertrophic, and restrictive.
    b. We will evaluate cardiomyopathy under 4.04 in part A, 104.02, 
or 104.05, depending on its effects on you.
    4. How will we evaluate valvular heart disease? We will evaluate 
aortic valvular disease under 4.07 in part A. We may also evaluate 
aortic valvular disease, as well as other forms of valvular disease, 
under 4.04 in part A, 104.02, 104.05, 104.06, or a listing in 
111.00, depending on its effects on you.
    5. * * *
    a. After your heart transplant, we will consider you disabled 
under 104.09 for 1 year following the surgery because there is a 
greater likelihood of rejection of the organ and infection during 
the first year. If you develop cardiac allograft vasculopathy after 
your transplant, we will evaluate this impairment under 104.16.
* * * * *
    d. When we do a continuing disability review to determine 
whether you are still disabled, we will evaluate your residual 
impairment(s), as shown by the evidence in your case record, 
including any side effects of medication. We will consider all 
evidence indicative of cardiac dysfunction in deciding whether 
medical improvement (as defined in Sec.  416.994a of this chapter) 
has occurred.
    6. How will we evaluate chronic rheumatic fever or rheumatic 
heart disease? We will evaluate rheumatic fever or rheumatic heart 
disease under the listing appropriate to its effects on you, which 
may include heart failure or recurrent arrhythmias. If you have 
evidence of chronic heart failure or recurrent arrhythmias 
associated with rheumatic heart disease, we will evaluate these 
disorders under 104.02 or 104.05, respectively.
* * * * *
    9. * * *
    a. * * *
    b. Lymphedema does not meet the requirements of 4.11 in part A, 
although it may medically equal the listing. We evaluate lymphedema 
by considering whether the underlying cause meets or medically 
equals any listing or whether the lymphedema medically equals a 
cardiovascular disorders listing, such as 4.11 in part A, or a 
listing in 101.00. If no listing is met or medically equaled, we 
will evaluate any functional limitations imposed by your lymphedema 
when we consider whether you have an impairment(s) that functionally 
equals the listings.
* * * * *
    11. What is cardiac allograft vasculopathy and how do we 
evaluate it? Cardiac allograft vasculopathy (CAV) may affect a 
person who has received a heart transplant and involves thickening 
in the walls of the coronary arteries that may progress quickly into 
serious vascular stenosis and heart dysfunction. Stenosis in CAV is 
caused by a pathological process different from classic 
atherosclerosis and treatment often is only palliative. We evaluate 
CAV under 104.16.
    G. How do we evaluate issues that affect the cardiovascular 
system?
    1. How do we consider the effects of obesity when we evaluate 
your cardiovascular disorder? Obesity is a medically determinable 
impairment that may be associated with cardiovascular disorders. The 
additional body mass may make it harder for the chest and lungs to 
expand or may cause the heart to work harder to pump blood to carry 
oxygen to the body. The combined effects of obesity with a 
cardiovascular disorder can be greater than the effects of each of 
the impairments considered separately. We consider the additional 
and cumulative effects of obesity when we determine whether you have 
a severe cardiovascular disorder, a listing-level cardiovascular 
disorder, a combination of impairments that medically equals the 
severity of a listed impairment, and when we determine whether your 
impairment(s) functionally equals the listings.
    2. How do we relate treatment to functional status? In general, 
conclusions about the severity of a cardiovascular disorder cannot 
be made on the basis of the type of treatment rendered or 
anticipated. * * *
    3. How do we consider hospitalizations? The hospitalizations in 
104.02E and 104.06E do not all have to be for the same exacerbation 
or complication of your cardiovascular disorder(s). They may be for 
three different exacerbations or complications resulting from your 
cardiovascular disorder. The hospitalizations must be at least 30 
days apart, and each one must last at least 48 hours, including 
hours in a hospital emergency department immediately before the 
hospitalization.
    H. How do we evaluate cardiovascular disorders that do not meet 
one of these listings?
    1. These listings are only examples of common cardiovascular 
disorders that we consider severe enough to result in marked and 
severe functional limitations. If your impairment(s) does not meet 
the criteria of any of these listings, we must also consider whether 
you have an impairment(s) that satisfies the criteria of a listing 
in another body system.
    2. If you have a severe medically determinable impairment(s) 
that does not meet a listing, we will determine whether your 
impairment(s) medically equals a listing. See Sec.  416.926 of this 
chapter. If your impairment(s) does not meet or medically equal a 
listing, we will also consider whether it functionally equals the 
listings. See Sec.  416.926a of this chapter. We will use the rules 
in Sec.  416.994a of this chapter when we decide whether you 
continue to be disabled.

104.01 Category of Impairments, Cardiovascular Disorders

    104.02 Chronic heart failure (see 104.00C) while on a regimen of 
prescribed treatment with symptoms and signs described in 104.00C2, 
and with A, B, C, D, or E:
    A. Persistent tachycardia at rest measured at least twice within 
a consecutive 12-month period and at least 90 days apart documented 
by apical heart rate greater than or equal to the value in Table I.

                      Table I--Tachycardia at Rest
------------------------------------------------------------------------
                                                           Apical heart
                           Age                              rate (beats
                                                            per minute)
------------------------------------------------------------------------
Under 1 year............................................             150
1 through 3 years.......................................             130
4 through 9 years.......................................             120
10 through 15 years.....................................             110
Over 15 years...........................................             100
------------------------------------------------------------------------

    OR
    B. Persistent tachypnea at rest measured at least twice within a 
consecutive 12-month period and at least 90 days apart documented by 
respiratory rate greater than or equal to the value in Table II or 
markedly decreased exercise tolerance (see 104.00C2b).

                       Table II--Tachypnea at Rest
------------------------------------------------------------------------
                                                            Respiratory
                           Age                               rate (per
                                                              minute)
------------------------------------------------------------------------
Under 1 year............................................              40
1 through 5 years.......................................              35
6 through 9 years.......................................              30
Over 9 years............................................              25
------------------------------------------------------------------------

    OR
    C. Growth failure as required in 1 or 2:
    1. For children from birth to attainment of age 2, three weight-
for-length measurements that are:
    a. Within a consecutive 12-month period; and
    b. At least 60 days apart; and
    c. Less than the third percentile on the appropriate weight-for-
length table under 105.08B1; or

[[Page 38867]]

    2. For children age 2 to attainment of age 18, three BMI-for-age 
measurements that are:
    a. Within a consecutive 12-month period; and
    b. At least 60 days apart; and
    c. Less than the third percentile on the appropriate BMI-for-age 
table under 105.08B2.
    OR
    D. Mechanical circulatory support device (except an 
extracorporeal membrane oxygenation (ECMO) while hospitalized, at 
home, or both (see 104.00C4). Consider under a disability for 12 
months from the date of implantation; after that, evaluate any 
residual impairment(s) under the criteria for the affected body 
system.
    OR
    E. Exacerbations or complications of chronic heart failure (see 
104.00C1b) requiring three hospitalizations within a consecutive 12-
month period and at least 30 days apart. Each hospitalization must 
last at least 48 hours, including hours in a hospital emergency 
department immediately before the hospitalization (see 104.00G3).
    104.03-104.04 [Reserved]
    104.05 Recurrent arrhythmias (see 104.00E), not related to 
reversible causes such as electrolyte abnormalities or digitalis 
glycoside or antiarrhythmic drug toxicity, while on a regimen of 
prescribed treatment (see 104.00B3 if there is no prescribed 
treatment), demonstrated by both A and B:
    A. Coincident with recurrent (see 104.00A3c) episodes of cardiac 
syncope or near syncope (see 104.00E3b).
    AND
    B. Documented by either 1 or 2:
    1. Resting or ambulatory (Holter) electrocardiography; or
    2. Other appropriate medically acceptable testing.
    104.06 Congenital heart disease (see 104.00D), documented by 
appropriate medically acceptable imaging (see 104.00A3d) or cardiac 
catheterization, with A, B, C, D, or E:
    A. Chronic hypoxemia, and 1, 2, or 3:
    1. Hematocrit of 55 percent or greater on two evaluations at 
least 90 days apart within a consecutive 12-month period (see 
104.00A3e); or
    2. Arterial blood gas test measurement obtained at rest while 
breathing room air, as described in either a or b:
    a. S<INF>a</INF>O<INF>2</INF> (arterial oxygen saturation) less 
than or equal to 89 percent; or
    b. PO<INF>2</INF> or P<INF>a</INF>O<INF>2</INF> (partial 
pressure of oxygen) less than or equal to 60 mmHg; or
    3. S<INF>p</INF>O<INF>2</INF> (percentage of oxygen saturation 
of blood hemoglobin) measured by pulse oximetry either at rest, or 
after activity, while breathing room air, less than or equal to 87 
percent on three evaluations at least 30 days apart within a 
consecutive 12-month period (see 104.00A3e).
    OR
    B. Pulmonary hypertension documented by cardiac catheterization 
while medically stable, as described in 1, 2, or 3:
    1. Pulmonary arterial systolic pressure elevated to at least 70 
percent of the systemic arterial systolic pressure; or
    2. Pulmonary arterial systolic pressure equal to or greater than 
70 mmHg; or
    3. Mean pulmonary artery pressure equal to or greater than 40 
mmHg.
    OR
    C. Single ventricle (for example, hypoplastic left or right 
ventricle) that has or will require Fontan procedures (see 
104.00D5).
    OR
    D. For infants under 1 year of age at the time of filing, with 
life-threatening congenital heart disease (see 104.00D3c) that will 
require or already has required surgical treatment in the first year 
of life, and the impairment is expected to be disabling (because of 
residual impairment following surgery, or the recovery time 
required, or both) until the attainment of at least 1 year of age, 
consider under a disability until the attainment of at least age 1; 
after that, evaluate impairment severity with the appropriate 
listing.
    OR
    E. Exacerbations or complications of congenital heart disease 
(see 104.00D) requiring three hospitalizations within a consecutive 
12-month period (see 104.00A3e) and at least 30 days apart. Each 
hospitalization must last at least 48 hours, including hours in a 
hospital emergency department immediately before the hospitalization 
(see 104.00G3).
    104.07-104.08 [Reserved]
    104.09 Heart transplantation (see 104.00F5). Consider under a 
disability for 1 year from the date of the transplant; after that, 
evaluate the residual impairment(s).
    104.10-104.15 [Reserved]
    104.16 Cardiac allograft vasculopathy (see 104.00F11), 
documented by appropriate medically acceptable imaging (for example, 
intravascular ultrasonography or coronary angiography).

[FR Doc. 2022-12980 Filed 6-28-22; 8:45 am]
BILLING CODE 4191-02-P