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## Complete Summary

#### GUIDELINE TITLE

Suspected congenital heart disease in the adult.

#### BIBLIOGRAPHIC SOURCE(S)

Ho VB, Yucel EK, Khan A, Haramati LB, Mammen L, Rozenshtein A, Rybicki
FJ, Schoepf UJ, Stanford W, Stein B, Woodard PK, Jaff M, Expert Panel on
Cardiovascular Imaging. Suspected congenital heart disease in the adult.
\[online publication\]. Reston (VA): American College of Radiology
(ACR); 2007. 8 p. \[55 references\]

#### GUIDELINE STATUS

This is the current release of the guideline.

It updates a previously published version: American College of Radiology
(ACR), Expert Panel on Cardiovascular Imaging. Suspected congenital
heart disease in the adult. Reston (VA): American College of Radiology
(ACR); 2002. 6 p.(ACR appropriateness criteria). \[38 references\]

The appropriateness criteria are reviewed annually and updated by the
panels as needed, depending on introduction of new and highly
significant scientific evidence.

### COMPLETE SUMMARY CONTENT

 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 CONTRAINDICATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Congenital heart disease

#### GUIDELINE CATEGORY

Diagnosis\
Evaluation

#### CLINICAL SPECIALTY

Cardiology\
Family Practice\
Internal Medicine\
Nuclear Medicine\
Radiology

#### INTENDED USERS

Health Plans\
Hospitals\
Managed Care Organizations\
Physicians\
Utilization Management

#### GUIDELINE OBJECTIVE(S)

To evaluate the appropriateness of initial radiologic examinations for
adults with suspected congenital heart disease

#### TARGET POPULATION

Adults with suspected congenital heart disease

#### INTERVENTIONS AND PRACTICES CONSIDERED

1.  X-ray, chest

2.  Ultrasound (US) echocardiography transthoracic with Doppler

3.  Magnetic resonance imaging (MRI) of the heart

4.  US echocardiography transesophageal

5.  Computed tomography angiogram (CTA), coronary arteries

6.  Computed tomography (CT) of the heart with contrast

7.  Magnetic resonance angiogram (MRA) of the chest

8.  Invasive (INV) heart cardiac catheterization with angiocardiography

9.  Nuclear medicine (NUC), scintigraphy of the heart

10. NUC shunt detection

#### MAJOR OUTCOMES CONSIDERED

Utility of radiologic examinations in differential diagnosis

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

The guideline developer performed literature searches of recent
peer-reviewed medical journals and the major applicable articles were
identified and collected.

#### NUMBER OF SOURCE DOCUMENTS

Not stated

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Weighting According to a Rating Scheme (Scheme Not Given)

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

Not stated

#### METHODS USED TO ANALYZE THE EVIDENCE

Systematic Review with Evidence Tables

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

One or two topic leaders within a panel assume the responsibility of
developing an evidence table for each clinical condition, based on
analysis of the current literature. These tables serve as a basis for
developing a narrative specific to each clinical condition.

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus (Delphi)

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

Since data available from existing scientific studies are usually
insufficient for meta-analysis, broad-based consensus techniques are
needed for reaching agreement in the formulation of the appropriateness
criteria. The American College of Radiology (ACR) Appropriateness
Criteria panels use a modified Delphi technique to arrive at consensus.
Serial surveys are conducted by distributing questionnaires to
consolidate expert opinions within each panel. These questionnaires are
distributed to the participants along with the evidence table and
narrative as developed by the topic leader(s). Questionnaires are
completed by participants in their own professional setting without
influence of the other members. Voting is conducted using a scoring
system from 1-9, indicating the least to the most appropriate imaging
examination or therapeutic procedure. The survey results are collected,
tabulated in anonymous fashion, and redistributed after each round. A
maximum of three rounds is conducted and opinions are unified to the
highest degree possible. Eighty percent agreement is considered a
consensus. This modified Delphi technique enables individual, unbiased
expression, is economical, easy to understand, and relatively simple to
conduct.

If consensus cannot be reached by the Delphi technique, the panel is
convened and group consensus techniques are utilized. The strengths and
weaknesses of each test or procedure are discussed and consensus reached
whenever possible. If \"No consensus\" appears in the rating column,
reasons for this decision are added to the comment sections.

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

Not applicable

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

Internal Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

Criteria developed by the Expert Panels are reviewed by the American
College of Radiology (ACR) Committee on Appropriateness Criteria.

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

**ACR Appropriateness Criteria®**

**Clinical Condition: Suspected Congenital Heart Disease in the Adult**

  -----------------------------------------------------------------------------------
  **Radiologic          **Rating**   **Comments**                      **RRL\***
  Procedure**                                                          
  --------------------- ------------ --------------------------------- --------------
  X-ray chest           9            Recommended in combination with   Min
                                     TTE.                              

  US echocardiography   9            Recommended in combination with   None
  transthoracic with                 chest x-ray.                      
  Doppler                                                              

  MRI heart             8            May be done as adjunct to TTE by  None
                                     trained operator if additional    
                                     information is required. See      
                                     comments regarding contrast in    
                                     text under \"Anticipated          
                                     Expectations.\"                   

  US echocardiography   7            May be done as adjunct to TTE by  None
  transesophageal                    trained operator if additional    
                                     information is required.          

  CTA coronary arteries 7            Preferred technique for suspected High
                                     coronary anomalies. Can also be   
                                     used for evaluation of coronary   
                                     artery disease.                   

  CT heart with         6            May be an alternative to MRI and  High
  contrast                           TTE/TEE.                          

  MRA chest             6            To evaluate associated vascular   None
                                     abnormalities. See comments       
                                     regarding contrast in text under  
                                     \"Anticipated Expectations.\"     

  INV heart cardiac     5            Adjunctive to noninvasive         IP
  catheterization with               testing, for hemodynamic          
  angiocardiography                  measurements or coronary artery   
                                     status, or if other diagnostic    
                                     information is required.          

  NUC scintigraphy      4            May be used for perfusion in      IP
  heart                              patients with suspected ischemic  
                                     heart disease.                    

  NUC shunt detection   4            Alternative to MRI for shunt      Low
                                     quantification.                   

  **[Rating                                                            **\*Relative
  Scale]{.underline}:                                                  Radiation
  1=Least appropriate,                                                 Level**
  9=Most appropriate**                                                 
  -----------------------------------------------------------------------------------

Note: Abbreviations used in the tables are listed at the end of the
\"Major Recommendations\" field.

**Summary of Literature Review**

Although patients with congenital heart disease (CHD) account for only a
small percentage of adults with recognized heart disease, the number of
adult patients in North America with CHD (ACHD) is increasing. A review
of data from the National Center for Health Statistics and the Centers
of Disease Control and Prevention showed a 39% decline in reported
mortality from the CHD in the United States between 1979 and 1997. This
decline is explained by 1) improvements in surgical treatment and
survival, which is greater than 90% at 10 years, 2) the increasing
numbers of immigrants, and 3) changes in the statistical methods used to
calculate incidence of ACHD. The underestimation of these figures is due
in part to measuring the incidence of CHD presenting in infancy and
childhood. However, at least 10% of patients diagnosed in ACHD clinics
(in particular, those with secundum atrial septal defect, Ebstein\'s
anomaly, and congenitally corrected transposition) are not diagnosed
until adulthood. In addition to adults newly diagnosed with CHD, there
is an increasing number of adults (estimated at \>500,000) who have
surgically treated CHD, with nearly 50% requiring two or more operations
and 23%, requiring three or more. In Toronto, for example, a 269%
expansion of outpatient workload in ACHD centers was noted between 1987
and 1997.

Adults with CHD also have acquired co-morbid factors, such as
hypertension, atherosclerosis, coronary artery occlusive disease,
pulmonary disease and renal disease, which may complicate their medical
and/or surgical management.

Congenital heart lesions may become symptomatic at any time from birth
until adulthood. Several common congenital heart defects often survive
into adulthood. These include bicuspid aortic valve, congenital forms of
mitral valve prolapse, aortic coarctation, atrial septal defect (ASD),
pulmonary valve stenosis, patent ductus arteriosus (PDA), and tetralogy
of Fallot. Uncommon congenital cardiac defects that may present in
adulthood include Ebstein\'s anomaly, corrected transposition of the
great vessels, pulmonary arteriovenous malformation, coronary artery
anomalies, and sinus of Valsalva aneurysm.

The most common congenital heart defect in children, ventricular septal
defect (VSD), may escape detection and present in adults either as a
small, physiologically insignificant defect or as a large defect with
Eisenmenger physiology. Anomalies of the great arteries such as complete
transposition and total anomalous pulmonary venous drainage are usually
symptomatic, whereas less severe anomalies such as a persistent left
superior vena cava and many anomalies of the origin of the great vessels
from the arch are often asymptomatic.

Imaging procedures for the diagnosis of suspected CHD in the adult
include plain chest film radiography, fluoroscopy, echocardiography
(transthoracic and transesophageal), nuclear scintigraphy, cardiac-gated
computed tomography (CT), magnetic resonance imaging (MRI), and cardiac
catheterization and angiography. The physician trying to diagnose these
often-complex conditions needs complete and reliable information that
includes details about intercardiac anatomy, vascular anatomy,
hemodynamics, and function.

**Chest Radiography**

The initial work-up of adults with suspected congenital heart disease
(CHD) usually includes a posteroanterior (PA) and lateral chest
radiograph. Occasionally the radiograph will be the first study to alert
the radiologist and the clinician to the possibility of a congenital
cardiac defect or great-vessel anomaly. This simple and inexpensive
examination remains a first-line test for patients with suspected CHD.

The chest radiograph quickly illustrates gross cardiac and mediastinal
contours, pulmonary vascularity, pathologic calcification, and the
presence of certain indwelling metallic devices. It also provides an
assessment of cardiac size, cardiac configuration, and position of the
aortic arch. The situs of the abdomen and thorax can usually be
determined. Thoracic cage anomalies associated with CHD and
postoperative changes may also be detected. The chest radiograph
continues to be an invaluable tool for following the patient with
surgically treated CHD.

**Echocardiography**

Transthoracic echocardiography (TTE) remains a first-line imaging
examination in adults with suspected CHD. This test has long been
established as a clinically useful diagnostic modality for CHD in
children, often eliminating the need for cardiac catheterization in
uncomplicated lesions. Although adults present certain technical
problems related to the need for lower frequency transducers, limited
acoustical windows, and postoperative changes, this examination provides
a unique, 2-dimensional, real-time evaluation of the anatomic and
hemodynamic relationships of intracardiac lesions.

Transthoracic echocardiography is widely available, reproducible, safe,
and painless. As such it remains a valuable tool in the investigation of
CHD.

Echocardiography using color flow Doppler is particularly well suited
for evaluating blood flow as seen across an atrial or a ventricular
septal defect or across a valve. In postoperative patients, VSD patch
defects can readily be visualized. Assessment of the valves (sclerosis,
fusion, estimation of valve gradients) and determination of right
ventricular systolic pressure can usually be achieved.

TTE, however, has difficulty in consistently providing high-quality
clinically useful information in some adult patients with intracardiac
defects. Imaging of the great vessel with TTE is difficult even in
children and is even more problematic in adults who have poorer
acoustical windows. In these situations, transesophageal
echocardiography (TEE) and MRI have roles to play. Echocardiography also
suffers from intraobserver variability in terms of exam reproducibility.

Current 2-dimensional TTE is limited by a field of view of 90 degrees
and the need for the examiner to assimilate tomographic slices into a 3-
or 4-dimensional diagnosis. The recent development of a rotational
acquisition format with dynamic volume rendering has allowed
presentation of TTE in a 3-dimensional display. In one study,
3-dimensional echocardiography was found to be particularly useful, when
compared to 2-dimensional echocardiography, for evaluating mitral valve,
aortoseptal continuity, and the intra-arterial septum. While 3D
echocardiography can provide additional information over traditional 2D
techniques, but has been reported to be nondiagnostic in up to 27% of
patients secondary to inadequate quality from morbid obesity, narrow
intercostal spaces, and severe pulmonary emphysema.

TEE has clear advantages over TTE in adolescents and adults with CHD.
TEE can provide a new or altered diagnosis (14%) or new information
(56%) in adults with CHD. New information obtained with TEE as compared
to TTE includes identification of the atrial appendages and atrial
septum, delineation of systemic and pulmonary venous connections,
improved morphologic assessment of the atrioventricular junction and
valves, improved definition of subaortic obstruction, improved
definition of the ascending aorta and coronary arteries, and better
evaluation of atrial baffle function and Fontan anatomy. Limitations of
TEE include limited planes of view, poor visualization of specific
regions (e.g., apical-anterior septum and right ventricular outflow
tract), and blind areas created by masking of flow by implanted
prosthetic material. Areas that may be difficult to visualize on TEE are
the right ventricular outflow tract, the pulmonary valve, the distal
right pulmonary artery, and the proximal left pulmonary artery. With the
addition of the vertical axis in the newer probes, these problem areas,
as well as the pulmonary veins, are better seen.

The standard TEE is an invasive examination that requires administration
of a local anesthetic to the pharynx and intravenous midazolam in small
doses. In large studies, it has been shown that the examination may be
unsuccessful in up to 5% of patients due to their inability to tolerate
the probe after intubation. Another 4%-5% of patients have the
examination while under general anesthesia as part of invasive or
surgical procedures. Although the risk of bacterial endocarditis from
TEE is small, and prophylactic antibiotics are not routinely
administered, endocarditis has been attributed to the procedure.

Transesophageal echocardiography is clearly operator dependent. In an
area as complex as congenital heart disease, the examiner must be
trained to interpret the findings in real time so that important
information is not missed.

With improvements and refinements in echocardiographic technology, most
adults attending an outpatient clinic undergo TTE and, when necessary,
complementary TEE and MRI. Two-dimensional imaging is more challenging
in this patient population because of large body size and often multiple
previous surgical scars. The use of TEE intraoperatively is also
increasing, and it has been shown to have a major impact on cardiac
surgical procedures in 6%-9% of cases (e.g., that it is desirable or
necessary for the patient to be put back onto cardiopulmonary bypass for
revision of the cardiac procedure). Physicians interpreting these
echocardiograms need to be experienced and have expertise in all aspects
of CHD.

A high rate of diagnostic error in pediatric echocardiograms performed
in community-based adult laboratories has been reported. One study found
that 53% of patients of varying ages, from 1 day to 18 years, had either
interpretive or technical errors that were of major or moderate
importance. There is reason to believe that diagnostic errors occur even
more frequently in older patients because image acquisition is typically
more challenging.

**Radionuclide Imaging**

Although quantitation of cardiac shunts is feasible using technetium Tc
99m first-pass techniques, it is seldom used today. There are, however,
a few selected uses for radionuclide imaging in evaluating adults with
CHD. Left ventricular dysfunction is known to complicate certain
long-standing congenital heart defects associated with right and left
heart volume overload. Left ventricular radionuclide scintigraphy with
ejection fraction calculation can be a useful noninvasive technique for
evaluating these patients. Gated radionuclide scintigraphy can improve
risk assessment in patient with single or systemic right ventricles by
assessing heart failure and ventricular dysfunction. In some adult
patients with abnormal pulmonary blood flow patterns related to
conditions such as pulmonary artery agenesis, ventilation-perfusion lung
scanning may assist in the diagnosis. Congenital anomalies of the
coronary artery origins, notably anomalous origin of the coronary artery
from the pulmonary artery and interarterial anomalous coronary artery,
may result in myocardial ischemia and/or silent infarction using
stress/rest radionuclide SPECT (single photon emission computed
tomography) imaging. Stress/rest radionuclide SPECT imaging can also be
used to evaluate myocardial perfusion and function of the systemic right
ventricle in patients following repair of transposition of the great
vessels, in which perfusion defects can commonly (54%) be seen on
long-term follow-up.

**Computed Tomography**

Cardiac-gated CT and CT angiography (CTA) can contribute valuable
information about congenital abnormalities of the coronary arteries and
thoracic aorta, including the identification of vascular rings and
postoperative complications such as pseudoaneurysm. The need for
intravenous contrast material and the exposure to radiation, however,
have limited their use in the pediatric population with CHD.

Current cardiac-gated CT scanners can evaluate the entire heart and
great vessel region in a 3-dimensional matrix of CT information.
Essentially all types of congenital cardiac malformations have been
accurately described with this technique. Cardiac-gated CT has been used
to calculate cardiac output, shunt flow, pulmonary-to-systemic flow
ratios, ventricular volumes, ejection fraction, regurgitant volumes, and
myocardial mass.

**Magnetic Resonance Imaging**

MRI is useful for evaluating CHD. Without the concerns related to
exposure to ionizing radiation or the use of nephrotoxic iodinated
contrast agents, it can provide morphologic and functional information
essential for detecting and managing CHD. Traditional \"black-blood\"
techniques (e.g., spin-echo MRI and double inversion recovery fast spin
echo) are useful for delineating cardiac and pericardiac anatomy.
\"Bright-blood\" techniques, notably using newer cine steady-state
free-precession pulse sequences, can demonstrate flow abnormalities
(e.g., a flow jet) related to lesions such as an interventricular or
interatrial septal defect, valvular insufficiency, valvular stenosis, or
coarctation. Defining the plane in which the jet velocity is maximal can
be difficult with MRI. However, with improvements in software and
real-time localization algorithms, this is becoming easier. Parallel
imaging and newer k-space schemes can shorten the acquisition times in
most instances such that cine bright-blood imaging can be performed
during a short breath hold. Bright-blood techniques also enable
volumetric coverage of cardiac chambers for determining cardiac metrics
such as ventricular volumes, ejection fractions, and myocardial mass.
Longer acquisitions as may be required for coronary magnetic resonance
angiography (MRA) are typically performed using navigator respiratory
gating methods.

Phase contrast techniques demonstrate directional blood flow information
for improved identification of subtle intra- or extracardiac shunt
lesions. Phase contrast also allows quantification of blood flow (e.g.,
estimation of the ratio of pulmonary to systemic blood flow \[Qp/Qs\]),
regurgitant fractions, and pressure gradients across valves.

MRI has been used for diagnosing essentially all congenital heart and
great-vessel abnormalities. Conventional spin-echo MRI has been shown to
have very high sensitivity and specificity for diagnosing common CHD. At
a specificity of 90%, spin echo was found to have high sensitivity in
diagnosing great-vessel relationships (100%), thoracic aortic
abnormalities (94%), ASDs (91%), VSDs (100%), visceroatrial situs
(100%), and the cardiac loop (100%). Pulmonary and systemic venous
anomalies and right ventricular outflow obstructions are also detected
with high sensitivity. Vascular rings can also be accurately diagnosed
without the need for angiography. MRI can also be performed using 3D
techniques for high spatial- resolution Gd-enhanced 3-D MRA, or to
provide volumetric coverage of cardiac chambers. Time-resolved MRA was
found to provide a very high diagnostic value (92% of diagnostic
parameters assessed) that included thoracic vascular anatomy, sequential
cardiac anatomy, and shunt detection with high sensitivity (93%-100%)
and high specificity (87%-100%).

Gradient-echo imaging acquisition viewed in a cine format facilitates
physiologic measurements, including stroke volume, ejection fraction,
and wall motion of both ventricles. Blood flow, valve gradients, shunt
flow, regurgitant flow, and pulmonary flow can all be measured using
velocity-encoded cine techniques.

MRI seems to be ideally suited for evaluating adults with suspected or
known CHD. Although claustrophobia in the gantry may require sedation in
a few patients, the study is noninvasive, and image quality is not
affected by body habitus. MRI can provide high-spatial-resolution images
even in more complex CHD without the limitation of imaging \"windows\"
or plane as experienced during echocardiography. MRI images can be
obtained in essentially any plane for improved 3-dimensional
presentation of cardiac anatomy. MRI is useful as well in evaluating the
postoperative patients with CHD, whether it is a palliative procedure, a
surgically created conduit, or reconstructed great vessels.

Pharmacologic stress MRI using dobutamine has also been shown to be
useful in evaluating systemic right ventricular function in patients
with transposition of the great vessels. However, one group of
researchers have shown significant differences in the responses between
exercise stress and dobutamine stress MRI in patients with patients with
intra-atrial correction of transposition of the great vessels,
suggesting the need for further evaluations.

MRI, however, does have a few contraindications and limitations. For
instance, pacemakers are generally considered an exclusion for MRI,
although it has been performed safely in patients with pacemakers under
rigorously safe conditions. The use of gadolinium (Gd) chelate contrast
agents may not be possible in a patient with known severe allergy to Gd.
Detection of calcification remains problematic for MRI, so adults with
homografts or bioprosthetic valved conduits in whom the detection of
calcification implies deterioration may not be optimally imaged. Motion
and respiratory artifacts also may pose a problem on some examinations.

Real-time MRI has recently been introduced and can facilitate evaluation
in patients with dysrhythmias or limited breath-hold capacity. Current
implementations have lower resolution than standard techniques.

Current cine studies represent a summation of acquisitions gated from
the ECG. Any factor that affects ECG gating therefore may degrade the
images, atrial fibrillation being the most common cause. Sternal wires,
vascular clips, arterial stents, biosynthetic valve rings, and
mechanical valves can all interfere with the quality of the images and
thereby limit the clinical value of the examination in some patients.

In terms of specific defects, MRI is probably not as accurate as color
flow Doppler in visualizing small ventricular and atrial defects.
Cardiac MR studies require supervision and monitoring of the procedure
by a physician who understands the clinical question and can acquire an
appropriate and optimal imaging study. This is essential for consistency
and reliable data. Detection of thickened atrioventricular valves and
the diagnosis of bicuspid or fused aortic valves are perhaps better
achieved with TEE.

Future developments in MRI may optimize the acquisition of functional
data and provide real-time visualization of cardiac structures, much as
echocardiography does now.

**Transthoracic and Transesophageal Echocardiography versus Magnetic
Resonance Imaging**

Few prospective studies are available to compare TTE and TEE with MRI.
Studies limited to specific congenital lesions (coarctation of the
aorta, subpulmonary and pulmonary artery anomalies) indicate that MRI
gives a more reliable assessment of severity and is technically more
successful than TTE. Studies comparing TTE with MRI in the evaluation of
patients who have had surgical correction or palliation of CHD indicate
that MRI information is additive to that from TTE. In patients who have
had palliative and corrective surgery for cyanotic heart disease, MRI
and TEE are equivalent for demonstrating abnormalities of the right
ventricular outflow tract, main pulmonary artery, and
systemic-to-pulmonary shunts. MRI is superior in demonstrating
abnormalities of the right and left pulmonary arteries. When TTE and MRI
are compared in a variety of congenital heart lesions, MRI is comparable
to echo in evaluating isolated intracardiac defects but more useful in
diagnosing complex congenital lesions.

Echocardiography has also been shown to have good agreement with MRI in
evaluating right ventricular volumes, but echocardiography has a much
wider interobserver variation. In the evaluation of extracardiac
ventriculopulmonary conduits and the right ventricle, MRI and
echocardiography can often provide complementary and diagnostic
information that, when in agreement, may obviate the need for cardiac
catheterization.

When TEE and MRI have been evaluated prospectively in adults with CHD,
TEE is shown to be superior in evaluating intracardiac anatomy; MRI is
superior for extracardiac anatomy and is slightly better than TEE for
hemodynamic and functional evaluation. Taken individually, the two
modalities provided similar overall diagnostic information; however,
when used in combination, they provide important complementary
information in all diagnostic categories.

**Cardiac Catheterization and Angiocardiography**

Cardiac catheterization has been the diagnostic \"gold standard\" for
CHD over the past 50 years. For the past 20 years, it has been
increasingly supplemented by noninvasive diagnostic
modalities--initially, cardiac ultrasound and more recently, CT scanning
and MRI. Advances in these technologies have been logarithmic, and it is
likely that in the coming decade, both morphologic and functional
assessments of this patient population will be increasingly accomplished
noninvasively.

In 2001, the Bethesda Conference Task Force 1, \"The Changing Profile on
Congenital Heart Disease in Adult Life,\" suggested the use of
diagnostic catheterization primarily \"for resolving specific issues
concerning operative interventions including 1) the preoperative
evaluation of coronary arteries; 2) the assessment of pulmonary vascular
disease and its response to vasoactive agents for planned, traditional
surgical intervention, and/or heart or heart/lung transplantation; and
3) as an adjunct to the noninvasive assessment of the morphologic and
functional characteristics of many complex congenital lesions (e.g.,
delineation of arterial and venous anatomy, patients with heterotaxy,
Fontan candidates, and patients who have had previous palliation in the
form of a shunt.)\" The group further suggested that only experienced
and trained operators who maintain an adequate volume annually should
perform such procedures. They further supported this suggestion by
noting that evaluation for possible interventional catheterization is an
increasingly common indication for diagnostic catheterization. Catheter
intervention, for instance, is commonly sought as the treatment of
choice for correcting valvular pulmonary stenosis, branch pulmonary
stenosis, residual or recurrent aortic coarctation, and arteriovenous
fistulae. Coil or device occlusion of lesions such as patent ductus or
secundum atrial septal defect are other preferred interventions for
treatment.

For many years, the purpose of cardiac catheterization and
angiocardiography for CHD was to acquire pressure, oximetric, and
morphologic data. Pressures defined gradients across stenosis and
between cardiac chambers connected by defects as well as the severity of
pulmonary hypertension. Oxygen saturations helped to define the volume
of shunts. Morphologic data of simple and complex anomalies were
achieved by cine angiograms using angulated views, contrast material,
and radiation. For the most part, these studies were accomplished safely
but with some morbidity (contrast reactions, renal failure, hematomas,
arterial and venous injuries, radiation exposure, etc.) and a small but
definite mortality.

Although cardiac catheterization continues to be performed and is
currently still considered by many to be the \"gold standard\" in
evaluating CHD, noninvasive methods increasingly limit the need for
catheterization unless intervention is considered. Many simple
congenital cardiac defects are now sent to surgery without
catheterization. In the future, cardiac catheterization and
angiocardiography may very well be reserved as a complement to these
noninvasive techniques in the evaluation of adults with suspected CHD.
However, until these less invasive studies provide an accurate depiction
of the coronary arteries, the catheterization laboratory will continue
to be involved in the assessment of this unique group of adult patients.

**Anticipated Exceptions**

Nephrogenic systemic fibrosis (NSF, also known as nephrogenic fibrosing
dermopathy) was first identified in 1997 and has recently generated
substantial concern among radiologists, referring doctors and lay
people. Until the last few years, gadolinium-based MR contrast agents
were widely believed to be almost universally well tolerated, extremely
safe and non-nephrotoxic, even when used in patients with impaired renal
function. All available experience suggests that these agents remain
generally very safe, but recently some patients with renal failure who
have been exposed to gadolinium contrast agents (the percentage is
unclear) have developed NSF, a syndrome that can be fatal. Further
studies are necessary to determine what the exact relationships are
between gadolinium-containing contrast agents, their specific components
and stoichiometry, patient renal function and NSF. Current theory links
the development of NSF to the administration of relatively high doses
(e.g., \>0.2 mM/kg) and to agents in which the gadolinium is least
strongly chelated. The FDA has recently issued a \"black box\" warning
concerning these contrast agents
(<http://www.fda.gov/cder/drug/InfoSheets/HCP/gcca_200705HCP.pdf>.)

This warning recommends that, until further information is available,
gadolinium contrast agents should not be administered to patients with
either acute or significant chronic kidney disease (estimated GFR \<30
mL/min/1.73m^2^), recent liver or kidney transplant or hepato-renal
syndrome, unless a risk-benefit assessment suggests that the benefit of
administration in the particular patient clearly outweighs the potential
risk(s).

**Abbreviations**

-   CT, computed tomography

-   CTA, computed tomography angiography

-   INV, invasive

-   IP, in progress

-   Min, minimal

-   MRA, magnetic resonance angiography

-   MRI, magnetic resonance imaging

-   NUC, nuclear imaging

-   TEE, transesophageal echocardiography

-   TTE, transthoracic echocardiography

-   US, ultrasound

#### CLINICAL ALGORITHM(S)

None provided

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The recommendations are based on analysis of the current literature and
expert panel consensus.

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

Appropriate selection of initial radiologic exam procedures for
suspected congenital heart disease

#### POTENTIAL HARMS

-   The relative radiation level is high for computed tomography (CT)
    angiography of the coronary arteries and CT of the heart with
    contrast.

-   There is a small risk of bacterial endocarditis from transesophageal
    echocardiography (TEE).

-   Sternal wires, vascular clips, biosynthetic valve rings, and
    mechanical valves can all interfere with the quality of magnetic
    resonance (MR) images and thereby limit their clinical value.

-   Some patients with renal failure who have been exposed to
    gadolinium-based MR contrast agents (the percentage is unclear) have
    developed nephrogenic systemic fibrosis, a syndrome that can be
    fatal. Until further information is available, gadolinium contrast
    agents should not be administered to patients with either acute or
    significant chronic kidney disease (estimated GFR \<30
    mL/min/1.73m^2^), recent liver or kidney transplant or hepato-renal
    syndrome, unless a risk-benefit assessment suggests that the benefit
    of administration in the particular patient clearly outweighs the
    potential risk(s)

### CONTRAINDICATIONS

#### CONTRAINDICATIONS

-   Pacemakers are generally considered an exclusion for magnetic
    resonance imaging (MRI), although it has been performed safely in
    patients with pacemakers under rigorously safe conditions.

-   The use of gadolinium (Gd) chelate contrast agents may not be
    possible in a patient with known severe allergy to Gd.

-   Detection of calcification is a problem for MRI, so adults with
    homografts or bioprosthetic valved conduits in whom the detection of
    calcification implies deterioration may not be optimally imaged.

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

An American College of Radiology (ACR) Committee on Appropriateness
Criteria and its expert panels have developed criteria for determining
appropriate imaging examinations for diagnosis and treatment of
specified medical condition(s). These criteria are intended to guide
radiologists, radiation oncologists, and referring physicians in making
decisions regarding radiologic imaging and treatment. Generally, the
complexity and severity of a patient\'s clinical condition should
dictate the selection of appropriate imaging procedures or treatments.
Only those exams generally used for evaluation of the patient\'s
condition are ranked. Other imaging studies necessary to evaluate other
co-existent diseases or other medical consequences of this condition are
not considered in this document. The availability of equipment or
personnel may influence the selection of appropriate imaging procedures
or treatments. Imaging techniques classified as investigational by the
U.S. Food and Drug Administration (FDA) have not been considered in
developing these criteria; however, study of new equipment and
applications should be encouraged. The ultimate decision regarding the
appropriateness of any specific radiologic examination or treatment must
be made by the referring physician and radiologist in light of all the
circumstances presented in an individual examination.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

An implementation strategy was not provided.

#### IMPLEMENTATION TOOLS

Personal Digital Assistant (PDA) Downloads

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Living with Illness

#### IOM DOMAIN

Effectiveness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Ho VB, Yucel EK, Khan A, Haramati LB, Mammen L, Rozenshtein A, Rybicki
FJ, Schoepf UJ, Stanford W, Stein B, Woodard PK, Jaff M, Expert Panel on
Cardiovascular Imaging. Suspected congenital heart disease in the adult.
\[online publication\]. Reston (VA): American College of Radiology
(ACR); 2007. 8 p. \[55 references\]

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

1998 (revised 2007)

#### GUIDELINE DEVELOPER(S)

American College of Radiology - Medical Specialty Society

#### SOURCE(S) OF FUNDING

The American College of Radiology (ACR) provided the funding and the
resources for these ACR Appropriateness Criteria®.

#### GUIDELINE COMMITTEE

Committee on Appropriateness Criteria, Expert Panel on Cardiovascular
Imaging

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Panel Members*: Vincent B. Ho, MD; E. Kent Yucel, MD; Arfa Khan, MD;
Linda B. Haramati, MD; Leena Mammen, MD; Anna Rozenshtein, MD; Frank J.
Rybicki, MD, PhD; U. Joseph Schoepf, MD; William Stanford, MD; Barry
Stein, MD; Pamela K. Woodard, MD; Michael Jaff, MD

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

Not stated

#### GUIDELINE STATUS

This is the current release of the guideline.

It updates a previously published version: American College of Radiology
(ACR), Expert Panel on Cardiovascular Imaging. Suspected congenital
heart disease in the adult. Reston (VA): American College of Radiology
(ACR); 2002. 6 p.(ACR appropriateness criteria). \[38 references\]

The appropriateness criteria are reviewed annually and updated by the
panels as needed, depending on introduction of new and highly
significant scientific evidence.

#### GUIDELINE AVAILABILITY

Electronic copies: Available in Portable Document Format (PDF) from the
[American College of Radiology (ACR) Web
site](http://www.acr.org/SecondaryMainMenuCategories/quality_safety/app_criteria/pdf/ExpertPanelonCardiovascularImaging/SuspectedCongenitalHeartDiseaseintheAdultUpdateinProgressDoc18.aspx).

ACR Appropriateness Criteria® *Anytime*, *Anywhere*™ (PDA application).
Available from the [ACR Web
site](http://www.acr.org/SecondaryMainMenuCategories/ACRStore/FeaturedCategories/QualityandSafety/ac_pda.aspx).

Print copies: Available from the American College of Radiology, 1891
Preston White Drive, Reston, VA 20191. Telephone: (703) 648-8900.

#### AVAILABILITY OF COMPANION DOCUMENTS

The following are available:

-   ACR Appropriateness Criteria®. Background and development. Reston
    (VA): American College of Radiology; 2 p. Electronic copies:
    Available in Portable Document Format (PDF) from the [American
    College of Radiology (ACR) Web
    site](http://www.acr.org/SecondaryMainMenuCategories/quality_safety/app_criteria.aspx).

-   ACR Appropriateness Criteria®. Relative radiation level information.
    Reston (VA): American College of Radiology; 2 p. Electronic copies:
    Available in Portable Document Format (PDF) from the [American
    College of Radiology (ACR) Web
    site](http://www.acr.org/SecondaryMainMenuCategories/quality_safety/app_criteria/RRLInformation.aspx).

#### PATIENT RESOURCES

None available

#### NGC STATUS

This summary was completed by ECRI on February 20, 2001. The information
was verified by the guideline developer on March 14, 2001. This summary
was updated by ECRI on March 31, 2003. The updated information was
verified by the guideline developer on April 21, 2003. This NGC summary
was updated by ECRI Institute on November 12, 2007.

#### COPYRIGHT STATEMENT

Instructions for downloading, use, and reproduction of the American
College of Radiology (ACR) Appropriateness Criteria® may be found on the
[ACR Web
site](http://www.acr.org/SecondaryMainMenuCategories/quality_safety/app_criteria/ACRAppropriatenessCriteriaTermsandConditionsDoc1.aspx).

### DISCLAIMER

#### NGC DISCLAIMER

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© 1998-2008 National Guideline Clearinghouse

Date Modified: 11/10/2008