GUIDELINES AND STANDARDS

Recommendations for Multimodality
Assessment of Congenital Coronary
Anomalies: A Guide from the American
Society of Echocardiography
Developed in Collaboration with the Society for Cardiovascular
Angiography and Interventions, Japanese Society of Echocardiography,
and Society for Cardiovascular Magnetic ResonancePeter Frommelt, MD, FASE, Leo Lopez, MD, FASE, V. Vivian Dimas, MD, FSCAI, Benjamin Eidem, MD, FASE,
B. Kelly Han, MD, FASE, H. Helen Ko, BS, ACS, RDMS, RDCS, RCCS, FASE, Richard Lorber, MD, FASE,
Masaki Nii, MD, PhD, Beth Printz, MD, PhD, FASE, Shubhika Srivastava, MBBS, FASE,
Anne Marie Valente, MD, FASE, FSCMR, and Meryl S. Cohen, MD, FASE, Milwaukee, Wisconsin; Palo Alto andSan Diego, California; Dallas and San Antonio, Texas; Rochester and Minneapolis, Minnesota; New York, New York;
Shizuoka, Shizuoka, Japan; Boston, Massachusetts; Philadelphia, Pennsylvania
Keywords: Coronary artery, Congenital heart disease, Pediatric, Echocardiography

In addition to the collaborating societies listed in the title, this document is endorsed by the following American Societyof Echocardiography International Alliance Partners: Argentine Federation of Cardiology, Argentine Society ofCardiology, Asian-Pacific Association of Echocardiography, Australasian Sonographers Association, British Society of
Echocardiography, Canadian Society of Echocardiography, Cardiovascular Imaging Society of the Interamerican
Society of Cardiology (SISIAC), Chinese Society of Echocardiography, Department of Cardiovascular Imaging of theBrazilian Society of Cardiology, Indian Academy of Echocardiography, Iranian Society of Echocardiography, Mexican
Society of Echocardiography and Cardiovascular Imaging (SOME-ic), National Society of Echocardiographyof Mexico (SONECOM), Philippine Society of Echocardiography, Saudi Arabian Society of Echocardiography, Thai
Society of Echocardiography, Venezuelan Society of Cardiology.
TABLE OF CONTENTS
Summary 260
I. Introduction 260
II. Imaging Techniques for Assessment of Coronary Anomalies
A. Echocardiography 261
B. Cardiac Magnetic Resonance Imaging
262
C. Cardiac Computed Tomography 263
D. Nuclear Myocardial Perfusion Imaging
264
E. Angiography 264
III. Isolated Congenital Coronary Anomalies 265

261

From the Children’s Hospital of Wisconsin and the Medical College of Wisconsin,
Milwaukee, Wisconsin (P.F.); Stanford University, Palo Alto, California (L.L.);
University of Texas Southwestern, Dallas, Texas (V.V.D.); Mayo Clinic,
Rochester, Minnesota (B.E.); Children’s Minnesota and the Minneapolis Heart
Institute, Minneapolis, Minnesota (B.K.H.); Kravis Children’s Hospital, Mount
Sinai Medical Center, New York, New York (H.H.K. and S.S.); Baylor College ofMedicine, Children’s Hospital of San Antonio, San Antonio, Texas (R.L.);
Shizuoka Children’s Hospital, Shizuoka, Shizuoka, Japan (M.N.); University ofCalifornia San Diego and Rady Children’s Hospital, San Diego, California (B.P.);
Boston Children’s Hospital, Brigham and Women’s Hospital, Harvard Medical
School, Boston, Massachusetts (A.M.V.), The Children’s Hospital of Philadelphia,
Philadelphia, Pennsylvania (M.S.C.).
The following authors reported no actual or potential conflicts of interest in relation
to this document: Peter Frommelt, MD, FASE, Leo Lopez, MD, FASE, V. Vivian Dimas, MD, FSCAI, Benjamin Eidem, MD, FASE, B. Kelly Han, MD, FASE, H. HelenKo, BS, ACS, RDCS, FASE, Richard Lorber, MD, FASE, Masaki Nii, MD, PhD,

A. Anomalous Aortic Origin of a Coronary Artery 265
1. Background 265
2. Goals of Echocardiographic Imaging 267
3. Additional Imaging Techniques
267
B. Anomalous Pulmonary Origin of a Coronary Artery 269
1. Background 269
2. Goals of Echocardiographic Imaging 270
a. Pre-operative Assessment 270
b. Post-operative Assessment 272

Beth Printz, MD, FASE, Shubhika Srivastava, MD, FASE, Anne Marie Valente,
MD, FASE, FSCMR, Meryl S. Cohen, MD, FASE.
Reprint requests: American Society of Echocardiography, Meridian Corporate Center,
2530 Meridian Parkway, Suite 450, Durham, NC 27713 (E-mail: ase@asecho.org).
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Copyright 2019 by the American Society of Echocardiography.
https://doi.org/10.1016/j.echo.2019.10.011

259

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Abbreviations

2D = two-dimensional
AAOCA = anomalous aortic origin of a coronary artery
AAOLCA = anomalous aortic origin of the left coronary artery
AAORCA = anomalous aortic origin of the right coronary artery
ALCAPA = anomalous left coronary artery from the pulmonary artery
ARCAPA = anomalous right coronary artery from the pulmonary artery
CA = coronary artery
CAF = coronary artery fistula
CHD = congenital heart disease
CMR = cardiac magnetic resonance
CCT = cardiac computed tomography
ECG = electrocardiographyb. Postoperative Assessment 283
3. Additional Imaging Techniques
283
C. Tetralogy of Fallot 284
1. Background 284
2. Goals of Echocardiographic Imaging 285
a. Preoperative Assessment 285
b. Postoperative Assessment 285
3. Additional Imaging Techniques
285
D. Truncus Arteriosus 286
1. Background 286
2. Goals of Echocardiographic Imaging 287
3. Additional Imaging Techniques
287
E. Coronary Anomalies Associated with Single Ventricle Lesions 288
1. Pulmonary Atresia With Intact Ventricular Septum 288
a. Background 288
b. Goals of Echocardiographic Imaging
288
c. Additional Imaging Techniques 288
2. Hypoplastic Left Heart Syndrome 289
a. Background 289
b. Goals of Echocardiographic Imaging
289
c. Additional Imaging Techniques 289

HLHS = hypoplastic left heart syndrome
LAD = left anterior descending

SUMMARY

LCx = left circumflex
LCA = left coronary artery
LV = left ventricle
MR = mitral regurgitation
PET = positron emission tomography
PA = pulmonary artery
RCA = right coronary artery
RV = right ventricle
SPECT = single-photon emission computed tomography
SVAS = supravalvular aortic stenosis
TOF = tetralogy of Fallot
TEE = transesophageal echocardiography
TGA = transposition of the great arteries
TTE = transthoracic echocardiography
VSD = ventricular septal defect
3. Additional Imaging Techniques 272
C. Isolated Congenital Coronary Artery Fistulas 274
1. Background 274
2. Goals of Echocardiographic Imaging
276
a. Pre-intervention Assessment 276
b. Post-interventional Assessment 276
3. Additional Imaging Techniques 276
IV. Congenital Coronary Anomalies Associated with Other Congenital
Heart Diseases 278
A. Supravalvular Aortic Stenosis 278
1. Background 278
2. Goals of Echocardiographic Imaging
279
3. Additional Imaging Techniques 280
B. Transposition of the Great Arteries 280
1. Background 280
2. Goals of Echocardiographic Imaging
281
a. Preoperative Assessment 281

Congenital coronary artery anomalies, both in isolation and associatedwith other forms of congenital heart disease, have been recognized asimportant lesions with significant potential morbidity and mortality,
including sudden cardiac death in children and adolescents.
Multimodality imaging techniques have demonstrated increasing utility in the characterization of most congenital coronary anomalies, bothin children and adults, and may reduce the need for diagnostic catheterization in many cases. This document provides multimodality
guidelines for optimization of imaging for congenital coronary anomalies, with a review of the benefits and limitations of the different imaging techniques (echocardiography, cardiac computed tomography,
cardiac magnetic resonance imaging, nuclear myocardial perfusion
imaging and angiography). Strategies for imaging congenital coronary
anomalies when the coronary anomaly is in isolation (anomalous
aortic origin of a coronary artery, anomalous left coronary from thepulmonary artery, and coronary artery fistulas) as well as coronary
anomalies associated with other congenital heart disease (supravalvular aortic stenosis, transposition of the great arteries, tetralogy of Fallot,
truncus arteriosus, pulmonary atresia with intact septum, and hypoplastic left heart syndrome) are described.
I. INTRODUCTION
Congenital coronary artery (CA) anomalies, both in isolation and associated with other forms of congenital heart disease (CHD), have beenrecognized as important lesions with significant potential morbidity
and mortality. Echocardiographic findings in patients with congenital
CA anomalies have been well characterized, and the feasibility and utility of complementary noninvasive CA imaging with cardiac computed
tomography (CCT) and cardiac magnetic resonance (CMR) have also
been described. Prospective identification of congenital CA anomalies
using transthoracic echocardiography (TTE) as a screening tool has
become routine and has helped dispel the notion that CA anatomy
cannot be identified noninvasively. In fact, multimodality imaging techniques have replaced cardiac catheterization as the primary tool forcharacterization of most congenital CA anomalies in children. Thishas become particularly relevant to those performing cardiac imaging,Frommelt et al 261

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with recognition that the prevalence of congenital CA anomalies associated with sudden cardiac death, specifically anomalous aortic originof a CA (AAOCA) from the opposite sinus of Valsalva, is estimatedat 0.7% [95% CI: 0.48-0.95%].1 These potentially lethal anomalies
are thus relatively common in the general population, and so evaluation of coronary artery takeoff and course should be included as partof a complete noninvasive imaging exam. With this background, it isapparent that a summary document providing multimodality guidelines for optimization of imaging for congenital CA anomalies isneeded. A review of the benefits and limitations of the different imaging techniques (Table 1) will preface descriptions of known congenital
CA conditions that warrant careful imaging assessment.

Table 1 Comparison of techniques for imaging coronary
arteries
Characteristic

TTE

IVUS

CMR

CCT

Cath

Spatial resolution++++++ +++++++ ++++

Temporal resolution+++++++ ++

varies +++Evaluation of origin/ostia

++

+++

+++

++++ +++

Evaluation of proximal course

++

+++

+++

++++ +++

Evaluation of distal courseand branches/dominance

+

-

++

+++

++++

Intracoronary structure+++++ ++++++++

Intracardiac morphology++++ -++++ +++++

Evaluation of extracardiac
structures++-++++ ++++ +Myocardial ischemia

++

-

+++

-

+++

Radiation exposure

-

-

-

+

+++

Iodinated contrast medium

-

-

-

++++ ++++

A. Echocardiography

Imaging duration

++

+++

+++

+

++++

Knowledge of the normal origin and course of the CAs is important inorder to recognize congenital CA anomalies. Each CA usually originates from its respective sinus above the aortic valve leaflets, withthe right CA (RCA) arising from the right sinus of Valsalva and theleft main CA (LCA) arising from the left sinus of Valsalva (Figure 1).
Echocardiographic techniques for imaging of CA origin and course
are well described.2-4 TTE is the ideal modality because it is risk-free,noninvasive, portable, and widely available. Echocardiography, with its
high temporal and spatial resolution, permits rapid visualization of soft tissue and cardiac chambers, and can be readily performed in all clinical settings. In young children, echocardiography usually does not require deep
sedation or general anesthesia as does cardiac catheterization or CMR
imaging, nor does it involve radiation exposure, unlike CCT or cardiac
catheterization. The CAs are small and superficial structures; thus,
optimal imaging is best performed with the highest frequency transducer
that allows for adequate penetration to maximize spatial resolution.
Depending on the ultrasound frequency that is used for scanning, theaxial resolution can range from 0.13 mm to 0.39 mm, corresponding
to 12 MHz and 4 MHz frequency transducers, respectively. In terms ofimaging the coronary arteries, one should follow basic ultrasound principles to optimize two-dimensional (2D) images by aligning the emitted Bmode pulses perpendicular to anatomic structures, and optimize color
flow mapping by orienting the color Doppler sector parallel to flow.
2D and color Doppler TTE imaging of CA origins should be part of acomplete examination in every child.5 This is best accomplished from aparasternal short-axis view, where both CA origins can be visualized byscanning the aortic sinuses above the aortic valve (Figure 2). If one considers the aortic root as a clock face when visualized in this view, theleft main CA origin is normally at approximately 4 o’clock and the
RCA origin is at approximately 11 o’clock. Additional color flow mappingof the interarterial space can also help identify an anomalous proximal
course. Clockwise rotation of the transducer in the parasternal shortaxis view (Figure 2B) frequently allows for imaging of the left main CA
as it bifurcates into the left anterior descending branch (LAD), whichcourses along the anterior interventricular groove, and the left circumflex
branch (LCx), which courses in the left anterior atrioventricular groove. In
contrast, counterclockwise transducer rotation can facilitate imaging of
the RCA. Color flow mapping is also important since documentationof direction and timing of flow is helpful in diagnosing anomalies(Figure 2C). It is paramount that the color flow map be positioned to profile flow from the sinus to the coronary ostium and then into its proximal

Sedation requirements in children ++++++ ++++ ++++++II. IMAGING TECHNIQUES FOR ASSESSMENT OF
CORONARY ANOMALIES

Cath, Cardiac catheterization; CCT, cardiac computed tomography;
CMR, cardiac magnetic resonance; IVUS, intravascular ultrasound;
TTE, transthoracic echocardiography.

course. The CAs usually have low-velocity flow; therefore, the Nyquist
limit should be decreased to about 20-40 cm/second, which is frequently
available as a CA evaluation preset on many ultrasound machines.
CA origin and course can also be assessed from other TTE views. In
infants and small children with good subcostal windows, the LCA
origin can be seen from a coronal plane, allowing for distinction between a coronary origin from the sinus, sinotubular junction, or
ascending aorta. The RCA origin is frequently best seen from a parasternal long-axis view with slight rightward tilt of the transducer, again
allowing identification of the coronary origin from the sinus, sinotubular junction, or ascending aorta. The LAD course along the anterior
interventricular groove can also be well seen in a parasternal long-axis
view with leftward anterior tilt as the imaging plane sweeps from theaortic root to the pulmonary trunk.
A limitation of TTE in imaging the CAs is that they are superficial cardiac structures, with potential acoustic interference by the ribs, lungs,
and pleura. In addition, the rapid heart rates in neonates can makethe evaluation of CAs even more challenging, while larger body habitus
in older children and adults may limit acoustic windows. Spatial resolution can sometimes be limited, making it difficult to distinguish whether
a CA is arising from a particular cusp or an area adjacent to that cusp.
When there is anomalous origin of a CA, false dropout can create theappearance of normal CA origin from the appropriate sinus, especiallyin the setting of a prominent transverse sinus. Therefore, still frames ofCA origins should never be used in isolation. Lastly, the distinction between an interarterial (between the aorta and the pulmonary artery[PA]) and an intramural course (traveling within the wall of the aorta)
is difficult by echocardiography. This is discussed in more detail below.
Transesophageal echocardiographic (TEE) imaging of CA origins andtheir proximal course can also be a complementary technique, particularly when there are poor TTE windows as seen in obese patients or thosewith lung disease. This is best accomplished with a multiplane probe fromthe mid-esophageal window just above the aortic valve. The LCA and its
branches are best seen with the imaging plane at approximately 20-50 ,whereas the RCA is best seen at 90-120 . Color flow mapping of bothCAs is also important to evaluate the direction and timing of flow.

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Figure 1 Illustration (A), pathology specimen (B), and cardiac CT (C) of normal coronary origins from the aorta (Ao), demonstrating the
epicardial position of the major coronary branches, including the right coronary artery (RCA), left coronary artery (LCA), left anterior
descending (LAD), and circumflex (CFX) coronary arteries. PA, Pulmonary artery; SVC, superior vena cava.
B. Cardiac Magnetic Resonance Imaging
CMR is frequently used as a complementary imaging modality toechocardiography for evaluation of suspected or confirmed congenital CA anomalies. CMR has some advantages over other imaging
modalities because it requires no radiation exposure and is not dependent on acoustic windows. Several techniques are typically utilized aspart of a comprehensive CMR exam to assess patients with known orsuspected congenital CA anomalies.6 Three-dimensional (3D) image
datasets acquired by CMR can be reformatted into any imaging planeto aid in identification of anomalies of CA origin and proximal course,
to assess the relation of the CAs to adjacent structures, and to evaluate
for the presence of associated anatomic abnormalities. CMR representation of CA anatomy can be displayed in standard axial, sagittal,
and coronal projections, or the images can be reformatted into ‘echoequivalent’ views that facilitate multimodality comparison of CA
anatomy. Specialized post-processing techniques and display formatting include virtual angiography, where images are presented as if theviewer is sitting within the CA lumen itself,7-9 allowing for theassessment of the shape and caliber of the ostium. CMR perfusion
imaging can be added to help identify perfusion defects in patientswith CA anomalies before and/or after surgical intervention, either
at rest or with pharmacologic stress.

Most commonly when screening for CA anomalies, an isotropic(i.e., of equal value from all orientations) set of images is acquired
from above the level of the aortic root to the ventricular apex utilizing
electrocardiographic (ECG) triggering, fat saturation (nulling the CMR
signal from fat), T2-preparation (to increase the ability to differentiate
CA flow from the adjacent myocardium), and a respiratory navigatorto track diaphragm motion and correct for cranial-caudal motion of
the heart during image acquisition.10 An alternative to acquisition ofthis so-called ‘whole heart’ method is volume-targeted imaging, whereseparate smaller 3D volumetric slabs targeted to the particular CA segment(s) of interest are acquired; this technique may be more suited toconfirmation of a known CA anomaly rather than comprehensive
evaluation of the entire CA system.11,12 ECG triggering to confine image acquisition to a specified time in the cardiac cycle with the least CA
motion has been used to visualize CAs in younger children and otherswith higher heart rates.13-15
CA anomalies are associated with increased risk of myocardialischemia, especially with exertion, which can be assessed by CMR
pharmacologic stress imaging. Agents used include those that thatcause increased myocardial oxygen demand (such as dobutamine)or increased CA blood flow (such as PersantineÒ, adenosine, or regadenoson).16-18 Intravenous gadolinium-based contrast agents are typically given to enhance intravascular signal intensity, optimize CAJournal of the American Society of Echocardiography
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Figure 2 Parasternal short-axis images of the normal CA origins from the aortic root (Ao). In A, the normal origins of the left main coronary
artery (LCA) from the left sinus of Valsalva and right coronary artery (RCA) from the right sinus of Valsalva are well visualized. With clockwise
rotation of the probe from this position (B), a longer length of the left main coronary and its bifurcation into the left anterior descending (LAD)
and circumflex (LCx) branches are visualized. Finally, color Doppler assessment of flow direction in the coronaries can be obtained (C) bylowering the color Nyquist limit (in this case to 23 cm/s) to visualize the low-velocity diastolic anterograde flow in the left coronary, seen as a
red color flow signal coursing appropriately toward the transducer and away from the aortic root during diastole.
image resolution, and visualize late myocardial enhancement as amarker of fibrosis/infarction. A matched defect on both contrastenhanced rest and stress imaging, with evidence of post-contrast
myocardial late gadolinium enhancement, identifies an irreversible
perfusion defect, while a perfusion defect during stress but not at
rest is classified as reversible. Recently, adenosine stress and rest imaging using T1 mapping has been shown to differentiate normal fromischemic or infarcted myocardium without the need for gadolinium
contrast.19
CMR limitations for CA imaging include: 1) the need for cooperative patients in order to obtain high quality imaging, thereby
requiring deep sedation or general anesthesia for infants and young
children; 2) lower spatial and temporal resolution of CMR
compared with TTE, which can be exacerbated in infants and smallchildren because of small patient size, high heart rates, and respiratory motion artifact,20 so that assessment of coronary origins, angleof origin, and/or length and course of anomalous segments may beinadequately demonstrated; 3) suboptimal imaging in the setting ofarrhythmias or irregular heart rate; 4) image degradation by artifact
from ferromagnetic materials following catheter-based or surgicalinterventions; 5) implanted pacemakers or cardioverter defibrillators (which are relative contraindications); 6) inability to use
gadolinium-based contrast agents in advanced renal disease; and7) poor correlation of CA measurements obtained by CMR with
2D echo-derived CA measurements, precluding the use of echocardiographic reference values for CMR-derived CA size measurements.15

C. Cardiac Computed Tomography
Modern cardiovascular multidetector CT is also an excellent complementary technique for evaluation of congenital CA anomalies.
Temporal resolution is as low as 66 msec, allowing definitive CA imaging even at fast heart rates, which is an advantage over CMR.
Modern multislice scanners with appropriate spatial resolution andthe use of ECG gating are required to adequately image the CAs inpediatric patients. Excellent visualization of CA origins can beachieved using CCT in infants with tetralogy of Fallot,21 and lower radiation exposure at an estimated dose of approximately 1 millisievert
has been reported with successful imaging of the CAs in pediatric patients with CHD.22,23
CCT imaging can provide high resolution images of the distal vessels with 3D display of the CAs and their relationship to extracardiac
structures and the sternum, as well as detection of calcified and stenotic CA segments. As with CMR imaging, ventricular function andassociated cardiovascular anomalies can be evaluated from thesame dataset used for CA imaging. CCT images of CAs should also
be projected in echo-equivalent views for consistent interpretation
and reporting. Optimizing CCT techniques for imaging specific CA
lesions should include: a) consideration of heart rate-lowering medications or CA vasodilators24 since lower radiation doses are achieved
for slow and steady heart rates25,26; b) consideration of suspended
respiration for high resolution imaging in patients unable to cooperate, particularly if distal CA vessel visualization is required; c) use
of low tube potential and automated tube current modulation27,28;d) use of ECG pulse modulation with a narrow acquisition window

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Figure 3 Variation in coronary course with anomalous aortic origin. Select illustrations of the possible variety of coronary artery anomalies. Panel A: Normal coronary anatomy; B: Anomalous aortic origin of the right coronary artery (AAORCA) with right coronary artery(RCA) originating from the left coronary sinus with a retro-aortic course; C: Interarterial AAORCA with the RCA originating from the left
sinus with an intramural course in the anterior aortic wall; D: ‘‘Single left’’ coronary artery with all coronaries arising from the left sinus
and RCA then coursing anterior to the PA. E: Interarterial anomalous aortic origin of the left coronary artery (AAOLCA) with the left
coronary originating from the right coronary sinus with an intramural course; F: Intraconal AAOLCA with the left coronary originatingfrom the right sinus and coursing within the myocardial sulcus below the right ventricular outflow tract; G: AAOLCA with the left coronary originating from the right sinus with a retro-aortic course; H: AAOLCA with the left coronary originating from the non-coronary
sinus with a retro-aortic course; I-K: Anomalous aortic origin of the left anterior descending coronary artery from the right sinus, withan anterior course in front of the pulmonary artery (I), an interarterial course (J), and a retro-aortic course after originating from the rightcoronary artery (K). Illustration created by Maansi Dayal Srivastava.
adjusted to heart rate; e) use of prospective ECG triggering sequence
whenever possible, reserving retrospective ECG gating for highly
irregular heart rates such as atrial fibrillation29; f) use of iterative
reconstruction techniques; g) patient-specific tailoring of image quality requirements and radiation dose optimization, since the image
quality required for high resolution CA imaging is often not needed
to determine CA location and course in patients with CHD andshould be reserved for patients with symptoms or those at risk for
CA lesions (such as those with supravalvular aortic stenosis); and h)patient-specific tailoring of the contrast administration when indicated so that all information for a patient with CHD can be obtainedin a single scan whenever possible (e.g., a patient with tetralogy of
Fallot should have a contrast injection protocol that will allow visualization of both right- and left-sided structures, including CAs, in thesame scan acquisition).
D. Nuclear Myocardial Perfusion Imaging
Nuclear myocardial perfusion imaging is not a sensitive or accurate
technique for detecting anomalous coronary artery origin, but isfrequently used to assess myocardial perfusion when a coronary
anomaly places a patient at risk for ischemia. Single-photon emission
computed tomography myocardial perfusion imaging (SPECT) is
extensively utilized in adults to evaluate cardiac perfusion and func-tion at rest and during dynamic exercise or pharmacologic stress forthe diagnosis and management of patients with chest pain andpossible CA ischemia. 99m-technetium (Tc99m)-labeled perfusion
agents (99m-Tc-sestamibi and 99m-Tc-tetrofosmin) are the mostcommonly used radioisotopes. The sensitivity of stress myocardial
perfusion imaging in children is reported to be 70-90%, while thespecificity is about 60%.30 The relatively high false-positive rate results from motion artifact during image acquisition, frequently related
to respiration, and slow liver isotope clearance. New ultrafast SPECT
cameras with cadmium-zinc-telluride-based detectors are faster andproduce higher quality images with lower isotope dose, resulting insignificantly reduced ionizing radiation exposure and decreased imaging times as compared to conventional SPECT cameras.31 A stressonly imaging protocol can decrease the length of the test from
3-4 hours to less than 90 minutes and decrease the radiation doseby 30–60%. Limited data are available on the utility of positron emission tomography (PET) in the evaluation of perfusion defects inchildren, and it is more expensive and not as readily available.
E. Angiography
Even as noninvasive imaging continues to improve, cardiac catheterization remains an important imaging modality in pediatric patientswith CA anomalies. Advantages lie in the ability to evaluate the entire
CA course and distribution in small infants and children with higher

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Figure 4 Illustration (A) and pathology specimens (B and C) of intramural anomalous left coronary artery originating from the rightsinus of Valsalva. The cartoon shows the typical long intramural course of the left main coronary artery (LMCA) as it arises fromthe right sinus and courses within the anterior aortic wall before exiting the aortic root from the left sinus of Valsalva to provide thenormally distributed left anterior descending (LAD) and circumflex (LCx) coronaries. Similar to A, Figure 4B demonstrates the anatomy
from the echocardiographer’s view, with the pulmonary valve (Pulm. valve) anterior, and the intramural segment of the left coronary
artery (LCA) has been opened (bracket) as it courses through the anterior aortic wall. In C, with the orientation inverted, the slit-likelength of the intramural segment within the aortic wall is clearly seen with the right coronary artery (RCA) also arising from the rightsinus of Valsalva in its normal position. The illustration is from Frommelt MA, Frommelt PC. Vascular abnormalities. In: Eidem BW,
O’Leary PW, Cetta F, eds. Echocardiography in pediatric and adult congenital heart disease. 2nd ed. Philadelphia, PA: Wolters Kluwer
Health; 2015:470-485. The pathology images are from the Web Portal of the Archiving Working Group of The International Society for
Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD) (http://ipccc-awg.net) and courtesy of Diane E. Spicer BS, PA(ASCP), University of Florida, Department of Pediatric Cardiology.

Figure 5 Short-axis image in a patient with interarterial AAOLCA with an intramural course of the anomalous coronary. In the 2dimensional image (A), the left coronary appears to be exiting the aorta (Ao) appropriately from the left sinus of Valsalva (large arrow);
however, on closer inspection, a long anomalous intramural segment can be appreciated coursing within the anterior aortic wall (small
arrows). Color Doppler imaging (B) shows the linear diastolic flow of the anomalous coronary within the anterior aortic wall (arrows);
the blue color signal confirms anomalous coronary flow away from the transducer, consistent with the coronary originating from theright sinus and coursing toward the more posteriorly positioned left sinus. A low Nyquist limit is needed to visualize the low-velocity
coronary flow signal.
heart rates, whereas ECG gating and spatial resolution can be challenging with other noninvasive imaging modalities.20 Selective angiography is also particularly useful in situations where small discrete
CA occlusion or interruption exists with vessel reconstitution by collaterals. Distal distribution of flow and patency is also best assessed bydirect imaging with catheterization as there remain limitations byother noninvasive means of imaging.32,33 Finally, intravascular ultrasound, to directly image CA wall architecture, and fractional flow
reserve, to quantify flow changes across CA stenosis, are additional
invasive adjunct techniques to assess anomalous CA pathology.
Angiography may also be preferentially used at institutions whereexpertise and technical support to perform pediatric CCT/CMR islacking. As with other modalities requiring radiation such as CCT,
the radiation dosing with newer equipment is significantly reduced
without reduction in image quality.34
III. ISOLATED CONGENITAL CORONARY ANOMALIES

A. Anomalous Aortic Origin of a Coronary Artery
1. Background. Anomalous aortic origin of a coronary artery(AAOCA) is a congenital anomaly whereby a CA arises from the266 Frommelt et al

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Figure 6 Short-axis imaging in a patient with interarterial AAORCA and an intramural course of the anomalous coronary. In A, theposition of the aortic sinuses at the level of the aortic valve are visualized; the commissure between the right (R) and left (L) cusps
is identified by an arrow. The non-coronary sinus (N) is seen posteriorly. Tilting the transducer more superiorly above the valve leaflets(B), the anomalous right coronary artery (RCA) can be seen arising at an acute angle from the left sinus of Valsalva near the origin of theleft main coronary artery (LCA) and coursing intramurally within the anterior aortic (Ao) wall (arrows) between the aorta and the rightventricular outflow tract toward the right sinus of Valsalva. Comparing A and B, it can be appreciated that the anomalous coronary
originates close to the commissure but clearly from the left sinus. Color Doppler imaging (C) shows the linear diastolic flow of theanomalous CA within the anterior aortic wall (arrows) between the aorta and pulmonary artery (PA); the red color signal confirms
anomalous coronary flow toward the transducer, consistent with the coronary originating from the left sinus and coursing toward
the more anteriorly positioned right sinus. Note the low Nyquist limit (12 cm/s) needed to visualize the low-velocity coronary flow
signal. From Frommelt MA, Frommelt PC. Vascular abnormalities. In: Eidem BW, O’Leary PW, Cetta F, eds. Echocardiography in pediatric and adult congenital heart disease. 2nd ed. Philadelphia, PA: Wolters Kluwer Health; 2015:470-485.
opposite or contralateral sinus of Valsalva; in rare instances a CA canarise from the non-coronary sinus. This anomaly has been associatedwith myocardial ischemia and sudden death in children and adolescents, particularly when the anomalous CA courses between the greatarteries.35-39 In fact, it is the second leading cause of sudden cardiac
death in young athletes.38 Noninvasive imaging is frequently the firstmodality utilized to make the diagnosis. The incidence of AAOCA is
estimated at 0.7%, and anomalous aortic origin of the RCA from theleft sinus of Valsalva (AAORCA) is six times more prevalent thananomalous origin of the LCA from the right sinus of Valsalva(AAOLCA).1 The anomalous CA can arise from the opposite sinus
and course anterior to the pulmonary artery (PA), posterior to theaorta (retro-aortic), or between them (interarterial) (Figure 3).
AAOCA with an interarterial course is associated with increased
risk for cardiac complications.
An interarterial CA can course within the myocardium between
the great arteries (septal, intramyocardial, or intraconal course) orwithin the anterior wall of the aorta between the great arteries (intramural). AAOLCA with an interarterial and intramural course is associated with the greatest risk for sudden cardiac death (Figure 4),although AAORCA with an intramural course has also been associated with sudden death. The etiology of sudden death in individuals
with an interarterial AAOCA is likely myocardial ischemia, especiallyin younger patients (age <40 years) during or following strenuous activity, resulting from proximal vessel narrowing, ostial stenosis, anacute angle of origin of the anomalous CA, and/or compression/
distortion of the interarterial segment with increased cardiac output
during vigorous activity.40,41 Anomalous origin of the LCA fromthe non-coronary sinus with an intramural course and sudden cardiac
death has also been reported.40
The need for and timing of surgical intervention for AAOCA isquite controversial, with documented symptoms/signs of myocardialischemia as the most common indication. Multiple surgical techniques have been used, including CA bypass graft placement, patch
enlargement of the anomalous CA origin, reimplantation of theanomalous CA to the appropriate sinus, translocation of the pulmonary trunk, and unroofing of the intramural segment of the anomalous CA. The unroofing procedure is commonly used, particularly
when there is an intramural CA, where the shared wall of the intramural segment is removed with creation of a neo-ostium in theJournal of the American Society of Echocardiography
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Figure 7 Parasternal long-axis imaging of an interarterial and intramural anomalous coronary coursing between the great arteries within the anterior aortic wall; the coronary can be seen
as a discrete circle (arrow) immediately anterior to the aortic(Ao) root near the sinotubular junction.
appropriate sinus.42 Imaging of AAOCA anatomic features should
involve delineation of the origin and course that provides the necessary information for optimal management, including the need andtype of surgical intervention. Unfortunately, despite multiple imaging
techniques that allow identification of AAOCA, effective risk stratification remains elusive with variable recommendations for best practices.9,43
2. Goals of Echocardiographic Imaging. TTE can prospectively
identify AAORCA and AAOLCA, particularly when there is an interarterial course.44 Moreover, TTE is often the screening tool used to
identify this lesion because many children are asymptomatic, andthe diagnosis is often made when TTE has been ordered for other reasons. The CA origins can frequently be imaged by TTE when goodtransthoracic parasternal windows are available. The intramuralcourse of AAOCA can be difficult to detect by echocardiography,
particularly because the CA exit from the aortic wall is frequently at
the usual or appropriate sinus of Valsalva (Figure 5A) with an unusually high origin from the sinus. In addition, the location of the ostium
relative to the commissure/sinuses can sometimes be difficult tojudge, especially when the anomalous coronary arises close to thecommissure and/or has a high take-off.
The intramural segment in AAOLCA is longer and usually traverses at least half of the right sinus before exiting the aortic wallfrom the left sinus (Figure 5A). In contrast, the intramural course of
AAORCA is usually characterized by a shorter (2 to 3 mm) intramuralsegment from the left sinus with the anomalous CA ostium arising
adjacent to the commissure between the right and left coronary cusps(Figure 6A and B). An acute angle of ostial takeoff and linear diastolic
flow adjacent to the aortic wall (visualized by color Doppler) are hallmark features of an intramural course by TTE.40,45,46 Color Doppler
with a low Nyquist limit (20-30 cm/sec) is particularly useful because
it helps to differentiate whether the anomalous CA arises from theright or left sinus. With AAOLCA, a blue Doppler signal is seen in
the intramural segment as flow moves away from the right sinus toward the more posteriorly positioned left sinus (Figure 5C). In

Frommelt et al 267

AAORCA from the left sinus, a red Doppler signal will be seen in
the intramural segment as flow moves toward the right sinus fromits origin in the left sinus (Figure 6C).
Other transthoracic windows can also provide imaging evidence
for AAOCA. From the parasternal long-axis view, the anomalous
CA between the great arteries can be seen as a discrete circle immediately anterior to the aortic root as an initial clue (Figure 7). The parasternal long-axis view may also help identify a higher origin of the
RCA from the sinotubular junction or ascending aorta. Subcostal
scanning from the aortic root more anteriorly to the pulmonary trunk
can identify an anomalous CA between the great arteries, suggesting
an intramural course.40 It is important to remember, however, that no
imaging modality defines the histologic diagnosis of a shared tunica
media. Ostial stenosis with AAOCA is frequently a concern, but
despite reports that this can be identified by Doppler echocardiography,47 this is very challenging to diagnose using echocardiography
because of the need to use lower Nyquist limits for CA flow assessment, and so other imaging modalities are generally utilized tomake that assessment. An intraconal CA course can be identified
by visualization of the CA within the muscular conal septum inferior
to the pulmonary valve annulus in subcostal and parasternal views(Figure 8A-C); these intraconal and interarterial AAOCAs usuallyarise from a single coronary orifice (usually a single right CA).
Using transverse/axial esophageal imaging planes as previously
described for CA visualization, TEE can provide complementary imaging in the assessment of CA origins, especially when the CA is interarterial and intramural (Figure 9).
3. Additional Imaging Techniques. Intravascular ultrasound(IVUS), although invasive, has been utilized to assess CA ostial stenosis, CA hypoplasia and localized systolic lateral compression of the intramural segments of an anomalous CA that courses within the aortic
wall.48 Not surprisingly, this degree of compression can be variable,
likely causing unpredictable responses to exercise in this patientgroup. The CA narrowing can be exacerbated by a pharmacologic
challenge that mimics exercise conditions with changes in aortic
wall tension and resultant intramural coronary distortion.
The sagittal stack with CCT can provide sequential images of the
CA lumen adjacent to the aortic wall in the interarterial space, allowing for recognition of the acute angle takeoff, the CA course‘‘hugging’’ the aortic wall, and abrupt change in caliber as it separates from the aortic wall more distally from the opposite sinus,
all highly suggestive of an intramural course (Figure 10). The distance of the intramural or interarterial segment can be measured.
The change in both caliber and sphericity at the levels of theostium, the interarterial segment, and the distal vessel should be
evaluated to assess for change from circular to oval shape.49,50
3D reconstructions can help determine ostial and proximal coronary artery anatomy as well as the relationship of the anomalous
CA to the great arteries (Figure 11). An intraseptal or intraconal
CA course, as described above, can be imaged by following the
CA from the aortic sinus inferiorly to the interventricular septum
where the ‘‘hammock sign’’ is seen when the LCA courses into
the conal septum from the right and then exits the conus to its final
distal epicardial location, thus mimicking the appearance of ahammock (Figure 8D). Both CCT and CMR can help differentiate
the intraconal and intramural forms of AAOCA.8
CMR also can provide high-quality imaging for AAOCA(Figure 12) and has potential added value in documentation of ostialabnormalities7 and myocardial fibrosis (suggestive of infarction), as268 Frommelt et al

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Figure 8 Echocardiographic and CT imaging in a patient with AAOLCA and an intraconal course of the anomalous coronary. In A, asingle coronary originates from the aorta (Ao), arising from the right sinus of Valsalva and immediately bifurcating into the right and left
main coronaries (arrows). No coronary is seen arising from the left aortic sinus. In B, the anomalous left coronary can be seen coursing
between the aorta and right ventricular outflow tract (RVOT) within the myocardial sulcus before bifurcating into the left anterior descending (LAD) and circumflex (Cx) branches. Again, no coronary is seen arising from the left aortic sinus, and the anomalous coronary is clearly separate from the anterior aortic wall (helping to differentiate it from an intramural interarterial course). In C and D, the‘‘hammock sign’’ appearance of the anomalous coronary is demonstrated as it courses through the myocardial sulcus of the rightventricular outflow tract (arrows) below the pulmonary artery (PA). The echocardiographic image (C) is a subcostal view of the coronary course, and D shows the anomalous coronary and hammock sign by CT reconstruction. Echocardiographic images are from
Frommelt MA, Frommelt PC. Vascular abnormalities. In: Eidem BW, O’Leary PW, Cetta F, eds. Echocardiography in pediatric andadult congenital heart disease. 2nd ed. Philadelphia, PA: Wolters Kluwer Health; 2015:470-485.
well as assessment of myocardial perfusion at rest and with stress imaging.9,16,17 Thus far, none of the techniques for provocative myocardial perfusion testing has yielded additional risk stratification for
AAOCA, particularly when found in an asymptomatic child or young
adult serendipitously.

Key Points for Anomalous Aortic Origin of a Coronary Artery AAOCA is relatively common and usually benign, but certain
forms, particularly when a CA courses between the great arteries, are associated with sudden death.
 Echocardiography identifies AAOCA with an interarterial
course, and important markers include:
B Oblique origin of the anomalous CA from the opposite sinus
B When intramural, an anomalous course within the anterioraortic wall with an acute angled origin that can be demonstrated by color Doppler mapping

When intraconal, an anomalous course within the muscularconal septum inferior to the pulmonary valve annulus
 Complementary imaging techniques, particularly CCT and
CMR, can help define AAOCA anatomy, specifically the ostia,
caliber, and distal branching, but accurate risk stratification for
sudden death remains elusive.
 The utility of myocardial stress imaging and IVUS in asymptomatic patients in decision-making about intervention is also unclear.
B

Recommended Imaging Strategy for Anomalous
Aortic Origin of a Coronary Artery TTE should be the initial screening tool for suspected AAOCA,
particularly in forms that are associated with myocardialischemia (those with an interarterial course).
 When an interarterial AAOCA is identified, delineation of
anomalous ostial origin/size and course should be confirmedwith CCT and/or CMR.

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Figure 9 Transesophageal images from a mid-esophageal short-axis view through the aortic root in a patient with AAOLCA (A and B)
and AAORCA (C and D), both with an intramural course of the anomalous coronary. In A, the anomalous left coronary artery (LCA) canbe seen arising from the right sinus of Valsalva (R) and coursing intramurally within the anterior aortic wall (arrows) between the aorta
and right ventricular outflow tract toward the left sinus of Valsalva (L). Color Doppler (B) shows the direction of flow toward the transducer (red color signal) as the anomalous coronary courses from the more anteriorly positioned right sinus to the more posteriorly
positioned left sinus. In C, the anomalous RCA can be seen arising from the left sinus of Valsalva and coursing intramurally within
the anterior aortic wall (arrows) between the aorta and right ventricular outflow tract toward the right sinus of Valsalva. Color Doppler(D) again shows the direction of flow away from the transducer (blue color signal) as the anomalous coronary courses from the moreposteriorly positioned left sinus to the more anterior right sinus. In both examples, the non-coronary cusp (N) is seen posteriorly. From
Frommelt PC. Congenital coronary anomalies. In: Wong, Miller-Hance, Silverman, eds. Transesophageal Echocardiography forCongenital Heart Disease. New York, NY: Springer; 2014.

 Resting and stress myocardial perfusion assessment may be
used to help identify ischemia and/or correlate symptoms in
AAOCA.

B. Anomalous Pulmonary Origin of a Coronary Artery
1. Background. Anomalous origin of the LCA from the PA(ALCAPA) is a rare but well recognized CA anomaly, occurring in
1 in 300,000 children.51 The majority of these CA anomalies present
in infancy and constitute one of the most significant causes of myocardial ischemia and heart failure in the pediatric age group. Anomalous
origin of the RCA from the PA (ARCAPA), as well as anomalous
origin of LCA branches (such as the LCx) from the PA are much rarer
than ALCAPA and have a variable presentation. ARCAPA incidence
has been reported as 0.002%,52 or 0.12% of all CHD cases.53
ARCAPA is often associated with other congenital cardiac abnormalities, most commonly aorto-pulmonary window or tetralogy of
Fallot.52,54 In rare instances, ALCAPA and origin of the circumflex
CA from the PA has also been noted with other forms of congenital
heart lesions and should be considered as a possible etiology for worsening ventricular function and mitral regurgitation after repair. This

discussion will focus on the clinical and imaging findings in isolated
ALCAPA.
The typical time and mode of clinical presentation in patientswith ALCAPA are related to the transitional fall in PA pressuresand vascular resistance in the first weeks of life and the subsequent development of collateral CA flow from the RCA to supply
the LV myocardium. Infants with inadequate collateralization
develop signs of myocardial ischemia, which may present clinicallyas feeding intolerance, tachypnea, pallor, and failure to thrive.
Myocardial ischemia results in LV dilation and dysfunction, papillary muscle fibrosis/scarring and mitral regurgitation (MR), with
eventual development of dilated cardiomyopathy, worsening
MR, and heart failure. If left unrecognized, there is an overallmortality of 90% in those infants who do not undergo surgicalrepair in the first year of life.55
Children with ALCAPA who have well-developed collateral CA
flow will often have preserved LV systolic function and a delayed clinical presentation into adolescence or adulthood.55-57 This cohort does
not characteristically present with heart failure but rather comes toclinical suspicion by the presence of a cardiac murmur or exercise
intolerance; rarely, this cohort may also present with sudden
cardiac death as adults.58 Patients with isolated ARCAPA may present
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Figure 10 2D and 3D CT imaging of a patient with AAOLCA with an interarterial intramural course (A and B) and a patient with
AAORCA with an interarterial intramural course (C and D). In both cases, the intramural course (arrows) can be seen from shortaxis 2D imaging (A and C) and from 3D reconstructions (B and D) of the coronaries as the anomalous coronary arises obliquelyfrom the opposite sinus with a proximal course within the anterior aortic wall between the aorta (Ao) and pulmonary artery (PA).
The longer intramural course of the anomalous left coronary artery (LCA) compared to the anomalous right coronary artery (RCA)
can be appreciated, and the more superior origin of the anomalous RCA from above the sinotubular junction is also apparent.
more commonly are diagnosed serendipitously when an echocardiogram is performed in an asymptomatic older child or adult for otherindications (such as a heart murmur or cardiomegaly), or when assessed for associated CHD.
Surgical correction in patients with ALCAPA is performed to reestablish anterograde flow from the aorta to the LCA.59 Surgical intervention is indicated in all patients whenever the lesion is identified,regardless of age. Direct reimplantation of the anomalous CA into
the aorta is the surgery of choice59 and often results in rapid recovery
of myocardial function with excellent overall survival.60-63
Alternative surgical approaches, such as the Takeuchi procedure,64
rely on the creation of a tunnel from the PA to the aorta in patientswith an unfavorable anatomy for direct reimplantation (LCA origin
far from the aorta), but this procedure is associated with a higher incidence of postoperative complications, including supravalvular pulmonary stenosis and baffle leaks.65,66 Long-term remodeling after
surgical repair most commonly results in improved LV geometry,
increased LV ejection fraction, and decreased MR. Diastolic dysfunc-tion from the previous myocardial ischemia and infarction frequently
persists.
2. Goals of Echocardiographic Imaging. a. Pre-operative
Assessment.–TTE is the diagnostic imaging modality of choicein the identification of ALCAPA in infants, whereas alternative imaging modalities such as cardiac CCT, CMR, and/or cardiac catheterization may be needed to confirm the diagnosis in some infants
and in older children and adults when the diagnosis is unclear/
acoustic windows are limited. This is particularly true when thereis associated CHD. Goals of the pre-operative TTE examination
with 2D and color Doppler imaging include demonstration of
the anomalous origin of the LCA from the PA as well as the direction of flow within the LCA.67 In addition, characterizing thenormal origin of the RCA from the right sinus of Valsalva as wellas any dilation of the RCA system by both color Doppler and
2D imaging is also important.68,69 Finally, quantitative assessmentof biventricular size and function, mitral valve function, andJournal of the American Society of Echocardiography
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Figure 11 3D CT reconstructions of interarterial AAORCA and AAOLCA. In A, the anomalous right coronary artery (RCA) can be seenarising from the left (L) sinus of Valsalva in a short-axis equivalent view. The acute angle of origin from the left sinus (in contrast to theperpendicular angle of origin of the normally arising left coronary artery (LCA)) and narrowed ostium of the RCA (arrows) and its relationship to the aortic valve leaflets and right sinus (R) are clearly demonstrated. Similarly, in B, the anomalous LCA is seen arising at anacute angle from the right sinus and coursing posteriorly towards the left sinus. Figure C shows an endoluminal posterior view that
demonstrates severe ostial stenosis and anomalous origin of the RCA from the superior aspect of the left aortic sinus.

Figure 12 MRI coronary imaging from a short-axis equivalentview of a single left coronary artery arising from the left sinus
of Valsalva with the right coronary branch coursing anterior to
the aortic root to its usual distribution.

exclusion of additional associated cardiac lesions are also important, both clinically and surgically.
Direct TTE visualization of the anomalous CA origin from the PA isbest accomplished from the parasternal short-axis and long-axis scanplanes (Figure 13A). The anomalous LCA typically inserts into theposterior and leftward aspect of the main PA, coursing close to its
normal origin in the left sinus of Valsalva. This is the least commonly
identified finding, however, because dropout of the tissue separating
the CA from the aorta can give the false impression of a normal LCA
origin.70 Identification of retrograde filling of the LCA using color
Doppler is an essential echocardiographic component of the diagnosis of ALCAPA (Figure 13B). Color flow in the normally originating
LCA progresses away from the aortic root (red Doppler signal in parasternal short-axis view). In ALCAPA, color Doppler identifies retrograde LCA filling (flow reversal in the LCA from myocardium toPA), and this retrograde flow can often be seen emptying into themain PA.67 Very rarely, persistent pulmonary hypertension can maintain anterograde flow in the anomalous left coronary, so that retrograde flow is not appreciated.71 In older patients with delayed
clinical presentation, extensive collateralization that provides the LV
myocardium with its necessary CA supply results in RCA vesselsthat become significantly dilated (Figure 14A) and tortuous withprominent color flow Doppler signals within the ventricular septum272 Frommelt et al

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Figure 13 Parasternal short-axis images of anomalous origin of the left coronary artery from the posterior aspect of the proximal main pulmonary artery (PA) in an infant with ALCAPA. In A, the left anterior descending (LAD) and circumflex (Cx) coronary arteries are both seen arising from the left main coronary segment, clearly a long distance from the normal entrance into
the aortic root (Ao). Color Doppler interrogation of the left coronary (B) identifies retrograde flow in the LAD. Flow in the LAD isblue as it moves away from the transducer toward the PA, which is abnormal since blood flow should be away from the aortic
root (and thus displaying a red color Doppler signal) rather than toward it. A turbulent flow signal (arrows) is also seen in the
PA as the anomalous left coronary empties into the low-pressure PA. From Frommelt MA, Frommelt PC. Vascular abnormalities. In: Eidem BW, O’Leary PW, Cetta F, eds. Echocardiography in pediatric and adult congenital heart disease. 2nd ed. Philadelphia, PA: Wolters Kluwer Health; 2015:470-485.
and LV myocardium (Figure 14B).69 This prominent flow can bemistaken for multiple muscular ventricular septal defects. The timingof flow in the ventricular septum is helpful to make the distinction.
Finally, in patients with inadequate RCA collateralization, LV dilation with both systolic and diastolic LV dysfunction is often demonstrated. Bright endocardial scarring, almost always inclusive of themitral papillary muscles, is often evident on 2D imaging and is consistent with endocardial fibroelastosis. This myocardial and/or papillarymuscle scarring can result in significant MR, chamber dilation, andworsening heart failure (Figure 15).71 Echocardiographic findings of
ALCAPA are variably identified and are summarized in Table 270;of note, no finding is 100% identified by echocardiography, so othermodalities may be required to make the diagnosis if there is high suspicion based on the clinical presentation and/or echocardiographic
demonstration of LV dilation and endocardial fibroelastosis in an
infant.
b. Post-operative Assessment.–Serial TTE evaluation after
ALCAPA repair is essential to characterize the presence and extent
of LV remodeling, improvement in ventricular performance, andthe decrease in degree of MR.59,71 In most patients, there is significant
improvement in all of these parameters. However, LV dysfunction
and MR may persist and occasionally require additional intervention,
including mitral valve repair/replacement and, in rare cases, hearttransplantation. Ongoing assessment of LCA patency and adequacyof CA supply to the LV myocardium is also essential, with stress echocardiography and deformation imaging providing insights into thelong-term viability and functional reserve of the myocardium.72,73
Post-operative imaging assessment should also include evaluation of
the main PA in patients who undergo Takeuchi-type repair, as main
PA stenosis or baffle leak in the region of the baffled CA artery may
occur (Figure 16).74
3. Additional Imaging Techniques. As previously stated, additional imaging modalities may be required when there is high suspicion for ALCAPA but TTE is inconclusive, particularly in those withassociated CHD. They are used to better delineate residual and/or
recurrent cardiac abnormalities following ALCAPA surgical repair.

Although angiography has been the gold standard for confirmationof ALCAPA, CCT is now commonly used to confirm the origin
and course of suspected cases of ALCAPA (Figure 17) or ARCAPAin infants and children, and to better characterize collaterals as wellas to delineate CA anatomy after surgical intervention. ALCAPA isbest evaluated with a double oblique centerline obtained throughthe PA and evaluated cranially until the anomalous CA origin is visualized. Once this origin is visualized, the centerline should be placed in
the CA, displaying the length of the CA and the relationship to boththe aorta and the PA.75 The CA arising normally from the aorta isoften enlarged if it has supplied a significant amount of the anomalous
CA territory. Both angiography and CCT are excellent techniques forpost-op assessment of ALCAPA after surgical intervention, particularly if there are concerns about CA patency, either with direct reimplantation and with baffling procedures.
CMR can also be quite useful in the assessment of older children
and adults with ALCAPA or ARCAPA, both prior to and following
surgical repair.72,76 In particular, CMR has been used to assess for residual CA stenosis and/or thrombosis, degree of MR, and for the presence of regional myocardial dysfunction or myocardial fibrosis, bothat rest and with CMR adenosine stress perfusion imaging.77

Key Points for Anomalous Left Coronary Arteryfrom the Pulmonary Artery ALCAPA is a rare but treatable cause of dilated cardiomyopathy in infants and children and should be ruled out at presentation.
 Echocardiography is the diagnostic imaging modality of choicein ALCAPA, but other imaging techniques may be necessary if
the diagnosis is uncertain.
 Characteristic echocardiographic hallmarks in the diagnosis of
ALCAPA include:
B Direct visualization of the origin of the LCA from the PA by
2D imaging
B Retrograde color flow Doppler from the LCA into the PA
B Extensive RCA dilation and tortuosity by 2D imaging

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Figure 14 Parasternal short-axis imaging of a 10-year-old child with ALCAPA. In A, the right coronary artery (RCA) arises appropriately from the anterior aspect of the aorta (Ao) and is markedly dilated (arrows) because of the chronically increased flow into that
coronary, which measured 5.6 mm in diameter proximally. Color Doppler interrogation of the ventricular septum (B) shows coronary
collateral flow. A turbulent linear diastolic flow signal is seen in the ventricular septum (arrows) anterior to the left ventricle (LV). The
color Doppler and spectral Doppler timing of flow in diastole helps differentiate this septal collateral from a VSD, which is generally
characterized by a high-velocity systolic flow signal.

Figure 15 Apical 4-chamber (A) and subcostal 4-chamber (B) images of an infant with dilated cardiomyopathy and ALCAPA showing
marked left atrial (LA) and left ventricular (LV) chamber dilation with echo-dense fibrotic changes of the mitral valve papillary muscles(arrows), consistent with chronic endocardial ischemia/fibroelastosis. From Frommelt MA, Frommelt PC. Vascular abnormalities. In:
Eidem BW, O’Leary PW, Cetta F, eds. Echocardiography in pediatric and adult congenital heart disease. 2nd ed. Philadelphia, PA:
Wolters Kluwer Health; 2015:470-485.

Table 2 Incidence of echocardiographic markers of ALCAPA from preoperative echocardiogramsMarkerTotal studies reviewed

Positive finding

Sensitivity (%)

Visualization of anomalous CA origin

35

19

54

Flow reversal in LCA

33

30

91

Collateral CA flow

33

28

85

RCA dilation

31

25

81

Abnormal Doppler signal in PA

34

27

79

Mitral valve insufficiency

35

26

74

LV systolic dysfunction (LVEF < 45%)

35

23

66

LV EFE

35

20

57

ALCAPA, Anomalous left coronary artery from the pulmonary artery; CA, coronary artery; EFE, endocardial fibroelastosis; LCA, left coronary artery;
LV, left ventricular; LVEF, left ventricular ejection fraction; PA, pulmonary artery.
Adapted from Patel SG, Frommelt MA, Frommelt PC, Kutty S, Cramer JW. Echocardiographic Diagnosis, Surgical Treatment, and Outcomes ofAnomalous Left Coronary Artery from the Pulmonary Artery. J Am Soc Echocardiogr. 2017; 30(9):896-903.

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Figure 16 Parasternal short-axis imaging in a young adult with ALCAPA s/p Takeuchi-type repair. In A, the widely patent baffling (arrows) of the anomalous left coronary to the aorta (Ao) through the main pulmonary artery (PA) is seen. In B, color Doppler interrogationshows 2 discrete jets (arrows) of baffle leaks from the coronary tunnel into the main PA.
Table 3 Location of coronary origin and termination site forcoronary fistula
Coronary artery fistula termination site
Origin

PA

RV

RA

LV

LA

Other

LCA

16%

41%

26%

5%

7%

LMT

50%

19%

19%

9%

3%

-

LAD

28%

57%

4%

11%

-

-

LCX

-

35%

31%

10%

6%

18%

RCA

13%

49%

26%

8%

1%

3%

5%

LCA, Left coronary artery; LMT, left main trunk; LAD, left anterior descending; LCX, left circumflex; RCA, right coronary artery; PA, pulmonary artery; RV, right ventricle; RA, right atrium; LV, left
ventricle; LA, left atrium.
Revised from Holzer R. et al. Cardiol Young 2004 14; 380-385.

Figure 17 Contrast CT image from a short-axis plane shows ALCAPA with the anomalous left coronary (arrow) arising from theposterior aspect of the pulmonary artery (PA). The separation
between the left coronary and aorta (Ao) is clearly evident. The
left anterior descending (LAD) and circumflex (Cx) branches
can be seen arising from the anomalous left main coronary.

 After repair, CCT and/or CMR may be used when there areconcerns about coronary anatomy and/or persistent myocardial perfusion abnormalities.

C. Isolated Congenital Coronary Artery Fistulas
Prominent intramyocardial color Doppler signals within ventricular septum and LV myocardium due to dilated RCA collaterals
B Significant LV chamber dilation and dysfunction
B Mitral valve papillary muscle fibrosis with significant MR
B

Recommended Imaging Strategy for Anomalous
Left Coronary Artery from the Pulmonary Artery TTE should be the primary screening tool and is usually diagnostic in suspected ALCAPA in infants and children.
 Confirmation of the diagnosis using CCT or CMR should bereserved for cases where the anatomy is unclear; cardiac catheterization is rarely needed for diagnosis.

1. Background. A congenital CA fistula (CAF) is a direct communication between a CA and any cardiac chambers, the coronary sinus, the systemic or pulmonary veins, or the pulmonary arteries,
bypassing the myocardial capillary bed.78,79 Although rare, a CAF
may cause myocardial ischemia, volume overload of the cardiac
chambers, or pulmonary hypertension when it increases pulmonary
blood flow. The majority of isolated CAFs involves only a single fistula, and the involved coronary bed is more commonly the LCA (3963%); less often the RCA (29-55%), and least often both (7-19%).8085
The site of drainage for CAFs is usually a right heart structure; theLV or left atrium are the least common termination sites. CAF
termination sites according to CA origin are summarized inTable 3. In individuals with CHD, pulmonary atresia with intact
septum and hypoplastic left heart syndrome are most commonly
associated with CAF, and these entities are discussed later in thisarticle. CAFs are otherwise rare in CHD, although acquired fistulas

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Frommelt et al 275

Figure 18 Subcostal images of a coronary artery fistula entering the right atrium in a child with a left coronary artery to right atrial
fistula. The markedly dilated left coronary artery (LCA) arising from the aorta (Ao) above the left ventricle (LV) is seen in A. The transducer is tilted more posteriorly in B as the fistula courses posterior to the aorta (arrows) with the terminal segment of the fistula
emptying into the right atrium (RA). With color Doppler (C), the turbulent flow throughout the length of the fistula as it courses toward
the right atrium is well visualized (arrows). From Frommelt MA, Frommelt PC. Vascular abnormalities. In: Eidem BW, O’Leary PW,
Cetta F, eds. Echocardiography in pediatric and adult congenital heart disease. 2nd ed. Philadelphia, PA: Wolters Kluwer Health;2015:470-485.

are seen after surgical RV muscle bundle resection, most commonlyas part of tetralogy of Fallot repair.
Size of the CAF is important, as the physiologic impact is related tothe amount of flow through that abnormal channel. A small CAF,
with low-volume fistulous flow, is usually benign, clinically silent,
and may close spontaneously. A large CAF, however, rarely closes
spontaneously and frequently enlarges with time because of thehigh-volume flow; there is a progressive increase in CA size proximal
to the fistulous site, which sometimes leads to aneurysmal dilation. A
large CAF can cause significant complications, including congestive
heart failure due to the large shunt through the fistulous connection,
myocardial ischemia related to steal of CA blood flow from the CAF,
thrombus formation in the dilated proximal CA segment, ventriculararrhythmias, infectious endocarditis, pulmonary hypertension, syncope, stroke, and even sudden death.86 American College of
Cardiology/American Heart Association guidelines recommend
closure of all large CAFs regardless of symptomatology; smaller
than moderate sized CAFs with symptoms; or CAFs associated with
documented myocardial ischemia, arrhythmia, otherwise unexplained ventricular systolic/diastolic dysfunction, ventricular enlargement, or endarteritis.87 Either transcatheter device closure or surgicalligation to obliterate the shunt without compromising the normal CA
branches is utilized when intervention is needed.

Figure 19 Longitudinal TEE image of the left ventricular outflow
tract and aorta in a child undergoing surgery for supravalvularaortic stenosis. The right coronary artery (RCA) arises immediately below the supravalvular ridge (arrow) from a more superior
position in the aortic sinus (AoS) near the sinotubular junction,with the potential for alternating coronary flow. AAo, Ascending
aorta.

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Figure 21 Aortography in a patient with Williams syndrome. Notethe proximal luminal stenosis of the left coronary artery (arrow) as
it arises from the aorta (Ao) near the sinotubular junction.
complementary tool to help guide device deployment during catheter
intervention.79,91,92
Figure 20 CT 3D reconstruction of the aortic root and ascending
aorta (AAo) in a child with supravalvular aortic stenosis. The origins of both the right (RCA) and left (LCA) coronary arteries are
immediately below the supravalvular narrowing (arrow) withboth coronaries arising from a more superior position in theaortic sinus. The left coronary origin is also covered by the left
coronary cusp, as the sinus appears walled off from the rest of
the aortic root.
2. Goals of Echocardiographic Imaging. a. Pre-intervention
Assessment.–TTE can be useful in identifying a CAF, with enlargement of the proximal CA that supplies the fistula as a constant observation when the shunt flow is significant.88 The proximal CAs canbecome quite dilated and tortuous (Figure 18A), and the recipientchambers can also become dilated.79,89 The parasternal short-axis
view is useful to identify this dilation. If the fistulous site is locateddistally, the dilation and tortuous deformation tends to involve theentire CA (Figure 18B). If the fistula is located more proximally, theproximal CA tends to develop aneurysmal dilation, and the CA distal
to the fistulous site is usually normal in shape.78 Significant CA dilation may progress to aortic sinus deformation, which in turn may
lead to aortic valve regurgitation.90
The origin, pathway, and exit of the CAF are usually visualized using color Doppler mapping (Figure 18C). When the CAF origin is unclear based on 2D imaging, high-velocity flow signals in the CA canidentify the artery from which the fistula originates.89 A continuous
high-velocity signal is observed when the CAF drains into a lowpressure chamber, such as the right atrium or coronary sinus; however, when it empties into a high-pressure chamber (the LV or a hypertensive RV), the shunt flow only occurs during diastole.89 Scanningthe cardiac chambers from all available views with color Doppler
mapping, particularly from the subcostal window, can be useful inevaluating the CAF, since continuous or diastolic high-velocity signals
can be imaged along the length of the CAF to the termination site.91
Although it is rare to observe dilation of cardiac chambers or diastolic
retrograde flow in the descending aorta with a CAF, it may present
when the shunt volume is significant. Although invasive, TEE can
improve sensitivity in detecting the CAF course, and it is certainly ab. Post-interventional Assessment.–The primary goal of postinterventional imaging is to document success in fistula closure; thesuccess rate is quite good for both transcatheter closure and surgicalligation.93-98 If a residual fistula is detected, careful follow-up isrequired as it tends to increase in size over time.82 The same strategies
utilized for pre-intervention imaging should be used in the postintervention assessment. Adverse CA events may occur in patientswith a significantly dilated distal CAF due to thrombus formation,
because of stagnant blood flow in a residually dilated CA after fistulaclosure and resultant occlusion of normal CA branches.99,100 Ectasiaof the CA can persist and is more significant when the fistula terminates in the atrium, and so long-term monitoring for developmentof thrombosis/late ischemia after closure is important.101 Discrete
intimal stenosis may also occur in a surgically modified large CAF after
closure, which can also be a cause of postoperative myocardialischemia.99,100,102-104 Wall motion abnormalities should beassessed, and stress echocardiography may be useful for detecting
ischemia if there is suspicion of CA insufficiency.105
3. Additional Imaging Techniques. Cardiac catheterization has
been the gold standard for complete anatomic and hemodynamic
characterization of a CAF. Although its spatial resolution of the
CAF course is superb, angiography is sometimes limited in outlining
the anatomical relationships with surrounding cardiac structuresand visualizing the drainage sites because of significant dilution of
the contrast when it drains into a low-pressure chamber.106 Cardiac
catheterization is indicated in patients with a suspected pathophysiologic burden identified or suspected on noninvasive imaging, and in
those undergoing planned transcatheter intervention. An ECGgated CCT provides excellent delineation of the precise location
and anatomy of the fistula. A volume-rendered 3D image of CCT
also provides an excellent overview of the spatial relationship of cardiac/vascular chambers to the fistula origin, course and terminationsite when surgical intervention is needed.107 CMR is also useful inevaluating CAF anatomy; it can also quantify the volume of cardiac
chambers and CAF flow by estimating Qp:Qs shunt fraction, assess
for ischemia in myocardial areas distal to the fistula take-off, anddefine the degree of myocardial fibrosis related to fistula steal usinglate gadolinium enhancement.83

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Figure 22 Anatomic coronary artery variations in transposition of the great arteries. The most common coronary artery patterns
seen in TGA are displayed in descending order of frequency. A: Usual pattern with the left coronary artery (LCA) arising fromthe left-facing sinus and the right coronary artery (RCA) arising from the posterior- and rightward-facing sinus. B: Origin of thecircumflex coronary artery (LCx) from the RCA, coursing posterior to the pulmonary trunk. C: Single RCA from the posteriorfacing sinus with the left coronary system coursing posterior to the pulmonary trunk. D: Single LCA from the left-facing sinus
with the right coronary coursing anterior to the aortic root. E: Inverted artery origins with the left coronary arising from theright-facing sinus and the right coronary arising from the left-facing sinus. F: Inverted RCA and LCx. G: Intramural LCA. H:
Intramural RCA. The intramural coronary artery in TGA usually arises from the opposite-facing sinus and passes within thewall of the aorta before exiting the adventitia. From Mertens LL, Vogt MO, Marek J, Cohen MS. Transposition of the greatarteries. In: Lai WW, Mertens LL, Cohen MS, Geva T, eds. Echocardiography in Pediatric and Congenital Heart Disease. Hoboken, NJ: Wiley-Blackwell; 2009.

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Figure 23 Parasternal short-axis imaging in an infant with TGA and the typical coronary artery pattern. The high right parasternal window (A) demonstrates the right coronary artery (RCA) arising from the right-facing sinus of the aorta (Ao), while the high left parasternal
window (B) provides excellent visualization of the left coronary artery (LCA) arising from the left-facing sinus. PA, Pulmonary artery.
 Alternative imaging techniques (CCT, CMR, angiography) can
clearly identify the CAF anatomy/hemodynamics and shouldbe employed when intervention is considered.

Recommended Imaging Strategy for Isolated Coronary Artery Fistulas
 TTE should be the initial screening tool for suspected CAF andis effective in serial monitoring of size/physiologic effects.
 When intervention for a significant CAF is planned, delineationof origin, course and exit should be confirmed with cardiac
catheterization, CCT or CMR.
 Supplementary hemodynamic information may be obtainedby cardiac catheterization or CMR when the need for closureis unclear.
 Coronary ectasia following CAF closure can persist and should
be monitored for development of thrombus/ischemia.
Figure 24 Parasternal short-axis imaging in an infant with TGA
and an intramural left coronary artery. The right coronary artery(RCA) can be seen arising from its usual origin from the rightfacing sinus of the aorta (Ao). The left coronary origin is also
from the right-facing sinus, immediately adjacent to the RCA,
with an intramural course within the posterior aortic wall (arrow).
PA, Pulmonary artery.

IV. CONGENITAL CORONARY ANOMALIES ASSOCIATED
WITH OTHER CONGENITAL HEART DISEASES

A. Supravalvular Aortic Stenosis

Key Points for Isolated Coronary Artery Fistulas
 CAFs are rare and frequently benign, but fistulas can enlarge
over time and lead to myocardial ischemia and/or heart failure.
 Echocardiography may identify CAFs, with common markers
including:
B Dilation of the involved CA with increased flow by color
Doppler
B Turbulent continuous flow throughout the length of theinvolved CA when draining into a low-pressure chamber(usually the right heart)
B Turbulent flow into the chamber at the exit point of the CAF
by color Doppler

1. Background. Supravalvular aortic stenosis (SVAS) is a congenital
localized or diffuse luminal narrowing of the ascending aorta at the sinotubular junction.108-110 SVAS is strongly associated with Williams
syndrome and familial forms of the disease, now recognized as
genetic mutations that affect elastin production. In some cases, all
the major arteries, including the pulmonary, carotid, and CAs, may
be affected.109,111 SVAS may be associated with CA pathologies,
which are mainly stenoses. However, a spectrum of mechanisms
listed below can occur alone or in combination:- CA ostial stenosis occurs in 9% of patients presenting in the first year oflife112,113 secondary to obstruction by a thickened aortic or sinus wall andby a thickened proximal CA wall, especially when the CA orifice is superiorly

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Figure 25 Parasternal short-axis (A) and apical (B) imaging in an infant with TGA and the circumflex coronary arising from the rightsinus. In A, the left circumflex coronary artery (arrows) is arising from the posterior right-facing sinus of the aorta (Ao), as it courses
posterior to the pulmonary artery (PA). The apical view shows a length of the circumflex coronary (arrow) as it courses just anterior tothe mitral valve.
- Diffuse CA dilation, aneurysm formation, and tortuosity can occur when the
CA orifices are below the sinotubular junction and exposed to high prestenotic systolic and pulse pressures.110,122,123

The myocardial perfusion reserve is already limited in patients withsignificant SVAS because of hypertrophied myocardium withincreased oxygen demand and intramyocardial pressure. A critical
perfusion mismatch can occur when coronary blood flow is compromised by stenosis.114,124 Chronic myocardial ischemia associated with
SVAS can result in LV dysfunction, acute myocardial infarction, or
sudden death.124,125 The estimated risk of sudden cardiac death is25-100 times higher in patients with Williams syndrome compared
to an age-matched normal population.126 Up to 58% of deaths are
related to general anesthesia or sedation, and 50-79% are associatedwith evidence of CA stenosis and myocardial ischemia.121,126-128

Figure 26 Contrast CT imaging of the coronary origins from ashort-axis plane in a child with TGA after arterial switch surgery.
The left coronary is severely narrowed (arrows) at it arises fromthe anterior aspect of the neo-aortic (Ao) root behind the pulmonary artery (PA).
displaced near the sinotubular ridge.108,110,114,115 Ostial stenosis affects the
LCA more frequently than the RCA, and it may be progressive in some caseseven after the effective surgical release of SVAS.116- Fusion of the free edge of the aortic valve with the sinotubular ridge, or shelf formation by a thickened aortic leaflet and protruded sinotubular ridge can cover
the CA orifice and compromise CA flow (CA hooding).110,117,118 This is mostfrequently found in the left coronary sinus of Valsalva.108,114,119 Total isolationof the LCA or RCA from the sinus as a result of complete fusion of the entire
leaflet edge to the prominent sinotubular ridge has also been reported.120- Diffuse CA narrowing with intimal and medial hyperplasia and fibrosis is
more frequently observed in patients with the diffuse form of SVAS thanin those with discrete SVAS.110,119 The luminal narrowing is usually
greater in the proximal segment of the affected CA than in the distalsegment.110,121

2. Goals of Echocardiographic Imaging. Deep sedation or general anesthesia involves substantial risk of sudden death in patients with
SVAS, especially in younger patients or those with biventricular outflow
tract obstruction, and so the choice of imaging modality must be considered in light of sedative needs. TTE can assess the severity of SVAS, ventricular wall thickness and motion, and additional anatomical
abnormalities. However, its capability for assessing CA ostial luminal stenosis or CA hooding is limited, and sedation may be needed in uncooperative infants and young children. TTE can identify the CA origins,providing insights into the risk for potential CA complications andmyocardial ischemia. If either CA ostium is located superiorly near the sinotubular ridge, then the risk of ostial luminal stenosis is significantly
increased.108,110,114,115 Flow turbulence by color Doppler at the CA
ostium may indicate stenosis, particularly when assessed at higher
Nyquist limits. Parasternal long-axis views are helpful in visualizing the position of the RCA ostium relative to the sinotubular ridge. Both the apicalfive-chamber and subcostal left anterior oblique views are useful in identifying the LCA ostium and its position relative to the sinotubular ridge.
Aneurysmal dilation or long-segment narrowing of the CAs can beassessed using parasternal and subcostal windows. Dilation of theCAs tends to be associated with discrete hourglass SVAS, and dilation
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Recommended Imaging Strategy for CongenitalCoronary Anomalies Associated with Supravalvular
Aortic Stenosis
 TTE may be an effective screening tool for CA anomalies with
SVAS, particularly in identification of anatomic subtypes associated with ischemia.
 CCT is the preferred imaging modality to document/confirm
coronary ostial abnormalities, since it can be performed
without deep sedation/anesthesia in this high-risk population.

B. Transposition of the Great Arteries

Figure 27 MRI of the coronary origins from a short-axis equivalent window in a child with TGA after arterial switch. Both the left
coronary artery (LCA) and right coronary artery (RCA) are widely
patent as they arise from the anterior aspect of the neo-aortic(Ao) root. The pulmonary artery (PA) is seen directly anterior to
the aorta.

the sinus of Valsalva, remote from the sinotubular ridge.108,119
Epicardial echocardiography or TEE during invasive evaluations orsurgery may also be helpful in visualizing the precise CA ostial location (Figure 19). Similarly, stress echocardiography can assess
myocardial perfusion mismatch but should be used with caution,
particularly preoperatively when there is associated significant
outflow obstruction.
3. Additional Imaging Techniques. ECG-gated CCT provides
excellent delineation of the precise location and anatomy of theCAs (Figure 20),118,129 thereby balancing the procedural risks in thispatient population. The spatial resolution of CMR can be challenging
for precise assessment of CA ostial anatomy, and the data acquisition
time is longer than that of CCT. Moreover, CMR usually requires general anesthesia or deep sedation in young children.130,131 Cardiac
catheterization has been the gold standard, especially in small children(Figure 21). However, the risk of cardiac arrest or sudden death is significant in SVAS patients, especially with severe biventricular outflow
tract obstruction or age less than 3 years.132 Myocardial perfusion imaging can assess CA perfusion without deep sedation. Although relatively low specificity in children is a major limitation, its usefulness inpatients with SVAS has been reported in children as young as
2.5 years.30,123

Key Points for Congenital Coronary Anomalies
Associated with Supravalvular Aortic Stenosis
 SVAS can be associated with potentially life-threatening CA stenosis.
 Echocardiography is limited in diagnosing CA stenosis in SVAS,
but identification of an ostial location near the sinotubular ridge
increases risk of stenosis.
 Alternative imaging techniques (CCT, CMR, angiography) can
help identify CA stenosis, but use of deep sedation during theseprocedures can trigger an ischemic event.

1. Background. Transposition of the great arteries (TGA) is a conotruncal malformation defined as concordance of the atrioventricularconnections and discordance of the ventriculo-arterial connections.
The aorta arises from the RV and the PA arises from the LV, resulting
in parallel systemic and pulmonary circulations and severe cyanosis atbirth.133 Approximately 40% of cases have a ventricular septal defect(VSD), which may affect the type of surgery performed. The primary
surgery for TGA (with intact ventricular septum or with simple typesof VSD) is the arterial switch operation (ASO), which entails transferof the aorta to the pulmonary valve, transfer of the PA to the aortic
valve, and translocation of the CAs to the ‘‘neo’’ aorta.134 In typical
TGA, the aorta is positioned rightward and anterior to the PA.
Thus, the CAs do not arise from the normal location.
There is significant variability in CA anatomy in TGA, and it is important to identify the anatomical pattern prior to the ASO (Figure 22). CA
imaging recommendations have been previously reviewed in theAmerican Society of Echocardiography imaging guidelines document
on TGA published in 2016.133 Particular variations of CA anatomy
have been associated with worse outcomes after the procedure.135
The most common CA pattern occurs in approximately 65% of
TGA cases: the left main CA arises from the left-facing aortic sinus
and divides into the LAD and the LCx, and the RCA arises from theright-facing aortic sinus. The second most common pattern (14% ofcases) is when the LCx arises from the RCA at the right-facing sinus
and the LAD arises separately from the left-facing sinus. In this case,the LCx courses behind the PA as it makes its way toward the left atrioventricular groove. The other 20% of TGA cases have more unusual
patterns that include single right and left CAs, and variations of inverted
CAs whereby the left main or one of its tributaries arises from the rightfacing sinus and the RCA arises from the left-facing sinus. Patients who
have TGA and a VSD are more likely to have an unusual CA patterncompared to patients with TGA and an intact ventricular septum.136
It is important to make a preoperative diagnosis of an intramural
CA pattern (Figure 22G and H), which occurs when one of theCAs is inverted but courses between the aorta and the PA rather
than anterior or posterior to these vessels. The interarterial course
makes it likely that the CA is also intramural. Translocation of an intramural CA is technically more challenging for the surgeon and more
likely to result in kinking or occlusion of the vessel with associatedworse outcome.135,137
The Rastelli operation may be performed for TGA with a large
VSD and LV outflow tract obstruction.138 In these cases, the ASO is
not a feasible strategy because the neo-aortic outflow would be obstructed. The Rastelli procedure entails a baffle from the LV to theaorta through the VSD and the creation of an unobstructed pathway

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Figure 28 Coronary artery variations in tetralogy of Fallot. The most common coronary artery patterns seen in TOF are displayed indescending order of frequency, with the frequency of each variation listed in the lower left corner of the illustration. Ant, Anterior; Conalbr, conal branch; Cx, left circumflex coronary artery; L, left; LAD, left anterior descending coronary artery; LCA, left coronary artery;
Post, posterior; R, right; RCA, right coronary artery. From Need LR, Powell AJ, del Nido P and Geva T. Coronary echocardiography
in tetralogy of Fallot: diagnostic accuracy, resource utilization and surgical implications over 13 years. J Am Coll Cardiol. 2000;
36:1371-7.
from the RV to the PA, usually with a valved conduit. The CAs do nothave to be mobilized for this procedure. Thus, preoperative assessment of CA anatomy may not have a significant impact on outcome.
The Nikaidoh operation is performed for TGA with LV outflow
tract obstruction when there is concern that the baffled pathway
from the LV to the aorta may become obstructed over time.139
During this procedure, the small posterior pulmonary annulus is transected, and the aortic root is mobilized and translocated to the region
where the pulmonary valve had been. This allows the aorta to sit
closer to the LV, which may prevent future LV outflow tract obstruction. The VSD is then closed and a pathway from the RV to the PA iscreated. The CAs require translocation in some but not all cases.
Some have reported that the Nikaidoh procedure cannot be performed if there are major CA anomalies such as single CA or
abnormal course of the LAD.140 Thus, identifying these variants
may help with surgical planning.
2. Goals of Echocardiographic Imaging. a. Preoperative
Assessment.–The goal of CA imaging in the preoperative patientwith TGA is to identify the origin and course of both CAs. This is typically achieved by TTE. Identification of the type of TGA is the first stepin localizing the CA. The great artery relationship and the position of

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Figure 29 Parasternal imaging and intraoperative photography from an infant with TOF and the left anterior descending coronary
artery (LAD) arising from the right coronary artery (RCA) and crossing the right ventricular outflow tract. From the parasternal longaxis view (A), the LCA can be seen in cross section anterior to the right ventricular infundibulum (Inf), raising concern for an anomalous coronary course. From the parasternal short-axis view (B), the LAD is clearly seen arising from the RCA and crossing anterior
to the pulmonary artery (PA). The intraoperative photo of the epicardial surface of the heart (C) confirms the anatomy, again
showing the LAD coursing across the right ventricular outflow tract below the main pulmonary artery (MPA). AoV, Aortic valve;
AO, aorta.
the heart in the chest should be noted. Once the spatial orientation
has been ascertained, there are five other points to keep in mind while
scanning for the coronaries: a) the semilunar valve cusp alignment, b)
the location of the CAs relative to the commissures, c) the verticallocation within the sinuses, d) the distance to the translocation siteon the PA, and e) the course of each CA.
Varying echocardiographic windows can aid in the localization of
the CAs. The parasternal short-axis view, employing either the rightor left parasternal window to get perpendicular to a particular CA,
is usually quite helpful because the semilunar valves appear en face.
The high right parasternal window may permit visualization of the
CA arising from the right-facing sinus (Figure 23A), whereas thehigh left parasternal window may yield a better view of the CA arisingfrom the left-facing sinus (Figure 23B). The parasternal short-axis viewis also important in identifying the cusp alignment and to help determine the distance the CA must be moved during translocation.

The parasternal short-axis view is also the best imaging plane todetect an intramural course between the two great arteries(Figure 24). Color Doppler can confirm the flow between the two
great arteries in patients suspected of having an intramural CA course,
as described in the section on AAOCA. This view can also be used toshow the LCA division into the LAD and LCx in patients with usual
CA anatomy. When the LCx arises from the right-facing sinus, it canoften be seen coursing behind the PA (Figure 25A). When the RCA
arises from the left-facing sinus, it can often be seen crossing anterior
to the aorta. Intraoperative TEE can provide analogous views that may
complement TTE imaging in identifying each CA origin and course.
Other echocardiographic planes can be used to detect unusual CA
patterns and courses. In the apical and subcostal views, the LCx fromthe right-facing sinus can often be seen coursing posterior to the PA
and anterior to the mitral valve in the frontal or left anterior oblique
sweep (Figure 25B). In patients with an inverted RCA (arising from

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Figure 30 Contrast CT imaging of the left coronary in a patient with TOF repair s/p right-ventricular-to-pulmonary artery (RV-PA)
conduit placement. From a short-axis plane (A), the left coronary (triangle) is seen in cross section coursing between the aorta(Ao) and the narrowed conduit, with the potential for compression during interventional dilatation of the conduit as part of percutaneous replacement. This relationship is well illustrated from a long-axis plane (B), where the potential for compression of the left coronary (arrows) between the aorta and the conduit can again be appreciated. LV, Left ventricle.

the left-facing sinus), further anterior sweeping in the subcostal view
will demonstrate the RCA coursing in front of the aorta.
b. Postoperative Assessment.–It is essential during the ASO (orthe Nikaidoh operation if the CAs are translocated) that the CAs
are reimplanted into the neo-aorta without narrowing of the orifices
or kinking of the vessels. Often, intraoperative TEE is performed toassess ventricular performance and assure that there are no regional
wall motion abnormalities suggestive of myocardial ischemia.133
Velocity and direction of flow in the CA, as assessed by TEE, may
be predictive of outcome.141,142 A velocity-time integral greaterthan 0.14 and CA peak systolic velocity of greater than 0.60.8 cm/sec are measures that have been associated with need forrevision of the CA anastomosis during the ASO,141 and systolic
reversal of flow in the LCA has also been associated with adverse
outcomes.142 Following surgical CA reimplantation, there should
be suspicion of myocardial ischemia if ventricular dysfunction
and/or ventricular arrhythmias are present, as CA events may still
occur.143 Serial TTE can screen for regional wall motion abnormalities, but CA narrowing or atresia can be challenging to detect.
Myocardial deformation imaging may add information about
regional ventricular function that could prompt further CA
investigation.
3. Additional Imaging Techniques. There are some circumstances where clarification of the anatomy may be helpful during
the preoperative evaluation. In patients where there is suspicion of
an intramural CA, CCT or CMR imaging may confirm this finding.
Although cardiac catheterization is primarily used for the performance of a balloon atrial septostomy to improve mixing prior to surgery, an angiogram of the aortic root can frequently identify the origin
and course of each CA.
After the ASO, CCT, CMR, or angiography can be used to visualize
the origin and course of the translocated CAs to determine if CA revision is required. CCT angiography is excellent for evaluation of CA
patency after the arterial switch operation144 and for definition of themechanism of obstruction (Figure 26).145,146 CMR can define theorigin and course of the CAs (Figure 27), and it can also determine

myocardial viability. Using late gadolinium enhancement, CMR canidentify areas of fibrosis, which may have resulted from ischemia.147
Some practitioners routinely assess CA perfusion after ASO. This canbe performed using stress echocardiography to detect regional wallmotion abnormalities or by perfusion imaging such as SPECT or PET.

Key Points for Congenital Coronary Anomalies
Associated with Transposition of the Great Arteries
 Variations in CA origin and course are common in TGA and
can impact surgical planning and risk of postoperative CA complications.
 Echocardiography may identify concerning CA patterns in
TGA preoperatively, particularly an intramural course between
the great arteries and CAs coursing anterior to the aorta or posterior to the pulmonary artery.
 Ventricular dysfunction and regional wall motion abnormalities
after the arterial switch operation should prompt alternative imaging techniques (CCT, CMR, angiography) to assess CA
patency.

Recommended Imaging Strategy for CongenitalCoronary Anomalies Associated with Transpositionof the Great Arteries
 TTE should be the primary imaging tool for delineation of coronary artery anatomy in the preoperative assessment of TGA.
 When an intramural coronary is suspected, clear delineation ofthis higher-risk variant using CCT may be warranted prior toarterial switch surgery.
 Postoperative CCT, CMR, and/or cardiac catheterization
should be performed when there is suspicion of coronary
compromise.

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Figure 31 Right coronary angiography during planned transcatheter pulmonary valve replacement in a patient with recurrent rightventricular outflow tract obstruction after TOF repair. Prior to test-balloon inflation (A) in the calcified right ventricular-topulmonary-artery conduit (large arrow), the right coronary (small arrows) can be seen arising without obstruction immediately adjacentto the conduit. With balloon inflation (BA) that expands the conduit (B), the right coronary artery is significantly narrowed (arrow),demonstrating the risk of coronary compression in placing a new pulmonary valve using interventional techniques.

C. Tetralogy of Fallot
1. Background. Tetralogy of Fallot (TOF) is the most common formof cyanotic CHD, with an estimated incidence of 32.6 per 100,000
live births. Approximately 5-14% of patients with TOF will have
CA anomalies.148-150 The most common CA anomalies in TOF(Figure 28) include the LAD originating from the RCA or right sinus,
dual LADs (with one crossing the RVOT), single CA, and the LCx
originating from the RCA. A large right conal CA branch is relatively
common, and a dominant LCA is present in 25% of TOF patients.148 Additionally, the orifice of the LCA is commonly locatedmore posteriorly and the RCA is located more anteriorly and leftward
than in the normal heart, related to the clockwise rotation of the aortic
root in TOF.148 Rare CA anomalies in TOF include CAF, CA atresia,
and origination of the LCA from the PA or from an aortopulmonary
collateral.151,152
Surgical intervention is always required for this lesion to close the
VSD and relieve RV outflow/pulmonary stenosis. Techniques torelieve the outflow obstruction can vary, based on anatomic findings,
but frequently involve pulmonary valvotomy, patch enlargement of
the RV outflow tract and pulmonary valve, or placement of a conduit
from the RV to the pulmonary artery. CA variants in which a major
branch crosses the RV outflow tract are important to recognize prior
to surgical intervention. In a retrospective single center study of 607
patients with TOF, 13% had a CA anomaly identified, and of those,
62% had a CA that crossed the RV outflow tract.153 It is critical todefine both CA origin and course prior to surgical intervention, sincean anomaly can determine the approach to relieving RVoutflow tract
obstruction, particularly if a transannular patch is considered. A CA
coursing anterior to the RV outflow tract may require a palliative procedure prior to definitive repair or an alternate surgical approach torelieve RV outflow tract obstruction such as a modified patch or
RV-to-PA conduit.154
Pulmonary valve replacement (either surgical or catheterdeployed) is commonly performed in adolescents and adults afterTOF repair because of residual pulmonary stenosis or regurgitation.
The 2018 ACC/AHA Guidelines for Managements of Adults with

Figure 32 Diagram of the variability in coronary artery origins intruncus arteriosus. The center circle represents the circumference of the truncal wall, divided into 4 quadrants, with the number and type of coronary arising from each quadrant. Post,
Posterior; Rt, right; Ant, anterior. From de la Cruz MV, Cayre
R, Angelini P, Noriega-Ramos N and Sadowinski S. Coronary arteries in truncus arteriosus. Am J Cardiol. 1990; 66:1482-6.
Congenital Heart Disease recommend that CA anatomy should
be delineated in any patient with repaired TOF prior to intervention
on the RV outflow tract.155 CAs that are directly substernal or
directly anterior to the RV outflow tract require careful planningfor sternal entry to avoid CA injury with re-intervention.
Advanced imaging prior to transcatheter pulmonary valve placement should place specific emphasis on definition of the CA anatomy and predict those at high risk for CA compromise with

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VSD and may be difficult to diagnose until after VSD closure
when PA pressures have fallen.
b. Postoperative Assessment.–Echocardiography is inadequate toprovide delineation of CA anatomy when additional procedures
are considered after initial complete repair, so additional imaging
techniques must be utilized. Circumstances when these techniques
are needed after repair of TOF include:

Figure 33 Contrast CT imaging from a short-axis plane in an infant with truncus arteriosus. The aortic valve is quadricuspid,with each of the leaflets numbered. The right coronary artery(RCA) can be seen arising from the anterior and rightward
cusp, while the left coronary artery arises from the posterior
and leftward cusp.

transcatheter valve placement and pre-stenting, which is commonlyused.
2. Goals of Echocardiographic Imaging. a. Preoperative
Assessment.–In a retrospective review of 598 children with TOF,
echocardiography for CA delineation exhibited a sensitivity of 82%
and a specificity of 99% with an accuracy of 98.5%.149 In this series,
5.4% of patients were found to have a major CA crossing the RV
outflow tract at the time of surgery (Figure 29C), a majority of which
did not necessitate the placement of an RV-to-PA conduit in patientswith TOF and pulmonary stenosis. CA imaging using TTE involves
primarily the parasternal short- and long-axis views (Figure 29A and
B). In the parasternal short-axis view, the left main CA is visualized
as it originates from the left sinus of Valsalva. Rotating the transducer
with a slight apical tilt will profile the LAD coursing in the anterior
interventricular groove and often identify the origin of the LCx. In order to image the RCA, it is often found slightly higher than the LCA in
the parasternal short-axis view. A sweep in the parasternal long-axis
view, tilting toward the left shoulder, is particularly helpful to identify
an anomalous origin of the LAD from the RCA (Figure 29A). In theparasternal long-axis view, the LCA and its branches are visualized between the aortic root and the main PA. It is important to examine theinfundibular free wall to identify any vessels coursing on the epicardial
surface. Occasionally, the apical and subcostal windows can provide
additional information.
Other than very rare anomalies, the CAs arise from the aorta
in a majority of TOF cases. Color Doppler settings that accentuate low-velocity CA flow can provide visualization of anterograde color flow into the CA ostia. Rarely, if a CA arises fromthe PA or an aortopulmonary collateral, flow may be biphasicor retrograde into the CA ostia depending on pulmonary pressures. A CA arising from a collateral or the PA will perfuse
with systemic pressure in the presence of a large and unrestrictive- Evaluation of the CA relationship to the sternum and the RVoutflow tract inpatients who will undergo additional cardiac surgical intervention.
- Evaluation of the CA relationship to the RVoutflow tract in patients who will
undergo either transcatheter or surgical intervention on the RVoutflow tract.
The relationship is particularly useful to identify CA anomalies and to predict
those at higher potential risk for CA compression with placement of thetranscatheter valve, often placed with pre-stenting of the RV outflow tract.
- Evaluation for CA lesions in patients with TOF who will undergo
another cardiac procedure who may need CA intervention, typicallymen over the age of 35 years, premenopausal women 35 years orolder with risk factors for atherosclerosis, postmenopausal women,
and any adult patient with symptoms of potential myocardialischemia.87

3. Additional Imaging Techniques. CMR is the most common
advanced imaging technique for post-operative assessment of
TOF.156 CMR is excellent at defining the relationship of the proximal
CA to the outflow tracts. However, artifact from metallic stents anddevices degrades CMR image quality, which can limit CA imaging,and there is a relatively high incidence of stent branch PA intervention
after TOF repair. In addition, older patients are more likely to have adefibrillator or pacemaker, some of which may cause artifact or arecontraindicated for MRI.21 Finally, pulmonary valve replacements(either surgical or percutaneous) often produce localized signal artifact with CMR.
CCT provides high-resolution CA imaging in patients of all ages
with TOF. A pre-operative CCT study in 100 patients less than one
year of age with TOF demonstrated 100% sensitivity and specificity
compared to surgical findings for CA course at a dose of less than 1
millisievert.21 Patients were spontaneously breathing and without
sedation for this study. CCT can show the course of the CA relative
to the RV outflow tract after surgical repair and help assess for potential CA compression prior to interventional pulmonary valve replacement (Figure 30). ECG-gated CCT can also be used to quantify
ventricular volumes and estimate EF with an accuracy comparable
to MRI as long as a scanner with appropriate temporal resolution isused and contrast is modified to opacify both the right and left sidesof the heart.
In the current era, non-invasive imaging of the CAs has largely
replaced invasive angiography for the preoperative evaluation ofpatients with TOF. However, angiography of the CAs in patientswith repaired TOF is commonly used during transcatheter pulmonary valve placement for relief of recurrent RV outflow tract
obstruction in previously placed conduits of appropriate size
(Figure 31A and B). In one series of patients undergoing transcatheter pulmonary valve placement, 6 of 124 patients had CA
compression.157 The largest series of transcatheter valves included
343 patients and demonstrated a 5% incidence of CA compression with test balloon angioplasty (Figure 31B). Of the 34 patientswith TOF and anomalous CA course included in this series, 21%
had CA compression.158

286 Frommelt et al

Journal of the American Society of Echocardiography
March 2020

Figure 34 Low-scale color Doppler mapping of the right ventricle from an apical view in an infant with PA-IVS and systolic RV hypertension showing a small jet of systolic flow from the RV apex into the myocardium, representing a small RV-to-coronary connection(A), and a dilated coronary segment along the anterior interventricular groove with anterior tilting of the imaging plane, representing
flow from the RV into that coronary segment (B).

 Alternative imaging techniques (CCT, CMR, angiography) assessing coronary artery relationships to the RVOT are necessaryprior to pulmonary valve replacement.

Recommended Imaging Strategy for CongenitalCoronary Anomalies Associated with Tetralogy of
Fallot TTE should be the primary screening tool for delineation of coronary anatomy in the preoperative assessment of TOF.
 After primary repair, delineation of coronary anatomy by CCT
or CMR should be performed prior to pulmonary valve
replacement.
 In patients undergoing transcatheter pulmonary valve replacement, coronary angiography may be sufficient to assess coronary anatomy during the procedure.

Figure 35 Right ventricular angiogram showing multiple rightventricular-to-coronary connections in an infant with PA-IVS.

Key Points for Congenital Coronary Anomalies
Associated with Tetralogy of Fallot Variations in CA origin and course are common in TOF and can
impact initial surgical planning and risk of CA complications
during later interventions.
 Echocardiography identifies CA branches from the RCA
crossing the RVOT prior to initial surgical repair.

D. Truncus Arteriosus
1. Background. Truncus arteriosus is an uncommon type of
CHD, in which a single arterial trunk arises from the heart, giving
rise to the CAs, pulmonary arteries, and systemic arteries, in thatorder. Infants with truncus arteriosus undergo surgical repair thatbaffles the LV to the truncal root, creating a ‘‘neo-aortic’’ valve
and outflow, with placement of an RV-to-PA conduit that must
be serially replaced over time. The single semilunar truncal valve
may have two, three, four, or more leaflets and usually overrides
the ventricular septum through an outlet VSD. The proximal CA
anatomy is most likely to be normal when there is a trileaflet truncal valve. However, CA anomalies are reported in up to 20% ofJournal of the American Society of Echocardiography
Volume 33 Number 3

patients with truncus arteriosus. In a review of 39 autopsy specimens of subjects with truncus arteriosus, there was great variability
in CA anatomy, with a tendency for the RCA to arise from theanterior right quadrant and for the LCA to arise from the anterior
left quadrant (Figure 32).159 Such a tendency was observed in 50%
of bicuspid, 59% of tricuspid, and 66% of quadricuspid valves. In
18% of cases, a single CA ostium is present, usually arising froma posterior sinus.159,160 Recognized variants of CA anatomy inthis condition include an LCx arising from the RCA (3.6%), and
an LAD arising from the RCA (2.4%).160 Patients with truncus arteriosus have marked variation in the location and shape of the CA
ostia, which are commonly located above the sinotubular junction.
The distal courses of the CAs are usually normal. The location
around the truncal root is unpredictable, and is a risk factor forpoor outcomes during surgical repair when the LCA arises near
the pulmonary artery origin or when the RCA courses anterior
to the truncal root.161 In a study of 30 autopsy specimens, 8 had
an RV-to-PA conduit, all of which had injury to the anteriorly
crossing RCA.162 Additionally, CA ostial stenosis due to a slit-likeorifice or a proximal intramural course may lead to ischemia, particularly with low aortic diastolic pressure (which can be seen with significant truncal valve regurgitation).163 There are rare case reports
of a CA arising from a PA.
Anomalous CA origins, where one or both CAs did not arise fromthe expected sinus, have been associated with a higher mortality
following truncus arteriosus repair. In a series of 106 subjects withtruncus arteriosus who underwent surgical repair between 1976
and 1998, 13 had an anomalous CA, and 7 deaths were related tocompression or distortion of that CA.164 In the largest series of truncus arteriosus outcomes, abnormal CA anatomy is shown as a risk factor for early mortality.165
2. Goals of Echocardiographic Imaging. TTE is an excellent
modality to delineate the CA origin(s) in infants with truncus arteriosus. Definition of the sinus from which the CA originates, the height of
the CA origin from the annulus or from the ascending aorta, the proximity of the CA to the pulmonary arteries, and the proximal CA
course should be defined. Rare reports of anomalous CA from thePA, CA ostial stenosis, or intramural proximal CA course have been
described.
TTE views for CA imaging in truncus arteriosus involve primarily
the parasternal short- and long-axis views. In the parasternal shortaxis view, the CAs may be visualized as they leave the truncal root,
and the relationship of the CA origin to the truncal cusp can be
defined. The parasternal long-axis and subcostal views can help define
the location of the CA origin within the truncal valve sinus or the
ascending aorta. Particular attention should be paid to the relationshipof the CA to the PA, often requiring tilting the transducer superiorly
and inferiorly to image these structures in the same plane. Color
Doppler can provide visualization of anterograde color flow into
the CA ostia. In exceedingly rare cases, if a CA arises from the PA,
flow may be biphasic or retrograde into the CA ostia, depending on
pulmonary vascular resistance.
3. Additional Imaging Techniques. In select cases, CCT is helpful in the preoperative assessment of anatomy (including CA origin
and course) in infants with truncus arteriosus (Figure 33).166 Similar
to the imaging evaluation of patients with repaired TOF, knowledgeof the CA relationships to the outflow tracts and sternum are important in patients with truncus arteriosus who may require transcatheter
pulmonary valve replacement.

Frommelt et al 287

Figure 36 Low-scale color Doppler mapping of the left ventricle
from an apical four-chamber view in an infant with the HLHS
variant involving mitral stenosis and aortic atresia, showing
flow from the hypoplastic left ventricle (LV) chamber into the
myocardium (open arrow), representing a tiny left ventricular tocoronary connection. RA, Right atrium.

Key Points for Congenital Coronary Anomalies
Associated with Truncus Arteriosus
 Variations in CA origin and course are common in truncus arteriosus, including single CA, and can impact risk of CA complications during surgical interventions.
 Echocardiography may identify CA origins and the location/
proximity of the CA ostia to the pulmonary arteries prior toinitial surgical repair.
 Alternative imaging techniques (CCT, CMR, angiography) arenecessary to assess CA course prior to subsequent pulmonary
valve replacements.

Recommended Imaging Strategy for CongenitalCoronary Anomalies Associated with Truncus Arteriosus
 TTE should be the primary screening tool for delineation of coronary anatomy in the preoperative assessment of truncus arteriosus.
 After primary repair, delineation of coronary anatomy by CCT
or CMR should be performed prior to pulmonary valve
replacement.
 In patients undergoing transcatheter pulmonary valve replacement, coronary angiography may be sufficient to assess coronary anatomy during the procedure.

288 Frommelt et al

E. Coronary Anomalies Associated with Single Ventricle
Lesions
Significant congenital CA anomalies in the setting of functionally univentricular hearts are seen primarily in two settings: pulmonary atresia
with intact ventricular septum (PA-IVS) and hypoplastic left heart syndrome (HLHS).
1. Pulmonary Atresia With Intact Ventricular Septum. a.
Background.–PA-IVS is a congenital lesion in which there is no bloodflow between the RV and the pulmonary arteries in the absence of a
VSD; there are varying degrees of tricuspid valve and RV hypoplasia.
Abnormal connections between the RV and the CAs are a common
feature related to high pressure in the RV cavity. The nomenclature
for these RV-CA connections has been variable and has included
coronary-cameral fistulae, myocardial sinusoids, and ventriculocoronary communications. Intrinsic abnormalities of the CAs havebeen seen in almost half of the cases of PA-IVS,167,168 with mostshowing myointimal hyperplasia resulting in abnormal vascular architecture and stenosis or complete atresia of the arterial lumen.169
Normal CA flow for the RV occurs during both systole and diastole, but the increased transmural pressure gradient of the hypertensive RV in PA-IVS results in only diastolic flow in both theright and LCA systems. In the presence of RV hypertension and
CA stenosis or atresia, aortic diastolic pressure becomes insufficientfor perfusion of the affected myocardium. Thus, the hypertensive
RV becomes the primary source of CA blood flow for the myocardial segments distal to the area of CA obstruction or interruption.170 RV-dependent CA circulation is seen in 5-34% ofcases.171-174 Recognition of this pathophysiologic state isimportant prior to establishing continuity between the RV andPA (also known as RV decompression) by surgical ortranscatheter intervention, as this will reduce RV systolicpressure, compromising CA flow to the RV-dependent myocardial
segments and resulting in ischemia and ultimately infarction.175 The
primary risk factor for RV-dependent CA circulation is tricuspid
valve annular size, with risk increasing at annular Z-score thresholdsof 2.5,171,176 below which the risk increases significantly. This isalso the approximate tricuspid valve Z-score below which the likelihood of a biventricular circulation is low.177
b. Goals of Echocardiographic Imaging.–The sensitivity of echocardiography for CA stenosis or atresia is low, and a normal-sized
tricuspid valve (and RV) does not necessarily preclude the presenceof RV-dependent CA circulation.178 Dilation in the proximal CA
may indicate fistulous connections to the RV. Recognition of tricuspid
valve hypoplasia using Z-scores for tricuspid annular diameter fromthe apical four-chamber and parasternal long-axis views should raiseawareness of potential sinusoidal connections.
Color Doppler using low Nyquist limits can identify flow between
the RV and the CAs (Figure 34); bidirectional flow in the proximalCAs (anterograde in diastole, retrograde in systole) is an indirect indicator of RV-CA connections. RV-CA connections can appear as areasof flow along the RVendocardial surface in subcostal, apical, and parasternal views. Occasionally, turbulent color flow at higher Nyquist
limits can identify areas of CA stenosis. Lack of identifiable flow
into a proximal CA suggests ostial atresia or stenosis. Finally, dilationof other CA segments besides the left main, proximal LAD, and proximal RCA suggests abnormal connections to the RV.
c. Additional Imaging Techniques.–Other imaging modalities arealmost always necessary to define CA anatomy in PA-IVS. TEE has

Journal of the American Society of Echocardiography
March 2020

provided little if any additional information as it relates to intrinsic
CA abnormalities. CCT is useful in assessing CA anatomy but canbe limited in very small patients with fast heart rates. CMR is usefulin the evaluation of the myocardial perfusion defects, since intrinsic
CA abnormalities in PA-IVS are not necessarily limited to the RV,
with some studies showing areas of ischemia, fibrosis, and infarctionin the LV as well.179-181 Additionally, particularly if there is evidenceof worsening myocardial dysfunction, CMR is a useful surveillance
tool to look for evidence of myocardial ischemia during long-term
follow-up.182
The primary advantage of catheterization with angiography is theability to evaluate the CA anatomy and perform transcatheter intervention (if amenable) in a single examination. The ability to evaluate
the CA flow via RV injection followed by aortic angiography and/or
selective CA injection provides the most comprehensive evaluationof the CA anatomy. It is important for the operator to recognize interruptions in the CA distribution, as this primarily affects the initial palliation. This can be accomplished by using similar views for both the RV
angiogram and aortic or selective CA angiogram to evaluate for absent segments, stenosis, and/or communications (Figure 35). If abnormalities are noted, angled angiography can be used as an adjunct tobetter delineate the specific area of concern. In addition to angiographic data, hemodynamic data can also be obtained that might
be useful in determining the most appropriate surgical or transcatheter intervention.
The angiographic definition of RV-dependent CA circulation ismade when atresia or severe stenosis of one or more ostial or CA interruptions is identified.170,177 RV-CA connections can exist without
stenosis and should be delineated angiographically as these patients
can still successfully undergo decompression.170

Key Points for Congenital Coronary Anomalies
Associated with Pulmonary Atresia with Intact Ventricular Septum
 Delineation of RV-dependent CA circulation is critical prior to
RV decompression.
 Identification of abnormal RV-CA connections can be madewith echocardiography, but confirmation of the anatomy isrequired prior to intervention and best accomplished by angiography.
 Tricuspid valve Z-score < 2.5 is a risk factor for poor prognosis with biventricular repair and for the presence of abnormal
RV-CA connections.
 CMR is a useful tool to evaluate for myocardial abnormalities
related to abnormal coronary perfusion both in the short andlong term.

Recommended Imaging Strategy for CongenitalCoronary Anomalies Associated with Pulmonary
Atresia with Intact Ventricular Septum
 TTE may identify anatomic/physiologic features concerning forcoronary anomalies, but it is usually inadequate as the sole tool
to assess coronary anatomy in PA/IVS.
 Coronary angiography should be performed prior to any interventions that would result in decompression of the RV.

Frommelt et al 289

Journal of the American Society of Echocardiography
Volume 33 Number 3

2. Hypoplastic Left Heart Syndrome. a. Background.–Hypoplastic left heart syndrome (HLHS), defined as significant underdevelopment of the LVand its associated structures, has several subtypes that
include atresia or hypoplasia of the aortic and mitral valves. Connections
between the LV and CA vascular bed (LV-CA connections) have been
seen in patients with HLHS by angiography and during autopsy183-186
These anomalies have been observed almost exclusively in the settingof mitral stenosis (MS) and aortic atresia (AA), suggesting that theabsence of flow into the hypoplastic LV with mitral atresia precludes
the formation of LV-CA connections. Pathologic review of postmortem
specimens evaluating for the presence of CA abnormalities and evidenceof abnormal RV histology suggestive of ischemia have not revealed any
differences between those patients with and without CA abnormalities,
including the subtype where they are usually found (hypoplastic left heart
syndrome with mitral stenosis and aortic atresia),187 and outcome data forthat subtype have not shown a consistent pattern of risk.188-191
b. Goals of Echocardiographic Imaging.–Imaging of patients with
HLHS, particularly in terms of LV-CA connections in the setting of
mitral stenosis and aortic atresia, is similar to the imaging protocol
for PA-IVS with CA abnormalities. Low Nyquist limits with color
mapping over the LV will often identify abnormal flow in the LV
myocardium (Figure 36), which is highly suspicious for LV-CA connections. CA imaging can be challenging, particularly in the settingof the severe ascending aortic hypoplasia that is usually characteristicof this lesion. Following the Norwood procedure (where the native
aorta is anastomosed to the ascending neo-aorta), imaging andDoppler interrogation of that connection is important in documenting patency, as it is the sole source of CA blood flow.
c. Additional Imaging Techniques.–TEE before and after stage I palliation can be useful in assessing RV function. CCTand CMR usually have
limited use in these patients in the preoperative period in part because the
LV is never decompressed with the Norwood palliation. Catheterization
with angiography may be helpful if there is concern for abnormal LV-CA
connections preoperatively, with evaluation primarily involving LV angiography to identify the abnormal communication. Direct injection of
the CAs in patients with aortic atresia is typically avoided due to therisk for CA compromise during catheter engagement of the hypoplastic
ascending aorta; this results from complete or near complete occlusion by
the catheter, which then prevents retrograde CA filling. Confirmation of
CA compromise can help guide clinical management in terms of standard
palliation versus hybrid palliation and/or transplantation. In addition, angiography is sometimes useful in the immediate postoperative period forevaluating etiologies of ischemia, including stenosis or distortion of theanastomosis between the ascending aorta and the PA or thrombosis of
the hypoplastic native aortic root.

Key Points for Congenital Coronary Anomalies
Associated with Hypoplastic Left Heart Syndrome CA abnormalities are more common in the HLHS subtype of
MS/AA.
 Echocardiography is typically adequate for the assessment ofabnormal CA communications in patients with HLHS.
 Patients with abnormal CA communications are palliated in asimilar manner to other subtypes of HLHS with outcomesthat are largely similar.
 Direct imaging with angiography is typically reserved for postoperative patients in whom there is evidence of ischemia.

Recommended Imaging Strategy for CongenitalCoronary Anomalies Associated with Hypoplastic
Left Heart Syndrome
 TTE should be the primary screening tool for delineation of coronary anatomy in the preoperative assessment of HLHS.
 Coronary angiography may be performed in the rare circumstance where abnormal LV-CA connections are suspected.

NOTICE AND DISCLAIMER: This report is made available by ASE
as a courtesy reference source for members. This report contains recommendations only and should not be used as the sole basis to makemedical practice decisions or for disciplinary action against any
employee. The statements and recommendations contained in thisreport are primarily based on the opinions of experts, rather thanon scientifically-verified data. ASE makes no express or implied warranties regarding the completeness or accuracy of the information inthis report, including the warranty of merchantability or fitness for aparticular purpose. In no event shall ASE be liable to you, your patients, or any other third parties for any decision made or action takenby you or such other parties in reliance on this information. Nor does
your use of this information constitute the offering of medical advice
by ASE or create any physician-patient relationship between ASE andyour patients or anyone else.
Reviewers: This document was reviewed by members of the ASE
Guidelines and Standards Committee, ASE Board of Directors, ASE
Executive Committee, and designated representatives from theJapanese Society of Echocardiography, and Society for
Cardiovascular Angiography and Interventions. Reviewers included
Azin Alizadehasl, MD, FASE, John P. Breinholt III, MD, Michael
Cheezum, MD, FSCCT, Scott D. Choyce, RDCS, RVT, RDMS,
FASE, Gregory Ensing, MD, FASE, Craig Fleishman, MD, FASE,
Benjamin Freed, MD, FASE, Mark K. Friedberg, MD, FASE, Edward
Gill, MD, FASE, Suzanne Golz, MS, RDCS, FASE, Renuka Jain,
MD, FASE, Pei-Ni Jone, MD, FASE, James N. Kirkpatrick, MD,
FASE, Wyman W. Lai, MD, MPH, FASE, Stephen H. Little, MD,
FASE, Anuj Mediratta, MD, FASE, Andrew Pellett, PhD, RDCS,
FASE, Dermot Phelan, MD, PhD, FASE, David H. Wiener, MD,
FASE, and Satoshi Yasukochi, MD.

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