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CASE REPORT

CLINICAL CASE

Echocardiographic Diagnosis ofBland-White-Garland Syndromein an Asymptomatic Adult

Shruti Hegde, MD,a Jennifer Bell, MD,a Benoy Zachariah, MD,b Emani Sitaram, MD,c Michael Maysky, MDa

ABSTRACT

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Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital defect and usually

diagnosed within the first 2 months of life. Only 10% of patients survive to adulthood largely in part to the formation of

extensive collaterals from the right to left coronary arteries. We present a case of ALCAPA diagnosed in an asymptomatic

adult through a transthoracic echocardiogram (TTE). (Level of Difficulty: Beginner.) (J Am Coll Cardiol Case Rep

2020;2:1021–4) © 2020 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation.

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

HISTORY OF PRESENT ILLNESS

A 37-year healthy man presented to establish carewith a primary care physician. He had no specificcomplaints. His vitals were within normal limits witha heart rate of 74 beats/min, blood pressure of 124/

EARNING OBJECTIVES

To highlight the importance of understand-ing the vascular anatomy of ALCAPA, clinicalpresentation in adults, and diagnosis.To identify the echocardiographic featurestypical of ALCAPA and use in combinationwith clinical data to significantly reducemisdiagnosis or nondiagnosis, therebyimproving survival.To understand the post-operative manage-ment of patients with ALCAPA.

N 2666-0849

m the aDepartment of Medicine, Division of Cardiology, St. Elizabeth’s Me

Medicine, Division of Cardiology, Good Samaritan Hospital, Brockton, Ma

rgery, Boston Children’s Hospital, Boston, Massachusetts. The authors hav

contents of this paper to disclose.

e authors attest they are in compliance with human studies committees

tutions and Food and Drug Administration guidelines, including patient co

JACC: Case Reports author instructions page.

nuscript received February 18, 2020; accepted March 19, 2020.

60 mm Hg, respiratory rate of 16 breaths/min, andoxygen saturation of 98% on room air. His physicalexamination was notable for a grade 3 of 6 hol-osystolic murmur heard best at the apex that radiatedto his back.

The patient had a history of measles when he was 2years of age. Otherwise, his past medical history wasunremarkable.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis for an apical holosystolicmurmur is mitral regurgitation owing to mitral valvedysfunction.

INVESTIGATIONS

Electrocardiography was notable for a left anteriorfascicular block. His laboratory work-up was within

https://doi.org/10.1016/j.jaccas.2020.03.036

dical Center, Brighton, Massachusetts; bDepartment

ssachusetts; and the cDepartment of Cardiothoracic

e reported that they have no relationships relevant to

and animal welfare regulations of the authors’ in-

nsent where appropriate. For more information, visit

FIGURE 1 Dilated

Angiogram

Coronary angiogram

nary artery with ex

ABBR EV I A T I ON S

AND ACRONYMS

ALCAPA = anomalous origin of

the left coronary artery from

the pulmonary artery

LCA = left coronary artery

RCA = right coronary artery

Hegde et al. J A C C : C A S E R E P O R T S , V O L . 2 , N O . 7 , 2 0 2 0

Echocardiographic Diagnosis of Bland-White-Garland Syndrome J U N E 1 7 , 2 0 2 0 : 1 0 2 1 – 4

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normal limits. Transthoracic echocardiogra-phy revealed an ejection fraction of 45%,moderate posteriorly directed mitral regur-gitation, mild hypokinesis of anteroseptumand anterior, anterolateral, and apical seg-ments of the left ventricle. Color Dopplershowed low-velocity, diastolic linear flow inthe septum from inferoseptum to ante-

roseptum, consistent with coronary vessel flow.Low-velocity diastolic flow was also noted into thepulmonary artery, representing reversed flow froman anomalous left main emptying into the pulmo-nary artery (Videos 1, 2, 3, and 4). Overall findingswere highly characteristic of an anomalous origin ofthe left coronary artery from the pulmonary artery(ALCAPA). He underwent a stress echocardiogram inwhich he achieved target heart rate and exercisedfor 13 min without experiencing chest pain ordiscomfort. Stress electrocardiography was notablefor 3-mm ST-segment depressions in the lateralleads (V4 to V6) and 1-mm ST-segment elevation inthe aVR lead. Post-exercise echocardiographyshowed more prominent hypokinesis of the anteriorwall and hypokinesis of anteroseptum. Mitral

Tortuous Right Coronary Artery as Seen on Coronary

(arrow) showing the dilated and tortuous right coro-

tensive collaterals to the left coronary artery.

regurgitation remained unchanged from baseline.Cardiac catheterization confirmed the presence ofan anomalous left coronary artery (LCA) originatingfrom the pulmonary artery. Right coronary angiog-raphy showed a very large right coronary artery(RCA) that filled the entire LCA retrograde viacollaterals with flow reversal in the LCA andcontrast emptying into the pulmonary artery(Figures 1 and 2). Ascending aortography confirmedthat there was no LCA arising from the ascendingaorta (Figure 3). His filling pressures were normal,and there was no step-up in oxygen saturationpresent.

MANAGEMENT

The patient then underwent successful reimplanta-tion of the LCA with concurrent mitral valve repair(Figures 4 to 6). His post-operative course remaineduneventful, and he was discharged on aspirin andwarfarin, given the very robust competing collateralflow with a dilated RCA, which carries a risk of coro-nary thrombosis.

FIGURE 2 Filling of Left Coronary Artery Through an Extensive

Network of Collaterals From the Right System

Coronary angiogram showing filling of left coronary artery via collat-

erals (red arrow). The blue arrow points to the origin of the left

coronary artery from the pulmonary artery.

FIGURE 3 The Origin of the Left Coronary Artery From the Pulmonary Artery and

Right Coronary From the Aorta

Aortogram shows origin of the left coronary artery from the pulmonary artery

(blue arrow). The red arrow points to the origin of the right coronary artery from the

right coronary sinus aorta.

FIGURE 4 Dilated Tortuous Epicardial Right Coronary Artery as Seen

Intraoperatively

Intraoperative photo showing the tortuous and dilated right coronary artery

(blue arrow) with extensive collaterals (red arrow).

J A C C : C A S E R E P O R T S , V O L . 2 , N O . 7 , 2 0 2 0 Hegde et al.J U N E 1 7 , 2 0 2 0 : 1 0 2 1 – 4 Echocardiographic Diagnosis of Bland-White-Garland Syndrome

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FOLLOW-UP

On his 1-month post-operative follow-up, he re-ported doing well. He admitted to realizing that hemay have had some chest discomfort whileclimbing hills but that this has completelyresolved since his surgery. A murmur was nolonger heard on a physical examination. He iscompliant with his aspirin and coumadin withoutbleeding issues.

DISCUSSION

Bland-White-Garland syndrome, also known asALCAPA, was first described in the literature in 1933by Bland, White, and Garland (1). ALCAPA is a veryrare congenital anomaly that occurs in 1 in 300,000births, with a 90% mortality within the first year oflife when left untreated (2). Although it mostcommonly presents in infancy, 10% of children withthis congenital anomaly will survive to adulthood.Case reports on adults with ALCAPA show that theirfirst presentation is on a spectrum of beingasymptomatic to acute heart failure, myocardialischemia, and sudden cardiac death (3,4). The de-gree of collateral formation between the RCA andLCA serves as a significant predictor for symptoms.Dilation of the RCA and the extensive collaterali-zation from the RCA to the left system occurs as acompensation for the lower blood oxygen level inthe LCA. Continuous blood from the RCA into theLCA and then into the low-pressure pulmonary ar-tery results in coronary steal phenomenon. Papil-lary muscle and LV lateral wall dysfunction inducedby chronic ischemia result in mitral regurgitation(5). Because the incidence of an adult type ofALCAPA is very low, often asymptomatic, andlacking ST-T changes on electrocardiography, pa-tients with this congenital anomaly go through lifeeither undiagnosed or misdiagnosed. Echocardiog-raphy is usually the initial diagnostic tool. Echo-cardiographic features including color Dopplermapping can show a continuous shunt retrogradeinto the pulmonary artery lumen at the abnormalorigin of the LCA. Abundant collateral bloodflow may be observed in the myocardium, espe-cially in the interventricular septum. Other sec-ondary features include left ventricular dilation anddysfunction, papillary muscle fibrosis, mitral valveprolapse or regurgitation, abnormal wall motion,and dilatated RCA (5). Although magnetic resonanceangiography and computed tomography angiog-raphy have been increasingly used for the diagnosisof ALCAPA, these modalities are unable to

determine intravascular blood flow conditions andrequire administration of contrast dye. Echocardi-ography is an indispensable diagnostic toolbecause it is noninvasive, is low cost, and hasthe ability to show the origin of the coronary

FIGURE 5 Intraoperative Image of Mitral Valve Repair

Intraoperative photo showing the pulmonary artery (blue arrow) being

opened and the deep-seated origin of left coronary artery (red arrow).

FIGURE 6 Intraoperative Image Showing Mitral Valve Repair

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artery. An obvious drawback is when the echocar-diographer does not have comprehensive knowl-edge of ALCAPA. Of note, the diagnosis may bedifficult in the setting of pulmonary hypertension,as the blood flow in the LCA might be forward, andthe “stealing blood” sign of the coronary artery isnot present.

CONCLUSIONS

ALCAPA is a rare congenital heart disease thatcarries a high mortality rate and may lead to suddendeath if left untreated. An understanding of thetypical echocardiographic features of ALCAPA iscritical in order to obtain the often elusive diag-nosis. Relatively young patients noted to have

depressed left ventricular systolic function andconcomitant mitral regurgitation without underlyingcoronary artery disease should raise suspicion foranomalous origin of a coronary artery. Ultrasoundimaging with transthoracic echocardiography servesas an essential tool for the early diagnosis of thiscongenital anomaly. An operator’s comprehensiveknowledge, diagnostic awareness, and echocardio-graphic skills are critical for the accurate diagnosisof ALCAPA.

ACKNOWLEDGMENT The authors would like tothank Robin Brides, RDCS, for performing thetransthoracic electrocardiography and obtaininggreat images.

ADDRESS FOR CORRESPONDENCE: Dr. ShrutiHegde, Cardiovascular Division, St. Elizabeth’s Med-ical Center, 736 Cambridge Street, Brighton, Massa-chusetts 02135. E-mail: shruti.hegde15@gmail.com.

RE F E RENCE S

1. Bland EF, White PD, Garland J. Congenitalanomalies of the coronary arteries: report of anunusual case associated with cardiac hypertrophy.Am Heart J 1933;8:787–801.

2. Yau JM, Singh R, Halpern EJ, Fischman D.Anomalous origin of the left coronary arteryfrom the pulmonary artery in adults: a compre-hensive review of 151 adult cases and a newdiagnosis in a 53-year-old woman. Clin Cardiol2011;34:204–10.

3. Fierens C, Budts W, Denef B, et al. A 72-year-oldwoman with ALCAPA. Heart 2000;83:e2.

4. Lin S, Xie M, Lv Q, et al. Misdiagnosis ofanomalous origin of the left coronary artery fromthe pulmonary artery by echocardiography: single-center experience from China. Echocardiography2020;37:104–13.

5. Ghaffari S, Ranjibar A, Hajizadeh R, et al. Asso-ciation of significant mitral regurgitation and leftventricular dysfunction with ALCAPA syndrome in

a young patient. Crescent J Med Biol Sci 2018;5:67–8.

KEY WORDS ALCAPA, coronary anomaly,dilated coronary artery, echocardiography

APPENDIX For supplemental videos,please see the online version of this paper.