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A 32-Year-Old Man With Chronic Left Ventricular Dysfunction

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 32-year-old man with a diagnosis of dilated cardiomyopathy sought care for angina and dyspnea with exertion. His chest pain and dyspnea were chronic and had recently increased in frequency. His cardiomyopathy followed a diagnosis of myocarditis after repeated putative chest colds and chest pain at age 14 years, at which time the ejection fraction was reported to be 30%. The results of a graded exercise stress test at 22 years were unremarkable.

On examination, he was afebrile. His heart rate was 68 beats per minute, his blood pressure was 114/77 mm Hg, and his examination was notable for an absence of jugular venous distension, peripheral edema, and murmurs or extra heart sounds. His electrocardiogram, shown in Figure 1, was notable for right bundle branch block and left anterior fascicular block, unchanged from his electrocardiogram at age 14 years. Transthoracic echocardiography revealed an ejection fraction of 40%. An exercise nuclear stress test revealed a fixed, severe apical defect, a partially reversible severe anterior wall defect, moderate global hypokinesis, and severe hypokinesis of the distal anterior wall and apex. His ejection fraction was estimated to be 32%. Given these findings, left-sided heart catheterization was performed.

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An anomalous left coronary artery from the pulmonary artery

C. Coronary artery reimplantation

The coronary angiogram is shown in Figure 2 and the Video. This case highlights the importance of vigilance and thoughtful diagnosis, because this patient’s initial clinical picture did not necessarily fit with the assigned diagnosis. Unfortunately, many patients with an anomalous left coronary artery from the pulmonary artery (ALCAPA) are compartmentalized into diagnoses that are more often found, such as myocarditis and dilated cardiomyopathy; however, the morbidity and mortality of this condition is such that there is little margin for error.1 

An ALCAPA is an exceedingly rare and potentially fatal congenital cardiac anomaly with an incidence of 1 in 300 000 live births, making up 0.24% to 0.46% of all congenital cardiac anomalies.2,3 Of infants born with an ALCAPA who do not undergo surgical correction, 90% die within the first year of life and the remaining 10% die of complications such as myocardial ischemia and arrhythmias.4 The mean life expectancy of patients who survive past infancy is 35 years.5 From 1908 to 2008, only 151 adults have been reported to have had an ALCAPA.6

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Article Information

Corresponding Author: M. Bilal Ahmed, MD, MBA, Warren Alpert School of Medicine, Department of Medicine, Brown University, 793 Eddy St, Providence, RI 02903 (mbahmed@crimson.ua.edu).

Published Online: May 13, 2020. doi:10.1001/jamacardio.2020.0295

Conflict of Interest Disclosures: None reported.

Additional Information: We thank the patient for granting permission to publish this information.

References
1.
Levitas  A , Krymko  H , Ioffe  V , Zalzstein  E , Broides  A .  Anomalous left coronary artery from the pulmonary artery in infants and toddlers misdiagnosed as myocarditis.   Pediatr Emerg Care. 2016;32(4):232-234. doi:10.1097/PEC.0000000000000511PubMedGoogle ScholarCrossref
2.
Bland  EF , White  PD , Garland  J .  Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy.   Am Heart J. 1933;8(6):787-801. doi:10.1016/S0002-8703(33)90140-4Google ScholarCrossref
3.
Frescura  C , Basso  C , Thiene  G ,  et al.  Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease.   Hum Pathol. 1998;29(7):689-695. doi:10.1016/S0046-8177(98)90277-5PubMedGoogle ScholarCrossref
4.
Wesselhoeft  H , Fawcett  JS , Johnson  AL .  Anomalous origin of the left coronary artery from the pulmonary trunk: its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases.   Circulation. 1968;38(2):403-425. doi:10.1161/01.CIR.38.2.403PubMedGoogle ScholarCrossref
5.
Alexi-Meskishvili  V , Hetzer  R , Weng  Y ,  et al.  Anomalous origin of the left coronary artery from the pulmonary artery: early results with direct aortic reimplantation.   J Thorac Cardiovasc Surg. 1994;108(2):354-362. doi:10.1016/S0022-5223(94)70018-4PubMedGoogle ScholarCrossref
6.
Yau  JM , Singh  R , Halpern  EJ , Fischman  D .  Anomalous origin of the left coronary artery from the pulmonary artery in adults: a comprehensive review of 151 adult cases and a new diagnosis in a 53-year-old woman.   Clin Cardiol. 2011;34(4):204-210. doi:10.1002/clc.20848PubMedGoogle ScholarCrossref
7.
Edwards  JE .  The direction of blood flow in coronary arteries arising from the pulmonary trunk.   Circulation. 1964;29:163-166. doi:10.1161/01.CIR.29.2.163PubMedGoogle ScholarCrossref
8.
Baue  AE , Baum  S , Blakemore  WS , Zinsser  HF .  A later stage of anomalous coronary circulation with origin of the left coronary artery from the pulmonary artery: coronary artery steal.   Circulation. 1967;36(6):878-885. doi:10.1161/01.CIR.36.6.878PubMedGoogle ScholarCrossref
9.
Gao  Y , Zhang  J , Huang  GY , Liang  XC , Jia  B , Ma  XJ .  Surgical outcomes of anomalous origin of the left coronary artery from the pulmonary artery in children: an echocardiography follow-up.   Chin Med J (Engl). 2017;130(19):2333-2338.PubMedGoogle Scholar
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