Abstract | October 29, 2020
Anomalous Left Coronary Artery from the Pulmonary Artery Presenting in an Adult with Heart Failure
Learning Objectives
- ALCAPA syndrome albeit sporadic is not confined only to childhood and should be considered in adults as well.
- Prognosis at time of presentation of ALCAPA depends on development of adequate collateral circulation.
- All anomalously arising coronary arteries from the pulmonary artery require surgical correction.
Introduction: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital anomaly, occurring in 1 in 300,000 live births. This condition will commonly lead to ischemia and heart failure and without intervention, results in a 90% mortality rate within the first year of life [1]. Survival beyond this time period is dependent on collateral circulation from the right coronary artery. Adult presentation is therefore rare with most patients presenting with left ventricular systolic dysfunction. [1,2]. Additionally, an 80 to 90% incidence of sudden cardiac death in patients with a mean age of 35 years has been reported [3].
Case Summary: A 45-year-old male presented to the emergency room with two weeks of worsening exertional dyspnea and edema with erythema of his lower extremities. He was admitted for suspected heart failure. He had TTE which showed reduced LVEF 40%. Patient underwent coronary angiography that demonstrated a markedly dilated right coronary artery with contrast enhancement of the left coronary system that arose from the pulmonary artery as seen in Figure 1. There was no atherosclerotic CAD. A CT coronary angiography demonstrated significant collateral circulation from the posterior descending artery to the left anterior descending artery and obtuse branches of the circumflex artery. The left main coronary artery originated in the posterolateral segment of the pulmonary artery. A diagnosis of ALCAPA was made.
Patient underwent coronary artery bypass surgery SVG to LAD and pulmonary artery was opened and the anomalous left main coronary ostium was patched Figure 2. Medical therapy for ischemic cardiomyopathy was initiated. At 6 month follow up patient has remained asymptomatic and LVEF improved to 60 %.
References
- Schwerzmann, M., Salehian, O., Elliot, T., Merchant, N., Siu, S. and Webb, G. (2004). Anomalous Origin of the Left Coronary Artery From the Main Pulmonary Artery in Adults. Circulation, 110(21).
- WESSELHOEFT, H., FAWCETT, J. and JOHNSON, A. (1968). Anomalous Origin of the Left Coronary Artery from the Pulmonary Trunk. Circulation, 38(2), pp.403-425.
- Dodge-Khatami, A., Mavroudis, C. and Backer, C. (2002). Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. The Annals of Thoracic Surgery, 74(3), pp.946-955.
