Surgical intervention in an incidentally diagnosed adult ALCAPA in the fourth decade or later-operate or observe?

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly with high infantile mortality, if untreated. Survival into adulthood is exceptional and typically depends on extensive inter-coronary collateral circulation. We present a 37-year-old asymptomatic man in whom ALCAPA was discovered incidentally during evaluation for a non-cardiac cerebrovascular event. Imaging revealed global left ventricular hypokinesia, reduced systolic function (ejection fraction 30-35%), and markedly dilated right coronary artery (RCA). Surgical repair was performed with saphenous vein grafting following ostial closure of the anomalous left main coronary artery (LMCA). At 6-month follow-up, the patient remained asymptomatic despite persistent left ventricular dysfunction. While surgery provides definitive restoration of dual coronary perfusion and protection against sudden cardiac death, some patients remain stable without intervention. A risk-stratified approach is therefore warranted, balancing operative risk, collateral circulation, and ischemic burden. Individualized decision-making, guided by multimodal imaging and clinical risk assessment, is essential in this rare but clinically significant anomaly.