Asymptomatic Adult Type ALCAPA Syndrome Coexisting with Bicuspid Aortic Valve- A Case Report

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome (BWGS) is a rare variant of the congenital anomalies of the coronary arteries. Two forms, the infantile (most common) and the adult types (rare) of these syndromic manifestations have been reported in the literature. Affected infants present with myocardial infarction and congestive heart failure. Few of them survive the first year of life, without appropriate surgical intervention. Adult type ALCAPA syndrome is one of the important causes of malignant cardiac arrhythmias, acute coronary syndromes, valvular pathologies and sudden cardiac death in young adults and adults. Asymptomatic adult type ALCAPA is uncommon, much more so its coexistence with bicuspid aortic valve (BAV). We present a 65-year-old patient with ALCAPA and BAV, who was largely asymptomatic. She had ligation of the ALCAPA, and aortic valve replacement, with a very good outcome.