Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA)

ALCAPA is a rare congenital heart lesion. It occurs when the left coronary arteryCoronary artery arises from the pulmonary trunk instead of the aorta. Patients usually present in infancy with acute heart failureHeart failure or circulatory collapse due to poor left ventricular function and the lesion is often fatal if not promptly recognised and treated. Occasionally patients present in adulthood with sudden cardiac deathSudden cardiac death or myocardial ischemiaIschemia. ALCAPA usually occurs in isolation. Diagnosis can be made easily by echocardiography and should be excluded in any pediatricPediatric patient presenting with left ventricular dysfunction. Colour Doppler can demonstrate flow from the left coronary arteryCoronary artery into the main pulmonary artery in diastole. Mitral regurgitationMitral regurgitation due to ischemiaIschemia of the papillary muscle and annular dilation can be severe and may also provide a clue to the diagnosis. Surgical repair to establish an aortic connection to the left coronary should be undertaken as soon as possible. Long term survivalSurvival is excellent, however survivalSurvival is dependent on recovery of ventricular function and the degree of mitral regurgitationMitral regurgitation. Rarely, a ventricular assist deviceVentricular assist devices (VADs) may be needed as a bridge to ventricular remodelling. Occasionally mitral valve repairMitral valve repair or replacement may be warranted depending on the degree of secondary regurgitation.