Concurrent finding of rheumatic severe mitral regurgitation and anomalous left coronary artery from the pulmonary artery (ALCAPA) in a 23-year-old patient consulting for ventricular tachycardia: A case report and a review of the literature

Abstract

Introduction

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease that in most cases manifests in the first months of life, being a well-studied entity of myocardial ischaemia in young patients. It has been reported that up to 90 % of infants die without treatment in the first year, although late diagnosis is increasingly reported in adults.

Case summary

We present the case of a 23-year-old woman of Moroccan origin with no medical history of interest, who was admitted to the cardiac intensive care unit after an episode of sustained ventricular tachycardia (VT) that required electrical cardioversion. On admission, the electrocardiogram and blood test were normal, but the transthoracic echocardiogram showed moderate left ventricular systolic dysfunction and severe mitral regurgitation of rheumatic aetiology which was later confirmed with a transesophageal echocardiogram. In view of the arrhythmic event presented, a complete study was performed with cardiac magnetic resonance imaging (MRI) which showed no pathological enhancements. Nevertheless, coronary artery angiography revealed an anomalous origin of the left coronary artery in the pulmonary artery which was confirmed by cardiac computed tomography (CT). Corrective surgery was performed with direct translocation of the left coronary artery to the aorta and mitral valve replacement.

Discussion

In ALCAPA, the coronary steal phenomenon produced from the pulmonary artery to the coronary circulation causes myocardial ischaemia and involves a left-to-right shunt. Myocardial ischaemia is cause of angina, systolic dysfunction, mitral regurgitation and sudden cardiac death in children and young adults.