Aortopulmonary window; hemitruncus

Abstract

Aortopulmonary (AP) window is a very rare congenital heart disease resulting from a defect in the shared wall between the great arteries during embryogenesis. There are four major types of AP window based on the latest accepted classification by the Society of Thoracic Surgeons Congenital Heart Surgery Database Committee. Hemitruncus is a misnomer because there are two semilunar valves in this lesion, and it should be correctly termed as the anomalous origin of the right (more common) or left pulmonary artery from the ascending aorta. AP window and anomalous branch pulmonary artery from ascending aorta lead to a left-to-right shunt. The patients present with symptoms of congestive heart failure and will develop pulmonary hypertension and pulmonary vascular obstructive disease if left untreated. Echocardiography remains the main diagnostic modality in detecting and defining these lesions. Cardiac computed tomography, magnetic resonance imaging, and cardiac catheterization can provide additional information in selected cases. Medical management includes treating the heart failure symptoms using medications. Transcatheter closure is feasible in selected cases. Surgical repair using patch closure is the preferred treatment and should be done early to avoid the development of pulmonary hypertension. Pulmonary hypertensive crisis can complicate the immediate postoperative care. Long-term survival as well as late results of surgical correction of AP window without associated anomalies is excellent.