Clinical Presentation and Therapy of Truncus Arteriosus

Abstract

Truncus arteriosus (TA, also known as common arterial trunk) consists of a ventricular septal defect and only one great artery (“the truncus”) arising from the heart (Fig. 46.1). This great artery is positioned above the ventricular septal defect and gives rise to the coronary arteries, the pulmonary arteries, and the aortic arch. TA has been classified as three types. In type 1 the right and left pulmonary arteries arise from a main pulmonary artery that arises from the aorta. In type 2 the right and left pulmonary arteries arise from separate orifice but close together. In type 3 the pulmonary arteries arise separately and distant from each other. Significant associated anomalies include truncal valve insufficiency, stenosis, and interrupted aortic arch. Truncus arteriosus constitutes approximately 0.7 % of all congenital heart defects. It results from abnormal conotruncal septation. There is a relatively common association between microdeletion 22q11.2 and truncus arteriosus especially if there is interruption of the aortic arch.