An Exceedingly Rare Combination of Truncus Arteriosus and Total Anomalous Pulmonary Venous Connection

Abstract

A neonate with truncus arteriosus (TA) and concomitant total anomalous pulmonary venous connection (TAPVC) is presented. The diagnosis of TA was made on antenatal ultrasound. An echocardiogram performed immediately after birth confirmed TA with a regurgitant quadrileaflet truncal valve. This echo further demonstrated all four pulmonary veins connected to a supracardiac confluence draining to the superior vena cava (SVC) via a partially obstructed ascending vein. Cardiac computed tomography confirmed TA and TAPVC, and demonstrated an ascending vein arising from the supracardiac confluence, passing over the aorta before inserting into the SVC. The patient underwent prompt single-stage repair. The postoperative course was complicated by a pulmonary embolism requiring surgical intervention, and severe truncal valve regurgitation necessitating a third surgery within the first 4 months of life. Review of the literature suggests this combination of defects is exceedingly rare and, of those cases reported, there is a high surgical and postsurgical complication rate.