Abstract
CASE SUMMARY: An 8-day-old boy with univentricular physiology presented with respiratory failure and persistent hypoxemia. Cardiac computed tomography angiography showed a dominant right ventricle with mitral atresia and an intact atrial septum, double-outlet right ventricle, and persistent ductus arteriosus. Pulmonary veins connected to the left atrium but were markedly dilated, with anomalous dual drainage through a levoatrial cardinal vein into the innominate and azygos veins. Despite emergent atrial septectomy, pulmonary artery banding, and ductal ligation, the outcome was unfavorable. TAKE-HOME MESSAGE: Distinguishing pulmonary venous connection from drainage is essential, and cardiac computed tomography angiography is key for surgical planning.