Supracardiac Total Anomalous Pulmonary Venous Connection with Left Main Bronchial Compression in an Infant

Abstract

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac malformation, and cases complicated with airway stenosis are even less common, posing substantial diagnostic and therapeutic challenges. We report a 4-month-old male infant with supracardiac TAPVC, accompanied by left main bronchial stenosis, a central atrial septal defect (ASD), and severe pulmonary hypertension. Following supportive management, the patient underwent TAPVC repair and atrial septal defect patch repair. Intraoperatively, weaning from cardiopulmonary bypass initially failed due to severe pulmonary hypertension, but was successfully achieved after fenestration of the atrial septal patch for decompression. Postoperative imaging confirmed unobstructed pulmonary venous return, resolution of pulmonary hypertension, and relief of airway compression. At 6-month follow-up, the infant demonstrated normal growth and development. TAPVC with airway stenosis markedly increases perioperative risk, and thorough preoperative assessment with timely surgical intervention is essential for favorable outcomes.