Total anomalous pulmonary venous return is a congenital heart malformation characterized by anomalous pulmonary venous inflow to the right atrium. Surgical repair typically occurs during the first month of life, and survival beyond that age in untreated patients is unlikely. We report an extreme case of supracardiac total anomalous pulmonary venous return in an infant who survived 7 months despite atypical anomalous inflow without atrial-level communication and with right-to-left shunting only through a patent ductus arteriosus. We stabilized the patient's left-sided heart function before surgically repairing the anomalous venous return 2 months later. Three years postoperatively, the patient was well.
Keywords: Cardiovascular abnormalities; disease-free survival; drainage; heart defects, congenital/diagnosis/surgery; infant; pulmonary veins/abnormalities/diagnostic imaging/surgery; treatment outcome; vascular surgical procedures/methods.
© 2022 by the Texas Heart® Institute, Houston.