Palliation of a Heterotaxy Single Ventricle Neonate with Pulmonary Atresia and Obstructed TAPVR

AbstractPatients born with obstructed total anomalous pulmonary venous return have a high risk of morbidity and mortality in the neonatal period, which only increases when combined with single ventricle physiology and non-cardiac congenital anomalies such as heterotaxy syndrome. Despite advances in management of congenital heart disease, surgery within the first weeks of life to repair the pulmonary venous connection and establish pulmonary blood flow with a systemic-to-pulmonary shunt has historically led to disappointing outcomes. A multidisciplinary approach with pediatric interventional cardiology and cardiac surgery is required to reduce morbidity and mortality in this extremely high-risk patient population. Extending the time between birth and cardiac surgery can lessen postoperative complications and mortality risk, especially in patients with abnormal thoracoabdominal relationships. Our team was able to successfully utilize transcatheter stent placement in a vertical vein and patent ductus arteriosus to delay and stage cardiac surgeries in an infant born with obstructed total anomalous pulmonary venous return, unbalanced atrioventricular septal defect with pulmonary atresia and heterotaxy, thus reducing the morbidity and mortality associated with this diagnosis.