Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly that leads to an anatomical left-to-right shunt. Termination of the intrahepatic inferior vena cava (IVC) with its azygos continuation associated with the hepatic venous connection to the left atrium (LA) is also a rare congenital anomaly that results in an anatomical right-to-left shunt. A 65-year-old male presented with severe dyspnea on exertion and pedal edema. He was further diagnosed at our clinic and was found to have both the aforementioned congenital abnormalities, creating a bidirectional shunt. On further investigation, he was found to have nocturnal hypoxemia on overnight oximetry. The patient was successfully treated via surgical corrections of the congenital anomalies leading to symptomatic improvement as well as the resolution of nocturnal hypoxemia.
Keywords: Congenital heart disease; left-to-right shunt; nocturnal hypoxemia; partial anomalous pulmonary venous connection.