Total anomalous pulmonary venous connection to the portal vein

Anomalous pulmonary venous return represents a rare congenital anomaly with wide anatomic and physiologic variability. We report a case of a newborn with a rare form of total infracardiac anomalous pulmonary venous connection (TAPVC). The pulmonary veins draining both lungs formed two vertical veins, which joined to a common pulmonary trunk below the diaphragm. This venous channel connected to the portal vein through the esophageal hiatus. The diagnosis was suggested by color Doppler sonography and confirmed by intravenous digital subtraction angiography, which allowed definition of the anatomy.