Total anomalous pulmonary venous return (TAPVR)

Abstract

In this anomaly the pulmonary veins are returning the oxygenated blood back to the systemic venous circuit. As in transposition of the great arteries, survival depends upon the existence of shunts between the two circuits. As a rule this obligatory shunting occurs at atrial level. Different types of TAPVR are recognized depending on the site of the abnormal pulmonary venous drainage. In the supracardiac type the pulmonary veins drain into a posteriorly located venous collector, from where blood then usually is directed into the superior vena cava via a left vertical and innominate vein. In the cardiac type the pulmonary veins connect, either separately or jointly, to the right atrium or the coronary sinus. In the infracardiac or infradiaphragmatic type the pulmonary veins connect to the inferior vena cava or to the hepatic-portal system. Obstruction to pulmonary venous drainage is practically always documented in the infradiaphragmatic type. In the cardiac or supracardiac types obstruction to drainage may or may not be associated.