Total anomalous pulmonary venous return (TAPVR) represents 1%-5% of congenital heart disease? It consists of an anomalous connection between the pulmonary veins with the right atrium, or with one or more systemic veins.
The classification of TAPVR is based on the location of the pulmonary venous drainage. There are 4 types: (1) supracardiac ( the most common type,44% of all cases ) (2) cardiac (22% ) (3) infracardiac ( 26% ) , and (4) mixed (9%).
In the infracardiac type, the drainage is located below the diaphragm, and may be either to a systemic vein such as inferior vena cava, hepatic veins or azygos system, or to the portal venous system. Any type of TAPVR may be associated with obstruction. However, the infracardiac type is the most frequently complicated by obstruction of the drainage vein, often located at the level of the diaphragm.
The echocardiography allows to raise the diagnosis in front of a dilatation of the right cavities with a small left atrium which does not receive any pulmonary vein, with an obligatory right-left shunt by the ASD
The CT scan allows the localization the collector and the drainage vein. Figure 1 Show the CT scan of our patient show pulmonary veins drain through a common trunk below the diaphragm.
The diagnosis of TAPVR is an indication for surgery. Thanks to better diagnostic tools and to improvements in surgical techniques, the repair of TAPVR has become a surgical procedure with very low risks of morbidity and mortality.
