Anomalous Pulmonary Venous Connections

Total anomalous pulmonary venous connection (TAPVC) may occur as an isolated lesion or in association with complex congenital heart disease. 2D echocardiography in patients with isolated TAPVC reveals significant enlargement of the right atrium, right ventricle and pulmonary arteries. Colour Doppler is a valuable tool to identify the pulmonary venous confluence and its connection to the right atrium or systemic veins. Possible sites of anomalous pulmonary venous connection, which should be checked by colour Doppler interrogation, include the right atrium, the coronary sinus, the right and left superior vena cava, the azygos vein, the innominate vein, the inferior vena cava, the portal vein system and the hepatic veins. Echocardiographic detection of TAPVC in the context of complex congenital heart disease requires a systematic sequential approach that starts with clarification of venous connections. Frequently these patients also have anomalies of visceral and atrial situs summarized as heterotaxy syndromes. Partial anomalous pulmonary venous connection (PAPVC) can be associated with various cardiovascular malformations or it may occur as an isolated lesion. Anomalous drainage of the right upper pulmonary vein is frequently encountered in patients with sinus venosus ASD, while PAPVC of the right lower pulmonary vein is an integral part of the Scimitar syndrome.