congenital total pulmonary venous return anomaly

Rare Disease

MONDO:0007130

Also Known As

TAPVRpulmonary venolobar syndrometotal anomalous pulmonary venous returnTAPVR1anomalous pulmonary Venous returnscimitar anomalyscimitar syndrometotal anomalous pulmonary VENOUS return 1

Definition

Total pulmonary venous return (TAPVR) is a form of congenital pulmonary venous return where all of the pulmonary veins drain into the right atrium or one of its tributaries, instead of the left atrium, leading to various manifestations such as fatigue, exertional dyspnea, pulmonary arterial hypertension, cyanosis and progressive congestive heart failure.