agenesis of the superior vena cava

Rare Disease

MONDO:0020445

Also Known As

absence of the SVCabsence of the superior caval veinabsence of the superior vena cavaagenesis of the SVCagenesis of the superior caval vein

Definition

Agenesis of the superior vena cava (SVC) is a rare congenital anomaly of the great veins characterized by unilateral or bilateral complete absence of the SVC. Unilateral agenesis is mainly asymptomatic (most of the time diagnosed incidentally) and patients usually have otherwise normal heart structure. Bilateral agenesis, however, is frequently associated with other congenital cardiac anomalies and/or conduction abnormalities (such as tetralogy of Fallot, atrial septal defect) and typically present symptoms of SVC syndrome.