Persistent left superior vena cava communicating with the left atrium through a systemic-pulmonary venous malformation.
A 14 year old white girl who presented with a brain abscess was discovered to have a left pulmonary vascular malformation on a chest roentgenogram. Angiograms revealed a left superior vena cava that drained into a venous malformation within the left lung, then communicated with the left atrium by way of the left superior pulmonary vein. The right superior vena cava was functionally absent and was anatomically an atretic cord. There was mild systemic arterial hemoglobin desaturation, but no evidence of cyanosis. The embryology, physiology and surgical repair of this rare lesion and the complication of a postoperative superior vena cava syndrome are discussed.
D000293:Adolescent; D003490:Cyanosis / Q000209:etiology; D005260:Female; D006325:Heart Atria / Q000002:abnormalities; D006439:Hemodynamics; D006801:Humans; D011651:Pulmonary Artery / Q000000981:diagnostic imaging; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D011859:Radiography; D014683:Vena Cava, Superior / Q000002:abnormalities*