Congenital Familial Vascular Anomalies: A Study of Patients with an Anomalous Inferior Vena Cava,and of Their First-Degree Relatives

Abstract

With magnetic resonance angiography and computed tomography, congenital anomalies of the inferior vena cava are diagnosed more frequently than they used to be. Accessory renal arteries identified by magnetic resonance angiography in a patient with an anomalous inferior vena cava indicated a combination of arterial and venous abnormalities. The study was initiated to screen consecutive patients with an anomalous inferior vena cava for concomitant abdominal and pelvic arterial abnormalities, and their first-degree relatives for congenital vascular anomalies. Magnetic resonance angiography identified in 2 of 5 patients with an anomalous inferior vena cava concomitant accessory renal arteries and in 5 of 11 first-degree relatives major abdominal vascular anomalies including accessory renal arteries, accessory renal veins, and anomalies of the hepatic artery. None of the relatives showed abnormalities of the inferior vena cava. The familial occurrence of vascular anomalies strongly suggests an underlying pathogenetic component in affected family members. In patients with a congenital anomaly of the inferior vena cava, concomitant arterial abnormalities should be considered. First-degree relatives may be at risk for congenital vascular anomalies.