Multiple arteriovenous aneurysms of soft tissues and bone (pelvis and vertebrae) resulting in cardiac failure

Abstract

A case is presented of a 34-year-old man who was hospitalized because of a three-month history of dependent edema and orthopnea. A diagnosis of rheumatic valvulitis was entertained, based upon the patient's age, the obvious signs of right and left heart failure, and a high pulse pressure. The absence of a murmur of aortic insufficiency in the presence of Corrigan's pulse prompted the consideration of arteriovenous fistula. Murmurs were heard in the lower vetebral and posterior pelvic regions and x-ray examinations of these areas revealed numerous lytic lesion. The coincidence of the pelvic murmurs and osseous lysis led to the clinical diagnosis of angiosarcoma with arteriovenous fistulas and resultant heart failure. An unusual low-salt syndrome, owing to salt deprivation and mercurial diuretics, was present. He expired five weeks after admission. Autopsy disclosed an extraordinary cardiac hypertrophy and multiple communications between the left hypogastric artery and the left common iliac vein. A very large aneurysmal sac was found occupying most of the posterior pelvic cavity. Extending posteriorly were dilated vessels which entered the pelvic and vertebral bones to form loculated, interconnected, intraosseous vascular channels. Evidence is presented which suggests that these vascular lessions are developmental anomalies. When one recalls that arteries and veins have a common embryonic origin from a capillary plexus, lying in close proximity to large vascular trunks, it is quite surprising that abnormal vascular communications do not occur more frequently. An alertness to this possibility may prompt the more frequent discovery of deeply hidden arteriovenous fistulas in those cases of decompensated heart disease in which the physical signs do not explain adequately the clinical findings.