Non malignant superior vena cava syndrome

A 62-year-old woman was admitted to our clinic for the evaluation of 2.5-ye ar history of progressive exertional facial flushing, and swelling of the h ead, face, and neck. Three years earlier, she had undergone implantation of a definitive pacemaker with an endocardial ventricular lead for a severe b radycardia. Physical examination revealed facial swelling and erythema incl uding the periorbital areas, distended neck and edema of the region above t he lower margins of the rib cage. Numerous dilated cutaneous venules were p resent on the ventral upper thoracic wall interspersed with patches of inte nse flush and large areas of normally colored skin (Fig. 1). There had been no weight loss within the previous months. History of angioedema, allergic reactions, autoimmune diseases, or medication was negative. Enlarged lymph nodes were not detected. Complete blood cell count, fibrinogen, Quick's te st for prothrombin, antithrombin ill, C-reactive protein, lipidic profile, renal functions, urinanalysis, and thyroid function test were within normal ranges. Standard X-ray and sectional radiography of the chest revealed no lung diseases or alterations of the mediastinum. Doppler examination of the supraaortic trunks and chest CT scan showed normal findings. A diagnosis o f superior vena cava syndrome (SCVS) was suspected and a cavography was per formed. by injecting contrast medium simultaneously through the basilic vei ns, that revealed marked narrowing of the confluence of the two brachioceph alic veins at the junction of the internal jugular and right subclavian vei ns with a total obliteration of the right brachiocephalic vein (Fig. 2). Th e diagnosis of pacemaker-induced SVCS was confirmed and the woman was refer red for angiosurgery.