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Severe Aortic Regurgitation in Von Recklinghausen’s Disease

A 71-year-old man with von Recklinghausen’s disease presented with NYHA class II exertional dyspnea and easy fatigability since about 20 days. Echocardiography and angiography demonstrated dilatation of aortic root, ascending aorta, and left ventricle, together with severe aortic regurgitation. Because of very few cases of cardiovascular abnormalities having been reported with von Recklinghausen’s disease, it is logical to concur that this is simply co-existence, whereas other possible mechanisms are endothelial dysfunction or sympathetic denervation.