Cardiovascular Features in a Case of Ehlers-Danlos Syndrome

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Diagnosis of Ehlers-Danlos syndrome was made in a 37-year-old man on the basis of hyperextensibility of the fingers joints, hyperelasticity and fragility of the skin, molluscoid pseudotumor, calcified subcutaneous spheroids, microscopic alterations of the dermis, megacolon, intestinal bleeding and bilateral inguinal hernia.A history of anginal pains, a loud ejection type systolic murmur over the aortic area and electro- and vectorcardiographic signs of unusually severe left ventricular hypertrophy simulated aortic valve stenosis. Cineangiograms of the left ventricle confirmed the existence of high degree concentric hypertrophy. Pressure, oxygen saturation, flow measurements, and cineangiography, however, excluded aortic stenosis and other hemo dynamic factors as causes of the hypertrophy. Finding of sudden narrowing of the aorta at the level of the arch reasonably accounted for the systolic murmur.Cardiac anomaly is interpreted as dependent upon a primary myocardial disease, possibly related to the tissue disorders of the Ehlers-Danlos syndrome.