[Calcification of the mitral valve ring, a cardiac manifestation of Marfan's syndrome (author's transl)].
A 29-year-old patient with calcification of the mitral valve annulus with Marfan's syndrome and severe malformation of the chest is reported. There are 8 other cases with calcification of the mitral annulus in this syndrome in the literature. It seems to be a specific but rare cardiac manifestation of Marfan's syndrome with calcification occurring also in the younger age group. These patients, in contrast to other patients with Marfan's syndrome who mostly have mitral insufficiency of minor degree mainly in late systole, present with hemodynamically severe mitral regurgitation. Our patient died 7 weeks after implantation of a prosthetic valve in low output syndrome, probably caused by a paraprosthetic leak and newly developed aortic insufficiency. It therefore seems that indication for prosthetic replacement of the mitral valve in these patients should be made with caution, particularly in regard to the technical difficulties at operation.
D000328:Adult; D002114:Calcinosis / Q000150:complications*; D006328:Cardiac Catheterization; D004562:Electrocardiography; D006321:Heart / Q000000981:diagnostic imaging; D006349:Heart Valve Diseases / Q000150:complications; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging; D008943:Mitral Valve; D011859:Radiography
Verkalkung des Mitralklappenringes als kardiale Manifestation des Marfan-Syndroms