A patient with Marfan's syndrome presented with severe rheumatic mitral stenosis and successfully treated with percutaneous transmitral balloon commissurotomy - Report of first case.

Marfan's syndrome (MFS) is an inherited autosomal dominant disorder of connective tissue with mutation on the fibrillin-1 gene encoding for fibrillin. This frequently involves the cardiovascular system with prevalence is 1:5000-10,000. The clinical major criteria involve the skeletal and ocular apparatus and the cardiovascular and central nervous system. In Marfan's syndrome mitral valve prolapse and aortic dilation are the main cardiovascular manifestations. According to the literature database the prevalence of aortic dilatation is 76%, 62% for mitral valve prolapse, 29% for mitral valve regurgitation and 26% for aortic regurgitation, in adult patients with Marfan's syndrome. We are reporting a case who presented with severe rheumatic mitral stenosis & later on examination found to be a case of Marfan's syndrome. This patient was successfully treated with percutaneous transmitral balloon commissurotomy. In medline search, so far there is no other case of Marfan's syndrome reported to have rheumatic mitral stenosis.