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Abstract
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Rheumatic heart disease (RHD) has a prevalence of 0.2 to 1.1/1000 in India. 25% patients with RHD develop mitral stenosis (MS). Marfan’s syndrome (MFS) is an autosomal dominant inherited defect in the FBN1 gene coding for fibrillin. Skeletal and cardiovascular involvement is common. Mitral valve prolapse or mitral regurgitation is common while mitral stenosis is seldom associated with MFS. Our patient had a rare combination of marfanoid habitus and severe rheumatic mitral stenosis (MS) which to the best of our knowledge has been scarcely reported. When indicated as per guidelines, percutaneous mitral balloon valvotomy (PBMV) is the procedure of choice for MS. MFS patients have a high genetic propensity towards developing MR and hence a PBMV would be associated with a high risk of MR. Our case was successfully managed with PBMV with good symptomatic relief and no post-procedural MR. |
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Keywords :
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Balloon Valvuloplasty, Cardiac Surgical Procedures, Heart Valves, Marfan Syndrome, Mitral Valve Stenosis.
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