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  • Title: [A case of Klinefelter's syndrome with acute mitral regurgitation caused by idiopathic chordal rupture].
    Author: Katsumata T, Tomizawa Y, Okoshi T, Ego Y, Iguchi N, Saigusa H, Nakagawa M, Kobayashi H, Kato T, Takahashi S.
    Journal: Kyobu Geka; 1991 Sep; 44(10):867-70. PubMed ID: 1921008.
    Abstract:
    It has been reported that the incidence of mitral valve prolapse was remarkably increased in patients with Klinefelter's syndrome. Some cases which have been previously reported were associated with hemodynamically significant transvalvular regurgitation, however, none of them underwent a surgical treatment such as valvular replacement. Here we report a case of Klinefelter's syndrome which underwent MVR for mitral regurgitation caused by idiopathic chordal rupture. Pathological microscopic examination showed topical and minor myxomatous changes on the free edge of the leaflet and no inflammatory changes such as in infectious endocarditis. It is, therefore, suggested that an echocardiographic follow-up should be carried out in case of Klinefelter's syndrome during its life and also in the case treated by a prophylactic therapy for endocarditis with antibiotics. And when, unfortunately, surgical treatment is required, valve replacement would be preferable to valvuloplasty because of a fragility of leaflet.
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