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  • Title: [Surgical treatment of Marfan's syndrome with annulo aortic ectasia and mitral regurgitation].
    Author: Yamaguchi T, Kazui T, Sakai E, Watanabe A, Inoue N, Tsukamoto M, Inaoka M, Yamada O, Komatsu S.
    Journal: Nihon Kyobu Geka Gakkai Zasshi; 1992 Mar; 40(3):393-8. PubMed ID: 1583363.
    Abstract:
    This report is concerned with results of surgical treatment for Marfan's syndrome combined with annulo-aortic ectasia (AAE) and mitral regurgitation (MR). Of the 23 patients with Marfan's syndrome who received Bentall's procedure during 14 year period, seven (30%) of these patients had both AAE and MR. The MR grade of seven patients by cardiac Doppler or left ventriculographic studies were grade 1 in 2, 2 in 1, 3 in 1, and 4 in 3. Atrial fibrillation was present in 4 patients. New York Heart Association Functional Class on admission in these 7 patients were II in 1, III in 4, and IV in 2. The mitral valve was replaced with mechanical valve in 4 patients by left atrial approach whose MR grade were over 3. In the 4 patients the mitral annuli were extremely dilated, both valve leaflets were massively redundant, and all chordae were elongated and turned chordae and vegetation were detected due to infective endocarditis. Only Bentall's procedure was performed in 3 patients whose MR were minimal. There were no early death, but two late deaths. One of them died of cardiac failure 2.3 years after Bentall's procedure because grade 2 MR was increased. Another one died from ventricular arrhythmia 1.6 years after MVR and Bentall's procedure. The remaining 5 patients are doing well for 3 months to 11.5 years after operation. For Marfan's syndrome combined with AAE and MR, early operation is recommended before left ventricular impairment. Mitral valve repair was not performed, both to save time and because anticoagulant therapy was need for aortic valve replacement. Concomitant MVR was to be done for moderate to severe MR.
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