Marfan's syndrome with annulo-aortic ectasia and ruptured mitral chorda--a case report of combined composite valve graft replacement of the aortic root and mitral valve replacement]

We report a case of Marfan's syndrome with acute heart failure caused by a ruptured mitral chorda that was successfully treated by one operation of combined composite valve graft replacement of aortic root and mitral valve replacement (MVR). A 23-year-old man was admitted to our hospital presenting severe dyspnea and chest pain. Echocardiography and cardiac catheterization studies demonstrated marked annulo-aortic ectasia, aortic regurgitation and significant mitral regurgitation due to a ruptured chorda. In operation, it was found that a chorda of the mitral posterior leaflet had been torn, with the leaflet completely prolapsed to the left atrium, and that the aortic root was dilated to 90 mm in diameter. The ascending aorta was extensively resected leaving those areas of aortic tissue involving the coronary ostia. Then the mobilized coronary arteries were reattached to the composite graft. MVR was performed with preservation of the whole anterior and posterior mitral valve apparatus except for that small part with the torn chorda. Histopathological findings of the aortic wall and mitral valve were compatible with those of Marfan's syndrome.