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原発性心筋症に僧帽弁閉鎖不全を伴う場合があり,その機序として左室内腔の拡大や乳頭筋の形態または機能不全などがあげられている1,2)。
著者らは高度の僧帽弁閉鎖不全の所見を示し,剖検上,冠動脈および弁膜には異常なく,心内膜の線維性肥厚および心筋脱落,線維化等の病変を示した2症例を経験したので報告し,本症例の病理的位置を検討した。
Two autopsied cases of primary myocardial disease presenting severe mitral regurgitation was described. Case 1 was 58-year-old male, who was found to have cogestive heart failure at theage of 55. There was no history of rheumatic fever. The physical examination revealed the apical holosystolic murmur (Levine 4 grade) and the fourth sound. The electrocardiogram showed left ventricular hypertrophy and right ventricular loading. Ecg after three years demonstrated the presence of the intraventricular conduction defects. The cardiac catheterization and angio-cardiogram revealed severe mitral regurgitation and elevated endodiastolic pressure and poor con-traction of the left ventricle.
Case 2 was 33 year-old male, who was found to have congestive heart failure at the age of 30. There was family history in which the elder brother was treated on because of congestive heart failure with progressive muscular dystrophy and the younger brother suddenly died of con-gestive heart failure at 28. The patient passed away suddenly on working. The physical ex-amination demonstrated the holosystolic murmur at the apex and in the fourth and fifth intercostal space. The cardiac catheterization and ACG demonstrated the presence of severe mitral regurgitation and dilatation and elevated endo-diastolic pressure of the left ventricle.
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