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冠状動脈のfibromuscular dysplasia(FMD)様病変と拡張相肥大型心筋症を合併した1例を報告した.症例は心不全の悪化により死亡した37歳の男性で,剖検で両心室肥大と各心室心房の拡大を認めた(心重量:800g).組織学的には心室中隔を主体とした著明な線維化と残存心筋線維の錯綜配列を認めた.左右冠状動脈には本幹中心に平滑筋,弾性線維の増生を主体とした内膜肥厚による高度の狭窄を認めた.また,全身血管では腎動脈,外腸骨動脈,肺動脈,大動脈に冠状動脈と類似する病変を軽度に認めた.主冠状動脈のFMD様病変が肥大型心筋症から拡張型心筋症への移行の成因の一つになったものと考え,両者の関連などにつき考察した.
An autopsy case involving the dilated phase of hyper-trophic cardiomyopathy (HCM) is reported. This case was also complicated with a lesion similar to fibromus-cular dysplasia of the coronary artery. A 37 year-old male died of cardiac insufficiency and an autopsy was performed. Macroscopically, both atriums and ventri-cles were dilated and the walls of both ventricles were markedly thickened. Also, the coronary artery was almost completely obstructed. Histlological examina-tion revealed bizzare myocardial hyperplasia with dis-organization and marked fibrosis of the myocardium.
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