特発性拘束型心筋症の1症例

Abstract

Most cases of restrictive cardiomyopathy (RCM) are caused by cardiac amyloidosis, cardiac hemochromatosis and endocardial fibrosis as the sequela of tropical or nontropical hypereosinophilia or endocardial fibroelastosis. We present herein a case of idiopathic RCM diagnosed by echocardiogram, cardiac catheterization and myocardial biopsy. This patient was a 33-year-old woman. She had been suffering from dyspnea on effort, abdominal fullness and pretibial edema. Persistent atrial fibrillation was seen on electrocardiogram and increase of cardiotrachic ratio (58.6%) on chest X-ray was seen on admission. Echocardiogram revealed a decrease of left ventricular diastolic properties with no decrease of left ventricular ejction fraction. Cardiac catheterization revealed an early diastolic dip and plateau pattern and increased end-diastolic pressure of both ventricles. A myocardial biopsy from the right ventricular wall disclosed mild hypertrophy of myocytes, interstitial fibrosis and endocardial thickening without the deposition of amyloid and hemosiderin.