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Journal of Medical Ultrasonics

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1987 - Vol.14

Vol.14 No.04

Case Report(症例報告)

(0339 - 0344)


A Case of Cardiac Hemochromatosis

原 和弘1, 山口 徹1, 樫田 光夫1, 桑子 賢司1, 遠田 栄一2

Kazuhiro HARA1, Tetsu YAMAGUCHI1, Mitsuo KASIDA1, Kenji KUWAKO1, Eiichi TODA2

1三井記念病院循環器センター内科, 2三井記念病院中央検査部

1Center for Cardiovascular Disease, Mitsui Memorial Hospital, 2Central Clinical Laboratory, Mitsui Memorial Hospital

キーワード : Cardiac hemochromatosis, Echocardiography

Echocardiographic and histological studies in a rare case of cardiac hemochromatosis were presented. A 39-year-old woman with six-years history of pure red cell aplasia was admitted for palpitation in April, 1983. She had received more than 80000 ml of blood transfusions. Two-dimensional echocardiography (2DE) demonstrated slight dilatation of both ventricles; diastolic dimension of left ventricle (LVDd) was 54 mm and fractional shortening (FS) 22%. She died from congestive heart failure five months later. 2DE immediately before her death showed RV dilatation (RVD=28 mm), normal left ventricle (LVDd=48%, FS=27%) and the "patchy granular and high intensity echoes" in the basal IVS.
Histological studies showed diffuse iron deposition, vacuolar degeneration and minimal fibrosis in the myocardium. Iron deposition was found more markedly in the basal IVS than in the free ventricular wall. Therefore, this abnormal echoes were supposed to be due to iron deposition rather than fibrosis in this patient.
In addition to the depressed ventricular function previously reported, the "patchy, granular and high intensity echoes" in the basal IVS was considered to be a characteristic echocardiographic finding in advanced cardiac hemochromatosis. Moreover, RV dilatation might be one of the signs of poor prognosis in this condition.