Hiromasa Suzuki, MD; Masataka Sumiyoshi, MD; Sachio Kawai, MD; Atsutoshi Takagi, MD; Akiko Wada, MD; Yuji Nakazato, MD; Hiroyuki Daida, MD; Hidehiko Sakurai, MD; Hiroshi Yamaguchi, MD
Abstract
A case of arrhythmogenic right ventricular cardiomyopathy (ARVC) with an initial manifestation of severe
impairment of the left ventricle (LV) and normal contraction of the right ventricle (RV) is presented. A 43-year-old
man was admitted to hospital because of congestive heart failure following a common cold. The LV function
was diffusely and severely hypokinetic. Coronary arteriogram revealed normal vessels. An endomyocardial
biopsy specimen obtained from the RV septum revealed mild infiltration of lymphocytes with focal myocytes
necrosis and so healing myocarditis was suspected. The specimen did not include any fatty replacement of
myocytes. Since then, the patient suffered from recurrent congestive heart failure as well as nonsustained
ventricular tachycardia and required frequent hospitalization. Progressive impairment, dilation, and thinning of
both ventricles were observed on serial echocardiographic examinations. Although the RV gradually enlarged
and became impaired, severe dilatation and impairment of the LV has always been predominant in the patient's
clinical course. After medical follow-up for 10 years, he died suddenly of ventricular fibrillation and pump
failure. The autopsy revealed extensive fibrofatty replacement of myocytes in both the ventricles, extending from
the outer layer to the inner layer of myocardium in the RV and to the middle layer in the LV. These features were
compatible with arrhythmogenic right ventricular cardiomyopathy or perimyocarditis, although only the right-sided
bundle of the interventricular septum was completely replaced by fatty tissue, which can not be explained
as a sequel of perimyocarditis. Moreover, apoptosis was present in the myocyte nuclei of the myocardial layers
bordering the area of fatty replacement. Therefore, myocarditis may have triggered or accelerated the process of
apoptosis leading to ARVC.
(Jpn Circ J 2000; 64: 209-213)
Key Words: Apoptosis; Arrhythmogenic right ventricular cardiomyopathy; Dilated cardiomyopathy; Myocarditis
Mailing address: Hiromasa Suzuki, MD, Department of Cardiology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan. E-mail: hisuzuki@med.juntendo.ac.jp