特発性心室性不整脈と心筋病変—電気生理学および心内膜心筋生検による病理組織学的検討

Electrophysiologic and histopathologic investigations in children with idiopathic ventricular arrhythmias

　内科領域では，最近基礎心疾患のない心室性不整脈のなかに失神発作，突然死，心不全を来たすものが見いだされており，危険な心室性不整脈としからざるものとの鑑別が大切になっている。それらのなかで，心内膜心筋生検（EMB）による積極的な検索により，いわゆる特発性心室性不整脈例に潜在性心筋疾患が見いだされるようになってきた、小児科領域では，このような検討の報告はなく，今回我々は心内精査で基礎心疾患の認められない，いわゆる特発性心室性不整脈のEMBを検討し若干の知見を得たので報告する。

Between April 1984 and December 1987, electro-physiological studies and endomyocardial biopsy were performed in 14 pediatric patients, aged from7 to 15 years, with idiopathic ventricular arrhythmias in whom diagnostic evaluation had revealed no struc-tural heart disease. They were 8 boys and 6 girls. Cardiac catheterization revealed regional wall moti-on abnormalities of the left ventricle in 3 patients, one of whom showed decreased ejection fraction (EF). Electrophysiologic examination showed sinus node dysfunction in 21%, AV nodal dysfunction in 14% and dual AV nodal pathway in 21%. Histopatho-logic examination by endomyocardial biopsy showedmyocellular hypertrophy, degeneration of myocytes, interstitial fibrosis and endomyocardial thickness in 86%, 36%, 35% and 14%, respectively.

Since idiopathic ventricular arrhythmias in pediat-ric age group included relatively high electrophy-siologic and histopathologic abnormalities, which were suggestive of occult myocardial disease and might be the early stage of cardiomyopathy, careful follow-up should be required.