著明なHegglin症候群を呈した褐色細胞腫の1治験例

A marked Hegglin syndrome in pheochromocytoma

　Hegglin症候群とは，心音図II音主節の開始が心電図T波の終末部よりも早期に生じる心音図上の病態異常1）で，II音主節の開始が心電図T波の終末部より40msec以上早期に記録される場合をいう。本症候群は，全身的な代謝障害（電解質・糖質代謝の異常，脱水状態），感染性・中毒状態（リウマチ性多発性関節炎，各種中毒，肝不全，ポルフィリン症）など，ことに甲状腺機能亢進で高頻度に発現するが^1〜5），褐色細胞腫にHegglin症候群を合併したとする報告はみあたらない。今回我々は，褐色細胞腫で著明なHegglin症候群を呈し，腫瘍剔出後早期にそれが正常化した1治験例について報告する。

A 46-year-old man was hospitalized with paroxys-mal headache and chest discomfort. His blood pres-sure varied, occasionally being up to 300/160 mmHg. Cardiac examination revealed a decrescendo type of diastolic murmur (aortic regurgitation) and S₄ gal-lop. Both blood and urine catecholamine levels were extremely high. Electrocardiogram and echocardiogr-am showed severe left ventricular hypertrophy pattern presenting hypertrophic cardiomyopathic changes. The phonocardiogram showed marked Heg-glin syndrome (QT ; 450 msec and QII ; 310 msec). Right adrenal tumor, pheochromocytoma was found on the abdominal CT scanning and ¹³¹I-metaiodo-benzylguanidine (MIBG). In the post-operative pe-riod, Hegglin syndrome was completely abolished (QT ; 360 msec and QII ; 345 msec,), and also elect-rocardiographic left ventricular hypertrophy pat-tern improved immediately after surgical removal of the pheochromocytoma.