モヤモヤ病に，洞不全症候群，肥大型心筋症，右冠動脈狭窄を合併した一例

A case of Moyamoya disease associated with the stenosis of the right coronary artery, sick sinus syndrome and hypertrophic cardiomyopathy

　モヤモヤ病は，1963年，工藤ら^1），鈴木ら^2）によって報告され，日本人に多発し脳血管写上きわめて特異な像を呈する疾患である。

　近年，モヤモヤ病と腎血管性高血圧症^3,4）・大動脈炎症候群^5）・原発性肺高血圧症^6）などの頭蓋外動脈病変との合併例が数多く報告されており，また，モヤモヤ病の剖検例で，内頸動脈と同様の細胞線維性あるいは線維性の内膜肥厚を，腎動脈・冠動脈に認めた報告^7,8）もある。

A case of Moyamoya disease is reported. The patient, 14-year-old boy, was diagnosed as Moya-moya disease in his 6 years of age on the cerebral angiogram performed because of headache fre-quently occurring after traffic accident. He was referred to our clinic for the close examination of his ECG abnormalities which revealed sinus brady-cardia and sinus arrest for 3.08 sec. On admission to our clinic, his physical examination was noncon-tributory. On echocardiogram both of the intraven-tricular septum and the posterior wall of the left ventricle were found to be thickened. Cardiac catherterization described slightly increased right ventricular end-diastolic pressure. Overdrive sup-pression test showed a marked prolongation of the corrected sinus node recovery time (1,213 msec). The left ventriculogram revealed the narrowing of the left ventricular apical space during the systolic phase and the coronary angiography showed local-ized 50% stenosis at segment 1 of the right coronary artery. The histology of biopsy specimens of the right ventricle showed hypertrophy and irregular arrangement of the myocardial muscle fiber. The diagnosis was made, with all these cardiologic ex-amination taken into consideration, as sick sinus syndrome, hypertrophic cardiomyopathy and local-ized stenosis of the right coronary artery.

In Moyamoya disease, several portions of extra-cranial arteries have been found to be involved, but exclusively on autopsy findings, and no papers have been so far presented which confirmed the coronary involvement on coronary angiogram. The present case indicates that we need to do check-up examination for extracranial vascular system including the heart in Moyamoya disease.