Romano-Ward症候群の1家系例—最近の文献的考察をふまえて

A case report on a family of Romano-Ward syndrome: deal with recent literatures

　遺伝性QT延長症候群とは，先天性心疾患，弁膜異常，心筋障害，電解質異常，脳血管障害，特殊薬剤の使用がないにもかかわらず，心電図上原因不明のQT延長があり，心室性頻拍による失神発作をくり返し，しばしば突然死に至る遺伝性疾患^1）である。同症候群は，先天性聾唖の有無および遺伝形式の差異により，Jervell—Lange-Nielson症候群^2）とRomano-Ward症候群^3,4）に分けられる。今回，我々は後者と思われる例でpropranol—olの内服により長期観察中の症例を経験したのでここに報告し，発生機序，治療に関する文献的考察も併せ行なった。

A case is reported of 22-year-old with syncopal attacks, ventricular tachycardia, and familial prolongation of QT interval. Hemodynamic, rentgenographic and chemical studies showed no abnormalities. Electroencephalogram and hearing activity are normal. The family history revealed 4 instance of QT interval prolongation over 4 generations. A diagnosis of Romano-Ward syndrome was established. Physical exercise made heart rate increased unsatisfactory. QT interval changed variously and often led to arrhythmias. She was treated successfuly with propranolol alone for past 2 years. The pathogenesis, treatment of the syndrome are discussed.