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Abstract

第14巻 2018年2月 第1号

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■症例報告

生後45日目に突然死をきたした2:1房室ブロックを伴う先天性QT延長症候群の1例
築野 香苗, 深澤 隆治, 橋本 佳亮, 橋本 康司, 伊藤 保彦
日本医科大学付属病院小児科

Congenital Long QT Syndrome with a 2:1 Atrioventricular Block Resulting in Sudden Death
Kanae Tsuno, Ryuji Fukazawa, Yoshiaki Hashimoto, Koji Hashimoto and Yasuhiko Ito
Department of Pediatrics, Nippon Medical School Hospital

Longer QT intervals have been shown to induce cardiovascular events, including atrioventricular (AV) block, ventricular tachycardia, and torsades de pointes in long QT syndrome (LQTS). We report the case of a male neonate with 2:1 AV block and congenital LQTS who died suddenly 45 days after birth. He was the first of dizygotic twins, born full-term, and weighed 2,516 g. His pulse was below 90 bpm, and electrocardiogram showed a 2:1 AV block and long QT interval (QTc 0.59). There was no history of sudden death in his family. In addition, no structural cardiac anomalies were detected on ultrasonography imaging. His pulse would rise to 120 bpm when he cried, and his QT interval tended to be shorter. He was discharged on day 7 and was kept under observation without medication. At day 45, he had syncope and was brought immediately to our hospital. He was in a state of cardiopulmonary arrest and died despite resuscitation. Although implantable cardioverter defibrillators have been successfully used in patients at high risk for LQTS, their use is controversial in asymptomatic patients or in those without a family history of sudden death. We report a case of neonatal LQTS and provide a review of the literature.

日医大医会誌 2018; 14(1), 14-17

Key words
congenital long QT syndrome, atrioventricular block, sudden death

Correspondence to
Ryuji Fukazawa, Department of Pediatrics, Nippon Medical School Hospital, 1-1-5 Sendagi, Bunkyo-ku Tokyo 113-8603, Japan
E-mail:oraora@nms.ac.jp

受付:2017年10月10日 受理:2018年1月17日

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