Kojima Katsumi, Kato Koichi, Fujii Yusuke, Okuyama Yusuke, Ohno Seiko, Ozawa Tomoya, Horie Minoru, Nakagawa Yoshihisa
Department of Cardiovascular Medicine, Shiga University of Medical Science, Japan.
Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Japan.
Intern Med. 2022 Apr 15;61(8):1179-1182. doi: 10.2169/internalmedicine.8093-21. Epub 2022 Feb 1.
We herein report a 14-year-old boy with repetitive nocturnal syncope related to medication-refractory long QT syndrome (LQTS). Although the use of an implantable cardioverter-defibrillator (ICD) was inevitable to prevent sudden cardiac death, he refused immediate implantation in order to play in a baseball competition six weeks away. Given his genetic diagnosis of type 2 LQTS, which is associated with cardiac events unrelated to exercise, we prescribed a wearable cardioverter defibrillator (WCD) to be donned at night, without limiting his exercise participation. An ICD was implanted after the competition. We successfully performed the preplanned treatment while maximizing the patient's quality-of-life with a WCD and genotype-specific risk stratification.
我们在此报告一名14岁男孩，患有与药物难治性长QT综合征（LQTS）相关的反复夜间晕厥。尽管为预防心源性猝死不可避免地要使用植入式心律转复除颤器（ICD），但他为了参加六周后的一场棒球比赛而拒绝立即植入。鉴于他的2型LQTS基因诊断结果，该型与非运动相关的心脏事件有关，我们给他开了一款夜间佩戴的可穿戴式心律转复除颤器（WCD），且不限制他参与运动。比赛结束后植入了ICD。我们成功实施了预先计划的治疗，同时通过WCD和基因型特异性风险分层使患者的生活质量最大化。
