短QT综合征患者血管外植入式心律转复除颤器植入成功一例报告

Q4 Medicine

Journal of Cardiology Cases Pub Date : 2026-10-01 Epub Date: 2026-06-16 DOI:10.1016/j.jccase.2026.06.002

Nobuhisa Kodera MD, Nobuhiro Nishii MD, PhD, Akira Ueoka MD, PhD, Koji Nakagawa MD, PhD, Hiroshi Morita MD, PhD, Shinsuke Yuasa MD, PhD, FJCC

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引用次数: 0

摘要

短QT综合征（SQTS）可出现危及生命的心室颤动（VF），需要及时复苏和二级预防。一名20多岁的男性患者从VF中复苏后被诊断为SQTS。基因检测可以发现罕见的变异，如SLC4A3，扩大了已知的SQTS遗传谱。高T波可能导致一些载体的皮下植入式心律转复除颤器（S-ICD）筛查失败，突出了S-ICD在某些SQTS患者中的局限性。当经静脉或皮下ICD不理想时，血管外ICD植入是SQTS患者可行且有效的选择。学习目标短QT综合征（SQTS）可出现危及生命的心室颤动，需要及时复苏和二级预防。基因检测可以发现罕见的变异，如SLC4A3，扩大了已知的SQTS遗传谱。高T波可能导致一些载体的皮下植入式心律转复除颤器（S-ICD）筛查失败，突出了S-ICD在某些SQTS患者中的局限性。当经静脉或皮下ICD不理想时，血管外ICD植入是SQTS患者可行且有效的选择。

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Successful extravascular implantable cardioverter-defibrillator implantation in a patient with short QT syndrome: A case report

Short QT syndrome (SQTS) can present with life-threatening ventricular fibrillation (VF), requiring timely resuscitation and secondary prevention. A male patient in his 20s was resuscitated from VF and diagnosed with SQTS. Genetic testing may identify rare variants, such as in SLC4A3, expanding the known genetic spectrum of SQTS. Tall T waves may cause subcutaneous implantable cardioverter-defibrillator (S-ICD) screening failure in some vectors, highlighting limitations of S-ICDs in certain SQTS patients. Extravascular ICD implantation can be a feasible and effective option for patients with SQTS when transvenous or subcutaneous ICDs are suboptimal.

Learning objectives

Short QT syndrome (SQTS) can present with life-threatening ventricular fibrillation, requiring timely resuscitation and secondary prevention.

Genetic testing may identify rare variants, such as in SLC4A3, expanding the known genetic spectrum of SQTS.

Tall T waves may cause subcutaneous implantable cardioverter-defibrillator (S-ICD) screening failure in some vectors, highlighting limitations of S-ICDs in certain SQTS patients.

Extravascular ICD implantation can be a feasible and effective option for patients with SQTS when transvenous or subcutaneous ICDs are suboptimal.

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