El-Battrawy Ibrahim, Erath Julia W, Vamos Mate, Aweimer Assem, Mügge Andreas, Lang Siegfried, Ansari Uzair, Gietzen Thorsten, Akin Ibrahim
First Department of Medicine, Faculty of Medicine, University Medical Centre Mannheim (UMM), University of Heidelberg, 68167 Mannheim, Germany.
DZHK (German Center for Cardiovascular Research), Partner Site, 69120 Heidelberg, Germany.
J Cardiovasc Dev Dis. 2022 Mar 9;9(3):79. doi: 10.3390/jcdd9030079.
The pathophysiology of Takotsubo Syndrome (TTS) is not completely understood and the trigger of sudden cardiac death (SCD) in TTS is not clear either. We therefore sought to find an association between TTS and primary electrical diseases. A total of 148 TTS patients were analyzed between 2003 and 2017 in a bi-centric manner. Additionally, a literature review was performed. The patients were included in an ongoing retrospective cohort database. The coexistence of TTS and primary electrical diseases was confirmed in five cases as the following: catecholaminergic polymorphic ventricular tachycardia (CPVT, 18-year-old female) ( = 1), LQTS 1 (72-year-old female and 65-year-old female) ( = 2), LQTS 2 (17-year-old female) ( = 1), and LQTS in the absence of mutations (22-year-old female). Four patients suffered from malignant tachyarrhythmia and recurrent syncope after TTS. Except for the CPVT patient and one LQTS 1 patient, all other cases underwent subcutaneous ICD implantation. An event recorder of the CPVT patient after starting beta-blocker did not detect arrhythmias. The diagnosis of primary electrical disease was in 80% of cases unmasked on a TTS event. This diagnosis triggered a family clinical and genetic screening confirming the diagnosis of primary electrical disease. A subsequent literature review identified five cases as the following: a congenital atrioventricular block ( = 1), a Jervell and Lange-Nielsen Syndrome ( = 1), and a family LQTS in the absence of a mutation ( = 2), LQTS 2 ( = 1). A primary electrical disease should be suspected in young and old TTS patients with a family history of sudden cardiac death. In suspected cases, e.g., ongoing QT interval prolongation, despite recovery of left ventricular ejection fraction a family screening is recommended.
应激性心肌病（TTS）的病理生理学尚未完全明确，TTS 患者心源性猝死（SCD）的触发因素也不清楚。因此，我们试图寻找 TTS 与原发性心电疾病之间的关联。2003 年至 2017 年期间，以双中心方式对 148 例 TTS 患者进行了分析。此外，还进行了文献综述。这些患者被纳入一个正在进行的回顾性队列数据库。TTS 与原发性心电疾病共存的情况在 5 例中得到证实，具体如下：儿茶酚胺能多形性室性心动过速（CPVT，18 岁女性）（=1）、长 QT 综合征 1 型（LQTS 1，72 岁女性和 65 岁女性）（=2）、LQTS 2 型（17 岁女性）（=1），以及无突变的 LQTS（22 岁女性）。4 例患者在 TTS 后出现恶性心律失常和反复晕厥。除 CPVT 患者和 1 例 LQTS 1 患者外，所有其他病例均接受了皮下植入式心律转复除颤器（ICD）植入。CPVT 患者开始使用β受体阻滞剂后，事件记录仪未检测到心律失常。80%的原发性心电疾病病例诊断是在 TTS 事件中被揭示的。这一诊断引发了家庭临床和基因筛查以确诊原发性心电疾病。随后的文献综述确定了如下 5 例：先天性房室传导阻滞（=1）、耶尔韦尔和朗格 - 尼尔森综合征（=1），以及无突变的家族性 LQTS（=2）、LQTS 2 型（=1）。对于有心脏性猝死家族史的年轻和老年 TTS 患者，应怀疑存在原发性心电疾病。在疑似病例中，例如尽管左心室射血分数已恢复，但仍存在持续性 QT 间期延长的情况，建议进行家族筛查。
