First Department of Medicine, Faculty of Medicine, University Medical Centre Mannheim (UMM), University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167, Mannheim, Germany.
DZHK (GermanCenter for Cardiovascular Research), Partner Site, Heidelberg-Mannheim, Mannheim, Germany.
Clin Res Cardiol. 2019 Oct;108(10):1140-1146. doi: 10.1007/s00392-019-01449-3. Epub 2019 Mar 16.
Short QT syndrome (SQTS) is associated with sudden cardiac death and implantable cardioverter-defibrillator (ICD) implantation is recommended in this rare disease. However, only a few SQTS families have been reported in literature with limited follow-up data.
In the recent study, we describe the outcome data of 57 SQTS patients receiving ICD implantation. This includes seven SQTS families consecutively admitted to our hospital between 2002 and 2017 as well as patients reported in published literature.
Seven SQTS patients admitted to our hospital were followed up. Additionally, 7 studies out of a total of 626 researched articles were identified through systematic database search (PubMed, Web of Science, Cochrane Library, and Cinahl) and their data analyzed according to our model.
Complications during a median follow-up time of 67.4 months (IQR 6-162 months) were documented in 31 (54%) patients. Inappropriate shocks were seen in 33% due to T wave oversensing (8.7%), supraventricular tachycardia (19%), lead failure and fracture (21%). Further complications were infection (10%), battery depletion (7%) and psychological distress (3.5%). Appropriate shocks were documented in 19%. Three patients (5%) were treated with s-ICD due to recurrent complications of transvenous ICD.
ICD therapy is an effective therapy in SQTS patients. However, it is also associated with significant risk of device-related complications.
短 QT 综合征（SQTS）与心源性猝死相关，因此在这种罕见疾病中推荐植入植入式心律转复除颤器（ICD）。然而，仅有少数 SQTS 家族在文献中被报道，且随访数据有限。
在最近的研究中，我们描述了 57 例接受 ICD 植入的 SQTS 患者的结局数据。这包括 2002 年至 2017 年间连续收治于我院的 7 例 SQTS 家族患者以及文献报道的患者。
对我院收治的 7 例 SQTS 患者进行随访。此外，通过系统的数据库搜索（PubMed、Web of Science、Cochrane Library 和 Cinahl）共确定了 7 项研究（共 626 篇研究文章），并根据我们的模型对其数据进行分析。
在中位随访时间为 67.4 个月（IQR 6-162 个月）期间，记录了 31 例（54%）患者的并发症。由于 T 波过感知（8.7%）、室上性心动过速（19%）、导联故障和断裂（21%），有 33%的患者出现了不恰当的电击。其他并发症包括感染（10%）、电池耗尽（7%）和心理困扰（3.5%）。记录到 19%的恰当电击。由于经静脉 ICD 反复出现并发症，有 3 例（5%）患者接受了 s-ICD 治疗。
ICD 治疗是 SQTS 患者的有效治疗方法，但也存在显著的器械相关并发症风险。
