Ibrahim El-Battrawy1,2, Johanna Besler3, Uzair Ansari3, Volker Liebe3, Rainer Schimpf3, Erol Tülümen3, Boris Rudic3, Siegfried Lang3,4, Katja Odening5, Lukas Cyganek6, Christian Wolpert3, Xiaobo Zhou3,4, Martin Borggrefe3,4, Ibrahim Akin3,4. 1. First Department of Medicine, Faculty of Medicine, University Medical Centre Mannheim (UMM), University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167, Mannheim, Germany. Ibrahim.el-battrawy@umm.de. 2. DZHK (GermanCenter for Cardiovascular Research), Partner Site, Heidelberg-Mannheim, Mannheim, Germany. Ibrahim.el-battrawy@umm.de. 3. First Department of Medicine, Faculty of Medicine, University Medical Centre Mannheim (UMM), University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167, Mannheim, Germany. 4. DZHK (GermanCenter for Cardiovascular Research), Partner Site, Heidelberg-Mannheim, Mannheim, Germany. 5. Faculty of Medicine, University of Freiburg, Freiburg, Germany. 6. Stem Cell Unit, Clinic for Cardiology and Pneumology, University Medical Center Göttingen, Göttingen, Germany.
Abstract
BACKGROUND: 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. OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSION: ICD therapy is an effective therapy in SQTS patients. However, it is also associated with significant risk of device-related complications.
BACKGROUND: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. OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSION:ICD therapy is an effective therapy in SQTS patients. However, it is also associated with significant risk of device-related complications.
Entities:
Keywords:
ICD-related complications; Short QT syndrome; Sudden cardiac death
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