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Long-term follow-up of implantable cardioverter-defibrillators in Short QT syndrome

  • Ibrahim El-BattrawyEmail author
  • Johanna Besler
  • Uzair Ansari
  • Volker Liebe
  • Rainer Schimpf
  • Erol Tülümen
  • Boris Rudic
  • Siegfried Lang
  • Katja Odening
  • Lukas Cyganek
  • Christian Wolpert
  • Xiaobo Zhou
  • Martin Borggrefe
  • Ibrahim Akin
Original Paper
  • 4 Downloads

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.

Keywords

Short QT syndrome Sudden cardiac death ICD-related complications 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no competing interests.

Supplementary material

392_2019_1449_MOESM1_ESM.docx (16 kb)
Supplementary material 1 (DOCX 15 KB)

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Ibrahim El-Battrawy
    • 1
    • 2
    Email author
  • Johanna Besler
    • 1
  • Uzair Ansari
    • 1
  • Volker Liebe
    • 1
  • Rainer Schimpf
    • 1
  • Erol Tülümen
    • 1
  • Boris Rudic
    • 1
  • Siegfried Lang
    • 1
    • 2
  • Katja Odening
    • 3
  • Lukas Cyganek
    • 4
  • Christian Wolpert
    • 1
  • Xiaobo Zhou
    • 1
    • 2
  • Martin Borggrefe
    • 1
    • 2
  • Ibrahim Akin
    • 1
    • 2
  1. 1.First Department of Medicine, Faculty of Medicine, University Medical Centre Mannheim (UMM)University of HeidelbergMannheimGermany
  2. 2.DZHK (GermanCenter for Cardiovascular Research), Partner Site, Heidelberg-MannheimMannheimGermany
  3. 3.Faculty of MedicineUniversity of FreiburgFreiburgGermany
  4. 4.Stem Cell Unit, Clinic for Cardiology and PneumologyUniversity Medical Center GöttingenGöttingenGermany

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