Literature DB >> 26681611

Implantable cardioverter-defibrillator explantation for overdiagnosed or overtreated congenital long QT syndrome.

Prakriti Gaba1, J Martijn Bos2, Bryan C Cannon3, Yong-Mei Cha4, Paul A Friedman4, Samuel J Asirvatham4, Michael J Ackerman5.   

Abstract

BACKGROUND: Primary treatment of long QT syndrome (LQTS) currently consists of beta-blocker therapy, although an implantable cardioverter-defibrillator (ICD) is considered for high-risk patients. However, both overdiagnosis and overtreatment must be avoided because their sequelae can be significant.
OBJECTIVE: The purpose of this study was to evaluate the prevalence and details of ICD explants in a cohort of patients from a tertiary genetic heart rhythm clinic for a previously rendered diagnosis of LQTS.
METHODS: Overall, 1227 consecutive patients were included in the study. All patients had been referred to the Mayo Clinic for evaluation of possible LQTS and subsequently were either diagnosed with LQTS or dismissed as normal. Further stratification of patients was conducted to assess how many patients had an ICD and how many had a subsequent ICD explant.
RESULTS: In total, 170 patients (14%) had an ICD, including 157 of 670 patients (23%) with confirmed LQTS and 13 of 557 patients (2%) who did not have LQTS. Among these, 12 of 1227 (1%) had the ICD removed: 7 of 157 LQTS patients (4.5%) compared to 5 of 14 non-LQTS patients (36%). Before explant, 5 of 12 patients (42%) experienced inappropriate shocks, ranging from 2 to as many as 54 shocks. In addition, 4 had a device-related infection, and 9 had device malfunction (including lead dysfunction or fracture). None of these patients had a breakthrough cardiac event since removal of their ICD during 5.5 ± 3.5 years of follow-up.
CONCLUSION: Implications of overdiagnosis and overtreatment are profound because unnecessary ICD placement can be associated with infection, malfunction, inappropriate shocks, and subsequent anxiety.
Copyright © 2016 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

Entities:  

Keywords:  Defibrillation; Explantation; Implantable cardioverter-defibrillator; Inappropriate shock; Long QT syndrome

Mesh:

Year:  2015        PMID: 26681611     DOI: 10.1016/j.hrthm.2015.12.008

Source DB:  PubMed          Journal:  Heart Rhythm        ISSN: 1547-5271            Impact factor:   6.343


  10 in total

Review 1.  The role of sympathectomy in long QT syndrome.

Authors:  Achilleas Antonopoulos; David Lawrence; Davide Patrini; Marco Scarci; Robert George; Martin Hayward; Sofoklis Mitsos; Nikolaos Panagiotopoulos
Journal:  J Thorac Dis       Date:  2017-09       Impact factor: 2.895

2.  The Outcome of Long QT Syndrome, a Korean Single Center Study.

Authors:  Kyung Jin Ahn; Mi Kyoung Song; Sang Yun Lee; Ja Kyoung Yoon; Gi Beom Kim; Seil Oh; Eun Jung Bae
Journal:  Korean Circ J       Date:  2022-10       Impact factor: 3.101

3.  The Outcome of Long QT Syndrome: What is the Optimal Therapy?

Authors:  Ji-Eun Ban
Journal:  Korean Circ J       Date:  2022-10       Impact factor: 3.101

4.  GENESIS: Gene-Specific Machine Learning Models for Variants of Uncertain Significance Found in Catecholaminergic Polymorphic Ventricular Tachycardia and Long QT Syndrome-Associated Genes.

Authors:  Rachel L Draelos; Jordan E Ezekian; Farica Zhuang; Mary E Moya-Mendez; Zhushan Zhang; Michael B Rosamilia; Perathu K R Manivannan; Ricardo Henao; Andrew P Landstrom
Journal:  Circ Arrhythm Electrophysiol       Date:  2022-03-31

5.  Clinical and genetic characteristics and course of congenital long QT syndrome in children: A nine-year single-center experience.

Authors:  Yakup Ergül; Gülhan Tunca Şahin; Hasan Candaş Kafalı; Erkut Öztürk; Senem Özgür; Sertaç Haydin; Alper Güzeltaş
Journal:  Anatol J Cardiol       Date:  2021-04       Impact factor: 1.596

6.  Establishment of a Dedicated Inherited Cardiomyopathy Clinic: From Challenges to Improved Patients' Outcome.

Authors:  Emily Smith; Paul D Thompson; Carolyn Burke-Martindale; Adaya Weissler-Snir
Journal:  J Am Heart Assoc       Date:  2022-04-26       Impact factor: 6.106

Review 7.  Management of Patients with Long QT Syndrome.

Authors:  Yongkeun Cho
Journal:  Korean Circ J       Date:  2016-10-20       Impact factor: 3.243

8.  Adaptation and validation of the ACMG/AMP variant classification framework for MYH7-associated inherited cardiomyopathies: recommendations by ClinGen's Inherited Cardiomyopathy Expert Panel.

Authors:  Melissa A Kelly; Colleen Caleshu; Ana Morales; Jillian Buchan; Zena Wolf; Steven M Harrison; Stuart Cook; Mitchell W Dillon; John Garcia; Eden Haverfield; Jan D H Jongbloed; Daniela Macaya; Arjun Manrai; Kate Orland; Gabriele Richard; Katherine Spoonamore; Matthew Thomas; Kate Thomson; Lisa M Vincent; Roddy Walsh; Hugh Watkins; Nicola Whiffin; Jodie Ingles; J Peter van Tintelen; Christopher Semsarian; James S Ware; Ray Hershberger; Birgit Funke
Journal:  Genet Med       Date:  2018-01-04       Impact factor: 8.822

9.  A Unique Interplay of Multiple Predisposing Factors Culminating in a Catastrophic QT Prolongation.

Authors:  Vrinda Vyas; Alisha Khan; Gowthami Kanagalingam; Luna Bhatta
Journal:  Cureus       Date:  2020-04-21

Review 10.  Long QT Syndrome Type 2: Emerging Strategies for Correcting Class 2 KCNH2 (hERG) Mutations and Identifying New Patients.

Authors:  Makoto Ono; Don E Burgess; Elizabeth A Schroder; Claude S Elayi; Corey L Anderson; Craig T January; Bin Sun; Kalyan Immadisetty; Peter M Kekenes-Huskey; Brian P Delisle
Journal:  Biomolecules       Date:  2020-08-04
  10 in total

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