Subcutaneous Implantable Cardioverter‐Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Transatlantic Experience

Background Despite growing use of the subcutaneous implantable cardioverter‐defibrillator (S‐ICD), its clinical role in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients remains undefined. We aim to elucidate the cardiac phenotype, implant characteristics, and long‐term eff...

Full description

Bibliographic Details
Main Authors: Gabriela M. Orgeron, Aditya Bhonsale, Federico Migliore, Cynthia A. James, Crystal Tichnell, Brittney Murray, Emanuele Bertaglia, Julia Cadrin‐Tourigny, Pietro De Franceschi, Jane Crosson, Harikrishna Tandri, Domenico Corrado, Hugh Calkins
Format: Article
Language:English
Published: Wiley 2018-11-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.118.008782
_version_ 1811334130437193728
author Gabriela M. Orgeron
Aditya Bhonsale
Federico Migliore
Cynthia A. James
Crystal Tichnell
Brittney Murray
Emanuele Bertaglia
Julia Cadrin‐Tourigny
Pietro De Franceschi
Jane Crosson
Harikrishna Tandri
Domenico Corrado
Hugh Calkins
author_facet Gabriela M. Orgeron
Aditya Bhonsale
Federico Migliore
Cynthia A. James
Crystal Tichnell
Brittney Murray
Emanuele Bertaglia
Julia Cadrin‐Tourigny
Pietro De Franceschi
Jane Crosson
Harikrishna Tandri
Domenico Corrado
Hugh Calkins
author_sort Gabriela M. Orgeron
collection DOAJ
description Background Despite growing use of the subcutaneous implantable cardioverter‐defibrillator (S‐ICD), its clinical role in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients remains undefined. We aim to elucidate the cardiac phenotype, implant characteristics, and long‐term efficacy regarding appropriate therapy and complications in ARVC/D patients with an S‐ICD implant. Methods and Results A transatlantic cohort of ARVC/D patients who underwent S‐ICD implantation was analyzed for clinical characteristics, S‐ICD therapy, and long‐term outcome including device‐related complications. The cohort included 29 patients (52% male, 76% probands, 59% with ARVC/D‐associated mutation, 59% primary prevention [no prior sustained ventricular arrhythmias], and 45% first‐generation S‐ICD devices). At implant, all inducible patients (27/29) had conversion of induced ventricular fibrillation. Two patients (7%) had superficial infections of the incision site that were treated conservatively. Over a median follow‐up of 3.16 years (interquartile range: 2.21–4.51 years), all episodes (6 patients, 4% per year) of sustained ventricular arrhythmias were appropriately detected and treated. Six patients (21%) experienced 39 inappropriate shocks, with 3 requiring device explantation. Oversensing of noncardiac signal (n=4; especially myopotentials) and cardiac signal (n=4) was the most frequent etiology. No lead or device dislodgement, infection, skin erosion, or explantation related to need for antitachycardia pacing was noted. Conclusions S‐ICD can effectively treat both induced and spontaneous ventricular arrhythmias in patients with ARVC/D. The rate of inappropriate shocks, although considerable, is comparable to that in ARVC/D patients treated with transvenous ICDs. When they occurred, inappropriate shocks were primarily due to cardiac and, uniquely, noncardiac oversensing. We suggest potential strategies for minimizing inappropriate therapy.
first_indexed 2024-04-13T17:03:14Z
format Article
id doaj.art-60b59eb1fe334f93b49ff497fed2b17a
institution Directory Open Access Journal
issn 2047-9980
language English
last_indexed 2024-04-13T17:03:14Z
publishDate 2018-11-01
publisher Wiley
record_format Article
series Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
spelling doaj.art-60b59eb1fe334f93b49ff497fed2b17a2022-12-22T02:38:34ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802018-11-0172110.1161/JAHA.118.008782Subcutaneous Implantable Cardioverter‐Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Transatlantic ExperienceGabriela M. Orgeron0Aditya Bhonsale1Federico Migliore2Cynthia A. James3Crystal Tichnell4Brittney Murray5Emanuele Bertaglia6Julia Cadrin‐Tourigny7Pietro De Franceschi8Jane Crosson9Harikrishna Tandri10Domenico Corrado11Hugh Calkins12Division of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MDDivision of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MDDivision of Cardiology Department of Cardiac Thoracic and Vascular Sciences University of Padova ItalyDivision of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MDDivision of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MDDivision of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MDDivision of Cardiology Department of Cardiac Thoracic and Vascular Sciences University of Padova ItalyDivision of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MDDivision of Cardiology Department of Cardiac Thoracic and Vascular Sciences University of Padova ItalyDivision of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MDDivision of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MDDivision of Cardiology Department of Cardiac Thoracic and Vascular Sciences University of Padova ItalyDivision of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MDBackground Despite growing use of the subcutaneous implantable cardioverter‐defibrillator (S‐ICD), its clinical role in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients remains undefined. We aim to elucidate the cardiac phenotype, implant characteristics, and long‐term efficacy regarding appropriate therapy and complications in ARVC/D patients with an S‐ICD implant. Methods and Results A transatlantic cohort of ARVC/D patients who underwent S‐ICD implantation was analyzed for clinical characteristics, S‐ICD therapy, and long‐term outcome including device‐related complications. The cohort included 29 patients (52% male, 76% probands, 59% with ARVC/D‐associated mutation, 59% primary prevention [no prior sustained ventricular arrhythmias], and 45% first‐generation S‐ICD devices). At implant, all inducible patients (27/29) had conversion of induced ventricular fibrillation. Two patients (7%) had superficial infections of the incision site that were treated conservatively. Over a median follow‐up of 3.16 years (interquartile range: 2.21–4.51 years), all episodes (6 patients, 4% per year) of sustained ventricular arrhythmias were appropriately detected and treated. Six patients (21%) experienced 39 inappropriate shocks, with 3 requiring device explantation. Oversensing of noncardiac signal (n=4; especially myopotentials) and cardiac signal (n=4) was the most frequent etiology. No lead or device dislodgement, infection, skin erosion, or explantation related to need for antitachycardia pacing was noted. Conclusions S‐ICD can effectively treat both induced and spontaneous ventricular arrhythmias in patients with ARVC/D. The rate of inappropriate shocks, although considerable, is comparable to that in ARVC/D patients treated with transvenous ICDs. When they occurred, inappropriate shocks were primarily due to cardiac and, uniquely, noncardiac oversensing. We suggest potential strategies for minimizing inappropriate therapy.https://www.ahajournals.org/doi/10.1161/JAHA.118.008782arrhythmogenic right ventricular cardiomyopathyimplanted cardioverter defibrillatorlong‐term follow‐upventricular tachycardia
spellingShingle Gabriela M. Orgeron
Aditya Bhonsale
Federico Migliore
Cynthia A. James
Crystal Tichnell
Brittney Murray
Emanuele Bertaglia
Julia Cadrin‐Tourigny
Pietro De Franceschi
Jane Crosson
Harikrishna Tandri
Domenico Corrado
Hugh Calkins
Subcutaneous Implantable Cardioverter‐Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Transatlantic Experience
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
arrhythmogenic right ventricular cardiomyopathy
implanted cardioverter defibrillator
long‐term follow‐up
ventricular tachycardia
title Subcutaneous Implantable Cardioverter‐Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Transatlantic Experience
title_full Subcutaneous Implantable Cardioverter‐Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Transatlantic Experience
title_fullStr Subcutaneous Implantable Cardioverter‐Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Transatlantic Experience
title_full_unstemmed Subcutaneous Implantable Cardioverter‐Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Transatlantic Experience
title_short Subcutaneous Implantable Cardioverter‐Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Transatlantic Experience
title_sort subcutaneous implantable cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy dysplasia a transatlantic experience
topic arrhythmogenic right ventricular cardiomyopathy
implanted cardioverter defibrillator
long‐term follow‐up
ventricular tachycardia
url https://www.ahajournals.org/doi/10.1161/JAHA.118.008782
work_keys_str_mv AT gabrielamorgeron subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT adityabhonsale subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT federicomigliore subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT cynthiaajames subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT crystaltichnell subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT brittneymurray subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT emanuelebertaglia subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT juliacadrintourigny subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT pietrodefranceschi subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT janecrosson subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT harikrishnatandri subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT domenicocorrado subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience
AT hughcalkins subcutaneousimplantablecardioverterdefibrillatorinpatientswitharrhythmogenicrightventricularcardiomyopathydysplasiaatransatlanticexperience