TY - JOUR
T1 - Subcutaneous implantable cardioverter-defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia
T2 - A transatlantic experience
AU - Orgeron, Gabriela M.
AU - Bhonsale, Aditya
AU - Migliore, Federico
AU - James, Cynthia A.
AU - Tichnell, Crystal
AU - Murray, Brittney
AU - Bertaglia, Emanuele
AU - Cadrin-Tourigny, Julia
AU - De Franceschi, Pietro
AU - Crosson, Jane
AU - Tandri, Harikrishna
AU - Corrado, Domenico
AU - Calkins, Hugh
N1 - Funding Information:
The authors wish to acknowledge funding from the Dr Francis P. Chiaramonte Private Foundation, the St. Jude Medical Foundation, and Boston Scientific Corp (for ICD studies only), and the Leducq Foundation-RHYTHM Network (all to Calkins). The Johns Hopkins arrhythmogenic right ventricular cardiomyopathy/dysplasia program is supported by the Leyla Erkan Family Fund for ARVD (arrhythmogenic right ventricular dysplasia) Research, the Dr Satish, Rupal, and Robin Shah ARVD Fund at Johns Hopkins, the Bogle Foundation, the Healing Hearts Foundation, the Campanella Family, the Patrick J. Harrison Family, the Peter French Memorial Foundation, and the Wilmerding Endowments.
Funding Information:
The Johns Hopkins arrhythmogenic right ventricular cardiomyopathy/dysplasia program receives research support from Boston Scientific. Dr Calkins has received honoraria for lectures from Boston Scientific and is a consultant to Medtronic. The remaining authors have no disclosures to report.
Funding Information:
The authors wish to acknowledge funding from the Dr Francis P. Chiaramonte Private Foundation, the St. Jude Medical Foundation, and Boston Scientific Corp (for ICD studies only), and the Leducq Foundation–RHYTHM Network (all to Calkins). The Johns Hopkins arrhythmogenic right ventricular cardiomyopathy/dysplasia program is supported by the Leyla Erkan Family Fund for ARVD (arrhythmogenic right ventricular dysplasia) Research, the Dr Satish, Rupal, and Robin Shah ARVD Fund at Johns Hopkins, the Bogle Foundation, the Healing Hearts Foundation, the Campanella Family, the Patrick J. Harrison Family, the Peter French Memorial Foundation, and the Wilmerding Endowments.
Publisher Copyright:
© 2018 The Authors.
PY - 2018/11/1
Y1 - 2018/11/1
N2 - 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 amedian 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.
AB - 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 amedian 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.
KW - Arrhythmogenic right ventricular cardiomyopathy
KW - Implanted cardioverter defibrillator
KW - Long-term follow-up
KW - Ventricular tachycardia
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U2 - 10.1161/JAHA.118.008782
DO - 10.1161/JAHA.118.008782
M3 - Article
C2 - 30608223
AN - SCOPUS:85056897653
VL - 7
JO - Journal of the American Heart Association
JF - Journal of the American Heart Association
SN - 2047-9980
IS - 21
M1 - e008782
ER -