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Arrhythmic outcome of arrhythmogenic right ventricular cardiomyopathy patients without implantable defibrillators
Author(s) -
Wang Weijia,
CadrinTourigny Julia,
Bhonsale Aditya,
Tichnell Crystal,
Murray Brittney,
Monfredi Oliver,
Chrispin Jonathan,
Crosson Jane,
Tandri Harikrishna,
James Cynthia A.,
Calkins Hugh
Publication year - 2018
Publication title -
journal of cardiovascular electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.193
H-Index - 138
eISSN - 1540-8167
pISSN - 1045-3873
DOI - 10.1111/jce.13668
Subject(s) - medicine , cardiology , interquartile range , sudden cardiac death , implantable cardioverter defibrillator , arrhythmogenic right ventricular dysplasia , proband , cardiomyopathy , heart failure , biochemistry , chemistry , mutation , gene
Abstract Background Implantable defibrillators (ICD) are an important therapy for arrhythmogenic right ventricular cardiomyopathy (ARVC) patients at high risk of sudden death. Given the high appropriate ICD therapy rate, some have argued that the mere act of implanting an ICD inflates the malignant arrhythmia rate in ARVC. Objective To report the arrhythmic course of ARVC patients without ICDs at the fulfillment of the 2010 Task Force Criteria and explore predictors of malignant ventricular arrhythmias. Methods We included 131 definite ARVC patients (age 32 ± 15 years, male 39%, proband 50%) either without ICDs (N = 47) or receiving an ICD at least 6 months after the fulfillment of the diagnostic criteria. The primary outcome was a composite of cardiac arrest (both resuscitated successfully and unsuccessfully) and sustained ventricular tachyarrhythmias (cycle length< 600 milliseconds, at least 30 seconds or requiring an intervention for termination). Results At the fulfillment of the diagnostic criteria, ICDs were not recommended to 59 (45%) patients and declined by 22 (17%) patients. Forty (31%) patients were not recognized as having ARVC by the treating physicians. Over 8 (interquartile interval: 3–12) years, 38 (29%) patients had primary outcomes (8 cardiac arrests [3 died] and 30 sustained ventricular arrhythmias) while not having ICDs. The 1‐year and 5‐year event‐free survival was 92% and 72%. Spontaneous sustained ventricular arrhythmias, cardiac syncope, men, proband, and inducibility in electrophysiology study were significantly associated with the primary outcome. Conclusion In a contemporary cohort, a considerable risk of malignant arrhythmias existed in ARVC when ICDs were not implanted.