TY - JOUR
T1 - Predicting arrhythmic risk in arrhythmogenic right ventricular cardiomyopathy
T2 - A systematic review and meta-analysis
AU - Bosman, Laurens P.
AU - Sammani, Arjan
AU - James, Cynthia A.
AU - Cadrin-Tourigny, Julia
AU - Calkins, Hugh
AU - van Tintelen, J. Peter
AU - Hauer, Richard N.W.
AU - Asselbergs, Folkert W.
AU - te Riele, Anneline S.J.M.
N1 - Funding Information:
This work was supported by the Netherlands CardioVascular Research Initiative (grant nos. CVON2015-12 eDETECT and CVON2014-40 DOSIS). Dr te Riele was supported by the Dutch Heart Foundation (grant no. 2015T058) and the UMC Utrecht Fellowship Clinical Research Talent. Dr Calkins and Dr James were supported by the Dr Francis P. Chiaramonte Private Foundation, the Leducq Foundation – RHYTHM Network, the Netherlands Organisation for Scientific Research (NWO), the Leyla Erkan Family Fund for ARVD 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 Heart Rhythm Society
PY - 2018/7/1
Y1 - 2018/7/1
N2 - While many studies evaluate predictors of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC), a systematic review consolidating this evidence is currently lacking. Therefore, we searched MEDLINE and Embase for studies analyzing predictors of ventricular arrhythmias (sustained ventricular tachycardia/fibrillation (VT/VF), appropriate implantable cardioverter-defibrillator therapy, or sudden cardiac death) in patients with definite ARVC, patients with borderline ARVC, and ARVC-associated mutation carriers. In the case of multiple publications on the same cohort, the study with the largest population was included. This yielded 45 studies with a median cohort size of 70 patients (interquartile range 60 patients) and a median follow-up of 5.0 years (interquartile range 3.3 - 6.7 years). The average proportion of arrhythmic events observed was 10.6%/y in patients with definite ARVC, 10.0%/y in patients with borderline ARVC, and 3.7%/y with mutation carriers. Predictors of ventricular arrhythmias were population dependent: consistently predictive risk factors in patients with definite ARVC were male sex, syncope, T-wave inversion in lead >V3, right ventricular dysfunction, and prior (non)sustained VT/VF; in patients with borderline ARVC, 2 additional predictors—inducibility during electrophysiology study and strenuous exercise—were identified; and with mutation carriers, all aforementioned predictors as well as ventricular ectopy, multiple ARVC-related pathogenic mutations, left ventricular dysfunction, and palpitations/presyncope determined arrhythmic risk. Most evidence originated from small observational cohort studies, with a moderate quality of evidence. In conclusion, the average risk of ventricular arrhythmia ranged from 3.7 to 10.6%/y depending on the population with ARVC. Male sex, syncope, T-wave inversion in lead >V3, right ventricular dysfunction, and prior (non)sustained VT/VF consistently predict ventricular arrhythmias in all populations with ARVC.
AB - While many studies evaluate predictors of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC), a systematic review consolidating this evidence is currently lacking. Therefore, we searched MEDLINE and Embase for studies analyzing predictors of ventricular arrhythmias (sustained ventricular tachycardia/fibrillation (VT/VF), appropriate implantable cardioverter-defibrillator therapy, or sudden cardiac death) in patients with definite ARVC, patients with borderline ARVC, and ARVC-associated mutation carriers. In the case of multiple publications on the same cohort, the study with the largest population was included. This yielded 45 studies with a median cohort size of 70 patients (interquartile range 60 patients) and a median follow-up of 5.0 years (interquartile range 3.3 - 6.7 years). The average proportion of arrhythmic events observed was 10.6%/y in patients with definite ARVC, 10.0%/y in patients with borderline ARVC, and 3.7%/y with mutation carriers. Predictors of ventricular arrhythmias were population dependent: consistently predictive risk factors in patients with definite ARVC were male sex, syncope, T-wave inversion in lead >V3, right ventricular dysfunction, and prior (non)sustained VT/VF; in patients with borderline ARVC, 2 additional predictors—inducibility during electrophysiology study and strenuous exercise—were identified; and with mutation carriers, all aforementioned predictors as well as ventricular ectopy, multiple ARVC-related pathogenic mutations, left ventricular dysfunction, and palpitations/presyncope determined arrhythmic risk. Most evidence originated from small observational cohort studies, with a moderate quality of evidence. In conclusion, the average risk of ventricular arrhythmia ranged from 3.7 to 10.6%/y depending on the population with ARVC. Male sex, syncope, T-wave inversion in lead >V3, right ventricular dysfunction, and prior (non)sustained VT/VF consistently predict ventricular arrhythmias in all populations with ARVC.
KW - Arrhythmogenic right ventricular cardiomyopathy
KW - Arrhythmogenic right ventricular dysplasia/cardiomyopathy
KW - Meta-analysis
KW - Prognosis
KW - Risk stratification
KW - Sudden cardiac death
KW - Systematic review
KW - Ventricular arrhythmias
KW - Humans
KW - Risk Factors
KW - Arrhythmogenic Right Ventricular Dysplasia/diagnosis
KW - Electrocardiography
KW - Risk Assessment/methods
KW - Cardiac Resynchronization Therapy Devices
UR - http://www.scopus.com/inward/record.url?scp=85048805644&partnerID=8YFLogxK
U2 - 10.1016/j.hrthm.2018.01.031
DO - 10.1016/j.hrthm.2018.01.031
M3 - Review article
C2 - 29408436
SN - 1547-5271
VL - 15
SP - 1097
EP - 1107
JO - Heart Rhythm
JF - Heart Rhythm
IS - 7
ER -