Predicting arrhythmic risk in arrhythmogenic right ventricular cardiomyopathy: A systematic review and meta-analysis

Laurens P. Bosman, Arjan Sammani, Cynthia Anne James, Julia Cadrin-Tourigny, Hugh Calkins, J. Peter van Tintelen, Richard N.W. Hauer, Folkert W. Asselbergs, Anneline S.J.M. te Riele

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1097-1107
Number of pages11
JournalHeart Rhythm
Volume15
Issue number7
DOIs
StatePublished - Jul 1 2018

Fingerprint

Arrhythmogenic Right Ventricular Dysplasia
Meta-Analysis
Cardiac Arrhythmias
Syncope
Right Ventricular Dysfunction
Mutation
Population
Cohort Studies
Implantable Defibrillators
Electrophysiology
Sudden Cardiac Death
Left Ventricular Dysfunction
Ventricular Fibrillation
Ventricular Tachycardia
MEDLINE
Observational Studies
Publications

Keywords

  • Arrhythmogenic right ventricular cardiomyopathy
  • Arrhythmogenic right ventricular dysplasia/cardiomyopathy
  • Meta-analysis
  • Prognosis
  • Risk stratification
  • Sudden cardiac death
  • Systematic review
  • Ventricular arrhythmias

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Predicting arrhythmic risk in arrhythmogenic right ventricular cardiomyopathy : A systematic review and meta-analysis. / Bosman, Laurens P.; Sammani, Arjan; James, Cynthia Anne; Cadrin-Tourigny, Julia; Calkins, Hugh; van Tintelen, J. Peter; Hauer, Richard N.W.; Asselbergs, Folkert W.; te Riele, Anneline S.J.M.

In: Heart Rhythm, Vol. 15, No. 7, 01.07.2018, p. 1097-1107.

Research output: Contribution to journalArticle

Bosman, LP, Sammani, A, James, CA, Cadrin-Tourigny, J, Calkins, H, van Tintelen, JP, Hauer, RNW, Asselbergs, FW & te Riele, ASJM 2018, 'Predicting arrhythmic risk in arrhythmogenic right ventricular cardiomyopathy: A systematic review and meta-analysis', Heart Rhythm, vol. 15, no. 7, pp. 1097-1107. https://doi.org/10.1016/j.hrthm.2018.01.031
Bosman, Laurens P. ; Sammani, Arjan ; James, Cynthia Anne ; Cadrin-Tourigny, Julia ; Calkins, Hugh ; van Tintelen, J. Peter ; Hauer, Richard N.W. ; Asselbergs, Folkert W. ; te Riele, Anneline S.J.M. / Predicting arrhythmic risk in arrhythmogenic right ventricular cardiomyopathy : A systematic review and meta-analysis. In: Heart Rhythm. 2018 ; Vol. 15, No. 7. pp. 1097-1107.
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