Author + information
- Received March 7, 2018
- Revision received March 19, 2018
- Accepted April 26, 2018
- Published online June 18, 2018.
- Carole Maupain, MDa,b,
- Nicolas Badenco, MDa,
- Françoise Pousset, MDa,b,
- Xavier Waintraub, MDa,
- Guillaume Duthoit, MDa,
- Thomas Chastre, MDa,
- Caroline Himbert, MDa,
- Jean-Louis Hébert, MD, PhDa,
- Robert Frank, MDa,
- Françoise Hidden-Lucet, MDa,b and
- Estelle Gandjbakhch, MD, PhDa,b,c,∗ ()
- aAP-HP, Groupe Hospitalier Pitié-Salpêtrière, L’institut de Cardiométabolisme et Nutrition, Département de Cardiologie, Paris, France
- bCentre de Référence des Maladies Cardiaques Héréditaires, Paris, France
- cSorbonne Universités, UPMC Univ Paris 06, Paris, France
- ↵∗Address for correspondence:
Dr. Estelle Gandjbakhch, Institut de Cardiologie, APHP, Hôpital Pitié-Salpêtrière, 45-87 boulevard de l’hôpital, 75013 Paris, France.
Objectives The purpose of this study was to identify clinical factors associated with arrhythmic events and sudden cardiac death (SCD), and to evaluate the prognostic value of electrophysiological study (EPS) in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients without implantable cardioverter-defibrillators (ICDs).
Background ARVC/D is an inherited cardiomyopathy characterized by a risk of SCD. Few studies have evaluated predictive factors of ventricular arrhythmias (VAs) in patients without ICDs.
Methods Between 2000 and 2010, all consecutive patients with ARVC/D without ICDs and with EPS at diagnosis were enrolled. Patients that received an ICD during follow-up were censored at the date of implantation, and in that case, only VAs that occurred before ICD implantation were analyzed. Risk factors for any VA event were determined by Cox regression. Patients that only experienced SCD or aborted cardiac arrest (ACA) were reported.
Results A total of 137 consecutive patients (78% male) diagnosed with ARVC/D without ICD were enrolled. 31% had sustained ventricular tachycardia at diagnosis. After mean follow-up of 42 ± 31 months, 19 patients experienced an episode of sustained VA and 5 patients experienced a SCD/ACA. No event occurred in asymptomatic patients. Left ventricular ejection fraction ≤50% (p = 0.024), positive EPS (p = 0.017), and physical activity >6 h/week (p = 0.025) were independently associated with occurrence of VAs. SCD/ACA exclusively occurred in male probands with definite diagnosis and syncope.
Conclusions In this cohort of ARVC/D patients without ICD, left ventricular ejection fraction ≤50%, positive EPS, and physical activity >6 h/week were independent predictors of VAs, whereas asymptomatic patients at diagnosis were at low risk. EPS predicted all VAs but had limited value to predict SCD/ACA.
- arrhythmogenic right ventricular cardiomyopathy/dysplasia
- electrophysiological study
- risk stratification
- sudden cardiac death
- ventricular arrhythmia
This work was supported by grants from the “Fédération Française de Cardiologie”/“Société Française de Cardiologie” and “Ligue contre la Cardiomyopathie.” The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
All authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Clinical Electrophysiology author instructions page.
- Received March 7, 2018.
- Revision received March 19, 2018.
- Accepted April 26, 2018.
- 2018 American College of Cardiology Foundation
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