Clinical Presentation and Outcomes by Sex in Arrhythmogenic Right Ventricular Cardiomyopathy: Findings from the North American ARVC Registry

J Cardiovasc Electrophysiol. 2016 May;27(5):555-62. doi: 10.1111/jce.12947. Epub 2016 Mar 21.

Abstract

Background: Sex differences in clinical presentation and outcomes of hereditary arrhythmias are commonly reported. We aimed to compare clinical presentation and outcomes in men and women with arrhythmogenic right ventricular cardiomyopathy (ARVC) enrolled in the North American ARVC Registry.

Methods: A total of 125 ARVC probands (55 females, mean age 38 ± 12; 70 males, mean age 41 ± 15) diagnosed, as either "affected" or "borderline" were included. Baseline clinical characteristics and time-dependent outcomes including syncope, ventricular tachycardia (VT), fast VT (>240 bpm), ventricular fibrillation (VF), and death were compared between males and females.

Results: The percentage of ARVC subjects diagnosed as "affected" (84% vs. 89%; P = 0.424) or "borderline" (16% vs. 11%; P = 0.424) was similar between females and males. Among the baseline characteristics, inverted T-waves in V2 trended to be more common in women (P = 0.09), whereas abnormal signal-averaged ECGs (SAECGs; P < 0.001) and inducible VT/VF (P = 0.026) were more frequent in men. During a mean follow-up of 37 ± 20 months, the probability of ICD-recorded VT/VF or death was not significantly different between men and women (P = 0.456). However, there was a trend toward lower risk of fast VT/VF or death in women compared to men (hazard ratio 0.41, 95% CI 0.151-1.113, P = 0.066). Abnormal SAECG and evidence of intramyocardial fat by cardiac MRI was associated with adverse outcomes in men (P = 0.006 and 0.02 respectively).

Conclusion: In the North American ARVC Registry, we found similar frequency of "affected" and "borderline" subjects between men and women. Sex-related differences were observed in baseline ECG, SAECG, Holter-recorded ventricular arrhythmias, and VT inducibility. Men showed a trend toward greater risk of fast VT than women.

Keywords: arrhythmogenic right ventricular cardiomyopathy, gender differences; clinical outcomes.

Publication types

  • Comparative Study
  • Multicenter Study

MeSH terms

  • Adult
  • Arrhythmogenic Right Ventricular Dysplasia / diagnosis
  • Arrhythmogenic Right Ventricular Dysplasia / epidemiology*
  • Arrhythmogenic Right Ventricular Dysplasia / genetics
  • Arrhythmogenic Right Ventricular Dysplasia / mortality
  • Biopsy
  • DNA Mutational Analysis
  • Electrocardiography
  • Female
  • Genetic Predisposition to Disease
  • Health Status Disparities*
  • Humans
  • Kaplan-Meier Estimate
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Mutation
  • North America / epidemiology
  • Phenotype
  • Proportional Hazards Models
  • Registries
  • Risk Factors
  • Sex Factors
  • Syncope / diagnosis
  • Syncope / epidemiology*
  • Syncope / genetics
  • Syncope / mortality
  • Tachycardia, Ventricular / diagnosis
  • Tachycardia, Ventricular / epidemiology*
  • Tachycardia, Ventricular / genetics
  • Tachycardia, Ventricular / mortality
  • Time Factors
  • Ventricular Fibrillation / diagnosis
  • Ventricular Fibrillation / epidemiology*
  • Ventricular Fibrillation / genetics
  • Ventricular Fibrillation / mortality