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

Abstract

Clinical Presentation and Outcomes 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.

Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy

“…Based on clinical experience and the current literature, particularly a recent published meta-analysis 13 and a prognostic model for predicting incident sustained VA in patients with ARVC, 2 8 potential predictors were preselected and recorded at the time of diagnosis. 2 , 11 – 15 These were sex, age at diagnosis, recent (<6 months) cardiac syncope, number of premature ventricular complexes (PVCs) on 24-hour Holter monitoring, prior sustained VA events, number of anterior and inferior leads with T-wave inversion (TWI), and left and right ventricular ejection fraction (RVEF). The definitions for these predictor variables are presented in Table II in the Data Supplement .…”

Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy

“…Men with ARVC are at higher arrhythmia risk than women 14, 41, 51–54 . While the specific pathophysiologic basis for this observation is unclear it has been suggested that this may be due to a more frequent and intense exercise in men.…”

Arrhythmogenic Right Ventricular Cardiomyopathy: From Pathophysiology to Diagnosis and Advances in Management

“…13,20,23,24,[62][63][64][65][66] The prognostic value of programmed ventricular stimulation in asymptomatic patients with ARVC remains unclear, but invasive evaluation of myocardial scar and fragmented, delayed electrograms may provide incremental information regarding SCD risk. 63,64,67 ICD therapy reduces SCD in high-risk patients with ARVC, but ICD implantation carries risks of complication, particularly lead-related adverse events and inappropriate shocks in younger patients.…”