Clinical Presentation and Outcomes by Sex in Arrhythmogenic Right Ventricular Cardiomyopathy: Findings from the North American ARVC Registry.
Naila Choudhary, Christine Tompkins, Bronislava Polonsky, Scott McNitt, Hugh Calkins, N A Mark Estes, Andrew D Krahn, Mark S Link, Frank I Marcus, Jeffrey A Towbin, Wojciech Zareba
Author Information
Naila Choudhary: Division of Cardiology, University of Florida College of Medicine, Jacksonville, Florida, USA.
Christine Tompkins: Division of Cardiology, University of Colorado School of Medicine, Aurora, Colorado, USA.
Bronislava Polonsky: Division of Cardiology, University of Rochester School of Medicine, Rochester, New York, USA.
Scott McNitt: Division of Cardiology, University of Rochester School of Medicine, Rochester, New York, USA.
Hugh Calkins: Division of Cardiology, John Hopkins University School of Medicine, Baltimore, Maryland, USA.
N A Mark Estes: Division of Cardiology, Tufts University School of Medicine, Boston, Massachusetts, USA.
Andrew D Krahn: Division of Cardiology, University of British Columbia, Vancouver, British Columbia, Canada.
Mark S Link: Division of Cardiology, Tufts University School of Medicine, Boston, Massachusetts, USA.
Frank I Marcus: Division of Cardiology, University of Arizona College of Medicine, Tucson, Arizona, USA.
Jeffrey A Towbin: Division of Pediatrics, University of Tennesse, Memphis, Tennessee, USA.
Wojciech Zareba: Division of Cardiology, University of Rochester School of Medicine, Rochester, New York, USA.
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.
