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 Towbin, Wojciech Zareba

Research output: Contribution to journalArticle

10 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)555-562
Number of pages8
JournalJournal of cardiovascular electrophysiology
Volume27
Issue number5
DOIs
StatePublished - May 1 2016

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Arrhythmogenic Right Ventricular Dysplasia
Ventricular Tachycardia
Registries
Ventricular Fibrillation
Sex Characteristics
Cardiac Arrhythmias
Electrocardiography
Syncope
Fats

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Clinical Presentation and Outcomes by Sex in Arrhythmogenic Right Ventricular Cardiomyopathy : Findings from the North American ARVC Registry. / Choudhary, Naila; Tompkins, Christine; Polonsky, Bronislava; McNitt, Scott; Calkins, Hugh; Mark Estes, N. A.; Krahn, Andrew D.; Link, Mark S.; Marcus, Frank I.; Towbin, Jeffrey; Zareba, Wojciech.

In: Journal of cardiovascular electrophysiology, Vol. 27, No. 5, 01.05.2016, p. 555-562.

Research output: Contribution to journalArticle

Choudhary, N, Tompkins, C, Polonsky, B, McNitt, S, Calkins, H, Mark Estes, NA, Krahn, AD, Link, MS, Marcus, FI, Towbin, J & Zareba, W 2016, 'Clinical Presentation and Outcomes by Sex in Arrhythmogenic Right Ventricular Cardiomyopathy: Findings from the North American ARVC Registry', Journal of cardiovascular electrophysiology, vol. 27, no. 5, pp. 555-562. https://doi.org/10.1111/jce.12947
Choudhary, Naila ; Tompkins, Christine ; Polonsky, Bronislava ; McNitt, Scott ; Calkins, Hugh ; Mark Estes, N. A. ; Krahn, Andrew D. ; Link, Mark S. ; Marcus, Frank I. ; Towbin, Jeffrey ; Zareba, Wojciech. / Clinical Presentation and Outcomes by Sex in Arrhythmogenic Right Ventricular Cardiomyopathy : Findings from the North American ARVC Registry. In: Journal of cardiovascular electrophysiology. 2016 ; Vol. 27, No. 5. pp. 555-562.
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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.",
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T1 - Clinical Presentation and Outcomes by Sex in Arrhythmogenic Right Ventricular Cardiomyopathy

T2 - Findings from the North American ARVC Registry

AU - Choudhary, Naila

AU - Tompkins, Christine

AU - Polonsky, Bronislava

AU - McNitt, Scott

AU - Calkins, Hugh

AU - Mark Estes, N. A.

AU - Krahn, Andrew D.

AU - Link, Mark S.

AU - Marcus, Frank I.

AU - Towbin, Jeffrey

AU - Zareba, Wojciech

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N2 - 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.

AB - 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.

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