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 A, Zareba Wojciech
Division of Cardiology, University of Florida College of Medicine, Jacksonville, Florida, USA.
Division of Cardiology, University of Colorado School of Medicine, Aurora, Colorado, USA.
J Cardiovasc Electrophysiol. 2016 May;27(5):555-62. doi: 10.1111/jce.12947. Epub 2016 Mar 21.
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.
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.
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).
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.
遗传性心律失常的临床表现和预后存在性别差异，这一现象已被广泛报道。我们旨在比较北美致心律失常性右室心肌病（ARVC）注册研究中男性和女性患者的临床表现及预后情况。
共纳入125例被诊断为“患病”或“临界”状态的ARVC先证者（55例女性，平均年龄38±12岁；70例男性，平均年龄41±15岁）。比较男性和女性患者的基线临床特征以及包括晕厥、室性心动过速（VT）、快速VT（>240次/分钟）、心室颤动（VF）和死亡在内的时间依赖性预后情况。
男性和女性中被诊断为“患病”（84%对89%；P = 0.424）或“临界”（16%对11%；P = 0.424）的ARVC患者比例相似。在基线特征方面，V2导联T波倒置在女性中更为常见（P = 0.09），而异常信号平均心电图（SAECG；P < 0.001）和可诱发性VT/VF（P = 0.026）在男性中更为频繁。在平均37±20个月的随访期间，男性和女性患者经植入式心律转复除颤器（ICD）记录的VT/VF或死亡概率无显著差异（P = 0.456）。然而，与男性相比，女性发生快速VT/VF或死亡的风险有降低趋势（风险比0.41，95%可信区间0.151 - 1.113，P = 0.066）。异常SAECG以及心脏磁共振成像显示的心肌内脂肪与男性的不良预后相关（分别为P = 0.006和P = 0.02）。
在北美ARVC注册研究中，我们发现男性和女性中“患病”和“临界”患者的比例相似。在基线心电图、SAECG、动态心电图记录的室性心律失常以及VT可诱发性方面观察到了性别相关差异。男性发生快速VT的风险比女性有增加趋势。
