Pearman Charles Michael, Lee David, Davies Brianna, Khan Habib, Tadros Rafik, Cadrin-Tourigny Julia, Roberts Jason D, Sanatani Shubhayan, Simpson Christopher, Angaran Paul, Hansom Simon, Ilhan Erkan, Seifer Colette, Green Martin, Gardner Martin, Talajic Mario, Laksman Zachary, Healey Jeff S, Krahn Andrew D
Center for Cardiovascular Innovation, Heart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, British Columbia; Unit of Cardiac Physiology, Division of Cardiovascular Sciences, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom.
Division of Cardiology, QEII Health Sciences Center, Halifax, Nova Scotia, Canada.
Heart Rhythm. 2023 Feb;20(2):224-230. doi: 10.1016/j.hrthm.2022.10.005. Epub 2022 Oct 14.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is currently diagnosed using a combination of clinical features, imaging, electrocardiography, and genetic investigations. An abnormal signal-averaged electrocardiogram (SAECG) is defined as a minor diagnostic criterion by the 2010 Task Force Criteria, but doubts remain about the value of this investigation.
We evaluated the utility of the SAECG in diagnosing ARVC using the Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry, a population representative registry of probands with ARVC and relatives, less influenced by referral bias.
Probands with ARVC and family members from the Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry underwent phenotype review. SAECG parameters were compared individually and in combination between those with varying degrees of ARVC severity and healthy controls (family members of probands with ARVC and unexplained sudden death, free of evidence of cardiac disease).
A total of 196 patients with ARVC and 205 controls were included (mean age 44 ± 15 years; 186 of 401 men [46%]). SAECG abnormalities were seen in 83 of 205 controls (40%), 33 of 68 patients with ARVC and mild disease (51%), and 31 of 42 with severe disease (74%). The SAECG associated strongly with imaging abnormalities (major: odds ratio 3.0, 95% confidence interval 1.3-6.9; minor: odds ratio 3.5, 95% confidence interval 0.7-16.5) but not with other aspects of phenotype. Patients carrying pathogenic variants but with minimal phenotype had similar SAECGs to healthy controls (filtered QRS duration 111.2 ± 11.2 ms vs 111 ± 7.6 ms, P = .93; duration of low amplitude signals < 40 μV 32.3 ± 8.9 ms vs 34.2 ± 7.2 ms, P = .32; root mean square of the terminal 40 ms of the filtered QRS complex 43.1 ± 25.2 ms vs 38.2 ± 20.2 ms, P = .38).
The SAECG appears to be a surrogate marker for structural abnormalities seen on imaging in those with ARVC. Great caution is required in interpreting SAECG findings in those without other corroborating evidence of an ARVC phenotype.
致心律失常性右室心肌病(ARVC)目前通过结合临床特征、影像学、心电图和基因检测来诊断。异常信号平均心电图(SAECG)被2010年工作组标准定义为次要诊断标准,但对这项检查的价值仍存在疑问。
我们使用加拿大致心律失常性右室心肌病注册研究评估了SAECG在诊断ARVC中的效用,该注册研究是一个具有人群代表性的ARVC先证者及其亲属的注册研究,较少受转诊偏倚影响。
对加拿大致心律失常性右室心肌病注册研究中的ARVC先证者及其家庭成员进行表型评估。比较不同ARVC严重程度的患者与健康对照者(ARVC先证者和不明原因猝死患者的家庭成员,无心脏病证据)的SAECG参数,包括单独参数及联合参数。
共纳入196例ARVC患者和205例对照者(平均年龄44±15岁;401例中有186例男性[46%])。205例对照者中有83例(40%)出现SAECG异常,68例轻度ARVC患者中有33例(51%)出现异常,42例重度患者中有31例(74%)出现异常。SAECG与影像学异常密切相关(主要异常:比值比3.0,95%置信区间1.3 - 6.9;次要异常:比值比3.5,95%置信区间0.7 - 16.5),但与表型的其他方面无关。携带致病变异但表型轻微的患者的SAECG与健康对照者相似(滤波QRS波时限111.2±11.2毫秒对111±7.6毫秒,P = 0.93;低振幅信号持续时间<40μV为32.3±8.9毫秒对34.2±7.2毫秒,P = 0.32;滤波QRS波群终末40毫秒的均方根为43.1±25.2毫秒对38.2±20.2毫秒,P = 0.38)。
SAECG似乎是ARVC患者影像学所见结构异常的替代标志物。在对没有其他ARVC表型确证证据的患者解释SAECG结果时需格外谨慎。