Al-Sabah Arrhythmia Institute, St. Luke's and Roosevelt Hospitals, and Columbia University College of Physicians & Surgeons, New York, New York 10025, USA.
Heart Rhythm. 2011 Feb;8(2):256-62. doi: 10.1016/j.hrthm.2010.10.007. Epub 2010 Oct 8.
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited disease that causes structural and functional abnormalities of the right ventricle (RV). The presence of late potentials as assessed by the signal-averaged electrocardiogram (SAECG) is a minor task force criterion.
The purpose of this study was to examine the diagnostic and clinical value of the SAECG in a large population of genotyped ARVC/D probands.
We compared the SAECGs of 87 ARVC/D probands (age 37 ± 13 years, 47 males) diagnosed as affected or borderline by task force criteria without using the SAECG criterion with 103 control subjects. The association of SAECG abnormalities was also correlated with clinical presentation, surface ECG, ventricular tachycardia (VT) inducibility at electrophysiologic testing, implantable cardioverter-defibrillator therapy for VT, and RV abnormalities as assessed by cardiac magnetic resonance imaging (cMRI).
Compared with controls, all three components of the SAECG were highly associated with the diagnosis of ARVC/D (P <.001). They include the filtered QRS duration (97.8 ± 8.7 ms vs 119.6 ± 23.8 ms), low-amplitude signal (24.4 ± 9.2 ms vs 46.2 ± 23.7 ms), and root mean square amplitude of the last 40 ms of the QRS (50.4 ± 26.9 μV vs 27.9 ± 36.3 μV). The sensitivity of using SAECG for diagnosis of ARVC/D was increased from 47% using the established 2 of 3 criteria (i.e., late potentials) to 69% by using a modified criterion of any 1 of 3 criteria, while maintaining a high specificity of 95%. Abnormal SAECG as defined by this modified criterion was associated with a dilated RV volume and decreased RV ejection fraction detected by cMRI (P <.05). SAECG abnormalities did not vary with clinical presentation or reliably predict spontaneous or inducible VT and had limited correlation with ECG findings.
Using 1 of 3 SAECG criteria contributed to increased sensitivity and specificity for the diagnosis of ARVC/D. This finding is incorporated in the recent modification of the task force criteria.
致心律失常性右室心肌病/发育不良（ARVC/D）是一种遗传性疾病，可导致右心室（RV）的结构和功能异常。通过信号平均心电图（SAECG）评估的晚电位是一个次要的工作组标准。
本研究旨在检查 SAECG 在大量 ARVC/D 先证者基因分型人群中的诊断和临床价值。
我们比较了 87 名 ARVC/D 先证者（年龄 37±13 岁，男性 47 名）的 SAECG，这些先证者根据工作组标准诊断为受累或边界型，而不使用 SAECG 标准，并与 103 名对照者进行比较。SAECG 异常的相关性还与临床表现、体表心电图、电生理检查时室性心动过速（VT）的可诱导性、植入式心脏复律除颤器治疗 VT 以及心脏磁共振成像（cMRI）评估的 RV 异常相关。
与对照组相比，SAECG 的所有三个成分均与 ARVC/D 的诊断高度相关（P<0.001）。它们包括滤波 QRS 持续时间（97.8±8.7 ms 比 119.6±23.8 ms）、低幅度信号（24.4±9.2 ms 比 46.2±23.7 ms）和 QRS 最后 40 ms 的均方根幅度（50.4±26.9 μV 比 27.9±36.3 μV）。通过使用修改后的标准（任何 3 个标准中的 1 个标准），将 SAECG 用于 ARVC/D 诊断的敏感性从使用现有的 2 个标准（即晚电位）的 47%增加到 69%，同时保持 95%的高特异性。通过此修改标准定义的异常 SAECG 与 cMRI 检测到的 RV 容积扩张和 RV 射血分数降低相关（P<0.05）。SAECG 异常与临床表现无关，不能可靠地预测自发性或可诱导性 VT，与 ECG 发现相关性有限。
使用 SAECG 的 1 个标准有助于提高 ARVC/D 的诊断敏感性和特异性。这一发现被纳入工作组标准的最新修改中。
