Skrzypczyńska-Banasik Urszula, Woźniak Olgierd, Kowalik Ilona, Fronczak-Jakubczyk Aneta, Borowiec Karolina, Hoffman Piotr, Biernacka Elżbieta Katarzyna
Cardinal Wyszynski National Institute of Cardiology, Alpejska 42, 04-628 Warsaw, Poland.
J Clin Med. 2024 Aug 25;13(17):5038. doi: 10.3390/jcm13175038.
: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder characterised by progressive fibrosis predominantly of the right ventricular (RV) myocardium, resulting in life-threatening arrhythmias and heart failure. The diagnosis is challenging due to a wide spectrum of clinical symptoms. The important role of ECG was covered in the current diagnostic criteria. The role of the epsilon wave (EW) is still under discussion. : The aim of the study was to examine a potential association between the EW and late ventricular potentials (LPs) in ARVC patients (pts). The correlation between RV dilatation or dysfunction and LPs/EW was also analysed. : The ARVC group consisted of 81 pts (53 men, aged 20-78 years) fulfilling 2010 International Task Force Criteria. 12-lead ECG, LPs, Holter, and ECHO were performed in all pts. The presence of EW was analysed in ECG by 3 investigators. LPs were detected by signal-averaged ECG (SAECG). SAECG was considered positive for LPs when at least two of the three following criteria were met: (1) the filtered QRS duration (fQRS) ≥ 114 msec; (2) the duration of the final QRS fragment in which low-amplitude signals lower than 40 μV are recorded (LAS-40 > 38 msec); and (3) the root mean square amplitude of the last 40 milliseconds of the fQRS complex (RMS-40 < 20 μV). The results were compared with a reference group consisting of 53 patients with RV damage in the course of atrial septum defect (ASD) or Ebstein's Anomaly (EA). Results: In the ARVC group, a significant relationship was observed between the occurrence of EW and the presence of LPs. EW was more common in the LP+ than in the LP- patients (48.1% vs. 6.9%, < 0001; OR 12.5; 95% CI [2.691-58.063]). In ARVC pts, RVOT > 36 mm, RVIT > 41 mm, and RV S' < 9 cm/s were observed significantly more often in the LPs+ than in the LPs- group (OR [95% CI]: 8.3 [2.9-1.5], 6.4 [2.2-19.0] and 3.6 [1.1-12.2], respectively). In the ARVC group, any of fQRS > 114 ms, LAS > 38 ms, and RMS < 20 μV were significantly more frequent in EW+ pts. In multivariate analysis, the independent factors of the EW were LAS-40 and RV S'. In the LPs- subgroup, RVOT > 36 mm was more frequent in ASD/EA than in ARVC (70.4% vs. 25%, = 0.002). Similarly, in the LPs- subgroup, RVIT > 41 mm was encountered more frequently in ASD/EA than in ARVC (85.2% vs. 48.3%, = 0.004). : In ARVC, there is an association between EW and LPs, with both probably resulting from the same process of fibrofatty substitution of the RV myocardium. Although RV dilatation is common in ASD and EA, it does not correlate with LPs.
致心律失常性右室心肌病(ARVC)是一种遗传性疾病,其特征是主要在右心室(RV)心肌出现进行性纤维化,导致危及生命的心律失常和心力衰竭。由于临床症状范围广泛,诊断具有挑战性。当前的诊断标准涵盖了心电图(ECG)的重要作用。ε波(EW)的作用仍在讨论中。:本研究的目的是检查ARVC患者中EW与晚期心室电位(LPs)之间的潜在关联。还分析了RV扩张或功能障碍与LPs/EW之间的相关性。:ARVC组由81例符合2010年国际工作组标准的患者(pts)组成(53名男性,年龄20 - 78岁)。对所有患者进行了12导联心电图、LPs、动态心电图监测(Holter)和超声心动图(ECHO)检查。由3名研究人员分析心电图中EW的存在情况。通过信号平均心电图(SAECG)检测LPs。当满足以下三个标准中的至少两个时,SAECG被认为LPs阳性:(1)滤波后的QRS波时限(fQRS)≥114毫秒;(2)记录到低于40微伏低振幅信号的最终QRS波片段的时限(LAS - 40 > 38毫秒);(3)fQRS复合波最后40毫秒的均方根振幅(RMS - 40 < 20微伏)。将结果与由53例在房间隔缺损(ASD)或埃布斯坦畸形(EA)病程中出现RV损害的患者组成的对照组进行比较。结果:在ARVC组中,观察到EW的出现与LPs的存在之间存在显著关系。EW在LPs阳性患者中比在LPs阴性患者中更常见(48.1%对6.9%,<0.0001;比值比[OR]12.5;95%置信区间[CI][2.691 - 58.063])。在ARVC患者中,LPs阳性组中RVOT > 36毫米、RVIT > 41毫米和RV S' < 9厘米/秒的情况明显比LPs阴性组更常见(OR[95%CI]:分别为8.3[2.9 - 1.5]、6.4[2.2 - 19.0]和3.6[1.1 - 12.2])。在ARVC组中,EW阳性患者中fQRS > 114毫秒、LAS > 38毫秒和RMS < 20微伏中的任何一项都明显更频繁。在多变量分析中,EW的独立因素是LAS - 40和RV S'。在LPs阴性亚组中,ASD/EA患者中RVOT > 36毫米比ARVC患者更常见(70.4%对25%,P = 0.