Background:Prior studies of structural and electrocardiographic changes in arrhythmogenic right ventricular (RV) cardiomyopathy and their role in predicting ventricular arrhythmias (ventricular tachycardia) have shown conflicting results.Methods:We reviewed 405 ECGs, 315 transthoracic echocardiographies, and 441 implantable cardioverter defibrillator interrogations in 64 arrhythmogenic RV cardiomyopathy patients (56% men, mean age [SD], 44.2 [14.6] years) over a mean follow-up of 10 (range, 2.3–19) years. Generalized estimating equations were used to identify the association between ECG abnormalities, clinical variables, and transthoracic echocardiographic measurements (>mild degree of tricuspid regurgitation, RV outflow tract diameter in parasternal long axis and short axis, RV end-diastolic area, fractional area change).Results:There was a 4.65 (95% CI, 0.51%–8.8%) increase in RV end-diastolic area, a 3.75 (95% CI, 1.17%–6.34%) decrease in fractional area change, and 1.9 (95% CI, 1.3–2.8) higher odds (odds ratio) of RV wall motion abnormality with every 5-year increase in age after patients’ first transthoracic echocardiography. >Mild tricuspid regurgitation was an independent predictor of RV enlargement and dysfunction (hazard ratio of >10% drop in fractional area change from baseline [95% CI], 3.51 [1.77–6.95] and hazard ratio of >10% increase in RV end-diastolic area from baseline [95% CI], 4.90 [2.52–9.52]). Patients with implantable cardioverter defibrillator were more likely to develop >mild tricuspid regurgitation and larger structural and functional disease progression. More pronounced increase in RV end-diastolic area was translated into higher rates of any ventricular tachycardia. Inferior T-wave inversions and sum of R waves (mm) in V1 to V3 were predictors of RV enlargement and dysfunction with the former also predicting risk of any ventricular tachycardia.Conclusions:Arrhythmogenic RV cardiomyopathy is a progressive disease. Tricuspid regurgitation is an independent predictor of structural disease progression, which may be exacerbated by use of a transvenous implantable cardioverter defibrillator lead.

中文翻译：

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致心律失常性右心室心肌病的长期心电图和超声心动图进展及其与室性快速性心律失常的相关性

背景：先前关于致心律失常性右心室 (RV) 心肌病的结构和心电图变化及其在预测室性心律失常（室性心动过速）中的作用的研究显示出相互矛盾的结果。方法：我们回顾了 405 份心电图、315 份经胸超声心动图和 441 份植入式心脏复律除颤器在 64 名致心律失常性右心室心肌病患者（56% 男性，平均年龄 [SD]，44.2 [14.6] 岁）中进行了 10（范围，2.3-19）年的平均随访。使用广义估计方程来确定心电图异常、临床变量和经胸超声心动图测量值（>轻度三尖瓣返流、胸骨旁长轴和短轴的 RV 流出道直径、RV 舒张末期面积、面积分数变化）之间的关联.结果：有一个 4。RV 舒张末期面积增加 65（95% CI，0.51%–8.8%），面积变化分数减少 3.75（95% CI，1.17%–6.34%），1.9（95% CI，1.3–2.8）患者第一次经胸超声心动图检查后，年龄每增加 5 年，右室壁运动异常的比值（比值比）就更高。>轻度三尖瓣反流是 RV 扩大和功能障碍的独立预测因子（风险比从基线下降 >10% [95% CI]，3.51 [1.77–6.95] 和 RV 末端增加 >10% 的风险比- 基线舒张面积 [95% CI], 4.90 [2.52–9.52])。植入式心脏复律除颤器的患者更可能发生>轻度三尖瓣反流和更大的结构和功能疾病进展。RV 舒张末期面积的更显着增加转化为更高的室性心动过速发生率。V1 至 V3 的低 T 波倒置和 R 波总和 (mm) 是 RV 扩大和功能障碍的预测因子，前者也可预测任何室性心动过速的风险。结论：致心律失常性 RV 心肌病是一种进行性疾病。三尖瓣反流是结构性疾病进展的独立预测因素，使用经静脉植入式心脏复律除颤器导线可能会加剧这种情况。