University of California San Francisco (S.K., S.S., E.V., L.K., M.M.S.).
Department of Clinical Physiology and Department of Health, Medicine and Caring Sciences, Linköping University, Sweden (M.A.A.).
Circ Heart Fail. 2021 Sep;14(9):e008121. doi: 10.1161/CIRCHEARTFAILURE.120.008121. Epub 2021 Sep 14.
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.
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).
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.
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.
先前关于致心律失常性右心室（RV）心肌病的结构和心电图改变及其在预测室性心律失常（室性心动过速）中的作用的研究结果存在矛盾。
我们对 64 例致心律失常性 RV 心肌病患者（56%为男性，平均年龄[标准差]为 44.2[14.6]岁）的 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检查进行了回顾性分析，平均随访时间为 10 年（范围为 2.3-19 年）。采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（中重度三尖瓣反流、胸骨旁长轴和短轴的 RV 流出道直径、RV 舒张末期面积、射血分数）之间的关联。
与患者首次经胸超声心动图检查后每增加 5 年相比，RV 舒张末期面积增加 4.65（95%置信区间：0.51%-8.8%），射血分数降低 3.75（95%置信区间：1.17%-6.34%），RV 壁运动异常的优势比（比值比）增加 1.9（95%置信区间：1.3-2.8）。中重度三尖瓣反流是 RV 扩大和功能障碍的独立预测因子（射血分数从基线下降 10%以上的风险比[95%置信区间]：3.51[1.77-6.95]；RV 舒张末期面积从基线增加 10%以上的风险比[95%置信区间]：4.90[2.52-9.52]）。植入式心脏复律除颤器患者更有可能发生中重度三尖瓣反流和更大的结构性和功能性疾病进展。RV 舒张末期面积的明显增加与任何室性心动过速的发生率更高相关。下壁 T 波倒置和 V1 至 V3 的 R 波总和（mm）是 RV 扩大和功能障碍的预测因子，前者也预测任何室性心动过速的风险。
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子，而经静脉植入式心脏复律除颤器导线可能会加剧这种进展。
