Department of Cardiology, University Heart Center Zurich, Zurich, Switzerland.
Circ Cardiovasc Imaging. 2014 Mar;7(2):230-9. doi: 10.1161/CIRCIMAGING.113.000210. Epub 2014 Feb 10.
The value of standard 2-dimensional transthoracic echocardiographic parameters for risk stratification in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is controversial.
We investigated the impact of RV fractional area change (FAC) and tricuspid annulus plane systolic excursion (TAPSE) for the prediction of major adverse cardiovascular events (MACE) defined as the occurrence of cardiac death, heart transplantation, survived sudden cardiac death, ventricular fibrillation, sustained ventricular tachycardia, or arrhythmogenic syncope. Among 70 patients who fulfilled the 2010 ARVC/D Revised Task Force Criteria and underwent baseline transthoracic echocardiography, 37 (53%) patients experienced MACE during a median follow-up period of 5.3 (interquartile range, 1.8-9.8) years. Average values for FAC, TAPSE, and TAPSE indexed to body surface area (BSA) decreased over time (P=0.03 for FAC, P=0.03 for TAPSE, and P=0.01 for TAPSE/BSA, each versus baseline). In contrast, median RV end-diastolic area increased (P=0.001 versus baseline). Based on the results of Kaplan-Meier estimates, the time between baseline transthoracic echocardiography and experiencing MACE was significantly shorter for patients with FAC <23% (P<0.001), TAPSE <17 mm (P=0.02), or right atrial short axis/BSA ≥25 mm/m(2) (P=0.04) at baseline. A reduced FAC constituted the strongest predictor of MACE (hazard ratio, 1.08 per 1% decrease; 95% confidence interval, 1.04-1.12; P<0.001) on bivariable analysis.
This long-term observational study indicates that TAPSE and dilation of right-sided cardiac chambers are associated with an increased risk for MACE in patients with ARVC/D with advanced disease and a high risk for adverse events. However, FAC is the strongest echocardiographic predictor of adverse outcome in these patients. Our data advocate a role for transthoracic echocardiography in risk stratification in patients with ARVC/D, although our results may not be generalizable to lower-risk ARVC/D cohorts.
标准二维经胸超声心动图参数在致心律失常性右室心肌病/发育不良（ARVC/D）患者风险分层中的价值存在争议。
我们研究了右室射血分数（FAC）和三尖瓣环平面收缩期位移（TAPSE）对主要不良心血管事件（MACE）的预测价值，MACE 定义为心脏性死亡、心脏移植、存活性猝死、心室颤动、持续性室性心动过速或心律失常性晕厥的发生。在符合 2010 年 ARVC/D 修订工作组标准并接受基线经胸超声心动图检查的 70 例患者中，37 例（53%）患者在中位随访 5.3 年（四分位距，1.8-9.8 年）期间发生 MACE。FAC、TAPSE 和 TAPSE 与体表面积（BSA）的比值随时间推移而降低（FAC 为 P=0.03，TAPSE 为 P=0.03，TAPSE/BSA 为 P=0.01，均与基线相比）。相比之下，中位 RV 舒张末期面积增加（P=0.001 与基线相比）。基于 Kaplan-Meier 估计的结果，在 FAC<23%（P<0.001）、TAPSE<17mm（P=0.02）或右心房短轴/BSA≥25mm/m²（P=0.04）的患者中，从基线经胸超声心动图到发生 MACE 的时间明显缩短。FAC 的降低是 MACE 的最强预测因素（风险比，每降低 1%增加 1.08；95%置信区间，1.04-1.12；P<0.001）。
这项长期观察性研究表明，在 ARVC/D 患者中，TAPSE 和右侧心腔扩张与疾病晚期和不良事件风险较高的患者的 MACE 风险增加相关。然而，FAC 是这些患者不良预后的最强超声心动图预测指标。我们的数据主张在 ARVC/D 患者中进行经胸超声心动图进行风险分层，尽管我们的结果可能不适用于风险较低的 ARVC/D 队列。
