Cardiovascular Department, Azienda Ospedaliera Ospedali Riuniti and University of Trieste, Trieste, Italy.
Eur Heart J. 2011 May;32(9):1105-13. doi: 10.1093/eurheartj/ehr040. Epub 2011 Feb 28.
We sought to examine the clinical presentation and natural history and to identify long-term prognostic predictors in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) as information concerning the natural history and risk stratification of ARVC is still incomplete.
A cohort of 96 ARVC patients (68% males, 35 ± 15 years) was enrolled and underwent structured diagnostic protocol and follow-up. Primary study endpoints were death and heart transplantation (HTx). Clinical and echo-Doppler data were assessed as prognostic indicators. Sixty-five per cent of patients had right ventricular (RV) systolic dysfunction (RV fractional area change < 33%) and 24% had left ventricular (LV) systolic dysfunction (LV ejection fraction <50%). During a mean follow-up of 128 ± 92 months, 20 patients (21%) experienced cardiac death or underwent HTx. At multivariate analysis (Model 1), RV dysfunction [hazard ratio (HR): 4.12; 95% confidence interval (CI): 1.01-18.0; P = 0.05], significant tricuspid regurgitation (HR: 7.6; 95% CI: 2.6-22.0; P < 0.001), and amiodarone treatment (HR: 3.4; 95% CI: 1.3-8.8; P = 0.01) resulted as predictors of death/HTx. When inserting in the model, the 'ordinal dysfunction' (Model 2), which considers the presence of both RV and LV dysfunctions, this variable emerged as an independent prognostic predictor (HR: 6.3; 95% CI: 2.17-17.45; P < 0.001). At the receiver operating characteristic analysis, Model 2 was significantly more accurate in predicting long-term outcome compared with Model 1 (area under the curve 0.84 vs. 0.78, respectively; P = 0.04).
In our tertiary referral centre ARVC population, the presence of LV dysfunction at diagnosis has an incremental power in predicting adverse outcome compared with RV dysfunction alone.
我们旨在研究心律失常性右心室心肌病（ARVC）患者的临床表现、自然病史，并确定长期预后预测因素，因为目前有关 ARVC 自然病史和风险分层的信息仍然不完整。
本研究纳入了 96 例 ARVC 患者（68%为男性，年龄 35±15 岁），并对其进行了结构化诊断方案和随访。主要研究终点为死亡和心脏移植（HTx）。评估临床和超声心动图数据作为预后指标。65%的患者存在右心室（RV）收缩功能障碍（RV 节段性面积变化 < 33%），24%的患者存在左心室（LV）收缩功能障碍（LV 射血分数 < 50%）。在平均 128±92 个月的随访期间，20 例患者（21%）发生心脏性死亡或接受了 HTx。多变量分析（模型 1）显示，RV 功能障碍[风险比（HR）：4.12；95%置信区间（CI）：1.01-18.0；P = 0.05]、严重三尖瓣反流（HR：7.6；95%CI：2.6-22.0；P < 0.001）和胺碘酮治疗（HR：3.4；95%CI：1.3-8.8；P = 0.01）是死亡/HTx 的预测因素。当将“有序功能障碍”（模型 2）插入模型中时，该变量被认为是一个独立的预后预测因素（HR：6.3；95%CI：2.17-17.45；P < 0.001）。在接受者操作特征分析中，与模型 1 相比，模型 2 预测长期预后的准确性显著提高（曲线下面积分别为 0.84 和 0.78，P = 0.04）。
在我们的三级转诊中心 ARVC 患者人群中，与单独的 RV 功能障碍相比，诊断时存在 LV 功能障碍对预测不良结局具有附加作用。
