Background

Patients with cardiomyopathy (CM) are at increased risk for pulmonary hypertension (PH). Data are lacking on the use of noninvasive PH measures by echocardiography in patients with CM. The aim of this study was to evaluate the correlation between Doppler-derived echocardiographic indices and catheterization-based measurement of pulmonary vascular resistance (PVR) in children and young adults with CM.

Methods

Imaging studies were retrospectively reviewed from pediatric patients with CM who underwent both echocardiography and cardiac catheterization within a 72-hour period. The ratio of peak tricuspid regurgitation velocity to right ventricular outflow tract velocity-time integral, the S/D ratio, and right ventricular myocardial performance index were correlated with invasive PVR. Receiver operating characteristic curves were developed to determine cutoffs for detecting PVR ≥ 6 indexed Wood units, a value associated with higher heart transplantation risk.

Results

Twenty-three patients with CM (median age, 11.7 years; range, 0.5–21 years) met the criteria for analysis, the majority (n = 17 [74%]) of whom had dilated CM. Linear regression showed significant correlations between echocardiography-based ratio of peak tricuspid regurgitation velocity to right ventricular outflow tract velocity-time integral, S/D ratio, and right ventricular myocardial performance index versus invasive PVR (r = 0.84, r = 0.72, and r = 0.72, respectively, P < .001). All echocardiographic measures showed high sensitivity, specificity, and predictive values to detect PVR ≥ 6 indexed Wood units, with ratio of peak tricuspid regurgitation velocity to right ventricular outflow tract velocity-time integral demonstrating the highest area under the curve (0.958; 95% CI, 0.866–1).

Conclusions

Right-sided Doppler-derived echocardiographic indices correlate with PVR measured by cardiac catheterization in children and young adults with CM. These parameters may serve as useful adjuncts in serial assessment of right ventricular hemodynamics in this population.

中文翻译：

---

儿童和青年成人心肌病的肺血管阻力的无创超声心动图测量。

背景

患有心肌病（CM）的患者患肺动脉高压（PH）的风险增加。缺少有关CM患者通过超声心动图进行无创PH测量的数据。这项研究的目的是评估多普勒超声心动图指数与以导管插入术为基础的儿童和年轻成人CM的肺血管阻力（PVR）测量之间的相关性。

方法

回顾性地回顾了在72小时内接受超声心动图和心脏导管检查的小儿CM患者的影像学研究。三尖瓣反流速度峰值与右心室流出道速度-时间积分之比，S / D比和右心室心肌性能指数与侵入性PVR相关。制定了接收器工作特性曲线，以确定用于检测PVR≥6分度的伍德单位的临界值，该值与更高的心脏移植风险相关。

结果

23例CM患者（中位年龄11.7岁；范围0.5-21岁）符合分析标准，其中大多数（n  = 17 [74％]）扩张了CM。线性回归显示基于超声心动图的三尖瓣反流峰值速度与右心室流出道速度-时间积分，S / D比和右心室心肌性能指数与侵入性PVR之间的显着相关性（r  = 0.84，r  = 0.72，r  = 0.72，分别P <.001）。所有超声心动图测量均显示出检测PVR≥6指数伍德单位的高灵敏度，特异性和预测值，三尖瓣反流峰值速度与右心室流出道速度-时间积分之比表明曲线下的最高面积（0.958； 95％CI ，0.866–1）。

结论

右侧多普勒超声心动图指数与通过心导管检查测得的CM儿童和年轻人的PVR相关。这些参数可以作为该人群右心室血流动力学系列评估的有用辅助手段。