Background Echocardiographic determination of the right ventricular end-systolic remodeling index (RVES RI) has clinical value for the assessment of pulmonary hypertension (PH) in adults. We aim to determine RVES RI values in pediatric PH and to correlate RVES RI data with echocardiographic variables and NYHA functional class (FC). Methods Prospective echocardiography study in 49 children with PH. The 49 matched control subjects were chosen from 123 healthy children used to construct pediatric normal reference values. The associations with invasive hemodynamic variables were also investigated in a validation cohort of 12 PH children and matched controls. Results RVES RI was increased in children with PH vs. healthy controls (1.45 ± 0.16 vs. 1.16 ± 0.06; p < 0.01; confirmed in the validation cohort). RVES RI was associated with invasive hemodynamic variables, i.e. the mean pulmonary artery pressure. RVES RI values increased with worsening NYHA-FC. The highest RVES RI values were observed in PH children with NYHA FC 3 (1.60 ± 0.12). Conclusions RVES RI is a useful indicator of RV remodeling and dilation in the setting of increased RV pressure load, especially when the degree of regurgitation of the tricuspid and pulmonary valves is insufficient to numerically estimate RV systolic pressure and mPAP, due to incomplete Doppler envelopes.

中文翻译：

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右心室收缩末期重构指数在评估小儿肺动脉高压中的作用。欧洲小儿肺血管疾病网络 (EPPVDN)

背景超声心动图测定右心室收缩末期重构指数 (RVES RI) 对评估成人肺动脉高压 (PH) 具有临床价值。我们的目标是确定儿科 PH 的 RVES RI 值，并将 RVES RI 数据与超声心动图变量和 NYHA 功能等级 (FC) 相关联。方法对49例PH儿童进行前瞻性超声心动图研究。49 名匹配的对照受试者选自用于构建儿科正常参考值的 123 名健康儿童。还在 12 名 PH 儿童和匹配对照的验证队列中研究了与侵入性血流动力学变量的关联。结果 与健康对照相比，PH 儿童的 RVES RI 增加（1.45 ± 0.16 与 1.16 ± 0.06；p < 0.01；在验证队列中得到证实）。RVES RI 与侵入性血液动力学变量相关，即平均肺动脉压力。RVES RI 值随着 NYHA-FC 的恶化而增加。在患有 NYHA FC 3 的 PH 儿童中观察到最高的 RVES RI 值 (1.60 ± 0.12)。结论 RVES RI 是在 RV 压力负荷增加的情况下 RV 重构和扩张的有用指标，特别是当三尖瓣和肺动脉瓣的返流程度不足以数值估计 RV 收缩压和 mPAP 时，由于多普勒包络不完整。