BACKGROUND Right ventricular (RV) failure, a determinant of outcomes in pulmonary hypertension (PH), occurs when the right ventricle cannot compensate for increased afterload. The authors showed that RV stroke work (RVSW) can be estimated in children with PH as the product of stroke volume and RV pressure and is related to adverse outcomes. The aim of this study was to test the hypothesis that ventricular-vascular (VV) mismatch (high afterload and low RVSW) is associated with echocardiographic measures of RV performance and adverse outcomes. METHODS Invasive hemodynamic data and concurrent echocardiograms were reviewed. Fifty subjects with PH were included. Four groups were created by dividing the patients using median RVSW and median pulmonary vascular resistance. For each group, tricuspid annular plane systolic excursion, fractional area change, myocardial performance index, and anterior RV wall thickness were determined. Both major (i.e., death) and minor (i.e., worsening World Health Organization class) clinical outcomes were tabulated. Groups were compared using the Kruskal-Wallis or Fisher exact test. RESULTS Patients in the high pulmonary vascular resistance/low RVSW cohort (VV mismatch) had the worst RV dysfunction: median tricuspid annular plane systolic excursion, 0.8 cm (interquartile range, 0.7-0.8 cm; P = .0002); median fractional area change, 0.29% (interquartile range, 0.27%-0.30%; P = .004); median myocardial performance index, 0.622 (interquartile range, 0.548-0.789; P = .0004). This group had the highest incidence of adverse outcomes: major events in 40%, minor events in 80%, and syncope in 60%. CONCLUSION VV mismatch in pediatric PH can be assessed using RVSW and pulmonary vascular resistance and is associated with RV performance and adverse events. RVSW increases in compensated high-afterload states and falls as the right ventricle fails to meet increased load; thus, VV matching status may be a sensitive predictor of outcomes in pediatric PH.

中文翻译：

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肺动脉高压患儿的右心室功能参数与右心室搏动对肺血管阻力的关系揭示了右心室不匹配。

背景技术右心室（RV）衰竭是决定肺动脉高压（PH）的结果，发生在右心室无法补偿增加的后负荷时。这组作者表明，PH患儿可将RV卒中工作（RVSW）估算为卒中量和RV压力的乘积，并且与不良结局有关。这项研究的目的是检验以下假设：心室-血管（VV）失配（高负荷量和低RVSW）与超声心动图测量RV表现和不良结局有关。方法回顾了有创血流动力学数据和并发超声心动图。纳入了50名患有PH的受试者。通过使用中位RVSW和中位肺血管阻力将患者划分为四组。对于每组，三尖瓣环平面收缩期偏移，面积分数变化，测定心肌性能指标和右室前壁厚度。将主要（即死亡）和次要（即世界卫生组织等级恶化）的临床结局都制成表格。使用Kruskal-Wallis或Fisher精确检验比较各组。结果高肺血管阻力/低RVSW队列（VV失配）患者的RV功能障碍最严重：中位三尖瓣环平面收缩期偏移为0.8 cm（四分位间距为0.7-0.8 cm； P = .0002）。分数变化中位数为0.29％（四分位间距为0.27％-0.30％; P = 0.004）; 心肌功能中位数指数为0.622（四分位间距为0.548-0.789； P = 0.0004）。该组不良事件发生率最高：重大事件占40％，次要事件占80％，晕厥占60％。结论可以通过RVSW和肺血管阻力评估小儿PH的VV失配，并与RV表现和不良事件相关。RVSW在补偿的高后负荷状态下增加，并在右心室无法满足负荷增加时下降；因此，VV匹配状态可能是小儿PH结局的敏感预测指标。