M3C-Necker, Hôpital Necker-Enfants malades, AP-HP Université de Paris Cité, Cardiologie Congénitale et Pédiatrique Paris France.
Université Paris-Saclay, Faculty of Medicine Le Kremlin-Bicêtre France.
J Am Heart Assoc. 2023 Apr 4;12(7):e029085. doi: 10.1161/JAHA.122.029085. Epub 2023 Mar 28.
Background Right heart catheterization (RHC) is a high-risk procedure in children with pulmonary arterial hypertension without clear guidelines for the indications and targets of invasive reassessment. Our objectives are to define the aims of repeated RHC and evaluate the correlation between noninvasive criteria and hemodynamic parameters. Methods and Results Clinical and hemodynamic characteristics from 71 incident treatment-naïve children (median age 6.2 years) with pulmonary arterial hypertension who had a baseline and reevaluation RHC were analyzed. Correlations between noninvasive predictors and hemodynamic parameters were tested. Adverse outcomes were defined as death, lung transplantation, or Potts shunt. At baseline, pulmonary vascular resistance index (hazard ratio [HR] 1.07 per 1 WU·m increase [95% CI, 1.02-1.12], =0.002), stroke volume index (HR 0.95 per 1 L·min·m increase [95% CI, 0.91-0.99], =0.012), pulmonary artery compliance index (HR 0.16 per 1 mL·mm Hgm increase [95% CI, 0.051-0.52], =0.002), and right atrial pressure (HR, 1.31 per 1 mm Hg increase [95% CI, 1.01-1.71], =0.043) were associated with adverse outcomes. Pulmonary vascular resistance index, pulmonary artery compliance index, and right atrial pressure were still associated with a worse outcome at second RHC. Noninvasive criteria accurately predicted hemodynamic evolution; however, 70% of the patients who had improved based on noninvasive criteria still presented at least 1 "at risk" hemodynamics at second RHC. Conclusions Pulmonary vascular resistance index, pulmonary artery compliance index, and right atrial pressure are solid predictors of adverse outcomes in pediatric pulmonary arterial hypertension and potential therapeutic targets. Noninvasive criteria accurately predict the evolution of hemodynamic parameters, but insufficiently. Repeated RHC are helpful to identify children with persistent higher risk after treatment introduction.
右心导管检查（RHC）是一种高危程序，适用于患有肺动脉高压但没有明确的介入性再评估适应证和目标的儿童。我们的目的是确定重复 RHC 的目的，并评估非侵入性标准与血流动力学参数之间的相关性。
对 71 例首次接受治疗的儿童（中位年龄 6.2 岁）进行了基线和重新评估 RHC 的临床和血流动力学特征分析。测试了非侵入性预测因子与血流动力学参数之间的相关性。将不良结局定义为死亡、肺移植或波氏分流术。基线时，肺血管阻力指数（每增加 1 WU·m 的危险比[HR]为 1.07[95%CI，1.02-1.12]，=0.002）、每增加 1 L·min·m 的每搏输出量指数（HR 为 0.95[95%CI，0.91-0.99]，=0.012）、肺动脉顺应性指数（每增加 1mmHg·mm·m 的 HR 为 0.16[95%CI，0.051-0.52]，=0.002）和右心房压（HR 每增加 1mmHg 为 1.31[95%CI，1.01-1.71]，=0.043）与不良结局相关。在第二次 RHC 时，肺血管阻力指数、肺动脉顺应性指数和右心房压仍与预后不良相关。非侵入性标准准确预测血流动力学的演变；然而，在基于非侵入性标准改善的患者中，70%的患者在第二次 RHC 时仍至少存在 1 种“高危”血流动力学。
肺血管阻力指数、肺动脉顺应性指数和右心房压是儿童肺动脉高压不良结局的可靠预测因子，也是潜在的治疗靶点。非侵入性标准准确预测血流动力学参数的演变，但不够充分。重复 RHC有助于确定治疗引入后持续存在更高风险的儿童。
