Baseline pulmonary vascular resistance index was significantly associated with adverse outcomes in pediatric pulmonary arterial hypertension (HR 1.07 per 1 WU·m2 increase; 95% CI 1.02-1.12; P=0.002).
Cohort (n=71)
Do hemodynamic parameters from repeated right heart catheterization predict adverse outcomes in children with pulmonary arterial hypertension?
Repeated right heart catheterization identifies children with pediatric pulmonary arterial hypertension who remain at high risk despite apparent improvement on noninvasive criteria.
Hazard Ratio: 1.07 (95% CI 1.02–1.12)
p-value: p=0.002
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 2 increase 95% CI, 1.02–1.12, P =0.002), stroke volume index (HR 0.95 per 1 L·min −1 ·m −2 increase 95% CI, 0.91–0.99, P =0.012), pulmonary artery compliance index (HR 0.16 per 1 mL·mm Hg −1· m −2 increase 95% CI, 0.051–0.52, P =0.002), and right atrial pressure (HR, 1.31 per 1 mm Hg increase 95% CI, 1.01–1.71, P =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.
Grynblat et al. (Tue,) conducted a cohort in Pulmonary arterial hypertension (n=71). Pulmonary vascular resistance index was evaluated on Adverse outcomes (death, lung transplantation, or Potts shunt) (HR 1.07, 95% CI 1.02-1.12, p=0.002). Baseline pulmonary vascular resistance index was significantly associated with adverse outcomes in pediatric pulmonary arterial hypertension (HR 1.07 per 1 WU·m2 increase; 95% CI 1.02-1.12; P=0.002).