Pulsatile haemodynamic parameters are predictors of survival in paediatric pulmonary arterial hypertension

Johannes M Douwes1, Marcus T R Roofthooft, Beatrijs Bartelds

  • 1Centre for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, The Netherlands.

Insights

Pulsatile parameters like pulmonary arterial compliance (PACi) and pulmonary stroke volume (PSVi) predict survival in pediatric pulmonary arterial hypertension (PAH). These, along with static measures, aid in assessing severity and guiding treatment for better outcomes.

Area of Science:

  • Cardiology
  • Pediatric Pulmonology
  • Hemodynamics

Background:

  • Pulmonary arterial hypertension (PAH) requires reliable prognostic markers, particularly in pediatric cases.
  • Pulsatile hemodynamic parameters, such as pulmonary arterial compliance (PACi) and pulmonary stroke volume (PSVi), may offer prognostic insights beyond static measures.
  • Assessing the prognostic value of PACi and PSVi in pediatric PAH is crucial for improving patient management.

Purpose of the Study:

  • To evaluate the prognostic significance of PACi and PSVi in children diagnosed with idiopathic/hereditary PAH or PAH associated with congenital heart disease.
  • To determine if pulsatile hemodynamic parameters can predict transplant-free survival in pediatric PAH patients.

Main Methods:

  • Retrospective review of right heart catheterization data from 52 pediatric PAH patients (1993-2010).
  • Comparison with a control group of patients with normal pulmonary vascular resistance.
  • Calculation of PACi and PSVi to assess their predictive value for transplant-free survival.

Main Results:

  • Pediatric PAH patients exhibited significantly lower PSVi and PACi compared to controls.
  • Lower PSVi and PACi correlated with higher WHO-functional class.
  • Higher PSVi, PACi, and mean systemic arterial pressure (mSAP), along with lower heart rate, were associated with improved survival, independent of functional class and therapy.
  • Multivariate analysis identified PSVi, heart rate, and mSAP as key hemodynamic predictors of survival.

Conclusions:

  • Both pulsatile and static hemodynamic parameters are robust, independent predictors of transplant-free survival in pediatric PAH.
  • These parameters are valuable for assessing disease severity, predicting prognosis, and guiding treatment decisions in pediatric PAH.
Abstract

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