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Updated: May 14, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
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.
Background:
There is a need for reliable prognostic parameters in pulmonary arterial hypertension (PAH), especially in children. Pulsatile components of the right ventricular afterload, represented by pulmonary arterial compliance (PACi) and pulmonary stroke volume (PSVi), may provide important additional prognostic information to conventional static haemodynamic parameters. The aim of this study was to determine the prognostic value of PACi and PSVi in paediatric PAH.
Methods:
Right heart catheterization data of 52 consecutive paediatric idiopathic/hereditary PAH and PAH associated with congenital heart disease patients with full haemodynamic evaluation seen at the Dutch national referral centre for paediatric pulmonary hypertension between 1993 and 2010 were reviewed. A control group was composed of patients with normal pulmonary vascular resistance. PSVi and PACi were calculated and tested for predictive value for transplant-free survival.
Results:
PAH patients had significantly lower PSVi and PACi compared to control patients. PSVi and PACi were lower in patients with higher WHO-functional class compared to those with lower functional classes. Higher PSVi, PACi and mSAP and lower mPAP/mSAP and heart rate were associated with improved survival, independent from WHO-functional class and PAH-targeted therapy. In multivariate analyses PSVi, heart rate and mSAP emerged as the strongest haemodynamic predictors of survival. The effect of vasodilator challenge on the haemodynamic variables did not provide additional prognostic information.
Conclusions:
The parameters of both the pulsatile and static pulmonary circulations are strong independent predictors for transplant-free survival, and therefore can be of complementary value in assessing disease severity, predicting survival and guiding treatment in paediatric PAH.
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