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Updated: Jun 14, 2025

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
Embracing the challenges of neonatal and paediatric pulmonary hypertension
Dunbar Ivy1, Erika B Rosenzweig2, Steven H Abman3
1Pediatric Cardiology, University of Colorado School of Medicine, and Children's Hospital Colorado, Aurora, CO, USA.
Insights
Paediatric pulmonary arterial hypertension (PAH) requires unique approaches due to associated disorders. This review updates diagnosis, risk stratification, and treatment for paediatric PH, highlighting knowledge gaps and challenges in clinical trials.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Critical Care Medicine
Background:
- Paediatric pulmonary arterial hypertension (PAH) shares traits with adult PAH but presents unique challenges.
- Associated disorders and specific patient populations necessitate distinct management strategies.
Purpose of the Study:
- To review recent advances, ongoing challenges, and distinct approaches in paediatric pulmonary hypertension (PH).
- To provide updates on diagnosis, classification, risk stratification, and treatment for paediatric PH.
- To identify critical knowledge gaps and propose solutions for paediatric PH management.
Main Methods:
- Consensus review by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension.
- Inclusion of updated risk stratification tools and treatment algorithms.
- Discussion of novel strategies for PAH associated with congenital heart disease and neonatal PH.
Main Results:
- An updated risk stratification tool and treatment algorithm for paediatric PH, including strategies for associated cardiopulmonary conditions.
- Highlighting advances in diagnosing neonatal PH using echocardiography and emphasizing a team approach.
- Discussion of challenges in paediatric drug approval and clinical trial design.
Conclusions:
- Paediatric PH management requires tailored approaches, addressing specific patient populations and associated conditions.
- Despite advances, treatment remains hindered by a lack of robust clinical trials and challenges in drug development.
- A multimodal and team-based approach is crucial for optimizing outcomes in infants and children with PH.
Abstract:
Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension. We provide updates on diagnosing, classifying, risk-stratifying and treating paediatric pulmonary hypertension (PH) and identify critical knowledge gaps. An updated risk stratification tool and treatment algorithm is provided, now also including strategies for patients with associated cardiopulmonary conditions. Treatment of paediatric PH continues to be hindered by the lack of randomised controlled clinical trials. The challenging management of children failing targeted PAH therapy is discussed, including balloon atrial septostomy, lung transplantation and pulmonary-to-systemic shunt (Potts). A novel strategy using a multimodal approach for the management of PAH associated with congenital heart diseases with borderline pulmonary vascular resistance is included. Advances in diagnosing neonatal PH, especially signs and interpretation of PH by echocardiography, are highlighted. A team approach to the rapidly changing physiology of neonatal PH is emphasised. Challenges in drug approval are discussed, particularly the challenges of designing accurate paediatric clinical trials with age-appropriate end-points and adequate enrolment.
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