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Updated: May 7, 2026
Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
Current challenges in pediatric pulmonary hypertension
Shinichi Takatsuki1, David Dunbar Ivy
1Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colorado.
Insights
Pediatric pulmonary arterial hypertension (PAH) has various causes, primarily idiopathic or linked to congenital heart disease (CHD). Current treatments rely on adult data, highlighting a need for pediatric-specific research and guidelines.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Clinical Pediatrics
Background:
- Pediatric pulmonary arterial hypertension (PAH) presents significant morbidity and mortality.
- Common causes include idiopathic PAH and PAH associated with congenital heart disease (CHD).
- Pulmonary hypertension (PH) linked to connective tissue disease is rare in children.
Purpose of the Study:
- To review the classification, incidence, and treatment of pediatric PAH.
- To highlight the development of a pediatric-specific PH classification.
- To discuss the challenges in pediatric PAH treatment due to reliance on adult studies.
Main Methods:
- Review of existing literature and classification systems for pediatric PH.
- Analysis of incidence data for idiopathic PAH and PAH-CHD from the Netherlands.
- Examination of treatment strategies, including selective pulmonary vasodilators.
Main Results:
- Incidence rates for idiopathic PAH and PAH-CHD per million children were reported.
- New pediatric-specific PH classification acknowledges developmental and fetal origins.
- Disparate treatment recommendations exist for sildenafil in the US and Europe.
Conclusions:
- Pediatric PAH requires tailored approaches due to unique etiologies and developmental factors.
- Evidence-based treatment for pediatric PAH is evolving, with ongoing research needed.
- Standardized pediatric guidelines are crucial for consistent and effective management.
Abstract:
Pulmonary arterial hypertension (PAH) in the pediatric population is associated with a variety of underlying diseases and causes, significantly morbidity and mortality. In the majority of patients, PAH in children is idiopathic or associated with congenital heart disease (CHD), with pulmonary hypertension (PH) associated with connective tissue disease, a rare cause in children. Classification of pediatric PH has generally followed the WHO classification, but recognition of the importance of fetal origins of PH and developmental abnormalities have led to the formation of a new pediatric-specific classification. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic PAH and 2.2 and 15.6 for associated pulmonary arterial hypertension-CHD cases per million children. Although the treatment with new selective pulmonary vasodilators offers hemodynamic and functional improvement in pediatric populations, the treatments in children largely depend on results from evidence-based adult studies and experience of clinicians treating children. A recent randomized clinical trial of sildenafil and its long-term extension has led to disparate recommendations in the United States and Europe.
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