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Updated: Apr 17, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
[Pediatric pulmonary hypertension]
Insights
Pediatric pulmonary hypertension (PH) is a serious condition in children, often linked to congenital heart disease. Management challenges persist due to reliance on adult data and expert experience.
Area of Science:
- Pediatric Cardiology
- Pulmonary Medicine
- Rare Diseases
Context:
- Pulmonary hypertension (PH) significantly impacts pediatric morbidity and mortality.
- Common causes in children include idiopathic PH and congenital heart disease (CHD).
- Incidence rates highlight the prevalence of PH associated with CHD in pediatric populations.
Purpose:
- To review the classification and management of pediatric pulmonary hypertension.
- To address the challenges in diagnosing and treating PH in infants, children, and newborns.
- To highlight the evolving understanding of PH in relation to CHD and developmental lung diseases.
Summary:
- Pediatric PH is rare but serious, with idiopathic and CHD-associated forms being most common.
- Updated classifications now better incorporate CHD and neonatal/developmental lung conditions.
- Current management relies heavily on adult studies and expert clinical judgment, posing challenges.
Impact:
- Improved classification aids in better understanding and diagnosis of pediatric PH.
- Highlights the need for pediatric-specific evidence in treatment strategies.
- Emphasizes the critical role of expert experience in managing complex pediatric cases.
Abstract:
Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence based adult studies and the clinical experience of pediatric experts. (J Am Coll Cardiol 2013;62:D117-26) ©2013 by the American College of Cardiology Foundation.
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