Related Experiment Video
Updated: Jan 16, 2026
Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
Chronic Lung Disease-Related Pulmonary Hypertension in Children
Jason E Lang1,2, Kevin D Hill1,3
1Duke Clinical Research Institute, Durham, North Carolina, USA.
Insights
Pediatric pulmonary hypertension (PH) linked to chronic lung disease (CLD-PH) requires specialized care. Key treatments include oxygen, phosphodiesterase inhibitors, endothelin receptor antagonists, and prostacyclin agonists.
Area of Science:
- Pediatric Pulmonology
- Cardiology
- Critical Care Medicine
Background:
- Pulmonary hypertension (PH) involves elevated pulmonary arterial pressures, with limited treatments for pediatric cases.
- WHO Group 3, chronic lung disease/hypoxia-related pulmonary hypertension (CLD-PH), is a significant concern in children.
Purpose of the Study:
- To review current diagnostic and treatment strategies for pediatric PH.
- To specifically emphasize management of CLD-PH in children.
Main Methods:
- A narrative review was conducted by two pediatric subspecialists.
- The review focused on expert guidelines and pharmacotherapeutic data for CLD-PH.
Main Results:
- Bronchopulmonary dysplasia (BPD) is the primary cause of CLD-PH, affecting up to 30% of infants with BPD.
- Factors like prematurity, ventilation, infection, and genetics contribute to vascular changes in CLD-PH.
- Common therapies include supplemental oxygen, phosphodiesterase inhibitors, endothelin receptor antagonists, and prostacyclin agonists.
Conclusions:
- CLD-PH in children is complex, necessitating a deep understanding of its causes and assessment.
- Effective management requires specialized teams, tailored pharmacotherapy, and family-centered care.
- Improving outcomes for children with CLD-PH depends on comprehensive management strategies.
Aim:
Pulmonary hypertension (PH) is characterised by elevated pressures in the pulmonary arterial system. Despite its high mortality, paediatric PH has few approved treatments. Our aim was to review the latest diagnostic and treatment considerations for paediatric PH, with particular emphasis on WHO group 3 chronic lung disease/hypoxia-related pulmonary hypertension (CLD-PH).
Methods:
Two subspecialists caring for children with CLD-PH conducted a narrative review of the latest expert guidelines and published pharmacotherapeutic data relevant to CLD-PH.
Results:
Bronchopulmonary dysplasia (BPD) is the leading cause of CLD-PH; up to 30% of infants with BPD develop PH. Extreme prematurity, mechanical ventilation, infection and genetic predisposition contribute to vascular growth arrest, remodelling and increased pulmonary vascular resistance. Other conditions with lung hypoplasia and CLD-PH include congenital diaphragmatic hernia (CDH) and Down syndrome. Children with PH should be monitored by a specialised team experienced in cardiac monitoring, lung function/exercise testing, PH pharmacotherapy and the typical CLD-PH comorbidities. The most common therapy options include supplemental oxygen, phosphodiesterase inhibitors, endothelin receptor antagonists and prostacyclin agonists.
Conclusion:
CLD-PH in children is a complex condition that requires a thorough understanding of its aetiology, assessment methods, management strategies and supportive, family-centred care to improve outcomes for children with CLD-PH.
More Related Videos
09:22Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
10:20Right Ventricular Systolic Pressure Measurements in Combination with Harvest of Lung and Immune Tissue Samples in Mice
Published on: January 16, 2013
Related Concept Videos
01:30Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
01:20Chronic Obstructive Pulmonary Disease-I: Introduction
01:25Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.
Symptoms of COPD can be classified as primary or systemic. Primary symptoms relate to reduced airflow, while systemic or extrapulmonary symptoms relate to COPD's broader impact on the body.
Primary Symptoms of COPD:
01:22Chronic Obstructive Pulmonary Disease
Smoking is a primary risk factor for COPD, with over 80% of patients having a history of it. Patients typically experience progressive dyspnea or labored breathing, frequent coughing, and recurrent pulmonary infections. Many eventually succumb to respiratory failure, characterized by...
01:20Chronic Obstructive Pulmonary Disease-II: Pathophysiology
Chronic Inflammation
01:20COPD: Pathogenesis and Clinical Features
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...