Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management

Area of Science:

• Pediatric Cardiology
• Pulmonology
• Genetics

Background:

• Pediatric pulmonary arterial hypertension (PAH) shares adult disease features but presents unique challenges.
• Neonatal PAH, particularly in preterm infants with lung disease, and novel genetic causes are significant concerns.

Purpose of the Study:

• To update the definition, epidemiology, classification, diagnostics, and treatment of pediatric PAH.
• To identify critical knowledge gaps and distinct approaches for managing pediatric PAH.
• To highlight recent advances and ongoing challenges in the care of children with PAH.

Main Methods:

• Review of current literature and guidelines presented by the Paediatric Task Force of the 6th World Symposium on Pulmonary Hypertension.
• Analysis of diagnostic modalities including cardiac catheterization and hemodynamic definitions.
• Evaluation of medical and interventional management strategies, including the Potts shunt.

Main Results:

• Updates provided on pediatric PAH definition, epidemiology, classification, and diagnostics.
• Emphasis on neonatal PAH, developmental lung diseases, and novel genetic etiologies.
• Discussion of cardiac catheterization, hemodynamic definitions, and management approaches, including the Potts shunt.

Conclusions:

• Despite persistent lack of clinical trial data for targeted therapies, emerging data aids in identifying appropriate targets for goal-oriented therapy in children.
• Improved identification of therapeutic targets is expected to enhance future clinical trial design and improve outcomes in pediatric PAH.
• Distinct approaches are necessary for the care of children with PAH, addressing unique disorders and challenges.