Chronic Lung Disease-Related Pulmonary Hypertension in Children.

Abstract

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.