Pulmonary Hypertension Community Call — September

The role of antithrombotic therapy in pulmonary hypertension (PH) remains uncertain, with conflicting adult data and limited pediatric evidence. This study aimed to describe real-world antithrombotic use in pediatric PH utilizing Pediatric Pulmonary Hypertension Network (PPHNet) registry data and a survey of PPHNet centers. 

Pediatric pulmonary arterial hypertension is a progressive vasculopathy with substantial early mortality. Subcutaneous treprostinil is an established prostacyclin-pathway therapy for higher-risk disease, but long-term pediatric data from Latin American referral centers remain limited.

Preterm birth survivors reaching adulthood are a growing population, with bronchopulmonary dysplasia (BPD), the chronic lung disease of prematurity, common among this cohort. Infants with BPD are at increased risk for pulmonary vascular disease (PVD) including the development of pulmonary hypertension (PH). 

Neonates and infants with FOXF1 mutation develop alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV), which is typically considered a uniformly fatal neonatal lung disorder due to marked hypoxemic respiratory failure with severe pulmonary hypertension (PH).