Abstract Body (Do not enter title and authors here): Introduction/Background:
Pulmonary hypertension (PH) is a frequent complication of congenital heart disease (CHD) that is estimated to account for 30-50% of pediatric group 1 PH patients, as defined by the World Symposium on Pulmonary Hypertension. Despite advancements in the field, many of the targeted therapies are understudied in the pediatric CHD population. While prior studies examined practice patterns in dedicated PH programs, medication use across pediatric heart centers with varying access to PH specialists and resources remains unclear.
Research Questions/Hypothesis:
Institutional site membership to Pediatric Pulmonary Hypertension Network (PPHNet) influences practice patterns in PH medication use in acute care cardiology units at participating Pediatric Acute Care Cardiology Collaborative (PAC³) centers.
Methods/Approach:
PAC³ registry data (2019–present) were analyzed for patients admitted to a participating ACCU with a PH diagnosis or who received new or chronic PH therapy during a PAC³ encounter. Centers were grouped by institutional site membership to PPHNet. Summary and comparative statistics were performed.
Results/Data:
There were 10,223 encounters from 4,468 unique patients (94% with CHD) across 34 PAC³ centers; 9 centers (26%) were PPHNet members, accounting for 41% of encounters. Phosphodiesterase-5 inhibitors (PDE5-i) were the most used PH medication, followed by endothelin receptor antagonists (ERAs). PDE5-i monotherapy was the most common treatment regimen (81.7% of encounters), followed by dual therapy with PDE5-i + ERA (9.6%) and triple therapy with PDE5-i + ERA + prostacyclin (4.6%). Among patients with CHD, 2.4% involved triple therapy. Compared to non-PPHNet centers, PPHNet centers used fewer PDE5-i but more ERAs and prostacyclins (Table 1). PPHNet centers exhibited a higher frequency of dual and triple PH therapies compared to non-PPHNet centers, while monotherapy was more prevalent in non-PPHNet centers (Table 2).
Conclusions:
PH monotherapy was more common in non-PPHNet participating centers while multi-drug PH regimens were more frequently used at PPHNet participating centers. Practice variations exist in the use of PH medications in participating PAC³ centers who are PPHNet institutional site members compared to non-PPHNet members, suggesting differing practice patterns in centers with a larger PH presence.