Direct prostacyclin transition in pediatric patients with pulmonary hypertension.

Pediatric patients with pulmonary arterial hypertension (PAH) are commonly treated with the prostacyclin analog treprostinil in IV, SQ, inhaled or oral form, or the prostacyclin receptor agonist selexipag. Patients who transition between these medications often follow recommendations for gradual up- and down-titrations that take place over several days in the hospital or several weeks as an outpatient. However, hospital resources are limited, and long transitions are inconvenient for patients and families. We report a case series of eight pediatric patients with PAH transitioned directly between prostacyclins with no overlapping doses. Direct medication transitions occurred in the cardiac intensive care unit (CICU), at home and in cardiology clinic. Equivalent doses for selexipag were estimated using information extrapolated from experience, published materials and selexipag study guidelines. All patients completed direct transition as planned and remained on transition dose for at least 1 week. In most cases selexipag was up-titrated at home after establishing initial transition dose. In select patients, direct prostacyclin transition in pediatric patients with PAH is safe, effective, convenient for families and reduces the use of hospital resources.

A multidisciplinary approach to severe bronchopulmonary dysplasia is associated with resolution of pulmonary hypertension.

Objective: To describe our multidisciplinary bronchopulmonary dysplasia (BPD) consult team's systematic approach to BPD associated pulmonary hypertension (PH), to report our center outcomes, and to evaluate clinical associations with outcomes. Study design: Retrospective cohort of 60 patients with BPD-PH who were referred to the Seattle Children's Hospital BPD team from 2018 to 2020. Patients with critical congenital heart disease were excluded. Demographics, comorbidities, treatments, closure of hemodynamically relevant intracardiac shunts, and clinical outcomes including time to BPD-PH resolution were reviewed. Results: Median gestational age of the 60 patients was 25 weeks (IQR: 24-26). 20% were small for gestational age (SGA), 65% were male, and 25% received a tracheostomy. With aggressive cardiopulmonary management including respiratory support optimization, patent ductus arteriosus (PDA) and atrial septal defect (ASD) closure (40% PDA, 5% ASD, 3% both), and limited use of pulmonary vasodilators (8%), all infants demonstrated resolution of PH during the follow-up period, including three (5%) who later died from non-BPD-PH morbidities. Neither SGA status nor the timing of PH diagnosis (<36 vs. ≥36 weeks PMA) impacted the time to BPD-PH resolution in our cohort [median 72 days (IQR 30.5-166.5)]. Conclusion: Our multidisciplinary, systematic approach to BPD-PH management was associated with complete resolution of PH with lower mortality despite less sildenafil use than reported in comparable cohorts. Unique features of our approach included aggressive PDA and ASD device closure and rare initiation of sildenafil only after lack of BPD-PH improvement with respiratory support optimization and diagnostic confirmation by cardiac catheterization.