MiscellaneousEffectiveness and Safety of Inhaled Treprostinil for the Treatment of Pulmonary Arterial Hypertension in Children
Section snippets
Methods
To evaluate the safety, efficacy, and tolerability of inhaled treprostinil in children, we retrospectively reviewed the data from all patients with group 1 PAH treated with inhaled treprostinil for ≥6 weeks at 2 large pediatric pulmonary hypertension centers (Columbia University Medical Center and Children's Hospital Colorado). Indications for initiation of inhaled treprostinil therapy included symptomatic PAH on background therapy or as a strategy to transition select patients off parenteral
Results
Twenty-nine consecutive pediatric patients with Group 1 PAH who received inhaled treprostinil for ≥6 weeks were included. Baseline demographic data are listed in Table 2. There were 14 male and 15 female subjects (mean age 11.3 ± 4.5 years, median 12, range 3.2 to 19) with idiopathic PAH (n = 19) or associated PAH (n = 10). Average treatment duration was 15.7 ± 8.2 months (median 17, range 1.5 to 27.5). All patients were on background therapy. Four patients were on calcium channel blockers, 26
Discussion
Inhaled iloprost was initially studied in children with PAH in 22 pediatric patients and was found to have acceptable safety and tolerability.20 Iloprost therapy requires treatments every 2 to 4 hours, with each treatment taking 5 to 20 minutes. Delivery of inhaled iloprost can be difficult for young children because of the need for actuated breaths with the Opti-Neb device. Availability of a longer-acting inhaled prostanoid, inhaled treprostinil, has enabled patients to transition from
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Cited by (0)
This study was funded by Specialized Centers of Clinically Oriented Research Grant HL-084923-02 from the National Institutes of Health, Bethesda, Maryland, Grant UL1RR025780 from the National Institutes of Health/National Center for Research Resources Colorado CTSI, Denver, Colorado, the Jayden DeLuca Foundation, and the Leah Bult PH Research Fund, Denver, Colorado. Columbia University and University of Colorado receive consultant fees from Actelion, Gilead, Pfizer, and United Therapeutics.