Abstract 20098: Treprostinil Improves Right Ventricular Function in Pediatric Pulmonary Hypertension
Introduction: Pulmonary hypertension (PH) is associated with significant morbidity and mortality in children due to right ventricular (RV) failure. Prostacyclin analogs improve outcomes in adult pulmonary arterial hypertension (PAH), however studies of children are limited. We sought to determine the effect of treprostinil on RV function assessed by echocardiogram in children with various forms of PH.
Methods: We conducted a retrospective cohort study of children with PH treated with treprostinil between 1/2001 and 8/2015 at our center. We collected data from baseline, 1-3 months, and 6-12 months after drug initiation. Transthoracic echocardiograms were reviewed offline using a standardized research protocol that included tricuspid annular plane systolic excursion (TAPSE) and RV global longitudinal strain (%, with more negative values indicating better systolic function). Generalized linear/logistic mixed-effects regression models were used to examine changes over time.
Results: Forty-seven subjects were included; 64% were female, 55% were white, and 17% were black. Median age at PH diagnosis was 2.4 months (range: 0 to 21.2). Disease type was idiopathic PAH (38%), PAH associated with congenital heart disease (15%), developmental lung disease (45%), and chronic thromboembolic PH (2%). Subjects received intravenous (68%), subcutaneous (17%), or inhaled (15%) treprostinil. TAPSE and RV strain were reduced at baseline (table 1). Treprostinil was associated with significant improvement in TAPSE (adjusted for growth), qualitative RV function and RV global longitudinal strain. Treprostinil decreased BNP levels, improved functional class assessment and increased 6-minute walk distance (in children able to perform testing).
Conclusions: In children with PH, treprostinil is associated with an early and sustained improvement in RV function as well as clinical markers of PH severity. RV strain may be a sensitive marker of RV function in children with PH.
Author Disclosures: R.K. Hopper: Research Grant; Significant; United Therapeutics, Inc.. Y. Wang: None. V. DeMatteo: None. S.M. Kawut: None. O. Elci: None. R. Rogers: None. L. Mercer-Rosa: Research Grant; Significant; United Therapeutics, Inc..
- © 2016 by American Heart Association, Inc.