Kaushik Parvathaneni, Justin J. Kochanski, Megha D. Tandel, Esther Liu, Alisa Arunamata, Jeffrey A. Feinstein, Rachel K. Hopper
Stanford University School of Medicine and Stanford University.
United States
Pulmonary Circulation
Pulm Circ 2026; 16:
DOI: 10.1002/pul2.70304
Abstract
Use of subcutaneous treprostinil (TRE) in children with pulmonary hypertension (PH) secondary to lung disease (group 3 PH) is not well described. We hypothesized that initiation of TRE is associated with improvement in PH severity and right ventricular (RV) function with minimal early side effects. We conducted a single-center retrospective analysis of children with group 3 PH treated with TRE between 2006 and 2022. We compared echocardiographic changes in PH severity and RV systolic function over the first 3-6 months of TRE and described adverse effects over the first 48 h of therapy. Forty-one patients (56% male), aged 6 days to 15 years old at the time of TRE initiation, were included. The most common associated diagnoses were bronchopulmonary dysplasia (63%), congenital diaphragmatic hernia (10%), and pulmonary hypoplasia (10%). TRE was associated with improvement in PH severity (p < 0.001), estimated by interventricular septal position at end-systole (p = 0.001), RV systolic pressure to systemic blood pressure ratio (p < 0.001), and flow direction across a simple shunt (p = 0.153). There were improvements in individual markers of RV function including tricuspid annular plane systolic excursion (p = 0.005), tricuspid annulus dilation (p = 0.004), RV global longitudinal strain (p < 0.001), RV free wall longitudinal strain (p < 0.001), and qualitative RV systolic function (p = 0.002). Over the first 48 h of TRE, the dose was reduced in 3 patients (7%) due to hypotension, hypoxemia, or emesis, all of whom tolerated re-escalation within 48 h. TRE is well tolerated and is associated with early improvement in PH severity and RV function in children with group 3 PH.
Category
Class III. Pulmonary Hypertension Associated with Lung Disease
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Medical Therapy. Efficacy or Lack of Efficacy
Medical Therapy. Adverse Effects or Lack of Adverse Effects
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes