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Treprostinil Is Associated With Early and Sustained Improvement in Group 3 Pulmonary Hypertension and Right
Kaushik Parvathaneni1, Justin J Kochanski1, Megha D Tandel2
1Divison of Cardiology, Department of Pediatrics Stanford University School of Medicine Palo Alto CA USA.
Insights
Subcutaneous treprostinil (TRE) improves pulmonary hypertension (PH) severity and right ventricular (RV) function in children with group 3 PH. This treatment is well-tolerated with minimal early side effects, showing early benefits in pediatric patients.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Cardiopulmonary Medicine
Background:
- Pulmonary hypertension (PH) in children, particularly group 3 PH secondary to lung disease, presents unique challenges.
- The use of subcutaneous treprostinil (TRE) in this pediatric population is not extensively documented.
- Understanding TRE's efficacy and safety is crucial for managing pediatric group 3 PH.
Purpose of the Study:
- To evaluate the impact of subcutaneous treprostinil (TRE) on PH severity in children with group 3 PH.
- To assess changes in right ventricular (RV) function following TRE initiation.
- To describe the early adverse effects associated with TRE therapy in this cohort.
Main Methods:
- Retrospective analysis of pediatric patients with group 3 PH treated with subcutaneous TRE.
- Comparison of echocardiographic parameters assessing PH severity and RV systolic function before and after TRE initiation (3-6 months).
- Documentation and analysis of adverse events occurring within the first 48 hours of TRE therapy.
Main Results:
- Subcutaneous TRE was associated with significant improvements in PH severity markers, including interventricular septal position and RV systolic pressure to systemic blood pressure ratio.
- TRE initiation led to notable enhancements in RV function, evidenced by improvements in tricuspid annular plane systolic excursion, RV global longitudinal strain, and qualitative RV systolic function.
- TRE was well-tolerated, with only a small percentage of patients requiring dose reduction for transient side effects like hypotension or emesis within the first 48 hours.
Conclusions:
- Subcutaneous treprostinil is a safe and effective treatment option for children diagnosed with group 3 pulmonary hypertension.
- Early initiation of TRE demonstrates significant improvements in both PH severity and right ventricular function.
- The findings support the use of TRE as a therapeutic strategy in pediatric patients with lung disease-related pulmonary hypertension.
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.
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