Key result
Bosentan therapy significantly reduced mean right ventricular systolic pressure from 79 to 52 mmHg and NT-proBNP levels from 21,071 to 2,037 in children less than two years of age with pulmonary hypertension.
Why the study?
Although bosentan has shown significant benefits in adults with pulmonary hypertension, few studies have assessed the efficacy and safety of endothelin receptor antagonists in infants and young children.
Does the addition of Bosentan to Sildenafil improve right ventricular systolic pressure and NT-proBNP levels in children less than two years of age with pulmonary hypertension?
Cohort (n=12)
No
Does the addition of Bosentan to Sildenafil improve right ventricular systolic pressure and NT-proBNP levels in children less than two years of age with pulmonary hypertension?
Absolute Event Rate: 52% vs 79%
p-value: p=<0.001
In infants and children under two years old with pulmonary hypertension, adding Bosentan to Sildenafil significantly improved right ventricular systolic pressure and NT-proBNP levels without notable adverse effects on liver function or blood counts.
Associated with RVSP and NT-proBNP reductions in young children with PH on sildenafil; leaves open randomized confirmation before practice change.
Pulmonary hypertension is a rare and potentially fatal disease in children if left untreated. Emerging therapies, including Bosentan, a dual endothelin receptor antagonist, have shown significant benefits in the adult pulmonary hypertension population; however, few studies have assessed the efficacy and safety of endothelin receptor antagonists in infants and young children. Our study was a single-center retrospective analysis of patients less than two years of age with a confirmed diagnosis of pulmonary hypertension and initiated on Bosentan therapy between 2017 and 2020. Twelve cases met eligibility criteria. Demographic data, laboratory data, echocardiographic, and cardiac catheterization data were analyzed. With treatment, there was a statistically significant decrease in mean right ventricular systolic pressure estimated by the tricuspid regurgitation jet (79 ± 23 mmHg reduced to 52 ± 25 mmHg; p < 0.001) N-terminal pro-hormone B-type natriuretic peptide levels (21,071 reduced to 2,037; p < 0.001). Additionally, improvement and eventual normalization of right ventricular function and septal geometry was seen within the first four months of therapy. Patients who underwent cardiac catheterization after therapy initiation (n = 4) demonstrated hemodynamic improvements; however, only the decrease in diastolic pulmonary artery pressure was statistically significant (p = 0.018). No significant differences in hemoglobin, platelet count, or liver function tests were observed between groups. In conclusion, these data suggest that Bosentan may be an effective and relatively safe treatment option for children less than two years of age with pulmonary hypertension. Further long-term randomized control studies are necessary to validate the potential clinical benefit of utilizing this drug therapy in young children.
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Landry et al. (2023) conducted a cohort in Pulmonary hypertension (n=12). Bosentan vs. Baseline was evaluated on Mean right ventricular systolic pressure (RVSP) estimated by tricuspid regurgitation jet (p=<0.001). Bosentan therapy significantly reduced mean right ventricular systolic pressure from 79 to 52 mmHg and NT-proBNP levels from 21,071 to 2,037 in children less than two years of age with pulmonary hypertension.
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