%0 Journal Article
%T Bosentan Is Associated with a Reduction in Right Ventricular Systolic Pressure N-Terminal Pro-Hormone B-Type Natriuretic Peptide Levels in Young Patients with Pulmonary Hypertension
%A Lily M. Landry
%A Andrew C. Burks
%A Onyekachukwu Osakwe
%A Jarrod D. Knudson
%A Christopher L. Jenks
%J Open Journal of Pediatrics
%P 32-42
%@ 2160-8776
%D 2023
%I Scientific Research Publishing
%R 10.4236/ojped.2023.131004
%X 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 ¡À 23 mmHg reduced to 52 ¡À 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.
%K Pulmonary Arterial Hypertension
%K Bronchopulmonary Dysplasia
%K Endothelial Receptor Antagonist
%K Congenital Heart Disease
%U http://www.scirp.org/journal/PaperInformation.aspx?PaperID=122319