TITLE:
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
AUTHORS:
Lily M. Landry, Andrew C. Burks, Onyekachukwu Osakwe, Jarrod D. Knudson, Christopher L. Jenks
KEYWORDS:
Pulmonary Arterial Hypertension, Bronchopulmonary Dysplasia, Endothelial Receptor Antagonist, Congenital Heart Disease
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.13 No.1,
January
9,
2023
ABSTRACT: 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