Key result
A single 10 mg dose of macitentan significantly improved pulmonary hemodynamics, functional capacity, and NT-proBNP levels compared to baseline in 4 patients with late-onset pulmonary hypertension.
Why the study?
Does macitentan improve pulmonary hemodynamics and functional capacity in patients with late-onset pulmonary hypertension after atrial septal defect operation?
Case Report (n=4)
No
Does macitentan improve pulmonary hemodynamics and functional capacity in patients with late-onset pulmonary hypertension after atrial septal defect operation?
Macitentan may improve pulmonary hemodynamics and functional capacity in patients with late-onset pulmonary hypertension following atrial septal defect repair.
May support macitentan in CHD-PAH; hypothesis-generating and requires randomized confirmation.
Background: Macitentan significantly improves pulmonary hemodynamics and survival in patients with primary pulmonary hypertension (PPH). Its beneficial effect, however, may be blunted due to the adverse impacts such as anemia and peripheral edema. Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity are associated with increased morbidity and mortality. We tried to evaluate that the effectiveness of the macitentan in patients with late-onset pulmonary hypertension after atrial septal defect operation in our center. Methods: The effect of a single dose of macitentan (10 mg) on pulmonary hemodynamics, functional capacity was examined in four patients with late-onset pulmonary hypertension after atrial septal defect operation. Results: The macitentan significantly improved mean pulmonary artery pressure (MPAP), cardiac output (CO), tricuspid annular plane systolic excursion (TAPSE), right ventricle systolic wave(RVS’), 6-minute walking test and NT-proBNP levels compared with before treatment. Conclusions: Macitentan can be used in patients with late-onset pulmonary hypertension after shunt operation especially atrial septal defect.
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Tarık Kıvrak (2018) conducted a case report in Late-onset pulmonary hypertension after atrial septal defect operation (n=4). Macitentan vs. Before treatment (baseline) was evaluated on Pulmonary hemodynamics and functional capacity. A single 10 mg dose of macitentan significantly improved pulmonary hemodynamics, functional capacity, and NT-proBNP levels compared to baseline in 4 patients with late-onset pulmonary hypertension.
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