Successful treatment of late-onset pulmonary hypertension after atrial septal defect operation with macitentan: Our center experience

Main Article Content

Tarik Kivrak
Zeynep Ulutas
Sena Sert
Bulent Mutlu

Abstract

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.

Article Details

Kivrak, T., Ulutas, Z., Sert, S., & Mutlu, B. (2018). Successful treatment of late-onset pulmonary hypertension after atrial septal defect operation with macitentan: Our center experience. Journal of Pulmonology and Respiratory Research, 2(1), 001–003. https://doi.org/10.29328/journal.jprr.1001006
Research Articles

Copyright (c) 2018 Kivrak T, et al.

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This work is licensed under a Creative Commons Attribution 4.0 International License.

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