Abstract

Research Article

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

Tarik Kivrak*, Zeynep Ulutas, Sena Sert and Bulent Mutlu

Published: 17 August, 2018 | Volume 2 - Issue 1 | Pages: 001-003

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.

Read Full Article HTML DOI: 10.29328/journal.jprr.1001006 Cite this Article Read Full Article PDF

Keywords:

Macitentan; Atrial septal defect; Acquired pulmonary hypertension

References

  1. Kidd L, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, et al. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation. 1993; 87: I38-151. Ref.: https://tinyurl.com/y8sn2nmy
  2. Engelfriet PM, Duffels MG, Möller T, Boersma E, Tijssen JG, et al. Pulmonary arterial hypertension in adults born with a septal heart defect: the Euro Heart Survey on adult congenital heart disease. Heart. 2007; 93: 682-687. Ref.: https://tinyurl.com/y7p9fj4s
  3. Duffels MG, Engelfriet PM, Berger RM, van Loon RL, Hoendermis E, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007; 21: 198-204. Ref.: https://tinyurl.com/y7xyzofa
  4. Galie N. ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2015; 46: 903-975.
  5. Bolli MH, Christoph Boss, Christoph Binkert, Stephan Buchmann, Daniel Bur, et al. The discovery N-[5-(4-bromophenyl)-6-[2-[(5-bromo-2-pyrimidinyl)oxy]ethoxy]-4-pyrimidinyl]-N’-propylsulfamide (macitentan), an orally active, potent, dual endothelin receptor antagonist. J Med Chem. 2012; 55: 7849-7861. Ref.: https://tinyurl.com/ycdbnrz3
  6. Iglarz M, Binkert C, Morrison K, Fischli W, Gatfield J, et al. Pharmacology of macitentan, an orally active tissue-targeting dual endothelin receptor antagonist. J Pharmacol Exp Ther. 2008; 327: 736-745. Ref.: https://tinyurl.com/yb9b767o
  7. Gatfield J, Mueller Grandjean C, Sasse T, Clozel M, Nayler O. Slow receptor dissociation kinetics differentiate macitentan from other endothelin receptor antagonists in pulmonary arterial smooth muscle cells. PLoS One. 2012; 7: e47662. Ref.: https://tinyurl.com/yd7xun9p
  8. Tomás Pulido, Adzerikho I, Channick RN, Delcroix M, Galiè N, et al. Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension. N Engl J Med. 2013; 369: 809-818. Ref.: https://tinyurl.com/ybu5tguz
  9. Bedan M, Grimm D, Wehland M, Simonsen U, Infanger M, et al. A Focus on Macitentan in the Treatment of Pulmonary Arterial Hypertension. A Focus on Macitentan in the Treatment of Pulmonary Arterial Hypertension. Basic Clin Pharmacol Toxicol. 2018; 123: 103-113. Ref.: https://tinyurl.com/ycmget86
  10. Galiè N, Simonneau G, Barst RJ, Badesch D, Rubin L. Clinical worsening in trials of pulmonary arterial hypertension: results and implications. Curr Opin Pulm Med 2010; 16: 11-19. Ref: https://tinyurl.com/ycx5ftxe
  11. Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997; 336: 111-1117. Ref.: https://tinyurl.com/yd42u2qu

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