What is the role of bosentan in the treatment of persistent pulmonary hypertension of the newborn (PPHN)?

Updated: Sep 03, 2019
  • Author: Kate A Tauber, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
  • Print


The endothelins (ETs) comprise a family of three 21-amino acid peptides, ET-1, ET-2, and ET-3. Of these, only ET-1 plays an important role in the regulation of vascular tone. ET-1 is a very potent vasoconstrictor and smooth muscle mutagen produced primarily by vascular endothelial cells. [57, 58]

The development of the endothelin receptor antagonists represents an important milestone in the therapeutic approach for pulmonary hypertension.

Bosentan is the first orally active treatment to show efficacy in a randomized trial of pulmonary arterial hypertension. Randomized controlled trials and systematic reviews in adults have shown that bosentan improves the outcomes of patients with pulmonary hypertension. [59, 60] A placebo-controlled trial with 47 infants diagnosed with PPHN at a center where iNO was not available found that bosentan was more effective than placebo in improving oxygenation and was well tolerated in term and late preterm neonates. [61]

There are 2 main concerns with the use of bosentan: the potential for serious hepatic injury and teratogenic effects. Monthly monitoring of liver function tests for the duration of treatment is mandatory, as approximately 10% of adult patients receiving bosentan show an increase in liver transaminase levels of 3-fold or greater. [62]

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!