Eur J Pediatr. 2008 Apr;167(4):437-40. Epub 2007 Aug 15.

Persistent pulmonary hypertension of the

newborn with transposition of the great arteries:
successful treatment with bosentan.
Goissen C, Ghyselen L, Tourneux P, Krim G, Storme L, Bou P, Maingourd Y.

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Abstract
Persistent pulmonary hypertension of the newborn (PPHN) occurs in 1-4% of neonates with
transposition of the great arteries with intact ventricular septum (TGA/IVS). This association is often
lethal. To our knowledge, only eight survivors have been described in the literature, two of whom
benefited from extracorporeal membrane oxygenation (ECMO). We report two cases of PPHN
complicating a TGA/IVS that were refractory to multiple therapies and resolved 48 hours after
initiation of bosentan therapy. Bosentan, an oral dual endothelin-1 receptor antagonist, is a new
treatment for pulmonary arterial hypertension that was both effective and safe in these two cases of
TGA/IVS with PPHN. To our knowledge, it is the first use of bosentan in newborns.
Acta Paediatr. 2009 Oct;98(10):1683-5. doi: 10.1111/j.1651-2227.2009.01386.x. Epub 2009 Jun 11.

Successful treatment of persistent pulmonary

hypertension of the newborn with bosentan.
Nakwan N, Choksuchat D, Saksawad R, Thammachote P, Nakwan N.

Author information
Abstract
The sophisticated and expensive treatment modalities of persistent pulmonary hypertension of the
newborn (PPHN), such as nitric oxide, are limited in developing countries. Alternative (less expensive)
treatments are being sought and bosentan, an oral dual endothelin-1 receptor antagonist, may be an
option for the treatment of PPHN. We report our experience of using bosentan in a neonate with severe
PPHN.
CONCLUSION:
Bosentan may be a useful adjuvant therapy in neonates with PPHN, providing significant improvement
in oxygenation, and thus may be particularly useful in the treatment of PPHN in countries with limited
resources.

Journal of Perinatology 32, 608-613 (August 2012) | doi:10.1038/jp.2011.157

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W A Mohamed
M Ismail

A randomized, double-blind, placebo-controlled, prospective

study of bosentan for the treatment of persistent pulmonary
hypertension of the newborn
W A Mohamed and M Ismail

Abstract
Objective:
To assess the efficacy and safety of bosentan as an adjuvant therapy of persistent pulmonary
hypertension of the newborn (PPHN).
Study Design:
Forty-seven neonates with PPHN were randomly assigned to receive either bosentan (n=24) or placebo
(n=23). Efficacy was evaluated with a favorable outcome defined as fulfilling all the following criteria
(for example, oxygenation index <15, normal pulmonary artery pressure (<20mmHg) and no
premature discontinuation of the drug because of drug-related toxicity or lack of efficacy). Evaluation
of safety was done by monitoring drug-related adverse events.
Result:
Bosentan treatment was superior to placebo with a favorable response in 87.5% of patients treated with
bosentan as compared with 20% of those who received placebo (P<0.0001). None of patients in the
bosentan group had drug-related clinical or laboratory adverse events.
Conclusion:
Bosentan may be a useful adjuvant therapy of PPHN.