Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art

Yuchen Wang; Selena Chen; Junbao Du

doi:10.3389/fped.2019.00302

Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art

Front Pediatr. 2019 Jul 23:7:302. doi: 10.3389/fped.2019.00302. eCollection 2019.

Authors

Yuchen Wang^{1

2}, Selena Chen³, Junbao Du^{1

4}

Affiliations

¹ Department of Pediatrics, Peking University First Hospital, Beijing, China.
² Department of Clinical Medicine, Peking University Health Science Center, Beijing, China.
³ Division of Biological Sciences, University of California, San Diego, La Jolla, CA, United States.
⁴ Key Laboratory of Molecular Cardiovascular Sciences, Ministry of Education, Beijing, China.

Abstract

Idiopathic pulmonary arterial hypertension (IPAH) is a complex disease associated with progressive deterioration. Targeted therapy for IPAH has improved in the last several decades. However, there remain many challenges to current treatment of children with IPAH, including poor prognosis and a median survival of 0.8 years. Endothelin-1 (ET-1) appears to be a key mediator in the pathogenesis of IPAH, with elevated concentrations in the plasma. Bosentan, an endothelin receptor antagonist, has been confirmed in Food and Drug Administration (FDA) to effectively treat IPAH when administered in recent studies. This review focuses on related studies and advance of bosentan in the treatment of IPAH in children.

Keywords: bosentan; idiopathic pulmonary hypertension; pediatrics; pharmacology; target therapy.

Publication types

Review