Current Hypertension Reports

, Volume 15, Issue 6, pp 614–622

An Update on Medical Therapy for Pulmonary Arterial Hypertension

Pulmonary Hypertension (Z-C Jing, Section Editor)

DOI: 10.1007/s11906-013-0394-8

Cite this article as:
Wu, Y., O’Callaghan, D.S. & Humbert, M. Curr Hypertens Rep (2013) 15: 614. doi:10.1007/s11906-013-0394-8


Over the past 20 years, great progress has been made in the treatment of pulmonary arterial hypertension (PAH). Available therapies target one of three principal pathways: the endothelin (ET), nitric oxide (NO) or the prostacyclin (PGI2) pathway. Evidence shows that current drugs, used either as monotherapy or in different combinations, can improve exercise capacity, clinical symptoms, hemodynamics and even survival in PAH. Unfortunately, the disease remains incurable and the prognosis of the disease is still poor. However, existing and novel potent antiproliferative therapies are being explored, and new agents targeting different and/or additional pathways are likely to become available to clinicians in the near future. Promising candidates include tyrosine kinase antagonists (e.g. imatinib); soluble guanylate cyclase stimulators (riociguat); an oral analog of prostacyclin (selexipag); and a tissue targeting endothelin receptor antagonist (macitentan). Phase II or III trials have either been completed or are underway to evaluate the safety and efficacy of these various therapies.


Pulmonary arterial hypertensionProstanoidsPhosphodiesterase type-5 inhibitorEndothelin receptor antagonistTyrosine kinase antagonistSelective serotonin receptor inhibitorVasoactive intestinal peptidRho-kinase inhibitorCombination therapy

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Yan Wu
    • 1
  • Dermot S. O’Callaghan
    • 2
  • Marc Humbert
    • 3
    • 4
    • 5
  1. 1.Thrombosis Medicine Center, State Key Lab of Cardiovascular DiseaseFu Wai Hospital & National Center for Cardiovascular DiseaseBeijingChina
  2. 2.Department of Respiratory MedicineMater Misericordiae University HospitalDublin 7Ireland
  3. 3.University Paris-Sud, Faculté de MédecineLe Kremlin-BicêtreFrance
  4. 4.AP-HP, Service de PneumologieDHU Thorax Innovation, Hôpital BicêtreLe Kremlin-BicêtreFrance
  5. 5.LabEx LERMITINSERM U999Le Kremlin-BicêtreFrance