Calcium channel blockers

Endothelin Receptor Antagonist

  • Bosentan (Tracleer) (62.5 mg/day x 2/day puis 125 mg x 2 /day).
  • Ambrisentan (Volibris) (5 mg/day puis 10 mg/day).
  • Macitentan (Opsumit) (10 mg/day).

Phosphodiesterase inhibitors

Guanylate cyclase stimulator

If thromboembolic disease and contra-indication to surgery.

  • Riociguat (Adempas) (0.5 mg x 3 mg/day up to 2.5 mg x 3/day).


  • Epoprostenol (Flolan) (IV 2-4 ng/kg/min, then according tolerance tolérance).
  • Treprostinil (Remodulin) (SC or IV 1-2 ng/kg/min, then according tolerance tolérance).
  • Iloprost (Ilomedin) [IV 0,5 ng/kg/min, then according tolerance], Iloprost (Ventavis) [inhal. 2,5-5 µg up to 6-9x/day]).


Pulmonary thrombo-endarterectomy if chronic pulmonary emboli.

Lung transplantation if idiopathic PAH.