Treatment

Calcium channel blockers

• Nifédipine (Adalat) (60-240 mg/day).
• Amlodipine (Norvasc) (10-20 mg/day).
• Diltiazem (Dilzem) (60 -720 mg/day).

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

• Sildenafil (Revatio) (20-80 mg x 3 /day).
• Tadalafil (Cialis) 2.5-40 mg/day).
• Vardenafil (Levitra) 5 mg x 2 /day à 80 mg x 3 /day).

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).

Prostanoids

• 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]).

Surgery

Pulmonary thrombo-endarterectomy if chronic pulmonary emboli.

Lung transplantation if idiopathic PAH.