Pharmacological group

Medication

Dose

Phosphodiesterase 5 inhibitors

Sildenafil 

Tadalafilo

20 mg/8 h

20 mg/24 h

Endothelin receptor antagonists

Bosentan

Ambrisentan

Macitentan

125 mg/12 h

10 mg/12 h

10 mg/12 h

Prostacyclin analogues and IP receptor agonists

Selexipag

Iloprosts inhaled

Intravenous Epoprostenol

200-1.600 mcg/12 h

Start 200 mcg 2/12 h, increase of 200 mcg 12/h, with intervals of 1 week between doses. Maximum dose 1,600 mcg/12 h

2.5 - 56 mcg/inhaled/6-9 times daily

20-40 ng/kg/min

Guanylate cyclase stimulators

Riociguat

1 mg 3/8 h for 2 weeks. Increase in increments of 0.5 mg/8 h every 2 weeks to a maximum of 2.5 mg/8 h