Figure 1. Treatment Algorithm for PAH
Oral anticoagulants (E/B) - IPAH/HPAH
Diuretics (E/A) Oxygen†
(E/A) Digoxin (E/C)
Supervised rehabilitation (E/B)
Expert referral (E/A)
Supportive therapy and general measures
Avoid excessive physical exertion (E/A)
Birth control (E/A)
Psychological and social support (E/C)
Infection prevention (E/A)
Acute vasoreactivity testing (A for IPAH) (E/C for APAH)
Acute responder
WHO Class I-IV
Amlodipine, diltiazem, nifedipine (B)
Sustained response
(WHO I-II) Non-responder WHO Class II WHO Class III WHO Class IV
• AmbrisentanA • BosentanA • SildenafilA • SitaxsentanB • TadalafilB
• AmbrisentanA • BosentanA • Epoprostenol IVA • Iloprost inhaledA • SildenafilA • SitaxsentanB • TadalafilB • Treprostinil subcutB
• BeraprostC • Iloprost IVEB • Treprostinil IVEB • Treprostinil inh.B
• Epoprostenol IVA • Iloprost inhaledB • Treprostinil subcutC • BosentanB • Iloprost IVEB • Treprostinil IVEB • Initial • combination • therapy (see below) • AmbrisentanEC • BosentanEC • SildenafilEC • SitaxsentanEC • TadalafilEC
YES NO Inadequate clinical response Sequential combination therapy
Inadequate clinical response
Amlodipine, diltiazem, nifedipine (B)
Prostanoids (B) PDE-5 (B) (B) ERA
Drugs within the same grade of evidence are listed in alphabetical order and not order of preference. Not all agents listed are approved or available for use in all countries.
and/or lung transplant (E/A) Atrial septostomy (E/B)
