Recommendations for efficacy of specific drug therapy, balloon atrial septostomy and lung transplantation for pulmonary arterial hypertension(group 1) according to World Health Organization functional class (WHO-FC)
| Measure/treatment | Classes of recommendation–level of evidence | |||
| WHO-FC II | WHO-FC III | WHO-FC IV | ||
| Calcium channel blockers | I–C# | I–C# | – | |
| Endothelin receptor antagonists | Ambrisentan | I–A | I–A | IIa–C |
| Bosentan | I–A | I–A | IIa–C | |
| Sitaxentan | IIa–C | I–A | IIa–C | |
| Phosphodiesterase type-5 inhibitors | Sildenafil | I–A | I–A | IIa–C |
| Tadalafil¶ | I–B | I–B | IIa–C | |
| Prostanoids | Beraprost | – | IIb–B | – |
| Epoprostenol (intravenous) | – | I–A | I–A | |
| Iloprost (inhaled) | – | I–A | IIa–C | |
| Iloprost (intravenous) | – | IIa–C | IIa–C | |
| Treprostinil (subcutaneous) | – | I–B | IIa–C | |
| Treprostinil (intravenous) | – | IIa–C | IIa–C | |
| Treprostinil (inhaled)¶ | – | I–B | IIa–C | |
| Initial drugs combination therapy | – | – | IIa–C | |
| Sequential drugs combination therapy | IIa–C | IIa–B | Iia–B | |
| Balloon atrial septostomy | – | I–C | I–C | |
| Lung transplantation | – | I–C | I–C | |
#: only in responders to acute vasoreactivity tests, I for idiopathic pulmonary arterial hypertension (PAH), heritable PAH and PAH due to anorexigens; IIa for APAH conditions; ¶: under regulatory review in the European Union.