Summary of CHEST/American College of Chest Physicians guidelines for pharmacological treatment of PAH18
| Patient status* | WHO FC II | WHO FC III | WHO FC IV |
|---|---|---|---|
| Treatment naïve | Monotherapy with any approved ERA, PDE5i or riociguat | Monotherapy with any approved ERA, PDE5i or riociguat | Monotherapy with parenteral prostanoid |
| Treatment naïve, unable or unwilling to receive parenteral prostanoids | – | – | Inhaled prostanoid plus ERA |
| Treatment naïve with evidence of rapid disease progression or markers of poor prognosis | – | Initial monotherapy with parenteral prostanoid or subcutaneous treprostinil | – |
| Receiving one or two oral therapies with evidence of rapid disease progression or markers of poor prognosis | – | Addition of parenteral or inhaled prostanoid | – |
| Receiving ERA or PDE5i but remains symptomatic | – | Addition of inhaled prostanoid | – |
| Receiving established monotherapy, with unacceptable clinical status | – | ▸ Add inhaled prostanoids to stable ERA/PDE5i ▸ Add sildenafil to epoprostenol ▸ Add riociguat to bosentan, ambrisentan or inhaled prostanoid ▸ Add macitentan to PDE5i | |
| Receiving dual combined therapy, with unacceptable clinical status | – | Add a third class of therapy. Patient should ideally be treated at an expert centre | |
*Pharmacotherapy is not recommended for patients with WHO FC I.
ERA, endothelin receptor antagonist; PAH, pulmonary arterial hypertension; PDE5i, phosphodiesterase 5 inhibitor; WHO FC, WHO functional class.