Figure 1
![Treatment algorithm in pulmonary arterial hypertension (modified from [20]).
ASI, activin signaling inhibitors; ERA, endothelin-1 receptor antagonists; PAH, pulmonary arterial hypertension; PDE5i, phosphodiesterase-5 inhibitors; i.v., intravenous; s.c., subcutaneous; PPA, prostacyclin pathway agents; sGCS: soluble guanylyl cyclase stimulators.](https://sciendo-parsed.s3.eu-central-1.amazonaws.com/647365084e662f30ba53c685/j_rjc-2025-0035_fig_001.jpg?X-Amz-Algorithm=AWS4-HMAC-SHA256&X-Amz-Content-Sha256=UNSIGNED-PAYLOAD&X-Amz-Credential=ASIA6AP2G7AKNOTHHMRS%2F20260127%2Feu-central-1%2Fs3%2Faws4_request&X-Amz-Date=20260127T000743Z&X-Amz-Expires=3600&X-Amz-Security-Token=IQoJb3JpZ2luX2VjEID%2F%2F%2F%2F%2F%2F%2F%2F%2F%2FwEaDGV1LWNlbnRyYWwtMSJHMEUCIBKmEbxkSV9hf%2BdaW108AGfI5G0KqyAIasH9lVp0uDISAiEAy8iv72XAzzGf4iQrdCJgzPJfQqxspUWjd9AO%2Fn2tR7UqvQUISRACGgw5NjMxMzQyODk5NDAiDEX6xqQ1%2BKoJye7HhSqaBcmDq2oVHHm%2ByzW66qDO1bDqZ39V1ppteHE0HTmm%2Bp%2FKtfdpj07W85rTQnMXroEhWnbcwEW9VCsWeoHds%2BUwclaskjR5E3D69QyNilGStYgId1hZ4NVP50wISKYfGMSMHTVoKIzBpYo3xAQLJsyZkuDaXQVe1KUZ1wR76Z2xOTb2UDFkjwOioCRBTZfhmdj7a5zhYOXFApc9d9beBYpNJJeZqubJ7NcFy%2FWVQeo7KOtFEDzDRmD0g14FYXCe%2BTUTKz5JxT%2BGw%2FaeEulq9xOdeMN%2BDHXsbMJKCCsYmi8kNoZaL7a%2FE%2BL9PcusdUZeaJxuAF5n9eDwoDnuvYspf2ysXOIO9yia8J5dq2HSDy%2BIgiAffU86El5Y5AoF3ODrWfFr%2BxEy4HMYO5g%2BdmwezZjXGbeNCPeo4SNBEhRSN%2FDv3LJ4ktnpc8FWT%2FTGPgP23RPnRrCbcGXDITc9vduHWN2pC1buevSjreY3uZOVnzttmEkXaNly8HrRR7%2FSZcaCIoaPLzQdp%2FGPq8wHU%2BYdeXiP2YGA1i%2B6jptpUpHnq5MxDODsDPW1G6Zg2dLvLu%2FsHxJKzhNxBFh0wC4MQi0qyQnTGOT2QiAO%2BLVkkvJVZA2y%2BD1P4fdLOE5KOEsaa7pHnpe7s5MB7%2BwHKCfPJRBNY1dnqZAVhvHHsMdMJEgunUCPyeV5gQWoNE1r9bKN0zaF9ZScnlANlbsmOZsU%2FYPG3BGRXKMLPB0NgmA3VKEms74thCsSd%2BE3O5AfBjOnWwWmTjezf7YiVdzIJFYapluMSPM%2F49D3p1wWCKvuY92CmW6m%2BKpDSA2Uq0uMjJGjA6qUEp3rOSCs6QrSIxOB%2FdY7RHLCCwpnr5BzIt13EuAjxt5SPQblAsZf6nrOPxJGzTC6geDLBjqxAcRs2ghfMLcd9AsSNEQiSzxu%2FfsaBlXzz4K21XJyxvWTj%2BD4O1AVYQYDLw%2BLKjf8pz7NO6eUfh1RO3M%2FE9cpjEjjInByBVD7MYcxn%2FLxTd0GuJndSlnnoxbvw0dshGv42vx3Mv2Q%2F8AFVDvCGHzcG%2FCv65DIe5tScZ%2Fjgc4OlA5jXgDNJg%2FQC9lcG628QgpysdHWcW0CZa3liUzlCeEvOoZ8eWKvuCZrV5IEzPDxw8S54A%3D%3D&X-Amz-Signature=105e6bac41e19fa8d49f2ed4c2ced5c66b06a8c43f4d860c9d1858531e97ffa4&X-Amz-SignedHeaders=host&x-amz-checksum-mode=ENABLED&x-id=GetObject)
The main drugs administred for pulmonary arterial hypertension treatment in Romania_
| Class of drugs | Availabledrugs | Doses |
|---|---|---|
| Calcium channel blockers | Diltiazem | 60–360 mg b.i.d. |
| Amlodipine | 5–30 mg o.d. | |
| Felodipine | 5–30 mg o.d. | |
| Nifedipine | 10–60 mg t.i.d. | |
| Endothelin receptor antagonists | Bosentan | 62.5–125 mg b.i.d. |
| Macitentan | 10 mg o.d. | |
| Phosphodiesterase 5 inhibitors | Sildenafil | 20 mg t.i.d. |
| Soluble guanylate cyclase stimulator | Riociguat | 1–2.5 mg t.i.d. |
| Prostacyclin receptor agonist | Selexipag | 200–1600 μg b.i.d. |
| Prostacyclin analogues | Treprostinil s.c. | 1.25 ng/kg/min–maximum tolerated dose |
Risk stratification in pulmonary arterial hypertension (modified from [1, 20])_
| Initial risk assessment (3 risk-strata) | |||||
|---|---|---|---|---|---|
| Determinants of prognosis | Low risk (<5%) | Intermediate risk (5–20%) | High risk (>20%) | ||
| Clinical parameters | Signs of right heart failure | Absent | Absent | Present | |
| Symptom progression | No | Slow | Rapid | ||
| Syncope | No | Occasional | Repeated | ||
| WHO-FC | I, II | III | IV | ||
| Exercise tests | 6MWD | >440 m | 165–440 m | <165 m | |
| CPET | peak V′O2 | >15 mL×kg−1×min−1 | 11-15 mL×kg−1×min−1 | <11 mL×kg−1×min−1 | |
| V′E/CO2 | <36 | 36–44 | >44 | ||
| Biomarkers | BNP | <50 ng/L | 50–800 ng/L | >800 ng/L | |
| NT-proBNP | <300 ng/L | 300–1100 ng/L | >1100 ng/L | ||
| Imaging | TEE | RA area | <18 cm2 | 18–26 cm2 | >26 cm2 |
| TAPSE/sPAP | >32 mm/mmHg | 19–32 mm/mmHg | <19 mm/mmHg | ||
| PE | No | Minimal | Moderate or large | ||
| cMRI | RVEF | >54% | 37–54% | <37% | |
| SVi | >40 mL/m2 | 26–40 mL/m2 | <26 mL/m2 | ||
| RVESVi | <42 mL/m2 | 42–54 mL/m2 | >54 mL/m2 | ||
| Hemodynamics | RAP | < 8 mmHg | 8–14 mmHg | >14 mmHg | |
| CI | >2.5 L×min−1×m−2 | 2.0-2.4 L×min−1×m−2 | <2.0 L×min−1×m−2 | ||
| SVi | >38 mL/m2 | 31–38 mL/m2 | <31 mL/m2 | ||
| SvO2 | >65% | 60–65% | <60% | ||
| Risk assessment at follow-up (4 risk-strata) | |||||
| Determinants of prognosis | Low | Intermediate-low | Intermediate-high | High | |
| WHO-FC | I, II | III | IV | ||
| 6MWD | >440 m | 320–440 | 164–319 | <165 m | |
| BNP | <50 ng/L | 50–199 ng/L | 200–800 ng/L | >800 ng/L | |
| NT–proBNP | <300 ng/‘l | 300–649 ng/L | 650–1100 ng/L | >1100 ng/L | |