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=ASIA6AP2G7AKLVYMQ3W3%2F20251210%2Feu-central-1%2Fs3%2Faws4_request&X-Amz-Date=20251210T221058Z&X-Amz-Expires=3600&X-Amz-Security-Token=IQoJb3JpZ2luX2VjEBUaDGV1LWNlbnRyYWwtMSJIMEYCIQC%2B07jPnMLk9F9kwxQcbNONyNDy0vpUDdrVbp1KMMQWswIhAMJOVXiWXI05Nfw%2FZW40jYqY6rfJC2a4q3GdpGYsySC8KsUFCN7%2F%2F%2F%2F%2F%2F%2F%2F%2F%2FwEQAhoMOTYzMTM0Mjg5OTQwIgz1JiswwRNE5CE7jrUqmQWmKimdZrrvuneTOboMH%2B3KHSOQeUMj7SqX6mW0pO14Q0HpvEgqyHGtECgm58rEvFvqX0zL0Bg1%2BZ%2Fxmws81JpKkStVASntUiMjriKAcAfZIl32nfykWRp5mmoIoFp81ANKTK23OT97LeitajKHAv2yZzvti3V6%2FzVUCxlV1sBaptTZcjRY8T0HBfIAyzNvXPbOXe%2FpdhtyvKdk0JrEUN6hkMsw9xVyoFKVDugs1BhWfg3px%2B0deB4flvGXhFqG8xmdc6hH9N1lrxgawDwQPEfMthVQlHZlubWm19euceYtz3XyKhgjPcz97czD2cu2t67LqESr0gzFB1loErdHawxMKDpvQhfFIdMYyf%2FBY1lCf906ev7LJBfT5drD1RQu70OD9FwCwU32wtCUZYdHmFDrtpXB1N6ASYeb3y7FvuUemu7c65WfB23VW9Y1kBulcmn%2BFTAAagiXIkK%2BoTlV1UAqZBdz5NPAlv%2FbdAfevZxlicsvhQeZXqt0o6bffnPzbbwr%2BeGY%2FkRVLAcDuDy6Zs5cLgknW%2FokwQxoEWt%2FJO1e%2BQ738e%2Ba6HHIpfoZSZdSloE4AN2YbWnd5Ec9%2BgSNshl6AyHI3HYFqj6fI5WUJ3vGGEmY3nvrPsDPud3nC94v%2BngW7kj%2FVDvMfZ7%2FmWOrn%2FDH7yCF30KP4%2BBzZgQo%2FcmgJ5gpvSXLlo97hym0s3e%2Fjfccxiqe3S%2FU4FzEc7HJsN9bx5ruMsN1p2Veq3kRjnG2MkiV9fO9K0i634XJJIVCT0If1PqnXSJpB5xhtawvh%2BfgsqwcOCkt8ysBlo9P3Q1IzTmCH%2FupikY8gdy%2BjX3IBLkQlX1XHa0y79EG%2F7Og%2BmMopjBwIBTPLzlfiN81EhZn9w25Ks0t6jm7DTCCu%2BfJBjqwAV9ojuN%2Btx6Nig9VkG%2BObn6XR2GW0QRGI50e3wb5wbmV2euqzCm6c0Lo0FGRxGkTgOK0FWSUeI%2BPwOp6ROaZCy5s3RYiRVI0AFyYdKgKSpKycGyWLGG5FfApLNK%2FW13nu7AmDs2%2BG8GoTvcGrb2cQlt2bqIaH3cBc7NHLMSfBWQ7wEHeitNYgTuYSNCP7eJQsnYutyaO8%2FgB7cJVvNL6yp%2Bkm9oojWb%2FDxAxugEqTvUP&X-Amz-Signature=965466477e9531252e82c65c473329ef0f83ded07bd3fab4e4f6b99453ebf393&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 | |