Table 4.
PAH-Specific Medications Among Adult PAH Patients in the Data Base of Pulmonary Hypertension in the Polish Population (BNP-PL) Registry at Enrollment.
| All Patients | Whole Sample: | I/HPAH | CTD | CHD | Portal | Drugs/Toxins | HIV |
|---|---|---|---|---|---|---|---|
| Monotherapy (n,%) | 366 (37.7) | 123 (27.7) | 58 (43.9) | 163 (45.8) | 15 (60) | 3 (42.9) | 4 (66.7) |
| -Oral (n,%) | 356 (36.7) | 117 (26.3) | 57 (43.2) | 160 (44.9) | 15 (60) | 3 (42.9) | 4 (66.7) |
| PDE5-I (n,%) | 201 | 105 | 22 | 54 | 13 | 3 | 4 |
| ERA (n,%) * | 148 | 4 | 141 | 1 | 2 | 0 | 0 |
| -Prostacyclin analogue (n,%) | 10 (1) | 6 (1.3) | 1 (0.7) | 3 (0.8) | 0 | 0 | 0 |
| Double comb. therapy (n,%) | 515 (53) | 254 (57.2) | 72 (54.5) | 173 (48.6) | 10 (40) | 4 (57.1) | 2 (33.3) |
| -Oral (n,%) * | 295 (30.4) | 118 (26.6) | 33 (27.3) | 141 (39.6) | 2 (8) | 0 | 1 (16.7) |
| -With prostacyclin analogue (n,%) | 220 (22.7) | 136 (30.6) | 39 (29.5) | 32 (8.9) | 8 (32) | 4 (57.1) | 1 (16.7) |
| Triple combination therapy (n,%) | 46 (4.7) | 29 (6.5) | 8 (6) | 9 (2.5) | 0 | 0 | 0 |
| Patients with WHO FC IV: | |||||||
| Monotherapy (n,%) | 7 (18.9) | 3 (14.3) | 0 | 4 (80) | 0 | 0 | 0 |
| -Oral (n,%) | 7 (18.9) | 3 (14.3) | 0 | 4 (80) | 0 | 0 | 0 |
| -Prostacyclin analogue (n,%) | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Double combination therapy (n,%) | 21 (56.8) | 13 (61.9) | 7 (70) | 0 | 0 | 1 (100) | 0 |
| -Oral (n,%) | 5 (13.5) | 3 (14.3) | 2 | 0 | 0 | 0 | 0 |
| -With prostacyclin analogue (n,%) | 16 (43.2) | 10 (47.6) | 5 | 0 | 0 | 1 (100) | 0 |
| Triple combination therapy (n,%) | 9 (24.3) | 5 (23.8) | 3 (30) | 1 (20) | 0 | 0 | 0 |
* Of 356 patients who used oral monotherapy 349 (98%) were receiving sildenafil or endothelin receptor antagonist. The other seven patients were receiving riociguat or selexipag which were available only in open label phases of clinical studies and were not formally reimbursed by the National Health Fund. ** Of 295 patients who used oral double combination therapy 292 (99%) patients used endothelin receptor antagonist and sildenafil, three other patients were using a combination of riociguat and bosentan, or selexipag and sildenafil. ERA—endothelin receptor antagonist, PDE5-I—phosphodiesterase type 5 inhibitor, WHO FC—World Health Organization Functional Class.