Table 4.
Pharmacotherapy of pediatric patients with Pulmonary Arterial Hypertension in the BNP-PL Registry at enrollment.
| Whole Group (IPAH, CHD-PAH, PoPAH) | IPAH | CHD-PAH | P | |
|---|---|---|---|---|
| N = 80 | N = 25 | N = 54 | ||
| PAH-specific therapies: | ||||
| Sildenafil [n,%] | 57 (71%) | 18 (72%) | 38 (70.4%) | 0.88 |
| Tadalafil [n,%] | 1 (1.3%) | 0 | 1 (1.9%) | 0.50 |
| Bosentan [n,%] | 61 (76.3%) | 17 (68%) | 44 (81.5%) | 0.29 |
| Treprostinil [n,%] | 3 (3.75%) | 3 (12%) | 0 | 0.009 |
| Epoprostenol [n,%] | 0 | 0 | 0 | - |
| Riociguat [n,%] | 1 (1.3%) | 1 (4.0%) | 0 | 0.14 |
| Ca blocker for PAH | 2 (2.5%) | 2 (8.0%) | 0 | |
| PAH-specific monotherapy [n,%] | 36 (45%) | 10 (40%) | 24 (44.4%) | 0.71 |
| PAH-specific combination therapy of two drugs [n,%] | 39 (49%) | 10 (40%) | 29 (53.7%) | 0.7 |
| Triple combination PAH-specific therapy [n,%] | 3 (3.8%) | 3 (12%) | 0 | 0.01 |
| Home oxygen therapy | 2 (2.5%) | 1 (4%) | 1 (1.9%) | 0.57 |
| Vitamin K antagonists | 12 (15%) | 6 (24%) | 6 (11.1%) | 0.14 |
| Low-molecular heparin | 1 (1.3%) | 1 (4%) | 0 | 0.14 |
| Beta blockers | 6 (7.5%) | 3 (12%) | 3 (5.6%) | 0.32 |
| ACEI | 17 (21.3%) | 4 (16%) | 13 (24.1%) | 0.42 |
| ARB | 1 (1.3%) | 0 | 1 (1.9%) | 0.49 |
| Loop diuretics | 10 (12.5%) | 3 (12%) | 7 (13%) | 0.9 |
| Thiazide diuretics | 1 (1.3%) | 0 | 1 (1.9%) | 0.49 |
| Potassium-sparing diuretics | 40 (50%) | 15 (60%) | 25 (46.3%) | 0.02 |
| SSRI | 1 (1.3%) | 0 | 1 (1.9%) | 0.49 |
| ASA | 10 (12.5%) | 1 (4%) | 9 (16.7%) | 0.12 |
| Proton pomp inhibitors | 1 (1.3%) | 1 (4%) | 0 | 0.14 |
| Immunosuppressive drugs | 1 (1.3%) | 0 | 1 (1.9%) | 0.49 |
ACEI, angiotensin convertase enzyme inhibitor; ASA, acetylsalicylic acid; ARB, angiotensin receptor antagonist; SSRI, serotonin reuptake inhibitor; PAH, pulmonary arterial hypertension.