Table 2.
WHO functional class of pediatric IPAH patients treated by bosentan.
| Numbers | F/M | Age, y | Treatment | WHO functional class before treatment | WHO functional class after treatment | References |
|---|---|---|---|---|---|---|
| 8 | 4/4 | 12.8 (8.5–17.8) | Bosentan add-on after epoprostenol for >1 year. | The mean class before treatment is 2.3 WHO II: n = 6 WHO III: n = 2 |
The mean class after treatment is 2.0 WHO I: n = 2 WHO II: n = 4 WHO III: n = 2 |
Ivy et al. (37) |
| 86 | 49/37 | 11.0 (0–18.0) | Bosentan concomitant Prostanoid (n = 42) Bosentan (n = 44) |
WHO I: n = 6 WHO II: n = 34 WHO III: n = 32 WHO IV: n = 6 |
Improved one class: n = 36, remained stable: n = 34, worsened one class: n = 8 (78 out of 86 patients had WHO assessments) | Rosenzweig et al. (40) |
| 20 | 15/5 | 8.0 (1.2–17.0) | Bosentan | WHO II: n = 1 WHO III: n = 11 WHO IV: n = 8 |
Improved one class: n = 8, remained stable: n = 9, worsened one class: n = 2 | Maiya et al. (34) |
| 7 | 3/4 | 9.6 (1.0–16.0) | Sildenafil (n = 5) Bosentan (n = 2) |
WHO II: n = 1 WHO III: n = 5 WHO IV: n = 1 |
All of them are in Class I or II | Raposo-Sonnenfeld et al. (35) |
| 36 | 15/21 | 7.0 (2.0–22.0) | Bosentan | WHO II: n = 23 WHO III: n = 13 |
Improved one class: n = 5 remained stable: n = 29, worsened one class: n = 1 WHO I: n = 2 WHO II: n = 23 WHO III: n = 10 |
Beghetti et al. (25) |
| 64 | 26/38 | 4.3 (1.5–8.9) | Bosentan (n = 23) Prostanoid (n = 15) Sildenafil (n = 9) combined therapy (n = 11) Calcium channel antagonists (n = 6) |
WHO II: n = 12 WHO III: n = 34 WHO IV: n = 18 |
They all get improved, and the mean class after treatment is 3.0 | Moledina et al. (36) |
| 36 | 16/20 | 10.5 (1.0–16.0) | Bosentan (n = 11) Bosentan concomitant prostanoid (n = 25) |
WHO I: n = 3 WHO II: n = 12 WHO III: n = 16 WHO IV: n = 3 |
Improved one class: n = 14, remained stable: n = 12, worsened one class: n = 6 (32 out of 36 patients had assessments) | Ivy et al. (38) |
| 42 | 26/16 | 9.7 (no data) | Bosentan | Mean class: 2.9 WHO I: n = 2 WHO II: n = 8 WHO III: n = 25 WHO IV: n = 7 |
The mean class is 2.4 No detailed data | Hislop et al. (16) |
| 36 | 21/15 | 6.8 (2.0–12.0) | Bosentan | WHO II: n = 17 WHO III: n = 11 |
Improved one class: n = 11, remained stable: n = 15, worsened one class: n = 2 | Berger et al. (39) |
F, Female; M, Male.