Baseline 6MWD, treatment duration and concomitant iron replacement therapy by treatment regimen
| Treatment regimen | ||||
|---|---|---|---|---|
| Bosentan monotherapy | Sildenafil monotherapy | Dual therapy | ||
| Monotherapy period | Dual therapy period | |||
| Patient group | ||||
| All patients, n | 54 | 16 | 12 | |
| Non-Down syndrome patients, n | 34 | 9 | 10 | |
| Down syndrome patients, n | 20 | 7 | 2 | |
| Parameter | Mean±SD 6MWD at baseline, m (median (range)) | |||
| All patients (n=82) | 268±109* (281 (29–480)*) | 235±95 (254 (30–370)) | 208±98† (210 (60–352)†) | |
| Non-Down syndrome patients (n=53) | 287±112* (296 (29–480)*) | 270±81 (270 (150–370)) | 246±73† (230 (165–352)†) | |
| Down syndrome patients (n=29) | 236±97 (238 (71–405)) | 190±99 (197 (30–300)) | 74.5±20.5 (75 (60–89)) | |
| Mean±SD Treatment duration, years (median (range)) | ||||
| All patients (n=82) | 2.7±1.8 (2.5 (0.1–6.9)) | 1.1±1.2 (0.6 (0.0–3.7)) | 2.4±1.5 (2.2 (0.2–4.4)) | 1.4±1.3 (1.0 (0.0–3.8)) |
| Non-Down syndrome patients (n=53) | 3.0±1.8 (3.2 (0.1–6.1)) | 1.1±1.2 (0.6 (0.0–3.6)) | 2.2±1.6 (1.9 (0.2–4.4)) | 1.5±1.4 (1.0 (0.0–3.8)) |
| Down syndrome patients (n=29) | 2.1±1.8 (1.7 (0.1–6.9)) | 1.1±1.4 (0.5 (0.0–3.7)) | 3.0±0.4 (3.0 (2.8–3.3)) | 0.8±0.6 (0.8 (0.4–1.2)) |
| Iron replacement therapy, n (%) | ||||
| All patients (n=82) | 7 (13.0) | 0 | 4 (33.3) | |
| Non-Down syndrome patients (n=53) | 4 (11.8) | 0 | 3 (30.0) | |
| Down syndrome patients (n=29) | 3 (15.0) | 0 | 1 (50.0) | |
*Baseline 6MWD unavailable for one patient.
†Baseline 6MWD unavailable for three patients.
6MWD, 6 min walk distance.