Demographics, baseline characteristics and on-treatment parameters for all patients and by Down syndrome status
| Parameter | All patients (n=82) | Non-Down syndrome patients (n=53) | Down syndrome patients (n=29) |
|---|---|---|---|
| Mean±SD age at first visit, years (range) | 38.1±14.0 (18–81) | 40.3±15.9 (18–81) | 33.9±8.4 (21–53) |
| Male/Female, n (%) | 34 (41.5)/48 (58.5) | 21 (39.6)/32 (60.4) | 13 (44.8)/16 (55.2) |
| Congenital heart defect in patients with Eisenmenger syndrome, n (%) | |||
| Total | 52 (63.4) | 26 (49.1) | 26 (89.7) |
| ASD | 3 (5.8) | 3 (11.5) | 0 (0) |
| VSD | 17 (32.7) | 9 (34.6) | 8 (30.8) |
| AVSD | 16 (30.8) | 1 (3.8) | 15 (57.7) |
| PDA | 4 (7.7) | 4 (15.4) | 0 (0) |
| TGA | 1 (1.9) | 1 (3.8) | 0 (0) |
| VSD and TGA | 2 (3.8) | 2 (7.7) | 0 (0) |
| VSD and PDA | 3 (5.8) | 2 (7.7) | 1 (3.8) |
| AVSD and TGA | 1 (1.9) | 1 (3.8) | 0 (0) |
| AVSD and PDA | 2 (3.8) | 0 (0) | 2 (7.7) |
| VSD and TGA and TA | 1 (1.9) | 1 (3.8) | 0 (0) |
| Aortopulmonary window | 2 (3.8) | 2 (7.7) | 0 (0) |
| Congenital heart defect in patients without Eisenmenger syndrome, n (%) | |||
| Total | 30 (36.6) | 27 (50.9) | 3 (10.3) |
| ASD | 3 (10.0) | 3 (11.1) | 0 (0) |
| VSD | 5 (16.7) | 4 (14.8) | 1 (33.3) |
| AVSD | 4 (13.3) | 2 (7.4) | 2 (66.7) |
| PA | 1 (3.3) | 1 (3.7) | 0 (0) |
| TA | 2 (6.7) | 2 (7.4) | 0 (0) |
| TGA | 3 (10.0) | 3 (11.1) | 0 (0) |
| TrA | 1 (3.3) | 1 (3.7) | 0 (0) |
| VSD and PA | 4 (13.3) | 4 (14.8) | 0 (0) |
| VSD and TGA | 2 (6.7) | 2 (7.4) | 0 (0) |
| AVSD and TGA | 1 (3.3) | 1 (3.7) | 0 (0) |
| VSD and TA and TGA | 1 (3.3) | 1 (3.7) | 0 (0) |
| AVSD and TA and PDA | 1 (3.3) | 1 (3.7) | 0 (0) |
| Not recorded | 2 (6.7) | 2 (7.4) | 0 (0) |
| WHO Functional Class (I–IV) | III | III | III |
| Mean±SD 6MWD at baseline, m (median (range)) | 254.1±106* (270 (29–480)*) | 278±102* (281 (29–480)*) | 214±102 (210 (30–405)) |
| Mean±SD treatment duration, years (median (range)) | 2.5±1.9 (2.4 (0.0–6.9)) | 2.8±1.9 (2.6 (0.0–6.1)) | 2.0±1.8 (1.4 (0.0–6.9)) |
| Iron replacement therapy, n (%) | 11 (13.4) | 7 (13.2) | 4 (13.7) |
*Baseline 6MWD unavailable for four patients.
6MWD, 6 min walk distance; ASD, atrial septal defect; AVSD, atrioventricular septal defect; PA, pulmonary atresia; PDA, patent ductus arteriosus; TA, tricuspid atresia; TGA, transposition of the great arteries; TrA, truncus arteriosus; VSD, ventricular septal defect.