TABLE 1.
Demographic and clinical characteristics of switch and control groups at diagnosis and baseline assessments.
| Characteristics | All | Switch | Control | p | |||
|---|---|---|---|---|---|---|---|
| N | 52 | 33 | 19 | ‐ | |||
| At diagnosis | |||||||
| Female | 37 (72) | 25 (76) | 12 (63) | 0.56 | |||
| Age (years) | 5.5 ± 3.9 | 5.4 ± 3.6 | 5.6 ± 4.4 | 0.85 | |||
| WSPH classificationb | |||||||
| 1.1: Idiopathic | 12 (23) | 8 (24) | 4 (21) | 0.97 | |||
| 1.2: Heritable | 2 (4) | 1 (3) | 1 (5) | ||||
| 1.4.3: a/w Portal hypertension | 2 (4) | 1 (3) | 1 (5) | ||||
| 1.4.4.3: a/w Co‐incidental shunt | 7 (13) | 4 (12) | 3 (16) | ||||
| 1.4.4.4: a/w Postoperative CHD | 29 (56) | 19 (58) | 10 (53) | ||||
| Co‐morbidities | |||||||
| Down syndrome | 8 (15) | 4 (12) | 4 (19) | 0.44 | |||
| Other chromosomal abnormality/associated gene variant | 4 (8) | 2 (6) | 2 (11) | 0.55 | |||
| Haemodynamicsb | n | n | n | ||||
| mPAP (mmHg) | 49 | 45.7 ± 12.1 | 30 | 44.1 ± 11.6 | 19 | 48.2 ± 12.8 | 0.27 |
| PVRi (WU.m2) | 48 | 13.5 ± 8.6 | 30 | 11.6 ± 5.9 | 18 | 16.5 ± 11.4 | 0.11 |
| At study initiation | |||||||
| Age (years) | 10.5 ± 3.6 | 11.1 ± 3.3 | 9.5 ± 4.1 | 0.17 | |||
| Sildenafil plus bosentan therapy | |||||||
| Age established (years) | 7.0 ± 3.9 | 7.0 ± 3.8 | 7.1 ± 4.2 | 0.94 | |||
| Duration of therapy (months) | 42.1 ± 35.8 | 49.2 ± 35.5 | 29.7 ± 33.7 | 0.06 | |||
| Other therapy | |||||||
| Prostanoid | 19 (37) | 11 (33) | 8 (42) | 0.77 | |||
| CCB | 4 (8) | 3 (9) | 1 (5) | 1.00 | |||
| Anthropometry | |||||||
| Weight z‐score | −0.37 ± 1.38 | −0.29 ± 1.46 | −0.51 ± 1.26 | 0.57 | |||
| Height z‐score | −0.5 ± 2.15 | −0.06 ± 1.47 | −0.65 ± 1.32 | 0.18 | |||
| WHO functional class | |||||||
| I or II | 29 (56) | 17 (52) | 12 (63) | 0.24 | |||
| III or IV | 21 (40) | 16 (48) | 5 (26) | ||||
| 6MWT | n | n | n | ||||
| Walk distance (m) | 40 | 378 ± 102 | 30 | 401 ± 92* | 10 | 308 ± 103* | <0.01 |
| Echocardiography | n | n | n | ||||
| RAA (cm2) | 30 | 13.3 ± 3.6 | 20 | 14.1 ± 3.3 | 10 | 11.5 ± 3.7 | 0.08 |
| RVSP (mmHg) | 48 | 68.9 ± 27.0 | 31 | 67.0 ± 24.5 | 17 | 72.3 ± 31.7 | 0.55 |
| TAPSE (mm) | 43 | 16.7 ± 4.7 | 30 | 15.9 ± 3.9 | 13 | 18.7 ± 5.8 | 0.13 |
| LVEI diastole | 43 | 1.33 ± 0.25 | 28 | 1.29 ± 0.21 | 15 | 1.40 ± 0.31 | 0.22 |
| LVEI systole | 44 | 1.75 ± 0.50 | 29 | 1.69 ± 0.51 | 15 | 1.88 ± 0.47 | 0.23 |
| Cardiac MRI | n | n | n | ||||
| RVEDVI (mL/m2) | 42 | 101.2 ± 36.7 | 32 | 98.9 ± 39.1 | 10 | 108.4 ± 28.1 | 0.41 |
| RVEF (%) | 42 | 51.7 ± 11.4 | 32 | 53.3 ± 8.9 | 10 | 46.5 ± 16.7 | 0.24 |
| Estimated mPAP (mmHg) | 40 | 56.0 ± 15.5 | 30 | 54.7 ± 14.7 | 10 | 59.8 ± 17.8 | 0.43 |
Note: Data represented as a number, n, percentage (%), and mean ± s.d. Statistically significant p values (<0.05) are shown in bold font.
Key: a/w., associated with; CHD, congenital heart disease; IV, intravenous; LVEI, left ventricular eccentricity index; mPAP, mean pulmonary artery pressure; MRI, magnetic resonance imaging; PVRi, pulmonary vascular resistance indexed to body surface area; RAA, right atrial area; RVEDVi, right ventricular end‐diastolic volume indexed to body surface area; RVEF, right ventricular ejection fraction; RVSP, estimate of right ventricular systolic pressure on echocardiography; s/c, sub‐cutaneous; TAPSE, tricuspid annular plane systolic excursion; WHO, World Health Organization; WSPH, World Symposium on Pulmonary Hypertension; 6MWT, 6‐min walk test.
a6th WSPH classification used. 40
First haemodynamic assessment at or after diagnosis.