Characteristics of control subjects and PAH patients studied
| Control (non-PAH) (n=8) | PAH (n=8) | P value | |
| Demographics | |||
| Age, years (mean, range) | 6.4 (0.4–17) | 7.9 (0.6–18) | n.s. (0.5627) |
| Male sex, n (%) | 4 (50) | 3 (38) | |
| Height (m) | 113.5±16.0 | 118.9±15.6 | n.s. (0.7209) |
| Weight (kg) | 24.1±6.3 | 29.1±8.2 | n.s. (0.9163) |
| BSA (m2) | 0.86±0.18 | 0.95±0.20 | n.s. (0.8330) |
| Disease subtypes (n) | Mild to moderate LVOTO (6), mediastinal teratoma (1), portal vein stenosis (1) | IPAH (3), PAH (2) PAH-repaired CHD (2), portopulmonary PH (1) | |
| Key haemodynamics | |||
| Cardiac catheterisation | |||
| sPAP (mm Hg) | 23.4±2.5 | 68.8±11.4 | 0.0024 |
| mPAP (mm Hg) | 17.3±2.2 | 60.4±8.9 | 0.0009 |
| dPAP (mm Hg) | 12.3±2.1 | 38.4±5.3 | 0.0052 |
| mPAP/mSAP | 0.28±0.03 | 0.82±0.12 | 0.0003 |
| mTPG (mm Hg) | 6.4±1.1 | 51.5±9.2 | 0.0014 |
| dTPG (mm Hg) | 1.6±0.6 | 35.5±8.0 | 0.0033 |
| PVRi (WU·m2) | 1.69±0.30 | 16.3±3.6 | 0.0003 |
| PVR/SVR | 0.11±0.02 | 0.82±0.14 | 0.0012 |
| Qpi | 4.08±0.36 | 3.64±0.52 | n.s. (0.5054) |
| Qsi | 4.46±0.42 | 3.78±0.60 | n.s.(0.3823) |
| Qp/Qs | 0.92±0.04 | 0.99±0.04 | n.s. (0.3823) |
| mRAP (mm Hg) | 5.5±0.6 | 6.6±1.2 | n.s. (0.1605) |
| Echocardiography | |||
| RVAWD (cm) | 0.34±0.03 | 0.72±0.09 | 0.0022 |
| RVEDD (cm) | 1.26±0.21 | 2.27±0.38 | n.s. (0.1098) |
| TAPSE (cm) | 1.95±0.09 | 1.62±0.12 | n.s. (0.1111) |
| LVEF (%) | 74.7±2.2 | 67.7±3.6 | n.s. (0.1419) |
Values are presented as mean±SEM. A Mann-Whitney U test was applied. P<0.05 was considered significant. All PAH patients with repaired CHD (PAH-CHD) had the repair >12 months prior to cardiac catheterisation. Two of the PAH patients had trisomy 21 (all with PAH-repaired CHD; patient ID #7 and #8 in online supplementary table S1).
BSA, body surface area; CHD, congenital heart disease; dPAP, diastolic pulmonary arterial pressure; dTPG, diastolic transpulmonary pressure gradient; IPAH, idiopathic PAH; LVEF, left ventricular ejection fraction; LVOTO, left ventricular outflow tract obstruction; mPAP, mean pulmonary arterial pressure; mRAP, mean right atrial pressure; mSAP, mean systemic arterial pressure; mTPG, mean transpulmonary pressure gradient; PAH, pulmonary arterial hypertension; PFO, patent foramen ovale; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; PVRi, PVR index; Qp, pulmonary blood flow; Qpi, pulmonary flow index; Qs, systemic blood flow; Qsi, systemic flow index; RVAWD, right ventricular anterior wall diameter; RVEDD, right ventricular end-diastolic diameter; sPAP, systolic pulmonary arterial pressure; SVR, systemic vascular resistance; TAPSE, tricuspid annular plane systolic excursion; WU, Wood units.