Table 1.
Characteristics of PH patients and non-PH controls.
| PH patients (N = 10) | non-PH controls (N = 10) | p -value | |
|---|---|---|---|
| Demographics | |||
| Age–years | 9.1 (3.9–18.5) | 10.5 (2.0–17.3) | n.s. (0.3822) |
| Male sex–n (%) | 4 (40%) | 6 (60%) | |
| Height – m | 1.30 ± 0.09 | 1.39 ± 0.09 | n.s. (0.3046) |
| Weight – kg | 30.2 ± 7.2 | 33.9 ± 4.6 | n.s. (0.2713) |
| BSA – m2 | 1.0 ± 0.1 | 1.1 ± 0.1 | n.s. (0.3061) |
| Clinical diagnosis | |||
| 1.1 IPAH−3 1.2 HPAH−3 1.4.3 Portal hypertension−1 1.4.4 PAH-repaired CHD−1 PH group 3 (lung disease)−2 |
LVOTO−9 s/p double aortic arch −1 |
||
| Functional status | |||
| WHO FC | 2.5 ± 0.2 | 1.0 | |
| 6 MWD–m, n = 5 | 344.0 ± 77.6 | N/A | |
| NTproBNP (SVC)–ng/l | 168.8 ± 50.8 | 79.6 ± 12.4 | n.s. (0.1594) |
| Key hemodynamics | |||
| Cardiac catheterization | |||
| mRAP–mm Hg | 3.7 ± 0.8 | 3.0 ± 0.7 | n.s. (0.4585) |
| RVSP–mmHg | 79.9 ± 10.2 | 25.6 ± 2.0 | <0.0001 |
| RVEDP–mm Hg | 7.6 ± 1.1 | 4.4 ± 1.0 | n.s. (0.0604) |
| sPAP–mm Hg | 81.8 ± 10.9 | 22.4 ± 2.1 | <0.0001 |
| dPAP–mm Hg | 35.9 ± 6.5 | 6.9 ± 1.5 | 0.0013 |
| mPAP–mm Hg | 56.9 ± 7.1 | 14.1 ± 1.5 | <0.0001 |
| mPAP/mSAP | 0.8 ± 0.1 | 0.2 ± 0.03 | <0.0001 |
| PAWP–mm Hg | 7.6 ± 1.5 | 8.0 ± 1.4 | n.s. (0.8890) |
| mTPG–mm Hg | 50.7 ± 6.9 | 6.0 ± 0.7 | <0.0001 |
| dTPG–mm Hg | 30.6 ± 5.9 | 0.6 ± 0.3 | 0.0003 |
| PVRi–WU·m2 | 13.3 ± 2.3 | 1.6 ± 0.2 | <0.0001 |
| SVRi–WU·m2 | 17.7 ± 2.0 | 16.1 ± 1.4 | n.s. (0.7394) |
| PVR/SVR | 0.7 ± 0.1 | 0.1 ± 0.01 | <0.0001 |
| Qpi–L/min/m2 | 4.0 ± 0.2 | 4.0 ± 0.1 | n.s. (0.9281) |
| Qsi–L/min/m2 | 4.2 ± 0.3 | 4.3 ± 0.2 | n.s. (0.6706) |
| Qp/Qs | 1.0 ± 0.03 | 0.94 ± 0.03 | n.s. (0.3033) |
| Echocardiography | |||
| RVAWD–cm, PSAX, n = 8 | 0.7 ± 0.1 | 0.3 ± 0.03 | 0.0003 |
| RVEDD–cm, PSAX, n = 7 | 2.2 ± 0.3 | 1.2 ± 0.1 | 0.0122 |
| RV/LV endsys. ratio, PSAX | 1.2 ± 0.1 | 0.5 ± 0.05 | <0.0001 |
| LV ecc. index (PSAX) | 1.3 ± 0.1 | 0.9 ± 0.03 | 0.0005 |
| S/D ratio, TRV jet, n = 8 | 1.5 ± 0.2 | N/A | |
| TAPSE–cm, apical | 1.7 ± 0.1 | 2.0 ± 0.08 | n.s. (0.0847) |
| PAAT–ms, PSAX | 85.8 ± 6.1 | 141.9 ± 9.3 | 0.0002 |
| LVEF n. Simpson–%, n = 8 | 61.1 ± 2.2 | 67.1 ± 2.9 | n.s. (0.0793) |
Values are presented as mean ± SEM. A Mann-Whitney U test was applied. P < 0.05 was considered significant. NTproBNP was measured in the superior vena cava (SVC) of all subjects. BSA, body surface area; D, diastolic; dPAP, diastolic pulmonary arterial pressure; dTPG, diastolic transpulmonary pressure gradient; HR, higher risk; HPAH, heritable pulmonary arterial hypertension; IPAH, idiopathic pulmonary arterial hypertension; LR, lower risk; LV, left ventricle; 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; NT-proBNP, N-terminal pro b-type natriuretic peptide; PAAT, pulmonary artery acceleration time; PAWP, pulmonary arterial wedge pressure; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension; PSAX, parasternal short axis; PVR, pulmonary vascular resistance; PVRi, pulmonary vascular resistance index; Qpi, pulmonary flow index; Qsi, systemic flow index; RV, right ventricle; RVAWD, right ventricular anterior wall diameter; RVEDD, right ventricular end-diastolic diameter; S, systolic; sPAP, systolic pulmonary arterial pressure; SVR, systemic vascular resistance; TAPSE, tricuspid annular plane systolic excursion; TRV, tricuspid regurgitation velocity; WHO, World Health Organization.