Table 1.
Demographic data of our 57 patients with PH.
| All PH patients | |
| Fulfilled inclusion criteria (n) | 57 |
| Female (n (%)) | 24 (42) |
| Age at baseline (years) (range) | 5.7 (0.6–18.2) |
| Body weight (kg) (range) | 20.8 (4.9–86.5) |
| Body length (cm) (range) | 127 (50–187) |
| BSA range (m2) | 0.2–1.92 |
| NYHA-FC/ROSS score | |
| I (n) | 18 |
| II (n) | 27 |
| III (n) | 12 |
| PH medication (n) | |
| Sildenafil (single) | 13 |
| Bosentan | 4 |
| Macitentan | 8 |
| Bosentan, sildenafil (comb) | 10 |
| Macitentan, sildenafil | 18 |
| Selexipag (triple comb) | 4 |
| Calcium antagonists | 3 |
| PAH-CHD (n = 26) | |
| TRV (m/s) | 3.9 (3.0–4.9) |
| sPAP/sSAP (%) | 71 (39–102) |
| mPAP (mmHg) (mean ± range) | 37 (27–55) |
| PVRi (WU) (mean ± range) | 3.9 (2.2–15.3) |
| TAPSE (mm) | 1.54 (1.21–1.74) |
| PAAT (ms) | 75 ± 19 |
| Diagnosis (n) | |
| AVSD | 12 |
| VSD | 10 |
| PA with VSD | 4 |
| IPAH (n = 12) | |
| TRV (m/s) | 4.4 (3.3–5.6) |
| sPAP/sSAP (%) | 88 (44–118) |
| mPAP (mmHg) (mean ± range) | 47 (32–91) |
| PVRi (WU) (mean ± range) | 8.9 (3.1–20.4) |
| TAPSE (mm) | 1.32 (1.24–1.66) |
| PAAT (ms) | 70 ± 16 |
| PH-BPD (n = 19) | |
| TRV (m/s) | 3.4 (2.9–4.3) |
| sPAP/sSAP (%) | 62 (38–85) |
| mPAP (mmHg) (mean ± range) | 35 (27–54) |
| PVRi (WU) (mean ± range) | 5.6 (3.0–15.8) |
| TAPSE (mm) | 1.12 (0.85–1.55) |
| PAAT (ms) | 65 ± 16 |
Values are presented as n or median (range) unless otherwise specified.
AVSD, atrioventricular septal defect; BSA, body surface area; BPD, bronchopulmonary dysplasia; IPAH, idiopathic pulmonary arterial hypertension; mPAP, mean pulmonary arterial pressure; NYHA, New York Heart Association; PA, pulmonary atresia; PAAT, pulmonary artery acceleration time; PH-CHD, pulmonary hypertension secondary to congenital heart disease; PVRi, pulmonary vascular resistance indexed; RV, right ventricle; sPAP/sSAP, systolic pulmonary arterial pressure/systolic systemic arterial pressure; SD, standard deviation; TAPSE, tricuspid annular plane systolic excursion; TRV, tricuspid regurgitation velocity; VSD, ventricular septal defect; WU, Wood Units.