Table 1.
Characteristics of the patients.
| Variables | All patients (N = 764) |
|---|---|
| Sex | Male 352/Female 402/unknown 10 |
| Preterm birth, N = 478 | 100 (21.9) |
| Birth weight, grams, N = 485 | 2,594 (2,166–2,958) |
| Congenital anomaly syndromes related to PH, (%) | 488 (63.9) |
| Past history of syncope or cardiac arrest due to pulmonary hypertension, (%), N = 560 | 14 (0.3) |
| Baseline WHO-FC, N = 323 | |
| I, (%) | 102 (31.6) |
| II, (%) | 117 (36.2) |
| III, (%) | 89 (27.6) |
| IV, (%) | 15 (4.6) |
| Cardiovascular death, (%), N = 568 | 54 (9.5) |
| Baseline laboratory tests | |
| BNP, pg/ml, N = 248 | 95 (36–232) |
| Baseline hemodynamics | |
| SvO2, %, N = 343 | 65 (59–69) |
| SaO2, %, N = 353 | 93 (88–96) |
| mPAP, mmHg, N = 399 | 44 (32–54) |
| CI, L/min/m2, N = 235 | 3.7 (3.2–4.5) |
| PVRI, Wood unit/m2, N = 384 | 5.4 (3.1–8.1) |
| PAWP, mmHg, N = 283 | 7 (5–10) |
| Positive pulmonary vasodilator testing, (%), N = 224 | 125 (55.8) |
| Lung biopsy | |
| Year of performing a lung biopsy | 2009 (2005–2013) |
| Age at lung biopsy, day | 150 (60–395) |
| Duration from lung biopsy to cardiovascular death/last medical check-up, year, N = 543 | 8.3 (3.3–13.1) |
| Heath-Edwards classification, N = 743 | Grade 0–2 587/Grade 3–6 156 |
| IPVD, N = 553 | <2 720/2≤ 24 |
| PAH treatment | |
| Epoprostenol, (%), N = 570 | 11 (1.9) |
| ERA, (%), N = 570 | 115 (20.2) |
| PDE5 inhibitor, (%), N = 570 | 139 (24.4) |
| Oral prostacyclin analogs, (%), N = 567 | 88 (15.4) |
| Selexipag, (%), N = 570 | 7 (1.2) |
| HOT, (%), N = 570 | 212 (37.2) |
For continuous variables, median and interquartile range are shown. For categorical variables, frequency with percentage is shown.
BNP, brain natriuretic peptide; CI, cardiac index; ERA, endothelin receptor antagonist; HOT, home oxygen therapy; IPVD, index of pulmonary vascular disease; mPAP, mean pulmonary arterial pressure; PAH, pulmonary arterial hypertension; PAWP, pulmonary arterial wedge pressure; PDE5, phosphodiesterase 5; PH, pulmonary hypertension; PVRI, pulmonary vascular resistance index; SaO2, saturation of arterial blood oxygen; SvO2, mixed venous oxygen saturation; WHO-FC, World Health Organization functional class.