Table 3.
Demographic and baseline characteristics of children with idiopathic pulmonary arterial hypertension (IPAH) and pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH).
| Characteristics | All (IPAH, CHD-PAH, PoPAH) | IPAH | CHD-PAH | P |
|---|---|---|---|---|
| N = 80 | N = 25 | N = 54 | ||
| Female, n (%) | 40 (50%) | 12 (48%) | 27 (50%) | 0.9 |
| Incident cases, n (%) | 10 (12.5%) | 9 (36%) | 1 (1.9%) | <0.0001 |
| Age at diagnosis | 5.1 (2.1–8.1) | 5.9 (2.5–8.3) | 4.6 (2.1–7.5) | 0.5 |
| Age at enrollment | 10.4 (7.9–15.2) | 9.8 (7.6–11.5) | 11.5 (7.9–15.4) | 0.15 |
| Functional class at diagnosis | ||||
| I | 9 (11.3%) | 2 (8%) | 7 (12.97%) | 0.5 |
| II | 17 (21.3%) | 7 (28%) | 9 (16.67%) | 0.2 |
| III | 53 (66.3%) | 16 (64%) | 37 (68.51%) | 0.7 |
| IV | 1 (1.3%) | 0 | 1 (1.85%) | 0.5 |
| Functional class at enrolment, n (%) | ||||
| I | 9 (11.3) | 2 (8%) | 7 (13%) | 0.5 |
| II | 46 (57.5) | 13 (52%) | 32 (59%) | 0.5 |
| III | 25 (31.3) | 10 (40%) | 15 (28%) | 0.3 |
| IV | 0 | 0 | 0 | - |
| RHC at diagnosis | ||||
| mPAP [mmHg] | 48.0 (37.0–57.0) | 48.0 (39–67) | 48.0 (36–55.5) | 0.54 |
| CI [l/min/m2] | 2.9 (1.9–3.9) | 2.6 (1.9–3.6) | 3.2 (2.0–3.9) | 0.86 |
| PVR [WU] | 11.1 (7.2–21.3) | 10.4 (6.7–22.9) | 11.9 (7.7–19.6) | 0.88 |
| PVRI [WU/m2] | 10.9 (6.5–21.1) | 11.4 (7.9–26.5) | 10.7 (6.4–18.5) | 0.34 |
| Clinical characterization at enrolment | ||||
| Height for age percentile | 14 (2.75–50.0) | 25.0 (7.0–62.0) | 6.5 (2.0–44.0) | 0.06 |
| Weight for age percentile | 22.0 (3.0–50) | 36.0 (17.0–62.0) | 13.5 (3.0–50.0) | 0.08 |
| BMI for age percentile | 40.6 (8.0–70.0) | 42.0 (17–62.0) | 36.0 (3.0–75.0) | 0.57 |
| 6 MWT [m] | 429 (360–500) | 450 (383–524) | 420 (353–469) | 0.46 |
| NT-proBNP [ng/L] | 272 (104.6–628.0) | 268.7 (69.2–502.7) | 290.0 (115.8–676.0) | 0.38 |
| RAA [cm2] | 14.3 (10.0–19.0) | 12.2 (8.8–14.6) | 16.4 (10.4–20.4) | 0.08 |
| Anthropometric measurements in patients without genetic defects | ||||
| Height for age percentile | 30.5 (5.0–62.5) | 37 (8.5–64.5) | 26 (3–62) | 0.6 |
| Weight for age percentile | 37.5 (6.5–64.8) | 39.5 (17.5–82.0) | 37 (25–62) | 0.4 |
| BMI for age percentile | 31.5 (3.5–64) | 43.5 (13.5–72.0) | 20 (3.0–55) | 0.1 |
| Anthropometric measurements in patients with genetic defects | ||||
| Height for age percentile | 3 (2–9) | 10 (3–25) | 3 (2–7) | 0.3 |
| Weight for age percentile | 7.5 (3–31) | 25 (10–25) | 7 (3–37) | 0.5 |
| BMI for age percentile | 47.5 (16–77.5) | 35 (25–30) | 50 (12–80) | 0.4 |
6 MWT, 6 min walk distance; NT-proBNP, N-terminal pro-B-type natriuretic peptide; RAA, right atrial area; mPAP, mean pulmonary artery pressure; CI, cardiac index; PVR, pulmonary vascular resistance; PVRI, pulmonary vascular resistance index.