Table 1.
Clinical, demographic and hemodynamical characteristics of the studied population. The clinical PH group shown in this table refers to the classification after genetic study. CI (cardiac index); DLD (developmental lung disease); IQr (interquartile range); iPVR (index pulmonary vascular resistance); iSVR (index systemic vascular resistance); mPAP (mean pulmonary artery pressure); N/A (not available); RAP (right atrial pressure); WU (Wood units). * p-value refers to global differences between groups.
| Total Cohort (n = 98) | Idiopathic (n = 28) | Heritable PAH (n = 18) | PVOD (n = 8) | PAH-CHD (n = 28) | TBX4 (n = 4) | PH-MSD (n = 12) | p Value | |
|---|---|---|---|---|---|---|---|---|
| Age—years (median—IQr) | 7.1 (1.5–14.7) | 6.3 (3.6–12.6) | 10.2 (5.6–17.7) | 15.7 (12.4–17.3) | 6.3 (2.1–10.5) | 0.4 (0.2–8.1) | 0.1 (0.1–0.4) | <0.001 |
| Gender—male (n—%) | 43 (43.9) | 13 (46.4) | 6 (33.3) | 6 (75.0) | 10 (35.7) | 2 (50.0) | 6 (50.0) | 0.412 |
|
Descendance
(n—%) |
<0.001 * | |||||||
| Arabic | 2 (2.1) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 2 (16.7) | |
| Asiatic | 1 (1.0) | 0 (0.0) | 1 (5.6) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | |
| Caucasian | 80 (81.6) | 27 (96.4) | 13 (72.2) | 2 (25.0) | 25 (89.3) | 4 (100.0) | 9 (75.0) | |
| Romani | 8 (8.2) | 0 (0.0) | 2 (11.1) | 6 (75.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | |
| Hispanic | 6 (6.1) | 1 (3.6) | 2 (11.1) | 0 (0.0) | 2 (7.1) | 0 (0.0) | 1 (8.3) | |
| Black | 1 (1.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 1 (3.6) | 0 (0.0) | 0 (0.0) | |
| Mode of diagnosis—Index case vs. family screening (n—%) | 91 (92.9) | 28 (100.0) | 14 (77.8) | 5 (62.5) | 28 (100.0) | 4 (100.0) | 12 (100.0) | 0.001 * |
| Baseline | ||||||||
| hemodynamics | N = 82 | N = 27 | N = 16 | N = 7 | N= 21 | N = 3 | N = 8 | |
| mPAP—mmHg (median—IQr) | 53.0 (40.0–63.0) | 54.0 (39.0–70.0) | 55.0 (42.5–68.5) | 44.0 (22.0–56.0) | 61.0 (46.0–65.0) | 39.0 (27.0–61.0) | 41.0 (32.0–49.5) | 0.081 |
| RAP—mmHg (median—IQr) | 7.0 (5.0–10.0) | 7.0 (6.0–10.0) | 5.5 (2.0–11.5) | 7.0 (4.0–10.0) | 7.5 (6.0–10.0) | 11.0 (8.0–12.0) | 6.5 (5.0–12.0) | 0.814 |
| iPVR—WU * m2 (median—IQr) | 11.3 (7.0–17.4) | 11.7 (7.9–16.9) | 15.6 (7.0–20.4) | 7.2 (3.2–13.7) | 11.8 (5.4–18.3) | 10.8 (3.8–16.2) | 8.6 (7.1–14.3) | 0.500 |
| CI—L/m/m2 | 3.1 (2.0–4.0) | 3.1 (1.9–3.9) | 2.6 (2.0–3.2) | 2.3 (1.8–2.7) | 4.0 (3.1–4.8) | 6.4 (4.8–8.0) | 2.2 (1.6–3.1) | 0.007 |
| (Median—IQr) | ||||||||
| iPVR/ iSVR (median—IQr) | 0.9 (0.6–1.0) | 0.8 (0.6–0.9) | 0.8 (0.6–0.9) | N/A | 1.0 (0.6–1.3) | 0.8 (0.8–2.2) | 0.9 (0.8–1.0) | 0.178 |