Table 2 Clinical characteristics at diagnosis
Baseline parameters | I/HPAH | CHD-APAH | Mutation carriers | Non carriers | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
n | 29 | 11 | 10 | 30 | ||||||
Female/male ratio | 18/11 | 7/4 | 5/5 | 20/10 | ||||||
Baseline and hemodynamic parameters | ||||||||||
p-value | Mean ± SD | 95% CI for the mean | Mean ± SD | 95% CI for the mean | p-value | Mean ± STD | 95% CI for the mean | Mean ± STD | 95% CI for the mean | |
Age of onset at first symptoms (years) | 0.974 | 4.4 ± 3.9 | 3.0-5.9 | 4.5 ± 3.9 | 2.1-6.8 | 0.419 | 5.3 ± 4.1 | 2.8-7.8 | 4.1 ± 3.8 | 2.8-5.5 |
Age at diagnosis by RHC (years) | 0.352 | 5,2 ± 4.3 | 3.1-7.3 | 6.9 ± 4.7 | 4.0-9.8 | 0.503 | 7.1 ± 4.1 | 3.5-10.7 | 5.6 ± 4.5 | 4.7-7.5 |
Heart rate per minute | 0.744 | 98 ± 35 | 79-118 | 94 ± 15 | 84-105 | 0.776 | 93.4 ± 21.3 | 74.7-112.1 | 97.8 ± 31.3 | 82.4-113.1 |
Oxygen Saturation (%) | 0.140 | 95.0 ± 6.5 | 91.6-98.4 | 81.8 ± 31.8 | 61.0-102.5 | 0.448 | 97.3 ± 2.2 | 95.1-99.4 | 88.3 ± 22.7 | 78.1-98.5 |
PASP (mm Hg) | 0.799 | 87.3 ± 49.4 | 61.4-113.2 | 82.1 ± 23.8 | 65.5-99.7 | 0.275 | 64.5 ± 5.7 | 58.9-70.1 | 90.5 ± 45.4 | 69.0-112.1 |
PADP (mm Hg) | 0.939 | 38.9 ± 26.1 | 24.7-53.1 | 39.8 ± 16.1 | 26.9-52.8 | 0.169 | 25.0 ± 11.1 | 14.1-35.9 | 43.0 ± 24.0 | 30.9-55.2 |
PAMP (mm Hg) | 0.864 | 58.0 ± 33.0 | 40.0-76.0 | 55.5 ± 16.3 | 42.4-68.6 | 0.298 | 43.6 ± 6.6 | 37.3-50.2 | 60.8 ± 31.0 | 45.1-76.5 |
PCWP (mm Hg) | 0.691 | 6.9 ± 3.3 | 5.1-8.8 | 6.2 ± 3.5 | 3.1-9.3 | 0.691 | 6.0 ± 4.4 | 1.1-10.9 | 6.9 ± 3.1 | 5.2-8.5 |
SASP (mm Hg) | 0.998 | 93.7 ± 14.1 | 86.3-101.1 | 93.7 ± 13.0 | 85.7-101.8 | 0.871 | 94.6 ± 8.7 | 87.0-102.2 | 93.5 ± 14.5 | 87.0-100.0 |
SADP (mm Hg) | 0.853 | 52.1 ± 16.2 | 43.6-60.6 | 53.1 ± 6.8 | 48.7-57.3 | 0.295 | 58.0 ± 5.4 | 53.2-62.8 | 51.1 ± 14.0 | 44.8-57.4 |
Cardiac Index (L/min/m2) | 0.563 | 3.3 ± 1.1 | 2.6-3.9 | 3.7 ± 0.8 | 2.8-4.5 | 0.644 | 3.6 ± 1.1 | 2.6-4.7 | 3.3 ± 1.0 | 2.7-3.9 |
PVRI (Wood U*m2) | 0.461 | 18.6 ± 16.1 | 9.8-27.4 | 12.9 ± 6.2 | 7.5-18.3 | 0.179 | 8.5 ± 2.5 | 6.1-10.9 | 19.4 ± 15.2 | 11.5-27.4 |
PVR (dyn*s*cm−5) | 0.804 | 2012.2 ± 1788.8 | 1039.8-2984.5 | 1795.4 ± 1038.8 | 884.9-2705.9 | 0.014* | 927.0 ± 284.4 | 1039.8-2984.5 | 2244.8 ± 1692.5 | 884.9-2705.9 |