Table 1 Clinical characteristics at diagnosis
t-test | n = 228 43.49 y ± 12.75 | ||||
|---|---|---|---|---|---|
Patients | Mutation carrier n = 49 | Mutation non carrier n = 179 | |||
Age at onset (years) | ** | 38.53 y ± 12.38 | 45.78 y ± 11.32 | ||
female/male (ratio) | 32/17 (1.9:1) | 134/45 (3:1) | |||
NYHA at diagnosis | III-IV | II-IV | |||
Pulmonary hemodynamic parameters | |||||
Heart rate per minute | * | 83.57 ± 11.79 | n = 28 | 77.38 ± 9.07 | n = 88 |
SaO 2 (%) | 92.85 ± 3.06 | n = 26 | 92.68 ± 3.29 | n = 83 | |
PASP (mm Hg) | * | 98.5 ± 16.35 | n = 26 | 87.73 ± 18.78 | n = 83 |
PADP (mm Hg) | 44.5 ± 7.5 | n = 26 | 36.37 ± 9.22 | n = 81 | |
mPAP (mm Hg) | *** | 62.63 ± 9.92 | n = 35 | 53.44 ± 12.18 | n = 135 |
PCWP | 7.08 ± 3.16 | n = 26 | 7.75 ± 2.42 | n = 83 | |
SASP (mm Hg) | * | 118.11 ± 14.34 | n = 27 | 128.13 ± 18.59 | n = 86 |
SADP (mm Hg) | 76.5 ± 11.81 | n = 27 | 76.67 ± 10.60 | n = 86 | |
CI (Litres/min/m 2 ) | *** | 1.67 ± 0.25 | n = 31 | 2.10 ± 0.53 | n = 124 |
PVRI | *** | 2306.53 ± 770.33 | n = 25 | 1503.25 ± 671.76 | n = 116 |
PVR | *** | 1519.65 ± 374.65 | n = 22 | 1000.36 ± 456.51 | n = 71 |