Table 2.
Clinical features and hemodynamic parameters of patients
| Clinical features and hemodynamic parameters | Total patients | Carriers of pathogenic mutationsa | Carriers of p.G191N mutationb | ||
|---|---|---|---|---|---|
| Clinical data | p-value | Clinical data | p-value | ||
| Number | 57 | 16 | ---------- | 10 | ---------- |
| Gender | 21 M/36 F | 6 M/10 F | n.s | 2 M/8 F | n.s |
| Age at diagnosis (years) | 49 ± 15 | 41 ± 16 | 0.040 | 39 ± 18 | 0.035 |
| mPaP (mmHg) | 49 ± 14 | 48 ± 11 | n.s | 45 ± 12 | n.s |
| sPaP (mmHg) | 70 ± 19 | 75 ± 16 | n.s | 67 ± 18 | n.s |
| PVR (mmHg.l−1.m−1) | 8.2 ± 3.5 | 8.6 ± 3.2 | n.s | 8.3 ± 0.9 | n.s |
| CI (l.m−1.m−2) | 2.6 ± 0.6 | 2.5 ± 0.7 | n.s | 2.1 ± 0.5 | 0.049 |
| 6MWT (m) | 445 ± 139 | 323 ± 162 | 0.040 | 457 ± 172 | n.s |
| PAH types | 29 IPAH/28 APAH | 7 IPAH/9 APAH | n.s | 8 IPAH/2 APAH | 0.040 |
Values are expressed as the mean ± standard deviation; F female, M male, mPaP mean pulmonary artery pressure, sPaP systolic pulmonary artery pressure, PVR pulmonary vascular resistance, CI cardiac index, 6MWT 6 min walking test, IPAH idiopathic pulmonary arterial hypertension, APAH associated pulmonary arterial hypertension
aWe have compared clinical features and hemodynamic parameters between patients with mutations in ENG gene and patients without mutations
bWe have compared clinical features and hemodynamic parameters between patients with p.G191N variation in ENG gene and patients without mutations