Skip to main content

Table 2 Clinical features and hemodynamic parameters of patients

From: Mutational and clinical analysis of the ENG gene in patients with pulmonary arterial hypertension

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
  1. 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
  2. aWe have compared clinical features and hemodynamic parameters between patients with mutations in ENG gene and patients without mutations
  3. bWe have compared clinical features and hemodynamic parameters between patients with p.G191N variation in ENG gene and patients without mutations