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Table 2 Baseline hemodynamic characteristics of BMPR2 mutation carriers according to mutation types.

From: Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension

  BMPR2 missense mutation carriers
n = 32
BMPR2 truncating mutation carriers
n = 51
BMPR2 large rearrangement or splice defect mutation carriers
n = 32
Age at diagnosis, yrs (mean ± SD) 35.8 ± 16.9 37.3 ± 14.0 33.5 ± 16.3
NYHA
I-II 5 (15.6%) 5 (9.8%) 7 (21.8%)
III 21 (65.6%) 37 (72.5%) 18 (56.3%)
IV 3 (9.4%) 7 (13.7%) 6 (18.8%)
Six-minute walk test distance, m 382 ± 107 351 ± 91 325 ± 109
mPAP, mmHg 63 ± 14 62 ± 12 62 ± 13
RAP, mmHg 6 ± 5* 9 ± 5 8 ± 5
PCWP, mmHg 8 ± 3 7 ± 2 8 ± 4
CI, L/min/m² 2.31 ± 0.68 1.94 ± 0.41 2.16 ± 0.75
PVRi, mmHg/L/min/m² 24.9 ± 10.8 25.0 ± 10.5 22.5 ± 14.9
SvO 2, % 60 ± 9 57 ± 10 60 ± 9
Acute vasodilator responders, % 1(3.1%) 1 (1.9%) 0.9
  1. *p = 0.02 compared to BMPR2 truncating mutation carriers
  2. p = 0.01 compared to BMPR2 truncating mutation carriers
  3. BMPR2: bone morphogenetic protein receptor type 2, PAH: pulmonary arterial hypertension, NYHA = New York Heart Association, mPAP = mean pulmonary artery pressure, RAP = right atrial pressure, PCWP = pulmonary capillary wedge pressure, CI = cardiac index, PVRi = indexed pulmonary vascular resistance, SvO2 = mixed venous oxygen saturation, NO = nitric oxide.
  4. Results are expressed as mean ± SD