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Table 2 Clinical characteristics of patients at the time of diagnosis of PAH, according to NMD pathway status of the BMPR2 mutation.

From: Truncating and missense BMPR2 mutations differentially affect the severity of heritable pulmonary arterial hypertension

NMD status of BMPR2 mutation 'NMD active' (Truncating)
(n = 55)
'NMD absent' (Missense)
(n = 41)
P value*
Age at Diagnosis, yrs 39.9 (36.3-43.5) 30.6 (25.9-35.3) 0.004
Gender, female/male 2.6/1 1.7/1 0.82
RAP, mm Hg 11.4 (7.4-15.3) 10.5 (8.1-13.0) 0.74
Mean PAP, mm Hg 56.5 (51.6-61.5) 60.0 (54.9-65.2) 0.42
PCWP, mm Hg 11.1 (8.5-13.6) 10.3 (8.4-12.2) 0.74
CI, L/min/m2 1.8 (1.5-2.1) 1.9 (1.8-2.1) 0.16
PVR, mm Hg/L/min 18.4 (11.4-25.3) 17.9 (12.7-23.1) 0.78
SvO2, % 58.6 (51.2-65.3) 57.3 (52.8-61.7) 0.19
  1. * All P values calculated using Mann-Whitney U test.
  2. † 95% Confidence Interval values calculated using univariable ANOVA
  3. Definition of abbreviations: CI = cardiac index; PAH = pulmonary arterial hypertension; PCWP = pulmonary capillary wedge pressure; PAP = pulmonary artery pressure; PVR = pulmonary vascular resistance; RAP = right atrial pressure; SvO2 = mixed venous oxygen saturation.