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Table 1 Demographics and clinical characteristics of patients at diagnosis with BMPR2 and with biallelic EIF2AK4 variants

From: Clinical characteristics and survival of Chinese patients diagnosed with pulmonary arterial hypertension who carry BMPR2 or EIF2KAK4 variants

 

BMPR2 (N = 45)

EIF2AK4 (N = 11)

p value

F/M, (F%)

30/15(66.7%)

8/3(72.7%)

0.7

Age at diagnosis, y

25.57 ± 10.17

31.6 ± 9.38

0.09

BMI, kg/m2

21.77 ± 3.99

20.78 ± 3.49

0.49

NYHA functional class

 I-II(n/%)

21/46.7%

5/45.5%

0.94

 III(n, %)

22/48.9%

5/45.5%

0.84

 IV(n, %)

2/4.4%

1/9.1%

0.54

NT-proBNP, pg/ml

1806.41 ± 1466.61

1703.5 ± 1778.76

0.85

DLCO, predicted %

67.74 ± 14.63

29.88 ± 6.01

0.00*

Peak VO2, predicted %

32.56 ± 12.83

29.12 ± 9.70

0.48

6MWD, m

365.77 ± 96.11

390.56 ± 130.86

0.53

SvO2, %

64.19 ± 6.96

70.87 ± 7.34

0.02*

SaO2, %

95.11 ± 3.54

92.00 ± 2.84

0.03*

RAP, mmHg

5.11 ± 3.99

4.29 ± 2.69

0.61

mPAP, mmHg

63.62 ± 14.97

51.00 ± 4.24

0.00

PAWP, mmHg

7.15 ± 3.45

7.57 ± 2.99

0.77

PVR, Wood units

18.85 ± 6.77

12.03 ± 2.00

0.01*

CI, L/min/m2

2.39 ± 0.52

3.24 ± 1.04

0.07

Vasodilator responder

0

1/9%

  1. Values are shown as mean ± SD or n (%). BMI body mass index, NT-proBNP N-terminal pro-B-type natriuretic peptide, DLCO diffusing lung capacity of carbon monoxide, PeakVO2 peak oxygen consumption, 6MWD 6 min walk distance, SvO2 mixed venous oxygen saturation, SaO2 arterial oxygen saturation, RAP right atrial pressure, mPAP mean pulmonary artery pressure, PAWP pulmonary artery wedge pressure, PVR pulmonary vascular resistance, CI cardiac index *:p<0.05