Table 1 Baseline characteristics of healthy controls and pulmonary arterial hypertension (PAH)

Demographics and clinical parameters

 Age, years

46 ± 14

46 ± 11

0.828

 Sex, M/F

17/34

7/38

0.059

 Height, cm

163 ± 8

159 ± 8

0.017

 Weight, kg

60 ± 11

61 ± 13

0.515

 Body surface area, m²

1.64 ± 0.17

1.64 ± 0.19

0.935

 Body mass index, kg/m²

22.4 ± 3.1

24.1 ± 4.6

0.032

 Systolic blood pressure, mmHg

126 ± 19

119 ± 20

0.074

 Diastolic blood pressure, mmHg

75 ± 13

71 ± 14

0.087

 Heart rate, bpm

71 ± 14

82 ± 16

0.001

 NT–proBNP, pg/mL*

–

207 (337)

–

 Functional class > WHO I, n (%)

–

18 (40%)

–

 Types of PAH

     Idiopathic PAH, n (%)

–

23 (51%)

–

     Heritable PAH, n (%)

–

4 (9%)

–

     PAH associated with connective tissue disease, n (%)

–

13 (29%)

–

        Systemic sclerosis, n (%)

–

4 (31%)

–

         Mixed connective tissue disease, n (%)

–

3 (23%)

–

        Systemic lupus erythematosus, n (%)

–

3 (23%)

–

         Sjogren syndrome, n (%)

–

2 (15%)

–

        Anti–synthetase syndrome, n (%)

–

1 (8%)

–

     PAH associated with congenital heart disease, n (%)

–

5 (11%)

–

       Atrial septal defect repaired, n (%)

–

3 (60%)

–

        Atrial septal defect unrepaired, n (%)

–

1 (20%)

–

       Pulmonary atresia with ventricular septal defect, n (%)

–

1 (20%)

–

Medical history

   Diabetes mellitus, n (%)

–

5 (11.1%)

–

   Hypertension, n (%)

–

7 (15.6%)

–

   Hyperlipidemia, n (%)

–

3 (6.7%)

–

 Medication

   β–Blocker, n (%)

–

3 (6.7%)

–

   Calcium channel blockers, n (%)

–

0 (0%)

–

   Diuretics, n (%)

–

6 (13.3%)

–

   Anticoagulants, n (%)

–

10 (22.2%)

–

   ERAs, n (%)

–

24 (53.3%)

–

    Prostanoids, n (%)

–

0 (0%)

–

   Digoxin, n (%)

–

12 (26.7%)

–

    Corticosteroids, n (%)

–

4 (8.9%)

–

    PDIs, n (%)

–

26 (57.8%)

–

Right heart catheterization

  RAP, mm Hg

–

9 ± 6

–

  mPAP, mm Hg

–

48 ± 14

–

  PCWP, mm Hg

–

12 ± 5

–

  PVR, Wood units

–

11.1 ± 7.0

–

  RV systolic pressure, mmHg

–

75 ± 25

–

LV function

  LV mass index, g/m²

39 ± 8

37 ± 15

0.383

  LVEDV index, ml/m²

76 ± 11

76 ± 34

0.993

  LVESV index, ml/m²

32 ± 6

30 ± 21

0.516

  LV stroke volume index, ml/m²

44 ± 7

47 ± 15

0.399

  LVEF, %

59 ± 5

62 ± 9

0.011

RV function

  RVEDV index, ml/m²

74 ± 13

102 ± 41

< 0.001

  RVESV index, ml/m²

35 ± 8

61 ± 31

< 0.001

  RV stroke volume index, ml/m²

40 ± 6

41 ± 20

0.728

  RVEF, %

54 ± 6

42 ± 12

< 0.001

  RVEDV/LVEDV

0.97 ± 0.10

1.43 ± 0.64

< 0.001

  RV mass, g

25.6 ± 5.3

39.2 ± 12.4

< 0.001

  RV/LV mass

0.39 ± 0.06

0.58 ± 0.19

< 0.001

 TAPSE, mm

19.8 ± 2.8

15.0 ± 3.9

< 0.001

 RV GLS, %

24.3 ± 3.9

17.9 ± 4.7

< 0.001

 Right atrial area (end–systole), cm²

19.3 ± 4.0

25.3 ± 11.2

0.001

 PA RAC, %

56 ± 20

25 ± 13

< 0.001