Table 1 Clinical characteristics of the patients enrolled in this study

N

9

9

28

28

Age, years

39.1 ± 10.5

39.3 ± 16.9

42.5 (35.3, 51.5)

39.0 (29.3, 60.5)

Female

6 (66.7%)

6 (66.7%)

22 (78.5%)

22 (78.5%)

BMI, kg•m⁻²

–

23.6 ± 3.6

–

22.9 ± 2.5

Comorbidity

CHD

0

0

0

4

Hypertension

0

1

0

9

Lung disease

0

0

0

6

Sleep disorder

0

2

0

1

Diabetes mellitus

0

0

0

2

Renal insufficiency

0

1

0

4

Malignant tumor

0

0

0

1

6MWD, m

–

340.3 ± 91.8

–

404.6 ± 102.5

WHO functional class

 I

–

0 (0%)

–

2 (7.1%)

 II

–

7 (77.8%)

–

13 (46.4%)

 III

–

2 (22.2%)

–

12 (42.9%)

 IV

–

0 (0%)

–

1 (3.6%)

Risk stratification

 Low risk

–

1 (11.1%)

–

5 (17.9%)

 Intermediate risk

–

6 (66.7%)

–

19 (67.9%)

 High risk

–

2 (22.2%)

–

4 (14.3%)

Hemodynamics

 mPAP, mmHg

–

54.0 (42.0, 90.5)

–

47.2 ± 15.4

 PVR, dyn•s•cm⁻⁵

–

1324.4 ± 721.2

–

746.4 ± 435.1

 CI, L/min

–

2.3 ± 0.6

–

2.1 (1.8, 2.5)

 NT-proBNP, ng/mL

–

1,063.1 ± 702.1

–

592.4 (179.0, 1,990.5)

Echocardiography

 sPAP, mmHg

–

84.0 ± 21.5

–

78.0 ± 27.5

 TAPSE, mm

–

15.0 (13.4, 15.0)

–

16.5 (14.0, 20.8)

 TAPSE/sPAP, mm/mmHg

–

0.18 ± 0.07

–

0.24 ± 0.12

Tarteted therapy

 sGC

–

0 (0.0%)

–

4 (14.3%)

 ERA

–

3 (33.3%)

–

18 (64.3%)

 PDE5i

–

5 (55.6%)

–

18 (64.3%)

 CCBs

–

2 (22.2%)

–

2 (7.1%)

 Monotherapy

–

2 (22.2%)

–

10 (35.7%)

 Combination therapy

–

4 (44.4%)

–

15 (53.6%)