Table 1 Clinical parameters

Age

25

30

32

Right heart catheterization

 PAP (S/D/M, mmHg)

65/29/43

87/29/50

–

 PAWP (mmHg)

8

8

–

 RAP (mmHg)

10

10

–

 CI (L/min/m²)

2.08

2.16

–

 PVR (Wood Units)

12.5

14.29

–

 SVR (Wood Units)

27.38

23.8

–

 SaO₂ (%)

94

93

–

Echocardiogram

 LVEDd (mm)

38

39

36

 LVEF (%)

59

50

52

 RVd (mm)

35

41

42

 PA (mm)

32

32

33

 Peak TRV (m/s)

3.2

2.8

3.1

 TAPSE (mm)

–

12

15

 RV-FAC (%)

–

26

29

 IVC (mm)

12

15

13

 Pericardial effusion

No

Minimal

No

 NT-proBNP (pg/ml)

628

3020

352

 6MWD (m)

496

294

482

 WHO-FC

II

III

II

 Medication

Silenafil, Bosentan

Sildenafil, Macitentan, Treprostinil

Sildenafil, Macitentan, Selexipag