Table 1 Characteristics of IPAH/HPAH patients (n = 105)

IPAH/HPAH, n (%)/n (%)

101(96.2)/4(3.8)

Females/males, n (%)

82/23 (78.1/21.9)

Age, years

50.4 ± 15.56

Age of PAH first symptoms, years

43.4 ± 17.69

Age at diagnosis, years

48.6 ± 16.78

Time from first symptoms to diagnosis, years

5.2 ± 10.76

Six-minute walking distance, m

367.2 ± 120.17

WHO functional class at time of diagnosis

 I, n (%)

2 (1.9)

 II, n (%)

25 (23.8)

 III, n (%)

74 (70.5)

 IV, n (%)

4 (3.8)

Peripheral oedema, n (%)

52 (49.5)

mPAP, mmHg

55.04 ± 15.928

PVR, Wood unit

13.17 (7.7;16.2)

Cardiac index, L/min/m²

2.48 (1.7;3.0)

RAP, mmHg

8.16 ± 5.354

Positive vasoreactivity test at time of diagnosis, n (%)

13 (12.4)

TAPSE, mm

16.88 (13;20)

NT-proBNP, pg/ml

2373.6 (221.3; 2479.5)