Table 1 Baseline characteristics of the patients

Age at initial symptoms, years

40 [35–43]

Age at diagnosis of IPV, years

42 [38–44]

Diagnosis delay, months

14 [10–40]

Body mass index, kg/m²

25.4 [18.3–26.7]

Female

5 (71)

Dyspnea

7 (100)

Hemoptysis

4 (57)

Six-minute walking distance, m

472 [407–534]

NYHA functional class

   II

2 (29)

   III

5 (71)

NT-pro BNP, pg/mL

92.1 [42.2–266.0]

C-reactive protein, mg/L

2.5 [1.6–8.8]

Erythrocyte sedimentation rate, mm/h

3.0 [2.0–10.0]

Hemodynamics

 Mean right atrial pressure, mm Hg

4 [3–6]

 Systolic pulmonary artery pressure, mm Hg

49 [26–78]

 Mean pulmonary artery pressure, mm Hg

33 [20–48]

 Cardiac index, L/min/m²

3.2 [2.9–4.6]

 Pulmonary vascular resistance, dyn.s.cm⁻⁵

234 [131–843]

Medications

 Anticoagulation

7 (100)

 PH‐targeted treatment

2 (29)