Table 1

 Demographic, clinical, and haemodynamic characteristics of patients at the time of PH diagnosis

Group A(n  =  7)Group B(n  =  15)
Group A, patients without pulmonary fibrosis on the chest radiograph; group B, patients with pulmonary fibrosis on the chest radiograph; mRAP, mean right atrial pressure; mPAP, mean pulmonary artery pressure; PWP, pulmonary wedge pressure; CI, cardiac index; PVRI, pulmonary vascular resistance index; SvO2, mixed venous oxygen saturation.
*Time interval between the first symptoms related to sarcoidosis and the diagnosis of PH.
†Acute vasodilator response was tested with a short term intravenous infusion of epoprostenol or inhaled nitric oxide. A positive response was defined by a fall both in mPAP and PVRI of ⩾20%.
‡Nine patients in group B were not tested.
Age (years)48 (13)45 (13)
Non/ex/current smokers4/3/06/7/2
Mean (SD) time interval between sarcoidosis and PH (years)*3.7 (3.1)12.5 (6.2)
NYHA functional class, n (%)
    I1 (14.3)
    II4 (57.1)2 (13.3)
    III2 (28.6)8 (53.3)
    IV5 (33.3)
Right heart failure, n (%)1 (14.3)4 (26.7)
Near syncope, n (%)03 (20)
Mean (SD) baseline haemodynamics
    mRAP (mm Hg)7.3 (4.2)6.4 (6.7)
    mPAP (mm Hg)51.7 (16.0)40.1 (11.6)
    PWP (mm Hg)8.8 (2.3)8.1 (3.6)
    CI (l/min/m2)2.45 (0.69)3.26 (0.09)
    PVRI (IU/m2)23.0 (10.4)14.6 (8.9)
    Svo2 (%)63 (16)68 (9)
Acute vasodilator response, n (%)†‡0 (0)0 (0)