Table 1 Baseline characteristics and haemodynamic parameters (mean (SD)) (n = 23)
Age (years)9.8 (7.1)
Gender (male/female)9/14
Familial/idiopathic (clinically)0/23
Haemodynamic parameters
    mPAP (mm Hg)71.7 (23.3)
    RAP (mm Hg)9.2 (3.9)
    CI (litres.min−1.m−2)2.5 (0.9)
    TPR (Wood.Unit−1.m−2)33.7 (12.6)
    PVR (Wood.Unit−1.m−2)27.5 (12.3)
    PAWP (mm Hg)10.2 (3.6)

  • CI, cardiac index; mPAP, mean pulmonary arterial pressure; PAWP, pulmonary artery wedge pressure; PVR, pulmonary vascular resistance; RAP, right atrial pressure; TPR, total pulmonary resistance.