Table 1

 Patient characteristics

HIV, human immunodeficiency virus infection; IPAH, idiopathic pulmonary arterial hypertension; NO, nitric oxide; PCWP, pulmonary capillary wedge pressure; Ca, calcium; ppm, parts per million; PVRI, pulmonary vascular resistance index; WHO, World Health Organization
Data are shown as mean (SD). In addition, the range is presented in brackets.
Patientsn = 39
Male:female17:22
Age (median)5.4 years (4 months–7 years)
Weight21.2 (15.3) kg (4.6–76.4 kg)
Aetiology of pulmonary arterial hypertension
    IPAHn = 25
    Pulmonary arterial hypertension associated with
        Congenital heart diseasen = 6
        Cardiomyopathyn = 3
        Connective tissue diseasen = 2
        Chronic/interstitial lung diseasen = 2
        HIVn = 1
WHO functional class I/II/III/IV0/0/27/12
Drugs taken before epoprostenol treatment
    Bosentann = 8
    Sildenafiln = 6
    Calcium channel blockern = 6
    Bosentan and sildenafiln = 2
    Bosentan and Ca channel blockern = 2
    Diureticsn = 20
    Digoxinn = 24 (8 warfarin, 13 aspirin, 3 dipyridamol)
    Anticoagulation/antiplateletn = 5
Haemodynamics at the onset of treatment
    Mean pulmonary arterial pressure59 (17) mmHg (25–88)
    Mean systemic arterial pressure62 (14) mmHg (42–95)
    Right atrial pressure8.1 (5.2) mmHg (3–28)
    Mean left atrial pressure/PCWP9.4 (4.6) (3–23)
    PVRI: baseline23.3 (11.6) units×m2 (3.5–49.8)
    PVRI: 100% FiO220.4 (10.8) units×m2 (8.7–44.0)
    PVRI: 65%FiO2/20 ppm NO19.5 (11.1) units×m2 (2.5–39.0)
    Pulmonary arterial saturation65.9 (12.5%) (23–87)
    Systemic saturation96 (4.7%) (83–100)