Table 2. Clinical and haemodynamic variables at baseline and after oral bosentan therapy in patients with and without Down's syndrome
With Down's syndrome#Without Down's syndrome
BasalFollow-upp-valueBasalFollow-upp-value
Clinical status
    Sp,O2 %83±984±90.0784±985±80.2
    Heart rate beats·min−190±1185±80.000191±1484±110.0003
    WHO functional class2.9±0.62.5±0.50.0052.9±0.52.5±0.50.000002
Exercise tolerance (6MWT)
    Distance travelled m239±74288±710.0007343±86389±800.00003
    Heart rate at end of exercise beats·min−1117±19109±90.03117±19111±200.04
    Sp,O2at end of exercise %72±1074±100.01169±1373±120.005
    Borg index5.7±2.63.5±1.60.00035.0±2.14.3±1.90.001
Heart catheterisation
    Pra mmHg13±512±60.1411±411±40.6
    mPAP mmHg66±2160±170.0674±1873±210.6
    mPpcw mmHg11±312±30.1611±312±30.9
    Qp L·m−1·m−23.5±1.44.0±1.60.0062.8±1.03.5±1.40.0005
    Qs L·m−1·m−23.6±1.33.4±1.70.373.3±1.33.7±1.90.064
    Qp/Qs1.0±0.41.4±0.70.0030.9±0.31.1±0.70.012
    PVRI Wood units20±1315±90.00726±1520±100.002
  • Data are presented as mean±sd, unless otherwise stated. Sp,O2: arterial oxygen saturation measured by pulse oximetry; WHO: World Health Organization; 6MWT: 6-min walk test; Pra: right atrial pressure; mPAP: mean pulmonary artery pressure; mPpcw: mean pulmonary capillary wedge pressure; Qp: pulmonary flow; Qs: systemic flow; PVRI: pulmonary vascular resistance index. #: n=18; : n=56. Reproduced from [48] with permission from the publisher.