Table 1—

Patient characteristics and outcome with bosentan

Time frameDiagnosis of PH6 months12 months18 months24 months
Oxygen therapy L·min−1 2.52.52.52.52.5
Noninvasive ventilation NoNoStartedYesYes
WHO functional class IIII/IIIIIIII
6-min walk distance m 198306294294360
Echocardiographic data
 Estimated sPAP mmHg11060
 Right cavitiesSevere dilatation, RV/LV size ratio 1.8UndilatedUndilatedUndilated
Right heart catheterisation
pa mmHg6534
 dPAP – Ppw mmHg347
 CI L·min−1·m−22.63.7
 PVR dyn·s·cm−5739214
Pulmonary function tests
 FVC % pred7675666981
 FEV1 % pred3427292839
Pa,O2 with 2.5 L·min−1 O2 kPa11.09.110.710.711.6
Pa,CO2 kPa7.99.67.87.07.2
 pH7.397.377.377.387.40
  • PH: pulmonary hypertension; WHO: World Health Organization; sPAP: systolic pulmonary artery pressure; pa: mean pulmonary artery pressure; dPAP: diastolic pulmonary artery pressure; Ppw: pulmonary artery wedge pressure; CI: cardiac index; PVR: pulmonary vascular resistance; FVC: forced vital capacity; % pred: % predicted; FEV1: forced expiratory volume in one second; Pa,O2: arterial oxygen tension; Pa,CO2: arterial carbon dioxide tension; RV: right ventricular; LV: left ventricular.