Age

10–80

Sex

ALL

Healthy volunteers

Not accepted

aged 10 to 80
treatment naive symptomatic PAH
6-MWD of 100-425 m
resting mean pulmonary artery pressure greater than 25 mm Hg, pulmonary capillary wedge pressure less than 15 mm Hg, and pulmonary vascular resistance of 240 dyn.s.cm-5 or greater.

Patients with thromboembolic disease,
untreated obstructive sleep apnea,
portal hypertension,
chronic liver disease or renal insufficiency,
left-sided or unrepaired congenital heart disease,
substantial obstructive (FEV1/FVC\<50% predicted) or restrictive (total lung capacity\<60% predicted) lung disease
Patients receiving phosphodiesterase inhibitors or other prostanoids and endothelin receptor antagonists

Study of the Initial Combination of Bosentan With Iloprost in the Treatment of Pulmonary Hypertension Patients