Prevention of Established Pulmonary Hypertension in High Risk patients with Fibrotic Lung Disease – a double-blinded, randomised, placebo controlled trial with endothelin-1 receptor antagonist therapy - Prevention of Pulmonary Hypertension in Interstitial Lung Disease

Conditions: Pulmonary hypertension in patients with interstitial lung disease. Specifically this trial looks at patients who have mild pulmonary hypertension, or are in an at-risk group to developing pulmonary hypertension, in the context of their interstitial lung disease. The interstitial lung diseases addressed include idiopathic pulmonary fibrosis and non-specific interstitial pneumonitis.
MedDRA version: 9.1Level: LLTClassification code 10064911Term: Pulmonary arterial hypertension

Registration Number: EUCTR2007-001645-17-GB

Lead Sponsor: Royal Brompton and Harefield NHS Trust

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: ot Recruiting

Sex: All

Target Recruitment: Not specified

Inclusion Criteria

1. Patients >=18yrs, <80yrs

2. Patients with Idiopathic Pulmonary Fibrosis or fibrotic Non-Specific Pneumonitis according to ATS/ERS criteria.

3. Patients with mild pulmonary hypertension or at risk of developing pulmonary hypertension (Pre-PH), as defined by:
o Mild-PH: RHC: mean PAH 25-35mmHg, PCWP<15mmHg, and 6-minute walk test oxygen desaturation <89%
o Pre-PH: RHC: mean PAP <25mmHg, PCWP<15mmHg, 6-minute walk test oxygen desaturation <89%

4. Patients providing written informed consent.
Are the trial subjects under 18? no
Number of subjects for this age range:
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range
F.1.3 Elderly (>=65 years) yes
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

1. Patients <18, >80yrs.

2. Patients with unstable disease, or an acute exacerbation of their underlying fibrotic lung disease.

3. Patients with significant other organ co-morbidity including hepatic or renal impairment.

4. Patients with systolic BP < 85mmHg

5. Patients with other conditions that may affect the ability to perform a 6-minute walk test.

6. Patients unable to provide informed consent and comply with the patient protocol.

7. Patients receiving excluded medications (including: epoprostenol, or prostacyclin analogues, phosphodiesterase inhibitors, other endothelin receptor antagonists, drugs with potential interaction with bosentan such as glibenclamide, fluconazole, cyclosporin A, or tacrolimus, and other invesgational agents).

8. Patients with planned surgical intervention during the study period.

9. Pregnant patients

10. Patients with resting oxygen saturation <90%

11. Patients with clinically overt ischaemic heart disease

12. Patients with predominant emphysema on CT scan (in excess of interstitial changes)