Evaluation of tolerability and efficacy of the combination Sildenafil/Bosentan in patients with severe pulmonary hypertension.

• Conditions: severe pulmonary hypertension; MedDRA version: 14.0Level: HLTClassification code 10037401Term: Pulmonary hypertensionsSystem Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders; Therapeutic area: Diseases [C] - Cardiovascular Diseases [C14]

• Registration Number: EUCTR2009-017155-10-IT
• Lead Sponsor: CASA DI CURA PRIVATA S.RAFFAELE - PISANA

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Study Start: 2011-12-06
• Last Update Posted: 19 March 2012

Recruitment & Eligibility

• Status: ot Recruiting
• Sex: All
• Target Recruitment: 60

Inclusion Criteria

Inclusion criteria 1) Patients with idiopathic or familial pulmonary arterial hypertension (PAH) 2) Patients with distal Chronic Thromboembolic Pulmonary Hypertension 3) PAH associated to connective tissue disease, anorexigen, congenital heart disease. 4) Age > 18 years 5) Mean pulmonary pressure > 25 mmHg, mean pulmonary capillary ''wedge'' pressure <15 mm Hg at right catheterization evaluation 6) No vasodilating response to inhaled nitric oxide, or ''vasoreactive'' patients in chronic calcium channel blockers 7) WHO functional class 2-3
Are the trial subjects under 18? no
Number of subjects for this age range: 0
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) Significant reduction in pulmonary function test (FVC, FEV1 < 70 % normal predicted value) 2) Treatment with other drug for PAH (endothelin receptor antagonist, prostanoids, PDE-5 inhibitors 3) Previous combination therapy with current drugs 4) Systemic hypotension (systolic blood pressure < 90 mmHg) 5) Chronic liver disease (Child-Pugh class B o C) or transaminases increased > 3 fold upper normal limits 6) Pshyschiatric disease or other condition that will be an impediment to the protocol 7) Pregnancy or lactation 8) Known hypersensitivity to bosentan or sildenafil 9) Treatment with other drugs that could have significant interaction with bosentan or sildenafil: calcineurine inhibitors (ciclosporine tacrolimus…), antimycotics (fluconazole, chetoconazole…), amiodarone, antiretroviral drug, glibencamide, nitrates, alfa-blockers.

Study & Design

• Study Type: Interventional clinical trial of medicinal product
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Main Objective: The purpose of this study is to evaluate the tolerability and clinical efficacy of an oral combination therapy Bosentan-Sildenafil, in patients with severe pulmonary hypertension due to PAH or distal chronic thromboembolic pulmonary hypertension.;Secondary Objective: The study will be extended also to patients with distal CTEPH because: - These patients present, beside proximal vessels obstruction, with distal lesion involving small and medium size pulmonary arteries similar to other form of severe pulmonary hypertension. - Furthermore an open uncontrolled study suggest beneficial hemodynamic effect after epoprostenol , sildenafil and bosentan therapy.;Primary end point(s): Primary endpoints will be: - Improvement of functional capacity compared to the basal conditions and to the period of monotherapy evaluated through distance 6 minutes walking test. - Improvement of the quality of life (WHOQOL-BRIEF questionnaire)