Status:

COMPLETED

Combination Therapy in Pulmonary Arterial Hypertension

Lead Sponsor:

Actelion

Conditions:

Pulmonary Arterial Hypertension

Eligibility:

All Genders

12+ years

Phase:

PHASE4

Brief Summary

An open label, non-comparative study design is appropriate for this Phase 4 study designed to assess whether a core therapy of bosentan, either as monotherapy or with the addition of sildenafil, enabl...

Eligibility Criteria

Inclusion

  • Signed informed consent prior to initiation of any study-mandated procedures.
  • Males or females ≥ 12 years of age (females of child-bearing potential must have been surgically sterilized or use a reliable method of contraception).
  • Symptomatic patients with the following types of PAH belonging to World Health Organization (WHO) Pulmonary Hypertension Classification Group I:
  • Idiopathic (IPAH)
  • Familial (FPAH)
  • Associated with PAH (APAH):
  • Collagen vascular disease
  • Drugs and toxins
  • Patients naïve to treatment with advanced PAH therapies (i.e., endothelin receptor antagonists (ERAs), phosphodiesterase-5 (PDE-5) inhibitors or prostacyclins) with a right heart catheterization (RHC) showing all of the following:
  • Mean pulmonary arterial pressure (mPAP) ≥ 25 mm Hg
  • Pulmonary capillary wedge pressure (PCWP) ≤ 15 mm Hg or left ventricular end diastolic pressure (LVEDP) ≤ 15 mm Hg when PCWP is not accurately obtained
  • Pulmonary vascular resistance ≥ 3 Wood units
  • 6-MWT distance ≥ 150 meters and \< 360 meters.

Exclusion

  • Patients with Pulmonary Hypertension (PH) belonging to WHO Classification Group II-V.
  • Patients with PAH (WHO PH Classification Group I) other than that listed in the Inclusion Criteria.
  • Pregnant and/or nursing.
  • Women of childbearing potential not using a reliable method of contraception.
  • Patients with known human immunodeficiency virus (HIV) infection.
  • Patients with significant vasoreactivity during right heart catheterization (i.e., a fall in mPAP to \< 40 mm Hg with a decrease of ≥ 10 mm Hg and with a normal cardiac index ≥ 2.5 l/min.m\^2).
  • Patients with restrictive lung disease (i.e., total lung capacity (TLC) \< 60% of normal predicted value).
  • Patients with obstructive lung disease (i.e., forced expiratory volume/ forced vital capacity (FEV1/FVC) \< 0.5).
  • Patients with impaired left ventricular function (LVEF \<50%) or diastolic dysfunction.
  • Patients with portal hypertension, cirrhosis, moderate to severe liver impairment (Child-Pugh Class B or C), or liver enzymes (Aspartate aminotransferase (AST) and/or alanine aminotransferase (ALT)) \> 3.0 times the upper limit of normal range.
  • Treatment with glibenclamide (glyburide) and calcineurin inhibitors (cyclosporine A, tacrolimus) sirolimus, everolimus up to 1 week prior to Baseline (Day 1).
  • Patients currently receiving or predicted to require treatment, during the course of the study, with nitrates, protease inhibitors, or alpha-blockers.
  • Patients with a hemoglobin concentration \< 75 % of the lower limit of the normal range or \< 8.5 g/dL.
  • Patients currently receiving or predicted to require treatment with a prostanoid during the course of the study.
  • Patients with systolic blood pressure \< 85 mm Hg.
  • Patients with body weight \< 40 kg.
  • Patients who have received any investigational product within 90 days prior to Baseline.
  • Patients who previously received any advanced therapy for PAH (e.g., ERAs, PDE-5 inhibitors or prostacyclins).
  • Patients with hypersensitivity to sildenafil or any excipients of its formulation.
  • Patients with any contraindication to sildenafil treatment (i.e., nitrates).
  • Patients with any recent medical condition limiting the ability to comply with the study requirements (i.e., stroke, myocardial infarction).
  • Patients with unstable PAH whose disease state would prohibit the completion of study procedures, in the opinion of the investigator.
  • Patients unable to complete a MRI scan (e.g., claustrophobia).
  • Patients with permanent cardiac pacemakers, automatic internal cardioverter defibrillators (AICD's), neurostimulators, hearing aides, and other implanted metallic devices that are contraindicated during a MRI study.
  • Patients with conditions that would interfere with proper cardiac gating during MRI, such as atrial fibrillation or multiple premature ventricular contractions (PVCs)/premature atrial contractions (PACs).
  • Patients with conditions that prevent compliance with the protocol or the ability to adhere to therapy.

Key Trial Info

Start Date :

March 1 2007

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

August 1 2010

Estimated Enrollment :

100 Patients enrolled

Trial Details

Trial ID

NCT00433329

Start Date

March 1 2007

End Date

August 1 2010

Last Update

February 4 2025

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