Pharmacogenomics in Pulmonary Arterial Hypertension

• Status: Unknown status
• Trial end date: 2013-07-01
• Target enrollment: 0
• Participant gender: All

Summary

Our goal is to determine clinically in Pulmonary Arterial Hypertension patients if associations exist between the efficacy and toxicity of sitaxsentan, bosentan, and ambrisentan and several gene polymorphisms in several key disease-specific and therapy specific genes. Also characterized is the relationship between these polymorphisms and the severity of Pulmonary Arterial Hypertension using either baseline hemodynamic or clinical surrogates for disease severity.

Hypothesis: Polymorphisms influence the efficacy and toxicity of specific Pulmonary Arterial Hypertension therapy as well as development/severity of PAH via their effect on PA remodeling, drug response, or metabolism.

This study requires a one time 8.5 ml blood sample and clinical data to be obtained at initiation of therapy, 4 months after initiation of therapy and 12 months after initiation of therapy.

• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: West Penn Allegheny Health System
• Collaborators: Bay Area Chest Physicians; Baylor College of Medicine; Chest Medical Associates; Children's Hospital Colorado; Columbia University; Duke University; Emory University; Johns Hopkins University; Latter Day Saints Hospital; London Health Sciences Centre; Louisiana State University Health Sciences Center in New Orleans; Lung Diagnostics, Ltd.; Massachusetts General Hospital; Mayo Clinic; Medical College of Wisconsin; National Institutes of Health (NIH); Ohio State University; Sentara Norfolk General Hospital; Sir Mortimer B. Davis - Jewish General Hospital; Southeastern Lung Care; Suncoast Lung Center; The University of Texas Medical Branch, Galveston; Tufts Medical Center; University Hospitals Cleveland Medical Center; University of Alabama at Birmingham; University of Calgary; University of California, Los Angeles; University of California, San Francisco; University of Chicago; University of Colorado, Denver; University of Maryland; University of Maryland, College Park; University of Michigan; University of Pittsburgh; University of Southern California; University of Texas Southwestern Medical Center; Vanderbilt University; Vanderbilt University Medical Center; Washington University School of Medicine; Wayne State University
• Treatments: Ambrisentan; Bosentan; Endothelin Receptor Antagonists; Sitaxsentan

Criteria

Inclusion Criteria:

GROUP 1

• Patients have to be currently enrolled or previously enrolled in STRIDE FPH01, FPH01-XC FPH02, FPH02x, FPH03, FPH04 or FPH06.

• WHO Group 1 Pulmonary Arterial Hypertension: Idiopathic, Familial, Associated with (APAH) Collagen vascular disease, congenital systemic-to-pulmonary shunts, portal hypertension, Drugs and toxins (e.g., anorexigens, rapeseed oil, L-tryptophan, methamphetamine, and cocaine), other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy) Associated with significant venous or capillary involvement, Pulmonary veno-occlusive disease, Pulmonary-capillary hemangiomatosis.

GROUP 2

• Patients currently receiving bosentan or ambrisentan OR who have previously received bosentan or ambrisentan for greater than 4 (four) months.

• WHO Group 1 Pulmonary Arterial Hypertension: Idiopathic, Familial, Associated with (APAH), collagen vascular disease, congenital systemic-to-pulmonary shunts, portal hypertension, drugs and toxins (e.g., anorexigens, rapeseed oil, L-tryptophan, methamphetamine, and cocaine), other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, or splenectomy), associated with significant venous or capillary involvement, pulmonary veno-occlusive disease, or pulmonary capillary hemangiomatosis.

Exclusion Criteria:

GROUP 1

• Not enrolled in the Encysive Pharmaceutical's STRIDE study(sitaxsentan).

• Known infectious disease (HIV, Hepatitis).

GROUP 2

• Never enrolled in the STRIDE study for sitaxsentan patients.

• Not currently or previously on bosentan or ambrisentan.

• Patients who were previously on bosentan or ambrisentan must have been on bosentan or ambrisentan for greater than 4 months.

• Known infectious disease (HIV, Hepatitis).