SEARCHING FOR CLUES TO THE PATHOBIOLOGY OF PULMONARY HYPERTENSION

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The trigger and mechanism by which pulmonary hypertension (increase in pressures in the arteries of the lungs) develops in scleroderma, congenital heart disease, and idiopathic pulmonary hypertension is not clear. Further, there are no reliable markers of early disease. A better understanding of the pathobiology of pulmonary hypertension could lead to earlier detection and novel therapies. We propose to investigate the role of certain markers of pulmonary vascular injury and dysfunction, factors involved in vascular remodeling, and the potential contribution of infection with chlamydia pneumonia in the pathobiology of pulmonary hypertension. The variables will be compared in patients with pulmonary arterial hypertension, idiopathic pulmonary fibrosis, scleroderma with and without pulmonary hypertension, and primary Raynaud s disease. There will be no gender, ethnic or racial restrictions. The study participants will be limited by age to 60 years in men and 65 years in women, and have no atherosclerotic diseases or major coronary risk factors so as to limit the potential impact of clinical or subclinical atherosclerosis on the results. There are no vulnerable populations. The initial requirement of the patient is to provide a 60ml sample of blood, have a cardiac ultrasound, and permission to review their records. We will correlate the levels of biomarkers with the presence and severity of pulmonary hypertension. The research database will identify each patient by numeric code. The code will be matched with the patient University CPI number and the file will be available to the senior investigators and project administrator. Eligible patients will be approached to participate by one of the investigators and provided written informed consent. If support is available participants will be followed for up to 5 years to determine whether any of the markers predict the development, clinical outcome, or rate of progression of pulmonary hypertension. Participants may be requested to return at intervals of 2 and 4 years for a physical exam, repeat blood draw, and a cardiac ultrasound. University patient records will be followed annually to determine the relationship between the biomarkers and the rate of progression and development of pulmonary hypertension. Patients not followed in the University will be requested to allow there records to be sent to the study team and may be requested to return for clinical reevaluation.