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. Pulmonary arterial hypertension (PAH) is a condition characterized by vasoconstriction and proliferation of the endothelial and smooth muscle cells within the pulmonary arteries, which leads to elevation of the pulmonary artery pressure. Left untreated, PAH is often progressive and fatal. PAH may be idiopathic (primary) without any known cause. Some cases of PAH are familial. PAH may also be secondary to a specific disease process such as portal hypertension, congenital heart disease, chronic lung disease, thromboembolic disease, connective tissue disease, HIV, and use of anorexigens. There is no cure for PAH. Therapy focuses upon treatment of secondary causes if present and reduction of the pulmonary artery pressure through medical therapy. The Children's Hospital Pulmonary Hypertension Program has been conducting a longitudinal study entitled 'Prospective, Longitudinal Study of Childhood Pulmonary Hypertension' since approved by COMIRB July 13, 1999. The focus of the longitudinal study was to evaluate the growth, development, quality of life, and cardiovascular response of chronic therapy for PAH in children. There have been many new developments within the past few years in the management of patients with PAH. Thus, this protocol will incorporate these newer therapies and findings as the incidence, clinical course, possible biomarkers of response, and long terms effects of treatment in children remains largely unknown.
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