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. Cystic fibrosis (CF) lung disease is characterized by persistent airway inflammation and parenchymal destruction. This leads to a progressive decline in pulmonary function and periods of chronic intermittent hypoxia. Progressive pulmonary vascular disease (PVD) due to hypoxia and inflammation increases pulmonary vascular resistance and may lead to overt right ventricular failure. Early clinical manifestations of PVD prior to the development of cardiac involvement include limitations in exercise tolerance and dyspnea. The extent to which PVD contributes to the decline in exercise tolerance and quality of life in CF patients is not known. Early evidence of PVD may be recognized in CF patients through standardized exercise testing and echocardiographic evaluation. Identifying those CF patients with PVD prior to the onset of right ventricular involvement may allow pharmacologic intervention to attenuate the progression of this disease. Recent studies have demonstrated the administration of the phosphodiesterase type 5-inhibitor sildenafil can markedly decrease pulmonary vascular resistance and improve exercise tolerance in non-CF patients. Sildenafil may prove to be an efficacious oral pharmacologic agent for the treatment of pulmonary vascular disease in patients with CF.
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