CLINICAL AND BIOLOGICAL PREDICTORS OF TREATMENT RESPONSE IN IDIOPATHIC PULMON

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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. GCRC Resource Utilization: Exhaled Nitric Oxide Collection and Forearm Pleythsmography Abstract: Medical Therapies have revolutionized the treatment of IPAH over the past decade and have delayed or eliminated the need for lung transplantation for many patients. However, lung transplantation continues to have a role in the care of patients for whom medical treatment fails. Unfortunately, this has resulted in a group of patients who are very sick and extremely high-risk presenting for transplantation. Thus, timing and patient selection has become a very difficult area in the treatment of these patients. Clinical markers of treatment failure, such as hemodynamic monitoring, are often very late signs and treatment failure is very difficult to define. Noninvasive testing for patients with pulmonary hypertension to evaluate the response to therapy is needed, but not available currently. We and others have shown the exhaled nitric oxide levels are lower in patients with pulmonary hypertension than in healthy controls and that nitric oxide reaction products in the bronchoalveolar lavage fluid is inversely related to the degree of pulmonary hypertension. In patients that begin vasodilator therapy for IPAH, an increase in their exhaled nitric oxide levels is seen. We have also seen that no measure over time can predict mortality in IPAH patients. We hypothesize that IPAH is a nitric oxide deficiency state and monitoring nitric oxide by various methods may be used to monitor IPAH over time and response to therapy.