Innovations in the management of pulmonary hypertension

Investigational projects within the Pulmonary Hypertension Clinic are intended to improve therapy of pulmonary hypertensive syndromes ? including idiopathic pulmonary arterial hypertension (idiopathic PAH), and pulmonary hypertension associated with connective tissue disease (CTD), liver disease, congenital heart disease (CHD), HIV infection, and appetite suppressant use. We have been, and continue to be, a major participant in clinical studies of investigational drugs. This has included in the past (1) the investigational use of intravenous epoprostenol (prostacyclin; Flolan) in idiopathic and scleroderma-related PAH, (2) clinical study protocols of the prostacyclin analogs UT-15 and beraprost in pulmonary hypertension and of the non-selective endothelin (ET) antagonist bosentan in PAH, (3)the assessment of a thromboxane inhibitor in PAH, and (4) the effect of sildenafil on PAH.

Clinical studies of selective ET-A antagonists sitaxsentan and ambrisentan are underway, and will be followed by open label use of the drug for participants. Neurohormonal profiles and responses of patients with various types of pulmonary hypertension undergoing treatment are being investigated by Dr. Robert Frantz. p>

From a diagnostic standpoint, a novel method of continuously monitoring pulmonary hemodynamic responses to treatment using an implanted device is currently under investigation.