National Institutes of Health   Department of Health and Human Services

Meeting Focus

Emerging Diseases

In 2003, the 3rd World Conference on Pulmonary Hypertension proposed a new classification system that groups various types of pulmonary hypertension (PH) by similarities in clinical presentation, pathology, and response to medical therapies:

With this new classification, the category of PAH now includes idiopathic pulmonary arterial hypertension (IPAH, formerly primary pulmonary hypertension), familial pulmonary arterial hypertension (FPAH), and PH associated with a variety of conditions including connective tissue disease, congenital heart disease, anorexigen use, human immunodeficiency virus (HIV), portal hypertension, hemoglobinopathies, and myeloproliferative disorders. All of the clinical disorders that comprise pulmonary hypertension are characterized by progressive increases in pulmonary vascular resistance ultimately leading to right heart failure and death.

Novel Therapeutics

Vasoconstriction, pulmonary vascular remodeling, and in-situ thrombosis have been implicated in the pathogenesis of pulmonary hypertension. Current pharmacologic therapies attempt to redress the imbalance between endothelial cell-derived mediators that regulate pulmonary vascular tone and cellular proliferation. These therapies focus on the endothelin pathway (endothelin receptor antagonists), the nitric oxide pathway (exogenous nitric oxide and phosphodiesterase type 5 inhibitors), and the prostacyclin pathway (prostacyclin analogues). Although these therapies have improved clinical outcomes to varying degrees in patients with PAH, long-term survival is still limited and there remains no cure.

Recent research has focused on molecular mechanisms and pathways involved in pulmonary vascular remodeling. Dysfunctional or dysregulated apoptosis, collagen and extracellular matrix deposition, smooth muscle cell proliferation, growth factors, endothelial cell migration, inflammation, platelet activity, and thrombosis have been demonstrated in animal models of PAH and in humans. Elucidation of these cellular and molecular perturbations has provided potential novel targets for therapy.

Education Goals

Our goals for this conference are threefold: to provide a forum to present new concepts in basic, translational, and clinical pulmonary hypertension research; to promote scientific exchange of ideas; and, ultimately, to foster research collaboration.

Target Audience

Researcher, clinicians and other medical professionals, doctoral and post doctoral candidates, associations, patient and patient advocates, industry representatives, and journalists.