Pulmonary arterial hypertension (PAH) is characterized by progressive obliteration of pulmonary arterioles leading to increased pulmonary vascular resistance, right heart failure, and death in 40-60% of PAH patients 5 years after diagnosis. Proliferation, migration, and resistance to apoptosis of vascular cells, as well as proliferative-inflammatory responses mediated by blood and fat cells contribute to disease development. Despite more than 15 published randomized controlled trials (RCT) that demonstrated moderate effectiveness in improving exercise capacity, the approved PH drugs have not led to a cure. Hence, basic science and translational research focusing on the discovery of novel pathways in pulmonary vascular disease and right ventricular dysfunction, new drug targets, development of novel therapeutic paradigms, cell-based and pharmacotherapies, and their translation into RCTs, is urgently needed.
This Keystone Symposia meeting will gather basic and clinical researchers working in the field of pulmonary vascular disease and right ventricular dysfunction. The focus is on basic science with strong impact on 1) our understanding of this fatal disease, and 2) discoveries with great potential to be translated into clinical practice in the near future. Late-breaking clinical studies on novel PAH therapies will also be presented.