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A Journal on Diseases of the Respiratory System
Official Journal of the Italian Society of Thoracic Endoscopy
Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index
REVIEWS WHAT’S NEW IN RESPIRATORY MEDICINE IN 2010 - PART II
Minerva Pneumologica 2010 June;49(2):271-83
Therapy for pulmonary hypertension
Dweik R. A.
Pulmonary Vascular Program, Department of Pulmonary, Allergy, and Critical Care Medicine/Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA
The treatment of pulmonary arterial hypertension (PAH) is a testament to the success of modern medicine. One of its distinguishing features is that it is based on our knowledge and understanding of the ptahobiologic basis of this disease. Discoveries in three main pathobiologic pathways (nitric oxide, endothelin and prostacyclin) have revolutionized our approach to the treatment of PAH and allowed the development of effective therapies that have changed the course of this previously uniformly fatal disease. Based on our understanding of the disease, several targeted treatment options are currently available and include parenteral and inhaled prostanoids, oral endothelin receptor antagonists and oral phosphodiesterase type-5 inhibitors. Due to their complex care, patients with PAH should be referred to centers with expertise in pulmonary hypertension. Newer and probably more effective therapies are being developed and / or tested based on our improved understanding of the contributions and underlying mechanisms of certain pathobiologic features of the disease including cell proliferation and remodeling. Future therapies will likely reflect our improved understanding of the proliferative and vascular remodeling pathways and improved phenotyping of the disease.