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Pulmonary arterial hypertension (PAH) may occur as an idiopathic process or as a component of a variety of diseases, including connective tissue diseases, congenital heart disease, and exposure to appetite suppressants or infectious agents such as HIV. Untreated, it is a potentially devastating disease; however, diagnosis can be difficult due to the non-specific nature of symptoms during the early stages, and the fact that patients often present to a range of different medical specialties. The past decade has seen remarkable improvements in our understanding of the pathology associated with the condition and the development of PAHspecific therapies with the ability to alter the natural history of the disease. This article reviews the evidence for screening and diagnosis of susceptible patient groups and discusses treatment selection and recommendations based on data available from randomized controlled trials. In addition, due to the complexity of the diagnostic evaluation required and the treatment options available, this review mandates for a multidisciplinary approach to the management of PAH. We discuss the roles and organizational structure of a specialized PAH center in Perth, Western Australia to highlight these issues.

Item Type:	Refereed Article
Keywords:	pulmonary hypertension, multidisciplinary care, systemic sclerosis, diagnostic
Research Division:	Medical and Health Sciences
Research Group:	Cardiorespiratory Medicine and Haematology
Research Field:	Cardiology (incl. Cardiovascular Diseases)
Objective Division:	Health
Objective Group:	Clinical Health (Organs, Diseases and Abnormal Conditions)
Objective Field:	Cardiovascular System and Diseases
Author:	Dalton, BS (Mr Brad Dalton)
ID Code:	58228
Year Published:	2009
Deposited By:	Health Sciences A
Deposited On:	2009-09-22
Last Modified:	2014-12-17
Downloads:	313 View Download Statistics