Integrated care and optimal management of pulmonary arterial hypertension

Abstract

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 PAH-specific 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.

Keywords:

• pulmonary hypertension
• multidisciplinary care
• systemic sclerosis
• diagnostic protocol

Disclosures

EG has received research support from Actelion Pharmaceuticals Australia and CSL as well as honoraria for speaking and consulting engagements, and as a member of the Actelion, CSL and GSK Advisory Boards. The Heart and Lung Transplant Foundation of WA, of which EG is chair has received educational grants from Actelion, and CSL. EG has also received travel support from Bayer-Schering the manufacturers of iloprost, Encysive Pharmaceuticals, the manufacturers of sitaxentan and GSK, the distributors of ambrisentan in Australia.

GS is an employee of Actelion Pharmaceuticals Australia. BD has received consulting fees from Actelion.