Current management of pulmonary arterial hypertension.

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State: Public
Version: Final published version
Serval ID
serval:BIB_FA8CF3CAA3EF
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Current management of pulmonary arterial hypertension.
Journal
Swiss Medical Weekly
Author(s)
Yerly P., Prella M., Aubert J.D.
ISSN
1424-3997 (Electronic)
ISSN-L
0036-7672
Publication state
Published
Issued date
2016
Peer-reviewed
Oui
Volume
146
Pages
w14305
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Pulmonary arterial hypertension (PAH) is a vascular disease of unknown aetiology, characterised by an abnormal thickening of the arterial wall that is responsible for an increase in pulmonary vascular resistance. The haemodynamic consequence of PAH is an increased afterload for the right ventricle and, eventually, right heart failure. When untreated, PAH has a grim prognosis with a median survival of about 2 to 4 years from diagnosis. In the last 10 years new orally administered compounds have demonstrated clinical efficacy in controlled trials using various surrogate endpoints to survival. Although the disease remains without cure until now, the available phase III trials have allowed evidence-based recommendations for the medical management of these patients to be established. It appears, however, that none of the compounds from the three main therapeutic classes, endothelin receptor antagonists, agents acting on the nitric oxide-cyclic guanosine monophosphate pathway (including phosphodiesterase type 5 inhibitors and guanylate cyclase stimulator), and prostanoid receptor agonists are able alone to control disease progression in every patient. Therefore combination therapy with two or three drugs may be necessary in a significant number of patients in order to maintain patients in, or bring them to, a low risk profile. Several recent studies have now validated this approach for specific double or triple drug regimens. It remains, however, unclear whether an upfront combination is preferable to a sequential step-up approach based on clinical response. In addition, some specific combination therapies have failed to demonstrate superiority to single drug alone in randomised controlled trials. Besides PAH-specific treatment, the place of nonspecific pharmaceutical and nonpharmaceutical treatment has been also recently clarified.
Pubmed
Web of science
Open Access
Yes
Create date
14/06/2016 17:11
Last modification date
20/08/2019 17:26
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