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    Challenges in the diagnosis and treatment of pulmonary arterial hypertension.

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    Authors
    Vachiéry, Jean-Luc
    Gaine, Sean
    Affiliation
    Pulmonary Vascular Diseases and Heart Failure Clinic, Cliniques Universitaires de Bruxelles, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium. # National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland.
    Issue Date
    2012-12-01
    
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    Citation
    Challenges in the diagnosis and treatment of pulmonary arterial hypertension. 2012, 21 (126):313-20 Eur Respir Rev
    Journal
    European respiratory review : an official journal of the European Respiratory Society
    URI
    http://hdl.handle.net/10147/255061
    http://err.ersjournals.com/cgi/content/full/21/126/313?ijkey=IfBwQvmSo9332&keytype=ref&siteid=ersjnls
    DOI
    10.1183/09059180.00005412
    PubMed ID
    23204119
    Abstract
    Advances in the diagnosis and management of pulmonary arterial hypertension (PAH) have resulted in significant improvements in outcomes for patients with this devastating and progressive disease. However, because of the non-specific nature of its symptoms, and the low level of suspicion among clinicians, prompt and accurate diagnosis of PAH as a rare disease remains a challenge. This article explains some of the issues that need to be addressed when faced with a patient with suspected PAH and describes how noninvasive and invasive techniques can be used effectively to ensure an accurate diagnosis. The availability of PAH-specific therapy means that once diagnosed, patients have a much greater chance of survival than they would have had in the past. However, despite improved survival, mortality is still high and, therefore, there is still room for improvement. It is currently recommended that patients with an inadequate clinical response to treatment receive sequential combination therapy; however, supportive data are still scarce. Although there is no clear explanation, these findings may be explained by the design and end-points chosen in clinical trials, the changing population of PAH and a need to improve the management strategy in this disease. Indeed, there is a clear need for randomised controlled studies that investigate whether adopting individualised treatment strategies, including upfront combination therapy, could help to optimise long-term management of patients with PAH.
    Item Type
    Article
    Language
    en
    ISSN
    1600-0617
    ae974a485f413a2113503eed53cd6c53
    10.1183/09059180.00005412
    Scopus Count
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    Mater Misericordiae Hospital

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