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Challenges in the diagnosis and treatment of pulmonary arterial hypertension

Jean-Luc Vachiéry, Sean Gaine
European Respiratory Review 2012 21: 313-320; DOI: 10.1183/09059180.00005412
Jean-Luc Vachiéry
*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.
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  • For correspondence: jvachier@ulb.ac.be
Sean Gaine
*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.
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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.

  • Combination therapy
  • diagnostic algorithms
  • pulmonary arterial hypertension-specific therapy
  • screening

Footnotes

  • Provenance

    Publication of this peer-reviewed article was supported by Actelion Pharmaceuticals Ltd, Switzerland (principal sponsor, European Respiratory Review issue 126).

  • Statement of Interest

    J-L. Vachiéry has served as a consultant and advisory board member for Actelion, GSK and United Therapeutics. He has received speaker fees from Actelion, Bayer Shering, GSK, Eli Lilly, Pfizer and United Therapeutics. S. Gaine has served as a consultant, advisory board member and has received speaker fees from Actelion, GSK, Pfizer and United Therapeutics.

  • Received September 10, 2012.
  • Accepted September 25, 2012.
  • ©ERS 2012
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Challenges in the diagnosis and treatment of pulmonary arterial hypertension
Jean-Luc Vachiéry, Sean Gaine
European Respiratory Review Dec 2012, 21 (126) 313-320; DOI: 10.1183/09059180.00005412

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Challenges in the diagnosis and treatment of pulmonary arterial hypertension
Jean-Luc Vachiéry, Sean Gaine
European Respiratory Review Dec 2012, 21 (126) 313-320; DOI: 10.1183/09059180.00005412
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  • Article
    • Abstract
    • THE CHALLENGE OF DIAGNOSIS IN PAH
    • CHALLENGES IN THE TREATMENT OF PAH
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