Please login or register to print this page.

The British Journal
of Cardiology

This website is intended for healthcare professionals only

Moving forward in pulmonary arterial hypertension

January 2009    Volume 16, Supplement 1   Br J Cardiol 2009;16(Suppl 1):S2-S3

Authors:
Henry Purcell

Sponsorship statement: The symposium was sponsored by GlaxoSmithKline, who also sponsored the writing of this report and this supplement. Volibris™ ▼ is a registered trademark of Gilead (Nasdaq: GILD), used under license by the GlaxoSmithKline group of companies. Ambrisentan has been licensed to GlaxoSmithKline by Gilead Sciences Inc. in all countries of the world, except the United States (US).

Pulmonary arterial hypertension (PAH) is a comparatively rare, chronic, progressive disease of unknown aetiology, which is characterised by increased pulmonary vascular resistance and which may ultimately lead to right heart failure and premature death.1 In a recent French registry the estimated prevalence was 15 cases per million, with approximately twice as many women as men being diagnosed.2 PAH is increasingly diagnosed in older people, who may have considerable co-morbidities compared to the younger PAH patients traditionally seen.2

Diagnosis can be challenging as its symptoms are often non-specific: they may include breathlessness, fatigue, weakness, angina, syncope and abdominal distension. In the mid-1980s, before the availability of ‘targeted’ therapy, median life expectancy from diagnosis in patients with idiopathic PAH (formerly termed primary pulmonary hypertension [PPH]) was only 2.8 years.3 In 1996, continuous intravenous prostacyclin (epoprostenol) was the first drug to demonstrate outcome benefit in PAH.4 Subsequently, over the past ten years, randomised, placebo-controlled trials of other prostacyclin analogues, endothelin receptor antagonists and phosphodiesterase inhibitors have shown significant benefit to patients with PAH, with improvements in exercise capacity, functional class and other parameters.5 For those patients who fail to respond to medical therapy, double-lung or heart-lung transplantation may be an option.6

This supplement is a report from the symposium ‘Moving forward in pulmonary arterial hypertension’, held on 1st September 2008 during the European Society of Cardiology Congress in Munich, Germany. The meeting was chaired by Dr Sean Gaine, Mater Misericordiae University Hospital, Dublin, Ireland, and Dr Simon Gibbs, Imperial College London and Hammersmith Hospital, London, UK and was sponsored by an educational grant from GSK.

The symposium highlighted how understanding of the pathobiology of PAH has evolved over the past two decades, as has the treatment of this condition. With the availability of newer treatment agents, and with increasing use of combination therapy to enhance clinical benefit, along with the need to begin treatment earlier, the PAH picture continues to unfold. It offers many challenges for the years to come, which makes this one of the most rapidly evolving fields within cardiology, and indeed within medicine as a whole. We hope that this is an objective and informative review of the symposium.

Br-J-Cardiol-2009-16-S1-S2-S3_WHO_Table

WHO Functional Classification of Pulmonary Hypertension

References

  1. Galie N, Simmoneau G. Pulmonary hypertension. In: The ESC Textbook of Cardiovascular Medicine. Eds Camm AJ, Luescher TF, Serruys PS. Blackwell Publishing, 2006.
  2. Humbert M, Sitbon O. Chaouat A et al. Pulmonary Arterial Hypertension in France. Results from a National Registry. Am J Respir Crit Care Med 2006;173:1023–30.
  3. D’Alonzo GE, Barst RJ, Ayres SM et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991;115:343–9.
  4. Barst RJ, Rubin LJ, Long WA et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996;334:296–302.
  5. National Pulmonary Hypertension Centres of the UK and Ireland. Consensus statement on the management of pulmonary arterial hypertension in clinical practice in the UK and Ireland. Heart 2008;94:i1–i41.
  6. Galie N, Torbicki A, Barst R et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology.Eur Heart J 2004;25:2243–78.

Switch from print to digital today and win a portable heart monitor

Register Now

Close

Disclaimer: UK prescribing information current at the date of publication of this supplement can be found by downloading the PDF. Medinews Cardiology Limited advises healthcare professionals to consult up-to-date Prescribing Information and the full Summary of Product Characteristics available from the manufacturers before prescribing any product. Medinews Cardiology Limited cannot accept responsibility for any errors in prescribing which may occur.

back to top

Comments

There are currently no comments for this article - leave a comment

You must be logged in to post a comment.
Not yet a member? Register now for free.

back to top

 For healthcare professionals only

Close

You are not logged in

You need to be a member to print this page.
Sign up for free membership, or log in.

Find out more about our membership benefits

Close

You are not logged in

You need to be a member to download PDF's.
Sign up for free membership, or log in.

Find out more about our membership benefits