Moving forward in pulmonary arterial hypertension

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 1^st 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.

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.