The past decade has witnessed unprecedented change in the field of pulmonary hypertension (PH). PH has previously been called an orphan disease; that is, a condition that affects few individuals and is overlooked by the medical profession and pharmaceutical companies [1]. Although undoubtedly rare, the concept that PH is “overlooked” cannot be considered to be the case today. In recent years there have been a number of important publications in the field that have significantly improved our understanding of PH, helped guide patient management and laid foundations for future research [2]. The articles in this issue of the European Respiratory Review aim to discuss such contemporary issues in PH. The authors, all experts in the field of PH, delivered the presentations upon which the papers are based at two international meetings sponsored by Actelion Pharmaceuticals (Allschwil, Switzerland). These meetings – the Fourth International Systemic Sclerosis Forum, Barcelona, Spain, February 5–6, 2011 and the Tenth International Pulmonary Hypertension Forum, Munich, Germany, March 26–27, 2011 – were each attended by over 1,000 healthcare professionals from all over the world, thus emphasising the increasing global interest in this devastating disease.
The clinical classification of PH has evolved since the initial proposal at the first international conference on primary PH in 1973 [3]. Two updates, one from the Second and Third World Symposia on Pulmonary Hypertension in Evian, France, 1998 and Venice, Italy, 2004 [4], and the other from the Fourth World Symposium on Pulmonary Hypertension in Dana Point, CA, USA, 2008 [5] have been published in the past 10 yrs. The more recent set of guidelines, based on the proceedings from Dana Point [5, 6], have been jointly published by the European Society of Cardiology (ESC) and European Respiratory Society …
