en years ago, the prognosis of patients with pulmonary hypertension
was poor. At that time, no pharmaceutical treatment was effective
in all patients. This has changed dramatically with the introduction of
inhaled prostacyclin as maintenance therapy. Endothelial antagonists and
phosphodiesterase inhibitors, both based on pathophysiological considera-
tions and used as monotherapy or in combination, have extended the
spectrum of treatment possibilities. All of the drugs used have been shown to
be efficient with regard to the different outcome measures, including
haemodynamic parameters, exercise tolerance and life quality. As a result,
observational studies have demonstrated an incredible increase in the life
expectancy of patients with PH.
When the development of new therapies began, idiopathic pulmonary arterial
hypertension (IPAH) was the focus of research. The therapeutic success in this
field initiated studies in patients with different forms of secondary PH, starting
with PH associated with connective tissue disease, followed by patients with
interstitial lung fibrosis or chronic thromboembolism, and then moving on to
those with chronic obstructive pulmonary disease (COPD) or congestive heart
failure. Huge randomised controlled trials have been performed for most of these
indications, meaning it has been possible to establish very specific algorithms for
diagnosis and treatment with regard to the baseline disease causing PH. Due to
the intensive collaboration of PH researchers all over the world and a special
effort within the PH community, guidelines have been developed and quickly
corrected in the light of new experiences and findings, making PH an example of
standardised management of disease on a worldwide scale.
However, the story of PH continues. Basic research in PH has raised
interesting new concepts for further treatment options. The pharmaceutical
industry and public research institutes are also working together to consider
different methods of diagnosis and treatment. And personalised medicine
based on pathophysiological concepts and risk stratification is no longer just
a vision, as work is underway to improve outcome of patients with PH.
This issue of the European Respiratory Monograph summarises the current
pathophysiology, diagnosis, and pharmacological and non-pharmacological
treatment of PH. The best experts in the field have contributed to this book,
which should be of interest not only to basic scientists and clinicians, but also
to those in the pharmaceutical industry, as it provides in-depth consideration
of the future of PH. The issue is also the first to include continuing medical
education (CME) questions (accredited by the European Board for
Accreditation in Pneumology (EBAP)), meaning it will be particularly
attractive to those in training. To earn 5 CME credits, simply read the issue
and answer the questions at the back of the book and provided online.
I want to personally congratulate the Guest Editors of this excellent
Monograph, which provides an excellent overview of PH. Marius and Marc,
thank you very much for a fantastic collaboration. I am convinced that this
Monograph will be a major success.
Editor in Chief
Eur Respir Monogr 2012; 57: vi. Copyright ERS 2012. DOI: 10.1183/1025448x.10024312. Print ISBN: 978-1-84984-025-5.
Online ISBN: 978-1-84984-026-2. Print ISSN: 1025-448x. Online ISSN: 2075-6674.