A National Pulmonary Hypertension Service for England & Wales: an orphan disease is adopted?
- National Pulmonary Hypertension Service, London Centre, Royal Brompton and Hammersmith Hospitals, London, UK
- Correspondence to:
Professor T W Evans, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK;
t.evans{at}rbh.nthames.nhs.uk
A National Pulmonary Hypertension Service has been set up in the UK, recognising the need for change in the way this complex and relatively rare condition is investigated and managed in response to therapeutic breakthroughs.
In pulmonary hypertension (PH) resting mean pulmonary artery pressure exceeds 25 mm Hg at rest or 30 mm Hg during exercise. PH may complicate many different clinical disease processes or may develop as a primary phenomenon (PPH). Post mortem studies estimate the prevalence to be 1300 per million.1 The incidence of PPH in Europe and the USA is 1–2 cases per million population per year, rising to as high as 25–50 per million per year in patients using certain appetite suppressants.2 The mean age at diagnosis is 36 years with a female preponderance (1.7–3.5:1). There is no ethnic predisposition but familial PPH accounts for roughly 10% of cases.3
In patients with PPH both vasoconstriction and later thickening of the peripheral “resistance” vessel walls (remodelling) with associated thrombosis leads to raised pulmonary vascular resistance. Most of the structural changes that occur in these vessels are attributable to the proliferation of vascular smooth muscle cells. The mechanisms …
