A National Pulmonary Hypertension Service for England & Wales: an orphan disease is adopted?

Evans, T. W.; Gatzoulis, M. A.; Gibbs, J. S. R.
June 2002
Thorax;Jun2002, Vol. 57 Issue 6, p471
Academic Journal
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. 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. The mean age at diagnosis is 36 years with a female preponderance. There is no ethnic predisposition but familial PPH accounts for roughly 10% of cases.


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