RT Journal Article
SR Electronic
T1 A critical analysis of survival in pulmonary arterial hypertension
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 218
OP 222
DO 10.1183/09059180.00003512
VO 21
IS 125
A1 Dermot S. O'Callaghan
A1 Marc Humbert
YR 2012
UL http://err.ersjournals.com/content/21/125/218.abstract
AB Pulmonary arterial hypertension (PAH) is a rare disease characterised by a progressive increase in pulmonary vascular resistance, leading to right heart failure and premature death. Over the last two decades, a better understanding of the pathogenesis of PAH has led to the approval of several targeted therapies that confer improvements in patients’ clinical and haemodynamic status, quality of life and survival. Analysis of recent survival data in PAH cohorts have been biased by the inclusion of prevalent patients included months or years after PAH diagnosis (“survivors”) who have a better prognosis than corresponding incident patients diagnosed at the time of recruitment. A critical analysis of recent multicentre incident cohorts has shown survival improvements in the current management era. However, idiopathic PAH remains a severe disease and still carries a poor prognosis. Modern survival figures also highlight the relevance of lung transplantation in eligible PAH patients who are refractory to current best standard of medical care.