Late-breaking abstract: Demographic trends and changes In long term outcome of incident idiopathic, heritable and anorexigen-associated pulmonary arterial hypertension between 2001 to 2009. Results from the Pulmonary Hypertension Registry of the United Kingdom and Ireland

Background: There have been significant changes in the management of pulmonary arterial hypertension (PAH) over the past decade. In the UK and Ireland, care of pulmonary hypertension (PH) is centralised to designated PH centres. This provides an excellent opportunity to study changes in demographic and survival trends of the disease within an entire region with a common healthcare system. Aim: To determine whether baseline characteristics and survival of incident IPAH, heritable and anorexigen-associated PAH has changed over the past decade. Methods: Retrospective observational study of all incident cases of IPAH, heritable and anorexigen-associated PAH diagnosed in the UK and Ireland between 1st January 2001 and 31st December 2009. Results: Total of 646 patients were diagnosed (22% in 2001-2003, 33% in 2004-2006 and 45% in 2007-2009). In recent years, patients were older, had higher BMI, lower % predicted diffusion capacity for carbon monoxide (DLCO) and had more co-morbidities (COPD, ischaemic heart disease and diabetes). There is no difference in unadjusted overall survival from 2001 to 2009. However, after adjusting for age, % DLCO, 6 minute walk distance and cardiac index, patients diagnosed in the 2001-2003 period were likely to have a shorter survival time compared to those diagnosed in 2007-2009 (hazard ratio 1.7, 95% CI 1.1-2.6) Conclusion: After adjusting for potential confounders, survival of incident IPAH, heritable and anorexigen-associated PAH in the UK and Ireland has improved over the last decade.