PW342 New scoring system for risk evaluation in Eisenmenger syndrome

O ST E R A B ST R A C T S Objectives: We sought to document the frequency and causes of death for those with a diagnosis of PAH complicating CHD, which has so far not been well described. Methods: We have established a Bi-National Registry of PAH-CHD cases from Australia and New Zealand, comprising 360 adults with a confirmed diagnosis of PAH and CHD and who have been seen at least once since Jan 1, 2000, at age over 16 years. Results: 70 patients are known to have died (31 males, age 37 13 years). The underlying causes of PAH were ASD (21%), PDA (23%), VSD (30%) and AVSD (16%); the others had more complex CHD lesions (12%). 73% had Eisenmenger Syndrome and 24% had Down’s Syndrome. Compared to survivors, the decedents were more likely to have a single ventricle (13% vs 4%; p<0.001) and were less likely to have Tetralogy of Fallot (1% vs 10%; p<0.02). There was no significant difference in the proportion of males, smokers or those with Eisenmenger Syndrome, Down’s, ASD, PDA, AVSD or associated lung disease in survivors compared with decedents. A similar proportion of patients in both groups (27%) had undergone an intervention for their congenital heart disease. The causes of death were varied and predominantly include cardiac arrest, right heart failure, renal failure and sepsis. Conclusion: Death is common in adults with PAH-CHD, often at a young age. The commonest causes are progressive heart failure and sudden cardiac deaths however sepsis and other causes have been documented. The effect of therapy on the risk and causes of death in this group requires further prospective study. Disclosure of Interest: None Declared