Eisenmenger syndrome - Factors relating to deterioration and death

Aims To assess the natural history, risk factors for death and deterioratio n of patients with Eisenmenger Syndrome. Methods The clinical course of 188 patients from three different cardiac ce ntres specialized in adult congenital heart defects, followed for a median period of 31 years, was retrospectively analysed. According to the diagnosi s, 64 males and 124 females, mean age at last follow-up 33.02 +/- 12.8 year s, were divided into two groups: simple (128) and complex (60) congenital h eart disease. Events analysed were: time variation of Ability Index, pulmon ary and cerebral complications, non-cardiac surgery, urate metabolism and r enal function, arrhythmias, pregnancy and contraception, medical therapy an d transplantation, death from all causes and sudden death. Results Eighty-four percent of the overall population showed a satisfactory Ability Index (1 and 2) at the first attendance (median age 25 years). Pat ients with complex heart disease and Eisenmenger reaction had earlier clini cal deterioration (18.6 +/- 11.3 vs 26.7 +/- 12.2) and shorter survival (25 .8 +/- 7.9 vs 32.5 +/- 14.6 years). Thirty-eight (20.2%) patients had at le ast one episode of haemoptysis between 20 and 40 years of age but it did no t modify overall survival; 25 (13.2%) had pulmonary thromboembolism at a me an age of 35.2 +/- 13.4 years. Fifteen (7.9%) had a stroke and 7 (3.7%) a c erebral abscess at a mean age of 31.4 +/- 15.7 and 24.1 +/- 4.9 years, resp ectively. Cerebral complications influenced the quality of life but did not modify survival. Patients who had venesection showed a 2.04 times greater hazard ratio for haemoptysis. Venesection did not reduce cerebral complicat ions and in 20% caused anaemia and iron deficiency. Other non-cardiac surge ry with general anaesthesia carried risks (23.5% of deaths). Significant ma ternal mortality (27%) in relation to pregnancy occurred with constant dete rioration in physical status, high incidence of spontaneous abortions (35.8 %) and cardiac abnormalities in offspring (20%). Sixty-one patients died du ring follow-up, mainly by sudden death (29.5%), heart failure (22.9%) and f rom haemoptysis (11.4%). Eight patients had heart and lung transplantation and five died 1 week to 4 years after transplant. Deterioration in Ability Index (worsening symptoms), age, complex defects, blood creatinine level, r ight ventricular dysfunction and noncardiac surgery were variables which af fected the prognosis adversely with uni- and multivariate analysis. Conclusions patients with Eisenmenger syndrome can survive to the seventh d ecade with informed medical care and protection from special risks.