title: Eisenmenger syndrome. Factors relating to deterioration and death.
abstract: OBJECTIVE
  To assess the natural history, risk factors for death and deterioration of patients with Eisenmenger Syndrome.
  
  
  METHODS
  The clinical course of 188 patients from three different cardiac centres specialized in adult congenital heart defects, followed for a median period of 31 years, was retrospectively analysed. According to the diagnosis, 64 males and 124 females, mean age at last follow-up 33.02+/-12.8 years, were divided into two groups: simple (128) and complex (60) congenital heart disease. Events analysed were: time variation of Ability Index, pulmonary and cerebral complications, non-cardiac surgery, urate metabolism and renal function, arrhythmias, pregnancy and contraception, medical therapy and 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). Patients with complex heart disease and Eisenmenger reaction had earlier clinical 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 least one episode of haemoptysis between 20 and 40 years of age but it did not modify overall survival; 25 (13.2%) had pulmonary thromboembolism at a mean age of 35.2+/-13.4 years. Fifteen (7.9%) had a stroke and 7 (3.7%) a cerebral abscess at a mean age of 31.4+/-15.7 and 24.1+/-4.9 years, respectively. 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 complications and in 20% caused anaemia and iron deficiency. Other non-cardiac surgery with general anaesthesia carried risks (23.5% of deaths). Significant maternal mortality (27%) in relation to pregnancy occurred with constant deterioration in physical status, high incidence of spontaneous abortions (35.8%) and cardiac abnormalities in offspring (20%). Sixty-one patients died during follow-up, mainly by sudden death (29.5%), heart failure (22.9%) and from 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, right ventricular dysfunction and non-cardiac surgery were variables which affected the prognosis adversely with uni- and multivariate analysis.
  
  
  CONCLUSIONS
  patients with Eisenmenger syndrome can survive to the seventh decade with informed medical care and protection from special risks
authors.affiliation: Division of Cardiology, University of Padua, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Daliento
authors.forename: J
authors.initials: J
authors.lastname: Somerville
authors.forename: P
authors.initials: P
authors.lastname: Presbitero
authors.forename: L
authors.initials: L
authors.lastname: Menti
authors.forename: S
authors.initials: S
authors.lastname: Brach-Prever
authors.forename: G
authors.initials: G
authors.lastname: Rizzoli
authors.forename: S
authors.initials: S
authors.lastname: Stone
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D002423:Cause of Death; D003267:Contraception; D004541:Eisenmenger Complex / Q000150:complications / Q000401:mortality* / Q000628:therapy; D004739:England / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D011247:Pregnancy; D018566:Pregnancy, High-Risk; D011379:Prognosis; D011788:Quality of Life; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D013997:Time Factors