title: Poor prognosis and related factors in adults with Eisenmenger syndrome.
abstract: BACKGROUND
  Little information is available regarding the clinical history and long-term prognosis of patients with Eisenmenger syndrome without diagnosis until adulthood. The purpose of this study was to investigate the long-term prognosis and factors predictive of the prognosis in patients with Eisenmenger syndrome diagnosis in adulthood.
  
  
  RESULTS
  We retrospectively reviewed the clinical data of 106 patients with Eisenmenger syndrome diagnosis in adulthood. Presence of congenital heart defects had neither been noticed nor confirmed with cardiac catheterization until adulthood in most of the patients in our study. After the diagnosis was confirmed, 42 patients died during a mean follow-up period of 7.6 years. The mean survival time from diagnostic catheterization to death was 5.4 years in nonsurvivors. Survival rate was 98% at 1 year, 77% at 5 years, and 58% at 10 years, with the Kaplan-Meier method. Elevated right atrial pressure (>7 mm Hg) and reduced systemic blood flow (<2.9 L/min) were independently associated with increased mortality rate of adults with Eisenmenger syndrome.
  
  
  CONCLUSIONS
  Patients with Eisenmenger syndrome without diagnosis until adulthood had a poor prognosis. Reduced systemic blood flow and elevated right atrial pressure were associated with high mortality rates in adults with Eisenmenger syndrome.
authors.affiliation: Cardiology Division of Medicine, National Cardiovascular Center, Suita, Osaka, Japan.
authors.forename: Hideo
authors.initials: H
authors.lastname: Oya
authors.forename: Noritoshi
authors.initials: N
authors.lastname: Nagaya
authors.forename: Masaaki
authors.initials: M
authors.lastname: Uematsu
authors.forename: Toru
authors.initials: T
authors.lastname: Satoh
authors.forename: Fumio
authors.initials: F
authors.lastname: Sakamaki
authors.forename: Shingo
authors.initials: S
authors.lastname: Kyotani
authors.forename: Nagato
authors.initials: N
authors.lastname: Sato
authors.forename: Norifumi
authors.initials: N
authors.lastname: Nakanishi
authors.forename: Kunio
authors.initials: K
authors.lastname: Miyatake
mesh_terms: D000328:Adult; D000367:Age Factors; D000704:Analysis of Variance; D006328:Cardiac Catheterization; D004541:Eisenmenger Complex / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate