title: B-type natriuretic peptide concentrations in contemporary Eisenmenger syndrome patients: predictive value and response to disease targeting therapy.
abstract: OBJECTIVE
  To assess the relationship between elevated levels of B-type natriuretic peptide (BNP) and outcome in patients with Eisenmenger syndrome.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  Tertiary centre for adult congenital heart disease.
  
  
  METHODS
  All patients with Eisenmenger syndrome (n=181, age 36.9±12.1 years, 31% with Down syndrome) in whom BNP concentrations were measured as part of routine clinical care were included.
  
  
  METHODS
  The study end point was all cause mortality.
  
  
  RESULTS
  During a median follow-up period of 3.3 years, 20 patients (7 with Down syndrome) died. Higher BNP concentrations were predictive of all cause mortality on univariate analysis in patients with or without Down syndrome. On multivariable Cox proportional hazard analysis, BNP predicted survival independently of renal function, Down syndrome, or 6 min walk test distance (p=0.004). Temporal increases in BNP concentration were also found to predict mortality. Treatment with disease targeting therapies was associated with a significant reduction in BNP concentrations.
  
  
  CONCLUSIONS
  BNP concentrations predict outcome in contemporary Eisenmenger patients. Increases in BNP concentrations over time are also of prognostic significance. In addition, disease targeting therapies may help to reduce BNP concentrations in this population, while treatment-naïve patients have static or rising BNP concentrations.
authors.affiliation: Adult Congenital Heart Disease Centre and, National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. g.diller@imperial.ac.uk
authors.forename: Gerhard-Paul
authors.initials: GP
authors.lastname: Diller
authors.forename: Rafael
authors.initials: R
authors.lastname: Alonso-Gonzalez
authors.forename: Aleksander
authors.initials: A
authors.lastname: Kempny
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.forename: Ryo
authors.initials: R
authors.lastname: Inuzuka
authors.forename: Georgios
authors.initials: G
authors.lastname: Giannakoulas
authors.forename: Lianne
authors.initials: L
authors.lastname: Castle
authors.forename: Astrid E
authors.initials: AE
authors.lastname: Lammers
authors.forename: James
authors.initials: J
authors.lastname: Hooper
authors.forename: Anselm
authors.initials: A
authors.lastname: Uebing
authors.forename: Lorna
authors.initials: L
authors.lastname: Swan
authors.forename: Michael
authors.initials: M
authors.lastname: Gatzoulis
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Wort
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D006348:Cardiac Surgical Procedures; D002423:Cause of Death / Q000639:trends; D004541:Eisenmenger Complex / Q000097:blood* / Q000401:mortality / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood*; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D006113:United Kingdom / Q000453:epidemiology
chemical_list: D015415:Biomarkers; D020097:Natriuretic Peptide, Brain