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B-type natriuretic peptide concentrations in contemporary Eisenmenger syndrome patients: predictive value and response to disease targeting therapy
  1. Gerhard-Paul Diller1,2,
  2. Rafael Alonso-Gonzalez1,
  3. Aleksander Kempny1,
  4. Konstantinos Dimopoulos1,2,
  5. Ryo Inuzuka1,
  6. Georgios Giannakoulas1,
  7. Lianne Castle1,
  8. Astrid E Lammers1,
  9. James Hooper3,
  10. Anselm Uebing1,
  11. Lorna Swan1,
  12. Michael Gatzoulis1,2,
  13. Stephen J Wort1,2
  1. 1Adult Congenital Heart Disease Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK
  2. 2National Heart and Lung Institute, Imperial College School of Medicine, London, UK
  3. 3Department of Clinical Biochemistry, Royal Brompton Hospital, London, UK
  1. Correspondence to Dr Gerhard-Paul Diller, Adult Congenital Heart Disease Centre and, National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; g.diller{at}


Objective To assess the relationship between elevated levels of B-type natriuretic peptide (BNP) and outcome in patients with Eisenmenger syndrome.

Design Retrospective study.

Setting Tertiary centre for adult congenital heart disease.

Patients 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.

Main outcome measures 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.

  • Brain natriuretic peptide
  • pulmonary arterial hypertension
  • Eisenmenger syndrome
  • prognosis
  • mortality
  • congenital heart disease
  • exercise tolerance
  • adult congenital heart disease
  • exercise physiology
  • exercise testing
  • heart failure
  • echocardiography
  • heart rate variability
  • pulmonary vascular disease
  • pulmonary hypertension
  • paediatric cardiology
  • endocrinology
  • metabolic medicine
  • cardiac function
  • paediatric echocardiology
  • interventional catheteris
  • paediatric cardiology
  • paediatric interventional cardiology
  • congenital - adult
  • congenital - paediatric

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  • See Editorial, p 681

  • Competing interests Dr Diller has received unrestricted educational grants from Actelion, UK and has served on the advisory board of Actelion, Germany. Professor Gatzoulis has served on the advisory board of Actelion, Pfizer, and GlaxoSmithKline and has received unrestricted educational grants from Actelion and Pfizer, UK. Dr SJ Wort has received educational grants from Actelion and has served on the advisory boards of Pfizer, Bayer and Eli-Lilly.

  • Ethics approval Ethics approval was provided by Royal Brompton Ethics Commitee.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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