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Original research article
Infective endocarditis in adults with congenital heart disease remains a lethal disease
  1. Oktay Tutarel1,2,
  2. Rafael Alonso-Gonzalez1,
  3. Claudia Montanaro1,
  4. Renee Schiff1,
  5. Aitor Uribarri1,
  6. Aleksander Kempny1,
  7. Martin R Grübler1,3,
  8. Anselm Uebing1,
  9. Lorna Swan1,
  10. Gerhard-Paul Diller1,4,5,
  11. Konstantinos Dimopoulos1,4,
  12. Michael A Gatzoulis1,4
  1. 1 Adult Congenital Heart Centre & National Centre for Pulmonary Hypertension, Royal Brompton Hospital and Harefield NHS Foundation Trust, London, UK
  2. 2 Department of Paediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich, Munich, Germany
  3. 3 Department of Cardiology, Swiss Cardiovascular Centre Bern, Bern University Hospital, Bern, Switzerland
  4. 4 National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, and National Heart and Lung Institute, Imperial College School of Medicine, London, UK
  5. 5 Division of Adult Congenital and Valvular Heart Disease Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany
  1. Correspondence to Dr Oktay Tutarel, Adult Congenital Heart Centre & National Centre for Pulmonary Hypertension, Royal Brompton Hospital, NIHR Cardiovascular Biomedical Research Unit, and National Heart and Lung Institute, Imperial College Sydney, London SW3 6NP, UK; otutarel{at}


Objective Infective endocarditis (IE) is associated with significant morbidity and mortality. Patients with adult congenital heart disease (ACHD) have an increased risk of developing IE. The aim of this study is to describe the incidence, predictors of outcome and mortality associated with IE in ACHD in a contemporary cohort.

Methods All episodes of IE in adults with congenital heart disease referred to our tertiary centre between 1999 and 2013 were included in the study. Patients were identified from the hospital database. The diagnosis of endocarditis was established according to the modified Duke criteria. The primary endpoint of the study was endocarditis-associated mortality.

Results There were 164 episodes of IE in 144 patients (male 102, 70.8%). Mean age at presentation was 32.3±22.7 years. Out of these, 43% had a simple, 23% a moderate and 32% a complex lesion. It was at least the second bout of IE in 37 episodes (23%). A predisposing event could be identified in only 26.2% of episodes. Surgical intervention during the same admission was performed in 61 episodes (37.2%). During a median follow-up of 6.7 years (IQR 2.9–11.4), 28 (19.4%) patients died. Out of these, 10 deaths were related to IE (IE mortality 6.9%). On unvariate regression analysis, the development of an abscess (OR: 7.23; 95% CI 1.81 to 28.94, p<0.01) and age (OR: 1.05; 95% CI 1.01 to 1.10, p=0.03) were the only predictors of IE-associated mortality. There was no increase in IE cases at our centre during the period of the study.

Conclusions IE-associated morbidity and mortality in a contemporary cohort of ACHD patients is still high in the current era.

  • infective endocarditis
  • adult congenital heart disease

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  • OT and RA-G contributed equally.

  • Contributors OT, RA-G, KD and MAG designed the study. OT, RA-G, CM, RS, AiU, AK, MRG, AnU, LS and G-PD made substantial contributions to conception and design as well as acquisition and interpretation of data. OT, CM and G-PD performed the data analysis. OT, RA-G, KD and MAG drafted the paper. All authors provided approval of the final version. OT and RA-G are responsible for the overall content as guarantors.

  • Funding This work was supported by a training grant from the European Society of Cardiology to Dr OT. Professor MAG and the Adult Congenital Heart Centre and the National Centre for Pulmonary Hypertension have received support from the Clinical Research Committee and the British Heart Foundation.

  • Competing interests None declared.

  • Patient consent As this was a retrospective analysis of data collected for routine clinical care and administrative purposes, individual informed consent was not required (UK National Research Ethics Service guidance).

  • Ethics approval The study was locally registered and approved.

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