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Original research article
Contemporary cardiac surgery for adults with congenital heart disease
  1. Sylvain Beurtheret1,2,
  2. Oktay Tutarel3,
  3. Gerhard Paul Diller3,4,
  4. Cathy West3,
  5. Evangelia Ntalarizou1,
  6. Noémie Resseguier5,
  7. Vasileios Papaioannou3,
  8. Richard Jabbour3,
  9. Victoria Simpkin3,
  10. Anthony J Bastin3,
  11. Sonya V Babu-Narayan3,4,
  12. Beatrice Bonello3,
  13. Wei Li3,
  14. ​Babulal Sethia1,
  15. Hideki Uemura1,
  16. Michael A Gatzoulis3,4,
  17. Darryl Shore1
  1. 1 Department of Congenital Cardiac Surgery, Royal Brompton Hospital, London, UK
  2. 2 Department of Adult Cardiac Surgery, Saint Joseph Hospital, Marseille, France
  3. 3 Adult Congenital Heart Centre & National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK
  4. 4 NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, London, UK
  5. 5 Department of Biostatistic and Public Health, La Timone Hospital, Marseille, France
  1. Correspondence to Dr Sylvain Beurtheret, Department of Congenital Cardiac Surgery, Royal Brompton Hospital, London SW3 6NP, UK; sylvainbeurtheret{at}


Objective Advances in early management of congenital heart disease (CHD) have led to an exponential growth in adults with CHD (ACHD). Many of these patients require cardiac surgery. This study sought to examine outcome and its predictors for ACHD cardiac surgery.

Methods This is an observational cohort study of prospectively collected data on 1090 consecutive adult patients with CHD, undergoing 1130 cardiac operations for CHD at the Royal Brompton Hospital between 2002 and 2011. Early mortality was the primary outcome measure. Midterm to longer-term survival, cumulative incidence of reoperation, other interventions and/or new-onset arrhythmia were secondary outcome measures. Predictors of early/total mortality were identified.

Results Age at surgery was 35±15 years, 53% male, 52.3% were in New York Heart Association (NYHA) class I, 37.2% in class II and 10.4% in class III/IV. Early mortality was 1.77% with independent predictors NYHA class ≥ III, tricuspid annular plane systolic excursion (TAPSE) <15 mm and female gender. Over a mean follow-up of 2.8±2.6 years, 46 patients died. Baseline predictors of total mortality were NYHA class ≥ III, TAPSE <15 mm and non-elective surgery. The number of sternotomies was not independently associated with neither early nor total mortality. At 10 years, probability of survival was 94%. NYHA class among survivors was significantly improved, compared with baseline.

Conclusions Contemporary cardiac surgery for ACHD performed at a single, tertiary reference centre with a multidisciplinary approach is associated with low mortality and improved functional status. Also, our findings emphasise the point that surgery should not be delayed because of reluctance to reoperate only.

  • adults – congenital heart disease – cardiac surgery

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  • Contributors SB, MAG, and DFS designed the study. SB, OT, CW, EN, VP, RJ, VS, AJB, SVB-N, and BB collected data. GPD and NR performed the statistical analysis. SB and OT wrote the manuscript. MAG, DFS, BS, WL and HU revised the manuscript. All authors contributed to analyses and interpretations of results and approved the final version for submission.

  • Funding MAG, SBN and the Adult Congenital Heart Centre & National Centre for Pulmonary Hypertension Royal Brompton Hospital Group have received support from the British Heart Foundation, London, UK. This work was supported by a training grant from the European Society of Cardiology to OT.

  • Competing interests None declared.

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