Article Text

Download PDFPDF

Adults with repaired tetralogy: low mortality but high morbidity up to middle age
  1. Mark Dennis1,2,
  2. Ben Moore2,
  3. Irina Kotchetkova2,
  4. Lynne Pressley2,
  5. Rachael Cordina1,2 and
  6. David S Celermajer1,2
  1. 1 Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia
  2. 2 Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
  1. Correspondence to Professor David S Celermajer; david.celermajer{at}


Objective Survival of patients with repaired tetralogy of Fallot (rToF) into young adulthood is very good. Concerns exist, however, over long-term morbidity and mortality as these subjects reach middle age. We aimed to assess survival and the prevalence of complications in patients with rToF seen in our Adult Congenital Heart Disease (ACHD) service.

Methods One hundred and sixty-eight consecutive patients with ‘simple rToF’, aged over 16 years, followed up at our tertiary-level ACHD service in Sydney, Australia since 2000, were included. We documented mortality and analysed the prospectively defined composite end points of (a) ‘Serious adverse events’, including death, heart failure hospitalisation and/or documented ventricular arrhythmia and (b) ‘Adverse events’ inclusive of the above and endocarditis, atrial arrhythmia, defibrillator and/or pacemaker implantation.

Results Mean age at the last follow-up was 34±12 years, and 55% were men. There were 10 (6%) deaths, and 26 patients (16%) experienced a ‘serious adverse event’. Fifty-one patients (30%) experienced an ‘adverse event’ and 29 patients had atrial arrhythmias. One hundred and one (61%) patients had at least one pulmonary valve replacement. By age 40 years, 93% were free of serious adverse events, and 83% were free of any adverse event. By age 50 years, only 56% had not had an adverse event. Older age and history of atrial arrhythmia were predictive of serious adverse events.

Conclusion Survival into mid-adulthood in patients with rToF is very good; however, a substantial number of survivors have adverse events by the age of 50 years.


This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:

View Full Text

Statistics from


  • Competing interests None declared.

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

  • Data sharing statement No additional unpublished data from this study is currently available. Any requests for data sharing should be made to the corresponding author.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.