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Is vWF really a very wonderful factor for risk stratification in adults with congenital heart disease?
  1. David S Celermajer1,2,
  2. Sophie Offen1,2
  1. 1 Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
  2. 2 Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia
  1. Correspondence to Dr David S Celermajer, Cardiology, Royal Prince Alfred Hospital, Camperdown, NSW 2050, Australia; david.celermajer{at}

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Adults with congenital heart disease (CHD) are indeed a 20th-century marvel. There were not too many adults with single ventricle hearts around, for example, before the 1980s. Extraordinary advances in paediatric cardiac care over the past decades have meant over 90% of babies born with cardiovascular anomalies are now expected to reach adulthood, a statistic that is likely to continue to improve.

Despite this great success, many of the adults now living with CHD will experience significant complications. Those with complex pathology are unlikely to reach their 40s without one or more adverse events, even after ‘successful’ repair in childhood (the most common complications including need for further surgeries, heart failure, arrhythmias, endocarditis and thromboembolism). The risk of premature mortality remains substantial, across the spectrum of CHDs.1

One pivotal question that remains to be answered is how we can best predict such adverse outcomes, in this heterogenous group of young and often complex patients. Following early initial care at an experienced paediatric heart centre, it has been shown that failure of transition into adult CHD care is associated with significantly poorer outcomes.2 Even in high volume paediatric centres, up to 60% of …

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  • Contributors DSC and SO contributed equally.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Patient consent for publication Not required.

  • Provenance and peer review Commissioned; internally peer reviewed.

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