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Original research article
Causes of death in a contemporary adult congenital heart disease cohort
  1. Christopher Yu1,
  2. Benjamin M Moore2,3,
  3. Irina Kotchetkova2,
  4. Rachael L Cordina2,3,
  5. David S Celermajer2,3
  1. 1 Concord Repatriation General Hospital, Concord, New South Wales, Australia
  2. 2 Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
  3. 3 Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia
  1. Correspondence to Professor David S Celermajer, Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, NSW 2050, Australia; David.Celermajer{at}


Objective The life expectancy of patients with congenital heart disease (CHD) has significantly improved with advances in their paediatric medical care. Mortality patterns are changing as a result. Our study aims to describe survival and causes of death in a contemporary cohort of adult patients with CHD.

Methods We reviewed 3068 patients in our adult CHD database (age ≥16 years, seen at least once in our centre between 2000 and 2015), and documented the number and causes of death, via Australia’s National Death Index. Survival and mortality patterns were analysed by complexity of CHD and by underlying congenital diagnosis.

Results Our cohort comprised 3068 adult patients (53% male). The distribution of patients (per the Bethesda classification) was 47% simple, 34% moderate and 18% complex (1% not classifiable). Over a median follow-up of 6.2 years (IQR 3.5–10.4), 341 patients (11%) died with an incidence of 0.4 deaths/100 patient years (py). Survival was significantly worse with increasing complexity of CHD (p<0.001); mortality rate in the simple group was 0.3 deaths/100 py with a median age of death 70 years, and in the complex group was 1.0 death/100 py with a median age of death 34 years. Overall, non-cardiac causes of death outnumbered cardiac causes, at 54% and 46%, respectively. The leading single cause of death was heart failure (17%), followed by malignancy (13%). Simple adult CHD patients mostly died due to non-cardiac causes such as malignancy. Perioperative mortality only accounted for 5% of deaths.

Conclusions Premature death is common in adults with CHD. Although heart failure remains the most common cause of death, in the contemporary era in a specialist CHD centre, non-cardiac related deaths outnumber cardiac deaths, particularly in those with simple CHD lesions.

  • heart failure
  • cardiac arrest

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  • Contributors CY was responsible for study design, data collection, statistical analysis and drafting of the manuscript. BM was responsible for drafting and editing of the manuscript, and assisted with analysis. IK contributed to statistical analysis and generation of figures. RLC contributed to study design, data collection, supervision and editing of the manuscript. DSC was responsible for study design, implementation and editing of the manuscript. All authors gave final approval for the manuscript and accept accountability for its contents.

  • Funding This research did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Not required.

  • Ethics approval The study protocol was approved by the Institutional Review Board (reference: HREC/11/RPAH/626) of Royal Prince Alfred Hospital, Sydney Local Health District.

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

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