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Original research article
Factors associated with coronary artery disease and stroke in adults with congenital heart disease
  1. Jouke P Bokma1,2,
  2. Ineke Zegstroo1,3,
  3. Joey M Kuijpers1,2,
  4. Thelma C Konings3,
  5. Roland R J van Kimmenade4,
  6. Joost P van Melle5,
  7. Philippine Kiès6,
  8. Barbara J M Mulder1,2,
  9. Berto J Bouma1,2
  1. 1 Department of Cardiology, Academic Medical Center Amsterdam, Amsterdam, The Netherlands
  2. 2 Netherlands Heart Institute, Utrecht, The Netherlands
  3. 3 Department of Cardiology, VU University Medical Center Amsterdam, Amsterdam, The Netherlands
  4. 4 Department of Cardiology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
  5. 5 Department of Cardiology, University Medical Center Groningen, Groningen, The Netherlands
  6. 6 Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands
  1. Correspondence to Dr Berto J Bouma, Department of Cardiology, Academic Medical Center Amsterdam, Meibergdreef 9, 1105AZ Amsterdam, The Netherlands; b.j.bouma{at}


Objective To determine factors associated with coronary artery disease (CAD) and ischaemic stroke in ageing adult congenital heart disease (ACHD) patients.

Methods We performed a multicentre case–control study, using data from the national CONgenital CORvitia (CONCOR) registry to identify ACHD patients within five participating centres. Patients with CAD were matched (1:2 ratio) with ACHD patients without CAD on age, CHD defect group and gender. Patients with ischaemic stroke (or transient ischaemic attack) were matched similarly. Medical charts were reviewed and a standardised questionnaire was used to determine presence of risk factors.

Results Of 6904 ACHD patients, a total of 55 cases with CAD (80% male, mean age 55.1±12.4 years) and 56 cases with stroke (46% male, mean age 46.9±15.2) were included and matched with control patients. In multivariable logistic regression analysis, traditional atherosclerotic risk factors (hypertension (OR 2.45; 95% CI 1.15 to 5.23), hypercholesterolaemia (OR 3.99; 95% CI 1.62 to 9.83) and smoking (OR 2.25; 95% CI 1.09 to 4.66)) were associated with CAD. In contrast, these risk factors were not associated with ischaemic stroke. In multivariable analysis, stroke was associated with previous shunt operations (OR 4.20; 95% CI 1.36 to 12.9), residual/unclosed septal defects (OR 2.38; 95% CI 1.03 to 5.51) and left-sided mechanical valves (OR 2.67; 95% CI 1.09 to 6.50).

Conclusions Traditional atherosclerotic risk factors were associated with CAD in ACHD patients. In contrast, ischaemic stroke was related to factors (previous shunts, septal defects, mechanical valves) suggesting a cardioembolic aetiology. These findings may inform surveillance and prevention strategies.

  • Congenital Heart Disease
  • Coronary Artery Disease
  • Stroke

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  • Contributors All authors attributed in both the conception, design, critical revision and final approval of this manuscript. JPB and IZ analysed and interpreted the data and drafted the manuscript under supervision of senior authors BJMM and BJB.

  • Funding This work was supported by the Netherlands Heart Institute (NL-HI) and Nuts Ohra foundation. The work described in this study was carried out in the context of the Parelsnoer Institute. Parelsnoer Institute is part of and funded by the Dutch Federation of University Medical Centers.

  • Competing interests None declared.

  • Ethics approval AMC Medical Ethics Committee.

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

  • Data sharing statement Unpublished data from the study are available on request.

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