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Acquired Coronary Artery Disease in Patients with Congenital Heart Disease: Issues in Diagnosis and Management

Sotiria C. Apostolopoulou1,*, Stella Brili2, Eftihia Sbarouni3, Dimitris Tousoulis2, Konstantinos Toutouzas2

1 Department of Pediatric Cardiology & Adult Congenital Heart Disease, Onassis Cardiac Surgery Center, Athens, Greece
2 First Department of Cardiology, University of Athens, Athens Medical School, Athens, Greece
3 Department of Cardiology, Onassis Cardiac Surgery Center, Athens, Greece

* Corresponding Author: Sotiria C. Apostolopoulou. Email:

Congenital Heart Disease 2020, 15(5), 369-375.


Objective: Acquired coronary artery disease, initially thought to rarely affect survivors of congenital heart disease, is increasingly recognized in this population, as these patients grow in age and numbers in the recent era. This study reports our experience with coronary artery disease in adults with congenital heart disease and discusses treatment issues and the existing literature. Methods: Retrospective review of all charts of adults with congenital heart disease and acquired coronary artery disease was performed. Patients’ clinical characteristics, diagnosis, risk factors, noninvasive and invasive imaging and management data were recorded. Results: Coronary artery disease was diagnosed at 35–70 of age in 17 patients out of a total of 1345 adult congenital heart disease patients followed. Congenital heart disease was moderate or complex in 5 patients (4 repaired Tetralogy of Fallot, 1 repaired ventricular septal defect) and simple unrepaired atrial septal defect diagnosed later in life before or after coronary artery disease identi- fication in 12 patients. Coronary artery disease symptoms were present in 12 patients (8 myocardial infarctions, 4 exercise intolerance), while all patients had 2–3 risk factors for coronary artery disease. Coronary angiography showed 1–3 vessel disease treated with percutaneous coronary intervention in 7 patients, coronary artery bypass graft in 2 patients and both in 2 patients. Patients received appropriate medical therapy and remained stable for 1–17 years, while 2 patients are awaiting surgical pulmonary valve replacement in the near future. Conclusion: Coronary artery disease can develop in adults with congenital heart disease later in life in the presence of traditional risk factors, and prevention, screening and treatment strategies should be applied in this population. Further studies are needed to fully elucidate the extent of this issue in this increasing and ageing population and to determine its optimal medical, interventional and surgical management.


Cite This Article

Apostolopoulou, S. C., Brili, S., Sbarouni, E., Tousoulis, D., Toutouzas, K. (2020). Acquired Coronary Artery Disease in Patients with Congenital Heart Disease: Issues in Diagnosis and Management. Congenital Heart Disease, 15(5), 369–375.

This work is licensed under a Creative Commons Attribution 4.0 International License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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