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

Objective: Acquired coronary artery disease, initially thought to rarelyaffect survivors of congenital heart disease, is increasingly recognized in thispopulation, as these patients grow in age and numbers in the recent era. This studyreports our experience with coronary artery disease in adults with congenital heartdisease and discusses treatment issues and the existing literature. Methods: Retrospective review of all charts of adults with congenital heart disease and acquiredcoronary artery disease was performed. Patients’ clinical characteristics, diagnosis, risk factors, noninvasive and invasive imaging and management data wererecorded. Results: Coronary artery disease was diagnosed at 35–70 of age in17 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 atrialseptal defect diagnosed later in life before or after coronary artery disease identi-fication in 12 patients. Coronary artery disease symptoms were present in12 patients (8 myocardial infarctions, 4 exercise intolerance), while all patientshad 2–3 risk factors for coronary artery disease. Coronary angiography showed1–3 vessel disease treated with percutaneous coronary intervention in 7 patients,coronary artery bypass graft in 2 patients and both in 2 patients. Patients receivedappropriate medical therapy and remained stable for 1–17 years, while 2 patientsare awaiting surgical pulmonary valve replacement in the near future. Conclusion:Coronary artery disease can develop in adults with congenital heart disease later inlife in the presence of traditional risk factors, and prevention, screening and treatment strategies should be applied in this population. Further studies are needed tofully elucidate the extent of this issue in this increasing and ageing populationand to determine its optimal medical, interventional and surgical management.