Coronary artery disease in adults with congenital heart disease

Abstract

The increasing population of adult patients with congenital heart disease (ACHD) is at risk of developing coronary artery disease (CAD) and other atherosclerotic cardiovascular diseases due to exposure to cardiovascular risk factors. The impact of this exposure is growing larger as life expectancy of these subjects increases with the progressive improvement in management of congenital heart disease. Studies have shown that ACHD patients have a higher risk for CAD than their non-ACHD matches, highlighting the need for awareness and prevention efforts among congenital heart disease specialists and non-ACHD cardiologists. At the same time, ACHD patients with CAD often present specific characteristics all practicing cardiologists should be aware of. While further research is needed to fully understand the mechanisms underlying the higher CAD risk in this population, this article summarizes key evidence on CAD in ACHD and emphasizes on one hand the importance of early screening and management of known cardiovascular risk factors in ACHD patients, particularly those who are younger, female, or have more complex/severe CHD. On the other hand, it calls for a broader knowledge of ACHD risk for CAD and its peculiarities among all cardiologists.

Keywords: Congenital heart disease; Coronary artery disease; Grown-up congenital heart disease.

Fig. 1

The figure depicts the main factors affecting the development of coronary artery disease (CAD) in adults with congenital heart disease (ACHD), including (clockwise starting on the upper left corner): surgical manipulation; altered hemodynamics; coronary anatomy anomalies; other anatomy-related factors (e.g. ab estrinseco compression of the LMCA); endothelial dysfunction; anatomic and clinical complexity of the congenital defect.

Fig. 2

The figure depicts the Leiden Convention for coronary coding, in the standard surgical view (left panel) and the universal/imaging view (right panel). Reprinted from The Journal of Thoracic and Cardiovascular Surgery, 156/6:6–10, Adriana C. Gittenberger-de Groot, Wilke M.C.Koenraadt, Margot M. Bartelings, Regina Bökenkamp,MarcoC. DeRuiter, Mark G. Hazekamp,Ad J.J. C. Bogers,Jan M.Quaegebeur, Martin J. Schalij, Hubert W. Vliegen, Robert E.Poelmann, Monique R.M. Jongbloed. Coding of coronary arterial origin and branching incongenital heart disease: The modified Leiden Convention, Copyright (2018), with permission from Elsevier (License Number: 5490310144912).