Coronary artery disease in patients with congenital heart defects

Jokinen E.J Intern Med. 2020 May 11. doi: 10.1111/joim.13080. Online ahead of print.PMID: 32391638



The prognosis of patients with congenital heart defects has improved significantly: more and more patients reach adulthood and old age. At the same time, the possibility of cardiovascular morbidity increases. The conventional risk factors for coronary artery disease are at least as high or even higher in patients than in the general population. Obesity and sedentary life style are more common in adults with congenital heart defect (ACHD) than in general population. In some patients, for example those with coarctation of the aorta or patients with operated coronary arteries in the infancy, the incidence of coronary artery disease (CAD) is clearly increased. In some patients with cyanotic heart defects (e.g. Fontan), the incidence of CAD might be lower, but it usually returns to the average level or higher after correction of the defect. Coronary artery disease is one of the most important reasons for mortality also in ACHD patients, and the consequences of a coronary event might be more fateful in a patient with a corrected congenital heart defect than in her/his peer. There should be a paradigm shift from operative mortality and short-term outcome to long-term morbidity and prevention of cardiovascular disease – a task that often has been forgotten during follow-up visits.