Sudden cardiac death during exercise in patients with congenital heart disease: the exercise paradox and the challenge of appropriate counselling.
This editorial refers to 'Sudden unexpected death in children with congenital heart defects'[1] by J. Jortveit et al on page 621. The population of patients with congenital heart disease (CHD) is growing rapidly. It is well appreciated by congenital cardiologists that the majority of CHD patients are not cured, and sequelae as well as late complications are prevalent.1 One of the major causes of mortality in this population is sudden, mostly arrhythmic, death.2 While not being the leading cause of mortality in the majority of CHD subgroups, sudden cardiac death (SCD) remains a particularly emotive subject inducing profound reactions from medical professionals, families, and society. This is due the fact that death occurs unexpectedly, often in patients who are otherwise well and lead a relatively normal life. Risk stratification has, therefore, been a major focus of CHD research at many institutions. Despite decades of effort, identifying CHD patients at particularly high risk of SCD and those who would most likely benefit from the implantation of defibrillator cardioverters (ICDs) remains challenging. While different risk factors and risk scores have been developed, their value for identifying patients at risk is still limited.3 5 On the other hand, implantation of ICDs in young CHD patients is associated with high morbidity and risk of inadequate shocks, ultimately also impacting on the quality of life in this population.6 Sudden death can occur both at rest and during exercise. However, it has been well appreciated that exercise is associated with an increased risk [1] #fn 2
European heart journal