Sudden Death in Congenital Heart Disease

Study Questions:

What is the rate of sudden unexpected death (SUD) in children with congenital heart disease (CHD) with particular attention to the risk of SUD during physical activity?


Data were retrieved from four Norwegian national or university databases to identify births from 1994-2009, incidence of CHD, CHD-specific data, and occurrence and cause of death. Individual data on children with CHD who died at age 2-18 years were collected from a university database, which included data on >80% of all CHD patients in Norway. Perioperative mortality was excluded from the definition of SUD.


There were 11,272 cases of CHD, accounting for 1.2% of live births; 23.7% had severe CHD. Follow-up through 2012 revealed 842 (7.5%) deaths (mean age 0.9 years). Risk of death was increased in children <2 years and those with severe CHD. Of the 10,459 children surviving past 2 years of age, 38 (1.4%) died with severe CHD and 40 (0.5%) died with nonsevere CHD. Overall rate of death for age 2-18 was 70 per 100,000 person-years of follow-up. Individual records were reviewable in 71 of 78 deaths after age 2. SUD occurred in 19 children, of which 7 were attributed to sudden cardiac death (SCD); none occurred during physical activity. Including two identified cases of aborted SCD during physical activity, the estimated rate of SCD related to physical activity in children >2 years of age with CHD was 2 per 100,000 person-years.


SCD in children with CHD is infrequent after age 2 and rarely occurs during physical activity. The authors opine that physical activity in children with CHD outweighs the low risk of SUD.


This is a thorough country-wide analysis of SUD in pediatric CHD, demonstrating the benefit of rigorous national databases to determine the rate of such events. While death during physical activity was found to be a rare occurrence, there is one important limitation identified by the authors—that the extent to which these children participated in athletics is not known and that personal-, family-, or physician-induced restrictions may bias these results.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Prevention, Sports and Exercise Cardiology, SCD/Ventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Prevention, Exercise, Sports and Exercise and Congenital Heart Disease and Pediatric Cardiology

Keywords: Cause of Death, Child, Death, Sudden, Cardiac, Death, Sudden, Exercise, Heart Defects, Congenital, Motor Activity, Sports

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