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?

Methods:

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.

Results:

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.

Conclusions:

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.

Perspective:

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 & Pediatrics and Arrhythmias, CHD & Pediatrics and Prevention, Exercise, Sports & Exercise and Congenital Heart Disease & Pediatric Cardiology

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


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