Conference Coverage

Primary arrhythmia syndromes: Common cause of pediatric sudden cardiac death




CHICAGO – Just over one-half of all sudden deaths in a large pediatric case series were due to a primary arrhythmia syndrome, Dr. Grazia Delle Donne reported at the annual meeting of the American College of Cardiology.

She presented an analysis of all patients under the age of 18 years who were referred to London’s Royal Brompton Hospital for post mortem examination following presumed sudden cardiac death during 1991-2013. Royal Brompton is a national referral center for sudden cardiac death.

The review was undertaken because sudden cardiac death in the pediatric population occurs infrequently. Little is known about the prevalence of the various causes, noted Dr. Delle Donne of Royal Brompton.

Bruce Jancin/Frontline Medical News

Of the 398 subjects, 266 (67%) were female. The median age at death was 14 years. Twenty-two percent of the fatalities occurred during or immediately after exercise. Thirty-nine percent occurred while at rest.

Thirty-one percent of subjects had a family history of sudden cardiac death, another 14% had a family history of cardiomyopathy, and in 5% of cases there was a significant family history of arrhythmia.

Five percent of the children were known to have congenital heart disease. Eighteen percent of the children had a history of syncope.

Investigators determined that a primary arrhythmia syndrome such as long QT or Brugada syndrome was the cause of sudden death in 54% of cases. Death was attributed to cardiomyopathy in 15% cases, congenital heart disease in 8%, myocarditis in 6%, and coronary anomalies in 5%, with miscellaneous causes accounting for the remainder.

Dr. Delle Donne reported having no financial conflicts of interest regarding her presentation.

Recommended Reading

VIDEO: Fire and Ice - Which catheter ablation approach is best in AF?
MDedge Cardiology
Similarities seen in rate and rhythm control for postsurgical AF
MDedge Cardiology
FIRE AND ICE trial called a win for cryoablation of AF
MDedge Cardiology
Early antiarrhythmic drugs boost survival in shock-refractory cardiac arrest
MDedge Cardiology
FDA approves first leadless pacemaker
MDedge Cardiology