Sudden cardiac death: clinical evaluation of paediatric family members.
OBJECTIVE
To evaluate paediatric relatives of first- and non-first-degree family victims with a history of premature sudden cardiac death (SCD) or aborted cardiac arrest (ACA).


RESULTS
Thirty-six consecutive referred families after SCD (n = 29) or ACA (n = 7) of a family member were analysed. Referral was either due to an inherited heart disease identified after autopsy, post-event, or family evaluation (n = 20 families) or due to sudden unexplained death (SUD, n = 16 families). In 3 of 16 (19%) SUD families, an inherited heart disease was diagnosed by evaluation of the paediatric relatives. In 5 of 25 (20%) referred paediatric relatives of SUD families, an inherited heart disease was identified, mainly sinus node dysfunction (n = 3). A total of 13 of 33 (39%) referred paediatric relatives of families with known inherited heart disease were affected, mainly with cardiomyopathy (n = 5) and primary electrical disease (n = 7). Prevention of SCD was initiated in 16 of the affected children by implantation of an antibradycardia device (n = 3), an implantable cardioverter defibrillator (ICD, n = 6), and/or antiarrhythmic medication (n = 8). Appropriate and successful ICD discharges occurred in four.


CONCLUSIONS
A stepwise, comprehensive clinical investigation of SCD or ACA families identifies a substantial number of paediatric relatives at risk of SCD. This allows for targeted prevention by effective treatments and evaluation of further relatives.
Division of Paediatric Cardiology, University Children's Hospital, Steinwiesstrasse 75, 8032, Zurich, Switzerland. maren.tomaske@kispi.uzh.ch
Maren
M
Tomaske
Dagmar I
DI
Keller
Urs
U
Bauersfeld
D000293:Adolescent; D000328:Adult; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D002304:Cardiac Pacing, Artificial / Q000379:methods; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000517:prevention & control; D017147:Defibrillators, Implantable; D004562:Electrocardiography; D005190:Family; D005260:Female; D006323:Heart Arrest / Q000453:epidemiology* / Q000517:prevention & control; D006331:Heart Diseases / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors; D012849:Sinoatrial Node / Q000503:physiopathology; D055815:Young Adult
D000889:Anti-Arrhythmia Agents