Clinical evaluation of paediatric relatives of sudden cardiac death victims identified inherited heart disease in 20% of SUD relatives and 39% of relatives with known familial disease.
Observational (n=36)
Does comprehensive clinical evaluation identify inherited heart diseases in paediatric relatives of sudden cardiac death or aborted cardiac arrest victims?
Comprehensive clinical evaluation of paediatric relatives of sudden cardiac death victims identifies a substantial number of at-risk individuals, allowing for targeted preventive treatments.
AIMS: 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). METHODS AND 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. CONCLUSION: 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.
Tomaske et al. (Tue,) conducted a observational in Family history of premature sudden cardiac death (SCD) or aborted cardiac arrest (ACA) (n=36). Clinical evaluation was evaluated on Identification of an inherited heart disease. Clinical evaluation of paediatric relatives of sudden cardiac death victims identified inherited heart disease in 20% of SUD relatives and 39% of relatives with known familial disease.
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