1 Sudden Arrhythmic Death Syndrome (sads): Diagnostic Yield of Comprehensive Clinical Evaluation of Paediatric First-degree Relatives

    loading  Checking for direct PDF access through Ovid

Abstract

Background

Sudden arrhythmic death syndrome (SADS) is most often caused by heritable cardiac diseases. Studies in adults have identified evidence of inherited cardiovascular diseases in up to 53% of families, but data on the prevalence of familial disease in children are scarce. The aim of this study was to evaluate the yield of clinical screening in paediatric first-degree relatives of victims of SADS or aborted cardiac arrest (ACA) using a systematic and comprehensive protocol.

Methods

All consecutive paediatric patients referred for family screening between 2003 and 2013 after a SCD or ACA of a family member were retrospectively enrolled into the study. Systematic evaluation of the children included clinical examination, family history, ECG, echocardiogram, 24-hour tape and signal averaged ECG. Older patients also underwent exercise testing, cardiac MRI and ajmaline provocation testing.

Results

A total of 110 children from 63 consecutive families were included in the study. An inherited cardiac disease was identified in 12 1st-degree children from 11 (17.5%) families (7 children were diagnosed with Brugada syndrome, 2 with long QT syndrome, 1 with CPVT, and 2 had late potentials on signal averaged ECGs).

Conclusions

These data show a high prevalence of inherited heart disease in paediatric first-degree relative in families with a history of SCD or ACA. The results highlight the importance of a systematic, comprehensive approach and ongoing screening of paediatric family members.

Related Topics

    loading  Loading Related Articles