Does clinical screening identify inherited arrhythmia syndromes in relatives of subjects who died suddenly before 45 years of age without autopsy selection?
Clinical screening, including pharmacological challenges, is highly effective for diagnosing inherited arrhythmia syndromes in relatives of young sudden death victims, even in the absence of an autopsy.
BACKGROUND: After sudden cardiac death with negative autopsy, clinical screening of relatives identifies a high proportion of inherited arrhythmia syndrome. However, the efficacy of this screening in families not selected by autopsy has never been assessed. We aim to investigate the value of clinical screening in relatives of all subjects who died suddenly before 45 years of age. METHODS AND RESULTS: <0.0001) with 17 Brugada syndromes and 15 long QT syndromes diagnosed based on pharmacological tests. CONCLUSIONS: Even without autopsy, familial screening after sudden death in young patients is effective. Broad screening of relatives and systematic tests, including pharmacological challenges, greatly increases the likelihood of diagnosis in families.
Quenin et al. (Fri,) studied this question.