Abstract

It has been more than 30 years since an association between sudden infant death syndrome (SIDS) and abnormal cardiac repolarization was first postulated.1,2 Enormous advances in our clinical understanding of heritable arrhythmia syndromes (aka “the cardiac channelopathies”), our scientific understanding of ion channels and how mutant ion channels impart their proarrhythmic phenotype to the patient, and our laboratory techniques, which include high-throughput mutational analysis, have given us the tools to begin to dissect a complex, multifactorial disease such as SIDS. Now, a clearer picture of how cardiac channelopathies can create the pathogenic substrate for sudden death during the first year of life as well as the prevalence of channelopathic SIDS among autopsy negative sudden infant deaths is emerging. This review highlights the substantial progress that has been made over the last decade, in particular highlighting the most recent findings that solidify the role that ion channels play in SIDS.

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