Molecular Diagnosis of the Inherited Long-QT Syndrome in a Woman Who Died after Near-Drowning

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Abstract
Drowning accounts for more accidental deaths in children and adolescents than all other causes except motor vehicle accidents.1-3 Many of these fatalities are attributed to lack of supervision, trauma, alcohol or drug use, or seizures. However, an appreciable number of drownings have no satisfactory explanation. In these situations, cardiac arrhythmias, particularly those associated with the long-QT syndrome, may be an important consideration. The long-QT syndrome comprises a group of genetically distinct arrhythmogenic cardiovascular disorders, each resulting from a mutation in one of five genes encoding cardiac ion channels or auxiliary ion-channel subunits: KVLQT1 (at the LQT1 locus), HERG (at LQT2), SCN5A (at LQT3), hKCNE1 (encoding minimal potassium-channel beta subunit [minK], at LQT5), and hKCNE2 (encoding minK-related peptide 1 [MiRP-1], at LQT6).4,5 As compared with other exertional activities, swimming seems to be particularly arrhythmogenic in patients with the long-QT syndrome.6-8