Dictionary of Human Evolution and Biology

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Long-QT Syndrome

Congenital, heritable condition predisposing affected individuals to heart arrhythmia, fainting, and sudden cardiac arrest in the presence of loud sounds or during exercise. Defective potassium channels result in the accumulation of potassium in the heart and inner ears, resulting in heart defects and deafness. The mutant autosomal dominant gene in LQT is KCNQ1. One of the ion channel diseases.Exhibits evidence for genomic imprinting. LQT has also been investigated in some SIDS cases. Aka Romano-Ward syndrome; includes a variant known as the Jervell and Lange-Nielson syndrome.

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