Compound heterozygous mutations in the SCN5A-encoded Nav1.5 cardiac sodium channel resulting in atrial standstill and His-Purkinje system disease.

Baskar, Shankar; Ackerman, Michael J; Clements, Diane; Mayuga, Kenneth A; Aziz, Peter F · J Pediatr · 2014

case_report · Level V

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Abstract

An 11-year-old girl on evaluation for syncope was found to have progressive sinus node dysfunction and His-Purkinje system disease with atrial standstill. Genetic analysis revealed compound heterozygous mutations of the SCN5A gene in a novel combination.

Medical subject headings