Key result
Genetic studies have identified 13 forms of congenital LQTS in 50-80% of clinically affected patients, whereas only one-third of Brugada syndrome patients can be genotyped, guiding genotype-specific management.
Population
Patients with inherited cardiac arrhythmias, specifically congenital long QT syndrome and Brugada syndrome
Design
Review
Authors
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Updated LQTS and BrS diagnostic criteria should inform immediate clinical decisions; extends genetic classification to 13 forms.
Advances in genetic testing and genotype-phenotype correlations are increasingly informing the diagnosis and genotype-specific management of inherited cardiac arrhythmias.
Wataru Shimizu (2013) conducted a review in Inherited Cardiac Arrhythmias (Congenital Long QT Syndrome and Brugada Syndrome). Genetic testing and genotype-specific management was evaluated. Genetic studies have identified 13 forms of congenital LQTS in 50-80% of clinically affected patients, whereas only one-third of Brugada syndrome patients can be genotyped, guiding genotype-specific management.
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