Key result
Mutations in the PRKAG2 gene were identified as the cause of a familial arrhythmogenic syndrome characterized by ventricular preexcitation, conduction system disease, and cardiac hypertrophy.
Population
Families with an inherited arrhythmogenic syndrome characterized by ventricular preexcitation, progressive…
Design
Review
Authors
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Prompts PRKAG2 inclusion in panels for hypertrophy with preexcitation; leaves open natural history and management questions.
The identification of PRKAG2 mutations as the cause of a familial syndrome involving ventricular preexcitation and hypertrophy suggests a novel cardiac glycogenosis syndrome.
Gollob et al. (2002) conducted a review in PRKAG2 cardiac syndrome. PRKAG2 mutations was evaluated. Mutations in the PRKAG2 gene were identified as the cause of a familial arrhythmogenic syndrome characterized by ventricular preexcitation, conduction system disease, and cardiac hypertrophy.
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