High risk of sudden death associated with a PRKAG2-related familial Wolff-Parkinson-White syndrome

Familial appearance of Wolff-Parkinson-White (WPW) syndrome is rare and displays an autosomal dominant inheritance. Here we report a Chinese kindred of WPW syndrome whose unique clinical features consist of a high risk of sudden cardiac death due to atrial fibrillation, causing a rapid antegrade conduct over the accessory pathway. The mutation in the PRKAG2 gene was identified as responsible for the familial form of WPW syndrome.