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. (C) 2011 Elsevier Inc. All rights reserved.