• PRKAG2;
  • Wolff–Parkinson–White syndrome;
  • Mahaim fiber;
  • fasciculoventricular pathway

Fasciculoventricular Pathway in PRKAG2 Disease.  Mutations in the PRKAG2 gene that regulates the gamma 2 subunit of the AMP-dependent protein kinase A have been associated with the development of AV accessory pathways, cardiac hypertrophy, and conduction system abnormalities. The accessory pathways described in PRKAG2 disease have mostly been AV bypass tracts, as mutations in the PRKAG2 gene disrupt the normal AV junction development. There have also been a few reports of nodoventricular tracts associated with PRKAG2 mutations, as these tracts also involve the AV junction. We describe a case of a fasciculoventricular pathway with PRKAG2 mutation suggesting a more widespread involvement of the PRKAG2 gene in the development of the cardiac conduction system. (J Cardiovasc Electrophysiol, Vol. 21, pp. 329–332, March 2010)