No conflicts of interest were declared.
A Rare Connection: Fasciculoventricular Pathway in PRKAG2 Disease
Article first published online: 1 SEP 2009
© 2009 Wiley Periodicals, Inc.
Journal of Cardiovascular Electrophysiology
Volume 21, Issue 3, pages 329–332, March 2010
How to Cite
GOVINDAN, M., WARD, D. and BEHR, E. (2010), A Rare Connection: Fasciculoventricular Pathway in PRKAG2 Disease. Journal of Cardiovascular Electrophysiology, 21: 329–332. doi: 10.1111/j.1540-8167.2009.01578.x
- Issue published online: 22 FEB 2010
- Article first published online: 1 SEP 2009
- Manuscript received 17 April 2009; Revised manuscript received 2 June 2009; Accepted for publication 17 June 2009.
- 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)