What is the association between Wolff-Parkinson-White (WPW) syndrome and autosomal dominant familial hypertrophic cardiomyopathy?

Updated: Jan 08, 2017
  • Author: Christopher R Ellis, MD, FACC, FHRS; Chief Editor: Mikhael F El-Chami, MD  more...
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Answer

Clinicians have long recognized the association of WPW syndrome with autosomal dominant familial hypertrophic cardiomyopathy. However, only comparatively recently was a genetic substrate linking hypertrophic cardiomyopathy to WPW syndrome and skeletal myopathy described. [2]

Patients with mutations in the gamma 2 subunit of adenosine monophosphate (AMP)-activated protein kinase (PRKAG2) develop cardiomyopathy characterized by ventricular hypertrophy, WPW syndrome, AV block, and progressive degenerative conduction system disease. The mutation is believed to produce disruption of the annulus fibrosus by accumulation of glycogen within myocytes, which causes preexcitation. This is thought to be the case in Pompe disease, Danon disease, and other glycogen-storage diseases.


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