Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation

Atrial Pathology Findings in a Patient With PRKAG2 Cardiomyopathy and Persistent Atrial Fibrillation

“…Patients with R302Q have a high incidence of atrial fibrillation. 3 Ventricular preexcitation and hypertrophy, which are commonly present in these patients, may be contributors. It has also been suggested that in the absence of fibrosis, a reduction of pH because of increased glycogen content could influence ionic channels function, which may contribute to atrial fibrillation maintenance.…”

Nodo- and fasciculoventricular pathways: Electrophysiological features and a proposed diagnostic algorithm for preexcitation variants

“…6 Patients with FVP and the PRKAG2 mutation have distinct clinical, electrocardiographic, and electrophysiological features. They have a high incidence of syncope/cardiac arrest, ventricular hypertrophy, atrial arrhythmias, sinus bradycardia, and complete AV block requiring device implantation.…”

AMP-Activated Protein Kinase

“…1,32,33 The pathology caused by the diseaseproducing PRKAG2 mutations Arg302Gln, Thr400Asn, and Asn488Ile is characterized by preexcitation, atrial fibrillation (AF), progressive conduction system disease, and cardiac hypertrophy. 32,33 Mice overexpressing PRKAG2 genes with disease-causing mutations R302Q, N488I, or R531G in the heart recapitulate the phenotype of human PRKAG2 cardiomyopathy. [34][35][36] The effects of these mutations on AMPK function is complex: they seem to enhance basal activity but impair activation because of AMP accumulation.…”