title: Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: the role of PRKAG2 mutation.
abstract: BACKGROUND
  The ECG, clinical, and electrophysiologic profiles of patients with a fasciculoventricular pathway are well described. Fasciculoventricular pathways occurring in the setting of glycogen storage cardiomyopathy possess unique features.
  
  
  OBJECTIVE
  The purpose of this study was to compare the clinical, ECG, and electrophysiologic characteristics of patients with a fasciculoventricular pathway, with or without glycogen storage cardiomyopathy.
  
  
  METHODS
  Two groups of patients with a fasciculoventricular pathway were compared: group A consisted of 10 patients with the PRKAG2 mutation (Arg302gln), and group B consisted of 9 patients without the mutation.
  
  
  RESULTS
  Thirty percent of group A patients had left ventricular hypertrophy, and none had an additional accessory pathway. Group B patients had no structural heart disease, and 33% had an additional accessory pathway. Group A patients had a slower resting heart rate (56 ± 7 vs 75 ± 10 bpm, P <0.0001), a wider QRS complex (0.15 ± 0.01 vs 0.11 ± 0.02 ms, P = .0004), and a longer HV interval (34 ± 1 vs 25 ± 3 ms, P = .0003). During long-term follow-up, 50% of group A patients developed complete AV block versus none in group B. Eighty percent of group A patients developed atrial flutter and/or atrial fibrillation. No Group B patient had any arrhythmia during follow-up after successful ablation of additional arrhythmia circuits. No sustained ventricular arrhythmia was induced in any patient from either group.
  
  
  CONCLUSIONS
  Patients with a fasciculoventricular pathway associated with the PRKAG2 mutation have distinct clinical, ECG, and electrophysiologic profiles and should be correctly identified because of their ominous long-term prognosis. Patients without the mutation have an excellent arrhythmia-free prognosis after treatment of additional circuits.
authors.affiliation: Arrhythmia Unit, Biocor Instituto, Nova Lima, Brazil. eduardosternick@terra.com.br
authors.forename: Eduardo Back
authors.initials: EB
authors.lastname: Sternick
authors.forename: Antonio
authors.initials: A
authors.lastname: Oliva
authors.forename: Luiz Márcio
authors.initials: LM
authors.lastname: Gerken
authors.forename: Luiz
authors.initials: L
authors.lastname: Magalhães
authors.forename: Ricardo
authors.initials: R
authors.lastname: Scarpelli
authors.forename: Frederico Soares
authors.initials: FS
authors.lastname: Correia
authors.forename: Silvia
authors.initials: S
authors.lastname: Rego
authors.forename: Oto
authors.initials: O
authors.lastname: Santana
authors.forename: Ramon
authors.initials: R
authors.lastname: Brugada
authors.forename: Hein J J
authors.initials: HJ
authors.lastname: Wellens
mesh_terms: D055372:AMP-Activated Protein Kinases / Q000235:genetics*; D058606:Accessory Atrioventricular Bundle / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics* / Q000503:physiopathology; D000328:Adult; D015897:Comorbidity; D004562:Electrocardiography; D022062:Electrophysiologic Techniques, Cardiac; D005260:Female; D052120:Glycogen Storage Disease Type IIb / Q000453:epidemiology; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000453:epidemiology / Q000235:genetics; D008297:Male; D008875:Middle Aged; D020125:Mutation, Missense; D011379:Prognosis; D012189:Retrospective Studies; D014927:Wolff-Parkinson-White Syndrome / Q000235:genetics; D055815:Young Adult
chemical_list: C526223:PRKAG2 protein, human; D055372:AMP-Activated Protein Kinases