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

Eduardo Back Sternick; Antonio Oliva; Luiz Marcio Gerken; Luiz Magalhaes; Ricardo Scarpelli; Frederico Soares Correia; Silvia Rego; Oto Santana; Ramon Brugada; Hein J. J. Wellens

doi:10.1016/j.hrthm.2010.09.081

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

Eduardo Back Sternick^*, Antonio Oliva, Luiz Marcio Gerken, Luiz Magalhaes, Ricardo Scarpelli, Frederico Soares Correia, Silvia Rego, Oto Santana, Ramon Brugada, Hein J. J. Wellens

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

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

Original language	English
Pages (from-to)	58-64
Journal	Heart Rhythm
Volume	8
Issue number	1
DOIs	https://doi.org/10.1016/j.hrthm.2010.09.081
Publication status	Published - Jan 2011

Keywords

Accessory atrioventricular pathway
Atrial fibrillation
Atrial flutter
Atrioventricular block
Fasciculoventricular pathway
Glycogen storage cardiomyopathy
PRKAG2 mutation
Wolff-Parkinson-White syndrome

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10.1016/j.hrthm.2010.09.081

Cite this

Sternick, E. B., Oliva, A., Gerken, L. M., Magalhaes, L., Scarpelli, R., Correia, F. S., Rego, S., Santana, O., Brugada, R., & Wellens, H. J. J. (2011). Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation. Heart Rhythm, 8(1), 58-64. https://doi.org/10.1016/j.hrthm.2010.09.081

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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",

keywords = "Accessory atrioventricular pathway, Atrial fibrillation, Atrial flutter, Atrioventricular block, Fasciculoventricular pathway, Glycogen storage cardiomyopathy, PRKAG2 mutation, Wolff-Parkinson-White syndrome",

author = "Sternick, {Eduardo Back} and Antonio Oliva and Gerken, {Luiz Marcio} and Luiz Magalhaes and Ricardo Scarpelli and Correia, {Frederico Soares} and Silvia Rego and Oto Santana and Ramon Brugada and Wellens, {Hein J. J.}",

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doi = "10.1016/j.hrthm.2010.09.081",

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T1 - Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation

AU - Sternick, Eduardo Back

AU - Oliva, Antonio

AU - Gerken, Luiz Marcio

AU - Magalhaes, Luiz

AU - Scarpelli, Ricardo

AU - Correia, Frederico Soares

AU - Rego, Silvia

AU - Santana, Oto

AU - Brugada, Ramon

AU - Wellens, Hein J. J.

PY - 2011/1

Y1 - 2011/1

N2 - 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

AB - 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

KW - Accessory atrioventricular pathway

KW - Atrial fibrillation

KW - Atrial flutter

KW - Atrioventricular block

KW - Fasciculoventricular pathway

KW - Glycogen storage cardiomyopathy

KW - PRKAG2 mutation

KW - Wolff-Parkinson-White syndrome

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DO - 10.1016/j.hrthm.2010.09.081

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SN - 1547-5271

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JO - Heart Rhythm

JF - Heart Rhythm

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