| Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation. | |
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MedLine Citation:
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PMID: 20888928 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
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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. CONCLUSION: 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. |
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Authors:
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Eduardo Back Sternick; Antonio Oliva; Luiz Márcio Gerken; Luiz Magalhães; Ricardo Scarpelli; Frederico Soares Correia; Silvia Rego; Oto Santana; Ramon Brugada; Hein J J Wellens |
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Publication Detail:
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Type: Journal Article Date: 2010-10-01 |
Journal Detail:
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Title: Heart rhythm : the official journal of the Heart Rhythm Society Volume: 8 ISSN: 1556-3871 ISO Abbreviation: Heart Rhythm Publication Date: 2011 Jan |
Date Detail:
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Created Date: 2010-12-27 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101200317 Medline TA: Heart Rhythm Country: United States |
Other Details:
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Languages: eng Pagination: 58-64 Citation Subset: IM |
Copyright Information:
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Copyright © 2011 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved. |
Affiliation:
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Arrhythmia Unit, Biocor Instituto, Nova Lima, Brazil. eduardosternick@terra.com.br |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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