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

Bibliographic Details
Main Author: Sternick, Eduardo Back
Publication Date: 2011
Other Authors: Oliva, Antonio, Gerken, Luiz Márcio, Magalhães, Luiz, Scarpelli, Ricardo, Correia, Frederico Soares, Rego, Silvia, Santana, Oto Oliviera, Brugada, Ramon, Wellens, Hein J. J.
Format: Article
Language: eng
Source: Repositório Institucional da UFBA
Download full: http://www.repositorio.ufba.br/ri/handle/ri/5554
Summary: Acesso restrito: Texto completo. p. 58-64
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spelling Sternick, Eduardo BackOliva, AntonioGerken, Luiz MárcioMagalhães, LuizScarpelli, RicardoCorreia, Frederico SoaresRego, SilviaSantana, Oto OlivieraBrugada, RamonWellens, Hein J. J.Sternick, Eduardo BackOliva, AntonioGerken, Luiz MárcioMagalhães, LuizScarpelli, RicardoCorreia, Frederico SoaresRego, SilviaSantana, Oto OlivieraBrugada, RamonWellens, Hein J. J.2012-03-22T13:54:11Z2011-011547-5271http://www.repositorio.ufba.br/ri/handle/ri/5554v. 8 n. 1Acesso restrito: Texto completo. p. 58-64BACKGROUND 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.Submitted by JURANDI DE SOUZA SILVA (jssufba@hotmail.com) on 2012-03-22T13:54:11Z No. of bitstreams: 1 __ac.els-cdn.com_S154752...f5e4534653cd08a1b170aac6c2c7c.pdf: 734057 bytes, checksum: d5d7c90cadd14c3d94fad8680a3a88d3 (MD5)Made available in DSpace on 2012-03-22T13:54:11Z (GMT). No. of bitstreams: 1 __ac.els-cdn.com_S154752...f5e4534653cd08a1b170aac6c2c7c.pdf: 734057 bytes, checksum: d5d7c90cadd14c3d94fad8680a3a88d3 (MD5) Previous issue date: 2011-01DOI: 10.1016/j.hrthm.2010.09.081reponame:Repositório Institucional da UFBAinstname:Universidade Federal da Bahia (UFBA)instacron:UFBAAccessory atrioventricular pathwayAtrial fibrillationAtrial flutterAtrioventricular blockFasciculoventricular pathwayGlycogen storage cardiomyopathyPRKAG2 mutationWolff-Parkinson- White syndromeClinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutationHEART RHYTHMinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article10000-01-01enginfo:eu-repo/semantics/openAccessORIGINAL__ac.els-cdn.com_S154752...f5e4534653cd08a1b170aac6c2c7c.pdf__ac.els-cdn.com_S154752...f5e4534653cd08a1b170aac6c2c7c.pdfapplication/pdf734057https://repositorio.ufba.br/bitstream/ri/5554/1/__ac.els-cdn.com_S154752...f5e4534653cd08a1b170aac6c2c7c.pdfd5d7c90cadd14c3d94fad8680a3a88d3MD51LICENSElicense.txtlicense.txttext/plain1762https://repositorio.ufba.br/bitstream/ri/5554/2/license.txt1b89a9a0548218172d7c829f87a0eab9MD52TEXT__ac.els-cdn.com_S154752...f5e4534653cd08a1b170aac6c2c7c.pdf.txt__ac.els-cdn.com_S154752...f5e4534653cd08a1b170aac6c2c7c.pdf.txtExtracted texttext/plain29733https://repositorio.ufba.br/bitstream/ri/5554/3/__ac.els-cdn.com_S154752...f5e4534653cd08a1b170aac6c2c7c.pdf.txt0348bcb83b4007dfe02fb0afa8c5481fMD53ri/55542022-07-05 14:03:21.688oai:repositorio.ufba.br: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Repositório InstitucionalPUBhttp://192.188.11.11:8080/oai/requestrepositorio@ufba.bropendoar:19322022-07-05T17:03:21Repositório Institucional da UFBA - Universidade Federal da Bahia (UFBA)false
dc.title.pt_BR.fl_str_mv Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation
dc.title.alternative.pt_BR.fl_str_mv HEART RHYTHM
title Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation
spellingShingle Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation
Sternick, Eduardo Back
Accessory atrioventricular pathway
Atrial fibrillation
Atrial flutter
Atrioventricular block
Fasciculoventricular pathway
Glycogen storage cardiomyopathy
PRKAG2 mutation
Wolff-Parkinson- White syndrome
title_short Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation
title_full Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation
title_fullStr Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation
title_full_unstemmed Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation
title_sort Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation
author Sternick, Eduardo Back
author_facet Sternick, Eduardo Back
Oliva, Antonio
Gerken, Luiz Márcio
Magalhães, Luiz
Scarpelli, Ricardo
Correia, Frederico Soares
Rego, Silvia
Santana, Oto Oliviera
Brugada, Ramon
Wellens, Hein J. J.
author_role author
author2 Oliva, Antonio
Gerken, Luiz Márcio
Magalhães, Luiz
Scarpelli, Ricardo
Correia, Frederico Soares
Rego, Silvia
Santana, Oto Oliviera
Brugada, Ramon
Wellens, Hein J. J.
author2_role author
author
author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Sternick, Eduardo Back
Oliva, Antonio
Gerken, Luiz Márcio
Magalhães, Luiz
Scarpelli, Ricardo
Correia, Frederico Soares
Rego, Silvia
Santana, Oto Oliviera
Brugada, Ramon
Wellens, Hein J. J.
Sternick, Eduardo Back
Oliva, Antonio
Gerken, Luiz Márcio
Magalhães, Luiz
Scarpelli, Ricardo
Correia, Frederico Soares
Rego, Silvia
Santana, Oto Oliviera
Brugada, Ramon
Wellens, Hein J. J.
dc.subject.por.fl_str_mv Accessory atrioventricular pathway
Atrial fibrillation
Atrial flutter
Atrioventricular block
Fasciculoventricular pathway
Glycogen storage cardiomyopathy
PRKAG2 mutation
Wolff-Parkinson- White syndrome
topic Accessory atrioventricular pathway
Atrial fibrillation
Atrial flutter
Atrioventricular block
Fasciculoventricular pathway
Glycogen storage cardiomyopathy
PRKAG2 mutation
Wolff-Parkinson- White syndrome
description Acesso restrito: Texto completo. p. 58-64
publishDate 2011
dc.date.issued.fl_str_mv 2011-01
dc.date.accessioned.fl_str_mv 2012-03-22T13:54:11Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://www.repositorio.ufba.br/ri/handle/ri/5554
dc.identifier.issn.none.fl_str_mv 1547-5271
dc.identifier.number.pt_BR.fl_str_mv v. 8 n. 1
identifier_str_mv 1547-5271
v. 8 n. 1
url http://www.repositorio.ufba.br/ri/handle/ri/5554
dc.language.iso.fl_str_mv eng
language eng
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.source.pt_BR.fl_str_mv DOI: 10.1016/j.hrthm.2010.09.081
dc.source.none.fl_str_mv reponame:Repositório Institucional da UFBA
instname:Universidade Federal da Bahia (UFBA)
instacron:UFBA
instname_str Universidade Federal da Bahia (UFBA)
instacron_str UFBA
institution UFBA
reponame_str Repositório Institucional da UFBA
collection Repositório Institucional da UFBA
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