Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: The role of PRKAG2 mutation
| Main Author: | |
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| Publication Date: | 2011 |
| Other Authors: | , , , , , , , , |
| 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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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 |
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2011 |
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2011-01 |
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2012-03-22T13:54:11Z |
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1547-5271 |
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v. 8 n. 1 |
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DOI: 10.1016/j.hrthm.2010.09.081 |
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