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.
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.
Authors: Andrea Giuseppe Porto; Francesca Brun; Giovanni Maria Severini; Pasquale Losurdo; Enrico Fabris; Matthew R G Taylor; Luisa Mestroni; Gianfranco Sinagra Journal: Circ Arrhythm Electrophysiol Date: 2016-01
Authors: Arthur A M Wilde; Christopher Semsarian; Manlio F Márquez; Alireza Sepehri Shamloo; Michael J Ackerman; Euan A Ashley; Back Sternick Eduardo; Héctor Barajas-Martinez; Elijah R Behr; Connie R Bezzina; Jeroen Breckpot; Philippe Charron; Priya Chockalingam; Lia Crotti; Michael H Gollob; Steven Lubitz; Naomasa Makita; Seiko Ohno; Martín Ortiz-Genga; Luciana Sacilotto; Eric Schulze-Bahr; Wataru Shimizu; Nona Sotoodehnia; Rafik Tadros; James S Ware; David S Winlaw; Elizabeth S Kaufman; Takeshi Aiba; Andreas Bollmann; Jong-Il Choi; Aarti Dalal; Francisco Darrieux; John Giudicessi; Mariana Guerchicoff; Kui Hong; Andrew D Krahn; Ciorsti Mac Intyre; Judith A Mackall; Lluís Mont; Carlo Napolitano; Pablo Ochoa Juan; Petr Peichl; Alexandre C Pereira; Peter J Schwartz; Jon Skinner; Christoph Stellbrink; Jacob Tfelt-Hansen; Thomas Deneke Journal: J Arrhythm Date: 2022-05-31
Authors: Arthur A M Wilde; Christopher Semsarian; Manlio F Márquez; Alireza Sepehri Shamloo; Michael J Ackerman; Euan A Ashley; Eduardo Back Sternick; Héctor Barajas-Martinez; Elijah R Behr; Connie R Bezzina; Jeroen Breckpot; Philippe Charron; Priya Chockalingam; Lia Crotti; Michael H Gollob; Steven Lubitz; Naomasa Makita; Seiko Ohno; Martín Ortiz-Genga; Luciana Sacilotto; Eric Schulze-Bahr; Wataru Shimizu; Nona Sotoodehnia; Rafik Tadros; James S Ware; David S Winlaw; Elizabeth S Kaufman; Takeshi Aiba; Andreas Bollmann; Jong Il Choi; Aarti Dalal; Francisco Darrieux; John Giudicessi; Mariana Guerchicoff; Kui Hong; Andrew D Krahn; Ciorsti MacIntyre; Judith A Mackall; Lluís Mont; Carlo Napolitano; Juan Pablo Ochoa; Petr Peichl; Alexandre C Pereira; Peter J Schwartz; Jon Skinner; Christoph Stellbrink; Jacob Tfelt-Hansen; Thomas Deneke Journal: Europace Date: 2022-09-01 Impact factor: 5.486
Authors: Yang Liu; Rong Bai; Lin Wang; Cuntai Zhang; Ruifu Zhao; Deli Wan; Xinshan Chen; Gabriel Caceres; Daniel Barr; Hector Barajas-Martinez; Charles Antzelevitch; Dan Hu Journal: PLoS One Date: 2013-05-31 Impact factor: 3.240
Authors: Arash Yavari; Mohamed Bellahcene; Annalisa Bucchi; Syevda Sirenko; Katalin Pinter; Neil Herring; Julia J Jung; Kirill V Tarasov; Emily J Sharpe; Markus Wolfien; Gabor Czibik; Violetta Steeples; Sahar Ghaffari; Chinh Nguyen; Alexander Stockenhuber; Joshua R St Clair; Christian Rimmbach; Yosuke Okamoto; Dongmei Yang; Mingyi Wang; Bruce D Ziman; Jack M Moen; Daniel R Riordon; Christopher Ramirez; Manuel Paina; Joonho Lee; Jing Zhang; Ismayil Ahmet; Michael G Matt; Yelena S Tarasova; Dilair Baban; Natasha Sahgal; Helen Lockstone; Rathi Puliyadi; Joseph de Bono; Owen M Siggs; John Gomes; Hannah Muskett; Mahon L Maguire; Youlia Beglov; Matthew Kelly; Pedro P N Dos Santos; Nicola J Bright; Angela Woods; Katja Gehmlich; Henrik Isackson; Gillian Douglas; David J P Ferguson; Jürgen E Schneider; Andrew Tinker; Olaf Wolkenhauer; Keith M Channon; Richard J Cornall; Eduardo B Sternick; David J Paterson; Charles S Redwood; David Carling; Catherine Proenza; Robert David; Mirko Baruscotti; Dario DiFrancesco; Edward G Lakatta; Hugh Watkins; Houman Ashrafian Journal: Nat Commun Date: 2017-11-02 Impact factor: 14.919
Authors: Lenises de Paula van der Steld; Oscar Campuzano; Alexandra Pérez-Serra; Mabel Moura de Barros Zamorano; Selma Sousa Matos; Ramon Brugada Journal: Am J Case Rep Date: 2017-07-10
Authors: José Luiz Barros Pena; Wander Costa Santos; Stanley de Almeida Araújo; Glauber Monteiro Dias; Eduardo Back Sternick Journal: Arq Bras Cardiol Date: 2018-11 Impact factor: 2.000