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1.
J Cardiovasc Electrophysiol ; 31(6): 1413-1419, 2020 06.
Article in English | MEDLINE | ID: mdl-32298040

ABSTRACT

INTRODUCTION: Catheter ablation of the parahisian accessory pathways (PHAP) has been established as the definitive therapy for this type of arrhythmia. However, the PHAP proximity to the normal atrioventricular conduction system makes the procedure technically challenging. Here, we have reported a case series of 20 patients with PHAP who underwent aortic access ablation to evaluate the safety and efficacy of this approach in the PHAP ablation. METHODS AND RESULTS: The ablation through the aortic cusps was the successful approach in 13 of 20 (65%) of the cases. In 11 patients, the aortic approach was the initial strategy for ablation, and the accessory pathway was eliminated in seven (63.6%) of them. The aortic approach followed a failed right-sided attempt in nine patients. In six (66.7%) patients, the ablation was successful with the aortic approach. The only independent predictor for the successful ablation with each approach was the earliest ventricular activation before delta wave (predelta time) and a right-sided earliest ventricular activation of more than 23 ms had high sensitivity and specificity for right-sided success. Systematically using the two strategies (right and left approaches), the ablation of the PHAP was successful in 18 (90%) patients. CONCLUSION: The aortic approach seems to be a safe and effective strategy for the ablation of PHAP. It can be used when the right-sided approach fails or even considered as an initial strategy when the predelta time is less than 23 ms in the right septal region. When combining the right- and left-sided approaches, the success rate is high. We believe that the retrograde aortic approach remains a key tool for this challenging ablation.


Subject(s)
Accessory Atrioventricular Bundle/surgery , Arrhythmias, Cardiac/surgery , Catheter Ablation , Electrophysiologic Techniques, Cardiac , Accessory Atrioventricular Bundle/physiopathology , Action Potentials , Adult , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/physiopathology , Catheter Ablation/adverse effects , Electrocardiography , Female , Heart Rate , Humans , Male , Predictive Value of Tests , Recurrence , Retrospective Studies , Treatment Outcome
2.
J Cardiovasc Electrophysiol ; 30(12): 3097-3115, 2019 12.
Article in English | MEDLINE | ID: mdl-31646696

ABSTRACT

The recognition of the presence, location, and properties of unusual accessory pathways for atrioventricular conduction is an exciting, but frequently a difficult, challenge for the clinical cardiac arrhythmologist. In this third part of our series of reviews, we discuss the different steps required to come to the correct diagnosis and management decision in patients with nodofascicular, nodoventricular, and fasciculo-ventricular pathways. We also discuss the concealed accessory atrioventricular pathways with the properties of decremental retrograde conduction that are associated with the so-called permanent form of junctional reciprocating tachycardia. Careful analysis of the 12-lead electrocardiogram during sinus rhythm and tachycardias should always precede the investigation in the catheterization room. When using programmed electrical stimulation of the heart from different intracardiac locations, combined with activation mapping, it should be possible to localize both the proximal and distal ends of the accessory connections. This, in turn, should then permit the determination of their electrophysiologic properties, providing the answer to the question "are they incorporated in a tachycardia circuit?". It is this information that is essential for decision-making with regard to the need for catheter ablation, and if necessary, its appropriate site.


Subject(s)
Accessory Atrioventricular Bundle/surgery , Action Potentials , Catheter Ablation , Heart Rate , Pre-Excitation, Mahaim-Type/surgery , Tachycardia, Reciprocating/surgery , Accessory Atrioventricular Bundle/physiopathology , Catheter Ablation/adverse effects , Electrocardiography , Electrophysiologic Techniques, Cardiac , Humans , Pre-Excitation, Mahaim-Type/diagnosis , Pre-Excitation, Mahaim-Type/physiopathology , Predictive Value of Tests , Tachycardia, Reciprocating/diagnosis , Tachycardia, Reciprocating/physiopathology , Treatment Outcome
3.
J Cardiovasc Electrophysiol ; 30(12): 3079-3096, 2019 12.
Article in English | MEDLINE | ID: mdl-31588593

ABSTRACT

Recognition of the presence, location, and properties of unusual accessory pathways for atrioventricular conduction is an exciting, frequently difficult, challenge for the clinical cardiac arrhythmologist. In this second part of our series of reviews relative to this topic, we discuss the steps required to achieve the correct diagnosis and appropriate management in patients with the so-called "Mahaim" variants of pre-excitation. We indicate that, nowadays, it is recognized that these abnormal rhythms are manifest because of the presence of atriofascicular pathways. These anatomical substrates, however, need to be distinguished from the other long and short accessory pathways which produce decremental atrioventricular conduction. The atriofascicular pathways, along with the long decrementally conducting pathways, have their atrial components located within the vestibule of the tricuspid valve. The short decremental pathways, in contrast, can originate in the vestibules of either the mitral or tricuspid valves. As a starting point, careful analysis of the 12-lead electrocardiogram, taken during both sinus rhythm and tachycardias, should precede any investigation in the catheterization room. When assessing the patient in the electrophysiological laboratory, the use of programmed electrical stimulation from different intracardiac locations, combined with entrainment technique and activation mapping, should permit the establishment of the properties of the accessory pathways, and localization of its proximal and distal ends. This should provide the answer to the question "is the pathway incorporated into the circuit underlying the clinical tachycardia". That information is essential for decision-making with regard to need, and localization of the proper site, for catheter ablation.


Subject(s)
Accessory Atrioventricular Bundle/surgery , Action Potentials , Catheter Ablation , Heart Rate , Pre-Excitation, Mahaim-Type/surgery , Accessory Atrioventricular Bundle/physiopathology , Catheter Ablation/adverse effects , Electrocardiography , Electrophysiologic Techniques, Cardiac , Humans , Pre-Excitation, Mahaim-Type/diagnosis , Pre-Excitation, Mahaim-Type/physiopathology , Predictive Value of Tests , Treatment Outcome
4.
Arch. cardiol. Méx ; Arch. cardiol. Méx;88(3): 212-218, jul.-sep. 2018. tab, graf
Article in Spanish | LILACS | ID: biblio-1088752

ABSTRACT

Resumen Objetivos: Las vías accesorias (VAc) fascículo-ventriculares (FV) tienen una localización anatómica similar a las VAcanteroseptales derechas (ASD) y comparten características electrocardiográficas. El objetivo es comparar características electrocardiográficas de las VAC FV con las de las ASD en pediatría. Métodos: Se incluyeron pacientes con preexcitación manifiesta sometidos a estudio electrofisiológico. Las VAc FV se definieron por un intervalo HV ≤ 32ms y un alargamiento del AH sin modificación del HV, del grado o patrón de preexcitación ventricular durante la estimulación auricular. Tres observadores independientes y ciegos analizaron los ECG en cada grupo. Resultados: De 288 pacientes, 15 (5.2%) presentaban VAC FV y 14 VAC ASD (4.9%). El intervalo PR fue más largo en las VAc FV que en las ASD (113 ± 21 vs. 86 ± 13 ms respectivamente; p = < 0.001) y la duración del QRS fue menor (95 ± 12 vs. 137 ± 24 ms respectivamente; p = < 0.001). El ECG de las VAc FV presentó una deflexión rápida de baja amplitud previa al inicio del QRS en 13 de 15 pacientes (87%) y en 2 con VAc AV ASD (14%); (p = 0.003). Conclusiones: El intervalo PR fue más largo y el complejo QRS más angosto en la VAC FV respecto de las ASD. La presencia de una deflexión rápida de baja amplitud previa al inicio del QRS permitiría diferenciarlas de las aurículo-ventriculares ASD de manera no invasiva.


Abstract Objectives: Fasciculo-ventricular (FV) accessory pathways (AP's) and right anteroseptal (RAS) AP's share similar anatomic locations and electrocardiographic characteristics. The objective of this article is to compare these features in children. Methods: All patients with manifest pre-excitation who underwent an electrophysiological study were included. Fasciculo-ventricular AP's were defined by the presence of an HV inter- val ≤ 32 ms and a prolongation of the AH without changes in the HV interval, or the level of pre-excitation during atrial pacing. Three independent and blind observers analysed the ECG's in both groups. Results: Out of 288 patients, 15 (5.2%) had FV AP's and 14 (4.9%) right AS AP's. The PR interval was longer in FV AP's than in RAS (113 ± 21 vs 86 ± 13 ms respectively; P < .001) and the QRS was narrower (95 ± 12 vs 137 ± 24 ms respectively; P < .001). The ECG in patients with FV AP's showed a rapid low amplitude deflection at the begining of the QRS in 13 out of 15 patients (87%) and in 2 (14%) the RAS AP group (P = .003). Conclusions: The PR interval was longer and the QRS complex was narrower in patients with FV AP's. The presence of a rapid low amplitude deflection at the beginning of the QRS complex would allow to differentiate them from RAS AP's non-invasively.


Subject(s)
Humans , Male , Female , Child , Adolescent , Wolff-Parkinson-White Syndrome/diagnosis , Electrocardiography/methods , Ventricular Septum/physiopathology , Accessory Atrioventricular Bundle/diagnosis , Wolff-Parkinson-White Syndrome/physiopathology , Retrospective Studies , Electrophysiologic Techniques, Cardiac , Accessory Atrioventricular Bundle/physiopathology
5.
Arch Cardiol Mex ; 88(3): 212-218, 2018.
Article in Spanish | MEDLINE | ID: mdl-28666599

ABSTRACT

OBJECTIVES: Fasciculo-ventricular (FV) accessory pathways (AP's) and right anteroseptal (RAS) AP's share similar anatomic locations and electrocardiographic characteristics. The objective of this article is to compare these features in children. METHODS: All patients with manifest pre-excitation who underwent an electrophysiological study were included. Fasciculo-ventricular AP's were defined by the presence of an HV interval≤32ms and a prolongation of the AH without changes in the HV interval, or the level of pre-excitation during atrial pacing. Three independent and blind observers analysed the ECG's in both groups. RESULTS: Out of 288 patients, 15 (5.2%) had FV AP's and 14 (4.9%) right AS AP's. The PR interval was longer in FV AP's than in RAS (113±21 vs 86±13ms respectively; P<.001) and the QRS was narrower (95±12 vs 137±24ms respectively; P<.001). The ECG in patients with FV AP's showed a rapid low amplitude deflection at the begining of the QRS in 13 out of 15 patients (87%) and in 2 (14%) the RAS AP group (P=.003). CONCLUSIONS: The PR interval was longer and the QRS complex was narrower in patients with FV AP's. The presence of a rapid low amplitude deflection at the beginning of the QRS complex would allow to differentiate them from RAS AP's non-invasively.


Subject(s)
Accessory Atrioventricular Bundle/diagnosis , Electrocardiography/methods , Ventricular Septum/physiopathology , Wolff-Parkinson-White Syndrome/diagnosis , Accessory Atrioventricular Bundle/physiopathology , Adolescent , Child , Electrophysiologic Techniques, Cardiac , Female , Humans , Male , Retrospective Studies , Wolff-Parkinson-White Syndrome/physiopathology
6.
Arq. bras. cardiol ; Arq. bras. cardiol;107(4): 331-338, Oct. 2016. tab, graf
Article in English | LILACS | ID: biblio-827850

ABSTRACT

Abstract Background: There are currently several electrocardiographic algorithms to locate the accessory pathway (AP) in patients with Wolff-Parkinson-White (WPW) syndrome. Objective: To compare the ability of electrocardiographic algorithms in identifying the location of the AP in patients with WPW pattern referred for ablation. Methods: Observational, cross-sectional, retrospective study with 111 patients with WPW syndrome referred for AP ablation. The electrocardiogram (ECG) obtained prior to the ablation was analyzed by an experienced observer who consecutively applied seven algorithms to identify non-invasively the AP. We then compared the location estimated with this assessment with that obtained in the electrophysiological study and calculated the agreement rates. Results: Among the APs, 59 (53.15%) were distributed around the mitral annulus and the remaining 52 (46.85%) were located around the tricuspid annulus. The overall absolute accuracy of the algorithms evaluated varied between 27% and 47%, increasing to between 40% and 76% when we included adjacent locations. The absolute agreement rate by AP location was 2.00-52.20% for septal APs (n = 51), increasing to 5.90-90.20% when considering adjacent locations; 7.70-69.20% for right APs (n = 13), increasing to 42.90-100% when considering adjacent locations; and 21.70-54.50% for left APs (n = 47), increasing to 50-87% when considering adjacent locations. Conclusion: The agreement rates observed for the analyzed scores indicated a low discriminative ability of the ECG in locating the AP in patients with WPW.


Resumo Fundamento: Existem atualmente vários algoritmos eletrocardiográficos para localizar a via acessória (VA) em pacientes com síndrome de Wolff-Parkinson-White (WPW). Objetivo: Comparar a capacidade discriminativa dos algoritmos eletrocardiográficos na localização da VA no padrão de WPW em pacientes encaminhados para ablação. Métodos: Estudo observacional, transversal e retrospectivo, incluindo 111 pacientes com síndrome de WPW encaminhados para ablação da VA. O eletrocardiograma (ECG) prévio à ablação foi analisado por um observador experiente que aplicou consecutivamente sete algoritmos para identificar a VA de forma não invasiva. A localização estimada com esta avaliação foi comparada à obtida no estudo eletrofisiológico e as taxas de acerto foram calculadas. Resultados: Entre as VAs, 59 (53,15%) estavam distribuídas ao redor do anel mitral e as restantes 52 (46,85%) em torno do anel tricúspide. O acerto global absoluto dos algoritmos em estudo variou entre 27% e 47%, aumentando para 40% a 76% quando incluímos localizações adjacentes. O acerto absoluto em função da localização da VA foi o seguinte: para as VAs septais (n = 51) variou entre 2% e 52,20% (5,90% e 90,20% incluindo localizações adjacentes), para as VAs direitas (n = 13) variou entre 7,70% e 69,20% (42,90% e 100%, incluindo localizações adjacentes), para as VAs esquerdas (n = 47) variou entre 21,70% e 54,50% (50% a 87%, incluindo as localizações adjacentes). Conclusões: Os índices de acerto observados para os escores analisados indicaram uma reduzida capacidade discriminativa do ECG na localização da VA em pacientes com WPW.


Subject(s)
Humans , Male , Female , Middle Aged , Young Adult , Wolff-Parkinson-White Syndrome/diagnosis , Algorithms , Electrocardiography/methods , Accessory Atrioventricular Bundle/diagnosis , Reference Standards , Reference Values , Wolff-Parkinson-White Syndrome/physiopathology , Cross-Sectional Studies , Reproducibility of Results , Retrospective Studies , Catheter Ablation , Statistics, Nonparametric , Accessory Atrioventricular Bundle/physiopathology
7.
Arq Bras Cardiol ; 107(4): 331-338, 2016 Oct.
Article in Portuguese, English | MEDLINE | ID: mdl-27627222

ABSTRACT

BACKGROUND: There are currently several electrocardiographic algorithms to locate the accessory pathway (AP) in patients with Wolff-Parkinson-White (WPW) syndrome. OBJECTIVE: To compare the ability of electrocardiographic algorithms in identifying the location of the AP in patients with WPW pattern referred for ablation. METHODS: Observational, cross-sectional, retrospective study with 111 patients with WPW syndrome referred for AP ablation. The electrocardiogram (ECG) obtained prior to the ablation was analyzed by an experienced observer who consecutively applied seven algorithms to identify non-invasively the AP. We then compared the location estimated with this assessment with that obtained in the electrophysiological study and calculated the agreement rates. RESULTS: Among the APs, 59 (53.15%) were distributed around the mitral annulus and the remaining 52 (46.85%) were located around the tricuspid annulus. The overall absolute accuracy of the algorithms evaluated varied between 27% and 47%, increasing to between 40% and 76% when we included adjacent locations. The absolute agreement rate by AP location was 2.00-52.20% for septal APs (n = 51), increasing to 5.90-90.20% when considering adjacent locations; 7.70-69.20% for right APs (n = 13), increasing to 42.90-100% when considering adjacent locations; and 21.70-54.50% for left APs (n = 47), increasing to 50-87% when considering adjacent locations. CONCLUSION: The agreement rates observed for the analyzed scores indicated a low discriminative ability of the ECG in locating the AP in patients with WPW.


Subject(s)
Accessory Atrioventricular Bundle/diagnosis , Algorithms , Electrocardiography/methods , Wolff-Parkinson-White Syndrome/diagnosis , Accessory Atrioventricular Bundle/physiopathology , Adult , Catheter Ablation , Cross-Sectional Studies , Female , Humans , Male , Middle Aged , Reference Standards , Reference Values , Reproducibility of Results , Retrospective Studies , Statistics, Nonparametric , Wolff-Parkinson-White Syndrome/physiopathology , Young Adult
8.
Circ Arrhythm Electrophysiol ; 8(1): 128-36, 2015 Feb.
Article in English | MEDLINE | ID: mdl-25527824

ABSTRACT

BACKGROUND: Epicardial mapping and ablation of accessory pathways through a subxiphoid approach can be an alternative when endocardial or epicardial transvenous mapping has failed. METHODS AND RESULTS: We reviewed acute and long-term follow-up of 21 patients (14 males) referred for percutaneous epicardial accessory pathway ablation. There was a median of 2 previous failed procedures. All patients were highly symptomatic, 8 had atrial fibrillation (3 with cardiac arrest) and 13 had frequent symptomatic episodes of atrioventricular reentrant tachycardia. Six patients (28.5%) had a successful epicardial ablation. Five patients (23.8%) underwent a successful repeated endocardial mapping, and ablation after epicardial mapping yielded no early activation site. Epicardial mapping was helpful in guiding endocardial ablation in 2 patients (9.5%), showing that the earliest activation was simultaneous at the epicardium and endocardium. Four patients (19%) underwent successful open-chest surgery after failing epicardial/endocardial ablation. Two patients (9.5%) remained controlled under antiarrhythmic drugs after unsuccessful endocardial/epicardial ablation. Two patients had a coronary sinus diverticulum and one a right atrium to right ventricle diverticulum. Three patients acquired postablation coronary sinus stenosis. There was no major complication related to pericardial access. CONCLUSIONS: Percutaneous epicardial approach is an alternative when conventional endocardial or transvenous epicardial ablation fails in the elimination of the accessory pathway. A new attempt by endocardial approach was successful in a significant number of patients. Open-chest surgery may be required in symptomatic cases refractory to endocardial-epicardial approach.


Subject(s)
Accessory Atrioventricular Bundle/surgery , Arrhythmias, Cardiac/surgery , Catheter Ablation/methods , Heart Conduction System/surgery , Pericardium/surgery , Accessory Atrioventricular Bundle/diagnosis , Accessory Atrioventricular Bundle/physiopathology , Adolescent , Adult , Aged , Anti-Arrhythmia Agents/therapeutic use , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/physiopathology , Brazil , Catheter Ablation/adverse effects , Epicardial Mapping , Female , Heart Conduction System/physiopathology , Humans , Male , Middle Aged , Pericardium/physiopathology , Predictive Value of Tests , Reoperation , Treatment Failure , Young Adult
10.
Europace ; 16(11): 1610-8, 2014 Nov.
Article in English | MEDLINE | ID: mdl-24681762

ABSTRACT

AIMS: Unlike in the Wolff-Parkinson-White syndrome, there has been no systematic study on the role of the pre-excitation pattern in predicting the ablation site in patients with atriofascicular (AF) pathways. We assessed in a large cohort the value of the 12-lead electrocardiogram (ECG) during antidromic tachycardia (ADT) to predict the site of ablation. METHODS AND RESULTS: Forty-five patients were studied, 23 males (51%), mean age of 27 ± 12 years with 46 AF pathways and 48 ADT using the AF pathway for A-V conduction. Inclusion required induction of a sustained ADT and successful ablation. Ablation site was assessed during LAO 45° projection and clockwise classified as hours in posteroseptal, posterolateral, lateral, anterolateral, and anteroseptal tricuspid annulus as follows: 05:00-07:00, >07:00-08:00, >08:00-09:00, >09:00-11:00, and >11:00-13:00 o'clock. The QRS axis was assessed during ADT and classified as normal (>+15°), horizontal (+15° to -30°), and superior (<-30°). During ADT axis was superior (-57° ± 10°) in 15 (31%), horizontal (-11° ± 14°) in 22 (46%), and normal (+45° ± 16°) in 11 (23%) patients. The correct ablation site did not differ between the different groups of QRS axis. QRS width during ADT was narrower in patients with a normal when compared with a horizontal and leftward axis (127 ± 14 vs. 145 ± 12 ms, P < 0.0001), and the V-H interval was shorter (4 ± 3 ms vs. 19 ± 22 ms, P = 0.03). CONCLUSIONS: There was no correlation between the AF pathway ablation site and the QRS axis during ADT. The 12-lead ECG during maximal pre-excitation does not predict the proper site of tricuspid annulus ablation in patients with A-V conduction over an AF pathway.


Subject(s)
Accessory Atrioventricular Bundle/diagnosis , Accessory Atrioventricular Bundle/surgery , Atrioventricular Node/surgery , Catheter Ablation , Electrocardiography , Tachycardia/diagnosis , Tachycardia/surgery , Accessory Atrioventricular Bundle/physiopathology , Adolescent , Adult , Atrioventricular Node/physiopathology , Brazil , Child , Child, Preschool , Female , Heart Rate , Humans , India , Male , Middle Aged , Netherlands , Predictive Value of Tests , Retrospective Studies , Tachycardia/physiopathology , Young Adult
11.
Heart Rhythm ; 9(6): 850-8, 2012 Jun.
Article in English | MEDLINE | ID: mdl-22338671

ABSTRACT

BACKGROUND: The electrophysiologic characteristics of decrementally conducting accessory pathways (APs) are well described; however, little is known about decrementally conducting APs caused by the radiofrequency ablation of a rapidly conducting AP. OBJECTIVE: To report the clinical, electrocardiographic, and electrophysiologic characteristics of 6 patients who developed a decremental AP after an attempt at ablation. METHODS: We compared the clinical and electrophysiologic characteristics of 295 consecutive patients with the Wolff-Parkinson-White syndrome who underwent radiofrequency ablation of 311 manifest APs (group A) with those of 6 patients with the Wolff-Parkinson-White syndrome in whom a decrementally conducting AP was detected after an attempt at ablation. RESULTS: The AP ablation site in group B patients was at the coronary sinus ostium region in 3 patients, middle cardiac vein in 2 patients, and left posteroseptal region in 1 patient. Sixty-two bypass tracts in group A patients and all 6 in group B patients were ablated at these locations, while 249 bypass tracts in group A patients and none in group B patients were ablated elsewhere (P = .0001). Five of the 6 patients (83%) with acquired Mahaim physiology had an AP located in the venous system. The odds for developing an acquired decremental antegrade atrioventricular AP when it was located inside the venous system were 1 in 6. All group B decremental APs were sensitive to adenosine, but none in 85 group A patients (P <.0001). CONCLUSIONS: The risk for developing decremental conduction after the ablation of a rapidly conducting AP is greater for APs inside the coronary venous system. Acquired decremental antegrade atrioventricular APs are electrophysiologically similar to de novo ones. They are capable of being part of an arrhythmia circuit and, therefore, should be targeted for ablation.


Subject(s)
Accessory Atrioventricular Bundle/physiopathology , Catheter Ablation/adverse effects , Electrocardiography/methods , Electrophysiological Phenomena/physiology , Pre-Excitation, Mahaim-Type/etiology , Wolff-Parkinson-White Syndrome/surgery , Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , Follow-Up Studies , Humans , Male , Middle Aged , Postoperative Complications , Pre-Excitation, Mahaim-Type/diagnosis , Pre-Excitation, Mahaim-Type/physiopathology , Prognosis , Retrospective Studies , Young Adult
12.
Heart Rhythm ; 8(1): 58-64, 2011 Jan.
Article in English | MEDLINE | ID: mdl-20888928

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 <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.


Subject(s)
AMP-Activated Protein Kinases/genetics , Accessory Atrioventricular Bundle/genetics , Accessory Atrioventricular Bundle/diagnosis , Accessory Atrioventricular Bundle/epidemiology , Accessory Atrioventricular Bundle/physiopathology , Adult , Comorbidity , Electrocardiography , Electrophysiologic Techniques, Cardiac , Female , Glycogen Storage Disease Type IIb/epidemiology , Humans , Hypertrophy, Left Ventricular/epidemiology , Hypertrophy, Left Ventricular/genetics , Male , Middle Aged , Mutation, Missense , Prognosis , Retrospective Studies , Wolff-Parkinson-White Syndrome/genetics , Young Adult
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