Electrocardiographic and Electrophysiologic Characteristics in Patients With Brugada Type Electrocardiogram and Inducible Ventricular Fibrillation  Single Center Experience

Ohkubo, Kimie; Watanabe, Ichiro; Takagi, Yasuhiro; Okumura, Yasuo; Ashino, Sonoko; Kofune, Masayoshi; Kawauchi, Kazunori; Yamada, Takeshi; Kofune, Tatsuya; Hashimoto, Kazuhito; Shindo, Atsushi; Sugimura, Hidezou; Nakai, Toshiko; Saito, Satoshi; Hirayama, Atsushi

doi:10.1253/circj.71.1437

Cited by 19 publications

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References 28 publications

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“…37) The electrical abnormality is localized in the free wall of the RVOT, as suggested by the frequent origin or induction of ventricular arrhythmia from this region, [38][39][40][41] and Haissaguerre, et al 42) showed the importance of focal premature ventricular beats, originating mainly from the RVOT, in triggering VF in patients with Brugada syndrome. Several studies based on noninvasive techniques have shown right ventricular abnormalities in patients with the clinical phenotype of Brugada syndrome.…”

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Right Ventricular Histological Substrate and Conduction Delay in Patients With Brugada Syndrome

et al. 2010

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SummaryThe reported pathogenesis of Brugada syndrome is phase 2 reentry resulting from shortening of the epicardial action potential duration at the right ventricular outflow tract (RVOT). However, several studies have revealed a high incidence of ventricular late potentials and high rate of ventricular fibrillation (VF) induced by programmed ventricular stimulation (PVS). The aim of the present study was to evaluate the role of slow conduction at the RVOT for the initiation of VF by PVS and any underlying pathological conditions in Brugada syndrome. Endocardial mapping of the RVOT and endomyocardial biopsy of the right ventricle were performed in 25 patients with Brugada syndrome with inducible VF. Late potentials were positive in 11 of the 25 (44%) patients. Low-amplitude fragmented and delayed electrograms were recorded at the RVOT in 13 of 18 (72.2%) patients. Histologic examination of the biopsy samples revealed fatty tissue infiltration, interstitial fibrosis, lymphocyte infiltration, and/or myocyte disorganization in 13 patients. Slow conduction at the RVOT may contribute to the induction of VF by PVS in Brugada syndrome. Various pathomorphologic changes may contribute to slow conduction at the RVOT. (Int Heart J 2010; 51: 17-23)

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Right Ventricular Histological Substrate and Conduction Delay in Patients With Brugada Syndrome

et al. 2010

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“…2 Programmed electrical stimulation can induce VF, and the His-ventricular (HV) conduction time is prolonged. [3][4][5] The findings are compatible with the abnormality of the cardiac Na channel, and in ~20% of patients with this syndrome, Chen et al found several mutations in the SCN5A that encodes the α-subunit of the voltage-gated cardiac Na channel. 6 The functional assay showed that these mutations resulted in a loss-offunction of the cardiac Na channel.…”

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confidence: 79%

Dynamic Change in ST-Segment and Spontaneous Occurrence of Ventricular Fibrillation in Brugada Syndrome With a Novel Nonsense Mutation in the SCN5A Gene During Long-Term Follow-up

Kawamura

Ozawa

Yao

et al. 2009

Circ J

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A 67-year-old male underwent genetic testing under the diagnosis of Brugada syndrome because of recurrent ventricular fibrillation with coincident ST-segment elevation in either right precordial, inferior leads or both since the age of 55 years. Screening of gene mutations using denaturing high-performance liquid chromatography (DHPLC) and direct sequencing identified a novel nonsense mutation (R179X) of SCN5A in a heterozygous manner. The functional assay for the identified mutation, using a whole-cell patch clamp in the heterologous expression system, revealed that the nonsense mutation, located in the second transmembrane segment of the first domain (DI-S2) of the α-subunit, failed to synthesize the complete structure of the cardiac sodium channel, thus causing the non-functional channel. Coding effects by the gene mutation was altered during the 12-year follow-up, which might affect the clinical features of the patient through the ion channel density in the ventricle, dynamics of repolarization abnormality and conduction disturbance. (Circ J 2009; 73: 584 -588)

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“…[15][16][17] We previously reported that low-amplitude fragmented and delayed potentials were recorded in the RVOT in patients with Brugada syndrome and that these electrograms showed more fractionation and disorganization with programmed ventricular stimulation that led to VF. 14) In addition, several reports have shown a high incidence of ventricular late potentials, 18,19) high rate of induction of ventricular fibrillation (VF) by programmed ventricular stimulation, [20][21][22][23][24] and that inducibility of VF is increased more by programmed stimulation from the RVOT than from the RV apex in Brugada syndrome. 25,26) The aim of this study was to investigate the characteristics of the induced ventricular tachycardia (VT) that degenerates to VF to evaluate whether the RVOT is the arrhythmogenic focus in Brugada syndrome in a retrospective manner.…”

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Surface ECG Characteristics of Ventricular Tachyarrhythmias Before Degeneration Into Ventricular Fibrillation in Patients With Brugada-Type ECG

et al. 2009

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SummaryThis study was designed to evaluate whether the right ventricular outflow tract (RVOT) is the arrhythmogenic focus in Brugada syndrome. We enrolled 45 patients with Brugadatype ECG who underwent programmed ventricular stimulation and inducible ventricular fibrillation (VF). In 25 of these 32 patients, repetitive VT was observed before degeneration into VF. The QRS morphology of surface ECG and intracardiac electrograms were evaluated to determine the origin of the ventricular tachycardia (VT) that degenerated into VF. The VT morphology was a left bundle branch block pattern with an inferior axis in 22 of 28 VTs and the intracardiac conduction sequence during VT revealed activation from the RVOT to the RV apex in these 22 VTs. The majority of the patients with Brugada syndrome showed repetitive VT originating from the RVOT that degenerated into VF. The RVOT may be an arrhythmogenic focus in patients with Brugada syndrome. (Int Heart J 2009; 50: 477-487)

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Electrocardiographic and Electrophysiologic Characteristics in Patients With Brugada Type Electrocardiogram and Inducible Ventricular Fibrillation Single Center Experience

Cited by 19 publications

References 28 publications

Right Ventricular Histological Substrate and Conduction Delay in Patients With Brugada Syndrome

Right Ventricular Histological Substrate and Conduction Delay in Patients With Brugada Syndrome

Dynamic Change in ST-Segment and Spontaneous Occurrence of Ventricular Fibrillation in Brugada Syndrome With a Novel Nonsense Mutation in the SCN5A Gene During Long-Term Follow-up

Surface ECG Characteristics of Ventricular Tachyarrhythmias Before Degeneration Into Ventricular Fibrillation in Patients With Brugada-Type ECG

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