A Slower Heart Rate and Therapeutic Hypothermia Unmasked Early Repolarization Syndrome in a Ventricular Fibrillation Survivor

Hasegawa, Kanae; Miyazaki, Shinsuke; Morishita, Tetsuji; Kaseno, Kenichi; Hisazaki, Kaori; Amaya, Naoki; Uzui, Hiroyasu; Tada, Hiroshi

doi:10.1536/ihj.18-024

Cited by 5 publications

(8 citation statements)

References 15 publications

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“…3) Regarding the cardiac phenotypes of our cases, ERS (increased J-wave amplitudes at a lower heart rate) and PAF (which predominantly occurs at night and early morning) can be explained by an increase of Kv4.3 in the ventricle and atrium, respectively, due to an increased current density and delayed inactivation. 3,4,14) In contrast, decreased J-wave amplitudes at a higher heart rate can be explained by a decrease of the increased Kv 4.3 due to a delayed recovery from inactivation in the ventricle. In addition, maladaptation of the QT interval to the heart rate can be explained by an increase of Kv4.3 at a lower heart rate and a decrease of the increased Kv4.3 at a higher heart rate in the ventricle.…”

Section: Discussionmentioning

confidence: 98%

Novel Cardiocerebral Channelopathy Associated with a <i>KCND3</i> V392I Mutation

et al. 2020

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While a KCND3 V392I mutation uniquely displays a mixed electrophysiological phenotype of Kv4.3, only limited clinical information on the mutation carriers is available. We report two teenage siblings exhibiting both cardiac (early repolarization syndrome and paroxysmal atrial fibrillation) and cerebral phenotypes (epilepsy and intellectual disability), in whom we identified the KCND3 V392I mutation. We propose a link between the KCND3 mutation with a mixed electrophysiological phenotype and cardiocerebral phenotypes, which may be defined as a novel cardiocerebral channelopathy.

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Section: Discussionmentioning

confidence: 98%

Novel Cardiocerebral Channelopathy Associated with a <i>KCND3</i> V392I Mutation

et al. 2020

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“…Nevertheless, it is possible that some myocardial damage in the preparation may have affected the results of this study. Finally, any correlation between hypothermic VA and Osborn wave (J-wave) was undetermined, 2,5,26) because we used a wedge preparation model and applied ventricular pacing.…”

Section: Discussionmentioning

confidence: 99%

“…1,2) On the other hand, hypothermic therapy using mild-cooling has often been successfully applied to patients after cardiopulmonary resuscitation in order to improve neurological outcomes, with infrequent VA events during the treatment. [3][4][5] These suggest that the arrhythmogenic potential and electrophysiological characteristics of the myocardium alter as the myocardial cooling is progressing, but this subject has not been well studied in a systematic experimental protocol.…”

mentioning

confidence: 99%

Therapy-Resistant Ventricular Arrhythmias Developed More Often in Advanced Than in Therapeutic Mild Hypothermic Condition

et al. 2019

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Therapy-resistant ventricular arrhythmias can occur during accidental advanced hypothermic conditions. On the other hand, hypothermic therapy using mild cooling has been successfully accomplished with infrequent ventricular arrhythmia events.To further clarify the therapeutic-resistant arrhythmogenic substrate which develops in hypothermic conditions, an experimental study was performed using a perfusion wedge preparation model of porcine ventricle, and electrophysiological characteristics, inducibility of ventricular arrhythmias, and effects of therapeutic interventions were assessed at 3 target temperatures (37, 32 and 28 ).As the myocardial temperature decreased, myocardial contractions and the number of spontaneous beats deceased. Depolarization (QRS width, stimulus-QRS interval) and repolarization (QT interval, ERP) parameters progressively increased, and dispersion of the ventricular repolarization increased. At 28 , VF tended to be inducible more frequently (1/11 at 37 , 1/11 at 32 , and 5/11 hearts at 28 ), and some VFs at 28 required greater defibrillation energy to resume basic rhythm.An advanced but not a mild hypothermic condition had an enhanced arrhythmogenic potential in our model. In the advanced hypothermic condition, VF with relatively prolonged F-F intervals and a greater defibrillation energy were occasionally inducible based on the arrhythmogenic substrate characterized as slowed conduction and prolonged repolarization of the ventricle.

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“…Hasegawa, et al reported that bradycardia and hypothermia may increase the voltage of the J waves in young adult patients surviving aborted sudden cardiac death. 15) Kimura, et al also reported that R-R interval prolongation and being male were independent factors associated with the presence of J waves. 16) We implanted an S-ICD in this patient.…”

Section: Figurementioning

confidence: 95%

Efficacy of a Subcutaneous Implantable Cardioverter Defibrillator in a Child with Early Repolarization Syndrome

et al. 2021

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An early repolarization (ER) pattern or J waves are considered to be a benign finding observed in the healthy population, however, it has been pointed out that the ER pattern seen in the inferolateral leads could be an independent risk factor for fatal arrhythmias. We present a pediatric case in which early repolarization syndrome (ERS) was suspected due to the presence of ER or J waves in the inferior leads, which eventually disappeared after the administration of pilsicainide. During the follow-up period, several fatal ventricular arrhythmias were recorded after implantation of a subcutaneous implantable cardiac defibrillator (S-ICD). This report describes the efficacy of S-ICDs in a child with an ER pattern after aborted sudden cardiac death.

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A Slower Heart Rate and Therapeutic Hypothermia Unmasked Early Repolarization Syndrome in a Ventricular Fibrillation Survivor

Cited by 5 publications

References 15 publications

Novel Cardiocerebral Channelopathy Associated with a <i>KCND3</i> V392I Mutation

Novel Cardiocerebral Channelopathy Associated with a <i>KCND3</i> V392I Mutation

Therapy-Resistant Ventricular Arrhythmias Developed More Often in Advanced Than in Therapeutic Mild Hypothermic Condition

Efficacy of a Subcutaneous Implantable Cardioverter Defibrillator in a Child with Early Repolarization Syndrome

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