Temporal Repolarization Lability in Hypertrophic Cardiomyopathy Caused by β-Myosin Heavy-Chain Gene Mutations

Atiga, Walter L.; Fananapazir, Lameh; McAreavey, Dorothea; Calkins, Hugh; Berger, Ronald D.

doi:10.1161/01.cir.101.11.1237

Cited by 96 publications

(67 citation statements)

References 31 publications

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“…Using the above described QTVI index, elevated variability has been reported in patients with dilated cardiomyopathy and in patients with hypertrophic cardiomyopathy, as compared with age-matched controls [79]. In [71] increased QTV in hypertrophic cardiomyopathy patients was also found using standard time and frequency domain variability indices.…”

Section: ) Qt Adaptation To Hr Changesmentioning

confidence: 90%

Techniques for Ventricular Repolarization Instability Assessment From the ECG

2016

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Abstract-Instabilities in ventricular repolarization have been documented to be tightly linked to arrhythmia vulnerability. Translation of the information contained in the repolarization phase of the ECG into valuable clinical decision-making tools remains challenging. This work aims at providing an overview of the last advances in the proposal and quantification of ECG-derived indices that describe repolarization properties and whose alterations are related with threatening arrhythmogenic conditions. A review of the state-of-the-art is provided, spanning from the electrophysiological basis of ventricular repolarization to its characterization on the surface ECG through a set of temporal and spatial risk markers.Index Terms-Electrophysiological basis of the ECG, ECG waves, ECG intervals, repolarization instabilities, spatial and temporal ventricular repolarization dispersion, cardiac arrhythmias, biophysical modeling of the ECG, ECG signal processing, repolarization risk markers, T wave alternans, QT variability.

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Section: ) Qt Adaptation To Hr Changesmentioning

confidence: 90%

Techniques for Ventricular Repolarization Instability Assessment From the ECG

2016

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“…These results confirm the association between STVMAPD and proarrhythmia, and suggest involvement of the SAC in BVR in the CAVB dog. infusion: 27 vs. 4 [2][3][4][5][6][7][8][9][10][11][12][13] (P=0.07, Figure 6). However, this could not prevent an increase of STVMAPD, from 0.6±0.2 ms to 4.0±3.3 ms (P=0.006), and is similar to the value obtained in the group that received only dofetilide.…”

Section: Streptomycin Abolishes Response Of Bvr To Preload Variation mentioning

confidence: 99%

Beat-to-Beat Variability in Preload Unmasks Latent Risk of Torsade de Pointes in Anesthetized Chronic Atrioventricular Block Dogs

Stams

Oosterhoff

Heijdel

et al. 2016

Circ J

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“…Since cardiac arrest is one of the most plausible mechanisms of unexpected death at young ages and in epileptic patients, [41][42][43] we assessed possible involvement of the Cacna2d2 product in cardiac function. Taking into consideration that ␣2␦-2 may serve as a regulatory subunit of multiple VDCCs, 8 -10 we first identified human heart areas and nervous tissue where ␣2␦-2 is expressed.…”

Section: Premature Death and Cardiac Functionmentioning

confidence: 99%

Cerebellar Ataxia, Seizures, Premature Death, and Cardiac Abnormalities in Mice with Targeted Disruption of the Cacna2d2 Gene

Ivanov

Ward

Tessarollo

et al. 2004

The American Journal of Pathology

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CACNA2D2 is a putative tumor suppressor gene located in the human chromosome 3p21.3 region that shows frequent allelic imbalances in lung, breast, and other cancers. The ␣2␦-2 protein encoded by the gene is a regulatory subunit of voltage-dependent calcium channels and is expressed in brain, heart, and other tissues. Here we report that mice homozygous for targeted disruption of the Cacna2d2 gene exhibit growth retardation, reduced life span, ataxic gait with apoptosis of cerebellar granule cells followed by Purkinje cell depletion, enhanced susceptibility to seizures, and cardiac abnormalities. The Cacna2d2 tm1NCIF null phenotype has much in common with that of Cacna1a mutants, such as cerebellar neuro-degeneration associated with ataxia, seizures, and premature death. A tendency to bradycardia and limited response of null mutants to isoflurane implicate ␣2␦-2 in sympathetic regulation of cardiac function. In summary, our findings provide genetic evidence that the ␣2␦-2 subunit serves in vivo as a component of P/Q-type calcium channels, is indispensable for the central nervous system function, and may be involved in hereditary cerebellar ataxias and epileptic disorders in humans. (Am J

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Temporal Repolarization Lability in Hypertrophic Cardiomyopathy Caused by β-Myosin Heavy-Chain Gene Mutations

Cited by 96 publications

References 31 publications

Techniques for Ventricular Repolarization Instability Assessment From the ECG

Techniques for Ventricular Repolarization Instability Assessment From the ECG

Beat-to-Beat Variability in Preload Unmasks Latent Risk of Torsade de Pointes in Anesthetized Chronic Atrioventricular Block Dogs

Cerebellar Ataxia, Seizures, Premature Death, and Cardiac Abnormalities in Mice with Targeted Disruption of the Cacna2d2 Gene

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