Structurally Abnormal Myocardium Underlies Ventricular Fibrillation Storms in a Patient Diagnosed With the Early Repolarization Pattern

Boukens, Bastiaan J.; Benjacholamas, Vichai; Amersfoort, Shirley van; Meijborg, Veronique M.F.; Schumacher, Cees A.; Jensen, Bjarke; Haı̈ssaguerre, Michel; Wilde, Arthur A.M.; Prechawat, Somchai; Huntrakul, Anurut; Nademanee, Koonlawee; Coronel, Ruben

doi:10.1016/j.jacep.2020.06.027

Cited by 18 publications

(26 citation statements)

References 13 publications

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“…Our computer simulation, however, indicates that when deeper penetration of the Purkinje system into the ventricular wall only activates part of the myocardium earlier, it may leave some areas being activated relatively late, which can give rise to notching and slurring at the end of the QRS complex (Figure 1B; Meijborg et al, 2016). This is in line with our recent view on the mechanisms underlying the early repolarization pattern (Boukens et al, 2019(Boukens et al, , 2020Haïssaguerre et al, 2019). The computer model was not tested for arrhythmogenesis.…”

Section: Discussionsupporting

confidence: 81%

Why Ablation of Sites With Purkinje Activation Is Antiarrhythmic: The Interplay Between Fast Activation and Arrhythmogenesis

et al. 2021

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Ablation of sites showing Purkinje activity is antiarrhythmic in some patients with idiopathic ventricular fibrillation (iVF). The mechanism for the therapeutic success of ablation is not fully understood. We propose that deeper penetrance of the Purkinje network allows faster activation of the ventricles and is proarrhythmic in the presence of steep repolarization gradients. Reduction of Purkinje penetrance, or its indirect reducing effect on apparent propagation velocity may be a therapeutic target in patients with iVF.

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Section: Discussionsupporting

confidence: 81%

Why Ablation of Sites With Purkinje Activation Is Antiarrhythmic: The Interplay Between Fast Activation and Arrhythmogenesis

et al. 2021

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“…It is thought that these so‐called J‐wave syndromes share early repolarization at a cellular level as a common mechanism underlying the J‐waves on the ECG and the occurrence of arrhythmias (Antzelevitch et al, 2016). However, recent studies have shown that delayed activation can lead to J‐waves as well (Boukens et al, 2020; Boukens, Opthof and Coronel, 2019; Hoogendijk et al, 2010). This may make the mechanism underlying J‐waves in mammals and birds with high heart rates different from that in human.…”

Section: Discussionmentioning

confidence: 99%

“…Some bird species exhibit a non‐isoelectric ST segment, which could be due to early repolarization. It is known from human studies, however, that multiple phenomena can give rise to positive deflections after the QRS, such as hypothermia or late activation due to structural abnormalities (Boukens et al, 2020). Therefore, action potential recordings are required to confirm early repolarization as an underlying mechanism.…”

Section: Introductionmentioning

confidence: 99%

High heart rate associated early repolarization causes J‐waves in both zebra finch and mouse

et al. 2021

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High heart rates are a feature of small endothermic—or warm‐blooded—mammals and birds. In small mammals, the QT interval is short, and local ventricular recordings reveal early repolarization that coincides with the J‐wave on the ECG, a positive deflection following the QRS complex. Early repolarization contributes to short QT‐intervals thereby enabling brief cardiac cycles and high heart rates. We therefore hypothesized high hearts rates associate with early repolarization and J‐waves on the ECG of endothermic birds. We tested this hypothesis by comparing isolated hearts of zebra finches and mice and recorded pseudo‐ECGs and optical action potentials (zebra finch, n = 8; mouse, n = 8). In both species, heart rate exceeded 300 beats per min, and total ventricular activation was fast (QRS < 10 ms). Ventricular activation progressed from the left to the right ventricle in zebra finch, whereas it progressed from apex‐to‐base in mouse. In both species, the early repolarization front followed the activation front, causing a positive J‐wave in the pseudo‐ECG. Inhibition of early repolarization by 4‐aminopyridine reduced J‐wave amplitude in both species. Action potential duration was similar between ventricles in zebra finch, whereas in mouse the left ventricular action potential was longer. Accordingly, late repolarization had opposite directions in zebra finch (left‐right) and mouse (right‐left). This caused a similar direction for the zebra finch J‐wave and T‐wave, whereas in the mouse they were discordant. Our findings demonstrate that early repolarization and the associated J‐wave may have evolved by convergence in association with high heart rates.

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“…We recently reported the first case of early repolarization syndrome in which localized fibrosis in the right ventricular inferior wall caused ventricular fibrillation and the early repolarization pattern in the ECG [16]. Echocardiography and magnetic resonance imaging (MRI) did not reveal gross structural abnormalities in this patient.…”

Section: Structural Abnormalities and Fractionated Potentialsmentioning

confidence: 78%

“…Sci. 2021, 22, 1570 2 of 10 caused by structural abnormalities led to the early repolarization syndrome [16]. Removal of the region containing the abnormalities cured the syndrome, indicating that abnormal conduction may be the mechanism causing early repolarization syndrome similar to the Brugada syndrome.…”

Section: Introductionmentioning

confidence: 99%

Fibrosis and Conduction Abnormalities as Basis for Overlap of Brugada Syndrome and Early Repolarization Syndrome

Boukens

Potse

Coronel

2021

IJMS

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Brugada syndrome and early repolarization syndrome are both classified as J-wave syndromes, with a similar mechanism of arrhythmogenesis and with the same basis for genesis of the characteristic electrocardiographic features. The Brugada syndrome is now considered a conduction disorder based on subtle structural abnormalities in the right ventricular outflow tract. Recent evidence suggests structural substrate in patients with the early repolarization syndrome as well. We propose a unifying mechanism based on these structural abnormalities explaining both arrhythmogenesis and the electrocardiographic changes. In addition, we speculate that, with increasing technical advances in imaging techniques and their spatial resolution, these syndromes will be reclassified as structural heart diseases or cardiomyopathies.

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Structurally Abnormal Myocardium Underlies Ventricular Fibrillation Storms in a Patient Diagnosed With the Early Repolarization Pattern

Cited by 18 publications

References 13 publications

Why Ablation of Sites With Purkinje Activation Is Antiarrhythmic: The Interplay Between Fast Activation and Arrhythmogenesis

Why Ablation of Sites With Purkinje Activation Is Antiarrhythmic: The Interplay Between Fast Activation and Arrhythmogenesis

High heart rate associated early repolarization causes J‐waves in both zebra finch and mouse

Fibrosis and Conduction Abnormalities as Basis for Overlap of Brugada Syndrome and Early Repolarization Syndrome

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