Malignant Early Repolarization Research Articles

Out of Hospital Cardiac Arrest from Malignant Early Repolarization Syndrome

Out of Hospital Cardiac Arrest from Malignant Early Repolarization Syndrome

Early Repolarization Pattern and Idiopatic Sustained Monomorphic Ventricular Tachycardia: an Infrequent Combination

Malignant early repolarization as cause of sudden death has taken on great importance in recent years. It has been described as an entity capable of producing ventricular arrhythmic events, causing from episodes of syncope to sudden cardiac death. Ventricular fibrillation is the typical arrhythmia in these patients, with no clear relationship to date with idiopathic monomorphic ventricular tachycardia. Electrocardiographic markers related to the development of arrhythmic events in early repolarization syndrome have been described. They seem not only related to the development of ventricular fibrillation, but also to sustained monomorphic ventricular tachycardia, as is the case described in the article.

Identification of malignant early repolarization pattern by late QRS activity in high‐resolution magnetocardiography

BackgroundThe early repolarization pattern (ERP) in electrocardiography (ECG) has been considered as a risk for ventricular fibrillation (VF), but effective methods for identification of malignant ERP are still required. We investigated whether high spatiotemporal resolution 64‐channel magnetocardiography (MCG) would enable distinction between benign and malignant ERPs.MethodsAmong all 2,636 subjects who received MCG in our facility, we identified 116 subjects (43 ± 18 years old, 54% male) with inferior and/or lateral ERP in ECG and without structural heart disease, including 13 survivors of VF (ERP‐VF(+)) and 103 with no history of VF (ERP‐VF(−)). We measured the following MCG parameters in a time‐domain waveform of relative current magnitude: (a) QRS duration (MCG‐QRSD), (b) root‐mean‐square of the last 40 ms (MCG‐RMS40), and (c) low amplitude (<10% of maximal) signal duration (MCG‐LAS).ResultsCompared to ERP‐VF(−), ERP‐VF(+) subjects presented a significantly longer MCG‐QRS (108 ± 24 vs. 91 ± 23 ms, p = .02) and lower MCG‐RMS40 (0.10 ± 0.08 vs. 0.25 ± 0.20, p = .01) but no difference in MCG‐LAS (38 ± 22 vs. 29 ± 23 ms, p = .17). MCG‐QRSD and MCG‐RMS40 showed significantly larger area under the ROC curve compared to J‐peak amplitude in ECG (0.72 and 0.71 vs. 0.50; p = .04 and 0.03). The sensitivity, specificity, and odds ratio for identifying VF(+) based on MCG‐QRSD ≥ 100 ms and MCG‐RMS40 ≤ 0.24 were 69%, 74%, and 6.33 (95% CI, 1.80–22.3), and 92%, 48%, and 10.9 (95% CI, 1.37–86.8), respectively.ConclusionMagnetocardiography is an effective tool to distinguish malignant and benign ERPs.

073_16836-Q1 Night Time Cardiac Arrest in Type Three Malignant Early Repolarization Syndrome: A Case Report

Malignant early repolarization syndrome (MERS) has recently been recognized as a rare arryhthmogenic entity for sudden cardiac death. Once considered a benign finding on ECG, recent studies establish a link between the presence of early repolarization and idiopathic ventricular fibrillation (VF).

Author Reply to Letter to the Editor: Silent and Malignant Early Repolarization Syndrome Mimicking Hyper-Acute ST Elevation Myocardial Infarction.

Author Reply to Letter to the Editor: Silent and Malignant Early Repolarization Syndrome Mimicking Hyper-Acute ST Elevation Myocardial Infarction.

Benign vs malignant inferolateral early repolarization: Focus on the T wave

Inferolateral early repolarization (ER) is highly prevalent and is associated with idiopathic ventricular fibrillation (VF). The purpose of this study was to evaluate the potential role of T-wave parameters to differentiate between malignant and benign ER. We compared the ECGs of patients with ER and VF (n = 92) with control subjects with asymptomatic ER (n = 247). We assessed J-wave amplitude, QTc interval, T-wave/R-wave (T/R) ratio in leads II and V5, and presence of low-amplitude T waves (T-wave amplitude <0.1 mV and <10% of R-wave amplitude in lead I, II, or V4-V6). Compared to controls, the VF group had longer QTc intervals (388 ms vs. 377 ms, P = .001), higher J-wave amplitudes (0.23 mV vs. 0.17 mV, P <.001), higher prevalence of low-amplitude T waves (29% vs. 3%, P <.001), and lower T/R ratio (0.18 vs. 0.30, P <.001). Logistic regression analysis demonstrated that QTc interval (odds ratio [OR] per 10 ms: 1.15, 95% confidence interval [CI} 1.02-1.30), maximal J-wave amplitude (OR per 0.1 mV: 1.68, 95% CI 1.23-2.31), lower T/R ratio (OR per 0.1 unit: 0.62, 95% CI 0.47-0.81), presence of low-amplitude T waves (OR 3.53, 95% CI 1.26-9.88). and presence of J waves in the inferior leads (OR 2.58, 95% CI 1.18-5.65) were associated with malignant ER. Patients with malignant ER have a higher prevalence of low-amplitude T waves, lower T/R ratio (lead II or V5), and longer QTc interval. The combination of these parameters with J-wave amplitude and distribution of J waves may allow for improved identification of malignant ER.

J-wave duration and slope as potential tools to discriminate between benign and malignant early repolarization

Yvonne Cristoforetti, MD, Luigi Biasco, MD, Carla Giustetto, MD, Ole De Backer, MD, PhD, Davide Castagno, MD, Piero Astegiano, MD, Gianpasquale Ganzit, MD, Carlo Gabriele Gribaudo, MD, Marco Moccetti, MD, Fiorenzo Gaita, MD From the Division of Cardiology, Department of Medical Science, University of Turin, Citta della Salute e della Scienza Hospital, Turin, Italy, Fondazione Cardiocentro Ticino, University of Zurich, Lugano, Switzerland, The Heart Centre, Rigshospitalet, Copenhagen, Denmark, and Institute of Sports Medicine, Turin, Italy.

Abstract 11668: High Resolution Magnetocardiography as a Novel Noninvasive Tool to Distinguish Between Benign and Malignant Early Repolarization Pattern

Introduction: Most of the early repolarization patterns (ERP) in electrocardiography (ECG) are benign but some of them are associated with ventricular fibrillation (VF). We evaluated whether or not a high spatio-temporal resolution magnetocardiography (MCG) could non-invasively detect malignant types of ERP. Methods: Sixty four-channel MCG, standard 12-lead ECG, and signal averaged ECG (SAECG) were recorded in 120 patients with inferolateral ERP in ECG without any major structural heart diseases; 13 of them had a history of VF (VF(+)-group) and the remaining 107 had no VF (VF(-)-group). We evaluated the following novel MCG indexes: MCG-QRS (msec), root mean square of terminal 40 msec magnetic field (MCG-RMS) (msec), and the duration under 10% of maximal amplitude (MCG-LAS) (msec) of the highest amplitude channel. Results: The amplitude and distribution of the J-wave, ST-T morphology in ECG, parameters of SAECG were not significantly different, whereas MCG-QRS and MCG-LAS were significantly longer and MCG-RMS was smaller in ERP-VF(+) compared with ERP-VF(-) group (107(SD=24) vs 84(13) msec, P&lt;0.01, 8(22) vs 22(11) msec, P&lt;0.01, 0.10(0.08) vs 0.28(0.19) msec, P&lt;0.01, respectively). In the multivariate logistic regression model, only MCG-QRS remained significant among the MCG indexes and the existing predictors (odds ratio (OR) 1.08, 95%CI 1.01 to1.17). The predictive ability of VF was significantly higher using MCG-QRS when the c-statistic was compared with that of the existing ECG measure (0.82 vs 0.56, P&lt;0.01, Figure). When cut-offs were set by the least squares method at MCG-QRS 100 msec, MCG-RMS 0.24 msec, MCG-LAS 27 msec, corresponding ORs were calculated as 12.7, 95%CI 3.6 to 45.0, 6.1, 95%CI 1.7 to 21.2, 6.2, 95%CI 1.8 to 20.8, respectively. Conclusions: MCG-QRS &gt;100 msec was a simple and effective criterion for prediction of high risk ERP subjects, thus MCG analysis is an useful screening tool to detect malignant ERP out of the numerous benign ones.

Early repolarization pattern: its ECG characteristics, arrhythmogeneity and heritability

Early repolarization (ER) has been accepted as a benign ECG variant for decades. Two seminal studies challenged this notion and have demonstrated that ER pattern is associated with an increased risk of arrhythmic and cardiac mortality in patients with idiopathic ventricular fibrillation (IVF) and in the general population. Recent clinical studies demonstrate its varying impact as an arrhythmogenic substrate on different diseases. For example, in ER syndrome, a primary electrical disease, ER appears as a major arrhythmogenic substrate for development of VF whereas in patients with coronary artery disease, an ER pattern may exist as a silent substrate, increasing the risk of VF during episodes of cardiac ischaemia. Due to the high prevalence of an ER pattern in the general population and a low VF event rate, it remains challenging to differentiate a malignant ER pattern from a benign form. Recent research suggests that a J-wave amplitude of more than 0.1 mV combined with a descending/horizontal ST segment may constitute a malignant ER pattern. Further studies are however necessary to evaluate its prognostic value for cardiac and arrhythmic death in the general population as well as in cases with a malignant ER pattern. While genetic testing has revealed putative causal DNA variants in sporadic cases, the lack of co-segregation with the disease in affected families suggests that ER syndrome is not monogenic but is likely a complex disorder influenced by multiple genetic as well as environmental factors.

Syncope in Hereditary Arrhythmogenic Syndromes

Since the discovery of the first mutation causing long QT syndrome (LQTS) in 1995, the field of hereditary arrhythmogenic syndromes has expanded greatly. Today, these syndromes include LQTS, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, and short QT syndrome. There is also evidence suggesting that the newly described malignant early repolarization syndrome also has a genetic cause.

Early repolarization with horizontal ST segment may be associated with aborted sudden cardiac arrest: a retrospective case control study

BackgroundRisk stratification of the early repolarization pattern (ERP) is needed to identify malignant early repolarization. J-point elevation with a horizontal ST segment was recently suggested as a malignant feature of the ERP. In this study, the prevalence of the ERP with a horizontal ST segment was examined among survivors of sudden cardiac arrest (SCA) without structural heart disease to evaluate the value of ST-segment morphology in risk stratification of the ERP.MethodsWe reviewed the data of 83 survivors of SCA who were admitted from August 2005 to August 2010. Among them, 25 subjects without structural heart disease were included. The control group comprised 60 healthy subjects who visited our health promotion center; all control subjects were matched for age, sex, and underlying disease (diabetes mellitus, hypertension). Early repolarization was defined as an elevation of the J point of at least 0.1 mV above the baseline in at least two continuous inferior or lateral leads that manifested as QRS slurring or notching. An ST-segment pattern of <0.1 mV within 100 ms after the J point was defined as a horizontal ST segment.ResultsThe SCA group included 17 men (64%) with a mean age of 49.7 ± 14.5 years. The corrected QTc was not significantly different between the SCA and control groups (432.7 ± 37.96 vs. 420.4 ± 26.3, respectively; p = 0.089). The prevalence of ERP was not statistically different between the SCA and control groups (5/25, 20% vs. 4/60, 6.7%, respectively; p = 0.116). The prevalence of early repolarization with a horizontal ST segment was more frequent in the SCA than in the control group (20% vs. 3.3%, respectively; p = 0.021). Four SCA subjects (16%) and one control subject (1.7%) had a J-point elevation of >2 mm (p = 0.025). Four SCA subjects (16%) and one (1.7%) control subject had an ERP in the inferior lead (p = 0.025).ConclusionThe prevalence of ERP with a horizontal ST segment was higher in patients with aborted SCA than in matched controls. This result suggests that ST morphology has value in the recognition of malignant early repolarization.

Distinguishing “benign” from “malignant early repolarization”: The value of the ST-segment morphology

Means for distinguishing the very common "benign early repolarization" from the very rare but malignant form are needed. Recently, the presence of early repolarization with "horizontal ST segment" was found to predict arrhythmic death during long-term follow-up in a large population study. We therefore speculated that the combination of "J waves with horizontal ST segment" would correlate with a history of idiopathic ventricular fibrillation (VF) better than the mere presence of J waves. To determine whether the morphology of the ST segment adds diagnostic value to the mere presence of J waves in a case-control series of idiopathic VF. We reanalyzed our case-control study showing that the presence of J waves strongly correlates with a history of idiopathic VF among 45 patients with this disorder, 124 controls matched for age and gender ("matched-control" group), and 121 young athletes. This time we focused only on those patients with J waves and graded their ST-segment morphology as either "horizontal" or "ascending" according to predefined criteria. The presence of J waves was associated with a history of idiopathic VF with an odds ratio of 4.0 (95% confidence intervals = 2.0-7.9), but having both J waves and horizontal ST segment yielded an odds ratio of 13.8 (95% confidence intervals = 5.1-37.2) for having idiopathic VF. We report, for the first time, that the combination of J waves with horizontal/descending ST segment improved our ability to distinguish patients with idiopathic VF from controls matched by gender and age.

A familial case of malignant early repolarization

Article history: Received 13 July 2010 Accepted 26 September 2010 Available online 20 October 2010 proclivity to life-threatening tachyarrhythmia [3]. The p findings have suggested that genetic variant encoding ion underlies prominent J wave [4]. However, the sporadic case by anecdotal literatures cannot signify the role of h prevalence of early repolarization associated with a hi catastrophic arrhythmia. The first-time reported familial cas

Ability of Terminal QRS Notching to Distinguish Benign from Malignant Electrocardiographic Forms of Early Repolarization

Recent studies have suggested that early repolarization (ER) might be associated with up to 1/3 of idiopathic ventricular tachycardia/ventricular fibrillation (VT/VF) cases ("malignant" ER). We sought to identify electrocardiographic features to distinguish benign from malignant variants of ER. We reviewed the medical records for implantable-cardioverter defibrillators implanted at a single institution (1988 to 2008) to identify cases of idiopathic VT/VF. The electrocardiograms were scored for ER, defined as a >or=0.1-mV elevation of the QRS-ST junction manifesting as J-point slurring or notching in 2 contiguous leads. We also identified a cohort of 200 healthy age- and gender-matched controls with electrocardiographic findings previously identified as normal ER ("benign" ER cohort). Of 1,224 consecutive implantable-cardioverter defibrillator implants, we identified 39 cases of idiopathic VT/VF. Of the 39 cases, 9 (23%) demonstrated ER. During a mean follow-up of 7.2 +/- 4.6 years, the combined end point of appropriate implantable-cardioverter defibrillator shocks or all-cause mortality occurred less frequently in cases of idiopathic VT/VF with ER than in those without ER (11% vs 30%, odds ratio 0.29, 95% confidence interval 0.03 to 2.69, p = 0.40). A comparison of the electrocardiograms between those with malignant ER and controls demonstrated that QRS notching was significantly more prevalent among cases when present in leads V4 (44% vs 5%, p = 0.001) and V5 (44% vs 8%, p = 0.006), with a similar trend in lead V6 (33% vs 5%, p = 0.013). In conclusion, left precordial terminal QRS notching is more prevalent in malignant variants of ER than in benign cases. These findings could have important implications for risk stratification of patients with ER.