Early repolarization pattern and syndrome — norm or pathology?

2020 ◽  
pp. 38-41
Author(s):  
A. L. Bobrov
Keyword(s):  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Structural Heart Disease ◽  
Polymorphic Ventricular Tachycardia ◽  
Early Repolarization ◽  
Multicenter Studies ◽  
St Segment ◽  
Increased Risk ◽  
Ecg Leads ◽  
J Wave

The review article is devoted to the diagnosis and clinical significance of early ventricular repolarization phenomenon and syndrome. Just 13 years ago, the phenomenon was recognized as an unambiguous version of the norm. However, the results of a series of multicenter studies have shown that the phenomenon is associated with an increased risk of sudden cardiac death. The following criteria are recognized as criteria for early repolarization: the presence of a notch or a junction wave on the descending part of the R wave with a concomitant (or absent) elevation of the ST segment (at the Jt point); J wave (point) ≥0.1mV peak elevation (at Jp point) in ≥2 adjacent 12-channel ECG leads, except for V1–3 leads; QRS duration, measured in leads with J wave (point) <120 ms. Early repolarization syndrome is a clinical condition involving a combination of the pattern of early repolarization and polymorphic ventricular tachycardia, ventricular fibrillation and/or sudden cardiac death in persons without structural heart disease. Treatment is required in patients with a symptom of ventricular tacharrhythmia or family history early repolarization with sudden cardiac death.

scholarly journals The Early Repolarization ECG Pattern – An Update

2017 ◽  
Vol 63 (4) ◽  
pp. 165-169
Author(s):  
István Adorján Szabó ◽  
Annamária Fárr ◽  
Ildikó Kocsis ◽  
Lehel Máthé ◽  
László Szilágyi ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Ventricular Fibrillation ◽  
Cardiac Death ◽  
Epidemiological Data ◽  
Early Repolarization ◽  
Future Directions ◽  
Idiopathic Ventricular Fibrillation ◽  
Increased Risk ◽  
Long Time ◽  
J Wave

AbstractEarly repolarization pattern (ERP), a form of J-wave syndromes, was considered long time a benign ECG phenomenon. However, recent data confirmed that certain phenotypes of ERP are related to an increased risk of sudden cardiac death (idiopathic ventricular fibrillation). Our paper gives a short and practical update regarding the main issues related to ERP: epidemiological data, molecular and electrophysiological background, clinical significance and risk stratification. At the end, the future directions of research and clinical management related to ERP are presented.

scholarly journals Integrin β1D Deficiency–Mediated RyR2 Dysfunction Contributes to Catecholamine-Sensitive Ventricular Tachycardia in Arrhythmogenic Right Ventricular Cardiomyopathy

Circulation ◽  
2020 ◽  
Vol 141 (18) ◽  
pp. 1477-1493 ◽  
Author(s):  
Yihui Wang ◽  
Chunyan Li ◽  
Ling Shi ◽  
Xiuyu Chen ◽  
Chen Cui ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Polymorphic Ventricular Tachycardia ◽  
Ventricular Cardiomyopathy ◽  
Open Probability ◽  
Increased Risk

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a hereditary heart disease characterized by fatty infiltration, life-threatening arrhythmias, and increased risk of sudden cardiac death. The guideline for management of ARVC in patients is to improve quality of life by reducing arrhythmic symptoms and to prevent sudden cardiac death. However, the mechanism underlying ARVC-associated cardiac arrhythmias remains poorly understood. Methods: Using protein mass spectrometry analyses, we identified that integrin β1 is downregulated in ARVC hearts without changes to Ca 2+ -handling proteins. As adult cardiomyocytes express only the β1D isoform, we generated a cardiac specific β1D knockout mouse model and performed functional imaging and biochemical analyses to determine the consequences of integrin β1D loss on function in the heart in vivo and in vitro. Results: Integrin β1D deficiency and RyR2 Ser-2030 hyperphosphorylation were detected by Western blotting in left ventricular tissues from patients with ARVC but not in patients with ischemic or hypertrophic cardiomyopathy. Using lipid bilayer patch clamp single channel recordings, we found that purified integrin β1D protein could stabilize RyR2 function by decreasing RyR2 open probability, mean open time, and increasing mean close time. Also, β1D knockout mice exhibited normal cardiac function and morphology but presented with catecholamine-sensitive polymorphic ventricular tachycardia, consistent with increased RyR2 Ser-2030 phosphorylation and aberrant Ca 2+ handling in β1D knockout cardiomyocytes. Mechanistically, we revealed that loss of DSP (desmoplakin) induces integrin β1D deficiency in ARVC mediated through an ERK1/2 (extracellular signal–regulated kinase 1 and 2)–fibronectin–ubiquitin/lysosome pathway. Conclusions: Our data suggest that integrin β1D deficiency represents a novel mechanism underlying the increased risk of ventricular arrhythmias in patients with ARVC.

scholarly journals The Role of Flecainide in the Management of Catecholaminergic Polymorphic Ventricular Tachycardia

2016 ◽  
Vol 5 (1) ◽  
pp. 45 ◽  
Author(s):  
Krystien VV Lieve ◽  
◽  
Arthur A Wilde ◽  
Christian van der Werf ◽  
◽  
...  
Keyword(s):  
Heart Disease ◽  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Structural Heart Disease ◽  
Current Data ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Β Blockers

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare but severe genetic cardiac arrhythmia disorder, with symptoms including syncope and sudden cardiac death due to polymorphic VT or ventricular fibrillation typically triggered by exercise or emotions in the absence of structural heart disease. The cornerstone of medical therapy for CPVT is β -blockers. However, recently flecainide has been added to the therapeutic arsenal for CPVT. In this review we summarise current data on the efficacy and role of flecainide in the treatment of CPVT.

scholarly journals Electrocardiographic Changes in Athletes of Black Ethnicity

2018 ◽  
Vol 13 ◽  
Author(s):  
Dillan Ismail ◽  
Anders Eriksson ◽  
Stellan Mörner ◽  
Annika Rydberg
Keyword(s):  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Heart Diseases ◽  
Cardiac Events ◽  
St Segment Elevation ◽  
Positive Effects ◽  
African Heritage ◽  
St Segment ◽  
Increased Risk ◽  
Electrocardiographic Changes

AbstractIntensive participation in sport has positive physiological effects on the heart. The contractility of the heart improves, the ejection fraction increases and the muscle mass of the heart increases, thus leading to a greater cardiac output. Despite these positive effects, there is still an increased risk for acute cardiac events. The workload of the heart can be very high in some sports and may in some cases be the reason for sudden cardiac death. In these cases, there is often an underlying heart disease (cardiomyopathy) unknown before the actual event. Electrocardiographic examination (ECG) may reveal some of these diseases but although ECG examinations can be a useful tool to discover pathological conditions, there could be difficulties in interpreting different ECG patterns, especially in athletes. In some cases, athletes may exhibit ECG patterns that are similar to those in heart diseases such as cardiomyopathies (QRS-amplitudes, ST-segment elevation and T wave inversions in lateral leads).  This pattern is even more common in athletes of African origins. Furthermore, cardiomyopathies such as hypertrophic cardiomyopathy (HCM) are more common among athletes with African heritage than in white athletes. Thus correct interpretation of ECG is crucial for several reasons: to distinguish between benign physiological (‘athlete’s heart’) and pathological changes, to lower the risk of sudden cardiac death, and to save time and money by not undertaking further examination of the heart.

The Role of Flecainide in the Management of Catecholaminergic Polymorphic Ventricular Tachycardia

2016 ◽  
pp. 1a
Author(s):  
Krystien VV Lieve ◽  
Arthur A Wilde ◽  
Christian van der Werf ◽  
◽  
◽  
...  
Keyword(s):  
Heart Disease ◽  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Structural Heart Disease ◽  
Current Data ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Β Blockers

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare but severe genetic cardiac arrhythmia disorder, with symptoms including syncope and sudden cardiac death due to polymorphic VT or ventricular fibrillation typically triggered by exercise or emotions in the absence of structural heart disease. The cornerstone of medical therapy for CPVT is β -blockers. However, recently flecainide has been added to the therapeutic arsenal for CPVT. In this review we summarise current data on the efficacy and role of flecainide in the treatment of CPVT.

scholarly journals Early Repolarization, Fragmented QRS and Tpeak-Tend Interval as Electrocardiographic Markers in Patients with Idiopathic Ventricular Arrhythmias: a Brief Review

2020 ◽  
Vol 33 (3) ◽  
pp. 142-146
Author(s):  
Javier Pinos Vásquez ◽  
Tiago Luiz Luz Leiria ◽  
Clóvis Froemming Jr ◽  
Bruno Schaaf Finkler ◽  
Danilo Barros Zanotta ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Randomized Clinical Trials ◽  
Early Repolarization ◽  
Specific Patient ◽  
Patient Profile ◽  
Published Evidence ◽  
Increased Risk ◽  
Electrocardiographic Markers

Introduction: Idiopathic ventricular tachycardia and ventricular fibrillation, as causes of sudden cardiac death, are entities with mechanisms poorly studied and understood to date. The electrocardiogram (ECG) is a simple tool, but with great diagnostic and prognostic value, which has allowed the identification of certain markers associated with increased risk of development of malignant ventricular arrhythmias and sudden cardiac death. Methods: To identify the electrocardiographic markers related to the risk of developing idiopathic malignant ventricular arrhythmias, a review of the literature was performed, looking for the most recent articles with the greatest scientific impact on the topic. Outcome: Although the number of studies published to date is scarce, the published evidence has shown three electrocardiographic risk markers that have emerged in recent years and which have been related to the development of idiopathic malignant ventricular arrhythmias: the early repolarization (ER) pattern, QRS fragmentation (QRSF) and the Tpeak-Tend (Tp-Te) interval. The ECG marker that has shown most evidence to date is the pattern of ER, as a cause of changes in both ventricular depolarization and repolarization. The QRSF and the Tp-Te interval have also been related to the development of idiopathic ventricular arrhythmias, although with less evidence in this regard. Conclusion: In the last years, three electrocardiographic markers have appeared as variables related to the development of malignant ventricular arrhythmias, as is the case of ER, QRSF and Tp-Te interval. However, evidence is scarce in this specific patient profile and further randomized clinical trials are necessary to demonstrate its true relationship and usefulness.

Abstract 13067: When Are 3 ECG Leads Better Than 12? Streamlining and Optimizing ECG Assessment in Sudden Death Prediction

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Isabelle Ruedisueli ◽  
Joyce Ma ◽  
Karishma Lakhani ◽  
Randy Nguyen ◽  
Jeffrey Gornbein ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Qt Interval ◽  
Cardiac Death ◽  
Qrs Complex ◽  
T Wave ◽  
Maximum Value ◽  
Increased Risk ◽  
Secondary Outcomes ◽  
Ecg Leads ◽  
Better Than

Introduction: Prolonged Tpeak to Tend (Tp-e) interval, an index of repolarization on the 12-lead ECG, is associated with increased risk for sudden cardiac death. However, there is no current consensus on which of the 12 leads is the most sensitive to measure the longest Tp-e interval. Aim: The aim of this study was to measure all 12 ECG leads and to analyze whether there are leads that are most sensitive to detect prolongation of Tp-e in order to optimize methodology for future investigations. Methods: Fifteen healthy volunteers (F/M 6/9; mean age 25 yrs) were included in our study. We recorded the 12-lead ECG for 5-minutes. Tp-e was defined as the interval from the peak of the T wave to the end of the T wave. QT is the interval from QRS complex onset to the end of the T wave. Using commercially available software (AdInstruments), three primary outcomes, Tp-e interval, Tp-e/QT, Tp-e/QTc ratios, and two secondary outcomes, QT, QTc intervals, were determined. Results: The location of maximum value for primary outcomes (Tp-e, Tp-e/QT, Tp-e/QTc) were not evenly distributed across the 12 leads, but most frequently was located in leads V2, V3 & V4. The maximum Tp-e was located in one of these three leads 79.7% of the time (CI 69.4, 89.9%) vs other leads (p=0.007, Figure). Conversely, maximum values for the secondary outcomes (QT, QTc) were located in AVL, AVR and III (Figure). Two of the leads, V5 and V6, never had a maximum value for any outcomes. Conclusion: Preliminary findings in our study suggest that investigators should focus on leads V2, V3 & V4 to detect prolongation of Tp-e intervals and leads AVL, AVR & III for prolongation of QT interval when investigating associations with sudden cardiac death.

Abstract 12925: Combination of Cardiac I-123 Metaiodobenzylguanidine Imaging Findings and Early Repolarization Pattern Improve the Predictive Power of Sudden Cardiac Death in Patients With Chronic Heart Failure

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Atsushi Kikuchi ◽  
Takahisa Yamada ◽  
Takashi Morita ◽  
Yoshio Furukawa ◽  
Shunsuke Tamaki ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sudden Cardiac Death ◽  
Chronic Heart Failure ◽  
Cardiac Death ◽  
Hazard Ratio ◽  
Mibg Imaging ◽  
Imaging Findings ◽  
Prognostic Information ◽  
Early Repolarization ◽  
Increased Risk

Background: Identification of patients with chronic heart failure (CHF) at risk for sudden cardiac death (SCD) is an important objective. Cardiac I-123 metaiodobenzylguanidine (MIBG) imaging findings provide prognostic information in patients with CHF. On the other hand, early repolarization pattern (ERP) is associated with life-threatening arrhythmia events. Moreover, it has been shown that ERP would be associated with an increased risk of SCD in CHF patients. However, there is no information available on the long-term prognostic value of combining cardiac MIBG imaging findings and ERP for the prediction of SCD in CHF patients. Methods: We studied 92 CHF outpatients (NYHA class: 2.1±0.6) with LVEF < 40% [30±7%) in our prospective cohort study. We performed MIBG scintigraphy, and measured the heart-to-mediastinum ratio on the delayed image (HMRd), of which the abnormal value was defined as < 1.7. Furthermore, we obtained the standard 12-lead electrocardiogram at enrollment. ERP was defined as J-point elevation ≤ 0.1 mV in at least 2 inferior or lateral leads. Results: At enrollment, 41 patients had abnormal HMRd and 19 patients had ERP. During a follow-up period of 7.7±4.3 yrs, 22 patients died suddenly. A multivariate Cox analysis revealed that HMRd (p=0.001) and ERP (p=0.017) were significantly and independently associated with SCD. Kaplan-Meier analysis showed that SCD was significantly more frequently observed in patients with both abnormal HMRd and ERP and those with either abnormal HMRd or ERP than those with neither abnormal HMRd nor ERP (55% vs 33% vs 7%, respectively, p<0.0001). The hazard ratio for SCD prediction in patients with both abnormal HMRd and ERP was 14.7 (95%CI 3.6 to 60.1), which was twofold of the hazard ratio in patients with either abnormal HMRd or ERP (HR 6.5 [95%CI 1.8 to 7.8]) Conclusion: The combination of cardiac MIBG imaging and ERP would provide the incremental prognostic information for the prediction of SCD in CHF patients.

scholarly journals Early Repolarization in Pediatric Athletes: A Dynamic Electrocardiographic Pattern With Benign Prognosis

2021 ◽  
Author(s):  
Geza Halasz ◽  
Mattia Cattaneo ◽  
Massimo Piepoli ◽  
Andrea Biagi ◽  
Silvio Romano ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Exclusion Criteria ◽  
Early Repolarization ◽  
St Segment ◽  
Pr Interval ◽  
St Elevation ◽  
Few Data

Background Early repolarization pattern (ERP) is considered a common training‐related and benign ECG finding in young adult athletes. Few data exist on ERP in the pediatric athletes population. Therefore, we aimed to evaluate the ERP prevalence, characteristics, and prognosis in pediatric athletes aged ≤16 years. Methods and Results Eight‐hundred eighty‐six consecutive pediatric athletes engaged in 17 different sports (mean age, 11.7±2.5 years; 7–16 years) were enrolled and prospectively evaluated with medical history, physical examination, resting and exercise ECGs, and transthoracic echocardiography during their preparticipation screening. Known cardiovascular diseases associated with sudden cardiac death was considered exclusion criteria. Athletes were followed up yearly for 4 years. The prevalence of ERP was 117 (13.2%), equally distributed in both sexes ( P =0.072), irrespectively of body mass index and classification of sports. The most common ERP localizations were inferolateral and inferior leads (53.8% and 27.3%, respectively). Notching J‐point morphology was the most prevalent (70%), and rapidly ascending ST elevation (96%) was the most common ST‐segment morphology. Athletes with ERP were older ( P <0.001) had lower rest and recovery heart rates ( P <0.001), increased precordial and limb R‐wave voltages ( P <0.001), increased R/S Sokolow index ( P <0.001), and longer PR interval ( P =0.006) in comparison with the athletes without ERP. Neither major cardiovascular nor arrhythmic events, nor sudden cardiac death were recorded over a median follow‐up of 4.2 years. One hundred seventeen (80.3%) athletes with ERP exhibited a persistent ERP. ERP localization and J‐point morphology changed during follow‐up in 11 (11.7%) and 17 (18%) of athletes, respectively. Conclusions ERP is common in pediatric athletes. It was mostly located in the inferolateral leads and associated with concave ascending ST segment with other training‐related ECG changes. The lack of either sudden cardiac death or cardiomyopathies linked to sudden cardiac death over follow‐up suggests that in pediatric athletes, ERP may be considered a benign training‐related ECG phenomenon with a potential dynamic pattern.

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