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Early Repolarization Syndrome

EKG Repolarization Early

Early repolarization syndrome (ERS) is a cardiac abnormality, once thought to be benign, that is characterized by J-point elevation. It is associated with fatal arrhythmic conditions and sudden cardiac death.


Presentation

Early repolarization syndrome (ERS) is a condition represented by the electrocardiographic finding of an elevated J-point, indicated by the presence of a notch on the QRS complex, or an unclear transition of the QRS to the ST segment [1]. ERS has been reported to have a higher prevalence in individuals of African or Southeast Asian descent [2]. In addition, males are more frequently affected than females [1]. ERS is also more common in the context of hypertrophic cardiac defects, overactive vagal tone, electrolyte abnormalities (particularly hypercalcemia), cocaine use, athletes, hypothermia, and certain ECG abnormalities [3].

In the past, ERS was considered a benign variation of normal physiology. Subsequent studies showed that it is linked to an increased incidence of various cardiac events, most prominently, idiopathic ventricular fibrillation (VF), even in the absence of cardiac comorbidities. Another major concern in ERS is the increased prevalence of sudden cardiac death (SCD). It is thought that a genetic component, in the form of inheritable mutations, may be involved in the etiology of ERS [4]. An autosomal dominant pattern of inheritance is suggested, with varying degrees of penetrance [5].

ERS can present in two ways, the first being symptomatic ERS. Patients often have arrhythmias and ischemia, where the latter can lead to ventricular fibrillation [6]. Those who have a history of myocardial infarction or syncope are also included in this subgroup [7]. Symptomatic ERS carries a higher risk of cardiac events in the future [6]. The alternative presentation of ERS is an asymptomatic disease that is often found incidentally. The clinical course for such individuals may be benign or consist of further cardiac disease.

Heart Disease
  • METHODS AND RESULTS: Fifty-six patients showing inferolateral ER in the standard ECG and spontaneous ventricular fibrillation (VF) not linked to structural heart disease underwent drug provocation tests by sodium channel blockade with right precordial[ncbi.nlm.nih.gov]
  • We report the case of a 52-year-old man with no structural heart disease whose electrocardiogram showed type 2 ER pattern (with evidence of J-point and ST-segment elevation in electrocardiogram leads II, III, and aVF). The patient presented with VF.[biomedsearch.com]
  • The majority of SCDs are associated with structural heart disease.[e-arrhythmia.org]
  • Subjects with acute or chronic ischemic heart disease and those with cardiac valve disease were not included. Frequency of echocardiographically detected LVFBs, as well as their number, size and location were compared between the two groups.[ingentaconnect.com]
  • Atherosclerosis Risk in Communities (ARIC) Study Investigators Vitelli, LL; Crow, RS; Shahar, E Ventricular fibrillation without apparent heart disease.[deepdyve.com]
Chest Pain
  • (but keep in mild BER can presented chest pain about 25 % of case) 1 Reply Sorry,keep in mind,chest pain about 15% 1 Reply @Gaurav Sarnaik: whts the history? T waves are too tall.. Give the history n get the diagnosis 0 Reply[dailyrounds.org]
  • The presence of S-T elevation in a patient with chest pain of possible cardiac origin mandates hospitalization and cardiac monitoring even if the ECG may confirm to a classic pattern of early repolarization; in this context, the diagnosis of benign early[ncbi.nlm.nih.gov]
  • Similar finding in a patient suffering from an acute chest pain may be much more challenging.[health-tutor.com]
  • pain are due to “normal” variants Hollander, JE; Lozano, M; Fairweather, P Echocardiographic study of the early repolarization syndrome.[deepdyve.com]
  • Benign early repolarization in a 15-year-old male Benign early repolarization also known as early repolarization is found on ECG in about 1% of those with chest pain. [1] It is diagnosed based on an elevated J-point / ST elevation where the ST segment[en.wikipedia.org]
Intracranial Hemorrhage
  • We present the case of a 34-year-old male with cardiac arrest in the context of spontaneous hypothalamic mediated thermal dysregulation after intracranial hemorrhage.[ncbi.nlm.nih.gov]

Workup

ERS is diagnosed via electrocardiography. The main finding is an elevation between the QRS and ST segments of over 1 millimeter above the baseline, as well as ST elevation with prominent T waves in at least two leads [1]. This may appear as slurring of the QRS complex or a notch just before the end of the segment. Both these abnormalities should be positive deflections. It has recently been agreed that ST elevation is not necessary for the diagnosis of ERS, however, in some cases ST elevation is the most obvious abnormality.

In asymptomatic patients, the electrocardiographic tracing is described as an ER pattern. ERS is diagnosed in those who have experienced symptoms due to serious arrhythmias such as ventricular fibrillation or ventricular tachycardia [8]. Patients diagnosed with ER pattern have a tripled risk of VF compared to the general population.

ERS is part of a spectrum of "J wave syndromes" which also include Brugada syndrome [9]. Events such as ST-segment elevation myocardial infarction (STEMI) and hypothermia are also known to produce a similar ECG pattern [10]. The above-mentioned disorders should be excluded as possible causes.

The degree of J-point elevation may correlate to the prognosis [11]. In addition, a descending ST segment is a poor prognostic indicator [12].

Testosterone Decreased
  • It is also true that with age testosterone decreases and so does the incidence of benign ERS.[medhelp.org]
ST Elevation
  • It has recently been agreed that ST elevation is not necessary for the diagnosis of ERS, however, in some cases ST elevation is the most obvious abnormality. In asymptomatic patients, the electrocardiographic tracing is described as an ER pattern.[symptoma.com]
  • Recent studies suggest that the electrocardiographic (ECG) finding of J-point ST-elevation, the early repolarization syndrome, is not as benign as earlier believed.[ncbi.nlm.nih.gov]
  • @article{abc472c2ed9242e396c95b889f35816c, title "Author reply to letter to the editor: Silent and malignant early repolarization syndrome mimicking hyper-acute ST elevation myocardial infarction", keywords "Early repolarization, Idiopathic ventricular[tmu.pure.elsevier.com]
  • Methods: We studied 67 subjects (23 with infero-lateral, horizontal ST elevation ERS- HST, 21 with infero-lateral, ascendant ST elevation ERS – AST, and 23 normals – C, with similar age and sex), by 2D and 3D echo, tissue Doppler, and speckle tracking[romanianjournalcardiology.ro]
  • The definition of ER pattern associated with sudden cardiac death was the presence of J point elevation 0.1mV in at least 2 contiguous inferior and/or lateral leads of a standard 12-lead ECG and not ST elevation as it was often the case in the studies[babymhospital.org]
Early Repolarization
  • The presence of "early repolarization" does not preclude diagnosis of exercise-induced myocardial ischemia by treadmill testing, and coronary vaso-occlusive lesions may be demonstrated in some patients with the early repolarization pattern on ECG.[ncbi.nlm.nih.gov]
  • Although early repolarization syndrome is considered a benign entity, its arrhythmogenic potential still remains unknown.[ncbi.nlm.nih.gov]
  • Early repolarization syndrome (ERS) was previously considered as a benign variant, but it has recently emerged as a risk marker for idiopathic ventricular fibrillation (VF) and sudden death.[ncbi.nlm.nih.gov]
  • Patients presenting with aborted cardiac arrest who display early repolarization generally are diagnosed with early repolarization syndrome.[ncbi.nlm.nih.gov]
  • The early repolarization (ER) pattern on ECG was originally described in the context of hypothermia.[ncbi.nlm.nih.gov]
Peaking T Wave
  • T waves on the ECG Gwinup, GR Altered ventricular repolarization in central sympathetic dysfunction associated with spinal cord injury Lehman, KG; Shandling, AH; Yusi, AU Unusual RT segment deviation in electrocardiograms of normal persons Chelton, LG[deepdyve.com]
Peaking T Wave
  • T waves on the ECG Gwinup, GR Altered ventricular repolarization in central sympathetic dysfunction associated with spinal cord injury Lehman, KG; Shandling, AH; Yusi, AU Unusual RT segment deviation in electrocardiograms of normal persons Chelton, LG[deepdyve.com]
Ventricular Repolarization Abnormalities
  • The following will be displayed on the cardiogram: the tooth P changes, indicating that the atria are depolarized; QRS complex indicates depolarization of the ventricular myocardium; T wave tells about the features of ventricular repolarization - abnormalities[iliveok.com]

Treatment

  • Treatment with quinidine, an Ito blocker, resulted in a normalization of J waves and suppression of VF. Interestingly, J wave amplitude correlated with the ventricular arrhythmia susceptibility and quinidine levels.[ncbi.nlm.nih.gov]
  • This book delineates the state of the art for the diagnosis and treatment of J wave syndromes and covers basic science, translational and clinical aspects of these syndromes.[amazon.com]
  • Only he can prescribe the necessary treatment.[iliveok.com]
  • Electrophysiologic Techniques, Cardiac Equipment Failure Female Humans Male Middle Aged Republic of Korea Retrospective Studies Seasons* Tachycardia, Ventricular/diagnosis Tachycardia, Ventricular/physiopathology Tachycardia, Ventricular/therapy* Time Factors Treatment[ncbi.nlm.nih.gov]
  • Management and available treatments Early repolarization pattern on ECG does not require any treatment.[ecgwaves.com]

Prognosis

  • The prognosis value of this test remains to be assessed. Copyright 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • The degree of J-point elevation may correlate to the prognosis. In addition, a descending ST segment is a poor prognostic indicator.[symptoma.com]
  • The prognosis value of this test remains to be assessed. Mis à jour le 23 juillet 2014 par (inconnu). Haut de page[umr1087.univ-nantes.fr]
  • Prognosis is favorable in the first case. In the presence of lesions of the ventricles, valves, narrowing of the holes, myocardial ischemia, the risk of complications increases significantly. SRRI can complicate: Ventricular fibrillation.[medicalj-center.info]
  • Evidence Rating Level: 2 (Good) Study Rundown: Early repolarization (ER), defined as the J-wave pattern (also known as QRS slur or QRS notch), is an electrocardiogram (ECG) finding with a heterogeneous definition and an unclear association with long-term prognosis[2minutemedicine.com]

Etiology

  • Early Repolarization Syndrome: Etiology and Therapeutics By Prof. Wataru Shimizu English PDF 2017 (2018 Edition) 108 Pages ISBN : 9811033781 5.91 MB This book presents the latest findings on early repolarization syndrome (ERS).[eb4all.com]
  • Readers will find essential information on ERS with regard to its etiology, epidemiology, clinical diagnosis and manifestation, genetic background, and risk stratification, as well as the relationship between ERS and Brugada syndrome.[geneeskundeboek.nl]
  • It is thought that a genetic component, in the form of inheritable mutations, may be involved in the etiology of ERS. An autosomal dominant pattern of inheritance is suggested, with varying degrees of penetrance.[symptoma.com]

Epidemiology

  • Readers will find essential information on ERS with regard to its etiology, epidemiology, clinical diagnosis and manifestation, genetic background, and risk stratification, as well as the relationship between ERS and Brugada syndrome.[geneeskundeboek.nl]
Sex distribution
Age distribution

Pathophysiology

  • Although clinical findings seem to differentiate the two syndromes, similarities between BS and ERS in terms of response to heart rate, pharmacologic agents, and neuromodulation could suggest a linkage in their pathophysiological mechanism.[ncbi.nlm.nih.gov]
  • The spectral nature of J-wave morphology/distribution and phenotypic transition/overlap suggest a common pathophysiologic background in patients with VEB, BS, and ERS.[ncbi.nlm.nih.gov]
  • While the pathophysiology is not completely understood, it is hypothesized that the J-point elevation is a result of more pronounced phase 1 notch of the epicardial action potential compared to the endocardium resulting in a greater ventricular transmural[omicsonline.org]
  • History, pathophysiology, and clinical significance Gussak, I; Bjerregaard, P; Egan, TM Experimental hypothermia: Respiratory and blood pH changes in relation to cardiac function Osborn, JJ Diagnostic value of ECG abnormalities observed in subjects accidentally[deepdyve.com]
  • Furthermore, the differential response to sodium-channel blocker suggests that ERS and BrS are caused by the different basic pathophysiological mechanisms [ 21 ].[journals.plos.org]

Prevention

  • Arrhythmias, Cardiac/diagnosis Arrhythmias, Cardiac/physiopathology Arrhythmias, Cardiac/therapy* Brugada Syndrome/diagnosis Brugada Syndrome/physiopathology Brugada Syndrome/therapy* Chi-Square Distribution Circadian Rhythm* Death, Sudden, Cardiac/prevention[ncbi.nlm.nih.gov]
  • Prevention The syndrome of early repolarization of the ventricles can not be prevented, since until now it has not been possible to identify the clear cause of its appearance.[iliveok.com]
  • An ICD was implanted in the patient for the secondary prevention of sudden cardiac death (SCD) due to idiopathic VF. Discussion SCD is defined as an unexpected death from a cardiac cause within a short period, generally 1 h from symptom onset.[e-arrhythmia.org]
  • Quinidine in addition to an ICD can be useful for secondary prevention of VF in patients with a diagnosis of ER syndrome. In the setting of resuscitated SCA, cascade/familial screening is needed.[acc.org]
  • […] with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC).[journals.plos.org]

References

Article

  1. Miyazaki S, Shah AJ, Haïssaguerre M. Early repolarization syndrome - a new electrical disorder associated with sudden cardiac death. Circ J. 2010;74(10):2039-2044.
  2. Haruta D, Matsuo K, Tsuneto A, et al. Incidence and prognostic value of early repolarization pattern in the 12-lead electrocardiogram. Circulation. 2011;123(25):2931-2937.
  3. Serra-Grima R, Doñate M, Álvarez-García J, et al. Long-term follow-up of early repolarization pattern in elite athletes. Am J Med. 2015;128(2):192.e1-9.
  4. Nunn LM, Bhar-Amato J, Lowe MD, et al. Prevalence of J-point elevation in sudden arrhythmic death syndrome families. J Am Coll Cardiol. 2011;58(3):286-290.
  5. Noseworthy PA, Tikkanen JT, Porthan K, et al. The early repolarization pattern in the general population: clinical correlates and heritability. J Am Coll Cardiol. 2011;57(22):2284–2289.
  6. Haïssaguerre M, Derval N, Sacher F, et al. Sudden cardiac arrest associated with early repolarization. N Engl J Med. 2008;358(19):2016–2023.
  7. Aizawa Y, Chinushi M, Hasegawa K, et al. Electrical storm in idiopathic ventricular fibrillation is associated with early repolarization. J Am Coll Cardiol. 2013;62(11):1015-1019.
  8. Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013;10(12):1932-1963.
  9. Derval N, Simpson CS, Birnie DH, et al. Prevalence and characteristics of early repolarization in the CASPER registry: cardiac arrest survivors with preserved ejection fraction registry. J Am Coll Cardiol.2011;58(7):722-728.
  10. Li G, Yang L, Cui CC, Sun C, Yan GX. J wave syndromes: a decade of progress. Chin Med J (Engl). 2015;128(7):969–975.
  11. Tikkanen JT, Anttonen O, Junttila MJ, et al. Long-term outcome associated with early repolarization on electrocardiography. N Engl J Med. 2009;361(26):2529-2537.
  12. Tikkanen JT, Junttila MJ, Anttonen O, et al. Early repolarization: electrocardiographic phenotypes associated with favorable long-term outcome. Circulation.2011;123(23):2666-2673.

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Last updated: 2019-06-28 09:44