MCID: ERL052
MIFTS: 22

Early Repolarization Associated with Ventricular Fibrillation

Aliases & Classifications for Early Repolarization Associated with Ventricular Fibrillation

MalaCards integrated aliases for Early Repolarization Associated with Ventricular Fibrillation:

Name: Early Repolarization Associated with Ventricular Fibrillation 57 29 6 71
Early Repolarization Syndrome 57

External Ids:

OMIM® 57 613601
MedGen 41 C3150852
UMLS 71 C3150852

Summaries for Early Repolarization Associated with Ventricular Fibrillation

OMIM® : 57 Electrocardiographic (ECG) early repolarization, defined as an elevation of the QRS-ST junction (J-point) of at least 1.0 mm (0.1 mV) from baseline in the inferior or lateral lead, manifest as QRS slurring or notching, is a common ECG finding that is generally considered to be benign but may be associated with ventricular fibrillation in some patients (summary by Haissaguerre et al., 2008). (613601) (Updated 05-Mar-2021)

MalaCards based summary : Early Repolarization Associated with Ventricular Fibrillation, also known as early repolarization syndrome, is related to ventricular fibrillation, paroxysmal familial, 1 and brugada syndrome. An important gene associated with Early Repolarization Associated with Ventricular Fibrillation is CACNB2 (Calcium Voltage-Gated Channel Auxiliary Subunit Beta 2). Affiliated tissues include heart and cardiac myocytes.

Related Diseases for Early Repolarization Associated with Ventricular Fibrillation

Diseases related to Early Repolarization Associated with Ventricular Fibrillation via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 20)
# Related Disease Score Top Affiliating Genes
1 ventricular fibrillation, paroxysmal familial, 1 10.7
2 brugada syndrome 10.6
3 cardiac conduction defect 10.5
4 syncope 10.4
5 cardiac arrest 10.3
6 progressive familial heart block, type ia 10.2
7 progressive familial heart block, type ib 10.2
8 right bundle branch block 10.2
9 pericarditis 10.1
10 arteries, anomalies of 10.1
11 cardiac arrhythmia 10.1
12 myocardial infarction 10.1
13 lipoprotein quantitative trait locus 10.1
14 fibrosis of extraocular muscles, congenital, 1 9.9
15 cantu syndrome 9.9
16 atrioventricular block 9.9
17 hypertrophic cardiomyopathy 9.9
18 ischemia 9.9
19 hypokalemia 9.9
20 acute disseminated encephalomyelitis 9.9

Graphical network of the top 20 diseases related to Early Repolarization Associated with Ventricular Fibrillation:



Diseases related to Early Repolarization Associated with Ventricular Fibrillation

Symptoms & Phenotypes for Early Repolarization Associated with Ventricular Fibrillation

Clinical features from OMIM®:

613601 (Updated 05-Mar-2021)

Drugs & Therapeutics for Early Repolarization Associated with Ventricular Fibrillation

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Early Repolarization Syndrome: Define the Risk, Stratify the Coverage and Understand the Causes - Clinical and Genetic Study Unknown status NCT02847039
2 China Inherited Ventricular Arrhythmias Registry, a Multicenter, Observational and Prospective Study Recruiting NCT03880708
3 Mapping of Ventricular Fibrillation (VF) Substrates and VF Arrhythmogenic Sources/Reentrant Circuits in Patients With Brugada and Early Repolarization Syndromes Recruiting NCT03764592
4 Derivation of Human Induced Pluripotent Stem (iPS) Cells to Heritable Cardiac Arrhythmias (Long QT Syndrome, Brugada Syndrome, CPVT and Early Repolarization Syndrome) Enrolling by invitation NCT02413450

Search NIH Clinical Center for Early Repolarization Associated with Ventricular Fibrillation

Genetic Tests for Early Repolarization Associated with Ventricular Fibrillation

Genetic tests related to Early Repolarization Associated with Ventricular Fibrillation:

# Genetic test Affiliating Genes
1 Early Repolarization Associated with Ventricular Fibrillation 29

Anatomical Context for Early Repolarization Associated with Ventricular Fibrillation

MalaCards organs/tissues related to Early Repolarization Associated with Ventricular Fibrillation:

40
Heart, Cardiac Myocytes

Publications for Early Repolarization Associated with Ventricular Fibrillation

Articles related to Early Repolarization Associated with Ventricular Fibrillation:

(show top 50) (show all 195)
# Title Authors PMID Year
1
Sudden cardiac arrest associated with early repolarization. 57
18463377 2008
2
Fibrosis and Conduction Abnormalities as Basis for Overlap of Brugada Syndrome and Early Repolarization Syndrome. 61
33557237 2021
3
Sexual Dimorphisms, Anti-Hormonal Therapy and Cardiac Arrhythmias. 61
33540539 2021
4
Ablation of premature ventricular contraction triggering ventricular fibrillation in a patient with early repolarization syndrome. 61
33263215 2021
5
Management of ventricular electrical storm: a contemporary appraisal. 61
32984880 2020
6
Diagnosis, family screening, and treatment of inherited arrhythmogenic diseases in Europe: results of the European Heart Rhythm Association Survey. 61
33367591 2020
7
Two siblings with early repolarization syndrome: clinical and genetic characterization by whole-exome sequencing. 61
33324992 2020
8
Prognostic significance of a low T/R ratio in Brugada syndrome. 61
33011474 2020
9
Transmural conduction time in an early repolarization syndrome model. 61
32855673 2020
10
Novel Cardiocerebral Channelopathy Associated with a KCND3 V392I Mutation. 61
32921676 2020
11
Electrocardiographic changes in the differentiation of ischemic and non-ischemic ST elevation. 61
31885293 2020
12
A novel mutation in GPD1‑L associated with early repolarization syndrome via modulation of cardiomyocyte fast sodium currents. 61
31922248 2020
13
The many faces of early repolarization syndrome: A single-center case series. 61
31521808 2020
14
[Elite athletes' electrocardiogram in Bobo-Dioulasso, Burkina Faso]. 61
33193973 2020
15
Susceptibility to Ventricular Arrhythmias Resulting from Mutations in FKBP1B, PXDNL, and SCN9A Evaluated in hiPSC Cardiomyocytes. 61
32952569 2020
16
Transient high-degree right bundle branch block masking the type 1 Brugada ECG pattern associated with possible transient early repolarization syndrome. 61
31343106 2020
17
A novel DPP6 variant in Chinese families causes early repolarization syndrome. 61
31476289 2019
18
A de novo gain-of-function KCND3 mutation in early repolarization syndrome. 61
31173922 2019
19
Tpeak-tend interval as a marker of arrhythmic risk in early repolarization syndrome. 61
31432584 2019
20
Experimental verification of the value of the Tpeak -Tend interval in ventricular arrhythmia inducibility in an early repolarization syndrome model. 61
31424133 2019
21
Mapping and Ablation of Ventricular Fibrillation Associated With Early Repolarization Syndrome. 61
31542949 2019
22
Successful treatment of electrical storm in a child with early repolarization syndrome with orciprenaline and radiofrequency ablation. 61
31375645 2019
23
Quinidine-A legacy within the modern era of antiarrhythmic therapy. 61
31026503 2019
24
Early repolarization and risk of lone atrial fibrillation. 61
30661277 2019
25
Revelation of early repolarization by eliminating accessory pathway in manifest Wolff-Parkinson-White syndrome: A case report. 61
31007798 2019
26
Prevalence of early repolarization syndrome and long-term clinical outcome in patients with the diagnosis of idiopathic ventricular fibrillation. 61
30288567 2019
27
Challenge and Impact of Quinidine Access in Sudden Death Syndromes: A National Experience. 61
30898241 2019
28
A Slower Heart Rate and Therapeutic Hypothermia Unmasked Early Repolarization Syndrome in a Ventricular Fibrillation Survivor. 61
30464125 2019
29
Sudden cardiac arrest in patients without overt heart disease: a limited value of next generation sequencing. 61
30403391 2018
30
SCN1Bβ mutations that affect their association with Kv4.3 underlie early repolarization syndrome. 61
30160358 2018
31
Control of voltage-driven instabilities in cardiac myocytes with memory. 61
30501225 2018
32
Criteria for ECG detection of acute myocardial ischemia: Sensitivity versus specificity. 61
30177366 2018
33
Update of the Diagnostic Criteria of J-Wave Syndrome: New Concepts and Their Relevance to Cardiology Practice (According to Materials of J-Wave Syndromes Expert Consensus Conference Report: Emerging Concepts and Gaps in Knowledge (2016). 61
30625077 2018
34
[FEATURES OF EARLY REPOLARIZATION SYNDROME DEPENDING ON EVIDENCES OF HEART HYPERTROPHY IN PROFESSIONAL ATHLETES]. 61
30516497 2018
35
Epicardial Substrate as a Target for Radiofrequency Ablation in an Experimental Model of Early Repolarization Syndrome. 61
30354293 2018
36
Sudden cardiac death in J wave syndrome with short QT associated to a novel mutation in Nav 1.8 coding gene SCN10A: First case report for a possible pharmacogenomic role. 61
30177317 2018
37
Repetitive-incessant electrical storm triggered by early repolarization. 61
29205672 2018
38
High rate of subcutaneous implantable cardioverter-defibrillator sensing screening failure in patients with Brugada syndrome: a comparison with other inherited primary arrhythmia syndromes. 61
28340026 2018
39
Ventricular Fibrillation Due to Early Repolarization Syndrome in the Wake of Hypothermia Due to Fulminant Acute Disseminated Encephalomyelitis. 61
28931785 2018
40
Ventricular Fibrillation Associated With Dynamic Changes in J-Point Elevation in a Patient With Silent Thyroiditis. 61
29383332 2018
41
Significance of Coronary Artery Spasm Diagnosis in Patients With Early Repolarization Syndrome. 61
29436392 2018
42
Epicardial ablation utilizing remote magnetic navigation in a patient with Brugada syndrome and inferior early repolarization. 61
28842979 2018
43
Mutation in KCNE1 associated to early repolarization syndrome by modulation of slowly activating delayed rectifier K+ current. 61
29395134 2018
44
The Role of Quinidine in the Pharmacological Therapy of Ventricular Arrhythmias 'Quinidine'. 61
28685701 2018
45
A Heterozygous Missense hERG Mutation Associated with Early Repolarization Syndrome. 61
30481776 2018
46
Early Repolarization Syndrome: Diagnostic and Therapeutic Approach. 61
30542653 2018
47
Electrophysiological Basis for Early Repolarization Syndrome. 61
30460246 2018
48
Early repolarization syndrome caused by de novo duplication of KCND3 detected by next-generation sequencing. 61
29296579 2017
49
Cardiac action potential repolarization revisited: early repolarization shows all-or-none behaviour. 61
28815597 2017
50
Intracoronary acetylcholine application as a possible probe inducing J waves in patients with early repolarization syndrome. 61
29021844 2017

Variations for Early Repolarization Associated with Ventricular Fibrillation

ClinVar genetic disease variations for Early Repolarization Associated with Ventricular Fibrillation:

6
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 CACNB2 NM_201596.3(CACNB2):c.641G>C (p.Ser214Thr) SNV Uncertain significance 161211 rs149253719 10:18795447-18795447 10:18506518-18506518

Expression for Early Repolarization Associated with Ventricular Fibrillation

Search GEO for disease gene expression data for Early Repolarization Associated with Ventricular Fibrillation.

Pathways for Early Repolarization Associated with Ventricular Fibrillation

GO Terms for Early Repolarization Associated with Ventricular Fibrillation

Sources for Early Repolarization Associated with Ventricular Fibrillation

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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