MCID: ATR081
MIFTS: 28

Atrial Standstill

Categories: Cardiovascular diseases, Genetic diseases, Rare diseases

Aliases & Classifications for Atrial Standstill

MalaCards integrated aliases for Atrial Standstill:

Name: Atrial Standstill 58 71
Cardiomyopathy, Familial, with Conduction Disturbance 71
Atrial Cardiomyopathy with Heart Block 58
Standstill, Atrial 39

Characteristics:

Orphanet epidemiological data:

58
atrial standstill
Inheritance: Autosomal dominant,Not applicable; Age of onset: Adult;

Classifications:



External Ids:

ICD10 via Orphanet 33 I45.5
UMLS via Orphanet 72 C0541782 C1838539
Orphanet 58 ORPHA1344
UMLS 71 C0541782 C1838539

Summaries for Atrial Standstill

MalaCards based summary : Atrial Standstill, also known as cardiomyopathy, familial, with conduction disturbance, is related to atrial standstill 1 and atrial standstill 2. An important gene associated with Atrial Standstill is SCN5A (Sodium Voltage-Gated Channel Alpha Subunit 5), and among its related pathways/superpathways is Antiarrhythmic Pathway, Pharmacodynamics. Affiliated tissues include heart, bone and testes.

Related Diseases for Atrial Standstill

Diseases in the Atrial Standstill family:

Atrial Standstill 1 Atrial Standstill 2

Diseases related to Atrial Standstill via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 100)
# Related Disease Score Top Affiliating Genes
1 atrial standstill 1 33.8 SCN5A NPPA GJA5
2 atrial standstill 2 33.4 NPPA-AS1 NPPA LOC114827827
3 syncope 30.6 SCN5A NPPA
4 progressive familial heart block, type ia 30.4 SCN5A GJA5
5 sinoatrial node disease 30.3 SCN5A GJA5
6 heart septal defect 30.2 NPPA GJA5
7 congestive heart failure 30.0 SCN5A NPPA GJA5
8 sick sinus syndrome 30.0 SCN5A NPPA GJA5
9 ebstein anomaly 30.0 SCN5A GJA5
10 atrial fibrillation 29.7 SCN5A NPPA GJA5
11 dilated cardiomyopathy 29.6 SCN5A NPPA GJA5
12 atrial heart septal defect 29.6 SCN5A NPPA GJA5
13 hypertrophic cardiomyopathy 29.4 SCN5A NPPA GJA5
14 tetralogy of fallot 29.3 SCN5A NPPA GJA5
15 heart disease 29.0 SCN5A NPPA GJA5
16 myocarditis 10.5
17 amyloidosis 10.4
18 tricuspid valve insufficiency 10.4
19 muscular dystrophy 10.4
20 intracranial embolism 10.3
21 progressive familial heart block, type ib 10.3
22 right bundle branch block 10.3
23 mitral valve stenosis 10.3
24 cardiac conduction defect 10.2
25 brugada syndrome 10.2
26 rheumatic heart disease 10.2
27 mitral valve insufficiency 10.2
28 megaloblastic anemia 10.2
29 heart valve disease 10.2
30 myopathy 10.2
31 neuropathy 10.2
32 systemic lupus erythematosus 10.0
33 sarcoidosis 1 10.0
34 thiamine-responsive megaloblastic anemia syndrome 10.0
35 anemia, x-linked, with or without neutropenia and/or platelet abnormalities 10.0
36 cardiomyopathy, dilated, 1e 10.0
37 branchiootic syndrome 1 10.0
38 ventricular fibrillation, paroxysmal familial, 1 10.0
39 sick sinus syndrome 1 10.0
40 atrial fibrillation, familial, 10 10.0
41 deficiency anemia 10.0
42 acute diarrhea 10.0
43 exanthem 10.0
44 autosomal recessive disease 10.0
45 metabolic acidosis 10.0
46 aphasia 10.0
47 stuttering 10.0
48 cardiac arrest 10.0
49 thalassemia 10.0
50 hemopericardium 10.0

Graphical network of the top 20 diseases related to Atrial Standstill:



Diseases related to Atrial Standstill

Symptoms & Phenotypes for Atrial Standstill

Drugs & Therapeutics for Atrial Standstill

Search Clinical Trials , NIH Clinical Center for Atrial Standstill

Genetic Tests for Atrial Standstill

Anatomical Context for Atrial Standstill

MalaCards organs/tissues related to Atrial Standstill:

40
Heart, Bone, Testes

Publications for Atrial Standstill

Articles related to Atrial Standstill:

(show top 50) (show all 224)
# Title Authors PMID Year
1
Autosomal recessive atrial dilated cardiomyopathy with standstill evolution associated with mutation of Natriuretic Peptide Precursor A. 61 6
23275345 2013
2
Familial endemic persistent atrial standstill in a small mountain community: review of eight cases. 61 6
6225642 1983
3
Left Atrial Standstill and Interatrial Block. 61
32359950 2020
4
Isolated Left Atrial Standstill in Patients with Hypertrophic Cardiomyopathy and Atrial Fibrillation after Restoration of Sinus Rhythm. 61
31351793 2019
5
A case of atrial standstill with the atrial lead of a dual-chamber pacemaker implanted in the coronary sinus. 61
31285994 2019
6
Indications for permanent pacing in dogs and cats. 61
30709617 2019
7
Cardiac Involvement in Emery-Dreifuss Muscular Dystrophy and Related Management Strategies. 61
30518714 2019
8
Atrial standstill presenting as cerebral infarction in a 7-year-old girl. 61
30783526 2019
9
Cardiac voltage-gated sodium channel mutations associated with left atrial dysfunction and stroke in children. 61
29579189 2018
10
Idiopathic isolated fibrotic atrial cardiomyopathy underlies unexplained scar-related atrial tachycardia in younger patients. 61
29293999 2018
11
Long-term follow-up of permanent atrial standstill in a German family with mutation in the SCN5A gene. 61
30116708 2018
12
A homozygous SCN5A mutation associated with atrial standstill and sudden death. 61
29781517 2018
13
Limited effects of long-term enzyme replacement therapy on the cardiac conduction system in Fabry disease. 61
30279886 2018
14
Cardiolaminopathies from bench to bedside: challenges in clinical decision-making with focus on arrhythmia-related outcomes. 61
30130999 2018
15
Atrial standstill in a pediatric patient with associated caveolin-3 mutation. 61
29387541 2017
16
Dysfunction of Myosin Light-Chain 4 (MYL4) Leads to Heritable Atrial Cardiomyopathy With Electrical, Contractile, and Structural Components: Evidence From Genetically-Engineered Rats. 61
29080865 2017
17
Progressive Atrial Conduction Defects Associated With Bone Malformation Caused by a Connexin-45 Mutation. 61
28705318 2017
18
Presumptive partial atrial standstill secondary to atrial cardiomyopathy in a Greyhound. 61
28314614 2017
19
Survival time with pacemaker implantation for dogs diagnosed with persistent atrial standstill. 61
28578822 2017
20
[Clinical features and outcomes of radiofrequency catheter ablation of atrial flutter in children]. 61
28441822 2017
21
Persistent atrial standstill following the Cox-maze III procedure: reversal with sustained atrial pacing. 61
27558131 2017
22
Anti-mitochondrial M2 Antibodies Enhance the Risk of Supraventricular Arrhythmias in Patients with Elevated Hepatobiliary Enzyme Levels. 61
28717071 2017
23
Persistent atrial standstill with coronary and cerebral embolism treated with left atrial appendage closure. 61
27723534 2016
24
Atrial standstill in suspected isolated cardiac sarcoidosis. 61
30546677 2016
25
The role of mutations in the SCN5A gene in cardiomyopathies. 61
26916278 2016
26
Coexisting Hyponatremia and Decline in Diastolic Blood Pressure Predispose to Atrial Standstill in Hyperkalemic Patients. 61
27301330 2016
27
Long-term management of atrial myopathy in two dogs with single chamber permanent transvenous pacemakers. 61
26923757 2016
28
Atrial standstill and thromboembolic complications. 61
26976773 2016
29
Survival of 4 dogs with persistent atrial standstill treated by pacemaker implantation. 61
26933268 2016
30
Atrial standstill in sinus node disease due to extensive atrial fibrosis: impact on dual chamber pacemaker implantation. 61
25995399 2016
31
Persistent Atrial Standstill in Acute Myocarditis. 61
26897154 2016
32
Intracardiac echocardiography for immediate detection of intracardiac thrombus formation. 61
26192113 2015
33
Stroke in a Young Individual with Left Ventricular Noncompaction and Left Atrium Standstill. 61
26413113 2015
34
Partial right atrial standstill enhancing SVC re-entry tachycardia. 61
25777938 2015
35
Persistent isolated right atrial standstill associated with left atrial tachycardia. 61
25785252 2014
36
Compound heterozygous mutations in the SCN5A-encoded Nav1.5 cardiac sodium channel resulting in atrial standstill and His-Purkinje system disease. 61
25171853 2014
37
Electroanatomic mapping and late gadolinium enhancement MRI in a genetic model of arrhythmogenic atrial cardiomyopathy. 61
24758425 2014
38
Heart failure after transvenous closure of atrial septal defect associated with atrial standstill and thiamine-responsive megaloblastic anemia. 61
24164997 2013
39
Cardiac sodium channelopathy associated with SCN5A mutations: electrophysiological, molecular and genetic aspects. 61
23818691 2013
40
Atrial standstill in a patient with progressive severe heart failure. 61
23529655 2013
41
Atrial standstill. 61
23194782 2013
42
The use of high-dose insulin therapy and intravenous lipid emulsion to treat severe, refractory diltiazem toxicosis in a dog. 61
23656275 2013
43
Isolated left atrial standstill identified during catheter ablation. 61
21070263 2013
44
Determinants of myocardial conduction velocity: implications for arrhythmogenesis. 61
23825462 2013
45
Sinus node dysfunction: recognition and treatment. 61
23114718 2012
46
Adult-onset nemaline myopathy in a dog presenting with persistent atrial standstill and primary hypothyroidism. 61
22647214 2012
47
Atrial standstill disease progression documented after 13 years follow-up. 61
21249473 2012
48
A pharmacological investigation of the venom extract of the Australian box jellyfish, Chironex fleckeri, in cardiac and vascular tissues. 61
22154831 2012
49
Biology of cardiac sodium channel Nav1.5 expression. 61
21937582 2012
50
[Heart failure due to atrial standstill in a cat]. 61
21939015 2011

Variations for Atrial Standstill

Expression for Atrial Standstill

Search GEO for disease gene expression data for Atrial Standstill.

Pathways for Atrial Standstill

Pathways related to Atrial Standstill according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.37 SCN5A GJA5

GO Terms for Atrial Standstill

Cellular components related to Atrial Standstill according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intercalated disc GO:0014704 8.62 SCN5A GJA5

Biological processes related to Atrial Standstill according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cardiac conduction GO:0061337 9.46 SCN5A GJA5
2 regulation of heart rate by cardiac conduction GO:0086091 9.43 SCN5A GJA5
3 regulation of ventricular cardiac muscle cell membrane repolarization GO:0060307 9.4 SCN5A GJA5
4 ventricular cardiac muscle cell action potential GO:0086005 9.37 SCN5A GJA5
5 regulation of atrial cardiac muscle cell membrane depolarization GO:0060371 9.32 SCN5A GJA5
6 regulation of ventricular cardiac muscle cell membrane depolarization GO:0060373 9.26 SCN5A GJA5
7 SA node cell action potential GO:0086015 9.16 SCN5A GJA5
8 regulation of atrial cardiac muscle cell membrane repolarization GO:0060372 8.96 SCN5A NPPA
9 AV node cell to bundle of His cell communication GO:0086067 8.62 SCN5A GJA5

Sources for Atrial Standstill

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....