MCID: ATR081
MIFTS: 29

Atrial Standstill

Categories: Cardiovascular diseases, Genetic diseases, Rare diseases

Aliases & Classifications for Atrial Standstill

MalaCards integrated aliases for Atrial Standstill:

Name: Atrial Standstill 59 73
Cardiomyopathy, Familial, with Conduction Disturbance 73
Atrial Cardiomyopathy with Heart Block 59

Characteristics:

Orphanet epidemiological data:

59
atrial standstill
Inheritance: Autosomal dominant,Not applicable; Age of onset: Adult;

Classifications:



External Ids:

Orphanet 59 ORPHA1344
ICD10 via Orphanet 34 I45.5
UMLS via Orphanet 74 C0541782 C1838539

Summaries for Atrial Standstill

MalaCards based summary : Atrial Standstill, also known as cardiomyopathy, familial, with conduction disturbance, is related to atrial standstill 2 and atrial standstill 1. 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. The drugs Lidocaine and Ropivacaine have been mentioned in the context of this disorder. Affiliated tissues include heart 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 all 41)
# Related Disease Score Top Affiliating Genes
1 atrial standstill 2 34.0 NPPA NPPA-AS1
2 atrial standstill 1 33.7 GJA5 LOC102723321 SCN5A
3 syncope 29.8 NPPA SCN5A
4 dilated cardiomyopathy 29.7 GJA5 NPPA SCN5A
5 atrial fibrillation 29.7 GJA5 NPPA SCN5A
6 heart disease 29.5 GJA5 NPPA SCN5A
7 myocarditis 10.4
8 congestive heart failure 10.1
9 megaloblastic anemia 10.1
10 atrial heart septal defect 10.1
11 progressive familial heart block, type ia 10.0 GJA5 SCN5A
12 progressive familial heart block 10.0 GJA5 SCN5A
13 first-degree atrioventricular block 10.0 GJA5 SCN5A
14 sinoatrial node disease 10.0 GJA5 SCN5A
15 systemic lupus erythematosus 10.0
16 thiamine-responsive megaloblastic anemia syndrome 10.0
17 myocardial infarction 10.0
18 acute myocardial infarction 10.0
19 thalassemia 10.0
20 brugada syndrome 10.0
21 rheumatic heart disease 10.0
22 diarrhea 10.0
23 cardiac sarcoidosis 10.0
24 sick sinus syndrome 10.0
25 hypothyroidism 10.0
26 mitral valve stenosis 10.0
27 nemaline myopathy 10.0
28 acute myocarditis 10.0
29 myopathy 10.0
30 axonal neuropathy 10.0
31 neuropathy 10.0
32 lupus erythematosus 10.0
33 amyloidosis 10.0
34 muscular dystrophy 10.0
35 adult-onset nemaline myopathy 10.0
36 limb-girdle muscular dystrophy 10.0
37 atrial fibrillation, familial, 6 10.0 NPPA NPPA-AS1
38 heart conduction disease 10.0 GJA5 SCN5A
39 atrial fibrillation, familial, 11 9.9 GJA5 LOC102723321
40 tetralogy of fallot 9.8 GJA5 NPPA
41 familial atrial fibrillation 9.5 GJA5 LOC102723321 NPPA SCN5A

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

Drugs for Atrial Standstill (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 60)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lidocaine Approved, Vet_approved Phase 4 137-58-6 3676
2
Ropivacaine Approved Phase 4 84057-95-4 175805 71273
3
Dexmedetomidine Approved, Vet_approved Phase 4,Not Applicable 113775-47-6 68602 5311068
4 Neurotransmitter Agents Phase 4,Phase 3,Not Applicable
5 Adrenergic Agents Phase 4,Not Applicable
6 Hypnotics and Sedatives Phase 4,Not Applicable
7 Analgesics, Non-Narcotic Phase 4,Not Applicable
8 Central Nervous System Depressants Phase 4,Not Applicable
9 Anesthetics Phase 4,Not Applicable
10 Adrenergic alpha-2 Receptor Agonists Phase 4,Not Applicable
11 Adrenergic alpha-Agonists Phase 4,Not Applicable
12 Anesthetics, Local Phase 4
13 Adrenergic Agonists Phase 4,Not Applicable
14 Analgesics Phase 4,Not Applicable
15 Peripheral Nervous System Agents Phase 4,Not Applicable
16 Cholinergic Antagonists Phase 4,Not Applicable
17 Cholinergic Agents Phase 4,Not Applicable
18 Anticonvulsants Phase 4
19 Muscarinic Antagonists Phase 4,Not Applicable
20 Bromides Phase 4
21
Adenosine Approved, Investigational Phase 3,Not Applicable 58-61-7 60961
22
Regadenoson Approved, Investigational Phase 3 313348-27-5 219024
23
Pyrimethamine Approved, Investigational, Vet_approved Phase 2 58-14-0 4993
24
leucovorin Approved Phase 2 58-05-9 6006 143
25
Sulfadoxine Approved, Investigational Phase 2 2447-57-6 17134
26
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
27
Piperaquine Experimental, Investigational Phase 2 4085-31-8 5079497
28
Dihydroartemisinin Experimental, Investigational Phase 2 71939-50-9 6918483
29 Folate Phase 2
30 Anti-Infective Agents, Urinary Phase 2
31 Vitamin B9 Phase 2
32 Vitamin B Complex Phase 2
33 Artemisinine Phase 2
34 Antiparasitic Agents Phase 2
35 Antiprotozoal Agents Phase 2
36 Fanasil, pyrimethamine drug combination Phase 2
37 Folic Acid Antagonists Phase 2
38 Antimalarials Phase 2
39 Renal Agents Phase 2
40 Artemisinins Phase 2
41 Anti-Infective Agents Phase 2
42
Atropine Approved, Vet_approved Not Applicable 51-55-8, 5908-99-6 174174
43
Cortisone acetate Approved, Investigational 1950-04-4, 50-04-4 5745
44
Cortisone Experimental 53-06-5 222786
45 Anti-Arrhythmia Agents Not Applicable
46 Vasodilator Agents Not Applicable
47 Natriuretic Peptide, Brain Not Applicable
48 Anti-Asthmatic Agents Not Applicable
49 Autonomic Agents Not Applicable
50 Mydriatics Not Applicable

Interventional clinical trials:

(show all 22)
# Name Status NCT ID Phase Drugs
1 Addition of Dexmedetomidine to Ropivacaine-induced Supraclavicular Block (ADRIB Trial) Completed NCT02630290 Phase 4 Ropivacaine;Ropivacaine + Dexmedetomidine
2 Evaluate the Effect of Aclidinium Bromide on Long-term Cardiovascular Safety and Exacerbations in Moderate to Very Severe COPD Patients. Completed NCT01966107 Phase 4 Aclidinium Bromide;Placebo
3 A Study to Assess Regadenoson Administration Following an Inadequate Exercise Stress Test as Compared to Regadenoson Alone for Myocardial Perfusion Imaging (MPI) Using Single Photon Emission Computed Tomography (SPECT) Completed NCT01618669 Phase 3 Regadenoson
4 An Extension of Study Fx-005 Evaluating Long-Term Safety And Clinical Outcomes Of Fx-1006A In Patients With Transthyretin Amyloid Polyneuropathy Completed NCT00791492 Phase 2, Phase 3 Fx-1006A
5 Cardio‑Safety of Dihydroartemisinin‑Piperaquine and Pharmacokinetics of Piperaquine Amongst Pregnant Women in Tanzania Unknown status NCT02909712 Phase 2 sulfadoxine-pyrimethamine (SP);dihydroartemisinin-piperaquine (DHA-PQP)
6 The Effects Of Fx-1006A On Transthyretin Stabilization And Clinical Outcome Measures In Patients With V122I Or Wild-Type TTR Amyloid Cardiomyopathy Completed NCT00694161 Phase 2 Fx-1006A
7 The Effects of Fx-1006A on Transthyretin Stabilization and Clinical Outcome Measures in Patients With Non-V30M Transthyretin Amyloidosis Completed NCT00630864 Phase 2 Fx-1006A
8 Pro-arrhythmic Potential of GSK3039294 in Healthy Subjects Withdrawn NCT03567499 Phase 1 GSK3039294;Placebo
9 Cardiac Arrhythmias in Dravet Syndrome Completed NCT02415686
10 Dexmedetomidine and Adenosine: Therapeutic Use for SVT Completed NCT01495481 Not Applicable Dexmedetomidine;Adenosine
11 Multicentre Retrospective Study to Understand Anti-thrombotic Treatment Patterns and Outcomes of Acute Coronary Syndrome (ACS) Patients Completed NCT01280630
12 Implantable Loop Recorder in Hemodialysis Patients Completed NCT01252823 Not Applicable
13 Diagnostic Yield of an Ambulatory Patch Monitor in Unexplained Emergency Department Syncope: A Pilot Study (PATCH-ED) Completed NCT02683174 Not Applicable
14 Effect of Atropine or Glycopyrrolate on the Prevention of Bradycardia During Sedation Using Dexmedetomidine in Adult Patients Undergoing Lower Extremity Surgery Under Spinal Anesthesia Recruiting NCT03322150 Not Applicable Atropine;Glycopyrrolate
15 Efficacy of Transcatheter Ablation Using Anatomic Approach of Ganglionated Plexi Located in the Right Atrium to Prevent Neuromediated Cardioinhibitory Syncope Recruiting NCT01814228 Not Applicable
16 Effect of His Bundle Pacing in Treatment of Slow Arrhythmia on Function of Left Atrial and Ventricle in Short Term Recruiting NCT03685617 Not Applicable
17 Effect of His Bundle Pacing in Treatment of Slow Arrhythmia on Function of Left Atrial and Ventricle Recruiting NCT03590353 Not Applicable
18 Implantable Cardiac Monitors in High-Risk Post-Infarction Patients With Cardiac Autonomic Dysfunction Recruiting NCT02594488 Not Applicable
19 Arrhythmias in Post-Myocardial Infarction Patients Recruiting NCT03231826
20 Conduction Abnormalities With Severe Aortic Stenosis Before and After Transcatheter Aortic Valve Replacement Active, not recruiting NCT03180073
21 Hypoglycaemia and Cardiac Arrhythmias in Type 2 Diabetes Enrolling by invitation NCT03150030
22 The Safety and Efficacy of FFR Protocol in NTUH System Not yet recruiting NCT03693157

Search NIH Clinical Center for Atrial Standstill

Genetic Tests for Atrial Standstill

Anatomical Context for Atrial Standstill

MalaCards organs/tissues related to Atrial Standstill:

41
Heart, Testes

Publications for Atrial Standstill

Articles related to Atrial Standstill:

(show top 50) (show all 110)
# Title Authors Year
1
A homozygous SCN5A mutation associated with atrial standstill and sudden death. ( 29781517 )
2018
2
Long-term follow-up of permanent atrial standstill in a German family with mutation in the SCN5A gene. ( 30116708 )
2018
3
Atrial standstill in a pediatric patient with associated caveolin-3 mutation. ( 29387541 )
2017
4
Presumptive partial atrial standstill secondary to atrial cardiomyopathy in a Greyhound. ( 28314614 )
2017
5
Survival time with pacemaker implantation for dogs diagnosed with persistent atrial standstill. ( 28578822 )
2017
6
Persistent atrial standstill following the Cox-maze III procedure: reversal with sustained atrial pacing. ( 27558131 )
2016
7
Persistent Atrial Standstill in Acute Myocarditis. ( 26897154 )
2016
8
Persistent atrial standstill with coronary and cerebral embolism treated with left atrial appendage closure. ( 27723534 )
2016
9
Atrial standstill in sinus node disease due to extensive atrial fibrosis: impact on dual chamber pacemaker implantation. ( 25995399 )
2016
10
Coexisting Hyponatremia and Decline in Diastolic Blood Pressure Predispose to Atrial Standstill in Hyperkalemic Patients. ( 27301330 )
2016
11
Atrial standstill and thromboembolic complications. ( 26976773 )
2016
12
Survival of 4 dogs with persistent atrial standstill treated by pacemaker implantation. ( 26933268 )
2016
13
Atrial standstill in suspected isolated cardiac sarcoidosis. ( 30546677 )
2016
14
Partial right atrial standstill enhancing SVC re-entry tachycardia. ( 25777938 )
2015
15
Persistent isolated right atrial standstill associated with left atrial tachycardia. ( 25785252 )
2014
16
Compound heterozygous mutations in the SCN5A-encoded Nav1.5 cardiac sodium channel resulting in atrial standstill and His-Purkinje system disease. ( 25171853 )
2014
17
Atrial standstill in a patient with progressive severe heart failure. ( 23529655 )
2013
18
Atrial standstill. ( 23194782 )
2013
19
Isolated left atrial standstill identified during catheter ablation. ( 21070263 )
2013
20
Heart failure after transvenous closure of atrial septal defect associated with atrial standstill and thiamine-responsive megaloblastic anemia. ( 24164997 )
2013
21
Adult-onset nemaline myopathy in a dog presenting with persistent atrial standstill and primary hypothyroidism. ( 22647214 )
2012
22
Atrial standstill disease progression documented after 13 years follow-up. ( 21249473 )
2012
23
Atrial standstill: a rare case. ( 23074623 )
2011
24
Permanent atrial standstill with irregular junctional ectopic rhythm mimicking atrial fibrillation. ( 21358233 )
2011
25
Thiamine-responsive megaloblastic anemia syndrome with atrial standstill: a case report. ( 21285901 )
2011
26
Dexmedetomidine-related atrial standstill and loss of capture in a pediatric patient after congenital heart surgery. ( 20959781 )
2011
27
Atrial standstill causing congestive heart failure in a child with ostium secundum atrial septal defect. ( 19937323 )
2010
28
Persistent atrial standstill and idioventricular rhythm in a patient with thalassemia intermedia. ( 19717959 )
2009
29
A cardio-neurological form of laminopathy: dilated cardiomyopathy with permanent partial atrial standstill and axonal neuropathy. ( 19272076 )
2009
30
Mapping and ablation of multiple atrial arrhythmias in a patient with persistent atrial standstill after remote viral myocarditis. ( 19170922 )
2009
31
Implantable cardioverter-defibrillator implantation in a patient with atrial standstill. ( 19259364 )
2009
32
Role of electroanatomic mapping in assessing the extent of atrial standstill: diagnostic and therapeutic implications. ( 19512940 )
2009
33
Atrial standstill associated with loss of atrial myocytes: a rare cause of fetal bradyarrhythmia. ( 19716093 )
2009
34
Electroanatomical mapping in partial atrial standstill for visualization of atrial viability and a suitable pacing site. ( 18373774 )
2008
35
Persistent atrial standstill in non-compaction cardiomyopathy. ( 19046733 )
2008
36
Idiopathic dilatation of right atrium with atrial standstill presenting as stroke. ( 16412183 )
2006
37
Persistent atrial standstill in noncompaction cardiomyopathy. ( 16565905 )
2006
38
Congenital atrial standstill associated with coinheritance of a novel SCN5A mutation and connexin 40 polymorphisms. ( 16188595 )
2005
39
Partial atrial standstill: a case report. ( 16003711 )
2005
40
Familial atrial standstill in association with dilated cardiomyopathy. ( 16176547 )
2005
41
Enormous bi-atrial enlargement in a persistent idiopathic atrial standstill. ( 16227313 )
2005
42
[Atrial standstill: a paralysis of cardiological relevance]. ( 15568605 )
2004
43
A cardiac sodium channel mutation identified in Brugada syndrome associated with atrial standstill. ( 14687250 )
2004
44
Atrial standstill with subnodal conduction defect and diffuse ventricular fibrosis managed with low right atrial pacing. ( 14764182 )
2004
45
A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstill. ( 12522116 )
2003
46
Left atrial standstill in a patient with mitral stenosis and sinus rhythm: a risk of thrombus hidden by left and right atrial electrical dissociation. ( 12975410 )
2003
47
Restored atrial excitability after late recanalization in a patient with atrial standstill and acute myocardial infarction. ( 11915991 )
2002
48
Permanent transvenous pacemaker implantation in an Ibizan hound cross with persistent atrial standstill. ( 12395764 )
2002
49
Noninvasive diagnosis of partial atrial standstill using magnetocardiograms. ( 12499629 )
2002
50
Serum atrial natriuretic peptide concentration is a useful predictor of atrial standstill in patients with heart failure. ( 10929785 )
2000

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 GJA5 SCN5A

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 GJA5 SCN5A

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

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

Sources for Atrial Standstill

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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