MCID: ATR081
MIFTS: 34

Atrial Standstill

Categories: Rare diseases, Cardiovascular diseases, Genetic 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 Dexmedetomidine have been mentioned in the context of this disorder. Affiliated tissues include heart, testes and brain.

Related Diseases for Atrial Standstill

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 57)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lidocaine Approved, Vet_approved Phase 4 137-58-6 3676
2
Dexmedetomidine Approved, Vet_approved Phase 4,Not Applicable 113775-47-6 68602 5311068
3
Ropivacaine Approved Phase 4 84057-95-4 71273 175805
4 Analgesics Phase 4,Not Applicable
5 Central Nervous System Depressants Phase 4,Not Applicable
6 Adrenergic Agents Phase 4,Not Applicable
7 Adrenergic Agonists Phase 4,Not Applicable
8 Adrenergic alpha-2 Receptor Agonists Phase 4,Not Applicable
9 Adrenergic alpha-Agonists Phase 4,Not Applicable
10 Neurotransmitter Agents Phase 4,Phase 3,Not Applicable
11 Analgesics, Non-Narcotic Phase 4,Not Applicable
12 Anesthetics Phase 4,Not Applicable
13 Anesthetics, Local Phase 4
14 Peripheral Nervous System Agents Phase 4,Not Applicable
15 Hypnotics and Sedatives Phase 4,Not Applicable
16
Regadenoson Approved, Investigational Phase 3 313348-27-5 219024
17
Adenosine Approved, Investigational Phase 3,Not Applicable 58-61-7 60961
18
Sulfadoxine Approved, Investigational Phase 2 2447-57-6 17134
19
Pyrimethamine Approved, Investigational, Vet_approved Phase 2 58-14-0 4993
20
Dihydroartemisinin Approved, Investigational Phase 2 71939-50-9 6918483
21
Piperaquine Approved, Investigational Phase 2 4085-31-8 5079497
22
leucovorin Approved, Nutraceutical Phase 2 58-05-9 143 6006
23
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
24 Renal Agents Phase 2
25 Folic Acid Antagonists Phase 2
26 Vitamin B Complex Phase 2
27 Fanasil, pyrimethamine drug combination Phase 2
28 Anti-Infective Agents Phase 2
29 Antiparasitic Agents Phase 2
30 Antiprotozoal Agents Phase 2
31 Antimalarials Phase 2
32 Artemisinine Phase 2
33 Artemisinins Phase 2
34 Folate Nutraceutical Phase 2
35 Vitamin B9 Nutraceutical Phase 2
36
Atropine Approved, Vet_approved Not Applicable 5908-99-6, 51-55-8 174174
37
Cortisone acetate Approved, Investigational 1950-04-4, 50-04-4 5745
38 Natriuretic Peptide, Brain Not Applicable
39 Anti-Arrhythmia Agents Not Applicable
40 Vasodilator Agents Not Applicable
41
Glycopyrrolate Not Applicable 596-51-0 3494
42 Respiratory System Agents Not Applicable
43 Adjuvants, Anesthesia Not Applicable
44 Cholinergic Agents Not Applicable
45 Muscarinic Antagonists Not Applicable
46 Cholinergic Antagonists Not Applicable
47 Mydriatics Not Applicable
48 Parasympatholytics Not Applicable
49 Anti-Asthmatic Agents Not Applicable
50 Autonomic Agents Not Applicable

Interventional clinical trials:

(show all 18)
# 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 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
3 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
4 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)
5 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
6 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
7 Pro-arrhythmic Potential of GSK3039294 in Healthy Subjects Not yet recruiting NCT03567499 Phase 1 GSK3039294;Placebo
8 Dexmedetomidine and Adenosine: Therapeutic Use for SVT Completed NCT01495481 Not Applicable Dexmedetomidine;Adenosine
9 Multicentre Retrospective Study to Understand Anti-thrombotic Treatment Patterns and Outcomes of Acute Coronary Syndrome (ACS) Patients Completed NCT01280630
10 Implantable Loop Recorder in Hemodialysis Patients Completed NCT01252823 Not Applicable
11 Diagnostic Yield of an Ambulatory Patch Monitor in Unexplained Emergency Department Syncope: A Pilot Study (PATCH-ED) Completed NCT02683174 Not Applicable
12 Conduction Abnormalities With Severe Aortic Stenosis Before and After Transcatheter Aortic Valve Replacement Recruiting NCT03180073
13 Cardiac Arrhythmias in Dravet Syndrome Recruiting NCT02415686
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 Implantable Cardiac Monitors in High-Risk Post-Infarction Patients With Cardiac Autonomic Dysfunction Recruiting NCT02594488 Not Applicable
17 Arrhythmias in Post-Myocardial Infarction Patients Recruiting NCT03231826
18 Hypoglycaemia and Cardiac Arrhythmias in Type 2 Diabetes Enrolling by invitation NCT03150030

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, Brain

Publications for Atrial Standstill

Articles related to Atrial Standstill:

(show all 40)
# Title Authors Year
1
A homozygous SCN5A mutation associated with atrial standstill and sudden death. ( 29781517 )
2018
2
Atrial standstill in a pediatric patient with associated caveolin-3 mutation. ( 29387541 )
2017
3
Presumptive partial atrial standstill secondary to atrial cardiomyopathy in a Greyhound. ( 28314614 )
2017
4
Survival time with pacemaker implantation for dogs diagnosed with persistent atrial standstill. ( 28578822 )
2017
5
Persistent atrial standstill following the Cox-maze III procedure: reversal with sustained atrial pacing. ( 27558131 )
2016
6
Persistent Atrial Standstill in Acute Myocarditis. ( 26897154 )
2016
7
Persistent atrial standstill with coronary and cerebral embolism treated with left atrial appendage closure. ( 27723534 )
2016
8
Atrial standstill in sinus node disease due to extensive atrial fibrosis: impact on dual chamber pacemaker implantation. ( 25995399 )
2016
9
Coexisting Hyponatremia and Decline in Diastolic Blood Pressure Predispose to Atrial Standstill in Hyperkalemic Patients. ( 27301330 )
2016
10
Atrial standstill and thromboembolic complications. ( 26976773 )
2016
11
Survival of 4 dogs with persistent atrial standstill treated by pacemaker implantation. ( 26933268 )
2016
12
Partial right atrial standstill enhancing SVC re-entry tachycardia. ( 25777938 )
2015
13
Persistent isolated right atrial standstill associated with left atrial tachycardia. ( 25785252 )
2014
14
Compound heterozygous mutations in the SCN5A-encoded Nav1.5 cardiac sodium channel resulting in atrial standstill and His-Purkinje system disease. ( 25171853 )
2014
15
Atrial standstill in a patient with progressive severe heart failure. ( 23529655 )
2013
16
Atrial standstill. ( 23194782 )
2013
17
Isolated left atrial standstill identified during catheter ablation. ( 21070263 )
2013
18
Heart failure after transvenous closure of atrial septal defect associated with atrial standstill and thiamine-responsive megaloblastic anemia. ( 24164997 )
2013
19
Adult-onset nemaline myopathy in a dog presenting with persistent atrial standstill and primary hypothyroidism. ( 22647214 )
2012
20
Atrial standstill disease progression documented after 13 years follow-up. ( 21249473 )
2012
21
Atrial standstill: a rare case. ( 23074623 )
2011
22
Permanent atrial standstill with irregular junctional ectopic rhythm mimicking atrial fibrillation. ( 21358233 )
2011
23
Thiamine-responsive megaloblastic anemia syndrome with atrial standstill: a case report. ( 21285901 )
2011
24
Dexmedetomidine-related atrial standstill and loss of capture in a pediatric patient after congenital heart surgery. ( 20959781 )
2011
25
Atrial standstill causing congestive heart failure in a child with ostium secundum atrial septal defect. ( 19937323 )
2010
26
Persistent atrial standstill and idioventricular rhythm in a patient with thalassemia intermedia. ( 19717959 )
2009
27
A cardio-neurological form of laminopathy: dilated cardiomyopathy with permanent partial atrial standstill and axonal neuropathy. ( 19272076 )
2009
28
Atrial standstill associated with loss of atrial myocytes: a rare cause of fetal bradyarrhythmia. ( 19716093 )
2009
29
Congenital atrial standstill associated with coinheritance of a novel SCN5A mutation and connexin 40 polymorphisms. ( 16188595 )
2005
30
[Atrial standstill: a paralysis of cardiological relevance]. ( 15568605 )
2004
31
A cardiac sodium channel mutation identified in Brugada syndrome associated with atrial standstill. ( 14687250 )
2004
32
A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstill. ( 12522116 )
2003
33
Serum atrial natriuretic peptide concentration is a useful predictor of atrial standstill in patients with heart failure. ( 10929785 )
2000
34
Atrial standstill in a case of Kugelberg-Welander syndrome with cardiac involvement: an electrophysiologic study. ( 10454312 )
1999
35
Left atrial appendage blood flow in a case of persistent atrial standstill. ( 8960950 )
1996
36
Resolution of atrial standstill in a child with myocarditis. ( 7505934 )
1993
37
Familial atrial standstill caused by amyloidosis. ( 3370185 )
1988
38
Partial atrial standstill in sick sinus syndrome; intraatrial and AV nodal block with short PR interval. ( 729599 )
1978
39
Persistent atrial standstill with limb girdle muscular dystrophy. ( 618588 )
1978
40
Persistent atrial standstill. ( 1084660 )
1975

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 cardiac conduction GO:0061337 9.46 GJA5 SCN5A
2 regulation of heart rate by cardiac conduction GO:0086091 9.43 GJA5 SCN5A
3 regulation of ventricular cardiac muscle cell membrane repolarization GO:0060307 9.4 GJA5 SCN5A
4 ventricular cardiac muscle cell action potential GO:0086005 9.37 GJA5 SCN5A
5 regulation of atrial cardiac muscle cell membrane depolarization GO:0060371 9.32 GJA5 SCN5A
6 regulation of ventricular cardiac muscle cell membrane depolarization GO:0060373 9.26 GJA5 SCN5A
7 regulation of atrial cardiac muscle cell membrane repolarization GO:0060372 9.16 NPPA SCN5A
8 SA node cell action potential GO:0086015 8.96 GJA5 SCN5A
9 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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