MCID: ATR048
MIFTS: 31

Atrial Tachyarrhythmia with Short Pr Interval

Categories: Rare diseases

Aliases & Classifications for Atrial Tachyarrhythmia with Short Pr Interval

MalaCards integrated aliases for Atrial Tachyarrhythmia with Short Pr Interval:

Name: Atrial Tachyarrhythmia with Short Pr Interval 56 12 58
Lown-Ganong-Levine Syndrome 12 58 43 15 71
Syndrome of Short P-R Interval, Normal Qrs Complexes, and Supraventricular Tachycardias 12
Lgl Syndrome 58

Characteristics:

Orphanet epidemiological data:

58
lown-ganong-levine syndrome
Inheritance: Autosomal dominant,Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: All ages;

OMIM:

56
Inheritance:
autosomal dominant


HPO:

31
atrial tachyarrhythmia with short pr interval:
Inheritance autosomal dominant inheritance


Classifications:



External Ids:

Disease Ontology 12 DOID:13087
OMIM 56 108950
ICD9CM 34 426.81
MeSH 43 D008151
SNOMED-CT 67 55475008
ICD10 32 I45.6
ICD10 via Orphanet 33 I45.6
UMLS via Orphanet 72 C0024054 C1862387
Orphanet 58 ORPHA844
MedGen 41 C1862387
UMLS 71 C0024054

Summaries for Atrial Tachyarrhythmia with Short Pr Interval

Disease Ontology : 12 A syndrome that involves pre-excitation of the ventricles due to an accessory pathway providing an abnormal electrical communication from the atria to the ventricles.

MalaCards based summary : Atrial Tachyarrhythmia with Short Pr Interval, also known as lown-ganong-levine syndrome, is related to wolff-parkinson-white syndrome and progressive familial heart block, type ia. An important gene associated with Atrial Tachyarrhythmia with Short Pr Interval is ZSCAN20 (Zinc Finger And SCAN Domain Containing 20). The drugs Verapamil and Hydroxyurea have been mentioned in the context of this disorder. Affiliated tissues include heart, and related phenotypes are paroxysmal atrial fibrillation and paroxysmal atrial tachycardia

More information from OMIM: 108950

Related Diseases for Atrial Tachyarrhythmia with Short Pr Interval

Diseases related to Atrial Tachyarrhythmia with Short Pr Interval via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 22)
# Related Disease Score Top Affiliating Genes
1 wolff-parkinson-white syndrome 10.9
2 progressive familial heart block, type ia 10.7
3 progressive familial heart block, type ib 10.7
4 right bundle branch block 10.7
5 atrial standstill 1 10.6
6 fabry disease 10.6
7 branchiootic syndrome 1 10.6
8 ventricular fibrillation, paroxysmal familial, 1 10.6
9 atrioventricular block 10.6
10 sinoatrial node disease 10.6
11 cardiac arrest 10.6
12 left ventricular noncompaction 10.6
13 hypertrophic cardiomyopathy 10.6
14 sick sinus syndrome 10.6
15 angiokeratoma 10.6
16 dystonia 10.6
17 felty syndrome 9.9
18 spastic paraplegia, intellectual disability, nystagmus, and obesity 9.9
19 neutropenia 9.9
20 heart valve disease 9.9
21 hyperthyroidism 9.9
22 splenomegaly 9.9

Comorbidity relations with Atrial Tachyarrhythmia with Short Pr Interval via Phenotypic Disease Network (PDN):


Familial Atrial Fibrillation

Graphical network of the top 20 diseases related to Atrial Tachyarrhythmia with Short Pr Interval:



Diseases related to Atrial Tachyarrhythmia with Short Pr Interval

Symptoms & Phenotypes for Atrial Tachyarrhythmia with Short Pr Interval

Human phenotypes related to Atrial Tachyarrhythmia with Short Pr Interval:

31
# Description HPO Frequency HPO Source Accession
1 paroxysmal atrial fibrillation 31 HP:0004757
2 paroxysmal atrial tachycardia 31 HP:0006671
3 shortened pr interval 31 HP:0005165
4 permanent atrial fibrillation 31 HP:0004754

Symptoms via clinical synopsis from OMIM:

56
Cardiac:
paroxysmal atrial tachycardia
paroxysmal or chronic atrial fibrillation

Lab:
electrocardiographic short pr interval

Clinical features from OMIM:

108950

Drugs & Therapeutics for Atrial Tachyarrhythmia with Short Pr Interval

Drugs for Atrial Tachyarrhythmia with Short Pr Interval (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 9)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Verapamil Approved Phase 2 52-53-9 2520
2
Hydroxyurea Approved Phase 2 127-07-1 3657
3 Hormones Phase 2
4 Anti-Arrhythmia Agents Phase 2
5 Vasodilator Agents Phase 2
6 Calcium, Dietary Phase 2
7 calcium channel blockers Phase 2
8 Anti-Retroviral Agents Phase 2
9
Calcium Nutraceutical Phase 2 7440-70-2 271

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Combination of Hydroxyurea and Verapamil for Refractory Meningiomas Completed NCT00706810 Phase 2 Hydroxyurea;Verapamil

Search NIH Clinical Center for Atrial Tachyarrhythmia with Short Pr Interval

Cochrane evidence based reviews: lown-ganong-levine syndrome

Genetic Tests for Atrial Tachyarrhythmia with Short Pr Interval

Anatomical Context for Atrial Tachyarrhythmia with Short Pr Interval

MalaCards organs/tissues related to Atrial Tachyarrhythmia with Short Pr Interval:

40
Heart

Publications for Atrial Tachyarrhythmia with Short Pr Interval

Articles related to Atrial Tachyarrhythmia with Short Pr Interval:

(show top 50) (show all 68)
# Title Authors PMID Year
1
Familial atrial tachyarrhythmia with short PR interval. 56 61
836114 1977
2
FAMILIAL ATRIAL FIBRILLATION. 56
14098892 1963
3
The syndrome of short P-R interval, normal QRS complex and paroxysmal rapid heart action. 56
14926053 1952
4
Perioperative management of patients with pre-excitation syndromes. 61
30393770 2018
5
A Case of Lown-Ganong-Levine Syndrome: Due to an Accessory Pathway of James Fibers or Enhanced Atrioventricular Nodal Conduction (EAVNC)? 61
29550833 2018
6
Anaesthetic management of a patient with Lown Ganong Levine syndrome-a case report. 61
27365828 2011
7
Lown-Ganong-Levine syndrome in a 3-month-old infant with isolated left ventricular noncompaction. 61
19859765 2010
8
Lown-Ganong-Levine syndrome in pregnancy. 61
14662232 2003
9
[Atypical symptoms of Fabry's disease: sudden bilateral deafness, lymphoedema and Lown-Ganong-Levine syndrome]. 61
12685250 2002
10
[Ventricular pre-excitation: electrophysiopathology, criteria for interpretation and clinical diagnosis. References for geriatrics]. 61
11279385 2001
11
High incidence of pre-excitation syndrome in Japanese families with Leber's hereditary optic neuropathy. 61
9147893 1996
12
Clinical and evolutive aspects in ventricular preexcitation syndromes in child. 61
9455443 1996
13
[Lown-Ganong-Levine syndrome]. 61
9047441 1996
14
Enhanced A-V nodal conduction (Lown-Ganong-Levine syndrome) by congenitally hypoplastic A-V node. 61
1464347 1992
15
The preexcitation syndrome: epidemiological and genetic study. 61
1572738 1992
16
Pulmonary developmental anomaly associated with Klippel-Feil syndrome and anomalous atrioventricular conduction. 61
1555440 1992
17
[Possible pro-arrhythmic effects of vagal stimulation maneuvers]. 61
2608187 1989
18
[Lown-Ganong-Levine syndrome: is it a true syndrome or just a coincidence?]. 61
2673902 1989
19
[Transesophageal pacing in the diagnosis of accelerated atrioventricular conduction]. 61
2720718 1989
20
Lown-Ganong-Levine syndrome. 61
3269321 1988
21
Supraventricular tachycardia in a patient with Lown-Ganong-Levine syndrome associated with apical hypertrophic cardiomyopathy. 61
3398258 1988
22
Adenosine: a clinical experience and comparison with verapamil for the termination of supraventricular tachycardias. 61
3295883 1987
23
Intrauterine supraventricular tachycardia with a familial history of Lown-Ganong-Levine syndrome. 61
3774583 1986
24
Multifocal Purkinje-like tumor of the heart. Occurrence with other anatomic abnormalities in the atrioventricular junction of an infant with junctional tachycardia, Lown-Ganong-Levine syndrome, and sudden death. 61
3971758 1985
25
[Apropos of a case of intra-uterine supraventricular tachycardia]. 61
6714551 1984
26
Lown-Ganong-Levine syndrome associated with Mahaim nodoventricular fibers. 61
15227113 1983
27
Mechanisms of junctional tachycardias in the Lown-Ganong-Levine syndrome. 61
6849236 1983
28
Treatment of drug resistant A-V reciprocating tachycardias with multiprogrammable dual demand A-V sequential (DVI,MN) pacemakers. 61
6184682 1982
29
Mitral valve prolapse and pre-excitation. 61
6182551 1982
30
The Lown-Ganong-Levine syndrome. 61
6182544 1982
31
[Case of Lown-Ganong-Levine syndrome in a patient with myeloblastic leukemia]. 61
6961379 1982
32
Analysis of anterograde and retrograde fast pathway properties in patients with dual atrioventricular nodal pathways: observations regarding the pathophysiology of the Lown-Ganong-Levine syndrome. 61
7058743 1982
33
How to spot...and care for...the patient with Lown-Ganong-Levine syndrome. 61
6907768 1981
34
Response of atrial flutter to overdrive atrial pacing and intravenous disopyramide phosphate, singly and in combination. 61
7426181 1980
35
Cycle length in atrioventricular nodal reentrant paroxysmal tachycardia with observations on the Lown-Ganong-Levine syndrome. 61
7377112 1980
36
[Diagnosis of Lown-Ganong-Levine syndrome]. 61
523211 1979
37
Atrial depolarization in Wolf-Parkinson-White and Lown-Ganong-Levine syndrome: vectorcardiographic features. 61
156108 1979
38
Electrocardiograms of the month: Lown-Ganong-Levine syndrome and right bundle-branch block. 61
287918 1979
39
[Arrhythmias. Diagnosis and therapy. LGL (Lown-Ganong-Levine) syndrome]. 61
34780 1979
40
Ventricular pre-excitation and prolonged Q-T interval syndromes in a patient with mitral valve prolapse. 61
696573 1978
41
Lown-Ganong-Levine syndrome. 61
679452 1978
42
Preservation of pre-excitation despite acute myocardial infarction complicated by complete heart block. 61
679113 1978
43
Association of sick sinus and Lown-Ganong-Levine syndromes. 61
279323 1978
44
[The syndrome of short AV-conduction time with normal ventricular complex and normal variation. Lown-Ganong-Levine syndrome]. 61
664719 1978
45
[Lown-Ganong-Levine syndrome]. 61
691631 1978
46
Characteristics of atrioventricular conduction and the spectrum of arrhythmias in lown-ganong-levine syndrome. 61
624155 1978
47
Abnormalities of atrioventricular conduction in patients with the Lown-Ganong-Levine syndrome. 61
747919 1978
48
Paroxysmal supraventricular tachycardia in patients with mitral valve prolapse. 61
618376 1978
49
Supraventricular tachycardia in Lown-Ganong-Levine syndrome: atrionodal versus intranodal reentry. 61
910716 1977
50
[Lown-Ganong-Levine syndrome]. 61
612967 1977

Variations for Atrial Tachyarrhythmia with Short Pr Interval

Expression for Atrial Tachyarrhythmia with Short Pr Interval

Search GEO for disease gene expression data for Atrial Tachyarrhythmia with Short Pr Interval.

Pathways for Atrial Tachyarrhythmia with Short Pr Interval

GO Terms for Atrial Tachyarrhythmia with Short Pr Interval

Cellular components related to Atrial Tachyarrhythmia with Short Pr Interval according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 mitochondrial membrane GO:0031966 9.16 NDUFA6 MT-ND4
2 respiratory chain GO:0070469 8.96 NDUFA6 MT-ND4
3 mitochondrial respiratory chain complex I GO:0005747 8.62 NDUFA6 MT-ND4

Biological processes related to Atrial Tachyarrhythmia with Short Pr Interval according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 mitochondrial respiratory chain complex I assembly GO:0032981 8.96 NDUFA6 MT-ND4
2 mitochondrial electron transport, NADH to ubiquinone GO:0006120 8.62 NDUFA6 MT-ND4

Molecular functions related to Atrial Tachyarrhythmia with Short Pr Interval according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 NADH dehydrogenase (ubiquinone) activity GO:0008137 8.62 NDUFA6 MT-ND4

Sources for Atrial Tachyarrhythmia with Short Pr Interval

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
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