TGA
MCID: TRN044
MIFTS: 49

Transposition of the Great Arteries (TGA) malady

Rare diseases category

Summaries for Transposition of the Great Arteries

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44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 Transposition of the great arteries (tga) is a type of congenital heart defect in which there is a reversal of the normal connections of the aorta and the pulmonary artery with the heart. the aorta and pulmonary artery are reversed, which causes oxygen-poor blood to be circulated to the body and oxygen-rich blood to be circulated between the lungs and the heart, rather than to the body. symptoms are apparent at birth and include great difficulty breathing and severe cyanosis (a bluish discoloration of the skin). the exact cause of tga in most cases is unknown. surgery is done to correct the abnormality during the first few days of life. last updated: 6/27/2011

MalaCards: Transposition of the Great Arteries, also known as transposition of the great arteries, dextro-looped 1, is related to ventricular septal defect and double outlet right ventricle. An important gene associated with Transposition of the Great Arteries is MED13L (mediator complex subunit 13-like), and among its related pathways are Signaling by NODAL and Transcriptional Regulatory Network in Embryonic Stem Cell. The compounds taurolidine and zoledronic acid have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and skin, and related mouse phenotypes are normal and digestive/alimentary.

Wikipedia:66 Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal... more...

Aliases & Classifications for Transposition of the Great Arteries

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44NIH Rare Diseases, 63UMLS
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases


Aliases & Descriptions:

transposition of the great arteries 44
transposition of the great arteries, dextro-looped 1 63
dextro-looped transposition of the great arteries 44
dtga 44
tga 44


Related Diseases for Transposition of the Great Arteries

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18GeneCards, 19GeneDecks
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Diseases related to Transposition of the Great Arteries via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 89)
idRelated DiseaseScoreTop Affiliating Genes
1ventricular septal defect31.8NKX2-5, GATA4
2double outlet right ventricle31.5CFC1, GDF1
3situs inversus31.4ZIC3, GDF1
4congenital heart disease31.1GATA4, ZIC3
5tetralogy of fallot31.0NKX2-5, GDF1
6atrioventricular block30.9NKX2-5, GATA4
7atrioventricular septal defect30.7NKX2-5, CRELD1, GATA4
8myocardial infarction30.4GATA4, ANXA5, ACE
9congenitally corrected transposition of the great arteries11.5
10left ventricular outflow tract obstruction11.0
11hypertension10.9
12dextrocardia10.9
13tricuspid atresia10.8
14aortopulmonary window10.7
15cor triatriatum10.7
16subpulmonary stenosis10.7
17aneurysm10.7
18transposition of the great arteries, dextro-looped 110.7
19hypoplastic left heart syndrome10.6
20transposition of the great arteries, dextro-looped 210.6
21right aortic arch10.6
22patent foramen ovale10.5
23aortic coarctation10.5
24brain injury10.5
25wolff-parkinson-white syndrome10.5
26tuberous sclerosis10.5
27cerebritis10.5
28communicating hydrocephalus10.5
29hydrocephalus10.5
30complete atrioventricular canal10.5
31pulmonary vein stenosis10.5
32transposition of great arteries, dextro-looped 310.5
33isolated congenitally uncorrected transposition of the great arteries10.5
34congenitally uncorrected transposition of the great arteries with cardiac malformation10.5
35criss-cross heart10.5
36congenitally uncorrected transposition of the great arteries with coarctation10.5
37accessory mitral valve tissue10.5
38transient global amnesia10.4
39klinefelter's syndrome10.3
40citrullinemia10.3
41endocardial fibroelastosis10.3
42scimitar syndrome10.3
43intestinal atresia10.3
44imperforate anus10.3
45biliary atresia10.3
46renal agenesis10.3
47twin-to-twin transfusion syndrome10.3
48congenital diaphragmatic hernia10.3
49sick sinus syndrome10.3
50subclavian steal syndrome10.3

Graphical network of the top 20 diseases related to Transposition of the Great Arteries:



Diseases related to transposition of the great arteries

Symptoms for Transposition of the Great Arteries

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Drugs & Therapeutics for Transposition of the Great Arteries

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Transposition of the Great Arteries

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Anatomical Context for Transposition of the Great Arteries

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34MalaCards
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MalaCards organs/tissues related to Transposition of the Great Arteries:

34
Heart, Lung, Skin, Brain, Liver, Testes, Atrioventricular node, Pituitary

Animal Models for Transposition of the Great Arteries or affiliated genes

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38MGI
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Publications for Transposition of the Great Arteries

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53PubMed
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Articles related to Transposition of the Great Arteries:

(show top 50)    (show all 639)
idTitleAuthorsYear
1
Earlier Arterial Switch Operation Improves Outcomes and Reduces Costs for Neonates with Transposition of the Great Arteries. (24184243)
2013
2
The phenotypic spectrum of ZIC3 mutations includes isolated d-transposition of the great arteries and double outlet right ventricle. (23427188)
2013
3
Slow pathway ablation using electro-anatomical mapping in a patient with corrected transposition of the great arteries, dextrocardia, and atrioventricular nodal reentrant tachycardia. (22764200)
2013
4
Cardiac catheterisation of patients with common arterial trunk and transposition of the great arteries. (23331588)
2012
5
Autonomic nervous system function in infants with transposition of the great arteries. (21613339)
2012
6
Complete atrioventricular block in an adult with congenitally corrected transposition of the great arteries with interrupted inferior vena cava. (23798952)
2012
7
Atrioventricular node reentrant tachycardia in patients with congenitally corrected transposition of the great arteries and results of radiofrequency catheter ablation. (23159417)
2012
8
Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries. (22551964)
2012
9
Percutaneous edge-to-edge repair of tricuspid regurgitation in congenitally corrected transposition of the great arteries. (21269414)
2011
10
Perioperative care of a child with transposition of the great arteries. (21706195)
2011
11
The modified Senning procedure as an integral part of an anatomical correction of congenitally corrected transposition of the great arteries. (24414198)
2011
12
Editorial comment Corrected transposition of the great arteries: is 'anatomic' repair the optimal option? (21515064)
2011
13
The role of the Fontan operation in the treatment of congenitally corrected transposition of the great arteries. (21976866)
2011
14
Myocardial perfusion and exercise capacity 12 years after arterial switch surgery for D-transposition of the great arteries. (21479909)
2011
15
Long-term outcomes after an arterial switch operation for simple complete transposition of the great arteries. (20111649)
2010
16
Do predictors exist for a successful withdrawal of preoperative prostaglandin E(1) from neonates with d-transposition of the great arteries and intact ventricular septum? (20886209)
2010
17
The right ventricular response to high afterload: comparison between healthy persons and patients with transposition of the great arteries: a 2D strain study. (20618391)
2010
18
Congenitally corrected transposition of the great arteries presenting in a nonagenarian. (20805436)
2010
19
A case of platypnea-orthodeoxia syndrome caused by the interaction between the presence of corrected transposition of the great arteries and patent foramen ovale. (18706706)
2010
20
Comparison of Simpson's method and three-dimensional reconstruction for measurement of right ventricular volume in patients with complete or corrected transposition of the great arteries. (20494670)
2010
21
A reminder from the past: a 49-year-old patient with transposition of the great arteries palliated with Dr. Thomas Baffes' and Dr. William Mustard's original operations. (19647403)
2009
22
Congenitally corrected transposition of the great arteries plus dextrocardia operated with an unusual operative technique. (18704862)
2008
23
Enlarged bronchial collateral artery complicating recovery after arterial switch for simple transposition of the great arteries. (18757452)
2008
24
Intraoperative coronary artery pulse Doppler patterns in patients with complete transposition of the great arteries undergoing the arterial switch operation. (18760127)
2008
25
Double aortic arch and d-transposition of the great arteries. (18186786)
2008
26
Cardiac defibrillation therapy for at risk patients with systemic right ventricular dysfunction secondary to atrial redirection surgery for dextro-transposition of the great arteries. (17383987)
2007
27
Metabolic alterations and neurodevelopmental outcome of infants with transposition of the great arteries. (16897317)
2006
28
That balloon atrial septostomy is associated with preoperative stroke in neonates with transposition of the great arteries is another powerful argument in favor of therapeutic closure of every patent foramen ovale. (16828611)
2006
29
Primary surgical correction of transposition of the great arteries and atrioventricular septal defect associated with left isomerism. (16391973)
2006
30
A case of transposition of the great arteries in a female infant of a HIV-1-infected woman. Potential teratogenic effects of antiretroviral drugs. (16941823)
2006
31
Ablation of atrioventricular conduction in corrected transposition of the great arteries. (16648068)
2006
32
Successful repair of transposition of the great arteries with right aortic arch and coarctation of the aorta. (16482932)
2006
33
Relation between right ventricular structural alterations and markers of adverse clinical outcome in adults with systemic right ventricle and either congenital complete (after Senning operation) or congenitally corrected transposition of the great arteries. (17056346)
2006
34
The arterial switch operation in Europe for transposition of the great arteries: a multi-institutional study from the European Congenital Heart Surgeons Association. (16935120)
2006
35
Wolff-Parkinson-white syndrome in d-transposition of the great arteries and mustard baffle. (16818221)
2006
36
Arterial switch operation for transposition of the great arteries combined with closure of paramembranous ventricular septal defect using the Amplatzer membranous ventricular septal occluder. (16098318)
2005
37
Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries. (15831655)
2005
38
Prenatal diagnosis of congenitally corrected transposition of the great arteries. A case report. (15608453)
2005
39
Repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction. (15082275)
2004
40
Combined arterial switch and Senning operation for congenitally corrected transposition of the great arteries: patient selection and intermediate results. (12658191)
2003
41
Neurocutaneous melanosis with transposition of the great arteries and renal agenesis. (12869156)
2003
42
Apico-pulmonary artery conduit repair of congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary outflow tract obstruction: a 10-year follow-up. (14602256)
2003
43
Outcome of 121 patients with congenitally corrected transposition of the great arteries. (11889523)
2002
44
Congenitally corrected transposition of the great arteries in a 65-year-old woman. (11804307)
2001
45
Transposition of the great arteries, pulmonary atresia, and multiple ventricular septal defects associated with multiple cardiac rhabdomyomas in a case of tuberous sclerosis. (10754091)
2000
46
Systemic ventricular inflow obstruction in congenitally corrected transposition of the great arteries. (9130433)
1997
47
Congenitally corrected transposition of the great arteries in the adult: functional status and complications. (8609349)
1996
48
Congenitally corrected transposition of the great arteries and exercise-induced ventricular tachycardia. (8640601)
1996
49
Tricuspid atresia with transposition of the great arteries in adolescents and adults: current state and late complications. (3342144)
1988
50
The Mustard procedure in transposition of the great arteries associated with juxtaposition of the atrial appendages with and without dextrocardia. (6827853)
1983

Variations for Transposition of the Great Arteries

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1 National Center for Biotechnology Information (Clinvar)
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Clinvar genetic disease variations for Transposition of the Great Arteries:

1
id Gene Name Type Significance SNP ID Assembly Location
1MED13LNM_015335.4(MED13L): c.752A> G (p.Glu251Gly)single nucleotide variantPathogenicrs28940309GRCh37Chr 12, 116457651: 116457651
2MED13LNM_015335.4(MED13L): c.5615G> A (p.Arg1872His)single nucleotide variantPathogenicrs28940310GRCh37Chr 12, 116413092: 116413092
3MED13LNM_015335.4(MED13L): c.6068A> G (p.Asp2023Gly)single nucleotide variantPathogenicrs121918333GRCh37Chr 12, 116406902: 116406902

Expression for genes affiliated with Transposition of the Great Arteries

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Transposition of the Great Arteries

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Pathways for genes affiliated with Transposition of the Great Arteries

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51PathCards, 56Reactome, 54QIAGEN, 39NCBI BioSystems Database, 31KEGG
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Compounds for genes affiliated with Transposition of the Great Arteries

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46Novoseek, 52PharmGKB, 12DrugBank, 30IUPHAR, 25HMDB
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Compounds related to Transposition of the Great Arteries according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1taurolidine469.6IL6, ANXA5
2zoledronic acid469.5ANXA5, IL6
3triptolide469.5IL6, ANXA5
4methylcellulose469.3ANXA5, IL6
5infliximab46 52 1211.1IL6, ANXA5
6fluvastatin46 52 30 1212.1IL6, ACE, ANXA5
7zinc46 2510.0ACE, ZIC3, NKX2-5, GATA4
8nitric oxide46 25 1210.6GATA4, NKX2-5, IL6, ACE
9retinoic acid46 259.2GATA4, IL6, MED24, ANXA5

GO Terms for genes affiliated with Transposition of the Great Arteries

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17Gene Ontology
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Cellular components related to Transposition of the Great Arteries according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1mediator complexGO:0165929.4MED24, MED13L
2external side of plasma membraneGO:0098978.6IL6, ACE, ANXA5

Biological processes related to Transposition of the Great Arteries according to GeneCards/GeneDecks:

(show all 15)
idNameGO IDScoreTop Affiliating Genes
1pulmonary myocardium developmentGO:00335010.0PITX2, NKX2-5
2positive regulation of cardioblast differentiationGO:05189110.0GATA4, NKX2-5
3endocardial cushion developmentGO:0031979.9GATA4, CRELD1
4ventricular cardiac muscle cell developmentGO:0550159.9PITX2, NKX2-5
5atrial septum morphogenesisGO:0604139.9NKX2-5, GATA4
6ventricular septum morphogenesisGO:0604129.8PITX2, NKX2-5
7embryonic digestive tract morphogenesisGO:0485579.8GATA4, PITX2
8determination of left/right symmetryGO:0073689.8ZIC3, CFC1, PITX2
9spleen developmentGO:0485369.8NKX2-5, PITX2
10BMP signaling pathwayGO:0305099.7GATA4, NKX2-5
11heart loopingGO:0019479.7GATA4, NKX2-5, ZIC3
12vasculogenesisGO:0015709.5NKX2-5, PITX2
13response to mechanical stimulusGO:0096129.3GATA4, IL6
14positive regulation of transcription from RNA polymerase II promoterGO:0459448.6PITX2, IL6, ZIC3, NKX2-5, GATA4
15positive regulation of transcription, DNA-templatedGO:0458938.3GATA4, NKX2-5, ZIC3, IL6, MED24

Molecular functions related to Transposition of the Great Arteries according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1transcription factor bindingGO:0081349.0PITX2, NKX2-5, GATA4
2sequence-specific DNA binding transcription factor activityGO:0037009.0GATA4, NKX2-5, ZIC3, PITX2
3RNA polymerase II transcription cofactor activityGO:0011049.0NKX2-5, MED24, MED13L

Products for genes affiliated with Transposition of the Great Arteries

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Sources for Transposition of the Great Arteries

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet