DTGA
MCID: TRN044
MIFTS: 41

Transposition of the Great Arteries (DTGA)

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Transposition of the Great Arteries

MalaCards integrated aliases for Transposition of the Great Arteries:

Name: Transposition of the Great Arteries 53 29 6
Transposition of the Great Arteries, Dextro-Looped 1 73
Dextro-Looped Transposition of the Great Arteries 53
Dtga 53

Classifications:



External Ids:

UMLS 73 C1837341

Summaries for Transposition of the Great Arteries

NIH Rare Diseases : 53 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.

MalaCards based summary : Transposition of the Great Arteries, also known as transposition of the great arteries, dextro-looped 1, is related to dextro-looped transposition of the great arteries and double outlet right ventricle. An important gene associated with Transposition of the Great Arteries is MED13L (Mediator Complex Subunit 13 Like). The drugs Eplerenone and Spironolactone have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and skin, and related phenotypes are Decreased Aire reporter expression and cardiovascular system

Related Diseases for Transposition of the Great Arteries

Diseases related to Transposition of the Great Arteries via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 131)
# Related Disease Score Top Affiliating Genes
1 dextro-looped transposition of the great arteries 33.6 GDF1 MED13L ZIC3
2 double outlet right ventricle 32.2 GDF1 NKX2-5 ZIC3
3 conotruncal heart malformations 31.9 GDF1 NKX2-5
4 ventricular septal defect 30.8 GDF1 NKX2-5 ZIC3
5 tetralogy of fallot 30.2 GDF1 NKX2-5
6 atrial heart septal defect 30.1 GDF1 NKX2-5
7 atrioventricular septal defect 30.0 GDF1 NKX2-5
8 patent foramen ovale 29.9 MED13L NKX2-5
9 heart disease 29.9 GDF1 MED13L NKX2-5 ZIC3
10 heterotaxy 29.9 CFAP53 ZIC3
11 tricuspid valve disease 29.4 GDF1 NKX2-5 ZIC3
12 congenitally corrected transposition of the great arteries 13.0
13 transposition of the great arteries, dextro-looped 1 12.9
14 dextro-looped transposition of the great arteries 3 12.7
15 isolated congenitally uncorrected transposition of the great arteries 12.5
16 congenitally uncorrected transposition of the great arteries with cardiac malformation 12.5
17 congenitally uncorrected transposition of the great arteries with coarctation 12.5
18 congenital heart defects, multiple types, 6 12.3
19 double discordia 11.8
20 mental retardation and distinctive facial features with or without cardiac defects 11.4
21 heterotaxy, visceral, 1, x-linked 11.2
22 heterotaxy, visceral, 6, autosomal 11.2
23 congenital heart defects, multiple types, 4 11.2
24 congenital heart defects, multiple types, 5 11.2
25 situs inversus 10.6
26 dextrocardia 10.6
27 pulmonary hypertension 10.6
28 tricuspid atresia 10.5
29 dextrocardia with situs inversus 10.4
30 aortic coarctation 10.4
31 single ventricular heart 10.4
32 aortopulmonary window 10.4
33 cardiac conduction defect 10.3
34 pulmonary hypertension, primary, 1 10.3
35 hypoplastic left heart syndrome 1 10.3
36 hypoplastic left heart syndrome 10.3
37 cor triatriatum 10.3
38 subpulmonary stenosis 10.3
39 diaphragmatic hernia, congenital 10.2
40 myocardial infarction 10.2
41 brain injury 10.2
42 atrioventricular block 10.2
43 endocarditis 10.2
44 univentricular heart 10.2
45 right aortic arch 10.2
46 tricuspid valve insufficiency 10.2
47 complete atrioventricular canal 10.2
48 criss-cross heart 10.2
49 aortic valve disease 1 10.1
50 wolff-parkinson-white syndrome 10.1

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



Diseases related to Transposition of the Great Arteries

Symptoms & Phenotypes for Transposition of the Great Arteries

GenomeRNAi Phenotypes related to Transposition of the Great Arteries according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased Aire reporter expression GR00304-A 8.62 MED13L NKX2-5

MGI Mouse Phenotypes related to Transposition of the Great Arteries:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.46 CFAP53 GDF1 NKX2-5 ZIC3
2 embryo MP:0005380 9.26 CFAP53 GDF1 NKX2-5 ZIC3
3 respiratory system MP:0005388 8.92 CFAP53 GDF1 NKX2-5 ZIC3

Drugs & Therapeutics for Transposition of the Great Arteries

Drugs for Transposition of the Great Arteries (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 55)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Eplerenone Approved Phase 4 107724-20-9 443872 150310
2
Spironolactone Approved Phase 4 52-01-7, 1952-01-7 5833
3
Mechlorethamine Approved, Investigational Phase 4 51-75-2 4033
4 Hormones Phase 4
5 Mineralocorticoids Phase 4
6 Hormone Antagonists Phase 4
7 Diuretics, Potassium Sparing Phase 4
8 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
9 diuretics Phase 4
10 Mineralocorticoid Receptor Antagonists Phase 4
11 Natriuretic Agents Phase 4
12
Nitric Oxide Approved Phase 3 10102-43-9 145068
13
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
14 Vasodilator Agents Phase 3,Phase 2
15 Free Radical Scavengers Phase 3,Phase 2
16 Antioxidants Phase 3,Phase 2
17 Neurotransmitter Agents Phase 3,Phase 1
18 Respiratory System Agents Phase 3,Phase 2
19 Peripheral Nervous System Agents Phase 3,Phase 1
20 Protective Agents Phase 3,Phase 2
21 Phosphodiesterase Inhibitors Phase 3,Phase 2
22 Autonomic Agents Phase 3
23 Anti-Asthmatic Agents Phase 3
24 Endothelium-Dependent Relaxing Factors Phase 3
25 Bronchodilator Agents Phase 3
26 Phosphodiesterase 5 Inhibitors Phase 3
27
Acetylcysteine Approved, Investigational Phase 2 616-91-1 12035
28 Simendan Investigational Phase 2 131741-08-7
29 Hematinics Phase 1, Phase 2
30 Epoetin alfa Phase 1, Phase 2 113427-24-0
31 Cardiotonic Agents Phase 2
32 Anti-Arrhythmia Agents Phase 2
33 Antiviral Agents Phase 2
34 Pharmaceutical Solutions Phase 2
35 Expectorants Phase 2
36 Antidotes Phase 2
37 Anti-Infective Agents Phase 2
38 N-monoacetylcystine Phase 2
39 Dextrans Phase 2
40 Anticoagulants Phase 2
41 Blood Substitutes Phase 2
42 Plasma Substitutes Phase 2
43
Dexmedetomidine Approved, Vet_approved Phase 1 113775-47-6 68602 5311068
44 Adrenergic alpha-Agonists Phase 1
45 Hypnotics and Sedatives Phase 1
46 Adrenergic Agents Phase 1
47 Central Nervous System Depressants Phase 1
48 Adrenergic alpha-2 Receptor Agonists Phase 1
49 Analgesics, Non-Narcotic Phase 1
50 Adrenergic Agonists Phase 1

Interventional clinical trials:

(show all 42)
# Name Status NCT ID Phase Drugs
1 Eplerenone in Systemic Right Ventricle Completed NCT00703352 Phase 4 Eplerenone
2 The Effects of Eplerenone on Markers of Myocardial Fibrosis in Adult Congenital Heart Disease Terminated NCT01971593 Phase 4 Eplerenone
3 Comparison of Hematocrit Levels in Infant Heart Surgery Completed NCT00006183 Phase 3
4 Infant Heart Surgery: Central Nervous System Sequelae of Circulatory Arrest Completed NCT00000470 Phase 3
5 Nitric Oxide During Bypass for Arterial Switch Operation Recruiting NCT03661385 Phase 3 Nitric Oxide
6 Effect of Phosphodiesterase-5 Inhibition With Tadalafil on SystEmic Right VEntricular Size and Function Recruiting NCT03049540 Phase 3 Tadalafil 20 MG;Placebo 20 MG
7 Erythropoetin Neuroprotection for Neonatal Cardiac Surgery Unknown status NCT00513240 Phase 1, Phase 2 Erythropoetin;Normal saline
8 Levosimendan Administration in Neonates With Transposition of the Great Arteries Completed NCT01120106 Phase 2 levosimendan;placebo
9 N-Acetylcysteine in Neonatal Congenital Heart Surgery (INACT Study) Completed NCT00374088 Phase 2 N-acetylcysteine;Placebo
10 Hypertonic Saline Dextran in Pediatric Cardiac Surgery Completed NCT00199771 Phase 2 7.5% NaCl in 6% dextran 70 solution
11 Autologous Umbilical Cord Blood Treatment of Neonate With CHD Not yet recruiting NCT03558269 Phase 1, Phase 2
12 A Phase I Study of Dexmedetomidine Bolus and Infusion in Corrective Infant Cardiac Surgery: Safety and Pharmacokinetics Completed NCT01915277 Phase 1 Dexmedetomidine
13 Cardiovascular MRI and Cardiopulmonary Exercise Capacity After Neonatal ASO) in Young Adults Unknown status NCT02415491 Not Applicable
14 Comparative Assessment of Coronary MR Angiography Vs Coronary Multislice CT After Coronary Artery Reimplantation in Children and Young Adults. Unknown status NCT02542345 Not Applicable
15 Assessing Neurodevelopment in Congenital Heart Disease. Unknown status NCT02996630 Not Applicable
16 T1 Mapping of Diffuse Myocardial Fibrosis in Congenital Heart Disease Unknown status NCT02350829 Not Applicable
17 Late Function After Surgery for Transposition of the Great Arteries Completed NCT03078413
18 MRI Study After Arterial Switch Operation in Patients With Transposition of the Great Arteries Completed NCT01916499
19 Effects of Beta-adrenergic in Adults w/Transposition of Great Arteries on Systemic Ventricular Function Completed NCT00313352
20 Physical Training in Transposition of the Great Arteries Completed NCT00837603 Not Applicable
21 CT Coronary Angiography After Arterial Switch Operation Completed NCT01153776 Not Applicable
22 Right Ventricular Hypertrophy After Atrial Switch and Cardiovascular Magnetic Resonance (CMR) Findings Completed NCT01430897
23 Myocardial Contrast Echocardiography in Congenital Heart Disease Completed NCT00412685
24 Assessment of Left and Right Ventricular Function in Patients With Ebstein Anomaly With Different Echocardiographic Methods Completed NCT01907971
25 Quality of Life in Young Adults With Congenital Heart Disease Completed NCT02463292
26 Prenatal Effects of Congenital Heart Disease (CHD) on Neurodevelopmental Outcome Completed NCT00713635
27 Reproduction and Survival After Cardiac Defect Repair Completed NCT00005190
28 Genes Causing Ebstein's Anomaly Completed NCT00497705
29 A Randomized Controlled Trial:Treatments on Infundibular Ventricular Septal Defect Completed NCT02361008 Not Applicable
30 Characterization of the Cardiac Reinnervation of Patients With Transposition of the Great Arteries Long After Repair With the Arterial Switch Operation. Correlation With Electrocardiographic and Exercise Test Parameters Recruiting NCT03469843
31 Fibrosis, Valvular and Ventricular Function in Patients With TGA Recruiting NCT02588989
32 Acute Maternal Hyperoxygenation for Fetal Transposition of the Great Arteries (TGA) Recruiting NCT03771534 Not Applicable Oxygen gas
33 Genetic Determinants of Congenital Heart Disease Outcomes Recruiting NCT01656941
34 Haemodynamics and Function of the Atria in Congenital Heart Disease by Cardiovascular Magnetic Resonance Recruiting NCT02161471
35 The Boston Circulatory Arrest Study: Antecedents and Correlates of Well-Being in Adults With Congenital Heart Disease Recruiting NCT03073122
36 Oxygen Consumption-based Assessments of Hemodynamics in Neonates Following Congenital Heart Surgery (Oxy-CAHN Study) Recruiting NCT02184169 Not Applicable
37 A Pilot Study- Monitoring Cerebral Blood Flow in Neonates With Congenital Heart Defects Recruiting NCT03512613
38 Xeltis Bioabsorbable Pulmonary Valved Conduit Early Feasibility Study Recruiting NCT03022708 Not Applicable
39 Initiation of Resuscitation While Attached to the Cord With Congenital Heart Disease Recruiting NCT03690245 Not Applicable
40 Surgical Planning for Reconstruction of Complex Heart Defects Recruiting NCT00972608
41 Study to Assess Safety of the Pulmonary Valved Conduit (PV-001) in Subjects Undergoing Right Ventricular Outflow Tract Reconstruction Active, not recruiting NCT02700100 Not Applicable
42 COMPASSION S3 - Evaluation of the SAPIEN 3 Transcatheter Heart Valve in Patients With Pulmonary Valve Dysfunction Active, not recruiting NCT02744677 Not Applicable

Search NIH Clinical Center for Transposition of the Great Arteries

Genetic Tests for Transposition of the Great Arteries

Genetic tests related to Transposition of the Great Arteries:

# Genetic test Affiliating Genes
1 Transposition of the Great Arteries 29

Anatomical Context for Transposition of the Great Arteries

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

41
Heart, Lung, Skin, Brain, Testes, Kidney, Pituitary

Publications for Transposition of the Great Arteries

Articles related to Transposition of the Great Arteries:

(show top 50) (show all 1141)
# Title Authors Year
1
Effect of prenatal diagnosis on hospital costs in complete transposition of the great arteries. ( 29675828 )
2018
2
Long-Term Outcomes of Tricuspid Valve Surgery in Patients With Congenitally Corrected Transposition of the Great Arteries. ( 29874165 )
2018
3
Left ventricular remodelling in long-term survivors after the arterial switch operation for transposition of the great arteries. ( 29800129 )
2018
4
Catheter ablation of manifest posteroseptal accessory pathway associated with coronary sinus diverticula in a child with congenitally corrected transposition of the great arteries. ( 29424737 )
2018
5
Role of myocardial collagen degradation and fibrosis in right ventricle dysfunction in transposition of the great arteries after atrial switch. ( 29433967 )
2018
6
Aorto-Right Ventricular Tunnel in Transposition of the Great Arteries. ( 29515986 )
2018
7
Analysis of DICER1 in familial and sporadic cases of transposition of the great arteries. ( 29399970 )
2018
8
Airway ciliary dysfunction and respiratory symptoms in patients with transposition of the great arteries. ( 29444099 )
2018
9
Rhythm disturbances and treatment strategies in children with congenitally corrected transposition of the great arteries. ( 29476598 )
2018
10
Attenuation of Pulse Pressure Amplification inA Patients with Complete Transposition of the Great Arteries After an Arterial Switch Operation in Children. ( 29923135 )
2018
11
Association of Habitual Activity and Body Mass Index in Survivors of Congenital Heart Surgery: A Study of Children and Adolescents With Tetralogy of Fallot, Transposition of the Great Arteries, and Fontan Palliation. ( 29544424 )
2018
12
Late diagnosis of a congenitally corrected transposition of the great arteries discovered at pacemaker implantation in a patient previously diagnosed with dextrocardia and situs solitus. ( 29881577 )
2018
13
Two Brothers With Dextro-Transposition of the Great Arteries. ( 29848184 )
2018
14
Aortopulmonary collaterals in neonates with d-transposition of the great arteries - Clinical significance early after arterial switch operation. ( 29444748 )
2018
15
The Use of Transesophageal Echocardiography in the Management of Baffle Leaks in a Patient With Transposition of the Great Arteries. ( 29851692 )
2018
16
Two-stage arterial switch for late-presenting transposition of the great arteries. ( 29912349 )
2018
17
Coronary sinus ostial atresia with persistent left superior caval vein in a patient with congenitally corrected transposition of the great arteries. ( 29233231 )
2018
18
Outcomes of the anatomical repair in patients with congenitally corrected transposition of the great arteries: lessons learned in a high-volume centre. ( 29566142 )
2018
19
Contemporary management and outcomes in congenitally corrected transposition of the great arteries. ( 29326110 )
2018
20
Right ventricular dysfunction in congenitally corrected transposition of the great arteries and risk of ventricular tachyarrhythmia and sudden death. ( 29463466 )
2018
21
A Rare Case of Coronary Artery Embolism in a Patient with d-Transposition of the Great Arteries with Prior Mustard Repair. ( 29657908 )
2018
22
An Analysis of Left Ventricular Retraining in Patients With Dextro- and Levo-Transposition of the Great Arteries. ( 29274314 )
2018
23
Effects of eplerenone on markers of myocardial fibrosis, 6-minute walk distance, and quality of life in adults with tetralogy of Fallot and complete transposition of the great arteries. ( 29686545 )
2018
24
Left ventricular function and exercise capacity after arterial switch operation for transposition of the great arteries: a systematic review and meta-analysis. ( 29848397 )
2018
25
Primary Arterial Switch Operation for Late Referral of Transposition of the Great Arteries with Intact Ventricular Septum in the Current Era: Do We Still Need a Rapid Two-Stage Operation? ( 29310554 )
2018
26
Case 3 / 2018 - Corrected Transposition of the Great Arteries with Natural Progression to Severe Biventricular Dysfunction and No Associated Defects in a 51-Year Old Man. ( 29898048 )
2018
27
Cerebral oximetry monitoring in the management of severe hypoxaemia associated with transposition of the great arteries with balloon atrial septostomy. ( 29366495 )
2018
28
<i>Bartonella</i> endocarditis with glomerulonephritis in a patient with complete transposition of the great arteries. ( 29686571 )
2018
29
Interpretation and Reporting of Coronary Arteries in Transposition of the Great Arteries: Cross-sectional Imaging Perspective. ( 29927871 )
2018
30
Congenitally Corrected Transposition of the Great Arteries in a Septuagenarian from the Developing Country of Pakistan. ( 30087813 )
2018
31
Implantation techniques and outcomes after cardiac resynchronization therapy for congenitally corrected transposition of the great arteries. ( 30125719 )
2018
32
A Rare Combination of Congenitally Corrected Transposition of the Great Arteries with Pulmonary Artery Aneurysm. ( 30370391 )
2018
33
Senning Procedure for Transposition of the Great Arteries in a Patient with Situs Inversus Totalis and Dextrocardia. ( 30173681 )
2018
34
A large posterior encephalocele associated with severe ventriculomegaly, cerebellar atrophy and transposition of the great arteries. ( 30105815 )
2018
35
Transposition of the great arteries: A laterality defect in the group of heterotaxy syndromes or an outflow tract malformation? ( 30271012 )
2018
36
Perioperative Cerebral Oxygenation Metabolism in Neonates with Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries. ( 30167749 )
2018
37
Acute Pulmonary Edema in a Pregnant Patient with Undiagnosed Levo-Transposition of the Great Arteries. ( 30518743 )
2018
38
Afterload dependence of right ventricular myocardial deformation: A comparison between tetralogy of Fallot and atrially corrected transposition of the great arteries in adult patients. ( 30261015 )
2018
39
The effect of the introduction of the three-vessel view on the detection rate of transposition of the great arteries and tetralogy of Fallot. ( 30132937 )
2018
40
Preoperative Coronary Anatomy Assessment with Echocardiography and Morbidity After Arterial Switch Operation of Transposition of the Great Arteries. ( 30003297 )
2018
41
Late Results of Half-Turned Truncal Switch Operation for Transposition of the Great Arteries. ( 30031843 )
2018
42
Implantable cardioverter defibrillator therapy in grown-up patients with transposition of the great arteries-role of anti-tachycardia pacing. ( 30034851 )
2018
43
Assessment of cardiac function in infants with transposition of the great arteries after surgery: comparison of two methods. ( 30062649 )
2018
44
Rotation of the outflow tracts for complex transposition of the great arteries. ( 30070785 )
2018
45
Longitudinal strain by two-dimensional speckle tracking to assess ventricular function in adults with transposition of the great arteries: Can serial assessment be simplified? ( 30122596 )
2018
46
Bridge to Transplantation With Long-Term Mechanical Assist Devices in Adults With Transposition of the Great Arteries. ( 30129258 )
2018
47
Two-dimensional speckle tracking strain in follow-up of adults with transposition of the great arteries: Should it be used in daily clinical practice? ( 30143349 )
2018
48
Transposition of the great arteries: When echocardiography does not match the clinical picture. ( 30160304 )
2018
49
Long-Term Survival After Arterial Versus Atrial Switch in d-Transposition of the Great Arteries. ( 30172857 )
2018
50
Clinical value of chromosomal microarray analysis in prenatally diagnosed dextro-transposition of the great arteries. ( 30176760 )
2018

Variations for Transposition of the Great Arteries

Expression for Transposition of the Great Arteries

Search GEO for disease gene expression data for Transposition of the Great Arteries.

Pathways for Transposition of the Great Arteries

GO Terms for Transposition of the Great Arteries

Biological processes related to Transposition of the Great Arteries according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 multicellular organism development GO:0007275 9.43 CFAP53 NKX2-5 ZIC3
2 BMP signaling pathway GO:0030509 9.16 GDF1 NKX2-5
3 determination of left/right symmetry GO:0007368 8.96 CFAP53 ZIC3
4 heart looping GO:0001947 8.62 NKX2-5 ZIC3

Sources for Transposition of the Great Arteries

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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