D-TGA
MCID: DXT004
MIFTS: 29

Dextro-Looped Transposition of the Great Arteries (D-TGA)

Categories: Cardiovascular diseases, Fetal diseases, Genetic diseases, Rare diseases

Aliases & Classifications for Dextro-Looped Transposition of the Great Arteries

MalaCards integrated aliases for Dextro-Looped Transposition of the Great Arteries:

Name: Dextro-Looped Transposition of the Great Arteries 12 15
Ventriculoarterial Discordance with Atrioventricular Concordance 12 59
Congenitally Uncorrected Transposition of the Great Arteries 12 59
Congenitally Uncorrected Transposition of the Great Vessels 12 59
Isolated Ventriculoarterial Discordance 12 59
Transposition of the Great Arteries, Dextro-Looped 1 73
Arteries, Great, Transposition, Dextro-Looped 40
D-Tga 12

Characteristics:

Orphanet epidemiological data:

59
congenitally uncorrected transposition of the great arteries
Inheritance: Multigenic/multifactorial,Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: Infancy,Neonatal;

Classifications:



External Ids:

Disease Ontology 12 DOID:0060770
ICD10 33 Q20.3
Orphanet 59 ORPHA860
ICD10 via Orphanet 34 Q20.3
UMLS 73 C1837341

Summaries for Dextro-Looped Transposition of the Great Arteries

Disease Ontology : 12 A congenital heart disease characterized by complete inversion of the great vessels where the aorta incorrectly arises from the right ventricle and the pulmonary artery incorrectly arises from the left ventricle.

MalaCards based summary : Dextro-Looped Transposition of the Great Arteries, also known as ventriculoarterial discordance with atrioventricular concordance, is related to isolated congenitally uncorrected transposition of the great arteries and transposition of the great arteries. An important gene associated with Dextro-Looped Transposition of the Great Arteries is GDF1 (Growth Differentiation Factor 1), and among its related pathways/superpathways are Developmental Biology and Mesodermal Commitment Pathway. The drugs Eplerenone and Spironolactone have been mentioned in the context of this disorder. Affiliated tissues include heart, and related phenotypes are cardiovascular system and embryo

Related Diseases for Dextro-Looped Transposition of the Great Arteries

Diseases in the Dextro-Looped Transposition of the Great Arteries family:

Transposition of the Great Arteries, Dextro-Looped 1 Dextro-Looped Transposition of the Great Arteries 3

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

(show all 26)
# Related Disease Score Top Affiliating Genes
1 isolated congenitally uncorrected transposition of the great arteries 34.0 CFC1 ZIC3
2 transposition of the great arteries 32.5 GDF1 MED13L ZIC3
3 ventricular septal defect 29.9 GDF1 NKX2-6 ZIC3
4 dextro-looped transposition of the great arteries 3 13.0
5 congenitally uncorrected transposition of the great arteries with cardiac malformation 12.6
6 congenitally uncorrected transposition of the great arteries with coarctation 12.6
7 transposition of the great arteries, dextro-looped 1 12.3
8 tricuspid atresia 10.1 GDF1 NKX2-6
9 total anomalous pulmonary venous return 1 10.1 GDF1 NKX2-6
10 ebstein anomaly 10.1 GDF1 NKX2-6
11 aortic coarctation 10.0
12 aortopulmonary window 10.0
13 conotruncal heart malformations 10.0 GDF1 NKX2-6
14 pulmonary valve insufficiency 10.0 LEFTY2 NKX2-6
15 right atrial isomerism 10.0 GDF1 LEFTY2
16 heterotaxy 10.0 CFC1 ZIC3
17 patent ductus arteriosus 1 10.0 GDF1 NKX2-6
18 atrioventricular septal defect 10.0 GDF1 NKX2-6
19 heart septal defect 9.9 CFC1 GDF1 NKX2-6
20 atrial heart septal defect 9.9 CFC1 GDF1 NKX2-6
21 mediastinitis 9.9 NKX2-6 RERE
22 tricuspid valve disease 9.9 GDF1 NKX2-6 ZIC3
23 patent foramen ovale 9.9 MED13L NKX2-6
24 double outlet right ventricle 9.7 CFC1 GDF1 NKX2-6 ZIC3
25 heart disease 9.6 CFC1 GDF1 MED13L NKX2-6 ZIC3
26 visceral heterotaxy 9.5 CFC1 GDF1 LEFTY2 NKX2-6 ZIC3

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



Diseases related to Dextro-Looped Transposition of the Great Arteries

Symptoms & Phenotypes for Dextro-Looped Transposition of the Great Arteries

MGI Mouse Phenotypes related to Dextro-Looped Transposition of the Great Arteries:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.85 FOXH1 GDF1 LEFTY2 NKX2-6 RERE ZIC3
2 embryo MP:0005380 9.73 FOXH1 GDF1 LEFTY2 NKX2-6 RERE ZIC3
3 growth/size/body region MP:0005378 9.63 FOXH1 GDF1 LEFTY2 NKX2-6 RERE ZIC3
4 craniofacial MP:0005382 9.62 FOXH1 GDF1 RERE ZIC3
5 normal MP:0002873 9.35 FOXH1 GDF1 NKX2-6 RERE ZIC3
6 respiratory system MP:0005388 9.02 FOXH1 GDF1 LEFTY2 NKX2-6 ZIC3

Drugs & Therapeutics for Dextro-Looped Transposition of the Great Arteries

Drugs for Dextro-Looped 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 Myocardial Contrast Echocardiography in Congenital Heart Disease Completed NCT00412685
23 Right Ventricular Hypertrophy After Atrial Switch and Cardiovascular Magnetic Resonance (CMR) Findings Completed NCT01430897
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 Reproduction and Survival After Cardiac Defect Repair Completed NCT00005190
27 Prenatal Effects of Congenital Heart Disease (CHD) on Neurodevelopmental Outcome Completed NCT00713635
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 Dextro-Looped Transposition of the Great Arteries

Genetic Tests for Dextro-Looped Transposition of the Great Arteries

Anatomical Context for Dextro-Looped Transposition of the Great Arteries

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

41
Heart

Publications for Dextro-Looped Transposition of the Great Arteries

Variations for Dextro-Looped Transposition of the Great Arteries

Expression for Dextro-Looped Transposition of the Great Arteries

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

Pathways for Dextro-Looped Transposition of the Great Arteries

Pathways related to Dextro-Looped Transposition of the Great Arteries according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.64 CFC1 FOXH1 GDF1 LEFTY2 MED13L ZIC3
2
Show member pathways
11.81 FOXH1 LEFTY2 ZIC3
3 11.02 FOXH1 LEFTY2
4
Show member pathways
10.62 CFC1 FOXH1 GDF1 LEFTY2

GO Terms for Dextro-Looped Transposition of the Great Arteries

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

# Name GO ID Score Top Affiliating Genes
1 multicellular organism development GO:0007275 9.55 CFC1 LEFTY2 NKX2-6 RERE ZIC3
2 transforming growth factor beta receptor signaling pathway GO:0007179 9.48 FOXH1 LEFTY2
3 BMP signaling pathway GO:0030509 9.46 CFC1 GDF1
4 cell development GO:0048468 9.43 GDF1 LEFTY2
5 heart looping GO:0001947 9.4 FOXH1 ZIC3
6 regulation of MAPK cascade GO:0043408 9.37 GDF1 LEFTY2
7 SMAD protein signal transduction GO:0060395 9.32 GDF1 LEFTY2
8 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.26 GDF1 LEFTY2
9 anterior/posterior pattern specification GO:0009952 9.13 CFC1 FOXH1 ZIC3
10 determination of left/right symmetry GO:0007368 8.8 CFC1 FOXH1 ZIC3

Molecular functions related to Dextro-Looped Transposition of the Great Arteries according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transforming growth factor beta receptor binding GO:0005160 8.96 GDF1 LEFTY2
2 sequence-specific DNA binding GO:0043565 8.92 FOXH1 NKX2-6 RERE ZIC3

Sources for Dextro-Looped 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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