TGA
MCID: DXT004
MIFTS: 54

Dextro-Looped Transposition of the Great Arteries (TGA)

Categories: Cardiovascular diseases, Fetal diseases, Genetic diseases, Rare diseases, Respiratory 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 52 15
Transposition of the Great Arteries 52 58 29 6
Ventriculoarterial Discordance with Atrioventricular Concordance 12 52 58
Congenitally Uncorrected Transposition of the Great Vessels 12 52 58
Congenitally Uncorrected Transposition of the Great Arteries 12 58
Isolated Ventriculoarterial Discordance 12 58
Transposition of the Great Arteries, Dextro-Looped 1 71
Arteries, Great, Transposition, Dextro-Looped 39
Dextro-Transposition of the Great Arteries 58
Ventriculoarterial Discordance, Isolated 52
D-Transposition of the Great Arteries 58
Transposition of the Great Vessels 58
Complete Transposition 58
D-Tga 12
Dtga 52
Tga 58
Tgv 58

Characteristics:

Orphanet epidemiological data:

58
congenitally uncorrected transposition of the great arteries
Inheritance: Multigenic/multifactorial,Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: Infancy,Neonatal;
transposition of the great arteries
Inheritance: Multigenic/multifactorial,Not applicable; Prevalence: 1-5/10000 (Europe),1-5/10000 (United States),1-5/10000 (Belgium),1-5/10000 (Denmark),1-5/10000 (France),1-5/10000 (Germany),1-5/10000 (Hungary),1-5/10000 (Ireland),1-5/10000 (Italy),1-5/10000 (Netherlands),1-5/10000 (Norway),1-5/10000 (Poland),1-9/100000 (Portugal),1-5/10000 (Spain),1-5/10000 (Switzerland),1-5/10000 (United Kingdom),1-5/10000 (Ukraine); Age of onset: Infancy,Neonatal;

Classifications:

Orphanet: 58  
Rare cardiac malformations
Developmental anomalies during embryogenesis


External Ids:

Disease Ontology 12 DOID:0060770
ICD10 32 Q20.3
ICD10 via Orphanet 33 Q20.3 Q20.5
UMLS via Orphanet 72 C0040761
UMLS 71 C1837341

Summaries for Dextro-Looped Transposition of the Great Arteries

NIH Rare Diseases : 52 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 : Dextro-Looped Transposition of the Great Arteries, also known as transposition of the great arteries, is related to isolated congenitally uncorrected transposition of the great arteries and situs inversus. An important gene associated with Dextro-Looped Transposition of the Great Arteries is MED13L (Mediator Complex Subunit 13L), and among its related pathways/superpathways are Developmental Biology and Mesodermal Commitment Pathway. The drugs Mechlorethamine and Eplerenone have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and brain, and related phenotypes are Decreased viability and Decreased viability

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.

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 top 50) (show all 335)
# Related Disease Score Top Affiliating Genes
1 isolated congenitally uncorrected transposition of the great arteries 35.0 ZIC3 CFC1
2 situs inversus 31.7 PKD1L1 DNAI1 DNAH5 DNAH11 ARMC4
3 dextrocardia with situs inversus 31.2 PKD1L1 NODAL
4 heterotaxy 31.2 ZIC3 NODAL CFC1
5 heart, malformation of 30.5 TBX5 NKX2-5
6 tricuspid atresia 30.2 ZIC3 TBX5 NKX2-5 GATA4
7 aortic valve disease 1 30.1 TBX5 NKX2-5 GDF1 GATA4
8 tricuspid valve disease 30.0 ZIC3 TBX5 NPPB NKX2-5
9 aortic valve disease 2 30.0 TBX5 NPPB NKX2-5 GATA4
10 heart valve disease 29.9 NPPB NKX2-5 GATA4
11 atrioventricular block 29.9 TBX5 NPPB NKX2-5 GATA4
12 eisenmenger syndrome 29.8 NKX2-5 GATA4
13 ventricular septal defect 29.7 ZIC3 TBX5 NPPB NODAL NKX2-5 GDF1
14 heart septal defect 29.6 ZIC3 TBX5 PITX2 NKX2-5 LEFTY2 GATA4
15 wolff-parkinson-white syndrome 29.6 TBX5 NPPB NKX2-5
16 digeorge syndrome 29.3 TBX5 PITX2 NKX2-5 GATA4
17 pulmonary valve stenosis 29.3 TBX5 NKX2-5 LEFTY2 GATA4 CFC1
18 patent foramen ovale 29.3 TBX5 NPPB NKX2-5 MED13L GATA4 CRELD1
19 ebstein anomaly 29.3 ZIC3 TBX5 NPPB NODAL NKX2-5 GATA4
20 total anomalous pulmonary venous return 1 29.2 ZIC3 TBX5 NODAL NKX2-5 GATA4 CRELD1
21 patent ductus arteriosus 1 29.1 ZIC3 TBX5 NPPB NKX2-5 LEFTY2 GATA4
22 atrial heart septal defect 28.6 ZIC3 TBX5 PITX2 NPPB NODAL NKX2-5
23 double outlet right ventricle 28.6 ZIC3 TBX5 PITX2 NODAL NKX2-5 LEFTY2
24 hypoplastic left heart syndrome 28.5 ZIC3 TBX5 NPPB NODAL NKX2-5 GATA4
25 atrioventricular septal defect 28.5 ZIC3 TBX5 NODAL NKX2-5 LEFTY2 GDF1
26 dextrocardia 28.4 ZIC3 NODAL LEFTY2 DNAI1 DNAH5 DNAH11
27 heart disease 28.3 ZIC3 TBX5 PITX2 NPPB NODAL NKX2-5
28 tetralogy of fallot 27.8 ZIC3 TBX5 PITX2 NPPB NODAL NKX2-5
29 visceral heterotaxy 27.5 ZIC3 PKD1L1 PITX2 NODAL LEFTY2 GDF1
30 primary ciliary dyskinesia 27.2 ZIC3 PKD1L1 PITX2 NODAL LEFTY2 DNAI1
31 congenitally corrected transposition of the great arteries 13.1
32 dextro-looped transposition of the great arteries 3 13.1
33 congenitally uncorrected transposition of the great arteries with cardiac malformation 12.8
34 transposition of the great arteries and conotruncal cardiac anomaly 12.6
35 transposition of the great arteries, dextro-looped 1 12.4
36 congenital heart defects, multiple types, 6 12.4
37 transient global amnesia 12.0
38 conotruncal heart malformations 11.9
39 mental retardation and distinctive facial features with or without cardiac defects 11.9
40 critical congenital heart disease 11.7
41 heterotaxy, visceral, 1, x-linked 11.7
42 heterotaxy, visceral, 5, autosomal 11.7
43 multicentric osteolysis, nodulosis, and arthropathy 11.4
44 heterotaxy, visceral, 6, autosomal 11.4
45 congenital heart defects, multiple types, 4 11.4
46 congenital heart defects, multiple types, 5 11.4
47 med13l haploinsufficiency syndrome 11.4
48 yorifuji okuno syndrome 11.4
49 amnestic disorder 10.5
50 epilepsy, progressive myoclonic, 8 10.5 GDF1 CERS1

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

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

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00240-S-1 10.03 NODAL
2 Decreased viability GR00381-A-1 10.03 NODAL
3 Decreased viability GR00381-A-2 10.03 NODAL
4 Decreased viability GR00381-A-3 10.03 NODAL
5 Decreased viability GR00402-S-2 10.03 ARMC4 CERS1 CFC1 CRELD1 DAW1 DNAH11
6 no effect GR00402-S-1 9.62 ARMC4 CERS1 CFC1 CRELD1 DAW1 DNAH11

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

45 (show all 15)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.42 ARMC4 CRELD1 DNAH11 DNAH5 DNAI1 FOXH1
2 growth/size/body region MP:0005378 10.35 ARMC4 CERS1 DNAH11 DNAH5 DNAI1 FOXH1
3 cellular MP:0005384 10.31 ARMC4 CERS1 CRELD1 DNAH11 DNAH5 DNAI1
4 digestive/alimentary MP:0005381 10.29 ARMC4 DNAH11 DNAH5 FOXH1 GATA4 GDF1
5 embryo MP:0005380 10.27 CRELD1 DNAH11 FOXH1 GATA4 GDF1 LEFTY2
6 hematopoietic system MP:0005397 10.27 ARMC4 CERS1 DNAH11 DNAH5 DNAI1 FOXH1
7 mortality/aging MP:0010768 10.27 ARMC4 CERS1 CRELD1 DNAH11 DNAH5 DNAI1
8 craniofacial MP:0005382 10.22 CRELD1 DNAH11 DNAH5 DNAI1 FOXH1 GDF1
9 immune system MP:0005387 10.17 ARMC4 DNAH11 DNAH5 DNAI1 FOXH1 GDF1
10 nervous system MP:0003631 10.1 ARMC4 CERS1 CRELD1 DNAH5 DNAI1 FOXH1
11 liver/biliary system MP:0005370 10.01 DNAH11 DNAH5 GATA4 GDF1 LEFTY2 NODAL
12 muscle MP:0005369 9.92 ARMC4 DNAH11 FOXH1 GATA4 NKX2-5 NODAL
13 normal MP:0002873 9.9 DNAH11 DNAI1 FOXH1 GATA4 GDF1 NKX2-5
14 respiratory system MP:0005388 9.77 ARMC4 DNAH11 DNAH5 DNAI1 FOXH1 GATA4
15 skeleton MP:0005390 9.32 DNAH11 DNAH5 DNAI1 FOXH1 GATA4 GDF1

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 54)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Mechlorethamine Approved, Investigational Phase 4 51-75-2 4033
2
Eplerenone Approved Phase 4 107724-20-9 150310 443872
3 Hormone Antagonists Phase 4
4 Antihypertensive Agents Phase 4
5 Mineralocorticoids Phase 4
6 Mineralocorticoid Receptor Antagonists Phase 4
7 Natriuretic Agents Phase 4
8 Diuretics, Potassium Sparing Phase 4
9 Hormones Phase 4
10 diuretics Phase 4
11
Nitric Oxide Approved Phase 3 10102-43-9 145068
12
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
13 Hemostatics Phase 2, Phase 3
14 Vasoconstrictor Agents Phase 2, Phase 3
15 Arginine Vasopressin Phase 2, Phase 3
16 Vasopressins Phase 2, Phase 3
17 Coagulants Phase 2, Phase 3
18 Neurotransmitter Agents Phase 3
19 Respiratory System Agents Phase 3
20 Free Radical Scavengers Phase 3
21 Antioxidants Phase 3
22 Protective Agents Phase 3
23 Anti-Asthmatic Agents Phase 3
24 Autonomic Agents Phase 3
25 Bronchodilator Agents Phase 3
26 Vasodilator Agents Phase 3
27 Phosphodiesterase 5 Inhibitors Phase 3
28 Phosphodiesterase Inhibitors Phase 3
29
Arginine Investigational, Nutraceutical Phase 2, Phase 3 74-79-3 6322
30
Acetylcysteine Approved, Investigational Phase 2 616-91-1 12035
31
Simendan Investigational Phase 2 131741-08-7
32 N-monoacetylcystine Phase 2
33 Anti-Infective Agents Phase 2
34 Pharmaceutical Solutions Phase 2
35 Antiviral Agents Phase 2
36 Expectorants Phase 2
37 Antidotes Phase 2
38 Phosphodiesterase 3 Inhibitors Phase 2
39
Dexmedetomidine Approved, Vet_approved Phase 1 113775-47-6 68602 5311068
40 Adrenergic Agents Phase 1
41 Adrenergic Agonists Phase 1
42 Adrenergic alpha-2 Receptor Agonists Phase 1
43 Hypnotics and Sedatives Phase 1
44 Analgesics, Non-Narcotic Phase 1
45 Central Nervous System Depressants Phase 1
46 Analgesics Phase 1
47
Dobutamine Approved 34368-04-2 36811
48
Calcium Approved, Nutraceutical 7440-70-2 271
49 Adrenergic beta-Antagonists
50 Adrenergic Antagonists

Interventional clinical trials:

(show all 46)
# Name Status NCT ID Phase Drugs
1 Aldosterone Antagonists in Systemic Right Ventricle: a Randomized Clinical Trial. Completed NCT00703352 Phase 4 Eplerenone
2 Eplerenone to Prevent Myocardial Fibrosis in Congenital Heart Disease Terminated NCT01971593 Phase 4 Eplerenone
3 Hematocrit Strategy in Infant Heart Surgery Completed NCT00006183 Phase 3
4 Use of Arginine Vasopressin in Early Postoperative Management After Fontan Palliation Completed NCT03088345 Phase 2, Phase 3 Vasopressin, Arginine;Placebos
5 A Phase III Single-Blind, Randomized, Placebo Controlled, Clinical Trial to Determine the Safety and Efficacy of Intravenous L-Citrulline Versus Placebo in Children Undergoing Cardiopulmonary Bypass Completed NCT00335244 Phase 3 L-citrulline;Placebo of intravenous L-citrulline
6 Infant Heart Surgery: Central Nervous System Sequelae of Circulatory Arrest Completed NCT00000470 Phase 3
7 Multicenter, Double-Blind, Placebo-Controlled Study to Evaluate Efficacy/Safety of CY-1503 (Cylexin) in Prevention of Reperfusion Injury in Neonates/Infants Undergoing Hypothermic Cardiopulmonary Bypass Completed NCT00226369 Phase 2, Phase 3 CY-1503
8 A Randomised Controlled Trial of Nitric Oxide Administration During Cardiopulmonary Bypass in Infants Undergoing Arterial Switch Operation for Repair of Transposition of the Great Arteries Recruiting NCT03661385 Phase 3 Nitric Oxide
9 Effect of Phosphodiesterase-5 Inhibition With Tadalafil on SystEmic Right VEntricular Size and Function - a Multi-center, Double-blind, Randomized, Placebo-controlled Clinical Trial - SERVE Trial Active, not recruiting NCT03049540 Phase 3 Tadalafil 20 MG;Placebo 20 MG
10 Attenuation of Myocardial Dysfunction by N-Acetylcysteine in Infants Undergoing Arterial Switch Procedure Completed NCT00374088 Phase 2 N-acetylcysteine;Placebo
11 Perioperative Levosimendan Administration in Neonates With Transposition of the Great Arteries: Randomized Controlled Trial Completed NCT01120106 Phase 2 levosimendan;placebo
12 Phase I/II Clinical Trial to Determine the Pharmacokinetics and Safety Profile of Citrulline in Children Undergoing Cardiopulmonary Bypass Completed NCT00201214 Phase 1, Phase 2 Citrulline
13 Phase IB Double Blind, Randomized, Placebo Controlled Clinical Trial to Determine the Pharmacokinetics and Safety of a Revised Protocol of Intravenous L-Citrulline (Citrupress®) Versus Placebo in Children Undergoing Cardiopulmonary Bypass Completed NCT01120964 Phase 1, Phase 2 Intravenous L-Citrulline;Placebo of Intravenous L-Citrulline
14 A Phase I Study of Dexmedetomidine Bolus and Infusion in Corrective Infant Cardiac Surgery: Safety and Pharmacokinetics Completed NCT01915277 Phase 1 Dexmedetomidine
15 Cardiovascular Magnetic Resonance at Rest and Stress in Relation to Cardiopulmonary Exercise Capacity in Young Adults After Arterial Switch Operation (ASO) for Transposition of the Great Arteries (TGA) Unknown status NCT02415491
16 Comparative Assessment of Coronary MR Angiography Vs Coronary Multislice CT After Coronary Artery Reimplantation in Children and Young Adults. Unknown status NCT02542345
17 Right Ventricular Hypertrophy After Atrial Switch Operation and Its Role in a Systemic RV: Normal Adaptation Process or Risk Factor? A Cardiovascular Magnetic Resonance Study Completed NCT01430897
18 Effects of Beta-adrenergic Blocking Agents in Adult Patients With Transposition of the Great Arteries on Systemic Ventricular Function Completed NCT00313352
19 Left Ventricular Function in Ebstein Anomaly: Assessment by 3D Echocardiography and Speckle Tracking. Assessment and Validation of the Right Ventricle by VentriPoint Method. Completed NCT01907971
20 Study of Adaptation of the Right Ventricle to Systemic Afterload Completed NCT02066506
21 MRI Study After Arterial Switch Operation in Patients With Transposition of the Great Arteries Completed NCT01916499
22 Follow up of Adults With Congenitally Malformed Hearts With Focus on Computer-Based Education and Psychosocial Support Completed NCT01234753
23 CT Coronary Angiography for the Detection of Reimplanted Coronary Lesions in Patients Who Had Undergone Arterial Switch Operation for Transposition of the Great Arteries Completed NCT01153776
24 Late Function After Surgery for Transposition of the Great Arteries Completed NCT03078413
25 Family Study of Congenital Cardiovascular Malformations Completed NCT00005258
26 Pulmonic SAPIEN XT™ THV A Multi-center, Observational Registry With Retrospective Enrollment of Patients That Underwent Transcatheter Pulmonic Valve Implantation and a Retrospective or Prospective Follow-up Completed NCT02302131
27 Pacemaker Therapy in Adults With Congenital Heart Defects Completed NCT00303511
28 Oxygen Consumption-based Assessments of Hemodynamics in Neonates Following Congenital Heart Surgery (Oxy-CAHN Study) Completed NCT02184169
29 Reproduction and Survival After Cardiac Defect Repair Completed NCT00005190
30 SPY Imaging System: Its Role in Pediatric Cardiac Surgery Completed NCT00446927
31 Biventricular Pacing in Children With Wide QRS After Surgery for Congenital Heart Disease Completed NCT02644824
32 Influence of Physical Training on Cardiopulmonary Exercise Capacity and Right Ventricular Function in Patients With D-TGA and Atrial Switch Operation. Completed NCT00837603
33 Evaluation of an Oral Health Intervention Program for Children With Congenital Heart Defects Completed NCT03311438
34 Sudden Cardiac Death in Congenital Heart Diseases With a Systemic Right Ventricle Recruiting NCT03833843
35 Fibrosis, Valvular and Ventricular Function in Patients With Transposition of the Great Arteries Recruiting NCT02588989
36 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
37 COngenital Multicenter Trial of Pulmonic vAlve Dysfunction Studying the SAPIEN 3 interventIONal THV Recruiting NCT02744677
38 Transposition of the Great Arteries: Prenatal Anatomical and Hemodynamic Findings Associated With Perinatal Outcomes Recruiting NCT03771534 Oxygen gas
39 Congenital Heart Disease (CHD): Hemodynamic Effects of Acute Maternal Hyperoxygenation in the Fetus Recruiting NCT03944837
40 The GECHO Trial: Genetic Determinants of Congenital Heart Disease Outcomes Active, not recruiting NCT01656941
41 Haemodynamics and Function of the Atria in Patients With Congenital Heart Defects - Pilot Study by Cardiovascular Magnetic Resonance Active, not recruiting NCT02161471
42 A Feasibility Study to Evaluate the Safety and Short-term Effectiveness of Implantation of Transcatheter Pulmonary Valve (TPV) for the Treatment of Congenital Heart Disease With Pulmonary Valve Disease Active, not recruiting NCT02555319
43 Pulmonic SAPIEN S3™ THV Registry A Multi-center, Observational Registry With Retrospective Enrollment of Patients That Underwent Transcatheter Pulmonic Valve Implantation and a Retrospective or Prospective Follow-up Active, not recruiting NCT02777892
44 Evaluation Of Performance & Complications of Pacemakers in Children Not yet recruiting NCT04163770
45 The Retrospective Analysis of Presenting Pulse Oximetry Readings and Hourly Variation in Oximetry Readings in Patients With Known Congenital Heart Disease Terminated NCT00261989
46 A Review of the Diagnosis, Treatment and Outcomes of Children With Critical Congenital Heart Defects Terminated NCT00208689

Search NIH Clinical Center for Dextro-Looped Transposition of the Great Arteries

Genetic Tests for Dextro-Looped Transposition of the Great Arteries

Genetic tests related to Dextro-Looped Transposition of the Great Arteries:

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

Anatomical Context for Dextro-Looped Transposition of the Great Arteries

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

40
Heart, Lung, Brain, Skin, Testes, Kidney, Liver

Publications for Dextro-Looped Transposition of the Great Arteries

Articles related to Dextro-Looped Transposition of the Great Arteries:

(show top 50) (show all 4477)
# Title Authors PMID Year
1
Loss-of-function mutations in growth differentiation factor-1 (GDF1) are associated with congenital heart defects in humans. 61 6
17924340 2007
2
Missense mutations and gene interruption in PROSIT240, a novel TRAP240-like gene, in patients with congenital heart defect (transposition of the great arteries). 61 6
14638541 2003
3
Contribution of rare inherited and de novo variants in 2,871 congenital heart disease probands. 6
28991257 2017
4
Recessively inherited right atrial isomerism caused by mutations in growth/differentiation factor 1 (GDF1). 6
20413652 2010
5
Right atrial isomerism in four siblings. 6
14648004 2004
6
Left Ventricular Stiffness in Adolescents and Young Adults After Arterial Switch Operation for Complete Transposition of the Great Arteries. 61
32006085 2020
7
Editorial for "Neopulmonary Outflow Tract Obstruction Assessment by 4D Flow Magnetic Resonance Imaging in Adults With Transposition of the Great Arteries After Arterial Switch Operation". 61
32034825 2020
8
Congenitally corrected transposition of the great arteries: is it really a transposition? An anatomical study of the right ventricular septal surface. 61
31657020 2020
9
A unique indication for the senning procedure: Atrioventricular discordance with ventriculoarterial concordance. 61
31793037 2020
10
Implantable pulmonary artery pressure monitoring device in patients with palliated congenital heart disease: Technical considerations and procedural outcomes. 61
31609082 2020
11
A case of Fontan-related hepatocellular carcinoma successfully treated with proton beam therapy. 61
31273674 2020
12
The Cardiac Genome Clinic: implementing genome sequencing in pediatric heart disease. 61
32037394 2020
13
Newborn pulse oximetry screening in the context of a high antenatal detection rate of critical congenital heart disease. 61
31332832 2020
14
Assessment and management of heart failure in the systemic right ventricle. 61
31970551 2020
15
Unusual association of transposition of great arteries with infradiaphragmatic pulmonary venous return. 61
32030039 2020
16
Comprehensive Functional Echocardiographic Assessment of Transposition of the Great Arteries: From Fetus to Newborn. 61
31919591 2020
17
Response to Evaluation of coronary artery variations using dual-source coronary computed tomography angiography in neonates with transposition of the great arteries. 61
31342268 2020
18
Myocardial blood flow and cardiac sympathetic innervation in young adults late after arterial switch operation for transposition of the great arteries. 61
31337551 2020
19
Heart rate response during exercise predicts exercise tolerance in adults with transposition of the great arteries and atrial switch operation. 61
31405586 2020
20
Prenatal Diagnosis and Outcome of Congenital Corrected Transposition of the Great Arteries - A Multicenter Report of 69 Cases. 61
31995816 2020
21
Global longitudinal strain correlates to systemic right ventricular function. 61
31987049 2020
22
Superior vena caval thrombosis after a neonatal arterial switch procedure. 61
32030040 2020
23
Epidural anesthesia for emergency cesarean section in a woman with Fontan circulation: A case report. 61
31977915 2020
24
Surgical repair of peripheral pulmonary artery stenosis in patients without williams or alagille syndromes. 61
31958550 2020
25
Attention-deficit/hyperactivity disorder symptoms in children with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot. 61
31928549 2020
26
Failure of Cellularization of Ventriculotomy Patch Leading to Right Ventricular Pseudoaneurysm. 61
31755355 2020
27
EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Echocardiographic assessment of transposition of the great arteries and congenitally corrected transposition of the great arteries 61
31729212 2019
28
Prenatal diagnosis of congenitally corrected transposition of the great arteries. 61
32021715 2019
29
Dextro-transposition of the great arteries: switching the switch. 61
31993361 2019
30
Mechanisms for heart failure in systemic right ventricle. 61
31853794 2019
31
[Pediatric coronary artery surgery. A new area in cardiac surgery?] 61
31786939 2019
32
Dual-source computed tomography coronary artery imaging in children. 61
31440884 2019
33
Prognostic power of anaerobic threshold parameters in patients with transposition of the great arteries and systemic right ventricle. 61
31623696 2019
34
Neopulmonary Outflow Tract Obstruction Assessment by 4D Flow MRI in Adults With Transposition of the Great Arteries After Arterial Switch Operation. 61
31794141 2019
35
Ventricular assist device implantation in patients with a failing systemic right ventricle: a call to expand current practice. 61
31420818 2019
36
A situs solitus transposition of great arteries with obstructed sub-diaphragmatic totally anomalous pulmonary venous connection: a rare case treated with anatomical repair. 61
31663489 2019
37
Partial Sternectomy to Prevent Rastelli Conduit Compression in Patients With Conotruncal Anomalies. 61
31152732 2019
38
The Benefits and Bias in Neurodevelopmental Evaluation for Children with Congenital Heart Disease. 61
31865442 2019
39
Quantitative assessment of technical performance during hands-on surgical training of the arterial switch operation using 3-dimensional printed heart models. 61
31983523 2019
40
Brain Injury in Infants with Critical Congenital Heart Disease: Insights from Two Clinical Cohorts with Different Practice Approaches. 61
31451185 2019
41
Development and validation of a procedure-specific assessment tool for hands-on surgical training in congenital heart surgery. 61
31973896 2019
42
Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge. 61
31596322 2019
43
Human Milk Use in the Preoperative Period Is Associated with a Lower Risk for Necrotizing Enterocolitis in Neonates with Complex Congenital Heart Disease. 61
31561958 2019
44
Systematic Evaluation of Systemic Right Ventricular Function. 61
31906129 2019
45
Results of a phase 1 multicentre investigation of dexmedetomidine bolus and infusion in corrective infant cardiac surgery. 61
31623840 2019
46
Arrhythmias in Adults With Congenital Heart Disease: What the Practicing Cardiologist Needs to Know. 61
31703824 2019
47
Late Survival and Patient-Perceived Health Status of the Congenital Heart Surgeons' Society dextro-Transposition of the Great Arteries Cohort. 61
31348901 2019
48
Arterial switch operation for transposition of the great arteries: A single-centre 32-year experience. 61
31508848 2019
49
Is Fetal Intelligent Navigation Echocardiography Helpful in Screening for d-Transposition of the Great Arteries? 61
31675129 2019
50
Repairing transposition of the great arteries: history of the challenge. 61
31771944 2019

Variations for Dextro-Looped Transposition of the Great Arteries

ClinVar genetic disease variations for Dextro-Looped Transposition of the Great Arteries:

6 ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 46;XY;t(2;14)(p22;q24.3)dnTranslocation Likely pathogenic 268037

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

GO Terms for Dextro-Looped Transposition of the Great Arteries

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

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.97 NPPB NODAL LEFTY2 GDF1 DNAI1 DNAH5
2 cell projection GO:0042995 9.88 PKD1L1 DNAI1 DNAH5 DNAH11 DAW1 ARMC4
3 axoneme GO:0005930 9.54 DNAH5 DNAH11 ARMC4
4 outer dynein arm GO:0036157 9.26 DNAI1 DNAH5
5 9+2 motile cilium GO:0097729 9.16 DNAH5 DNAH11
6 dynein complex GO:0030286 9.13 DNAI1 DNAH5 DNAH11
7 cilium GO:0005929 9.1 PKD1L1 DNAI1 DNAH5 DNAH11 DAW1 ARMC4

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

(show all 36)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 10.16 ZIC3 TBX5 PITX2 NODAL NKX2-5 GATA4
2 multicellular organism development GO:0007275 10.15 ZIC3 TBX5 PITX2 NODAL NKX2-5 LEFTY2
3 positive regulation of transcription, DNA-templated GO:0045893 10.06 ZIC3 TBX5 NKX2-5 GATA4 FOXH1
4 anterior/posterior pattern specification GO:0009952 9.83 ZIC3 NODAL FOXH1 CFC1
5 lung development GO:0030324 9.82 ZIC3 TBX5 NODAL
6 microtubule-based movement GO:0007018 9.81 DNAI1 DNAH5 DNAH11
7 flagellated sperm motility GO:0030317 9.79 DNAI1 DNAH5 DNAH11
8 BMP signaling pathway GO:0030509 9.78 NODAL LEFTY2 GDF1 CFC1
9 regulation of MAPK cascade GO:0043408 9.77 NODAL LEFTY2 GDF1
10 SMAD protein signal transduction GO:0060395 9.77 NODAL LEFTY2 GDF1
11 cell development GO:0048468 9.76 NODAL LEFTY2 GDF1
12 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.75 NODAL LEFTY2 GDF1
13 cardiac muscle tissue development GO:0048738 9.67 NKX2-5 GATA4
14 cardiac muscle cell differentiation GO:0055007 9.67 TBX5 NKX2-5 GATA4
15 embryonic heart tube development GO:0035050 9.66 NODAL NKX2-5
16 cardiac septum development GO:0003279 9.66 DNAH11 CRELD1
17 ventricular trabecula myocardium morphogenesis GO:0003222 9.65 NKX2-5 FOXH1
18 negative regulation of androgen receptor signaling pathway GO:0060766 9.65 NODAL FOXH1
19 left/right axis specification GO:0070986 9.64 PKD1L1 PITX2
20 cardiac right ventricle morphogenesis GO:0003215 9.64 GATA4 FOXH1
21 nodal signaling pathway GO:0038092 9.63 NODAL CFC1
22 epithelial cilium movement GO:0003351 9.63 DNAI1 DNAH5 DNAH11
23 cardiac ventricle morphogenesis GO:0003208 9.62 NKX2-5 GATA4
24 cilium movement GO:0003341 9.62 DNAI1 DNAH5 DNAH11 ARMC4
25 outer dynein arm assembly GO:0036158 9.61 DNAI1 DNAH5 ARMC4
26 atrial septum morphogenesis GO:0060413 9.58 TBX5 NKX2-5 GATA4
27 regulation of cilium beat frequency GO:0003356 9.57 DNAH11 ARMC4
28 nodal signaling pathway involved in determination of lateral mesoderm left/right asymmetry GO:1900164 9.56 NODAL FOXH1
29 heart development GO:0007507 9.56 TBX5 NODAL NKX2-5 DNAI1 DNAH5 DNAH11
30 embryonic heart tube anterior/posterior pattern specification GO:0035054 9.55 GATA4 FOXH1
31 heart looping GO:0001947 9.55 ZIC3 NODAL NKX2-5 GATA4 FOXH1
32 bundle of His development GO:0003166 9.54 TBX5 NKX2-5
33 endocardial cushion development GO:0003197 9.54 TBX5 GATA4 CRELD1
34 determination of left/right asymmetry in nervous system GO:0035545 9.51 ZIC3 DNAH11
35 positive regulation of cardioblast differentiation GO:0051891 9.43 TBX5 NKX2-5 GATA4
36 determination of left/right symmetry GO:0007368 9.28 ZIC3 PITX2 NODAL FOXH1 DNAI1 DNAH5

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 DNA-binding transcription factor activity, RNA polymerase II-specific GO:0000981 9.93 ZIC3 TBX5 PITX2 NKX2-5 GATA4 FOXH1
2 DNA-binding transcription factor activity GO:0003700 9.88 ZIC3 TBX5 PITX2 NKX2-5 GATA4 FOXH1
3 RNA polymerase II proximal promoter sequence-specific DNA binding GO:0000978 9.72 ZIC3 TBX5 PITX2 NKX2-5 GATA4
4 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 9.65 ZIC3 TBX5 NKX2-5 GATA4 FOXH1
5 sequence-specific DNA binding GO:0043565 9.63 ZIC3 TBX5 PITX2 NKX2-5 GATA4 FOXH1
6 motor activity GO:0003774 9.61 DNAI1 DNAH5 DNAH11
7 ATP-dependent microtubule motor activity, minus-end-directed GO:0008569 9.48 DNAH5 DNAH11
8 co-SMAD binding GO:0070410 9.37 GATA4 FOXH1
9 growth factor activity GO:0008083 9.26 NODAL LEFTY2 GDF1 CERS1
10 transforming growth factor beta receptor binding GO:0005160 8.8 NODAL LEFTY2 GDF1

Sources for Dextro-Looped Transposition of the Great Arteries

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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