MCID: DXT001
MIFTS: 55

Dextrocardia

Categories: Rare diseases, Cardiovascular diseases, Fetal diseases

Aliases & Classifications for Dextrocardia

MalaCards integrated aliases for Dextrocardia:

Name: Dextrocardia 12 72 49 55 28 41 14
Heart Predominantly in Right Hemithorax 12

Classifications:



External Ids:

Disease Ontology 12 DOID:9565
ICD10 32 Q24.0
MeSH 41 D003914
NCIt 46 C84669
SNOMED-CT 64 156925007 27637000
Orphanet 55 ORPHA1666
MESH via Orphanet 42 D003914
UMLS via Orphanet 70 C0011813 C0265863
ICD10 via Orphanet 33 Q24.0
UMLS 69 C0011813

Summaries for Dextrocardia

NIH Rare Diseases : 49 Dextrocardia is a condition in which the heart is located in the right side of the chest instead of the left. It is usually present from birth (congenital). There are several types of dextrocardia.  The simplest type occurs when the shape and structure of the heart is a mirror image of a normal heart.  Other types of dextrocardia may involve defects of the walls of the heart, nearby blood vessels, or other organs in the abdomen. Chest X-raxys and echocardiograms can be used to determine which type of dextrocardia is present.  Last updated: 1/14/2016

MalaCards based summary : Dextrocardia, also known as heart predominantly in right hemithorax, is related to dextrocardia with situs inversus and kartagener syndrome, and has symptoms including t-wave inversion, situs inversus totalis and congenital malformation of the great arteries. An important gene associated with Dextrocardia is NODAL (Nodal Growth Differentiation Factor), and among its related pathways/superpathways are Mesodermal Commitment Pathway and TGF-beta signaling pathway (KEGG). The drugs Spironolactone and diuretics have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and liver, and related phenotypes are growth/size/body region and mortality/aging

Wikipedia : 72 Dextrocardia (from Latin dexter, meaning \"right,\" and Greek kardia, meaning \"heart\") is a rare... more...

Related Diseases for Dextrocardia

Diseases related to Dextrocardia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 145)
# Related Disease Score Top Affiliating Genes
1 dextrocardia with situs inversus 33.7 HES7 NODAL
2 kartagener syndrome 32.0 DNAI1 NODAL
3 situs inversus 31.1 DNAI1 NODAL
4 heterotaxy 30.0 ACVR2B NODAL
5 heart disease 29.0 ACTC1 NODAL TNNT2
6 dextrocardia with unusual facies and microphthalmia 12.1
7 short stature wormian bones dextrocardia 12.1
8 heterotaxy, visceral, 1, x-linked 11.7
9 ciliary dyskinesia, primary, 1 11.5
10 levocardia 11.2
11 heterotaxy, visceral, 5, autosomal 11.2
12 pagod syndrome 11.1
13 heterotaxy, visceral, 4, autosomal 10.9
14 stratton-parker syndrome 10.9
15 van allen myhre syndrome 10.9
16 myocardial infarction 10.3
17 double discordia 10.2
18 transposition of the great arteries 10.2
19 acute myocardial infarction 10.1
20 poland syndrome 10.1
21 ventricular septal defect 10.1
22 atrial heart septal defect 10.1
23 atrial fibrillation 10.0
24 conotruncal heart malformations 10.0
25 congenitally corrected transposition of the great arteries 10.0
26 arteries, anomalies of 9.9
27 progressive familial heart block, type ia 9.9
28 tetralogy of fallot 9.9
29 lung agenesis 9.9
30 coronary artery anomaly 9.9
31 bronchiectasis 9.9
32 visceral heterotaxy 9.9 ACVR2B DNAI1 NODAL
33 transposition of the great arteries, dextro-looped 1 9.9
34 aneurysm 9.9
35 atrial standstill 1 9.9
36 tricuspid atresia 9.9
37 sinusitis 9.9
38 sick sinus syndrome 9.8
39 total anomalous pulmonary venous return 1 9.7
40 cleft palate, isolated 9.7
41 lipomatosis, multiple 9.7
42 tracheoesophageal fistula with or without esophageal atresia 9.7
43 wolff-parkinson-white syndrome 9.7
44 progressive familial heart block, type ib 9.7
45 atrioventricular septal defect 9.7
46 pulmonary hypertension 9.7
47 rheumatic heart disease 9.7
48 diaphragmatic eventration 9.7
49 esophageal atresia 9.7
50 esophagitis 9.7

Graphical network of the top 20 diseases related to Dextrocardia:



Diseases related to Dextrocardia

Symptoms & Phenotypes for Dextrocardia

Human phenotypes related to Dextrocardia:

55 31 (show all 28)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 t-wave inversion 55 31 hallmark (90%) Very frequent (99-80%) HP:0010872
2 situs inversus totalis 55 31 frequent (33%) Frequent (79-30%) HP:0001696
3 congenital malformation of the great arteries 55 31 frequent (33%) Frequent (79-30%) HP:0011603
4 abnormality of the ureter 55 31 occasional (7.5%) Occasional (29-5%) HP:0000069
5 abnormality of the spleen 55 31 occasional (7.5%) Occasional (29-5%) HP:0001743
6 abnormal lung lobation 55 31 occasional (7.5%) Occasional (29-5%) HP:0002101
7 intestinal malrotation 55 31 occasional (7.5%) Occasional (29-5%) HP:0002566
8 abnormality of the pulmonary artery 55 31 occasional (7.5%) Occasional (29-5%) HP:0004414
9 abnormality of abdominal situs 55 31 occasional (7.5%) Occasional (29-5%) HP:0011620
10 abnormal renal morphology 55 31 occasional (7.5%) Occasional (29-5%) HP:0012210
11 hydrocephalus 55 31 very rare (1%) Very rare (<4-1%) HP:0000238
12 preauricular skin tag 55 31 very rare (1%) Very rare (<4-1%) HP:0000384
13 webbed neck 55 31 very rare (1%) Very rare (<4-1%) HP:0000465
14 abnormality of the ribs 55 31 very rare (1%) Very rare (<4-1%) HP:0000772
15 global developmental delay 55 31 very rare (1%) Very rare (<4-1%) HP:0001263
16 congenital hip dislocation 55 31 very rare (1%) Very rare (<4-1%) HP:0001374
17 abnormality of the foot 55 31 very rare (1%) Very rare (<4-1%) HP:0001760
18 meckel diverticulum 55 31 very rare (1%) Very rare (<4-1%) HP:0002245
19 pancreatic hypoplasia 55 31 very rare (1%) Very rare (<4-1%) HP:0002594
20 neuroblastoma 55 31 very rare (1%) Very rare (<4-1%) HP:0003006
21 aplasia/hypoplasia of the ear 55 31 very rare (1%) Very rare (<4-1%) HP:0008771
22 dextrocardia 55 Obligate (100%)
23 abnormal heart morphology 55 Very frequent (99-80%)
24 abnormal ekg 55 Very frequent (99-80%)
25 abnormality of pulmonary situs 55 Occasional (29-5%)
26 abnormal genital system morphology 55 Occasional (29-5%)
27 abnormal pulmonary situs morphology 31 occasional (7.5%) HP:0011615
28 abnormal reproductive system morphology 31 occasional (7.5%) HP:0012243

MGI Mouse Phenotypes related to Dextrocardia:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 9.5 ACTC1 ACVR2B CTC1 DNAI1 HES7 NODAL
2 mortality/aging MP:0010768 9.17 NODAL TNNT2 ACTC1 ACVR2B CTC1 DNAI1

Drugs & Therapeutics for Dextrocardia

Drugs for Dextrocardia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 27)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Spironolactone Approved Phase 4 1952-01-7, 52-01-7 5833
2 diuretics Phase 4
3 Mineralocorticoid Receptor Antagonists Phase 4
4 Mineralocorticoids Phase 4
5 Sodium Channel Blockers Phase 4
6 Natriuretic Agents Phase 4,Phase 3
7 Hormone Antagonists Phase 4
8 Hormones Phase 4
9 Diuretics, Potassium Sparing Phase 4
10 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
11 Natriuretic Peptide, Brain Phase 4,Phase 3
12
Nitroglycerin Approved, Investigational Phase 3 55-63-0 4510
13 Vasodilator Agents Phase 3
14 Pharmaceutical Solutions Phase 2
15 Antihypertensive Agents Phase 2
16
Nitric Oxide Approved 10102-43-9 145068
17
Menthol Approved 2216-51-5 16666
18 Respiratory System Agents
19 Neurotransmitter Agents
20 Peripheral Nervous System Agents
21 Anti-Asthmatic Agents
22 Endothelium-Dependent Relaxing Factors
23 Protective Agents
24 Antioxidants
25 Autonomic Agents
26 Bronchodilator Agents
27 Liver Extracts

Interventional clinical trials:

(show all 38)

# Name Status NCT ID Phase Drugs
1 Effects of Spironolactone on Collagen Metabolism in Patients With Pulmonary Arterial Hypertension Unknown status NCT01468571 Phase 4 Spironolactone;Placebo
2 Water Immersion in Right-Sided Heart Failure: A Pilot Study Completed NCT00654264 Phase 4
3 Spironolactone Therapy in Chronic Stable Right HF Trial Not yet recruiting NCT03344159 Phase 4 Spironolactone;Placebo
4 A Study Comparing Blood Flow and Clinical and Safety Effects of the Addition of Natrecor (Nesiritide), Placebo or Intravenous Nitroglycerin to Standard Care for the Treatment of Worsening Congestive Heart Failure. Completed NCT00270374 Phase 3 nesiritide
5 Ketones in Heart Failure - Effects on Cardiac Efficiency Completed NCT03073356 Phase 2
6 Ambrisentan in Single Ventricle Recruiting NCT02080637 Phase 2 Ambrisentan
7 Clearing Lungs With ENaC Inhibition in Primary Ciliary Dyskinesia Recruiting NCT02871778 Phase 2 VX-371;Hypertonic Saline;Placebo (0.17% saline);Ivacaftor
8 Behaviour of Tricuspid Valve Regurgitation in Volume and/or Pressure Loaded Right Heart Unknown status NCT01444222
9 Early Detection and Characterization of Primary Ciliary Dyskinesia Unknown status NCT01070914
10 Screening for Primary Ciliary Dyskinesia Using Nasal Nitric Oxide Unknown status NCT00739817
11 Comparison of On-line and Off-line Measurements of Exhaled Nitric Oxide (NO) Unknown status NCT00686309
12 Transcatheter Valve Implantation in Patients With Dysfunctional Left and Right Sided Heart Valves Completed NCT02119442
13 Non-invasive Assessment of Liver Stiffness/Fibrosis by Transient Elastography (Fibroscan) in Patients With Left and/or Right Sided Heart Failure Completed NCT00777725
14 Evaluation of Monitoring TECHNOlogies in the PERI-OPerative Care of Cardiac Surgical Patients Completed NCT02658006
15 Portal Flow Pulsatility as a Risk Factor for Acute Kidney Injury After Cardiac Surgery Completed NCT02831907
16 NIOX VERO Nasal Application in Primary Ciliary Dyskinesia Completed NCT02622061
17 Otolith Function in Patients With Primary Ciliary Dyskinesia Completed NCT01246258
18 Inflammatory and Microbiologic Markers in Sputum: Comparing Cystic Fibrosis With Primary Ciliary Dyskinesia Completed NCT01155115
19 Diagnosis of Primary Ciliary Dyskinesia Completed NCT00783887
20 Dyskinesia, Heterotaxy and Congenital Heart Disease Completed NCT00608556
21 Genetic Study of Patients With Primary Ciliary Dyskinesia Completed NCT00005650
22 Molecular Genetics of Heterotaxy and Related Congenital Heart Defects Recruiting NCT02432079
23 Global Congestive Heart Failure Registry Recruiting NCT03078166
24 Respiratory Muscle Strength, Exercise Capacity and Physical Activity Levels in Children Primary Ciliary Dyskinesia Recruiting NCT03370029
25 Multiple Breath Washout, a Clinimetric Dataset Recruiting NCT03320382
26 MRI in Cystic Fibrosis and Primary Ciliary Dyskinesia Recruiting NCT03279965
27 Registry for Primary Ciliary Dyskinesia Recruiting NCT03271840
28 High Resolution Micro OCT Imaging Recruiting NCT03256773
29 International Prospective Patient Registry for Primary Ciliary Dyskinesia (PCD) Recruiting NCT02419365
30 Genetics of Primary Ciliary Dyskinesia Recruiting NCT02389049
31 Pathogenesis of Primary Ciliary Dyskinesia (PCD) Lung Disease Recruiting NCT00807482
32 Open Lung Strategy in Critically Ill Morbid Obese Patients Active, not recruiting NCT02503241
33 Long-term Lung Function and Disease Progression in Children With Early Onset Primary Ciliary Dyskinesia Lung Disease Active, not recruiting NCT00722878
34 Evaluating Progression of and Diagnostic Tools for Primary Ciliary Dyskinesia in Children and Adolescents Active, not recruiting NCT00450918
35 Rare Genetic Disorders of the Breathing Airways Active, not recruiting NCT00323167
36 Registry Study on Primary Ciliary Dyskinesia in Chinese Children Not yet recruiting NCT02704455
37 In Vivo Measurements of Nasal Ciliary Beat Frequency by Using Interferometry Not yet recruiting NCT02699177
38 Chest Physiotherapy and Lung Function in Primary Ciliary Dyskinesia Withdrawn NCT01929356

Search NIH Clinical Center for Dextrocardia

Cochrane evidence based reviews: dextrocardia

Genetic Tests for Dextrocardia

Genetic tests related to Dextrocardia:

# Genetic test Affiliating Genes
1 Dextrocardia 28

Anatomical Context for Dextrocardia

MalaCards organs/tissues related to Dextrocardia:

38
Heart, Lung, Liver, Spleen, Kidney, Skin, Brain

Publications for Dextrocardia

Articles related to Dextrocardia:

(show top 50) (show all 586)
# Title Authors Year
1
Management of scar-related atrial flutter in a patient with dextrocardia, inferior vena cava interruption, and azygos continuation. ( 29424736 )
2018
2
Acute Myocardial Infarction in a Patient with Isolated Dextrocardia. ( 29431312 )
2018
3
Mitral valve replacement via a left thoracotomy in dextrocardia and situs solitus. ( 29338299 )
2018
4
Quantitation in Dextrocardia on myocardial perfusion imaging: how to perform quantitative analysis using Cedars-Sinai software. ( 29442348 )
2018
5
Clipping through a mirror: First reported successful MitraClip in a patient with dextrocardia. ( 29087542 )
2017
6
Assessment of coronary artery disease based on coronary flow velocity reserve by transthoracic Doppler echocardiography and coronary computed tomography angiography in a patient with dextrocardia: a case report. ( 29247297 )
2017
7
Unexpected mirror-image dextrocardia in a patient with ST elevation myocardial infarction. ( 28891179 )
2017
8
Successful left transradial coronary angiography in an elderly patient with dextrocardia and severe aortic stenosis. ( 28529182 )
2017
9
Concomitant surgical treatment of coronary artery disease and coarctation of the aorta in a patient with dextrocardia. ( 29281034 )
2017
10
Dextrocardia: Why Significant Left-Axis Deviation? ( 29061578 )
2017
11
Image Diagnosis: Multivessel Percutaneous Coronary Intervention in Dextrocardia: Success with Usual Techniques in a Case of Mirror-Image Heart. ( 28633731 )
2017
12
Case 4/2017 - Double-Chambered Right Ventricle with Dextrocardia and Hypoxemia Due to Atrial Shunt in a 4-Year-Old Girl. ( 28699979 )
2017
13
Radiofrequency Pulmonary Vein Isolation in a Patient with Dextrocardia With Situs Inversus Totalis. ( 28462889 )
2017
14
Aeroembolism in left atrium during catheter ablation of atrial fibrillation in a patient with dextrocardia: a case report and review of the literature. ( 28577360 )
2017
15
Implantation of VVI pacemaker in a patient with dextrocardia, persistent left superior vena cava, and sick sinus syndrome: A case report. ( 28151908 )
2017
16
Off-pump Coronary Artery Bypass Surgery in a Patient with Dextrocardia and Situs Inversus: Anesthetic, Surgical Consideration and Role of Transesophageal Echocardiography. ( 29184618 )
2017
17
An unusual combination of mirror-image dextrocardia with familial medulloblastoma : Is there a histogenetic relationship? ( 28844926 )
2017
18
Dextrocardia with asymptomatic right atrial appendage aneurysm: a case report. ( 29152268 )
2017
19
Successful Drug-eluting Stent Implantation in a Male Patient with Dextrocardia: A Case Report. ( 29340305 )
2017
20
Acute myocardial infarction in a patient with dextrocardia and successful angioplasty. ( 29296253 )
2017
21
Three-dimensional electroanatomical mapping for non-pulmonary vein foci in a patient with complete situs inversus and dextrocardia. ( 29274801 )
2017
22
Surgical challenge in situs inversus with dextrocardia and Lutembacher syndrome. ( 29214824 )
2017
23
An unusual cause of 'dextrocardia'. ( 28087601 )
2017
24
Catheter ablation of premature ventricular contractions originating from aortic sinus cusps in a patient with dextrocardia and situs solitus: A case report. ( 29310391 )
2017
25
Severe aortic stenosis in dextrocardia with situs invertus and anomalous single coronary ostium treated with transcatheter aortic valve replacement. ( 28578896 )
2017
26
Dextrocardia With Situs Inversus and Levo-Transposition of Great Vessels. ( 28641729 )
2017
27
Comparing levocardia and dextrocardia in fetuses with heterotaxy syndrome: prenatal features, clinical significance and outcomes. ( 29169330 )
2017
28
Prenatal Diagnosis of Dextrocardia with Complex Congenital Heart Disease Using Fetal Intelligent Navigation Echocardiography (FINE) and a Literature Review. ( 28641300 )
2017
29
Successful percutaneous coronary intervention for an in-stent chronic total occlusion in a patient with dextrocardia: a case report. ( 29145812 )
2017
30
Review of surgical and anesthetic management for esophageal atresia with tracheoesophageal fistula, unilateral pulmonary agenesis and dextrocardia. ( 28417152 )
2017
31
Goldenhar Syndrome with Dextrocardia and Right Pulmonary Hypoplasia: An Unusual Association. ( 28377826 )
2017
32
Left side approach for aortic valve replacement in patient with dextrocardia and situs inversus totalis. ( 28983174 )
2017
33
Repair of Posterior Infarct Ventricular Septal Defect in a Patient with Dextrocardia and Situs Inversus. ( 26785697 )
2016
34
Creating a Fontan fenestration in a child with dextrocardia and interrupted inferior vena cava. ( 27212858 )
2016
35
Status asthmaticus with pseudo-dextrocardia, complicated by iatrogenic tension pneumothorax. ( 27583095 )
2016
36
Transcatheter aortic valve replacement for severe aortic stenosis in dextrocardia with situs inversus: First reported case in the United States. ( 26889599 )
2016
37
Successful transradial coronary angioplasty in a patient with dextrocardia and acute myocardial infarction. ( 28190738 )
2016
38
Triple vessel percutaneous coronary intervention in a patient with situs inversus dextrocardia using a transradial approach. ( 27751321 )
2016
39
Aortic Valve Replacement in Dextrocardia and Anomalous Origin of the Coronary Artery. ( 28238249 )
2016
40
Valve sparing surgery in an adult patient with dextrocardia and annuloaortic ectasia. ( 27212978 )
2016
41
Percutaneous Left Main Coronary Artery Intervention in a Case of Dextrocardia. ( 27127445 )
2016
42
Robotic atrial septal defect closure and tricuspid annuloplasty in a case of situs inversus totalis with dextrocardia. ( 27344445 )
2016
43
Criss-cross heart with dextrocardia and transposition of the great arteries: a rare pathology. ( 26875142 )
2016
44
Myocardial Perfusion SPECT Imaging in Dextrocardia with Situs Inversus: A Case Report. ( 27408900 )
2016
45
Successful Obstetric Outcome in Dextrocardia with Situs Inversus and Moderate Pulmonary Hypertension- Rare Case. ( 27891408 )
2016
46
Emergent percutaneous coronary intervention for acute myocardial infarction in patients with mirror dextrocardia: case reports and brief review. ( 27280091 )
2016
47
Implantable cardioverter-defibrillator in a patient with dextrocardia situs inversus. ( 27999649 )
2016
48
Successful Percutaneous Coronary Intervention for Chronic Total Occlusion of Left Anterior Descending Coronary Artery in a Patient with Dextrocardia. ( 28031659 )
2016
49
PERFORATED DUODENAL ULCER ASSOCIATED WITH SITUS INVERSUS AND DEXTROCARDIA. ( 27323596 )
2016
50
Fontan's circulation with dextrocardia, recent pulmonary embolism, and inferior vena cava filter: Anesthetic challenges for urgent hysterectomy. ( 26750698 )
2016

Variations for Dextrocardia

Expression for Dextrocardia

Search GEO for disease gene expression data for Dextrocardia.

Pathways for Dextrocardia

Pathways related to Dextrocardia according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.61 ACVR2B HES7 NODAL
2 11.29 ACVR2B NODAL
3 11.21 ACTC1 TNNT2
4 11.11 ACTC1 TNNT2
5
Show member pathways
10.85 ACVR2B NODAL
6 10.52 ACTC1 NODAL TNNT2

GO Terms for Dextrocardia

Cellular components related to Dextrocardia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 sarcomere GO:0030017 8.62 ACTC1 TNNT2

Biological processes related to Dextrocardia according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 anterior/posterior pattern specification GO:0009952 9.49 ACVR2B NODAL
2 lung development GO:0030324 9.48 ACVR2B NODAL
3 BMP signaling pathway GO:0030509 9.46 ACVR2B NODAL
4 cardiac muscle contraction GO:0060048 9.43 ACTC1 TNNT2
5 heart development GO:0007507 9.43 ACVR2B DNAI1 NODAL
6 regulation of signal transduction GO:0009966 9.4 ACVR2B NODAL
7 muscle filament sliding GO:0030049 9.37 ACTC1 TNNT2
8 mesoderm development GO:0007498 9.32 ACVR2B HES7
9 gastrulation with mouth forming second GO:0001702 9.16 ACVR2B NODAL
10 positive regulation of activin receptor signaling pathway GO:0032927 8.96 ACVR2B NODAL
11 determination of left/right symmetry GO:0007368 8.8 ACVR2B DNAI1 NODAL

Sources for Dextrocardia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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