MCID: DXT001
MIFTS: 52

Dextrocardia

Categories: Cardiovascular diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Dextrocardia

MalaCards integrated aliases for Dextrocardia:

Name: Dextrocardia 12 75 53 59 29 6 44 15 33
Heart Predominantly in Right Hemithorax 12

Classifications:



External Ids:

Disease Ontology 12 DOID:9565
MeSH 44 D003914
NCIt 50 C84669
SNOMED-CT 68 27637000
ICD10 33 Q24.0
MESH via Orphanet 45 D003914
ICD10 via Orphanet 34 Q24.0
UMLS via Orphanet 73 C0011813 C0265863
Orphanet 59 ORPHA1666
UMLS 72 C0011813

Summaries for Dextrocardia

NIH Rare Diseases : 53 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.

MalaCards based summary : Dextrocardia, also known as heart predominantly in right hemithorax, is related to dextrocardia with situs inversus and situs inversus. An important gene associated with Dextrocardia is HES7 (Hes Family BHLH Transcription Factor 7), and among its related pathways/superpathways are Mesodermal Commitment Pathway and Signaling by BMP. The drugs Spironolactone and Natriuretic Peptide, Brain have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and liver, and related phenotypes are t-wave inversion and situs inversus totalis

Wikipedia : 75 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 262)
# Related Disease Score Top Affiliating Genes
1 dextrocardia with situs inversus 33.4 NODAL HES7 CFAP53
2 situs inversus 31.2 NODAL DNAI1 CFAP53
3 heterotaxy 29.8 NODAL CFAP53 ACVR2B
4 visceral heterotaxy 29.3 NODAL DNAI1 CFAP53 ACVR2B
5 short stature wormian bones dextrocardia 12.5
6 dextrocardia with unusual facies and microphthalmia 12.4
7 heterotaxy, visceral, 1, x-linked 12.0
8 kartagener syndrome 12.0
9 stratton-parker syndrome 12.0
10 ciliary dyskinesia, primary, 1 12.0
11 poland syndrome 11.5
12 levocardia 11.5
13 heterotaxy, visceral, 5, autosomal 11.5
14 pagod syndrome 11.4
15 carpenter syndrome 1 11.1
16 marden-walker syndrome 11.1
17 heterotaxy, visceral, 4, autosomal 11.1
18 intellectual disability - athetosis - microphthalmia 11.1
19 heart septal defect 10.7
20 transposition of the great arteries 10.6
21 double discordia 10.6
22 ventricular septal defect 10.6
23 myocardial infarction 10.5
24 atrial heart septal defect 10.4
25 mitral valve insufficiency 10.4
26 acute myocardial infarction 10.4
27 double outlet right ventricle 10.4
28 tetralogy of fallot 10.4
29 hyperlipoproteinemia, type iii 10.3
30 coronary artery anomaly 10.3
31 mitral valve stenosis 10.3
32 congenitally corrected transposition of the great arteries 10.3
33 progressive familial heart block, type ia 10.3
34 single ventricular heart 10.3
35 pulmonary hypertension 10.3
36 atrial fibrillation 10.3
37 congestive heart failure 10.3
38 bronchiectasis 10.3
39 arteries, anomalies of 10.2
40 pulmonary hypoplasia, primary 10.2
41 aortic coarctation 10.2
42 cyanosis, transient neonatal 10.2
43 sick sinus syndrome 10.2
44 tricuspid atresia 10.1
45 heart disease 10.1
46 tricuspid valve insufficiency 10.1
47 inferior myocardial infarction 10.1
48 atrial standstill 1 10.1
49 cardiac conduction defect 10.1
50 wolff-parkinson-white syndrome 10.1

Graphical network of the top 20 diseases related to Dextrocardia:



Diseases related to Dextrocardia

Symptoms & Phenotypes for Dextrocardia

Human phenotypes related to Dextrocardia:

59 32 (show all 27)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 t-wave inversion 59 32 hallmark (90%) Very frequent (99-80%) HP:0010872
2 situs inversus totalis 59 32 frequent (33%) Frequent (79-30%) HP:0001696
3 congenital malformation of the great arteries 59 32 frequent (33%) Frequent (79-30%) HP:0011603
4 intestinal malrotation 59 32 occasional (7.5%) Occasional (29-5%) HP:0002566
5 abnormality of the spleen 59 32 occasional (7.5%) Occasional (29-5%) HP:0001743
6 abnormality of the pulmonary artery 59 32 occasional (7.5%) Occasional (29-5%) HP:0004414
7 abnormality of the ureter 59 32 occasional (7.5%) Occasional (29-5%) HP:0000069
8 abnormal lung lobation 59 32 occasional (7.5%) Occasional (29-5%) HP:0002101
9 abnormal renal morphology 59 32 occasional (7.5%) Occasional (29-5%) HP:0012210
10 abnormal reproductive system morphology 59 32 occasional (7.5%) Occasional (29-5%) HP:0012243
11 abnormality of abdominal situs 59 32 occasional (7.5%) Occasional (29-5%) HP:0011620
12 abnormal pulmonary situs morphology 32 occasional (7.5%) HP:0011615
13 hydrocephalus 59 32 very rare (1%) Very rare (<4-1%) HP:0000238
14 global developmental delay 59 32 very rare (1%) Very rare (<4-1%) HP:0001263
15 webbed neck 59 32 very rare (1%) Very rare (<4-1%) HP:0000465
16 abnormality of the foot 59 32 very rare (1%) Very rare (<4-1%) HP:0001760
17 congenital hip dislocation 59 32 very rare (1%) Very rare (<4-1%) HP:0001374
18 preauricular skin tag 59 32 very rare (1%) Very rare (<4-1%) HP:0000384
19 abnormality of the ribs 59 32 very rare (1%) Very rare (<4-1%) HP:0000772
20 neuroblastoma 59 32 very rare (1%) Very rare (<4-1%) HP:0003006
21 meckel diverticulum 59 32 very rare (1%) Very rare (<4-1%) HP:0002245
22 pancreatic hypoplasia 59 32 very rare (1%) Very rare (<4-1%) HP:0002594
23 aplasia/hypoplasia of the ear 59 32 very rare (1%) Very rare (<4-1%) HP:0008771
24 dextrocardia 59 Obligate (100%)
25 abnormal heart morphology 59 Very frequent (99-80%)
26 abnormal ekg 59 Very frequent (99-80%)
27 abnormality of pulmonary situs 59 Occasional (29-5%)

MGI Mouse Phenotypes related to Dextrocardia:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 9.7 ACVR2B CFAP53 CTC1 DNAI1 HES7 NODAL
2 embryo MP:0005380 9.65 ACVR2B CFAP53 HES7 NODAL STRA6
3 immune system MP:0005387 9.43 ACVR2B CFAP53 CTC1 DNAI1 NODAL STRA6
4 skeleton MP:0005390 9.02 ACVR2B DNAI1 HES7 NODAL STRA6

Drugs & Therapeutics for Dextrocardia

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

(show all 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Spironolactone Approved Phase 4 1952-01-7, 52-01-7 5833
2 Natriuretic Peptide, Brain Phase 4
3 Natriuretic Agents Phase 4
4 Hormones Phase 4
5 diuretics Phase 4
6 Mineralocorticoid Receptor Antagonists Phase 4
7 Diuretics, Potassium Sparing Phase 4
8 Mineralocorticoids Phase 4
9 Hormone Antagonists Phase 4
10 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
11
Ambrisentan Approved, Investigational Phase 2 177036-94-1 6918493
12 Simendan Investigational Phase 2 131741-08-7
13 Cardiotonic Agents Phase 2
14 Phosphodiesterase Inhibitors Phase 2
15 Protective Agents Phase 2
16 Pharmaceutical Solutions Phase 2
17 Vasodilator Agents Phase 2
18 Phosphodiesterase 3 Inhibitors Phase 2
19 Antihypertensive Agents Phase 2
20 Liver Extracts
21 Respiratory System Agents

Interventional clinical trials:

(show all 11)
# Name Status NCT ID Phase Drugs
1 Water Immersion in Right-Sided Heart Failure: A Pilot Study Completed NCT00654264 Phase 4
2 Spironolactone Therapy in Chronic Stable Right HF Trial Recruiting NCT03344159 Phase 4 Spironolactone;Placebo
3 Open-Label Rollover Study of Levosimendan in Patients With Pulmonary Hypertension With Heart Failure and Preserved Left Ventricular Ejection Fraction (PH-HFpEF) Recruiting NCT03624010 Phase 2 Levosimendan 2.5 mg/ml Injectable Solution
4 Safety, Pharmacokinetics (PK) and Hemodynamic Effects of Ambrisentan in Single Ventricle Pediatric Patients Active, not recruiting NCT02080637 Phase 2 Ambrisentan
5 Assessment of Portal Flow Using Bedside Doppler Ultrasonography for the Detection of Portal Pulsatility as a Risk Factor for Acute Kidney Injury in Cardiac Surgery Patients Completed NCT02831907
6 Non-invasive Assessment of Liver Stiffness/Fibrosis by Transient Elastography (Fibroscan) in Patients With Left and/or Right Sided Heart Failure Completed NCT00777725
7 Evaluation of Multimodal Monitoring Technologies in the Peri-operative Care of Cardiac Surgical Patients Completed NCT02658006
8 Transcatheter Valve Implantation in Patients With Dysfunctional Left and Right Sided Heart Valves Completed NCT02119442
9 Molecular Genetics of Heterotaxy and Related Congenital Heart Defects Recruiting NCT02432079
10 Severe and Transient Hypoxemia During Selective Intra-arterial Chemotherapy for Retinoblastoma in Children: Evaluation of the Right-sided Heart Function. Active, not recruiting NCT03559894
11 Open Lung Strategy in Critically Ill Morbid Obese Patients Lung Imaging and Heart-lung Interaction Active, not recruiting NCT02503241

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 29

Anatomical Context for Dextrocardia

MalaCards organs/tissues related to Dextrocardia:

41
Heart, Lung, Liver, Kidney, Spleen, Brain, Skin

Publications for Dextrocardia

Articles related to Dextrocardia:

(show top 50) (show all 1362)
# Title Authors PMID Year
1
Surgical ductal stent implantation in total anomalous pulmonary venous connection to vena porta with right pulmonary sequestration in a mature newborn. 38
31360457 2019
2
[A Total Right Heart Bypass Operation for Polysplenia with Unilateral Pulmonary Arteriovenous Fistula;the Usefulness of the Anastomosis of the Hepatic Vein to the Azygous Vein Combined with Dilatable Pulmonary Artery Banding]. 38
31353348 2019
3
[Mitral Valve and Tricuspid Valve Regurgitation in a Patient with Dextrocardia and Absence of the Inferior Vena Cava]. 38
31353349 2019
4
Prolonged sustained ventricular fibrillation in a patient with dextrocardia and a left ventricular assist device. 38
31389822 2019
5
Situs Inversus: Inferior-Lateral ST-Elevation Myocardial Infarction on Right-Sided Electrocardiogram. 38
31404172 2019
6
Successful high-density mapping and ablation of atrial tachycardia in a patient with dextrocardia and situs inversus: a complex case. 38
31400210 2019
7
SPECT myocardial perfusion imaging in patients with Dextrocardia. 38
31062220 2019
8
Coronary angioplasty in an adult with dextrocardia and single coronary artery with the right coronary artery originating from the left anterior descending artery. 38
30707111 2019
9
Jarvik 2000 axial flow ventricular assist device in right single ventricle after Fontan operation. 38
31392523 2019
10
Interstitial 5p15.2-p13.3 deletion in association with situs inversus, dextrocardia, L-loop of the ventricles, and transposition of great arteries in a newborn infant. 38
31331405 2019
11
Management of a young patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome with venous-venous extracorporeal membrane oxygenation and heart-lung transplantation. 38
31332841 2019
12
Right Lung Agenesis Associated with Dextrocardia and Pulmonary Hypertension. 38
31300255 2019
13
Isthmic Aortic False Aneurysm Post-Patch Coartaction Aortoplasty: A Hybrid Solution. 38
31376546 2019
14
Redo robotic cardiac surgery and concomitant cesarean section in a pregnant patient with dextrocardia and situs inversus totalis. 38
31233237 2019
15
Percutaneous coronary intervention for left main coronary artery stenosis in a patient with dextrocardia. 38
31087254 2019
16
Commentary: Doing the "right" thing: Cardiac transplantation in patients with dextrocardia. 38
30630604 2019
17
Transradial Approach to Coronary Angiography and Percutaneous Intervention in Patients With Dextrocardia. 38
31034439 2019
18
Technical Considerations for Lung Transplantation in Kartagener's Syndrome. 38
30612988 2019
19
Characteristics and outcomes of fetuses with laterality defects are the current outcomes better? A single center study. 38
31106637 2019
20
Computed Tomography Angiography and Magnetic Resonance Angiography of Congenital Anomalies of Pulmonary Veins. 38
31082974 2019
21
Computed Tomography Angiography and Magnetic Resonance Angiography of Congenital Anomalies of Pulmonary Veins. 38
31082945 2019
22
Dextrocardia with pulmonary hypoplasia: an unusual cause of unilateral lung white-out. 38
31142489 2019
23
Persistent dextrocardia after adult orthotopic heart transplantation in a patient with complex congenital heart disease. 38
30528511 2019
24
3D Printing to Model Surgical Repair of Complex Congenitally Corrected Transposition of the Great Arteries. 38
28825385 2019
25
Dextrocardia with complete AV block and the implantation of a temporary pacemaker before cesarean section: A case report. 38
31027067 2019
26
Balloon Mitral Valvuloplasty in Mirror Image Dextrocardia with Rheumatic Mitral Stenosis with Suprasystemic Pulmonary Arterial Hypertension. 38
31311229 2019
27
Catheter ablation of supraventricular tachycardia in patients with dextrocardia and situs inversus. 38
30661266 2019
28
A rare association of left pulmonary artery sling with right pulmonary hypoplasia and total anomalous pulmonary venous connection. 38
30968804 2019
29
[Valve Replacement for Tricuspid Regurgitation with Congenitally Corrected Transposition of the Great Arteries]. 38
30923295 2019
30
Right coronary artery originating from the left anterior descending artery in a patient with mirror dextrocardia. 38
31222235 2019
31
Transcatheter atrioventricular valve-in-valve implantation for Fontan circulation and dextrocardia. 38
30312393 2019
32
Implantation of a completely right sided subcutaneous cardioverter-defibrillator in a patient with situs inversus dextrocardia. 38
30468861 2019
33
His-bundle pacing in a patient with dextrocardia, severe systolic dysfunction, and complete atrioventricular block. 38
30891412 2019
34
Successful Heart Transplantation in Two Infants With Dextrocardia-Heterotaxy Syndrome. 38
30081023 2019
35
Comparison of Morphologic Findings in Patients with Dextrocardia with Situs Solitus vs Situs Inversus: a Retrospective Study. 38
30334087 2019
36
Transcatheter Aortic Valve Replacement in a Patient With Dextrocardia and Situs Inversus Totalis. 38
29908190 2019
37
Cryoballoon ablation under transesophageal echocardiographic guidance in dextrocardia. 38
29704566 2019
38
Partition of Common Atrioventricular Valve in a Patient With Dextrocardia and Univentricular Circulation. 38
30273646 2019
39
Novel use of covered stents to treat profound cyanosis in a hepatic vein exclusion Fontan. 38
30745773 2019
40
Right-Sided Subcutaneous Implantable Cardioverter Defibrillator System Implantation in a Patient with Complex Congenital Heart Disease and Dextrocardia: A Case Report and Literature Review. 38
30867968 2019
41
Left-sided Poland's syndrome associated with dextrocardia. 38
31297489 2019
42
A retrospective review of right-sided hearts at a South African tertiary hospital. 38
30462143 2019
43
Rare case of septum primum malposition defect in dextrocardia and situs inversus totalis without heterotaxy syndrome. 38
30585568 2018
44
Just flip it!-CRT implantation in a patient with dextrocardia and situs inversus totalis. 38
30555613 2018
45
Surgical challenge in situs inversus with dextrocardia and Lutembacher syndrome. 38
29214824 2018
46
Percutaneous Transhepatic Fontan-Kreutzer Completion of Hepatic Vein Inclusion. 38
28055329 2018
47
Trans-aortic pulmonary vein isolation using magnetic navigation system for paroxysmal atrial fibrillation in a patient with dextrocardia, situs inversus, and inferior vena cava continuity with azygos vein. 38
30327707 2018
48
d-transposition of great arteries, dextrocardia with aberrant origin of right subclavian artery from pulmonary artery. 38
30206995 2018
49
Atrioventricular Canal and Dextrocardia: A Case Report with Implications for Community-Based Providers. 38
30567810 2018
50
Senning Procedure for Transposition of the Great Arteries in a Patient with Situs Inversus Totalis and Dextrocardia. 38
30173681 2018

Variations for Dextrocardia

ClinVar genetic disease variations for Dextrocardia:

6
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 CFAP53 NM_145020.5(CFAP53): c.301_473+1del deletion Likely pathogenic rs1555672928 18:47787434-47787607 18:50261064-50261237
2 CFAP53 NM_145020.5(CFAP53): c.472A> G (p.Arg158Gly) single nucleotide variant Likely pathogenic rs886037751 18:47787435-47787435 18:50261065-50261065
3 ROCK2 NM_004850.5(ROCK2): c.3724C> T (p.Gln1242Ter) single nucleotide variant Uncertain significance 2:11332802-11332802 2:11192676-11192676
4 ZFYVE16 NM_014733.6(ZFYVE16): c.1798G> A (p.Asp600Asn) single nucleotide variant Uncertain significance 5:79734302-79734302 5:80438483-80438483
5 ZFYVE16 NM_014733.6(ZFYVE16): c.3755G> C (p.Gly1252Ala) single nucleotide variant Uncertain significance 5:79752344-79752344 5:80456525-80456525
6 RAI2 NM_021785.5(RAI2): c.712G> A (p.Val238Ile) single nucleotide variant Uncertain significance X:17819419-17819419 X:17801299-17801299
7 SUPT16H NM_007192.4(SUPT16H): c.956-1G> A single nucleotide variant Uncertain significance 14:21834689-21834689 14:21366530-21366530

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 NODAL HES7 ACVR2B
2
Show member pathways
11.11 ZFYVE16 ACVR2B
3
Show member pathways
10.85 NODAL ACVR2B
4 10.77 ZFYVE16 NODAL ACVR2B

GO Terms for Dextrocardia

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

# Name GO ID Score Top Affiliating Genes
1 lung development GO:0030324 9.5 STRA6 NODAL ACVR2B
2 regulation of signal transduction GO:0009966 9.48 NODAL ACVR2B
3 mesoderm development GO:0007498 9.46 HES7 ACVR2B
4 cilium movement GO:0003341 9.43 DNAI1 CFAP53
5 BMP signaling pathway GO:0030509 9.43 ZFYVE16 NODAL ACVR2B
6 gastrulation with mouth forming second GO:0001702 9.4 NODAL ACVR2B
7 digestive tract morphogenesis GO:0048546 9.37 STRA6 NODAL
8 heart development GO:0007507 9.26 STRA6 NODAL DNAI1 ACVR2B
9 positive regulation of activin receptor signaling pathway GO:0032927 9.16 NODAL ACVR2B
10 determination of left/right symmetry GO:0007368 8.92 NODAL DNAI1 CFAP53 ACVR2B

Sources for Dextrocardia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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