MCID: VSC013
MIFTS: 36

Visceral Heterotaxy malady

Genetic diseases, Cardiovascular diseases, Fetal diseases, Rare diseases categories

Aliases & Classifications for Visceral Heterotaxy

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Sources:
9Disease Ontology, 47Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet
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Visceral Heterotaxy, Aliases & Descriptions:

Name: Visceral Heterotaxy 9
Situs Ambiguus 9 47
Incomplete Situs Inversus 47
 
Partial Situs Inversus 47
Situs Ambiguous 47
Heterotaxia 9


Classifications:



External Ids:

Disease Ontology9 DOID:0050545
Orphanet47 157769
ICD10 via Orphanet26 Q89.3
UMLS via Orphanet61 C0266642, C1167664

Summaries for Visceral Heterotaxy

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Disease Ontology:9 A physical disorder characterized by the abnormal distribution of the major visceral organs within the chest and abdomen.

MalaCards based summary: Visceral Heterotaxy, also known as situs ambiguus, is related to congenital heart disease and holoprosencephaly. An important gene associated with Visceral Heterotaxy is NODAL (nodal growth differentiation factor), and among its related pathways are TGF-beta Signaling Pathway and TGF-beta Signaling Pathway. Affiliated tissues include heart and spleen, and related mouse phenotypes are craniofacial and digestive/alimentary.

Related Diseases for Visceral Heterotaxy

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Diseases in the Visceral Heterotaxy family:

Heterotaxy, Visceral, 2, Autosomal Heterotaxy, Visceral, 4, Autosomal
Heterotaxy, Visceral, 3, Autosomal Heterotaxy, Visceral, 5
Heterotaxy, Visceral, 6, Autosomal Recessive Zic3-Related Visceral Heterotaxy
Cfc1-Related Visceral Heterotaxy Nodal-Related Visceral Heterotaxy
Acvr2b-Related Visceral Heterotaxy Lefty2-Related Visceral Heterotaxy

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

(show top 50)    (show all 66)
idRelated DiseaseScoreTop Affiliating Genes
1congenital heart disease30.7ZIC3
2holoprosencephaly30.2NODAL, ZIC5
3situs inversus30.1ZIC3, CCDC11, LEFTY2
4heterotaxy10.9
5heterotaxy, visceral, 1, x-linked10.5
6duodenitis10.4
7duodenal obstruction10.4
8zic3-related visceral heterotaxy10.4
9cfc1-related visceral heterotaxy10.4
10nodal-related visceral heterotaxy10.4
11acvr2b-related visceral heterotaxy10.4
12lefty2-related visceral heterotaxy10.4
13levocardia10.4
14annular pancreas10.3
15isolated levocardia10.3
16peritonitis10.2
17intestinal volvulus10.2
18pancreatitis10.2
19heterotaxy, visceral, 2, autosomal10.2
20heterotaxy, visceral, 4, autosomal10.2
21heterotaxy, visceral, 3, autosomal10.2
22bacteremia10.2
23heterotaxy, visceral, 510.2
24dilated cardiomyopathy10.2
25vascular disease10.2
26ventricular septal defect10.2
27aneurysm10.2
28dextrocardia10.1
29splenic infarction10.1
30congenital pseudoarthrosis of clavicle10.1
31restrictive dermopathy, lethal9.9
32visceral myopathy9.9
33ellis-van creveld syndrome9.9
34joubert syndrome 19.9
35meacham syndrome9.9
36smith-lemli-opitz syndrome9.9
37johanson-blizzard syndrome9.9
38marden-walker syndrome9.9
39renpenning syndrome9.9
40alport syndrome9.9
41escobar syndrome9.9
42meckel syndrome 19.9
43craniorachischisis9.9
44hepatitis9.9
45primary ciliary dyskinesia9.9
46polydactyly9.9
47poland syndrome9.9
48esophageal atresia9.9
49hallermann-streiff syndrome9.9
50lymphocele9.9

Graphical network of the top 20 diseases related to Visceral Heterotaxy:



Diseases related to visceral heterotaxy

Symptoms for Visceral Heterotaxy

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Drugs & Therapeutics for Visceral Heterotaxy

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Drug clinical trials:

Search ClinicalTrials for Visceral Heterotaxy

Search NIH Clinical Center for Visceral Heterotaxy

Genetic Tests for Visceral Heterotaxy

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Anatomical Context for Visceral Heterotaxy

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MalaCards organs/tissues related to Visceral Heterotaxy:

31
Heart, Spleen

Animal Models for Visceral Heterotaxy or affiliated genes

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Publications for Visceral Heterotaxy

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Articles related to Visceral Heterotaxy:

(show all 27)
idTitleAuthorsYear
1
Slow pathway ablation in abdominal visceral heterotaxy with azygos continuation. (25829475)
2015
2
A case of unusual visceral heterotaxy syndrome with isolated levocardia. (24255657)
2013
3
Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy. (22843201)
2013
4
Visceral heterotaxy in the developing world. (22726404)
2012
5
Visceral heterotaxy and malrotation in a neonate. (22411653)
2012
6
Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy. (21233261)
2011
7
Raoultella ornithinolytica bacteremia in an infant with visceral heterotaxy. (20072080)
2010
8
Membranous septal aneurysm: an unusual cause for right ventricular outflow tract obstruction in a malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle) associated with visceral heterotaxy. (18704548)
2009
9
Surgery for the functionally univentricular heart in patients with visceral heterotaxy. (16401367)
2006
10
Visceral heterotaxy, isomerism, and splenic structure. (16164784)
2005
11
Splenic state in surviving patients with visceral heterotaxy. (16164783)
2005
12
Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy. (12031727)
2002
13
Isomeric arrangement of the left atrial appendages and visceral heterotaxy. (11117404)
2000
14
Atrial structure in the presence of visceral heterotaxy. (10950326)
2000
15
Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome. (11045684)
2000
16
Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases. (10817298)
2000
17
The Fontan type procedure in patients with visceral heterotaxy. (9855091)
1998
18
Aortic outflow obstruction in visceral heterotaxy: a study based on twenty postmortem cases. (9141378)
1997
19
Venoarterial connections in visceral heterotaxy. (8622310)
1996
20
Visceral heterotaxy syndrome induced by retinoids in mouse embryo. (8593204)
1995
21
Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. (7564430)
1995
22
Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. (7677481)
1995
23
Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. (7572671)
1995
24
Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. (8279925)
1994
25
99mTechnetium pyridoxylidene glutamate imaging in visceral heterotaxy (Ivemark's syndrome). (488637)
1979
26
Levocardia with visceral heterotaxy--isolated levocardia: pathologic anatomy and its clinical implications. (4682005)
1973
27
VISCERAL HETEROTAXY WITH MALFORMED HEART. SIMILAR FINDINGS IN A CHILD AND A PUPPY. (14173800)
1964

Variations for Visceral Heterotaxy

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Expression for genes affiliated with Visceral Heterotaxy

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Search GEO for disease gene expression data for Visceral Heterotaxy.

Pathways for genes affiliated with Visceral Heterotaxy

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Compounds for genes affiliated with Visceral Heterotaxy

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GO Terms for genes affiliated with Visceral Heterotaxy

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Biological processes related to Visceral Heterotaxy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of activin receptor signaling pathwayGO:00329279.7NODAL, ACVR2B
2heart loopingGO:00019479.7ZIC3, NODAL
3anterior/posterior pattern specificationGO:00099529.6ZIC3, ACVR2B
4lung developmentGO:00303249.3ZIC3, ACVR2B
5determination of left/right symmetryGO:00073689.3ZIC3, ACVR2B, CFC1

Molecular functions related to Visceral Heterotaxy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:00051259.6NODAL, LEFTY2
2growth factor activityGO:00080839.3NODAL, LEFTY2
3metal ion bindingGO:00468728.2ZIC3, ZIC5, ZIC4, ACVR2B

Products for genes affiliated with Visceral Heterotaxy

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Sources for Visceral Heterotaxy

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet