MCID: VSC013
MIFTS: 30

Visceral Heterotaxy malady

Summaries for Visceral Heterotaxy

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46OMIM, 32MalaCards
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MalaCards: Visceral Heterotaxy, also known as heterotaxy, visceral, 1, x-linked, is related to ventricular septal defect and heterotaxy, visceral, 2, autosomal. An important gene associated with Visceral Heterotaxy is ZIC3 (Zic family member 3), and among its related pathways are TGF-beta Signaling Pathway and Axon guidance. Affiliated tissues include heart and spleen, and related mouse phenotypes are liver/biliary system and respiratory system.

Description from OMIM:46 605376,306955,613751

Aliases & Classifications for Visceral Heterotaxy

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8Disease Ontology, 60UMLS, 46OMIM
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Aliases & Descriptions:

visceral heterotaxy 8
heterotaxy, visceral, 1, x-linked 60


External Ids:

Disease Ontology8 DOID:0050545

Related Diseases for Visceral Heterotaxy

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17GeneCards, 18GeneDecks
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Graphical network of diseases related to Visceral Heterotaxy:



Diseases related to visceral heterotaxy

Clinical Features for Visceral Heterotaxy

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46OMIM
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Clinical features from OMIM:

605376,306955,613751

Drugs & Therapeutics for Visceral Heterotaxy

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Search NIH Clinical Center for Visceral Heterotaxy

Search CenterWatch for Visceral Heterotaxy

Genetic Tests for Visceral Heterotaxy

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Anatomical Context for Visceral Heterotaxy

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32MalaCards
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MalaCards organs/tissues related to Visceral Heterotaxy:

32
Heart, Spleen

Animal Models for Visceral Heterotaxy or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Visceral Heterotaxy:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.7LEFTY2, ZIC3, CFC1, NODAL, ACVR2B
2MP:00053888.7ACVR2B, NODAL, CFC1, LEFTY2, ZIC3
3MP:00053818.7ZIC3, LEFTY2, NODAL, CFC1
4MP:00053858.6NODAL, CFC1, LEFTY2, ZIC3, ACVR2B
5MP:00053878.6LEFTY2, ZIC3, NODAL, CFC1, ACVR2B
6MP:00053908.5ACVR2B, NODAL, CFC1, ZIC5, ZIC3
7MP:00053978.5CFC1, ACVR2B, LEFTY2, ZIC3, NODAL
8MP:00053808.2ZIC3, ACVR2B, NODAL, CFC1, LEFTY2, ZIC5
9MP:00053787.3ZIC3, ZIC5, ZIC4, LEFTY2, CFC1, ACVR2B
10MP:00107687.2ZIC3, ZIC5, ZIC4, LEFTY2, CFC1, ACVR2B

Publications for Visceral Heterotaxy

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50PubMed
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Articles related to Visceral Heterotaxy:

(show all 30)
idTitleAuthorsYear
1
A case of unusual visceral heterotaxy syndrome with isolated levocardia. (24255657)
2013
2
Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy. (22843201)
2013
3
Visceral heterotaxy in the developing world. (22726404)
2012
4
Visceral heterotaxy and malrotation in a neonate. (22411653)
2012
5
Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy. (21233261)
2011
6
Raoultella ornithinolytica bacteremia in an infant with visceral heterotaxy. (20072080)
2010
7
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
8
Surgery for the functionally univentricular heart in patients with visceral heterotaxy. (16401367)
2006
9
Heterotaxy syndrome -- asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. (16113522)
2005
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
Heterotaxy syndrome in an adult, with polysplenia, visceral and cardiovascular malposition. (11307871)
2001
14
Isomeric arrangement of the left atrial appendages and visceral heterotaxy. (11117404)
2000
15
Atrial structure in the presence of visceral heterotaxy. (10950326)
2000
16
Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome. (11045684)
2000
17
Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases. (10817298)
2000
18
The Fontan type procedure in patients with visceral heterotaxy. (9855091)
1998
19
Aortic outflow obstruction in visceral heterotaxy: a study based on twenty postmortem cases. (9141378)
1997
20
Venoarterial connections in visceral heterotaxy. (8622310)
1996
21
Visceral heterotaxy syndrome induced by retinoids in mouse embryo. (8593204)
1995
22
Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. (7564430)
1995
23
Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. (7677481)
1995
24
Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. (7572671)
1995
25
Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. (8279925)
1994
26
Spectrum of heart malformations in mice with situs solitus, situs inversus, and associated visceral heterotaxy. (1959204)
1991
27
99mTechnetium pyridoxylidene glutamate imaging in visceral heterotaxy (Ivemark's syndrome). (488637)
1979
28
Levocardia with visceral heterotaxy--isolated levocardia: pathologic anatomy and its clinical implications. (4682005)
1973
29
VISCERAL HETEROTAXY WITH MALFORMED HEART. SIMILAR FINDINGS IN A CHILD AND A PUPPY. (14173800)
1964
30
Heterotaxy; a bibliography of visceral transposition in man. (21026789)
1945

Genetic Variations for Visceral Heterotaxy

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Expression for genes affiliated with Visceral Heterotaxy

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Visceral Heterotaxy

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Pathways for genes affiliated with Visceral Heterotaxy

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55SinoBiological, 53Reactome, 12EMD Millipore, 29KEGG
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Pathways related to Visceral Heterotaxy according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6ACVR2B, LEFTY2
2
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9.3ACVR2B, NODAL, CFC1
3
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9.2ACVR2B, NODAL, LEFTY2
49.2LEFTY2, NODAL, ACVR2B
5
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8.9ACVR2B, NODAL, CFC1, LEFTY2

Compounds for genes affiliated with Visceral Heterotaxy

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GO Terms for genes affiliated with Visceral Heterotaxy

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16Gene Ontology
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Biological processes related to Visceral Heterotaxy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1anterior/posterior pattern specificationGO:0099529.7ACVR2B, ZIC3
2positive regulation of activin receptor signaling pathwayGO:0329279.7ACVR2B, NODAL
3heart loopingGO:0019479.7ZIC3, NODAL
4determination of left/right symmetryGO:0073689.5ACVR2B, CFC1, ZIC3
5lung developmentGO:0303249.4ACVR2B, ZIC3

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
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet