MCID: VSC013
MIFTS: 35

Visceral Heterotaxy malady

Genetic diseases (common) category

Summaries for Visceral Heterotaxy

About this section
Sources:
48OMIM, 34MalaCards
See all sources

Fully expand this MalaCard

Download this MalaCard
MalaCards: Visceral Heterotaxy, also known as heterotaxy, visceral, 1, x-linked, is related to congenital heart disease and situs inversus. An important gene associated with Visceral Heterotaxy is ZIC3 (Zic family member 3), and among its related pathways are Ca-dependent events and TGF-beta Signaling Pathway. Affiliated tissues include heart and spleen, and related mouse phenotypes are digestive/alimentary and liver/biliary system.

Description from OMIM:48 613751,605376,306955

Aliases & Classifications for Visceral Heterotaxy

About this section
Sources:
9Disease Ontology, 63UMLS, 48OMIM
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic diseases (common)


Aliases & Descriptions:

visceral heterotaxy 9
heterotaxy, visceral, 1, x-linked 63


External Ids:

Disease Ontology9 DOID:0050545

Related Diseases for Visceral Heterotaxy

About this section
Sources:
18GeneCards, 19GeneDecks
See all sources

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



Diseases related to visceral heterotaxy

Symptoms for Visceral Heterotaxy

About this section
Sources:
48OMIM
See all sources


Clinical features from OMIM:

613751,605376,306955

Drugs & Therapeutics for Visceral Heterotaxy

About this section
Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
See all sources

Approved drugs:

Search CenterWatch for Visceral Heterotaxy

Drug clinical trials:

Search ClinicalTrials for Visceral Heterotaxy

Search NIH Clinical Center for Visceral Heterotaxy

Search CenterWatch for Visceral Heterotaxy

Genetic Tests for Visceral Heterotaxy

About this section

Anatomical Context for Visceral Heterotaxy

About this section
Sources:
34MalaCards
See all sources

MalaCards organs/tissues related to Visceral Heterotaxy:

34
Heart, Spleen

Animal Models for Visceral Heterotaxy or affiliated genes

About this section
Sources:
38MGI
See all sources

MGI Mouse Phenotypes related to Visceral Heterotaxy:

38 (show all 13)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053818.9CFC1, ZIC3, NODAL
2MP:00053708.9NODAL, ZIC3, ACVR2B, CFC1
3MP:00053888.9CFC1, ACVR2B, ZIC3, NODAL
4MP:00053828.9NODAL, ZIC3, ZIC5
5MP:00053858.9NODAL, ZIC3, ACVR2B, CFC1
6MP:00053978.8CFC1, ACVR2B, ZIC3, NODAL
7MP:00053918.8NODAL, ZIC3, ZIC5
8MP:00053878.7CFC1, ACVR2B, ZIC3, NODAL
9MP:00036318.2NODAL, ZIC3, ZIC5, ZIC4
10MP:00053908.1CFC1, ACVR2B, ZIC5, ZIC3, NODAL
11MP:00053808.1CFC1, ACVR2B, ZIC5, ZIC3, NODAL
12MP:00053787.6NODAL, ZIC3, ZIC5, ZIC4, ACVR2B, CFC1
13MP:00107687.6CFC1, ACVR2B, ZIC4, ZIC5, ZIC3, NODAL

Publications for Visceral Heterotaxy

About this section
Sources:
53PubMed
See all sources

Articles related to Visceral Heterotaxy:

(show all 27)
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
Visceral heterotaxy, isomerism, and splenic structure. (16164784)
2005
10
Splenic state in surviving patients with visceral heterotaxy. (16164783)
2005
11
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
12
Isomeric arrangement of the left atrial appendages and visceral heterotaxy. (11117404)
2000
13
Atrial structure in the presence of visceral heterotaxy. (10950326)
2000
14
Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome. (11045684)
2000
15
Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases. (10817298)
2000
16
The Fontan type procedure in patients with visceral heterotaxy. (9855091)
1998
17
Aortic outflow obstruction in visceral heterotaxy: a study based on twenty postmortem cases. (9141378)
1997
18
Venoarterial connections in visceral heterotaxy. (8622310)
1996
19
Visceral heterotaxy syndrome induced by retinoids in mouse embryo. (8593204)
1995
20
Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. (7564430)
1995
21
Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. (7677481)
1995
22
Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. (7572671)
1995
23
Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. (8279925)
1994
24
Spectrum of heart malformations in mice with situs solitus, situs inversus, and associated visceral heterotaxy. (1959204)
1991
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

About this section

Expression for genes affiliated with Visceral Heterotaxy

About this section
Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Visceral Heterotaxy

Search GEO for disease gene expression data for Visceral Heterotaxy.

Pathways for genes affiliated with Visceral Heterotaxy

About this section
Sources:
51PathCards, 56Reactome, 61Thomson Reuters, 58SinoBiological, 31KEGG
See all sources

Compounds for genes affiliated with Visceral Heterotaxy

About this section

GO Terms for genes affiliated with Visceral Heterotaxy

About this section
Sources:
17Gene Ontology
See all sources

Biological processes related to Visceral Heterotaxy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1anterior/posterior pattern specificationGO:0099529.6ZIC3, ACVR2B
2heart loopingGO:0019479.5ZIC3, NODAL
3positive regulation of activin receptor signaling pathwayGO:0329279.5NODAL, ACVR2B
4lung developmentGO:0303249.3ZIC3, ACVR2B
5determination of left/right symmetryGO:0073689.2ZIC3, ACVR2B, CFC1

Molecular functions related to Visceral Heterotaxy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1metal ion bindingGO:0468728.2ZIC3, ZIC5, ZIC4, ACVR2B

Products for genes affiliated with Visceral Heterotaxy

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Visceral Heterotaxy

About this section
4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet