LVNC
MCID: LFT003
MIFTS: 48

Left Ventricular Noncompaction (LVNC) malady

Summaries for Left Ventricular Noncompaction

Sources:
43NIH Rare Diseases, 33MalaCards
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NIH Rare Diseases:43 Left ventricular noncompaction (lvnc) is a rare heart condition. in lvnc the inside wall of the heart is spongy or grooved, instead of smooth. signs and symptoms of lvnc vary, but may cause life-threatening abnormal heart rhythms and weakness of the heart muscle. treatments, such as blood thinning medication and defibrillators, are available to control these heart symptoms. in rare cases, heart transplantation is needed. last updated: 12/29/2013

MalaCards: Left Ventricular Noncompaction, also known as left ventricular hypertrabeculation, is related to barth syndrome and hypertrophic cardiomyopathy. An important gene associated with Left Ventricular Noncompaction is TAZ (tafazzin), and among its related pathways are Cardiac Progenitor Differentiation and Muscular Dystrophies and Dystrophin-Glycoprotein Complex. The compounds valine and ruthenium have been mentioned in the context of this disorder. Affiliated tissues include heart, smooth muscle and kidney, and related mouse phenotypes are behavior/neurological and normal.

Aliases & Classifications for Left Ventricular Noncompaction

Sources:
43NIH Rare Diseases, 22GTR, 61UMLS
See all sources

Aliases & Descriptions:

left ventricular noncompaction 43 22 61
left ventricular hypertrabeculation 43
spongy myocardium 43
lvnc 43


Related Diseases for Left Ventricular Noncompaction

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the left ventricular noncompaction 1, with or without congenital heart defects family:

left ventricular noncompaction left ventricular noncompaction 5
left ventricular noncompaction 6 left ventricular noncompaction 4
left ventricular noncompaction 8 left ventricular noncompaction 3, with or without dilated cardiomyopathy
left ventricular noncompaction 2 left ventricular noncompaction 10
left ventricular noncompaction 9 left ventricular noncompaction 7

Diseases related to Left Ventricular Noncompaction via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 80)
idRelated DiseaseScoreTop Affiliating Genes
1barth syndrome30.9DTNA, TAZ, LDB3
2hypertrophic cardiomyopathy30.9MYBPC3, MYH7
3left ventricular noncompaction 630.7TNNT2, CMD1D
4duchenne muscular dystrophy30.5SNTA1, DTNB
5sudden infant death syndrome30.4SCN5A
6atrioventricular block30.3LMNA, SCN5A
7n syndrome10.8
8left ventricular noncompaction 1, with or without congenital heart defects10.6
9ebstein anomaly10.6
10left ventricular noncompaction 510.6
11left ventricular noncompaction 410.6
12left ventricular noncompaction 210.6
13peripartum cardiomyopathy10.5
14double-orifice mitral valve10.4
15sotos syndrome10.4
16sensorineural hearing loss10.4
17brugada syndrome10.4
18ventricular septal defect10.4
19left ventricular noncompaction 810.4
20left ventricular noncompaction 3, with or without dilated cardiomyopathy10.4
21left ventricular noncompaction 1010.4
22left ventricular noncompaction 910.4
23left ventricular noncompaction 710.4
24inclusion body myositis10.3
25adult syndrome10.3
26tooth disease10.3
27toxoplasmosis10.3
28autonomic dysfunction10.3
29lown-ganong-levine syndrome10.2
30marfan syndrome10.2
31mitral valve prolapse10.2
32periventricular nodular heterotopia10.2
33pancytopenia10.2
34endomyocardial fibrosis10.2
35becker muscular dystrophy10.2
36fabry disease10.2
37coffin-lowry syndrome10.2
38patent ductus arteriosus10.2
39thalassemia10.2
40multiminicore disease10.2
41polymicrogyria10.2
42corpus callosum agenesis10.2
43bilateral perisylvian polymicrogyria10.2
44familial dilated cardiomyopathy10.2
45cutaneous sclerosis10.2
46hydrops fetalis10.2
47pierre robin sequence10.2
48partial atrioventricular canal10.2
49single ventricular heart10.2
50familial isolated noncompaction of left ventricular myocardium10.2

Graphical network of the top 20 diseases related to Left Ventricular Noncompaction:



Diseases related to left ventricular noncompaction

Clinical Features for Left Ventricular Noncompaction

Drugs & Therapeutics for Left Ventricular Noncompaction

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Left Ventricular Noncompaction

Drug clinical trials:

Search ClinicalTrials for Left Ventricular Noncompaction

Search NIH Clinical Center for Left Ventricular Noncompaction

Search CenterWatch for Left Ventricular Noncompaction

Genetic Tests for Left Ventricular Noncompaction

Sources:
22GTR
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Genetic tests related to Left Ventricular Noncompaction:

id Genetic test Affiliating Genes
1 Left Ventricular Noncompaction22

Anatomical Context for Left Ventricular Noncompaction

Sources:
33MalaCards
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MalaCards organs/tissues related to Left Ventricular Noncompaction:

33
Heart, Smooth muscle, Kidney, Atrioventricular node

Animal Models for Left Ventricular Noncompaction or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Left Ventricular Noncompaction

Sources:
51PubMed
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Articles related to Left Ventricular Noncompaction:

(show top 50)    (show all 288)
idTitleAuthorsYear
1
Incidental diagnosis of a familial left ventricular noncompaction on a chest CT angiography. (24018053)
2014
2
Juvenile neuronal ceroid-lipofuscinosis with hypertrophic cardiomyopathy and left ventricular noncompaction: a case report. (24429647)
2014
3
Refinement of echocardiographic criteria for left ventricular noncompaction. (21944384)
2013
4
Features of left ventricular noncompaction in peripartum cardiomyopathy: a case series. (23103144)
2013
5
An adult patient with left ventricular noncompaction detected on radionuclide myocardial perfusion imaging. (23503407)
2013
6
Human immunodeficiency virus and left ventricular noncompaction. (23958416)
2013
7
Hypertrophic cardiomyopathy associated with left ventricular noncompaction cardiomyopathy and coronary fistulae: A case report. One genotype, three phenotypes? (24268868)
2013
8
Rare combination of left ventricular noncompaction, bicuspid aortic valve and myocardial bridging. Rare case or common genetic mutations? (24360154)
2013
9
Compacta-thickness--a valuable or unhelpful criterion in the diagnosis of left ventricular noncompaction/hypertrabeculation? (23176775)
2013
10
Left ventricular noncompaction in Duchenne muscular dystrophy. (23914774)
2013
11
Fetal diagnosis of left-ventricular noncompaction cardiomyopathy in identical twins with discordant congenital heart disease. (22695766)
2013
12
Left ventricular noncompaction cardiomyopathy: updated review. (24132556)
2013
13
Surgery for congenital aortic stenosis in children with left ventricular noncompaction. (23763604)
2013
14
Three-dimensional echocardiography for the evaluation of left ventricular noncompaction. (22658560)
2012
15
Loss of function of hNav1.5 by a ZASP1 mutation associated with intraventricular conduction disturbances in left ventricular noncompaction. (22929165)
2012
16
Left ventricular noncompaction and aneurysm revealed by left ventriculography. (21954168)
2012
17
Left ventricular noncompaction. (22565075)
2012
18
Failure of transvenous ICD to terminate ventricular fibrillation in a patient with left ventricular noncompaction and polycystic kidneys. (20946291)
2012
19
Left ventricular noncompaction presenting as peripartum cardiomyopathy. (21741100)
2012
20
Left ventricular noncompaction in a patient with multiminicore disease. (20729748)
2012
21
Multiple left ventricular thrombi in a patient with left ventricular noncompaction. (22949776)
2012
22
Left-ventricular noncompaction in a pediatric population: predictors of survival. (21188370)
2011
23
Histopathological features of delayed enhancement cardiovascular magnetic resonance in isolated left ventricular noncompaction. (21737026)
2011
24
Cardiac magnetic resonance imaging characteristics of isolated left ventricular noncompaction in a Chinese adult Han population. (21046254)
2011
25
Transition m.3308T>C in the ND1 gene is associated with left ventricular hypertrabeculation/noncompaction. (21625124)
2011
26
Left ventricular noncompaction associated with a sinoatrial node artery originating from the posterolateral branch of the right coronary artery. (21446386)
2011
27
Isolated left ventricular noncompaction in a case of sotos syndrome: a casual or causal link? (21747990)
2011
28
Coffin-Lowry syndrome and left ventricular noncompaction cardiomyopathy with a restrictive pattern. (22009732)
2011
29
Left ventricular noncompaction: analysis of a pediatric population. (21638988)
2011
30
Left ventricular noncompaction: travelling the road from diagnosis to outcomes. (20122495)
2010
31
The importance of genetic counseling, DNA diagnostics, and cardiologic family screening in left ventricular noncompaction cardiomyopathy. (20530761)
2010
32
Images in cardiovascular medicine. Overlapping phenotypes: left ventricular noncompaction and hypertrophic cardiomyopathy. (19528341)
2009
33
Left ventricular noncompaction: diagnosis by three-dimensional echocardiography. (20222350)
2009
34
Aortic valve replacement for aortic regurgitation in a patient with left ventricular noncompaction. (19101315)
2009
35
Left ventricular noncompaction: a cardiomyopathy often mistaken. (19700911)
2009
36
An unusual cause of cardioembolic stroke: isolated left ventricular noncompaction. (19131862)
2009
37
Deficiency of mannose-binding lectin, myopathy, calcified endomyocardial fibrosis, and left ventricular noncompaction. (18992631)
2008
38
Diagnosing left ventricular noncompaction by echocardiography and cardiac magnetic resonance imaging and its dependency on neuromuscular disorders. (18727062)
2008
39
Isolated noncompaction of the left ventricular myocardium complicated by thromboembolic cerebrovascular accident in a patient with essential thrombocythemia. (17669523)
2008
40
Isolated left ventricular noncompaction in association with rheumatic mitral stenosis. (17336412)
2008
41
Left ventricular noncompaction or papillary muscle? (17700403)
2007
42
Left ventricular restoration surgery for isolated left ventricular noncompaction: report of the first successful case. (17599523)
2007
43
Left ventricular noncompaction with a single coronary artery of anomalous origin. (17442437)
2007
44
Differential diagnoses and extracardiac manifestations in left ventricular noncompaction. (17700404)
2007
45
Isolated left ventricular noncompaction: echocardiographic diagnosis. (16640720)
2006
46
Live/real time three-dimensional echocardiographic demonstration of left ventricular noncompaction and thrombi. (16970725)
2006
47
Left ventricular noncompaction: a pathological study of 14 cases. (15892002)
2005
48
Left ventricular noncompaction with hypothyroidism and sensorineural hearing loss. (15939692)
2005
49
Novel gene locus for autosomal dominant left ventricular noncompaction maps to chromosome 11p15. (15173023)
2004
50
Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. (14623814)
2003

Genetic Variations for Left Ventricular Noncompaction

Expression for genes affiliated with Left Ventricular Noncompaction

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Left Ventricular Noncompaction

Search GEO for disease gene expression data for Left Ventricular Noncompaction.

Pathways for genes affiliated with Left Ventricular Noncompaction

Sources:
38NCBI BioSystems Database, 52QIAGEN, 54Reactome, 4Cell Signaling Technology, 30KEGG, 50PharmGKB
See all sources

Compounds for genes affiliated with Left Ventricular Noncompaction

Sources:
45Novoseek, 24HMDB, 29IUPHAR, 60Tocris Bioscience, 50PharmGKB, 2BitterDB, 11DrugBank
See all sources

Compounds related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1valine459.6MYH7, MT-ND1, SCN5A, MBL2
2ruthenium459.4RYR2, FKBP1A
3creatinine459.3MBL2, TNNT2, MYH7, LMNA, ACTC1
4sodium45 2410.2MBL2, SCN5A, SNTA1, MYH7, RYR2
5ryanodine45 29 6011.0FKBP1B, FKBP1A, MYH7, RYR2
6arginine458.8LMNA, MYH7, MYBPC3, FKBP1A, SCN5A
7caffeine45 50 29 2 11 2413.7FKBP1B, FKBP1A, RYR2
8atp45 299.3MBL2, MT-ND1, FKBP1A, MYBPC3, MYH7, RYR2
9lipid458.1MBL2, FKBP1B, FKBP1A, MYH7, RYR2, TAZ
10calcium45 50 11 2410.3TPM1, MBL2, TNNT2, SCN5A, SNTA1, FKBP1B

GO Terms for genes affiliated with Left Ventricular Noncompaction

Sources:
16Gene Ontology
See all sources

Cellular components related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1calcium channel complexGO:0347049.6RYR2, FKBP1B
2sarcomereGO:0300179.4TPM1, ACTC1, MYH7, MYBPC3, TNNT2
3cytoskeletonGO:0058569.1CSRP3, SNTA1, LDB3, TPM1
4sarcoplasmic reticulum membraneGO:0330178.9RYR2, FKBP1A, FKBP1B
5Z discGO:0300188.2CSRP3, FKBP1B, FKBP1A, MYH7, RYR2, LDB3

Biological processes related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

(show all 31)
idNameGO IDScoreTop Affiliating Genes
1regulation of sodium ion transmembrane transportGO:190230510.3SCN5A, SNTA1
2positive regulation of ATPase activityGO:03278110.2TNNT2, MYBPC3, TPM1
3ATP catabolic processGO:00620010.1ACTC1, MYH7, TNNT2
4cardiac myofibril assemblyGO:05500310.1ACTC1, CSRP3
5cardiac muscle tissue developmentGO:04873810.1CSRP3, TAZ
6regulation of muscle contractionGO:00693710.1TNNT2, TPM1
7cardiac muscle hypertrophyGO:00330010.1RYR2, CSRP3
8regulation of ventricular cardiac muscle cell membrane repolarizationGO:06030710.1SCN5A, SNTA1
9regulation of ventricular cardiac muscle cell action potentialGO:08600510.0RYR2, SNTA1, SCN5A
10sarcomere organizationGO:04521410.0TPM1, LDB3, MYBPC3, TNNT2
11positive regulation of sequestering of calcium ionGO:05128410.0RYR2, FKBP1B
12release of sequestered calcium ion into cytosol by sarcoplasmic reticulumGO:0148089.9FKBP1B, RYR2
13cell communication by electrical coupling involved in cardiac conductionGO:0860649.9FKBP1B, RYR2
14response to redox stateGO:0517759.9FKBP1B, RYR2
15calcium-mediated signaling using intracellular calcium sourceGO:0355849.8FKBP1B, RYR2
16muscle filament slidingGO:0300499.8TNNT2, MYBPC3, MYH7, ACTC1, TPM1
17negative regulation of release of sequestered calcium ion into cytosolGO:0512809.7FKBP1B, FKBP1A
18negative regulation of protein phosphatase type 2B activityGO:0325139.7FKBP1B, FKBP1A
19de novo protein foldingGO:0064589.7FKBP1B, FKBP1A
20protein maturation by protein foldingGO:0224179.6FKBP1B, FKBP1A
21protein refoldingGO:0420269.6FKBP1B, FKBP1A
22negative regulation of ryanodine-sensitive calcium-release channel activityGO:0603159.6FKBP1B, FKBP1A
23regulation of heart rateGO:0020279.5SCN5A, SNTA1, MYBPC3, MYH7, RYR2
24regulation of ryanodine-sensitive calcium-release channel activityGO:0603149.5FKBP1B, FKBP1A
25cytosolic calcium ion homeostasisGO:0514809.5FKBP1B, RYR2
26muscle contractionGO:0069369.5SNTA1, FKBP1B, MYH7, TAZ, TPM1
27ventricular cardiac muscle tissue morphogenesisGO:0550109.3TPM1, MYH7, MYBPC3, FKBP1A, TNNT2
28peptidyl-proline modificationGO:0182089.3FKBP1A, FKBP1B
29protein peptidyl-prolyl isomerizationGO:0004139.3FKBP1A, FKBP1B
30regulation of cardiac muscle contraction by regulation of the release of sequestered calcium ionGO:0108819.3FKBP1B, RYR2
31cardiac muscle contractionGO:0600489.0TPM1, ACTC1, TAZ, RYR2, MYBPC3, SCN5A

Molecular functions related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1nitric-oxide synthase bindingGO:05099810.0SCN5A, SNTA1
2myosin bindingGO:01702210.0ACTC1, MYBPC3
3structural constituent of muscleGO:0083079.6TPM1, MYH7, MYBPC3, CSRP3
4ATPase activityGO:0168879.6ACTC1, MYH7, TNNT2
5calmodulin bindingGO:0055169.5RYR2, MYH7, SNTA1, SCN5A
6actin bindingGO:0037799.5TNNT2, SNTA1, MYBPC3, MYH7, TPM1
7calcium channel inhibitor activityGO:0198559.3FKBP1B, FKBP1A
8FK506 bindingGO:0055289.2FKBP1A, FKBP1B
9ryanodine-sensitive calcium-release channel activityGO:0052199.2FKBP1B, FKBP1A, RYR2
10ion channel bindingGO:0443258.7RYR2, FKBP1A, FKBP1B, SNTA1, SCN5A

Products for genes affiliated with Left Ventricular Noncompaction

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Sources for Left Ventricular Noncompaction

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet