LVNC
MCID: LFT003
MIFTS: 43

Left Ventricular Noncompaction (LVNC) malady

Genetic diseases, Rare diseases, Cardiovascular diseases categories
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Summaries for Left Ventricular Noncompaction

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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 hypertrophic cardiomyopathy and barth syndrome. An important gene associated with Left Ventricular Noncompaction is DTNA (dystrobrevin, alpha), and among its related pathways are Hypertrophic cardiomyopathy (HCM) and Striated Muscle Contraction. Affiliated tissues include heart and kidney, and related mouse phenotypes are muscle and cardiovascular system.

Aliases & Classifications for Left Ventricular Noncompaction

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43NIH Rare Diseases, 22GTR, 62UMLS
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic diseases, Rare diseases
Anatomical: Cardiovascular diseases


Aliases & Descriptions:

left ventricular noncompaction 43 22 62
left ventricular hypertrabeculation 43
spongy myocardium 43
lvnc 43


Related Diseases for Left Ventricular Noncompaction

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17GeneCards, 18GeneDecks
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Diseases in the Left Ventricular Noncompaction 6 family:

left ventricular noncompaction Left Ventricular Noncompaction 5
Left Ventricular Noncompaction 4 Left Ventricular Noncompaction 8
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 78)
idRelated DiseaseScoreTop Affiliating Genes
1hypertrophic cardiomyopathy30.9TPM1, MYBPC3
2barth syndrome30.9DTNA, TAZ, LDB3
3myopathy30.9TAZ, LDB3, DTNA, TPM1
4dilated cardiomyopathy30.6TAZ, LDB3, MYBPC3, TPM1
5congenital heart disease30.2MYBPC3, TAZ
6left ventricular noncompaction 1, with or without congenital heart defects10.6
7left ventricular noncompaction 510.6
8left ventricular noncompaction 410.6
9peripartum cardiomyopathy10.5
10left ventricular noncompaction 610.5
11left ventricular noncompaction 210.5
12double-orifice mitral valve10.5
13myotonic dystrophy10.4
14myotonic dystrophy type 110.4
15sensorineural hearing loss10.4
16brugada syndrome10.4
17ebstein anomaly10.4
18hypothyroidism10.4
19long qt syndrome10.4
20muscular dystrophy10.4
21myocardial infarction10.4
22aneurysm10.4
23left ventricular noncompaction 810.4
24left ventricular noncompaction 3, with or without dilated cardiomyopathy10.4
25left ventricular noncompaction 1010.4
26left ventricular noncompaction 910.4
27left ventricular noncompaction 710.4
28inclusion body myositis10.3
29sudden infant death syndrome10.3
30charcot-marie-tooth disease10.3
31myositis10.3
32poliomyelitis10.3
33tooth disease10.3
34toxoplasmosis10.3
35charcot-marie-tooth disease type 1a10.3
36mannose-binding lectin deficiency10.3
37autonomic dysfunction10.3
38atrioventricular block10.2
39mitral valve prolapse10.2
40neuronitis10.2
41endomyocardial fibrosis10.2
42becker muscular dystrophy10.2
43duchenne muscular dystrophy10.2
44tetralogy of fallot10.2
45sotos syndrome10.2
46periventricular nodular heterotopia10.2
47pancytopenia10.2
48fabry disease10.2
49patent ductus arteriosus10.2
50coffin-lowry syndrome10.2

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



Diseases related to left ventricular noncompaction

Symptoms for Left Ventricular Noncompaction

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Drugs & Therapeutics for Left Ventricular Noncompaction

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Sources:
42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for Left Ventricular Noncompaction

Search NIH Clinical Center for Left Ventricular Noncompaction

Genetic Tests for Left Ventricular Noncompaction

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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

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

33
Heart, Kidney

Animal Models for Left Ventricular Noncompaction or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Left Ventricular Noncompaction:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053698.6LDB3, DTNA, MYBPC3
2MP:00053858.0LDB3, DTNA, MYBPC3, TPM1
3MP:00053787.8TPM1, MYBPC3, DTNA, LDB3

Publications for Left Ventricular Noncompaction

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52PubMed
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Articles related to Left Ventricular Noncompaction:

(show top 50)    (show all 263)
idTitleAuthorsYear
1
Magnetic resonance assessment of prevalence and correlates of right ventricular abnormalities in isolated left ventricular noncompaction. (24176065)
2014
2
Molecular mechanism of ventricular trabeculation/compaction and the pathogenesis of the left ventricular noncompaction cardiomyopathy (LVNC). (23843320)
2013
3
Refinement of echocardiographic criteria for left ventricular noncompaction. (21944384)
2013
4
Mutations in the NOTCH pathway regulator MIB1 cause left ventricular noncompaction cardiomyopathy. (23314057)
2013
5
Focal left atrial tachycardia in a patient with left ventricular noncompaction. (23762071)
2013
6
Features of left ventricular noncompaction in peripartum cardiomyopathy: a case series. (23103144)
2013
7
Coexistence of left ventricular noncompaction and aortic interruption. (23724972)
2013
8
Left ventricular noncompaction cardiomyopathy. (24282766)
2013
9
Three-dimensional echocardiography for the evaluation of left ventricular noncompaction. (22658560)
2012
10
Predictors of adverse outcome in adolescents and adults with isolated left ventricular noncompaction. (22036106)
2012
11
Loss of function of hNav1.5 by a ZASP1 mutation associated with intraventricular conduction disturbances in left ventricular noncompaction. (22929165)
2012
12
Left ventricular noncompaction and aneurysm revealed by left ventriculography. (21954168)
2012
13
Left ventricular noncompaction: a proposal of new diagnostic criteria by multidetector computed tomography. (22981859)
2012
14
Left ventricular noncompaction cardiomyopathy and aortopathy in a patient with recessive dystrophic epidermolysis bullosa. (22991407)
2012
15
Isolated left ventricular noncompaction in a 90-year-old man. (22740746)
2012
16
Three-dimensional echocardiographic characterization of patients with left ventricular noncompaction. (22154390)
2012
17
Left ventricular noncompaction. (22565075)
2012
18
Left ventricular noncompaction: a 25-year odyssey. (22284845)
2012
19
Isolated left ventricular noncompaction cardiomyopathy diagnosed by transesophageal echocardiography. (21487457)
2011
20
Electrocardiographic findings at initial diagnosis in children with isolated left ventricular noncompaction. (21496170)
2011
21
Left ventricular noncompaction cardiomyopathy. (22218001)
2011
22
Implantable cardioverter defibrillator in a young child with left ventricular noncompaction. (21798475)
2011
23
Long-term follow-up of patients with isolated left ventricular noncompaction: role of electrocardiography in predicting poor outcome. (21617326)
2011
24
Left ventricular noncompaction in a patient with fabry disease: overdiagnosis, morphological manifestation of fabry disease or two unrelated rare conditions in the same patient? (21952291)
2011
25
Left-ventricular noncompaction in a pediatric population: predictors of survival. (21188370)
2011
26
Sudden unexpected death due to left ventricular noncompaction of myocardium: case report and review of the literature. (20032775)
2010
27
Left ventricular noncompaction and myocardial fibrosis: a case report. (20843341)
2010
28
Left ventricular noncompaction: a new form of heart failure. (20869646)
2010
29
Isolated left ventricular noncompaction syndrome. (19801037)
2009
30
Genetic heterogeneity of left-ventricular noncompaction cardiomyopathy. (17729299)
2008
31
New classification scheme of left ventricular noncompaction and correlation with ventricular performance. (18572043)
2008
32
SCN5A variants in Japanese patients with left ventricular noncompaction and arrhythmia. (18368697)
2008
33
Sinus bradycardia, Wolff Parkinson White, and left ventricular noncompaction: an embryologic connection? (17786379)
2008
34
Echocardiography in the diagnosis left ventricular noncompaction. (19105818)
2008
35
Left ventricular noncompaction/hypertrabeculation cardiomyopathy. (19400533)
2008
36
An unusual cause of cardioembolic stroke: isolated left ventricular noncompaction. (18332842)
2008
37
Left ventricular noncompaction or papillary muscle? (17700403)
2007
38
Reply to 'Left ventricular noncompaction or papillary muscle?'. (17700418)
2007
39
Left ventricular restoration surgery for isolated left ventricular noncompaction: report of the first successful case. (17599523)
2007
40
Comparison of regional systolic myocardial velocities in patients with isolated left ventricular noncompaction and patients with idiopathic dilated cardiomyopathy. (17543758)
2007
41
Sporadic isolated left ventricular noncompaction: dread disease or not? (17437566)
2007
42
Images in cardiovascular medicine. Isolated left ventricular noncompaction enhanced by echocontrast agent. (17646590)
2007
43
Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. (17885073)
2007
44
Comparison of regional systolic myocardial velocities in patients with isolated left ventricular noncompaction and patients with idiopathic dilated cardiomyopathy. (17098133)
2006
45
Cardiomyopathy and myopathy in left ventricular noncompaction. (17126673)
2006
46
Isolated left ventricular noncompaction of the myocardium as a cause of embolic superior mesenteric artery occlusion. (15947775)
2005
47
Left ventricular noncompaction cardiomyopathy in association with trisomy 13. (15549619)
2005
48
Double-orifice mitral valve associated with nonisolated left ventricular noncompaction--a case report. (15547659)
2004
49
Do cypher gene mutations cause left ventricular noncompaction with subclinical myopathy? (15337232)
2004
50
Isolated left ventricular noncompaction in an adult. (12216935)
2002

Variations for Left Ventricular Noncompaction

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Expression for genes affiliated with Left Ventricular Noncompaction

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2BioGPS, 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

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50PathCards, 30KEGG, 38NCBI BioSystems Database, 55Reactome
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Pathways related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
Arrhythmogenic right ventricular cardiomyopathy38
9.1MYBPC3, TPM1
2
Show member pathways
9.1MYBPC3, TPM1

Compounds for genes affiliated with Left Ventricular Noncompaction

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GO Terms for genes affiliated with Left Ventricular Noncompaction

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16Gene Ontology
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Cellular components related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytoskeletonGO:0058569.3LDB3, TPM1
2sarcomereGO:0300178.8MYBPC3, TPM1

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

idNameGO IDScoreTop Affiliating Genes
1muscle contractionGO:0069369.4TAZ, TPM1
2positive regulation of ATPase activityGO:0327819.1TPM1, MYBPC3
3ventricular cardiac muscle tissue morphogenesisGO:0550109.0MYBPC3, TPM1
4muscle filament slidingGO:0300498.8MYBPC3, TPM1
5cardiac muscle contractionGO:0600488.7TAZ, MYBPC3, TPM1
6sarcomere organizationGO:0452148.6LDB3, MYBPC3, TPM1

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

idNameGO IDScoreTop Affiliating Genes
1actin bindingGO:0037799.1MYBPC3, TPM1
2cytoskeletal protein bindingGO:0080929.0LDB3, TPM1
3structural constituent of muscleGO:0083079.0MYBPC3, TPM1

Products for genes affiliated with Left Ventricular Noncompaction

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Sources for Left Ventricular Noncompaction

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet