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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NIH Rare Diseases:42 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 based summary: 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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Left Ventricular Noncompaction, Aliases & Descriptions:

Name: Left Ventricular Noncompaction 42 22 62
Left Ventricular Hypertrabeculation 42
 
Spongy Myocardium 42
Lvnc 42


Classifications:

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


Related Diseases for Left Ventricular Noncompaction

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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 85)
idRelated DiseaseScoreTop Affiliating Genes
1hypertrophic cardiomyopathy30.8TPM1, MYBPC3
2barth syndrome30.7DTNA, TAZ, LDB3
3myopathy30.4TAZ, LDB3, DTNA, TPM1
4congenital heart disease30.2MYBPC3, TAZ
5dilated cardiomyopathy29.9TAZ, LDB3, MYBPC3, TPM1
6left ventricular noncompaction 510.6
7left ventricular noncompaction 410.6
8left ventricular noncompaction 1, with or without congenital heart defects10.6
9peripartum cardiomyopathy10.5
10left ventricular noncompaction 610.5
11left ventricular noncompaction 210.5
12left ventricular noncompaction 710.5
13double-orifice mitral valve10.5
14myotonic dystrophy type 110.4
15myotonic dystrophy10.4
16myocardial infarction10.4
17sotos syndrome10.4
18fabry disease10.4
19ebstein anomaly10.4
20sensorineural hearing loss10.4
21brugada syndrome10.4
22hypothyroidism10.4
23long qt syndrome10.4
24muscular dystrophy10.4
25aneurysm10.4
26left ventricular noncompaction 810.4
27cardiomyopathy, dilated, 1e10.4
28left ventricular noncompaction 3, with or without dilated cardiomyopathy10.4
29left ventricular noncompaction 1010.4
30left ventricular noncompaction 910.4
31charcot-marie-tooth disease10.3
32sudden infant death syndrome10.3
33inclusion body myositis10.3
34myositis10.3
35neuromuscular disease10.3
36poliomyelitis10.3
37tooth disease10.3
38toxoplasmosis10.3
39charcot-marie-tooth disease type 1a10.3
40mannose-binding lectin deficiency10.3
41autonomic dysfunction10.3
42becker muscular dystrophy10.2
43celiac disease10.2
44congestive heart failure10.2
45duchenne muscular dystrophy10.2
46hypertension10.2
47pulmonary hypertension10.2
48thalassemia10.2
49marfan syndrome10.2
50mitral valve prolapse10.2

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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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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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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MalaCards organs/tissues related to Left Ventricular Noncompaction:

32
Heart, Kidney

Animal Models for Left Ventricular Noncompaction or affiliated genes

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MGI Mouse Phenotypes related to Left Ventricular Noncompaction:

36
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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Articles related to Left Ventricular Noncompaction:

(show top 50)    (show all 284)
idTitleAuthorsYear
1
HCN4 mutations in multiple families with bradycardia and left ventricular noncompaction cardiomyopathy. (25145517)
2014
2
Possible link between right ventricular coronary sinusoids and noncompaction sinusoids in pulmonary atresia with intact ventricular septum patients that later develop left ventricular noncompaction. (24767937)
2014
3
A case of an infant with compound heterozygous mutations for hypertrophic cardiomyopathy producing a phenotype of left ventricular noncompaction. (25262865)
2014
4
Ischemic stroke in left ventricular noncompaction and celiac disease. (25086466)
2014
5
Left ventricular noncompaction - a case report. (25178616)
2014
6
Guiding the management of ventricular arrhythmias in patients with left ventricular noncompaction cardiomyopathy: a knowledge gap. (25290726)
2014
7
Magnetic resonance assessment of prevalence and correlates of right ventricular abnormalities in isolated left ventricular noncompaction. (24176065)
2014
8
Molecular mechanism of ventricular trabeculation/compaction and the pathogenesis of the left ventricular noncompaction cardiomyopathy (LVNC). (23843320)
2013
9
Refinement of echocardiographic criteria for left ventricular noncompaction. (21944384)
2013
10
Focal left atrial tachycardia in a patient with left ventricular noncompaction. (23762071)
2013
11
Features of left ventricular noncompaction in peripartum cardiomyopathy: a case series. (23103144)
2013
12
Coexistence of left ventricular noncompaction and aortic interruption. (23724972)
2013
13
Left ventricular noncompaction cardiomyopathy. (24282766)
2013
14
Three-dimensional echocardiography for the evaluation of left ventricular noncompaction. (22658560)
2012
15
Predictors of adverse outcome in adolescents and adults with isolated left ventricular noncompaction. (22036106)
2012
16
Loss of function of hNav1.5 by a ZASP1 mutation associated with intraventricular conduction disturbances in left ventricular noncompaction. (22929165)
2012
17
Left ventricular noncompaction and aneurysm revealed by left ventriculography. (21954168)
2012
18
Left ventricular noncompaction: a proposal of new diagnostic criteria by multidetector computed tomography. (22981859)
2012
19
Left ventricular noncompaction cardiomyopathy and aortopathy in a patient with recessive dystrophic epidermolysis bullosa. (22991407)
2012
20
Isolated left ventricular noncompaction in a 90-year-old man. (22740746)
2012
21
Three-dimensional echocardiographic characterization of patients with left ventricular noncompaction. (22154390)
2012
22
Left ventricular noncompaction. (22565075)
2012
23
Isolated left ventricular noncompaction cardiomyopathy diagnosed by transesophageal echocardiography. (21487457)
2011
24
Electrocardiographic findings at initial diagnosis in children with isolated left ventricular noncompaction. (21496170)
2011
25
Left ventricular noncompaction cardiomyopathy. (22218001)
2011
26
Implantable cardioverter defibrillator in a young child with left ventricular noncompaction. (21798475)
2011
27
Long-term follow-up of patients with isolated left ventricular noncompaction: role of electrocardiography in predicting poor outcome. (21617326)
2011
28
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
29
Left-ventricular noncompaction in a pediatric population: predictors of survival. (21188370)
2011
30
Sudden unexpected death due to left ventricular noncompaction of myocardium: case report and review of the literature. (20032775)
2010
31
Left ventricular noncompaction and myocardial fibrosis: a case report. (20843341)
2010
32
Left ventricular noncompaction: a new form of heart failure. (20869646)
2010
33
Isolated left ventricular noncompaction syndrome. (19801037)
2009
34
Genetic heterogeneity of left-ventricular noncompaction cardiomyopathy. (17729299)
2008
35
New classification scheme of left ventricular noncompaction and correlation with ventricular performance. (18572043)
2008
36
SCN5A variants in Japanese patients with left ventricular noncompaction and arrhythmia. (18368697)
2008
37
Sinus bradycardia, Wolff Parkinson White, and left ventricular noncompaction: an embryologic connection? (17786379)
2008
38
Echocardiography in the diagnosis left ventricular noncompaction. (19105818)
2008
39
Left ventricular noncompaction/hypertrabeculation cardiomyopathy. (19400533)
2008
40
Left ventricular noncompaction or papillary muscle? (17700403)
2007
41
Reply to 'Left ventricular noncompaction or papillary muscle?'. (17700418)
2007
42
Left ventricular restoration surgery for isolated left ventricular noncompaction: report of the first successful case. (17599523)
2007
43
Comparison of regional systolic myocardial velocities in patients with isolated left ventricular noncompaction and patients with idiopathic dilated cardiomyopathy. (17543758)
2007
44
Sporadic isolated left ventricular noncompaction: dread disease or not? (17437566)
2007
45
Images in cardiovascular medicine. Isolated left ventricular noncompaction enhanced by echocontrast agent. (17646590)
2007
46
Comparison of regional systolic myocardial velocities in patients with isolated left ventricular noncompaction and patients with idiopathic dilated cardiomyopathy. (17098133)
2006
47
Isolated left ventricular noncompaction of the myocardium as a cause of embolic superior mesenteric artery occlusion. (15947775)
2005
48
Left ventricular noncompaction cardiomyopathy in association with trisomy 13. (15549619)
2005
49
Double-orifice mitral valve associated with nonisolated left ventricular noncompaction--a case report. (15547659)
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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Expression patterns in normal tissues for genes affiliated with Left Ventricular Noncompaction

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Pathways for genes affiliated with Left Ventricular Noncompaction

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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 cardiomyopathy37
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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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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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet