Page last updated: 2024-09-27

Isolated Non-compaction of the Ventricular Myocardium

Rare congenital cardiomyopathies characterized by the lack of left ventricular myocardium compaction. The noncompaction results in numerous prominent trabeculations and a loose myocardial meshwork (spongy myocardium) in the LEFT VENTRICLE. Heterogeneous clinical features include diminished systolic function sometimes associated with left ventricular dilation, that presents either neonatally or progressively. Often, the RIGHT VENTRICLE is also affected. CONGESTIVE HEART FAILURE; PULMONARY EMBOLISM; and ventricular ARRHYTHMIA are commonly seen.

Synonyms(4)

Synonym
Isolated Noncompaction of the Left Ventricular Myocardium, X-Linked
Isolated Non-compaction of the Ventricular Myocardium
Noncompaction of the Left Ventricular Myocardium, Autosomal Dominant
Isolated Noncompaction of the Ventricular Myocardium

Research

Studies (55)

TimeframeStudies, This Condition (%)All Conditions %
pre-19900 (0.00)23.3326
1990's0 (0.00)12.5806
2000's2 (3.64)18.1394
2010's46 (83.64)28.8240
2020's7 (12.73)9.53
DrugIndicatedRelationship StrengthStudiesTrials
carnitine0low20
methylmalonic acid0low10
aspirin0low30
bisoprolol0low20
flecainide0low10
furosemide0low20
ml 70low10
ticlopidine0low10
spironolactone0low10
valine0low10
perflutren0low10
methylergonovine0low10
gadolinium0medium121
glutamic acid0low10
captopril0low10
clopidogrel0low10
3-iodobenzylguanidine0low10
fluorodeoxyglucose f180low10
16-iodo-3-methylhexadecanoic acid0low10
malonyl coenzyme a0low10
enalapril0low10
emerin0low10
gadolinium dtpa0low60
rivaroxaban0low10
endothelin-10low10
cardiovascular agents0low70
natriuretic peptide, brain0low40
warfarin0low40
phenprocoumon0low10
cytochalasin d0low10
folic acid0low10

Protein Targets (415)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
dopamine D1 receptor2002
thioredoxin reductase6006
GLS protein6006
Thrombopoietin2002
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)1001
cytochrome P450 2D6 isoform 13003
cytochrome P450 2C19 precursor3003
ras-related protein Rab-9A2002
histone acetyltransferase KAT2A isoform 13003
lethal factor (plasmid)3003
POU domain, class 2, transcription factor 10001
Spike glycoprotein7007
ATP-dependent phosphofructokinase6006
glp-1 receptor, partial5005
regulator of G-protein signaling 45005
estrogen-related nuclear receptor alpha5005
estrogen nuclear receptor alpha110011
NPYLR7B0011
arylsulfatase A4004
euchromatic histone-lysine N-methyltransferase 28008
thyroid stimulating hormone receptor2002
flap endonuclease 12002
peripheral myelin protein 224004
ATP-binding cassette sub-family C member 30707
Multidrug resistance-associated protein 40708
Bile salt export pump012012
Beta-2 adrenergic receptor0549
Beta-1 adrenergic receptor0246
60 kDa chaperonin0101
60 kDa heat shock protein, mitochondrial0101
Beta-3 adrenergic receptor0033
Delta-type opioid receptor0011
10 kDa heat shock protein, mitochondrial0101
Thiosulfate sulfurtransferase0101
Beta-2 adrenergic receptor 0013
60 kDa chaperonin 0101
10 kDa chaperonin 0101
Beta-2 adrenergic receptor0134
Canalicular multispecific organic anion transporter 10707
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE5005
Chain A, TYROSYL-DNA PHOSPHODIESTERASE3003
Chain A, Phospholipase A2 isoform 30011
Chain A, HADH2 protein4004
Chain B, HADH2 protein4004
Chain A, 2-oxoglutarate Oxygenase4004
GTP-binding protein (rab7)0011
ras protein, partial0011
interleukin 81001
AR protein110011
aldehyde dehydrogenase 1 family, member A16006
thyroid stimulating hormone receptor3003
hypothetical protein, conserved1001
cytochrome P450 family 3 subfamily A polypeptide 46006
EWS/FLI fusion protein6006
glucocorticoid receptor [Homo sapiens]7007
retinoic acid nuclear receptor alpha variant 15005
cytochrome P450 2D64004
glucocerebrosidase2002
peroxisome proliferator-activated receptor delta2002
Rac1 protein0011
IDH12002
cell division cycle 42 (GTP binding protein, 25kDa), partial0011
Bloom syndrome protein isoform 11001
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 12002
DNA polymerase iota isoform a (long)3003
DNA polymerase kappa isoform 13003
muscarinic acetylcholine receptor M11001
Prostaglandin-H2 D-isomerase0101
Solute carrier family 22 member 60202
Prostaglandin G/H synthase 1 0101
Prostaglandin G/H synthase 2 0101
Epidermal growth factor receptor0101
Carbonic anhydrase 10305
Carbonic anhydrase 20305
Fatty acid-binding protein, liver0202
Integrin beta-30112
Myeloperoxidase0101
Prostaglandin G/H synthase 10101
Seed linoleate 13S-lipoxygenase-10101
Integrin alpha-IIb0112
Glutathione hydrolase 1 proenzyme0101
Prostaglandin G/H synthase 10303
Adenosine receptor A10101
Adenosine receptor A2a0101
Substance-P receptor0101
Prostaglandin G/H synthase 20303
Urotensin-2 receptor0101
4-aminobutyrate aminotransferase, mitochondrial0101
Nuclear receptor ROR-gamma1001
Ras-related protein Rab-2A0011
Gamma-aminobutyric acid receptor subunit alpha-12114
Prostaglandin G/H synthase 20101
Rho-associated protein kinase 20101
Nicotinate phosphoribosyltransferase0101
Solute carrier family 22 member 200101
Solute carrier family 22 member 60101
Sigma non-opioid intracellular receptor 10213
Sigma non-opioid intracellular receptor 10011
Beta-1 adrenergic receptor 0011
D(1A) dopamine receptor3003
geminin6006
lamin isoform A-delta107007
Gamma-aminobutyric acid receptor subunit pi2013
Urease0101
Gamma-aminobutyric acid receptor subunit beta-12013
Gamma-aminobutyric acid receptor subunit delta2013
Gamma-aminobutyric acid receptor subunit gamma-22013
5-hydroxytryptamine receptor 1A0314
Gamma-aminobutyric acid receptor subunit alpha-52013
Gamma-aminobutyric acid receptor subunit alpha-32013
Gamma-aminobutyric acid receptor subunit gamma-12013
Gamma-aminobutyric acid receptor subunit alpha-22013
Alpha-1D adrenergic receptor0101
Gamma-aminobutyric acid receptor subunit alpha-42013
Gamma-aminobutyric acid receptor subunit gamma-32013
Gamma-aminobutyric acid receptor subunit alpha-62013
Alpha-1A adrenergic receptor0101
Alpha-1B adrenergic receptor0101
Gamma-aminobutyric acid receptor subunit beta-32013
Gamma-aminobutyric acid receptor subunit beta-22013
TAR DNA-binding protein 432002
GABA theta subunit2013
Gamma-aminobutyric acid receptor subunit epsilon2013
ATAD5 protein, partial4004
chromobox protein homolog 16006
Beta-1 adrenergic receptor0101
Chain A, angiotensin converting enzyme0101
Chain A, angiotensin converting enzyme0101
15-lipoxygenase, partial1001
phosphopantetheinyl transferase3003
Microtubule-associated protein tau2002
thioredoxin glutathione reductase1001
Metallo-beta-lactamase type 20112
peripheral myelin protein 22 isoform 11001
M-phase phosphoprotein 82002
transient receptor potential cation channel subfamily V member 11001
Prothrombin0213
Renin0101
Neprilysin0101
Neprilysin0101
Leukotriene A-4 hydrolase0213
EEF1AKMT4-ECE2 readthrough transcript protein0101
Angiotensin-converting enzyme 0314
Angiotensin-converting enzyme0304
Leukotriene A-4 hydrolase0101
Thymidine phosphorylase0011
Endothelin-converting enzyme 10101
Succinyl-diaminopimelate desuccinylase0303
Angiotensin-converting enzyme0303
Solute carrier family 15 member 10202
Beta-lactamase TEM0101
Mu-type opioid receptor0011
Beta-lactamase 0203
Beta-lactamase 0202
Angiotensin-converting enzyme 2 0202
Beta-lactamase class B VIM-2 0112
Solute carrier family 22 member 50001
Solute carrier family 22 member 50001
Solute carrier family 22 member 160001
Solute carrier family 22 member 210001
Solute carrier family 22 member 50001
nonstructural protein 11001
parathyroid hormone/parathyroid hormone-related peptide receptor precursor3003
Cytochrome P450 2C80202
Cytochrome P450 2B60303
Liver carboxylesterase 10001
Sodium-dependent serotonin transporter0404
Cytochrome P450 2C190404
Rap guanine nucleotide exchange factor 41001
P2Y purinoceptor 120101
endonuclease IV1001
USP1 protein, partial5005
nuclear receptor subfamily 1, group I, member 35005
farnesoid X nuclear receptor4004
Type-2 angiotensin II receptor0101
Solute carrier family 15 member 10101
Solute carrier organic anion transporter family member 1A10001
Multidrug and toxin extrusion protein 10404
Endothelin-1 receptor0101
TDP1 protein6006
potassium voltage-gated channel subfamily H member 2 isoform d2002
thyroid hormone receptor beta isoform 25005
Voltage-dependent L-type calcium channel subunit alpha-1F0101
Cytochrome P450 2C9 0404
Potassium voltage-gated channel subfamily A member 50101
Cytochrome P450 2J20303
Voltage-dependent L-type calcium channel subunit alpha-1D 0101
Potassium voltage-gated channel subfamily A member 10101
Potassium voltage-gated channel subfamily H member 20101
Voltage-dependent L-type calcium channel subunit alpha-1S0101
Voltage-dependent L-type calcium channel subunit alpha-1C0101
Sodium channel protein type 5 subunit alpha0101
Potassium voltage-gated channel subfamily D member 20101
Chain A, Dihydrofolate reductase0011
Chain A, Putative fructose-1,6-bisphosphate aldolase2002
Chain A, Ferritin light chain2002
acid sphingomyelinase1001
GLI family zinc finger 33003
retinoid X nuclear receptor alpha2002
vitamin D (1,25- dihydroxyvitamin D3) receptor2002
alpha-galactosidase1001
activating transcription factor 62002
lysosomal alpha-glucosidase preproprotein1001
nuclear factor erythroid 2-related factor 2 isoform 21001
DNA polymerase beta1001
Cellular tumor antigen p534004
Thymidylate synthase0202
Thymidylate synthase0101
Dihydrofolate reductase0011
Aldo-keto reductase family 1 member B10101
Alpha-synuclein1001
Folylpolyglutamate synthase, mitochondrial0001
Solute carrier organic anion transporter family member 1A30101
Folylpolyglutamate synthase, mitochondrial0001
Multidrug resistance associated protein0001
RAR-related orphan receptor gamma4004
estrogen receptor 2 (ER beta)2002
aryl hydrocarbon receptor2002
thyrotropin-releasing hormone receptor1001
nuclear factor erythroid 2-related factor 2 isoform 12002
Carbonic anhydrase 120102
Bile salt export pump0202
Albumin0112
Carbonic anhydrase 30101
Glucose-6-phosphate 1-dehydrogenase0101
Carbonic anhydrase 40102
Carbonic anhydrase 60102
Serum paraoxonase/arylesterase 10101
Corticosteroid 11-beta-dehydrogenase isozyme 10101
Carbonic anhydrase 5A, mitochondrial0102
Carbonic anhydrase 70102
Adenosine receptor A10101
Corticosteroid 11-beta-dehydrogenase isozyme 10101
6-phosphogluconate dehydrogenase, decarboxylating0101
Holo-[acyl-carrier-protein] synthase0101
Solute carrier organic anion transporter family member 2A10101
Sodium/bile acid cotransporter0101
Carbonic anhydrase 90102
Carbonic anhydrase 130102
G-protein coupled receptor 350112
CDGSH iron-sulfur domain-containing protein 10202
Carbonic anhydrase 140102
Broad substrate specificity ATP-binding cassette transporter ABCG20101
Carbonic anhydrase 5B, mitochondrial0102
Chain A, GLUTAMATE RECEPTOR SUBUNIT 20101
Chain A, Glutamate Receptor Subunit 20101
Chain B, Glutamate Receptor Subunit 20101
Chain A, Slr1257 protein0011
Chain A, Glucosamine--fructose-6-phosphate aminotransferase [isomerizing]0101
beta-2 adrenergic receptor1001
peptidyl-prolyl cis-trans isomerase NIMA-interacting 13003
Metabotropic glutamate receptor 80202
Metabotropic glutamate receptor 60113
Glutamate receptor ionotropic, NMDA 2D0101
Excitatory amino acid transporter 40101
Glutamate receptor ionotropic, NMDA 3B0101
Glutathione reductase, mitochondrial0202
Bifunctional aspartokinase/homoserine dehydrogenase 10101
ATP-citrate synthase 0101
Glutamate receptor 10213
Glutamate receptor 21214
Glutamate receptor 30213
Glutamate receptor 40213
Glutamate receptor ionotropic, kainate 10213
Metabotropic glutamate receptor 10112
Metabotropic glutamate receptor 20011
Metabotropic glutamate receptor 30011
Metabotropic glutamate receptor 40112
Metabotropic glutamate receptor 50011
Metabotropic glutamate receptor 60011
Metabotropic glutamate receptor 70011
Glutamate receptor ionotropic, NMDA 1 0213
Glutamate receptor ionotropic, kainate 10112
Metabotropic glutamate receptor 50113
Glutamate receptor ionotropic, kainate 20213
Glutamate receptor 10213
Glutamate receptor 20213
Glutamate receptor 30112
Glutamate receptor ionotropic, kainate 30202
Excitatory amino acid transporter 10203
Excitatory amino acid transporter 20203
Excitatory amino acid transporter 30203
Metabotropic glutamate receptor 80011
Glutamate receptor 40213
Excitatory amino acid transporter 3 0001
Glutamate racemase0001
Metabotropic glutamate receptor 80112
Fatty-acid amide hydrolase 10101
Glutamate receptor ionotropic, NMDA 2A 0213
Glutamate receptor ionotropic, NMDA 2B0213
Glutamate receptor ionotropic, NMDA 2C0213
Glutamate receptor ionotropic, kainate 40202
Glutamate carboxypeptidase 20101
Glutamate receptor ionotropic, NMDA 10101
Glutamate receptor ionotropic, NMDA 2A0101
Glutamate receptor ionotropic, kainate 20112
Glutamate receptor ionotropic, kainate 30101
Glutamate receptor ionotropic, NMDA 2B0101
Metabotropic glutamate receptor 10113
Metabotropic glutamate receptor 20113
Metabotropic glutamate receptor 70113
Metabotropic glutamate receptor 30113
Metabotropic glutamate receptor 40113
Glutamate receptor ionotropic, NMDA 2C0101
Glutamate receptor ionotropic, kainate 50101
Carbonic anhydrase-like protein, putative0001
Glutamate receptor ionotropic, NMDA 2D0213
Glutamate receptor ionotropic, kainate 50202
Glutamate racemase0001
Prolyl 4-hydroxylase0101
Glutamate receptor ionotropic, NMDA 3A0101
Glutamate receptor ionotropic, NMDA 3B0213
Alpha-ketoglutarate-dependent dioxygenase FTO0101
Glutamate receptor ionotropic, NMDA 3A0213
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a2002
nuclear receptor subfamily 1, group I, member 21001
pregnane X nuclear receptor4004
importin subunit beta-1 isoform 12002
snurportin-12002
GTP-binding nuclear protein Ran isoform 12002
Carnitine O-palmitoyltransferase 1, muscle isoform0101
Chain A, ATP-DEPENDENT DNA HELICASE Q11001
acetylcholinesterase2002
DNA polymerase III, partial1001
vitamin D3 receptor isoform VDRA1001
ubiquitin carboxyl-terminal hydrolase 2 isoform a2002
tumor susceptibility gene 101 protein2002
survival motor neuron protein isoform d1001
5-hydroxytryptamine receptor 40202
Alpha-2A adrenergic receptor0404
Cytochrome P450 2D60303
D(2) dopamine receptor0202
Alpha-2B adrenergic receptor0404
D(1A) dopamine receptor0202
5-hydroxytryptamine receptor 2A0202
5-hydroxytryptamine receptor 2C0202
5-hydroxytryptamine receptor 1B0202
D(3) dopamine receptor0202
5-hydroxytryptamine receptor 2B0213
5-hydroxytryptamine receptor 60202
Chain A, Beta-lactamase2002
Chain A, JmjC domain-containing histone demethylation protein 3A2002
Guanine nucleotide-binding protein G1001
Serine racemase0101
Sodium-dependent noradrenaline transporter 0202
Sodium-dependent dopamine transporter0101
Sodium-dependent dopamine transporter 0202
Fumarate hydratase1001
NFKB1 protein, partial1001
polyprotein1001
cellular tumor antigen p53 isoform a1001
atrial natriuretic peptide receptor 2 precursor1001
serine/threonine-protein kinase mTOR isoform 11001
nuclear receptor ROR-gamma isoform 11001
cytochrome P450 3A4 isoform 12002
D(1A) dopamine receptor1001
MO15-related protein kinase Pfmrk 0101
Rho-associated protein kinase 10101
Myosin light chain kinase, smooth muscle0101
Ataxin-22002
Atrial natriuretic peptide receptor 10101
Gag-Pol polyprotein0101
Gag-Pol polyprotein0101
Protease 0303
Chain A, Activated Factor Xa Heavy Chain0101
Coagulation factor X0202
Coagulation factor XII0101
Suppressor of tumorigenicity 14 protein0101
Chain A, Mineralocorticoid receptor0101
Chain A, Mineralocorticoid receptor0101
pregnane X receptor1001
SMAD family member 21001
SMAD family member 31001
Smad31001
progesterone receptor3003
67.9K protein1001
peroxisome proliferator activated receptor gamma2002
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a2002
Histone H2A.x1001
thyroid hormone receptor beta isoform a2002
Vpr1001
Voltage-dependent calcium channel gamma-2 subunit1001
Estrogen receptor0213
Glucocorticoid receptor0213
Progesterone receptor0213
Glycine receptor subunit alpha-10202
ATP-dependent translocase ABCB10101
Mineralocorticoid receptor 0203
Androgen receptor0213
Androgen receptor0203
Glycine receptor subunit beta0202
Glycine receptor subunit alpha-20202
Glycine receptor subunit alpha-30202
Sodium-dependent serotonin transporter0101
Estrogen receptor beta0112
ATPase family AAA domain-containing protein 51001
Luciferase1001
G1001
v-jun sarcoma virus 17 oncogene homolog (avian)1001
heat shock protein beta-11001
Interferon beta2002
HLA class I histocompatibility antigen, B alpha chain 1001
Alpha-2C adrenergic receptor0202
Disintegrin and metalloproteinase domain-containing protein 171001
Inositol hexakisphosphate kinase 11001
cytochrome P450 2C9, partial1001
Chain A, Arginase 10101
Large neutral amino acids transporter small subunit 10101
PPM1D protein1001
Albumin0011
Proprotein convertase subtilisin/kexin type 70101
Vitamin K epoxide reductase complex subunit 1-like protein 10101
Vitamin K epoxide reductase complex subunit 10101
Vitamin K epoxide reductase complex subunit 1-like protein 10101
Vitamin K epoxide reductase complex subunit 1 0202
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]