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Left ventricular noncompaction
10 OMIM references -
12 associated genes
242 connected diseases
No signs/symptoms info
Disease Type of connection
Familial isolated dilated cardiomyopathy
Autosomal dominant Emery-Dreifuss muscular dystrophy
Familial isolated restrictive cardiomyopathy
Familial isolated arrhythmogenic ventricular dysplasia, biventricular form
Familial isolated arrhythmogenic ventricular dysplasia, left dominant form
Familial isolated arrhythmogenic ventricular dysplasia, right dominant form
1p36 deletion syndrome
Atrial septal defect, ostium secundum type
Atypical Werner syndrome
Autosomal codominant severe lipodystrophic laminopathy
Autosomal dominant limb-girdle muscular dystrophy type 1B
Autosomal recessive Emery-Dreifuss muscular dystrophy
Barth syndrome
Charcot-Marie-Tooth disease type 2B1
Classic multiminicore myopathy
Congenital muscular dystrophy due to LMNA mutation
Dilated cardiomyopathy - hypergonadotropic hypogonadism
Ebstein malformation
Familial dilated cardiomyopathy with conduction defect due to LMNA mutation
Familial partial lipodystrophy, Dunnigan type
Familial partial lipodystrophy, Köbberling type
Heart-hand syndrome, Slovenian type
Hutchinson-Gilford progeria syndrome
Hyaline body myopathy
LMNA-related cardiocutaneous progeria syndrome
Laing distal myopathy
Laminopathy type Decaudain-Vigouroux
Lethal restrictive dermopathy
Mandibuloacral dysplasia with type A lipodystrophy
Progeria-associated arthropathy
ZASP-related myofibrillar myopathy
Familial thoracic aortic aneurysm and aortic dissection
Precursor T-cell acute lymphoblastic leukemia
Young adult-onset Parkinsonism
Baraitser-Winter syndrome
Developmental malformations - deafness - dystonia
Amyotrophic lateral sclerosis
Familial idiopathic steroid-resistant nephrotic syndrome with focal segmental hyalinosis
Inflammatory myofibroblastic tumor
Burkitt lymphoma
Estrogen resistance syndrome
Childhood-onset nemaline myopathy
Congenital fiber-type disproportion myopathy
Hereditary breast and ovarian cancer syndrome
Becker muscular dystrophy
Duchenne muscular dystrophy
Inclusion body myopathy with Paget disease of bone and frontotemporal dementia
Symptomatic form of muscular dystrophy of Duchenne and Becker in female carriers
Cap myopathy
Typical nemaline myopathy
Autosomal recessive ataxia, Beauce type
Autosomal recessive myogenic arthrogryposis multiplex congenita
X-linked Emery-Dreifuss muscular dystrophy
Cystic fibrosis
Dedifferentiated liposarcoma
Well-differentiated liposarcoma
Wiskott-Aldrich syndrome
Aneurysm - osteoarthritis syndrome
Papillary or follicular thyroid carcinoma
46,XY partial gonadal dysgenesis
Autosomal recessive cerebellar ataxia - epilepsy - intellectual deficit
Behavioral variant of frontotemporal dementia
Extraskeletal Ewing sarcoma
Neuroblastoma
Progressive non-fluent aphasia
Semantic dementia
Intermediate nemaline myopathy
Early-onset autosomal dominant Alzheimer disease
Moyamoya disease
Romano-Ward syndrome
Acute promyelocytic leukemia
Craniolenticulosutural dysplasia
Amish nemaline myopathy
Digitotalar dysmorphism
Sheldon-Hall syndrome
Trismus - pseudocamptodactyly
Familial retinoblastoma
Monosomy 13q14
Unilateral retinoblastoma
Adult-onset autosomal dominant leukodystrophy
Split hand-split foot malformation
Familial pancreatic carcinoma
Familial gastric cancer
Gastric linitis plastica
Autosomal recessive centronuclear myopathy
Autosomal recessive limb-girdle muscular dystrophy type 2J
Early-onset generalized limb-onset dystonia
Early-onset myopathy with fatal cardiomyopathy
Familial prostate cancer
Helicoid peripapillary chorioretinal degeneration
Hereditary breast cancer
Hereditary proximal myopathy with early respiratory failure
Hereditary site-specific ovarian cancer syndrome
Herpetic encephalitis
Myoclonus-dystonia syndrome
Tibial muscular dystrophy
Chronic intestinal pseudoobstruction
Congenital short bowel syndrome
Congenital valvular dysplasia
Ehlers-Danlos syndrome with periventricular heterotopia
Frontometaphyseal dysplasia
Osteodysplasty, Melnick-Needles type
Otopalatodigital syndrome type 1
Otopalatodigital syndrome type 2
Periventricular nodular heterotopia
Terminal osseous dysplasia - pigmentary defects
Congenital analbuminemia
Hepatoencephalopathy due to combined oxidative phosphorylation deficiency type 1
Nuclear cataract
Primary peritoneal carcinoma
Hermansky-Pudlak syndrome type 7
Adult hepatocellular carcinoma
Berardinelli-Seip congenital lipodystrophy
Congenital non-bullous ichthyosiform erythroderma
Familial primary hypomagnesemia with normocalcuria and normocalcemia
Lamellar ichthyosis
Self-healing collodion baby
Autosomal dominant nonsyndromic sensorineural deafness type DFNA
Autosomal dominant distal renal tubular acidosis
Bloom syndrome
Distal renal tubular acidosis with anemia
Hereditary spherocytosis
Southeast Asian ovalocytosis
3C syndrome
ALK-positive anaplastic large cell lymphoma
ALK-positive large B-cell lymphoma
Adult-onset distal myopathy due to VCP mutation
Alagille syndrome due to 20p12 microdeletion
Alagille syndrome due to a JAG1 point mutation
Alobar holoprosencephaly
Autosomal agammaglobulinemia
Autosomal dominant nonsyndromic intellectual deficit
Autosomal dominant optic atrophy plus syndrome
Autosomal dominant optic atrophy, classic type
Autosomal dominant spastic paraplegia type 8
Autosomal recessive limb-girdle muscular dystrophy type 2T
Cerebellar ataxia - hypogonadism
Chronic granulomatous disease
Coffin-Siris syndrome
Combined immunodeficiency due to STK4 deficiency
Congenital bilateral absence of vas deferens
Congenital glaucoma
Congenital muscular dystrophy with cerebellar involvement
Congenital muscular dystrophy with intellectual disability
Craniopharyngioma
Desmoid tumor
Ewing sarcoma
Familial hemophagocytic lymphohistiocytosis
Familial rhabdoid tumor
Fibronectin glomerulopathy
Giant cell glioblastoma
Gliosarcoma
Glycogen storage disease due to muscle glycogen phosphorylase deficiency
Gray platelet syndrome
Hepatocellular carcinoma, childhood-onset
Hereditary chronic pancreatitis
Hereditary persistence of fetal hemoglobin - beta-thalassemia
Idiopathic bronchiectasis
Intellectual disability-feeding difficulties-developmental delay-microcephaly syndrome
Juvenile glaucoma
Lobar holoprosencephaly
Loeys-Dietz syndrome type 1
Male infertility with normal virilization due to meiosis defect
Megalencephalic leukoencephalopathy with subcortical cysts
Mendelian susceptibility to mycobacterial diseases due to complete ISG15 deficiency
Microform holoprosencephaly
Midline interhemispheric variant of holoprosencephaly
Multiple acyl-CoA dehydrogenation deficiency, mild type
Multiple acyl-CoA dehydrogenation deficiency, severe neonatal type
Multiple keratoacanthoma, Ferguson-Smith type
Muscle-eye-brain disease
Muscular dystrophy, Selcen type
Nestor-Guillermo progeria syndrome
Peripheral primitive neuroectodermal tumor
Pilomatrixoma
Proximal spinal muscular atrophy type 1
Proximal spinal muscular atrophy type 2
Proximal spinal muscular atrophy type 3
Proximal spinal muscular atrophy type 4
Richieri Costa-Pereira syndrome
Semilobar holoprosencephaly
Septopreoptic holoprosencephaly
Severe combined immunodeficiency due to DNA-PKcs deficiency
Shprintzen-Goldberg syndrome
Spastic paraplegia - Paget disease of bone
Squamous cell carcinoma of head and neck
Tetralogy of Fallot
Wolf-Hirschhorn syndrome
X-linked severe congenital neutropenia
X-linked thrombocytopenia with normal platelets
Fanconi anemia
Translocation renal cell carcinoma
Hereditary cerebral hemorrhage with amyloidosis, Arctic type
Hereditary cerebral hemorrhage with amyloidosis, Dutch type
Hereditary cerebral hemorrhage with amyloidosis, Flemish type
Hereditary cerebral hemorrhage with amyloidosis, Iowa type
Hereditary cerebral hemorrhage with amyloidosis, Italian type
Hereditary cerebral hemorrhage with amyloidosis, Piedmont type
Knobloch syndrome
Acute myeloid leukemia with t(8;21)(q22;q22) translocation
Chronic myeloid leukemia
Congenital myopathy with excess of thin filaments
Familial platelet syndrome with predisposition to acute myelogenous leukemia
Isolated delta-storage pool disease
Precursor B-cell acute lymphoblastic leukemia
Severe congenital nemaline myopathy
17p13.3 microduplication syndrome
Autosomal dominant Charcot-Marie-Tooth disease type 2F
Distal 17p13.3 microdeletion syndrome
Distal hereditary motor neuropathy type 2
Miller-Dieker syndrome
12q14 microdeletion syndrome
Acute necrotizing encephalopathy of childhood
Ataxia-telangiectasia-like disorder
Autosomal thrombocytopenia with normal platelets
Buschke-Ollendorff syndrome
Constitutional mismatch repair deficiency syndrome
Distal 22q11.2 microdeletion syndrome
Familial acute necrotizing encephalopathy
Familial atrial fibrillation
Familial infantile bilateral striatal necrosis
Frontotemporal dementia with motor neuron disease
Greenberg dysplasia
Hereditary nonpolyposis colon cancer
Isolated osteopoikilosis
Juvenile amyotrophic lateral sclerosis
Melorheostosis with osteopoikilosis
Methylmalonic acidemia with homocystinuria, type cblX
Myxofibrosarcoma
Myxoid / round cell liposarcoma
Non-polyposis Turcot syndrome
Partial acquired lipodystrophy
Reducing body myopathy
Renal tubular dysgenesis of genetic origin
Reynolds syndrome
Sjögren-Larsson syndrome
TARP syndrome
Williams syndrome
Wolfram syndrome
X-linked myopathy with postural muscle atrophy
X-linked non-syndromic intellectual deficit
Inherited cancer-predisposing syndrome due to biallelic BRCA2 mutations
Synonym(s):
- LVNC
- Left ventricular hypertrabeculation
- Spongy myocardium

Classification (Orphanet):
- Rare cardiac disease
- Rare genetic disease

Classification (ICD10):
- Diseases of the circulatory system -

Epidemiological data:
Class of prevalence: unknown
Average age onset: variable
Average age of death: -
Type of inheritance: autosomal dominant
External references:
10 OMIM references -
No MeSH references

No signs/symptoms info available.