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Descriptor English: Isolated Noncompaction of the Ventricular Myocardium
Descriptor Spanish: No Compactación Aislada del Miocardio Ventricular
Descriptor Portuguese: Miocárdio Ventricular não Compactado Isolado
Descriptor French: Non-compaction isolée du ventricule
Entry term(s): Isolated Non-compaction of the Ventricular Myocardium
Isolated Noncompaction of the Left Ventricular Myocardium, X-Linked
Noncompaction of the Left Ventricular Myocardium, Autosomal Dominant
Tree number(s): C14.240.400.660
C14.280.238.281.500
C14.280.400.660
C16.131.077.477
C16.131.240.400.655
C16.320.322.370
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D056830
Scope note: 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.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Public MeSH Note: 2010
History Note: 2010
DeCS ID: 53536
Unique ID: D056830
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2010/01/01
Date of Entry: 2009/07/06
Isolated Noncompaction of the Ventricular Myocardium - Preferred
Concept UI M0529360
Scope note 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.
Preferred term Isolated Noncompaction of the Ventricular Myocardium
Entry term(s) Isolated Non-compaction of the Ventricular Myocardium
Noncompaction of the Left Ventricular Myocardium, Autosomal Dominant - Narrower
Concept UI M0529361
Scope note Mutations in the alpha-dystrobrevin gene are associated with Autosomal Dominant type of Noncompaction of the Left Ventricular Myocardium.
Preferred term Noncompaction of the Left Ventricular Myocardium, Autosomal Dominant
Isolated Noncompaction of the Left Ventricular Myocardium, X-Linked - Narrower
Concept UI M0529362
Scope note Mutations in the tafazzin gene are associated with X-Linked Isolated Noncompaction of the Left Ventricular Myocardium. BARTH SYNDROME is an allelic disorder.
Preferred term Isolated Noncompaction of the Left Ventricular Myocardium, X-Linked



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