| Descriptor English: | Isolated Noncompaction of the Ventricular Myocardium | ||||||
| Descriptor Spanish: |
No Compactación Aislada del Miocardio Ventricular
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| 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 |
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| 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 |
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| 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. |
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| 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 |
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| Public MeSH Note: | 2010 |
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| History Note: | 2010 |
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| 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 |
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Isolated Noncompaction of the Ventricular Myocardium
- Preferred
Noncompaction of the Left Ventricular Myocardium, Autosomal Dominant
- Narrower
Isolated Noncompaction of the Left Ventricular Myocardium, X-Linked
- Narrower
| 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 |
| 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 |
| 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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