Concept information
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Congenital Abnormalities
Cardiovascular Abnormalities
Heart Defects, Congenital
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Genetic Diseases, Inborn
Genetic Diseases, X-Linked
Preferred term
Isolated Noncompaction of the Ventricular Myocardium
Type
-
Topical Descriptor
Broader concept
Entry terms
- Isolated Non-compaction of the Ventricular Myocardium
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.
History note
- 2010
In other languages
-
Finnish
-
sydänlihaksen tiivistymättömyys
URI
http://www.yso.fi/onto/mesh/D056830
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