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DeCS
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Descriptor English:
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Isolated Noncompaction of the Ventricular Myocardium
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Descriptor Spanish:
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No Compactación Aislada del Miocardio Ventricular
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Descriptor Portuguese:
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Miocárdio Ventricular não Compactado Isolado
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Synonyms English:
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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:
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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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Definition English:
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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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History Note English:
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2010
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Allowable Qualifiers English:
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Record Number:
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53536
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Unique Identifier:
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D056830
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Occurrence in VHL:
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Similar:
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DeCS
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