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Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Congenital Abnormalities > Abnormalities, Multiple > Isolated Noncompaction of the Ventricular Myocardium
Cardiovascular Diseases > Heart Diseases > Heart Defects, Congenital > Isolated Noncompaction of the Ventricular Myocardium
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Congenital Abnormalities > Cardiovascular Abnormalities > Heart Defects, Congenital > Isolated Noncompaction of the Ventricular Myocardium
Cardiovascular Diseases > Cardiovascular Abnormalities > Heart Defects, Congenital > Isolated Noncompaction of the Ventricular Myocardium
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Genetic Diseases, X-Linked > Isolated Noncompaction of the Ventricular Myocardium
Cardiovascular Diseases > Heart Diseases > Cardiomyopathies > Endocardial Fibroelastosis > Isolated Noncompaction of the Ventricular Myocardium

Preferred term

Isolated Noncompaction of the Ventricular Myocardium  

Type

  • mesh:Descriptor

Definition

  • 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. Mutations in the alpha-dystrobrevin gene are associated with Autosomal Dominant type of Noncompaction of the Left Ventricular Myocardium. Mutations in the tafazzin gene are associated with X-Linked Isolated Noncompaction of the Left Ventricular Myocardium. BARTH SYNDROME is an allelic disorder.

Broader concept

Related concepts

Entry terms

  • Isolated Non-compaction of the Ventricular Myocardium

Allowable Qualifier(s)

In other languages

  • Non-compaction isolée du ventricule

    French

  • NCIVG
  • NCVI
  • Non-compaction isolée du ventricule du myocarde
  • Non-compaction isolée du ventricule gauche
  • Non-compaction isolée du ventricule myocardique
  • Non-compaction ventriculaire isolée

URI

http://data.loterre.fr/ark:/67375/JVR-NXTG79SM-J

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