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Left ventricular noncompaction

ORPHA54260
Synonym(s) LVNC
Left ventricular hypertrabeculation
Spongy myocardium
Prevalence >1 / 1000
Inheritance Mitochondrial inheritance
or Autosomal dominant
or X-linked recessive
Age of onset All ages
ICD-10
  • I42.8
OMIM
UMLS
  • C1960469
MeSH -
MedDRA -

Summary

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized anatomically by prominent left ventricular trabeculae and deep intratrabecular recesses causing progressive systolic and diastolic dysfunction, conduction abnormalities, and occasionally thromboembolic events.


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