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Left ventricular noncompaction

Orpha number ORPHA54260
Synonym(s) LVNC
Left ventricular hypertrabeculation
Spongy myocardium
Prevalence Unknown
Inheritance
  • Autosomal dominant
  • X-linked recessive
  • Mitochondrial inheritance
Age of onset Variable
ICD-10
  • I42.8
OMIM
UMLS
  • C1960469
MeSH -
MedDRA -
SNOMED CT
  • 427608000

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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