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Left ventricular noncompaction

Disease definition

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.

ORPHA:54260

  • Synonym(s):
    • LVNC
    • Left ventricular hypertrabeculation
    • Spongy myocardium
  • Prevalence: -
  • Inheritance: Autosomal dominant or X-linked recessive or Mitochondrial inheritance or Autosomal recessive 
  • Age of onset: All ages
  • ICD-10: I42.8
  • OMIM: 601493  601494  604169  609470  611878  613424  613426  615092  615373  615396
  • UMLS: C1960469
  • MeSH: -
  • GARD: 10985
  • MedDRA: -

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