x

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.

Attention

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Orphanet doesn't provide personalised answers. To get in touch with the Orphanet team, please contact

Information provided in your contribution (including your email address) will be stocked in .CSV files that will be sent as an email to Orphanet's teams. These emails might be conserved in the teams' mailboxes, in our backoffice servers but will not be registered in our databases (for more information see our section General Data Protection Regulation and data privacy (GDPR) and Confidentiality).

Captcha image

Left ventricular noncompaction

Disease definition

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

Classification level: Disorder
  • 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
  • ICD-11: BC44
  • OMIM: 601493  601494  604169  609470  611878  613424  613426  615092  615373  615396
  • UMLS: C1960469
  • MeSH: -
  • GARD: 10985
  • MedDRA: -

  A summary on this disease is available in Deutsch (2004) Español (2014) Français (2014) Nederlands (2014)

Detailed information

General public

Guidelines

Genetic Testing

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.