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Condition report · Cardiovascular

Left Ventricular Noncompaction

Inherited conditions in which the inner wall of the left ventricle stays spongy, with deep folds, instead of becoming compact during development. It can be found without symptoms or can lead to heart failure, rhythm problems or blood clots, and often occurs with other cardiomyopathies.

A condition report looks for rare variants known to cause the conditions it covers.

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What your report covers

The 2 conditions we analyse

Left Ventricular Noncompaction

A heart muscle condition with a thick, spongy inner layer of the left ventricle, linked to sarcomere genes such as MYH7, ACTC1, TNNT2, TPM1 and MYBPC3, and to LDB3, PRDM16 and MIB1.

What a diagnosis changes

Knowing about it supports heart imaging and rhythm monitoring and screening of relatives.

Genes analysed

Also known as

left ventricular hypertrabeculation; left ventricular non-compaction cardiomyopathy; left ventricular non-compaction syndrome; left ventricular noncompaction (disease); Lv non-compaction syndrome; LVNC; spongy myocardium

Includes 13 related subtypes ↓
  • dilated cardiomyopathy 1C
  • dilated cardiomyopathy 1D
  • dilated cardiomyopathy 1R
  • dilated cardiomyopathy 1S
  • dilated cardiomyopathy 1Y
  • left ventricular noncompaction 1
  • left ventricular noncompaction 10
  • left ventricular noncompaction 2
  • left ventricular noncompaction 4
  • left ventricular noncompaction 5
  • left ventricular noncompaction 7
  • left ventricular noncompaction 8
  • left ventricular noncompaction 9
MONDO:0018901Orphanet 54260MedGen 450531

MIPEP-Related Left Ventricular Non-Compaction Syndrome

Autosomal recessive

A severe condition caused by changes in MIPEP that combines left ventricular non-compaction or other cardiomyopathy with seizures, low muscle tone, cataracts and developmental delay.

Genes analysed

Also known as

combined oxidative phosphorylation deficiency 31; combined oxidative phosphorylation deficiency caused by mutation in MIPEP; combined oxidative phosphorylation deficiency type 31; COXPD31; MIPEP combined oxidative phosphorylation deficiency

MONDO:0014976OMIM 617228Orphanet 478049MedGen 934628

Other cardiovascular conditions

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