left ventricular noncompaction
Findings
No curated finding names left ventricular noncompaction yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
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.
Definition from the Mondo Disease Ontology (MONDO:0018901), read 2026-09-29. CC BY 4.0.
Features
1 feature
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, hp/releases/2026-09-01.
- Left ventricular noncompactionMondoHP:0030682
Genes
9 genes
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- MIB1HGNC:21086
- Supportive · Orphanet · Autosomal dominant · 2021
- MYH7HGNC:7577
- Supportive · Orphanet · Autosomal dominant · 2021
- MYH7BHGNC:15906
- Supportive · Orphanet · Autosomal dominant · 2021
- PKP2HGNC:9024
- Supportive · Orphanet · Autosomal dominant · 2021
- PRDM16HGNC:14000
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
- Narrower terms (13)
- 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
Other names
7 names
Resolves to: left ventricular noncompaction
- Also called
- left ventricular hypertrabeculationleft ventricular non-compaction cardiomyopathyleft ventricular non-compaction syndromeleft ventricular noncompaction (disease)Lv non-compaction syndromeLVNCspongy myocardium