left ventricular noncompaction 1
Findings
No curated finding names left ventricular noncompaction 1 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Any left ventricular noncompaction in which the cause of the disease is a mutation in the DTNA gene.
Definition from the Mondo Disease Ontology (MONDO:0011403), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Congenital onset · Adult onset · Young adult onset
HPO, annotations 2026-09-02
Features
11 features
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, annotations 2026-09-02.
- Chest tightnessHPOHP:0031352
- 1 of 1 reported patient
- First degree atrioventricular blockHPOHP:0011705
- 1 of 1 reported patient
- Left ventricular noncompactionHPOHP:0030682
- 7 of 7 reported patients
- Noncompaction cardiomyopathyHPOHP:0012817
- 1 of 1 reported patient
- PalpitationsHPOHP:0001962
- 1 of 1 reported patient
- Ventricular septal defectHPOHP:0001629
- 5 of 6 reported patients
- Hypoplastic left ventricleHPO
Genes
1 gene
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.
- DTNAHGNC:3057
- Limited · Ambry Genetics · Autosomal dominant · 2018
- Limited · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2020
Where it sits
- A kind of
Other names
4 names
Resolves to: left ventricular noncompaction 1
- Also called
- DTNA left ventricular noncompactionleft ventricular noncompaction 1, with or without congenital heart defectsleft ventricular noncompaction caused by mutation in DTNAleft ventricular noncompaction type 1