arrhythmogenic right ventricular dysplasia 10
Findings
No curated finding names arrhythmogenic right ventricular dysplasia 10 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Any arrhythmogenic right ventricular cardiomyopathy in which the cause of the disease is a mutation in the DSG2 gene.
Definition from the Mondo Disease Ontology (MONDO:0012434), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Late onset · Middle age onset · Juvenile onset · Young adult onset
HPO, annotations 2026-09-02
Features
13 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.
- Ventricular arrhythmiaHPOHP:0004308
- 8 of 8 reported patients
- Premature ventricular contractionHPOHP:0006682
- 5 of 8 reported patients
- Epsilon waveHPOHP:0034304
- 3 of 8 reported patients
- PalpitationsHPOHP:0001962
- 3 of 8 reported patients
- Prolonged PR intervalHPOHP:0012248
- 3 of 8 reported patients
- Ventricular tachycardiaHPOHP:0004756
- 3 of 8 reported patients
- Chest painHPOHP:0100749
Show the remaining 1
- Right ventricular cardiomyopathyHPOHP:0011663
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.
- DSG2HGNC:3049
- Definitive · Ambry Genetics · Autosomal dominant · 2018
- Definitive · G2P · Autosomal dominant · 2024
- Definitive · G2P · Autosomal recessive · 2024
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2021
- Moderate · Ambry Genetics · Autosomal recessive · 2023
Where it sits
Other names
7 names
Resolves to: arrhythmogenic right ventricular dysplasia 10
- Also called
- arrhythmogenic right ventricular cardiomyopathy 10arrhythmogenic right ventricular cardiomyopathy caused by mutation in DSG2arrhythmogenic right ventricular dysplasia type 10arrhythmogenic right ventricular dysplasia, familial, type 10ARVC10ARVD10DSG2 arrhythmogenic right ventricular cardiomyopathy