What's arrhythmogenic right ventricular cardiomyopathy?
Arrhythmogenic right ventricular cardiomyopathy is an inherited heart-muscle disease in which heart-muscle cells are gradually lost and replaced by fibrous and fatty tissue, disrupting the heart's rhythm.
| Also indexed as | OMIM:609040, MONDO:0012180 |
|---|---|
| Features mapped | 6 |
| Treatments mapped | 1 |
| Published sources | 8 |
| Last reviewed | 2026-08-04 |
Signs and symptoms
Fibrofatty replacement of right ventricular myocardium
Arrhythmogenic right ventricular cardiomyopathy is an inherited heart muscle condition in which muscle in the right pumping chamber is gradually replaced by fibrous and fatty tissue. That change is linked to abnormal heart rhythms and a raised risk of sudden cardiac death.
Epsilon wave
An epsilon wave is a small extra deflection on an ECG reflecting delayed electrical signalling in heart muscle. In one group it appeared in about a quarter of people, in the leads over the right side of the heart, while inverted T waves in those same leads were more common still.
Right ventricular cardiomyopathy
Arrhythmogenic right ventricular cardiomyopathy is an inherited disease of the right pumping chamber of the heart. It was first described around forty years ago.
Sudden cardiac death
Arrhythmogenic cardiomyopathy carries a significant risk of sudden cardiac death caused by dangerous ventricular rhythms, especially in young people and endurance athletes.
Ventricular tachycardia
Ventricular tachycardia, a dangerously fast rhythm arising from the heart's lower chambers, can occur; it was seen, for example, in an endurance runner with a dilated, poorly contracting right ventricle.
Autosomal dominant inheritance
One form of this condition, linked to changes in the TMEM43 gene, is passed down in an autosomal dominant pattern, meaning a single altered copy of the gene is enough. Everyone who inherits it goes on to develop the condition, and it tends to be more severe and to begin earlier in men.
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
implantable cardioverter-defibrillator (ICD)
An implantable cardioverter-defibrillator (ICD) is a device that can prevent sudden cardiac death by detecting and stopping a dangerous rhythm; deciding who needs one rests on careful arrhythmic risk assessment.
Used to help with: Arrhythmogenic right ventricular dysplasia, familial, 9.
“Although its absolute incidence is low, the possibility of preventing SCD through implantable cardioverter-defibrillators renders accurate arrhythmic risk stratification a central clinical priority.”
How to read the evidence labels
Where this comes from
This guide is built from 8 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
Take it further
Printed, source-linked documents built from this condition's graph — ready to bring to an appointment or attach to a coverage request. Every claim carries its published source, the same as this guide.