Cardiovascular

Arrhythmogenic Right Ventricular Cardiomyopathy

Also known as ARVC, ARVD, arrhythmogenic cardiomyopathy, desmoplakin/PKP2

Arrhythmogenic Right Ventricular Cardiomyopathy is an inherited heart muscle disease caused primarily by mutations in desmosomal genes, leading to progressive fibro-fatty replacement of right ventricular myocardium, ventricular arrhythmias,

ORPHA:247 ↗Gene PKP2Gene DSPGene DSG2Gene DSC2Prevalence 1 per 2,000–5,000Onset Adolescence to young adulthood; rarely in childhoodAutosomal dominant (most cases)

20

studies recruiting now

as of 7 Sept 2026

54

studies registered in total

as of 7 Sept 2026

3

countries with a recruiting site

as of 7 Sept 2026

24 Sept 2024

most recent study posted

among recruiting studies

Recruiting trials

Showing the 5 most recently updated recruiting studies, as recorded 7 Sept 2026. Live status on each study page.

See all 20 recruiting studies

Where recruiting studies are running

Countries with at least one recruiting site among the studies above, 7 Sept 2026. Tap a country to search trials there.

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Patient organisations

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Registry: North American ARVC Registry · Join ↗. Registries connect patients to researchers and often hear about trials first.

About Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic Right Ventricular Cardiomyopathy is an inherited heart muscle disease caused primarily by mutations in desmosomal genes, leading to progressive fibro-fatty replacement of right ventricular myocardium, ventricular arrhythmias, and an elevated risk of sudden cardiac death, particularly in young individuals and athletes. PKP2, encoding plakophilin-2, is the most frequently mutated gene, accounting for approximately 70–80% of mutation-positive cases in North American and European cohorts. The diagnosis is made using the 2010 Revised Task Force Criteria, which integrate imaging, electrocardiographic, histological, and genetic findings.

Common clinical features

Palpitations and symptomatic ventricular ectopySyncope or pre-syncope due to ventricular tachycardiaSudden cardiac arrest (may be the first presentation)Right ventricular dilation and wall motion abnormalities on MRI or echoLeft bundle branch block morphology ventricular tachycardiaEpsilon waves on ECGFibro-fatty myocardial replacement on cardiac MRI (late gadolinium enhancement)Exercise-triggered arrhythmia

From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.

Treatments being studied

2 in clinical development, from Open Targets (CC BY 4.0). Not medical advice.

Phase 2Flecainide (Flecainide component of thn102)
Phase 2Spironolactone (Abbolactone)

Before you apply

Things trial teams commonly ask about for Arrhythmogenic Right Ventricular Cardiomyopathy. Not eligibility rules; those are set by each study.

  • Fulfilling the 2010 Task Force Criteria (definite, borderline, or possible ARVC) is typically required for enrolment; ensure your diagnosis is formally documented with criterion scoring.
  • Competitive sport participation history and ongoing activity level are important for risk stratification and trial eligibility; a detailed exercise history should be prepared.
  • Implantable cardioverter-defibrillator (ICD) implantation status affects eligibility for some electrophysiology-focused trials; disclose device type and programming details.

Related conditions

Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).