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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Arrhythmogenic right ventricular cardiomyopathy

Cardiology · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

RapidFull
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Escalate

Sustained VT, resuscitated arrest, arrhythmic syncope or acute myocardial-injury 'hot phase' with instability requires monitored emergency care and inherited-cardiac/electrophysiology input.

Synopsis

Recognise arrhythmogenic cardiomyopathy, integrate electrical, imaging and genetic evidence, and reduce exercise-related and sudden-death risk.

  • ARVC is a genetic arrhythmogenic cardiomyopathy with fibrofatty myocardial replacement; biventricular and left-dominant disease occur.
  • Diagnosis is multiparametric: no single ECG, echo, CMR or genetic finding is sufficient in isolation.
  • Clues include T-wave inversion V1-V3 beyond the expected age, terminal activation abnormalities and VT with left-bundle morphology.
  • CMR must assess regional motion and quantitative RV/LV size and function; isolated fat is non-specific.
  • Exercise can accelerate penetrance and arrhythmia: competitive and high-intensity exercise is generally discouraged in affected people and pathogenic-variant carriers.
  • A negative family history does not exclude disease; penetrance and expression vary markedly.
  • Risk assessment integrates prior arrest/VT, syncope, ventricular function, arrhythmia burden, genotype and extent of disease.
  • Inflammatory 'hot phases', especially with desmosomal variants, can mimic myocarditis and should prompt inherited-disease reassessment.

Key red flags

Hot phase

Chest pain and troponin rise with non-ischaemic scar, recurrent 'myocarditis' or a DSP family history should raise arrhythmogenic cardiomyopathy.

High-risk symptoms

Exercise-related syncope, sustained VT or aborted sudden death needs urgent risk assessment and exercise cessation.

Investigation priorities

01
12-lead and signal-averaged ECGFirst step

Identify repolarisation/depolarisation criteria and conduction alternatives.

Management branches

DiagnosisSuspected ARVC

Arrhythmic symptoms, suggestive ECG/CMR or family diagnosis.

  1. Stop competitive/high-intensity exercise pending assessment; obtain a three-generation pedigree and details of sudden deaths or recurrent myocarditis.
  2. Perform ECG, ambulatory monitoring, expert TTE and CMR with quantitative biventricular assessment.
  3. Exclude mimics including athlete remodelling, RV volume/pressure loading, cardiac sarcoidosis, myocarditis and congenital shunts.
  4. Apply accepted multiparametric criteria in an inherited-cardiac service; add genomic counselling rather than treating a genetic result as a standalone diagnosis.
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Sources and review status4 sources · checked 27 Aug 2026 · clinical review pending+
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Apply principles in context and verify current guidance when a decision affects care. Source check completed 27 Aug 2026; clinical approval remains outstanding.

  • 2023 ESC Guidelines for the management of cardiomyopathiesCurrent diagnosis, exercise, risk and family framework.
  • NHS Genomics Education: Arrhythmogenic right ventricular cardiomyopathyUK genomic and family context.
  • ESC imaging toolbox: Arrhythmogenic cardiomyopathySpecialist imaging and risk resources.
  • NICE TA314: Implantable cardioverter defibrillators and CRTUK device framework.

Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom