DPDoctor's PassportEducation
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.
RapidARVCarrhythmogenic cardiomyopathyventricular tachycardiageneticsexerciseICD

Arrhythmogenic right ventricular cardiomyopathy

Essential points for quick revision.

!
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.

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.

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.
Open full textbook Answer 2 questionsCardiology check
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. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom