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Arrhythmogenic Right Ventricular Cardiomyopathy

Formerly called arrhythmogenic right ventricular dysplasia, arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterised by progressive fibro-fatty replacement of right ventricular myocardium with progressive effects on the right ventricle, a strong familial transmission, and presentation with symptomatic arrhythmias or sudden death.1 The precise aetiology remains unknown.

There are separate articles which discuss Cardiomyopathies, Hypertrophic Cardiomyopathies, Dilated Cardiomyopathy and Restrictive Cardiomyopathy.

Epidemiology
  • Most often presents in adolescence and early adulthood.
  • More common in males.
  • Presents as familial disease in at least 30% of patients: autosomal dominant inheritance is typical.2
Clinical presentation

Dependent on which phase of disease progression:

  • Concealed phase:
    • Subtle right ventricular changes, with or without minor ventricular arrhythmias.
    • These may occasionally be the first manifestation as sudden death, usually in the young engaged in competitive sports or intense exercise.
  • Overt electrical disorder:
    • Symptomatic right ventricular arrhythmias associated with functional and structural abnormalities of the right ventricle.
    • Usually presents with palpitations or syncope.
    • Arrhythmias and sudden death are common. Previously undiagnosed ARVC accounts for about 20% of cardiac sudden deaths.
  • Right ventricular failure:
    • Extension of disease to the whole of the right ventricle causes dysfunction.
  • Biventricular pump failure - end stage:
    • Left ventricular involvement leads to heart failure and may mimic dilated cardiomyopathy.
Investigations

A Task Force set up by the European and International Society of Cardiology has proposed diagnostic criteria in some detail.3

Management
  • For patients with well tolerated or non life-threatening ventricular arrhythmias, beta-blockers, such as sotalol, or amiodarone with/without beta-blocker, are the most effective drugs with a relatively low proarrhythmic risk.
  • For sustained VT or VF, serial therapeutic drug trials are used, using programmed ventricular stimulation to assess effectiveness.
  • Patients who remain inducible of arrhythmias usually require an implantable cardioverter defibrillator, except for rare cases with localised disease where catheter ablation may be an option.
  • Ablation can have up to a 90% success rate in the short term, but a 60% recurrence rate which may lead to sudden death.
  • In patients who are not inducible, and present in cardiac arrest or syncope, an automatic implantable cardioverter-defibrillator is the first option, and is the most effective intervention in prevention of arrhythmic sudden death.
  • Treatment of heart failure.
  • Heart transplantation may need to be considered in refractory cases in end stage failure.
Prognosis
  • Arrhythmogenic right ventricular dysplasia tends to be progressive with deterioration of right ventricular function.
  • The left ventricle may become involved with progression of the degenerative process.
  • The death rate is approximate 2.5% per year.4


Document references
  1. Franz WM, Muller OJ, Katus HA; Cardiomyopathies: from genetics to the prospect of treatment. Lancet. 2001 Nov 10;358(9293):1627-37. [abstract]
  2. Sen-Chowdhry S, Lowe MD, Sporton SC, et al; Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management. Am J Med. 2004 Nov 1;117(9):685-95. [abstract]
  3. Corrado D, Basso C, Thiene G; Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment. Heart. 2000 May;83(5):588-95.
  4. Pennell DJ et al; Arrhythmogenic right ventricular cardiomyopathy. European Society of Cardiology; E-Journal - Volume 1.

Internet and further reading Acknowledgements EMIS is grateful to Dr Colin Tidy for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2008.
DocID: 1820
Document Version: 20
DocRef: bgp25252
Last Updated: 21 Feb 2008
Review Date: 20 Feb 2010


















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See also MAKEPOVERTYHISTORY North East for details and links to campaigns against poverty.

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