Arrhythmogenic Right Ventricular Cardiomyopathy

Author(s)	J.S.S.G. de Jong, MD
Moderator	J.S.S.G. de Jong, MD
Supervisor
some notes about authorship

What is ARVC?

Arrythmogenic Right Ventricular Cardiomyopathy, (ARVC, or ARVD: Arrythmogenic Right Ventricular Disease) is characterized by fatty replacement and fibrosis of the heart. Especially the right ventricle apex and outflow tract are involved. However the left ventricle can be affected to.

A section throught the heart of a ARVC patient. (A) Transmural fatty replacement of the right ventricular free wall. (B) Myocardial atrophy is confined to the right ventricle and substantially spares the interventricular septum as well as the left ventricular free wall. [1] Reproduced with permission from BMJ Publishing Group Ltd.

As a result of the fatty replacement and fibrosis, ventricular arrhythmias are common in this disease and can lead to palpitations, syncope and sudden death. At older age right ventricular pump failure can occur.

The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.[2]

ARVC is a progressive disease. The incidence is estimated to be 1:3.000-1:10.000. Manifestations is usually during teenage. Although the diagnosis is more often made in athletes, sports are not thought to have a causative relationship with the disease. ARVD can occur in families; more than 9 different chromosomal defects have been described, most often with autosomal dominant inheritance.

One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.

Diagnosis

Een epsilon golf in V1

ARVC is a difficult diagnosis to make. ECG characteristics that can occur in ARVC are:

• Epsilon waves
• QRS prolongation (>110ms) in the precordial leads (V1–V3)
• Late potentials on a signal averaged ECG

The European Society of Cardiology has created a list of diagnositc criteria for the diagnosis of ARVC.

Major diagnostic criteria for Aritmogenic Right Ventricular Cardiomyopathy[2]
Familial disease confirmed at necroscopy or surgery Severe dilatation and reduciton of right ventricular ejection fraction with no (or only mild) LV impairment Localised irhgt ventricular aneurysms (akinetic or diskinetic areas with diastolic bulging) Severe segmental dilataion of the right ventricle Fibrofatty replacement of myocardium on endomyocardial biopsy
Diagnostic criteria that can be diagnosed on the ECG
(major) Epsilon wave or localised prolongation (>110ms) of the QRS complex in right precordial leads (V1-V3) (minor) Inverted T waves in right precordial leads (V2 and V3) (people aged more than 12 yr; in absence of RBBB (minor) Late potentials (signal averaged ECG) (minor) Left bundle branch block type ventricular tachycardia (sutained and non-sustained) (ECG, Holter, exercise testing (minor) Frequent ventricular extrasystoles (more than 1000/24h) (Holter)

Treatment

Treatment focusses on avoiding complications.[3]

• Medication:
  • anti-arrithmics: Amiodarone, Sotalol
  • ACE-inhibitors to prevent cardiac remodelling
• Preventive ICD placements in patients in whom optimal medical treatment did not prevent ventricular tachycardia or ventricular fibrillation. Other patients who qualify for ICD placement: patients with severe disease with left ventricular involvement, patients with more family members who died of ARVD, patients who had a syncope while on medication and in whom arrhythmias cannot be excluded as a cause.
• Radiofrequency ablation in patients with recurrent ventricular tachycardia.

Referenties

1. Corrado D, Basso C, and Thiene G. Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment. Heart. 2000 May;83(5):588-95. DOI:10.1136/heart.83.5.588 |
2. McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist C, Fontaine G, and Camerini F. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology. Br Heart J. 1994 Mar;71(3):215-8. DOI:10.1136/hrt.71.3.215 |
3. European Heart Rhythm Association, Heart Rhythm Society, Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC Jr, Jacobs AK, Adams CD, Antman EM, Anderson JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B, Priori SG, Blanc JJ, Budaj A, Camm AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL, American College of Cardiology, American Heart Association Task Force, and European Society of Cardiology Committee for Practice Guidelines. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). J Am Coll Cardiol. 2006 Sep 5;48(5):e247-346. DOI:10.1016/j.jacc.2006.07.010 |