Arrhythmogenic right ventricular dysplasia surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Three methods which employ surgical treatment can be used in patients with arrhythmogenic right ventricular dysplasia. Catheter ablation may be used to treat intractable ventricular tachycardia. An implantable cardiac difibrillator is the best preventative method against sudden cardiac death. Cardiac transplant surgery is rarely considered in severe or intractable conditions.

Surgery

Catheter ablation

Catheter ablation may be used to treat intractable ventricular tachycardia. It has a 60-90% success rate.[1] Unfortunately, due to the progressive nature of the disease, recurrence is common (60% recurrence rate), with the creation of new arrhythmogenic foci. Indications for catheter ablation include drug-refractory VT and frequent recurrence of VT after ICD placement, causing frequent discharges of the ICD.

Implantable Cardioverter-defibrillator

An ICD is the most effective prevention against sudden cardiac death. Due to the prohibitive cost of ICDs, they are not routinely placed in all individuals with ARVD.

Indications for ICD placement in the setting of ARVD include:

  • Cardiac arrest due to VT or VF
  • Symptomatic VT that is not inducible during programmed stimulation
  • Failed programmed stimulation-guided drug therapy
  • Severe RV involvement with poor tolerance of VT
  • Sudden death of immediate family member

Since ICDs are typically placed via a transvenous approach into the right ventricle, there are complications associated with ICD placement and follow-up.

Due to the extreme thinning of the RV free wall, it is possible to perforation the RV during implantation, potentially causing pericardial tamponade. Because of this, every attempt is made at placing the defibrillator lead on the ventricular septum.

After a successful implantation, the progressive nature of the disease may lead to fibro-fatty replacement of the myocardium at the site of lead placement. This may lead to under-sensing of the individual's electrical activity (potentially causing inability to sense VT or VF), and inability to pace the ventricle.

Cardiac Transplant Surgery

Cardiac transplant surgery is rarely performed in ARVD. It may be indicated if the arrhythmias associated with the disease are uncontrollable or if there is severe bi-ventricular heart failure that is not manageable with pharmacological therapy.

ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death - Arrhythmogenic Right Ventricular Cardiomyopathy (DO NOT EDIT) [2]

Class I
"1. ICD implantation is recommended for the prevention of SCD in patients with ARVC with documented sustained VT or VF who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: B)"
Class IIa
"1. ICD implantation can be effective for the prevention of SCD in patients with ARVC with extensive disease, including those with LV involvement, 1 or more affected family member with SCD, or undiagnosed syncope when VT or VF has not been excluded as the cause of syncope, who are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)"
"2. Amiodarone or sotalol can be effective for treatment of sustained VT or VF in patients with ARVC when ICD implantation is not feasible. (Level of Evidence: C)"
"3. Ablation can be useful as adjunctive therapy in management of patients with ARVC with recurrent VT, despite optimal antiarrhythmic drug therapy. (Level of Evidence: C)"
Class IIb
"1. EP testing might be useful for risk assessment of SCD in patients with ARVC. (Level of Evidence: C)"

References

  1. Fontaine G, Tonet J, Gallais Y, Lascault G, Hidden-Lucet F, Aouate P, Halimi F, Poulain F, Johnson N, Charfeddine H, Frank R. (2000). "Ventricular tachycardia catheter ablation in arrhythmogenic right ventricular dysplasia: a 16-year experience". Curr Cardiol Rep. 2 (6): 498–506. PMID 11203287.
  2. Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M; et al. (2006). "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): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society". Circulation. 114 (10): e385–484. doi:10.1161/CIRCULATIONAHA.106.178233. PMID 16935995.

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