Right ventricular disarticulation for arrythmogenic right ventricular dysplasia: an 18 year single centre experience. J Zacharias, J Forty, C Doig*, J Bourke*, CJ Hilton. Departments of academic cardiology and cardiac surgery, Freeman Hospital, Newcastle upon Tyne. England.
ARVD A Type of cardiomyopathy Affects all age groups Presentation with syncope / sudden death Ventricular Tachycardia / Fibrillation Familial occurrence Structural changes in R V Myocardium
Histology
Treatment options with ARVD patients: Antiarrhythmic medication Percutaneous catheter ablation Automatic internal cardiac defibrillators. (AICD) Surgery
Right Ventricular Disarticulation Described initially by Guiraudon Animal studies done by Guiraudon & Cox et al Medium term results from Cox and Hilton et al Few surgical reports lately
Retrospective Case series: Institution: Tertiary referral centre : Freeman hospital. Time Frame: July 1985 - July 2003. Patient Numbers: 17, age range (14 - 72), 15 M / 2 F Surgeons: CJ Hilton (14) & J Forty (3)
Pre Op Details: Presentation: Management; Syncope: 12 Intractable VT : 3 patients Electrical storms (AICD): 2 Management; Drugs: 17 (Range 3 - 8) EPS: 17 AICD: 2 Echo: 14 Angio; 14 CT Scan: 2
Preop Echo Of ARVD patient
Operative details: Complete: 10 Partial: 7 Full Cardiopulmonary Bypass Cold Blood Cardioplegia Induce VT in the disarticulated segment
Gross Appearance Of ARVD Heart
Completed RV Disarticulation
Post operative events: immediate I Mortality : 1 (6%) multi-organ failure Overall complication rate: 64% (11/17) Reopening for bleeding: 4 Renal failure: 3 Tracheostomy: 2 Derangement of liver function: 1 Pleural effusions: 3
Post operative events: immediate II Mean post -operative hospital stay : 17.6 days (range 9 - 35 days) Electrophysiological studies : 16 Medication at discharge: Anticoagulation: 4 Antiarrhythmic drugs: 2 Digoxin & Diuretics: 6 Biventricular pacing : 2
Post operative events: medium term Further VT episodes : 4 Partial 3/7 Complete 1/10 Supraventricular Tachycardia: 10
Echo findings14 yrs post RV Disarticulation.
Post operative Events: Long Term Follow up : 94% (15/16) 1 lost to follow up alive & well at 5 years. Median 13 years (range 0 - 18) Death: 3 ( 9,11, 17 years post op) Transplantation: 2 ( 3, 8 yrs post op) Awaiting transplantation: 2 ( 3, 14 yrs post op)
Kaplan-Meier actuarial survival 100 1 6 1 5 1 4 1 3 80 1 2 1 1 Survival % 60 40 20 5 10 1 5 2 S u r v i v a l i n y e a r s
Discussion points: Natural history of ARVD Prevention of sudden death Affects young patients Biventricular failure affects some Heart transplantation Prevention of sudden death Can occur despite AICD International registry details awaited Long term effects of biventricular pacing
Effects of Right ventricular pacing
Conclusions: Excellent antiarrhythmic procedure 77% event free survival at 10 years Heart failure may be related to natural history of ARVD Consider as an option in young patients with ARVD who cannot be managed with medication or AICD