1. 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.

2. 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

3. Histology

4. Treatment options with ARVD patients:
Antiarrhythmic medication
Percutaneous catheter ablation
Automatic internal cardiac defibrillators. (AICD)
Surgery

5. 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

6. Retrospective Case series:
Institution: Tertiary referral centre : Freeman hospital.
Time Frame: July July 2003.
Patient Numbers: 17, age range ( ), 15 M / 2 F
Surgeons: CJ Hilton (14) & J Forty (3)

7. 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

8. Preop Echo Of ARVD patient

9. Operative details: Complete: 10 Partial: 7 Full Cardiopulmonary Bypass
Cold Blood Cardioplegia
Induce VT in the disarticulated segment

10. Gross Appearance Of ARVD Heart

15. Completed RV Disarticulation

16. Post operative events: immediate I
Mortality : 1 (6%) multi-organ failure
Overall complication rate: % (11/17)
Reopening for bleeding:
Renal failure:
Tracheostomy:
Derangement of liver function:
Pleural effusions:

17. Post operative events: immediate II
Mean post -operative hospital stay : 17.6 days (range days)
Electrophysiological studies : 16
Medication at discharge:
Anticoagulation: 4
Antiarrhythmic drugs: 2
Digoxin & Diuretics: 6
Biventricular pacing : 2

18. Post operative events: medium term
Further VT episodes : 4
Partial 3/7
Complete 1/10
Supraventricular Tachycardia: 10

19. Echo findings14 yrs post RV Disarticulation.

20. Post operative Events: Long Term
Follow up : 94% (15/16)
1 lost to follow up alive & well at 5 years.
Median 13 years (range )
Death: 3 ( 9,11, 17 years post op)
Transplantation: 2 ( 3, 8 yrs post op)
Awaiting transplantation: 2 ( 3, 14 yrs post op)

21. 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

22. 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

23. Effects of Right ventricular pacing

24. 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