Transcript Right Ventricular disarticulation for arrythmogenic right ventricular

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
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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:
» Syncope: 12
» Intractable VT : 3 patients
» Electrical storms (AICD): 2
• Management;
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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:
 Renal failure:
 Tracheostomy:
 Derangement of liver function:
 Pleural effusions:
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2
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3
Post operative events: immediate II
o Mean post -operative hospital stay : 17.6 days
(range 9 - 35 days)
o Electrophysiological studies : 16
o Medication at discharge:
 Anticoagulation: 4
 Antiarrhythmic drugs: 2
 Digoxin & Diuretics: 6
o 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
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80
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Survival % 60
40
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Survival in yea rs
15
20
Discussion points:
• Natural history of ARVD
• 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
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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