Right ventricular disarticulation for arrythmogenic right ventricular dysplasia

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Right ventricular disarticulation for arrythmogenic right ventricular dysplasia: an 18 year single centre experience.

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

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

Histology

Treatment options with ARVD patients: • Antiarrhythmic medication • Percutaneous catheter ablation • Automatic

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

Right Ventricular Disarticulation v Described initially by Guiraudon v Animal studies done by Guiraudon

Right Ventricular Disarticulation v Described initially by Guiraudon v Animal studies done by Guiraudon & Cox et al v Medium term results from Cox and Hilton et al v Few surgical reports lately

Retrospective Case series: • Institution: Tertiary referral centre : Freeman hospital. • Time Frame:

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

Pre Op Details: • Presentation: » 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

Preop Echo Of ARVD patient

Operative details: • Complete: 10 • Partial: 7 • Full Cardiopulmonary Bypass • Cold

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

Gross Appearance Of ARVD Heart

Gross Appearance Of ARVD Heart

Completed RV Disarticulation

Completed RV Disarticulation

Post operative events: immediate I § Mortality : 1 (6%) multi-organ failure § Overall

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: 4 3 2 1 3

Post operative events: immediate II o Mean post -operative hospital stay : 17. 6

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: ü Anticoagulation: 4 ü Antiarrhythmic drugs: 2 ü Digoxin & Diuretics: 6 o Biventricular pacing : 2

Post operative events: medium term • Further VT episodes : 4 • Partial 3/7

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

Echo findings 14 yrs post RV Disarticulation.

Echo findings 14 yrs post RV Disarticulation.

Post operative Events: Long Term • Follow up : 94% (15/16) – 1 lost

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 16 15 14 80 13 12 11 Survival % 60

Kaplan-Meier actuarial survival 100 16 15 14 80 13 12 11 Survival % 60 40 20 0 0 5 10 Survival in yea rs 15 20

Discussion points: • Natural history of ARVD • Affects young patients • Biventricular failure

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

Effects of Right ventricular pacing

Conclusions: • Excellent antiarrhythmic procedure • 77% event free survival at 10 years •

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

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Ventricular Tachycardia VT Ventricular tachycardia occurs when electricalVentricular Tachycardia VT Ventricular tachycardia occurs when electrical