When to implant an ICD in systemic right ventricle?

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1 When to implant an ICD in systemic right ventricle? Département de rythmologie et de stimulation cardiaque Nicolas Combes Pôle de cardiologie pédiatrique et congénitale

2 Risk of sudden death ICD related complications

3 Systemic RV Ejection Fraction Functional class (NYHA) Complex ventricular arrhythmias Factors influencing sudden death Ventricular scars Electrophysiological markers - QRS duration - QRS dispersion - Programmed ventricular stimulation Supraventricular arrhythmias

4 A wide spectrum of cardiac abnormalities and surgery Congenitally corrected transposition of great arteries - native without surgery - ventricular surgery for VSD - conduit between LV and pulmonary artery - double switch Complete transposition of great arteries after atrial switch (Mustard and Senning) with possible ventricular sugery Single right ventricle physiology +/- Fontan circulation or aortopulmonary shunt - Hypoplastic left heart syndrome - Mitral atresia - double inlet right ventricle - unbalanced common AV canal defect Not the same sudden death risk

5 Koyak Z et al. Circulation 2012;126:1944 Database with adults (Netherlands, Belgium and Canada) with CHD 1189 death (5%), 213 sudden death (171 arrythmic) X cctga (n=12): 75% with associated defect and surgery (VSD, pulmonary stenosis) dtga (n=18) : 83% Mustard or Senning repair

6 Koyak Z et al. Circulation 2012;126:1944 No specific datas for single ventricle physiology

7 Supraventricular arrhythmias can lead to sudden death

8 Aggressive management of these arrhythmias

9 Potential treatable causes of sudden death especially in congenitally corrected transposition of great arteries AV block 1%/year Secondary to AV discordance AV accessory pathway (Kent) ++ in case of Ebstein tricuspid valve Anderson RH et al. Circulation 1974;50:911 Possible AV reentrant tachycardia with poor tolerance or atrial fibrillation inducing VF

10 Ventricular scar and QRS dispersion Piers SR et al. Circ Arrythm Electrophysiol 2012

11 Age and sudden death X Koyak Z et al. Circulation 2012;126:1944

12 Silka MJ et al. J Am Coll Cardiol 2008;32: patients, 41 sudden deaths monocentric, oregon Maximal risk for Aortic stenosis, coarctation, tetralogiy of Fallot and d-tga (most of them with Mustard or Senning)

13 Silka MJ et al. J Am Coll Cardiol 2008;32:245 80% of cardiovascular death in CHD : - 1/3 sudden death (80% arrhythmic) - 1/3 heart failure - 1/3 others (embolic, anevrysm dissection endocarditis ) Increase risk for sudden death 25 to 100 times greater than matched population control

14 ICD related complications Problems for transvenous leads : - venous access - intracardiac residual shunt with thromboembolic risk (avoid) - young patients with significant activity and important risk of lead fracture Problems for epicardial or thoracic leads : - which lead? - risk of epicardial approach and redo surgery in these complex patients with poor hemodynamics - frequent need for pacing in association with shock delivery (subcutaneous ICD alone rarely indicated)

15 ICD Follow up limited with very small dedicated studies only in D TGA with atrial switch Khairy P et al. Circulation 2008 Bouzeman A et al. Int J Cardiol 2014 Annual incidence of therapies (mean FU 3,4 years) : - Appropriate : 0,5% in primary prevention, 6% in secondary prevention - Inappropriate : 6 to 8% ICD/lead malfunction : up to 20% Implantation associated death : 1 secondary to defibrillation testing 50% of patients with sustained SVT in ICD recording during follow up No appropriate therapy in patients waiting for heart transplantation

16 Consensus for ICD implant in patients with systemic right ventricle Khairy P et al. Heart Rhythm 2014 Class I Survivors of cardiac arrest due to VF or hemodynamically unstable VT after evaluation to define the cause of the event and exclude any completely reversible etiology (Level B) Spontaneous sustained VT after hemodynamic and electrophysiologic evaluation (Level B). Catheter ablation or surgery may offer a reasonable alternative or adjunct to ICD therapy in carefully selected patients (Level C) Secondary prevention

17 Consensus for ICD implant in patients with systemic right ventricle Khairy P et al. Heart Rhythm 2014 May be reasonable in adults with single or systemic right ventricular EF < 35%, particularly in the presence of additional risk factors such as complex ventricular arrhythmias, unexplained syncope, NYHA II or III, QRS duration 140 ms, severe systemic AV valve regurgitation (Level C) Class IIB May be considered in case of syncope of unknown origin and hemodynamic significant VT or FV inducible at electrophysiologic study (Level B) Nonhospitalized adults awaiting heart transplantation May be considered in adults with systemic ventricular function < 35% alone and in adults with moderate or complex CHD with syncope and «high clinical suspicion of ventricular arrhythmias» (Level C)

18 Conclusion ICD indication in systemic right ventricle is relatively simple with consensus in cases of secondary prevention, very complex in cases of primary prevention with few datas available. A case by case discussion should by made in each patient balancing risk factors of sudden death and potential life threatening events associated with ICD implant A global management should be performed in each case with aggressive management of supraventricular arrhythmias, close follow up for severe conduction disorders, discussion for specific treatment for residual hemodynamic lesions (intracardiac shunts, valvular disease ) In advanced cases with very poor hemodynamic parameters, ICD seems not to be a good option alone without hemodynamic project (assistance, transplantation, other?)

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