Fontan Global Case Conference Richard J. Czosek, MD 6/28/17 The Role of Catheter Ablation in Fontan Arrhythmia Control: Patient Based Decision Making Fontan Global Case Conference Richard J. Czosek, MD 6/28/17

Patient 1 20 yo with non-fenestrated extra-cardiac Fontan who recently had their first episode of atrial tachycardia They were cardioverted without issue and are now on Coumadin Currently 3 months from this first episode of tachycardia currently on no anti-arrhythmic medications Patient 2 43 yo with a atrio-pulmonary Fontan with history of multiple cardioversions for atrial tachycardia with recent increase in symptomatology and 3-4 episodes in the past 12 months History previous ablation procedure 4 years prior Currently on Coumadin as well as amiodarone with continued breakthrough episodes of tachycardia

Prevalence of Atrial Flutter/IART in Fontan 1973 - 1991 Fishberger, et al., Jour. Thor. Card. Surgery, 1997

Role of Trans-catheter Ablation Medication DCCV Surgical Ablation Pacing Rate Control

Recurrence of Atrial Tachycardia Egbe et al., Int. Journ. Of Cardiology, 2016

Risk of Atrial Tachycardia Recurrence Egbe et al, American Heart Journal, 2017

To ablate or not to ablate Don’t Ablate

When to ablate? Ablation DCCV/ Pacing Fontan + Arrhythmia Medications/ Rate Control

Fontan Patient Decision Tools Trans-catheter Ablation Medications Surgical Ablation Medications Trans-catheter Ablation Surgical Ablation Decision Fontan Patient Atrial Arrhythmia Surgical Ablation Medications Decision Tools Patient Age Fontan Type Tachycardia Type Frequency of Tachycardia Patient Risk Factors Rate of Tachycardia Patient Preference Physician Preference Center philosophy Monitor for Recurrence Medications Trans-catheter Ablation

Trans-catheter Ablation Patient Decision Tools Fontan Type Tachycardia Type Frequency of Tachycardia Patient Risk Factors Rate of Tachycardia Patient Preference Physician Preference Center philosophy Patient Age

Trans-catheter Ablation Patient Decision Tools Fontan Type Tachycardia Type Frequency of Tachycardia Patient Risk Factors Rate of Tachycardia Patient Preference Physician Preference Center philosophy Patient Age

Atrial Arrhythmia and Fontan Type “Classic” Style Fontan Atrio-pulmonary Fontan Lateral Tunnel Fontan/Intra-atrial Conduit Extra-cardiac Conduit Fontan Fenestrated Kutty et al., Heart, 2015

Fontan Cardiac Type Fishberger, et al., Jour. Thor. Card. Surgery, 1997

Classic vs Lateral Tunnel Fontan Atrio-pulmonary Fontan Stamm et al., Journ. Thor. Card. Surgery, 2001

Effect of Fontan Type Balaji et al., Cong. Heart Disease, 2013

Atrio-pulmonary Fontan Effect of Fontan Type Extra-Cardiac Fontan Lateral Tunnel Fontan Atrio-pulmonary Fontan Pundi et al., Cong. Heart Disease, 2016

Collins et al, Amer. Heart Journal, 2000

Access to Tachycardia Substrate Kutty et al., Heart, 2015

Access in Differing Styles of Fontan Frequency of Tachycardia Classic Fontan Lateral Tunnel Extra-Cardiac Ease of Ablation Classic Fontan Lateral Tunnel Extra-Cardiac

Accessing Substrate In Extra-cardiac Fontan Moore et al., Heart Rhythm, 2016 Moore et al., Circulation Arr. Elect., 2015

Risks of Fontan Ablation Correa et al., JAHA, 2013

Risks of Fontan Ablation Correa et al., JAHA, 2013

Trans-catheter Ablation Decision Tools Fontan Type Tachycardia Type Frequency of Tachycardia Patient Risk Factors Rate of Tachycardia Patient Preference Physician Preference Center philosophy Patient Age

Fontan Arrhythmia Mechanism Arrhythmias Number % of All (n=80) % of Ablations (n=59) Macro-reentrant 25 31% 42% Focal Atrial Tachycardia 8 10% 14% AVNRT 13 16% 22% AVRT 4 5% 7% VT 5 6% 8% Twin Node Undefined 21 26% n/a 57 procedures on 52 patients 80 arrhythmias identified Correa et al, Circ Arrhythm Electrophys, 2014

Atrial Fibrillation Takahashi et al, Europace, 2008

Trans-catheter Ablation Decision Tools Fontan Type Tachycardia Type Frequency of Tachycardia Patient Risk Factors Rate of Tachycardia Patient Preference Physician Preference Center philosophy Patient Age

Trans-catheter Ablation Decision Tools Fontan Type Tachycardia Type Frequency of Tachycardia Patient Risk Factors Rate of Tachycardia Patient Preference Physician Preference Center philosophy Patient Age

Risk Factors for SCD in Fontan Walsh E, et al, Heart Rhythm, 2014

Arrhythmia Influence on Fontan Diller et al, European Heart Journal, 2010

Risk of Mortality in Fontan Fontan Type Clinical arrhythmias Symptoms of heart failure Diller et al, Eur. Heart Journal, 2010

Trans-catheter Ablation Decision Tools Fontan Type Tachycardia Type Frequency of Tachycardia Patient Risk Factors Rate of Tachycardia Patient Preference Physician Preference Center philosophy

What is The Goal of Fontan Ablation? Hiroshi Nakagawa et al. Circulation, 2001

Outcomes in Fontan Patients With Atrial Arrhythmias Egbe et al, Amer. Heart Journal., 2016

Outcomes in Fontan Patients With Atrial Arrhythmias Egbe et al, Amer. Heart Journal., 2016

Correa et al., Circulation Arrhythmia Elect., 2015

Fontan Conversion/Arrhythmia Surgery Deal et al., Brit. Med. Journal, 2012

Risk of Atrial Tachycardia Recurrence Egbe et al, American Heart Journal, 2017

Trans-catheter Ablation Recurrent Atrial Arrhythmia Decision Patient Age Fontan Type Frequency of Tachycardia Patient Preference Fontan Patient Atrial Arrhythmia DCCV +/- Meds Recurrent Atrial Arrhythmia Surgical Ablation Decision Patient Age Fontan Type Patient Risk Factors Patient Preference Physician Preference Decision Patient Age Fontan Type Frequency of Tachycardia Patient Preference

Conclusions Fontan patients with SVT are likely to have recurrence of SVT Anti-arrhythmic medications are reasonable but unlikely to keep patients tachycardia free New onset Atrial Tachycardia or increased tachycardia burden is often an early indicator of other issues Catheter ablation plays a major role in management of SVT in Fontan patients and is reasonable as first or second line management We need to clearly define our goals The role of catheter ablation varies based on center and patient preference The role of catheter ablation is likely to change significantly over the next decades with change in Fontan anatomy

Patient 1 20 yo with non-fenestrated extra-cardiac Fontan who recently had their first episode of atrial tachycardia They were cardioverted without issue and are now on Coumadin Currently 3 months from this first episode of tachycardia currently on no anti-arrhythmic medications Question 1 Recommend catheter ablation as a first line treatment strategy Recommend monitoring without ablation or anti-arrythmic medication Recommend starting anti-arrhythmic medication without ablation Other

Patient 2 43 yo with a atrio-pulmonary Fontan with history of multiple cardioversions for atrial tachycardia with recent increase in symptomatology and 3-4 episodes in the past 12 months History previous ablation procedure 4 years prior Currently on Coumadin as well as amiodarone with continued breakthrough episodes of tachycardia Question 2 Recommend catheter ablation with continued anti- arrhythmic mediations Recommend monitoring without ablation or anti-arrythmic medication change Recommend starting a new anti-arrhythmic medication or changing anti-arrhythmic medication without ablation Other

Cincinnati Children's Hospital Medical Center

Sudden Death in Fontan Gallego et al, Amer. Journ. Card., 2012 Diller et al, European Heart Journal, 2010

Is There Disease Specific Risk of SCD? Gallego et al, Amer. Journ. Card., 2012

Sudden Death in Fontan’s Khairy et al., Circulation

Sudden Death in Fontan Pundi et al., Cong. Heart Disease, 2016

The Role of Surgical Ablation Deal et al., Brit. Med. Journal, 2012

Definition of Success in Fontan Ablation

Arrhythmic Influence on Single Fontan Diller et al, European Heart Journal, 2010

Fontan Arrhythmia Mechanism Arrhythmias Number % of All (n=80) % of Ablations (n=59) Macro-reentrant 25 31% 42% Focal Atrial Tachycardia 8 10% 14% AVNRT 13 16% 22% AVRT 4 5% 7% VT 5 6% 8% Twin Node Undefined 21 26% n/a 57 procedures on 52 patients 80 arrhythmias identified Access to pulmonary venous atrium Fenestration - 16 Trans-septal - 17 Retrograde - 2 Correa et al, Circ Arrhythm Electrophys, 2014