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Update on surgical practice and current state on Fontan conversion surgery
James S. Tweddell, MD Warren W. Bailey Endowed Chair, Director of Cardiothoracic Surgery Executive Co-Director, The Heart Institute Cincinnati Children’s Hospital Medical Center Professor of Surgery and Pediatrics University of Cincinnati June 28, 2017
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Disclosures: None
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Fontan = Severe Chronic Right Heart Failure
Venous hypertension Hepatic congestion ➔ cirrhosis Lymphatic congestion ➔ PLE, PB Restrictive lung disease Altered pulmonary vasculature Single ventricle dysfunction
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Post Fontan surgical options
Fontan Takedown Fontan Conversion Heart Transplant Lymphatic Decompression
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Fontan takedown
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Fontan Takedown to previous stage
For early Fontan failure Low cardiac output/elevated CVP Early post op Early revision – if anatomic problem clearly established ECMO – (primary arrhythmias, temporary ventricular dysfunction) Immediate takedown to previous stage
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Fontan Takedown to previous stage
For late Fontan failure Hepatic congestion, protein losing enteropathy, variceal bleeding Poor outcome
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Fontan Takedown to previous stage - outcome
Trezzi M et al, Bambino Gesù Hospital Rome Results: n = 18, 2 – immediate post op 16 - within 2 months of Fontan 17 early survivors 3 underwent subsequent Fontan 4 underwent transplant 10 remained as BDG Median O2 saturation = 83% (76-89%) Median follow-up = 7.0 years (0.9 – 26.5 years) Fontan Takedown n = 18 Early Death After Takedown n = 1 Extended Intermediate Palliation n = 10 Heart Transplant n = 4 Redo Fontan completion n = 3 Death After Heart Transplant n = 2 Long-Term Outcomes After Extra-Cardiac Fontan Takedown to an Intermediate Stage. STS 2017
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Fontan Takedown to previous stage - outcome Multi-institutional study
Fontan to takedown (0.6±1.9 years) Follow up (FU) of 6.7±6.7 years (0–23.7years) Early mortality = 26.3% (10/15 patients) 5 Late deaths 4 patients (10.5%) underwent HTX after Fontan takedown After a mean period of 12.9±21.1 months, with two patients alive at the end of follow-up. 2 takedown patients (5.3%) underwent subsequent TCPC LT operation after a mean period of 34.1±14.1 months. 17 patients (44.7%) had reached the primary end point mortality/HTX at the end of follow-up Fontan Takedown n = 38 Early Death After Takedown n = 10 Late Death n = 5 Heart Transplant n = 4 Redo Fontan completion n = 2 Death After Heart Transplant n = 2 van Melle JP et al Heart 2016
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Fontan Takedown to previous stage - outcome Multi-institutional study
van Melle JP et al Heart 2016
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Fontan conversion
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Fontan conversion Backer CL et al Cardiol Young 2006
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Fontan conversion Backer CL et al Cardiol Young 2006
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Fontan conversion Backer CL et al Cardiol Young 2006
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Fontan conversion
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Fontan conversion – freedom from arrhythmias
The more complete the arrhythmia procedure the lower risk of recurrence Bi-atrial maze Right atrial maze Isthmus ablation Deal B et al Ann Thorac Surg 2016
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Fontan conversion - survival
Multi-center studies show early mortality of ~10%2,3 Operative mortality was 1.4%1 Deal B et al Ann Thorac Surg 2016 Fuller SM et al Ann Thorac Surg 2015 van Melle JP et al Heart 2016
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Prophylactic Fontan conversion? n = 32, 1991 – 2012
Ann Thorac Surg 2017 Prophylactic Fontan conversion? n = 32, 1991 – 2012 Group 1, n = 25; tachyarrhythmias and PLE Group 2, n = 7; no late Fontan compications
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Fontan conversion Who is a candidate for conversion? Risk factors*
Primarily an arrhythmia procedure Preserved ventricular function Preserved end-organ function Risk factors* Protein losing enteropathy Older age Ascites Right or indeterminate ventricular morphology Bi-atrial arrhythmia operation *Deal B et al Ann Thorac Surg 2016
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Fontan conversion only extracardiac Fontan in Australia and New Zealand since 2007 d'Udekem Y et al Circ 2014
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Heart transplantation
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Heart transplantation
SVC IVC Aorta LA cuff Aorta SVC Extracardiac Fontan RA IVC
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Heart transplantation
Sughimoto K et al Ann Thorac Surg 2015
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Heart transplantation
SVC IVC Aorta
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Thoracic duct decompression
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Thoracic duct decompression
The lymphatic circulation drains into the venous system Venous hypertension will result in lymphatic hypertension Lymphatic hypertension Protein losing enteropathy Plastic bronchitis
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Thoracic duct decompression
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Thoracic duct decompression
Hraska V Ann Thorac Surg 2013
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Summary and conclusions
Fontan takedown - life saving for some patients with early failure Fontan conversion is an option for individuals with an aortopulmonary Fontan, arrhythmias and preserved function The future role of Fontan conversion is unclear as the number of individuals with an aortopulmonary Fontan decreases Lymphatic decompression may provide palliation for PLE and PB with preserved function Ultimately transplant remains the best chance for long term survival Better mechanical support strategies Better appreciation of the impact of liver dysfunction and alterations of the pulmonary vasculature on survival Improved understanding of sensitization and immunosuppression
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