1. 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

2. Disclosures:
None

3. Fontan = Severe Chronic Right Heart Failure
Venous hypertension
Hepatic congestion ➔ cirrhosis
Lymphatic congestion ➔ PLE, PB
Restrictive lung disease
Altered pulmonary vasculature
Single ventricle dysfunction

4. Post Fontan surgical options
Fontan Takedown
Fontan Conversion
Heart Transplant
Lymphatic Decompression

5. Fontan takedown

6. 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

7. Fontan Takedown to previous stage
For late Fontan failure
Hepatic congestion, protein losing enteropathy, variceal bleeding
Poor outcome

8. 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

9. 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

10. Fontan Takedown to previous stage - outcome Multi-institutional study
van Melle JP et al Heart 2016

11. Fontan conversion

12. Fontan conversion
Backer CL et al Cardiol Young 2006

13. Fontan conversion
Backer CL et al Cardiol Young 2006

14. Fontan conversion
Backer CL et al Cardiol Young 2006

15. Fontan conversion

16. 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

17. 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

18. 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

19. 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

20. Fontan conversion
only extracardiac Fontan in Australia and New Zealand since 2007
d'Udekem Y et al Circ 2014

21. Heart transplantation

22. Heart transplantation
SVC
IVC
Aorta
LA cuff
Aorta
SVC
Extracardiac
Fontan RA
IVC

23. Heart transplantation
Sughimoto K et al Ann Thorac Surg 2015

24. Heart transplantation
SVC
IVC
Aorta

25. Thoracic duct decompression

26. 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

27. Thoracic duct decompression

28. Thoracic duct decompression
Hraska V Ann Thorac Surg 2013

29. 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