1. Angela Lorts MD Cincinnati Children’s Hospital Medical Center
The Failing Fontan Transplant and VAD Options Angela Lorts MD Medical Director, Ventricular Assist Devices
Angela Lorts MD
Cincinnati Children’s Hospital Medical Center

2. Disclosures: Off-label use of various devices will be discussed

3. Simplifying Fontan Failure
Early
Late
End-Stage
Long standing PLE
Cirrhosis
Renal Failure
Plastic Bronchitis
Poor Hemodynamics
Arrhythmias
New onset of symptoms
Arrhythmia
New onset of end-organ dysfunction
Early PLE and/or plastic bronchitis
Myocardial Dysfunction

4. Early Fontan Failure? Case:
4 year-old girl POD #15 from Extra- cardiac Fontan
Pancreatitis
Pleural Effusions
Fluid Overload
High Fontan Pressures

5. Is transplant a good option for the early failing Fontan?
Fontan failure
Early
Poor hemodynamics
Early Onset of symptoms
Risk factors for death while awaiting transplantation included:
A. Requirement for mechanical ventilation at listing;
B. Younger age (<4 years) at listing;
C. UNOS status 1 at listing
D. Shorter time interval from Fontan
Anatomic/physiologic problem
Bernstein D et al. Circulation 2006;114:
Fontan Conversion/Take Down

6. High mortality for early failing Fontans
Bernstein D et al. Circulation 2006;114:

7. Failed Medical Management
Fontan failure
Early
Late
Arrhythmia
Onset of early liver & renal dysfunction
Early PLE/plastic bronchitis
Myocardial Dysfunction
Onset of symptoms
Hemodynamic compromise
No anatomic problem
Failed Medical Management
Heart Transplant

8. Late Fontan Failure? Case:
12 year-old boy with “Failing Fontan”, 35 kg
EC Fontan at age 5
Referred for unremitting protein losing enteropathy
Albumin 1.5, IgG 150
1 year since diagnosis of PLE
No anatomic abnormalities
Severe myocardial dysfunction, EDP elevated to 15 mm hg

9. Protein Losing Enteropathy: Accrual of risk factors for VAD and Transplant
Clinical phase
Edema
Hypo-Alb
Body Electrolyte depletion
Hypo gammaglobulinemia
Hypo proteinemia
Malnutrition
Lymphopenia
Prothrombosis
>60% Albumin pool lost
Preclinical phase
All bad for patients awaiting transplant
Follow-up Time
Thorne 1998, EHJ

10. When to refer to transplant for PLE
When to refer to transplant for PLE? Continue to attempt medical management? PLE Outcomes
50% survival 5 years after diagnosis
85% survival 5 years after diagnosis
Mertens, 1998
John, 2014

11. Freedom from death and/or heart transplant in plastic bronchitis patients
50% mortality or transplant at 5 years
Freedom from death and/or heart transplant (HTx) in plastic bronchitis (PB) patients.
Kurt R. Schumacher et al. J Am Heart Assoc 2014;3:e000865

12. How do these compare to transplant outcomes?
Year
Author
Centres N
Age
EM (n)
EM % 1y 5y
10y
1995
Hsu
Columbia 9 3
33.3%
1998
Carey
Newcastle
2003
Michielon
Rome 6
8.6 4
66.7%
2004
Jayakumar
Multiple 24
15.7
37.5%
62.5%
58.0%
53.6%
Gamba
Bergamo 14
17.2 2
14.3%
86.0%
77.0%
62.0%
2006
Bernstein
PHTS 70
10.7
76.0%
68.0%
2008
Davies
CHONY 40
15.9
35.0%
2009
Lamour
PHTS/CTRD
107 15
70.0%
Griffiths
Boston 20
15.00%
85.0%
2012 43
32.6%
2011
Kantor
Emory 27 1
81%
66%
Average
31.5%
79.6%
1 year pediatric survival – 92%
5 year survival – 86%
All Pediatrics
1 year pediatric survival – approx 80%
5 year survival – approx 75%
Fontans

13. Summary of post transplant Fontan multi-center studies
Higher waitlist time and mortality
Standard listing criteria underestimates degree of illness
Increased risk for early graft failure
Death from sepsis more common
Bleeding common
PLE resolves in survivors

14. Fontan with PLE will wait along time.. not be a status 1a or a 1b

15. Question
Patient (12 year old, 35 kg, with dysfunction and PLE) comes into ED with 1 week history of difficulty breathing, Cr has doubled, he complains of headache and is vomiting. He is intubated with good respiratory response and now with poor UOP and escalating inotropes? Listed for transplant
If you decided to use mechanical support what device would you consider?
ECMO
Centrimag
Berlin
HVAD
Syncardia

16. (failure of medical management)
What if our patient has poor cardiac output while waiting?
Fontan VAD options
Fontan failure
(failure of medical management)
>35kg
Urgent
Semi-Urgent
Syncardia
Centrimag
<25 kg
>25 kg
Berlin Heart
HVAD
Pulsatile flow
Continuous flow
Continuous flow
Pulsatile flow

17. Paucity of multi-center published data for VADs in single ventricle
Weinstein, JTCS 2014
VanderPluym, JHLT 2016

18. Can we make them better transplant candidates with VAD or TAH support?
End-organ resuscitation
Nutritional and physical rehabilitation
Allow for desensitization
Possibly improve PLE?
Allow for discharge
Status 1a or 1b

19. Fontan failure Early Late End-Stage
PLE
Cirrhosis
Renal Failure
Plastic Bronchitis
Onset of symptoms
Arrhythmia (Afib)
Onset of liver and renal dysfunction
SVAD
Not a Candidate
AP Fontan/
Anatomic prob
Heart/Liver Transplant
If sx worsens
 EDP
Heart Transplant
VAD
Syncardia TAH
Fontan Conversion
Heart Transplant

20. Support for the late failing Fontan with myocardial dysfunction and multiple anatomic issues
Fontan with multi- factorial failure
Residual lesions + systolic/diastolic dysfunction
Capacitance chamber if there are not 2 AV valves
2 have been placed in single ventricle in US
70cc
50cc

21. Why Early Referral? – An adult awaiting transplant on medical therapy
Only 1/3 of adults with CHD on medical therapy will be transplanted at a year
UNOS, Proposal for Adult Allocation Change

22. 5 opportunities to improve peri-transplant outcomes in Fontan patients
Improved surveillance of end-organs and early referral.
Share experiences, via a learning network, regarding VAD in the Fontan to further the understand which patients will benefit from VAD and best support strategy.
Advocate for alterations of the organ allocation system.
Earlier referral for late Fontan failures before they are End-Stage.
Clear criteria for transplant referral
Seamless collaboration between transplant team and congenital team.

23. Thank you