Download The Failing Fontan Transplant and VAD Options

The Failing Fontan
Transplant and VAD Options
Angela Lorts MD
Medical Director, Ventricular Assist Devices
Angela Lorts MD
Cincinnati Children’s Hospital Medical Center
Disclosures:
Off-label use of various devices will be discussed
Simplifying Fontan Failure
Fontan
Failure
Early
• Poor Hemodynamics
• Arrhythmias
• New onset of symptoms
Late
• Arrhythmia
• New onset of endorgan dysfunction
• Early PLE and/or
plastic bronchitis
• Myocardial
Dysfunction
End-Stage
• Long standing
PLE
• Cirrhosis
• Renal Failure
• Plastic Bronchitis
Early Fontan Failure?
Case:
4 year-old girl POD #15 from Extracardiac Fontan
• Pancreatitis
• Pleural Effusions
• Fluid Overload
• High Fontan Pressures
Is transplant a good option for the early failing
Fontan?
Fontan failure
Early
• Poor hemodynamics
• Early Onset of symptoms
Anatomic/physiologic
problem
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
Bernstein D et al. Circulation 2006;114:273-280
Fontan Conversion/Take Down
High mortality for early failing Fontans
Bernstein D et al. Circulation 2006;114:273-280
Fontan failure
Late
Early
• Onset of symptoms
• Hemodynamic compromise
•
•
•
•
Arrhythmia
Onset of early liver &
renal dysfunction
Early PLE/plastic
bronchitis
Myocardial Dysfunction
No anatomic
problem
Failed Medical
Management
Heart Transplant
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
Protein Losing Enteropathy: Accrual of
risk factors for VAD and Transplant
Clinical phase
>60%
Albumin pool
lost
Preclinical phase
Follow-up Time
Thorne 1998, EHJ
•
•
•
•
•
•
•
•
Edema
Hypo-Alb
Body Electrolyte depletion
Hypo gammaglobulinemia
Hypo proteinemia
Malnutrition
Lymphopenia
Prothrombosis
All bad for
patients
awaiting
transplant
When to refer to transplant for PLE? Continue to
attempt medical management? PLE Outcomes
50% survival 5 years
after diagnosis
Mertens, 1998
85% survival 5 years
after diagnosis
John, 2014
Freedom from death and/or heart
transplant in plastic bronchitis patients
50% mortality or
transplant at 5 years
Kurt R. Schumacher et al. J Am Heart Assoc
2014;3:e000865
How do these compare to transplant
outcomes?
Year
Author
Centres
N
1995
Hsu
Columbia
1998
Carey
Newcastle
2003
AllMichielon
Pediatrics
Rome
Age
EM (n)
EM %
9
3
33.3%
9
3
33.3%
1y
5y
10y
1 year pediatric survival – 92%
6
8.6
4
66.7%
5 year survival – 86%
2004
Jayakumar
Multiple
24
15.7
9
37.5%
62.5%
58.0%
53.6%
2004
Gamba
Bergamo
14
17.2
2
14.3%
86.0%
77.0%
62.0%
1 year pediatric survival – approx 80%
70
10.7
76.0%
5 year survival – approx 75%
68.0%
2006
Bernstein
Fontans
PHTS
2008
Davies
CHONY
40
15.9
2009
Lamour
PHTS/CTR
D
107
15
2009
Griffiths
Boston
20
3
15.00%
2012
Davies
Columbia
43
14
32.6%
2011
Kantor
Emory
27
1
Average
14
35.0%
31.5%
77.0%
70.0%
85.0%
85.0%
81%
66%
79.6%
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
Fontan with PLE will wait along time.. not be a
status 1a or a 1b
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?
A. ECMO
B. Centrimag
C. Berlin
D. HVAD
E. Syncardia
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
Berlin Heart
>25 kg
HVAD
Pulsatile flow
Continuous
flow
Pulsatile flow
Continuous
flow
Paucity of multi-center published data for VADs
in single ventricle
Weinstein, JTCS 2014
VanderPluym, JHLT 2016
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
Fontan failure
Early
• Onset of symptoms
Late
End-Stage
• Arrhythmia (Afib)
• Onset of liver and
renal dysfunction
SVAD
AP Fontan/
Anatomic prob
If sx worsens
 EDP
•
•
•
•
PLE
Cirrhosis
Renal Failure
Plastic Bronchitis
Not a
Candidate
Heart Transplant
VAD
Fontan Conversion
Heart Transplant
Heart/Liver
Transplant
Syncardia TAH
Support for the late failing Fontan
with myocardial dysfunction and
multiple anatomic issues
• Fontan with multifactorial failure
• Residual lesions +
systolic/diastolic
dysfunction
• Capacitance chamber if
there are not 2 AV valves
• 2 have been placed in
single ventricle in US
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
5 opportunities to improve peri-transplant
outcomes in Fontan patients
1. Improved surveillance of end-organs and early referral.
2. 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.
3. Advocate for alterations of the organ allocation system.
4. Earlier referral for late Fontan failures before they are
End-Stage.
5. Clear criteria for transplant referral
6. Seamless collaboration between transplant team and
congenital team.
Thank you