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

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

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

Early Fontan Failure? Case: 4 year-old girl POD #15 from Extra- cardiac 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 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;114:273-280 Fontan Conversion/Take Down

High mortality for early failing Fontans Bernstein D et al. Circulation 2006;114:273-280

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

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 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

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

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

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

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? ECMO Centrimag Berlin HVAD Syncardia

(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

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 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

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

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 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.

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