The Failing Fontan Transplant and VAD Options Angela

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 • 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, Ig. G 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: e 000865

How do these compare to transplant outcomes? Year Author Centres N 1995 Hsu Columbia 1998 Carey Newcastle 2003 All. Michielon Pediatrics Rome Age EM (n) EM % 9 3 33. 3% 1 y 5 y 10 y 1 year pediatric survival – 92% 6 5 year 8. 6 survival 4– 86%66. 7% 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% 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 1 a or a 1 b

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) >35 kg Urgent Semi-Urgent Syncardia Centrimag <25 kg Berlin Heart >25 kg HVAD Pulsatile flow Continuous flow

Paucity of multi-center published data for VADs in single ventricle Weinstein, JTCS 2014 Vander. Pluym, 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 1 a or 1 b

Fontan failure Early • Onset of symptoms AP Fontan/ Anatomic prob Late End-Stage • Arrhythmia (Afib) • Onset of liver and renal dysfunction SVAD 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 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 70 cc 50 cc

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