1. + Liver Transplantation for PSC Patients A Transplant Surgeon’s Perspective Tiffany Anthony, MD Annette C. and Harold C. Simmons Transplant Institute Baylor University Medical Center

2. + Liver Transplant: The Journey to and Through Liver Transplant Recipient Selection Transplant Listing Donor Selection Liver Offers THE LIVER TRANSPLANT EVENT Post-Op Recovery Post-Op Complications Long Term Complications Outcomes/Survival

3. + Liver Transplant: When is time to pursue Tranplant Listing? Cirrhosis Compensated Cirrhosis Decompensated Cirrhosis: Complications of Portal Hypertension Ascites Encephalopathy Esophageal Varices Hepato-renal Syndrome/Hepato-pulmonary/Porto- Pulmonary Hepatocellular Carcinoma: HCC

4. + Liver Transplant: When is time to pursue Transplant Listing? Indications for Liver Transplant Listing Specific to PSC patients Cholangitis Pruritis Cholangiocarcinoma: Special Protocols/Tight Restrictions/Few Centers

5. + Liver Transplant: Indications for Transplant Listing Portal Hypertension Fibrotic Stiff Liver Toll Booth Analogy Hormonal Cascade Dilated and “Leaky” Venous System Causing Downstream Symptoms Of Liver Disease

6. + Liver Transplant: Indications for Transplant Listing Ascites

7. + Liver Transplant: Indications for Transplant Listing Hepatic Encephalopathy

8. + Liver Transplant: Indications for Transplant Listing Esophageal Varices: Nadolol and Banding

9. + Liver Transplant: Indications for Transplant Listing Hepato-renal Syndrome Reversible kidney failure caused by worsening liver failure. Treatment OLT Hepato-Pulmonary Syndrome Hypoxemia-low blood oxygen levels Porto-Pulmonary Syndrome Portal Hypertension and Pulmonary Hypertension

10. + Liver Transplant: Indications for Transplant Listing Special Considerations for PSC patients Cholangitis: RRB Points Pruritis: RRB Points Cholangiocarcinoma

11. + Liver Transplant: Indications for Transplant Listing ERCP/PTC: Two different ways to stent the bile duct. ERCP

12. + Liver Transplant: Indications for Transplant Listing PTC

13. + Liver Transplant: Transplant Listing Considerations Liver Transplant Evaluation Risk-Benefit Ratio Contraindications to Liver Transplant MELD Score: Total Bilirubin INR Creatinine Total Bilirubin PSC disadvantage Options The Wait Alternative to Deceased Donor Transplant/The Wait List Living Donor Liver Transplant

14. + Liver Transplant: Donor Selection Considerations Types of Liver Donors Brain Dead Donors-5% DCD (Donor After Cardiac Death)-95% Living Donor Donor Selection Criteria Age Medical History BMI Laboratory Values Size CDC High Risk What does the Transplant Surgeon think about the liver about the liver on visualization?

15. + Liver Transplant: Donor Selection- Visualization Normal Liver

16. + Liver Transplant: Donor Selection- Visualization Cirrhotic Liver

17. + Liver Transplant: Donor Selection- Visualization Steatotic “Fatty” Liver

18. + Liver Transplant: Donor Selection Fatty Liver: Pathology

19. + Liver Transplant: Liver Offers OPO: Organ Procure Organization

20. + Liver Transplant: Liver Offers DonorNet: Electronic Notification Review Donor information in DonorNet Liver Biopsy Imaging Coordination of Donor OR time with OPO, other Transplant Centers and Aviation company Procurement Transplant OR timing

21. + Liver Transplant: Procurement

22. + Liver Transplant: THE OPERATION Incisions

23. + Liver Transplant: THE OPERATION

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25. + Liver Transplant: Post Op Recovery ICU Stay What to expect in the first 24-48 hours. Regular Ward Stay What to expect the next 5-10 days after ICU stay Post-Op Clinic Twice Weekly appts Weekly apps 1-3 month stay in close proximity to Transplant Center

26. + Liver Transplant: Early Complications Operative Death 3-5% Primary Non-Function 1-3% Post-op Bleeding 5% Biliary Complications 10-15 Vascular Complications 2-4% Post Op Infections Acute Rejection 10-30%

27. + Liver Transplant: Early Complications-Vasculcar 2-4% Hepatic Artery Thrombosis (HAT) Early vs. Late: 4 week cut-off period Surgery Relist patient Status 1 if < 1 week from LT Re-Transplant-Late Hepatic Artery Stenosis (HAS) Arterial Revision Stent Placement Portal Vein Stenosis/Thrombosis

28. + Liver Transplant: Early Complications-Biliary 10-15% Biliary Anastomotic Strictures Technical problem Dx: rising canalicular enzymes, PTC/ERCP Operative revision Biliary Leak Rare, more often seen with split livers Dx: PTC/ERCP Rx: ERCP/stent with drainage Operative revision

29. + Liver Transplant: Immunosuppression Side-Effects/Long Term Effects of Immunosuppression Prograf/tacrolimus Cyclosporine Cell Cept/Myfortic Prednisone

30. + Liver Transplant: Long Term Complications Obesity Diabetes Hypertension Hyperlipidemia Renal Dysfunction Bone Loss Cardiovascular Complications PSC Recurrence

31. + Liver Transplant: Long Term Complications PSC Recurrence Estimated to occur in 20-25% of transplant recipients any where from 5-10 years post transplant. Largest meta-analysis reported a recurrence of 17% in 940 PSC patients Can lead to graft failure but this is rare. No proven preventative measures.

32. + Liver Transplant: Outcomes 1 Year Survival: 90% 5 Year Survival: 75-80% Very Long Term Survival Data on PSC Patient Post Transplant Survival Survival Deceased Donor Living Donor 1 year 93% 97.2% 5 year 87.5% 95%

33. + Liver Transplant: The Future/Other Technologies Tissue Engineering Using a scaffold Infusing liver cells Cell signals to differentiate into the different cells/structures ?In my career/lifetime? Liver Dialysis Bridge to Transplant Donor Liver Perfusion Machines Extend Time from Procurement to Transplant Salvage “Marginal Livers”

34. + Liver Transplant: Our patients/Our Friends

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36. + The Organ Grinders

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38. + Questions?