Mortality Case after Liver Transplantation “Death and Life” Gustav Klimt ( )

40-year-old-male

Kidney Donor

40-year-old-male Kidney Donor Borderline renal function and proteinuira

40-year-old-male Kidney Donor Borderline renal function and proteinuira Borderline LFT

40-year-old-male Kidney Donor Borderline renal function and proteinuira Borderline LFT (alcoholic liver disease?)

40-year-old-male Kidney Donor Borderline renal function and proteinuira Borderline LFT (alcoholic liver disease?) Jaundice and increased Amylase

40-year-old-male Kidney Donor Borderline renal function and proteinuira Borderline LFT (alcoholic liver disease?) Jaundice and increased Amylase (gallstone pancreatitis?)

40-year-old-male Kidney Donor Borderline renal function and proteinuira Borderline LFT (alcoholic liver disease?) Jaundice and increased Amylase (gallstone pancreatitis?) No improvement even after alcohol abstinence and ERCP (renal failure, ascites and encephalopathy)

40-year-old-male Kidney donor Acute pancreatitis Acute liver failure Acute renal failure

40-year-old-male Kidney donor Acute pancreatitis Acute liver failure Acute renal failure + Hantaan virus antigen (+) (Hemorrhagic fever with renal syndrome?)

Suitable donor matched stable vital sign aggravating clinical course

:00 Induction of anesthesia hypotension even before the surgery Immediately before the reperfusion Cardiac arrest Intraoperative CPR Serious hypothermia and coagulopathy

Warm ischemic time 13 min Cold ischemic time 470 min Operation time 7hr 20 min 18:40 – 02:00 Transfusion RBC 23 U

Operation Findings Large amount of serous ascites - not bloody, nor turbid No bowel edema No retroperitoneal edema/inflammation Severe hepatomegaly without cirrhosis Significant portal hypertension Very hard and heavy liver

Operation Findings Hepatic veinHepatic arteryPortal vein

Immediate postoperative finding At ICU Serious Hypotension epinephreine, dopamine, norepinephrine, and vasopressin infusion Echocardiogram 10:29 preserved LV function Hypothermia Failing liver

Postoperative follow-up US 15:58 Very weak, if any, blood flow in the liver Bradycardia and Cardiac Arrest 16:50 Expire 18:00

Hepatic failure due to secondary amyloidosis Am J Med 1962;33:349 J Korean Med Sci 1988;3:151-5 Dig Liver Dis 2006;38: *No Liver Transplantation for secondary amyloidosis has been reported.

Korean J Hematol 1997;32:

Amyloidosis Primary amyloidosis Secondary amyloidosis Familial amyloidotic polyneuropathy

Amyloidosis Primary amyloidosis Secondary amyloidosis Familial amyloidotic polyneuropathy

Familial Amyloidotic Polyneuropathy A/D Sweden, Portugal, Japan Painful polyneuropathy GI Sx, Autonomic neuropathy Kidney/heart involvement Die 9-13 years after onset

Familial Amyloidotic Polyneuropathy Latent period: about 20 years

Familial Amyloidotic Polyneuropathy Refractory intraoperative orthostatic hypotension Some patients die during transplantation because of circulatory insufficiency Am J Transpl 2007;7: Amyloid Int J Exp Clin Invest 1994;1:138 Transplantation 1995;60:

Refractory intraoperative orthostatic hypotension Cardiomyopathy Conduction disorder Peripheral vasodilation secondary to autonomic neuropathy

Refractory intraoperative orthostatic hypotension Cardiomyopathy Conduction disorder Peripheral vasodilation secondary to autonomic neuropathy – major cause

Refractory intraoperative orthostatic hypotension Echocardiographic findings and Routine preoperative Holter monitoring: not helpful Spectral analysis of heart rate variability Transplantation 1997;63:

Refractory intraoperative orthostatic hypotension Routine cardiac pacemaker before anesthesia TEE during liver transplantation Low cardiac output is rare More vigorous volume loading before anesthesia Maintaining higher hemoglobin level Pure vasoconstictor such as phenylephrine is more frequently requred Serious bradyarrhythmia – atropine is ineffective, response to isoproterenol is preserved Am J Transpl 2007;7: J Clin Anesth 2005;17: Trnasplant proc 2000;32:

Consequentialism, every surgeon’s virtue, no doubt.