Selecting Patients for Liver Transplantation: Who, When

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Transcript Selecting Patients for Liver Transplantation: Who, When

Selecting Patients for Liver Transplantation: Who, When and Unusual Cases

Ahmet Gurakar, MD Johns Hopkins School of Medicine Medical Director, Liver Transplant Program Nov 26 , 20112 Florence Nightingale Liver Transplant Program Istanbul

LIVER TRANSPLANT TEAM

Transplant Hepatology

-Patient Selection -Maintenance on the list -New Policy Develop.

Liver Transplant Surgery

-Donor selection -New Policy Develop.

MICU-SICU-NCCU Anesthesia

Consultative Services

Cardio-Pulmonary-Nephro-Neuro-Psychology-Soc.W

Referral for Liver Transplantation Evaluation

Acute Liver failure ( ALF )  Complications of cirrhosis; i.e. Encephalopathy, Ascites, Hepatorenal Syndrome, Bleeding from Portal Hypertension, all indicating a change in the Natural History of the Disease  Detection of HCC  Consider referral at the first appearance of the above complications, even in the presence of low MELD score JG O’Leary, R Lepe. GL Davis. Gastro 2008:134:1764-1776

Expanding Indications for Liver Transplantation (OLT)

 Metabolic Disorders: 1) Primary defect in the liver; a) Hepatic Complications: Deficiency, Hereditary tyrosinemia I, Glycogen storage I/IV, PFIC(Byler Dis.), Arteriohepatic dysplasia(Alagille synd.), neonatal hemochromatosis Wilson’s Disease, A1AntiTrypsin b)Extrahepatic Complications: Polyneuropathy), Primary hyperoxaluria I, Crigler-Najjar I, Familiar hypercholesterolemia, Urea Cycle Defects, Hemophilia 2)Primary defect extra hepatic; FAP(Familiar Amyloidotic a) b) May Recur: Hereditary Hemochromatosis, Gaucher, Erytopoietic protoporphyria, NASH Curative for Hepatic component: Cystic Fibrosis Zhang KY Clin Liver Dis 11(2007) 265-281

Expanding Indications for OLT

    ALF ( Acute Liver Failure ): Hep A,B, DILI ( drug induced liver failure ) ESLD ( End Stage Liver Disease ):Hep B/C, ETOH, Autoimmune, PBC, PSC, Crytogenic/NASH, Secondary Biliary Cirrhosis) Malignancy: a) Primary: HCC. uncommon forms; Fibrolamellar cancer, epitheliod hemangioendothelioma, hepatoblastoma b) Metastatic: Carcinoid, Islet Cell Misc: Polycystic Liver Disease, Budd-Chiari Syndrome ( with cirrhotic changes ).

Relative Contraindications to OLT

      Advanced age Cholangiocarcinoma (undergoing study with strict protocols to include chemotherapy, radiation and staging surgery) HIV without AIDS ( under NIH protocol study ) HIV+HCV with less survival compared to HIV alone. Portal Vein Thrombosis Psychological Instability BMI > 40 Ahmed A: Clin Liver Dis 11(2007):227-247

2007 UNOS

      Primary oxaluria: MELD: 16 Familial Amyloidosis (FAP): MELD: 16 Polycytic Liver /Kidney Disease: MELD: 16 Hepatopulmonary Syndrome: MELD: 20 Post TIPS, persistant ascites and/or pleural effusion: MELD: 16 Post TIPS Portal Hypertensive Bleeding: MELD: 20 ( >4 Unit PRBC/month)

   External drainage of a biliary stricture for more than 30 days: MELD: 20 3 episodes of Biliary Sepsis in 6 months: MELD: 20 Urea cycle defect : PELD: 20

JHH Adult Liver Transplant Cardiac Evaluation Protocol

• • Low Risk Candidates: Intermediate and High Risk Candidates: - High Risk: - Intermediate Risk: . Porto Pulmonary Hypertension (PPHTN ) . Hepato Pulmonary syndrome ( HPS ) . Annual Cardiovascular Assessment

• • • • • Low Risk Candidates: 18-45 without risk factors will have a standard resting 2D ECHO >45 without risk factors will undergo resting 2D ECHO and dobutamine stress echocardiography to evaluate for obstructive coronary disease.

(RVSP) as an estimate of pulmonary artery pressure (TAPSE) as an index of right ventricular function ECHO Bubble Study if RA O2 SAT <93% or signs of platypnea or orthodeoxia.

• • • • • • • • • Intermediate Risk: History of coronary artery disease History of compensated or prior heart failure Diabetes Renal Insufficiency Peripheral arterial disease Family history of premature coronary artery disease or sudden death Dyslipidemia NASH Heavy prior tobacco use

Cardiovascular issues post LTx

• • • • • • Pre Tx Troponin 1 > 0.07, DM, CV Disease associated, with post LTX Cardiac events.

Happens 25-70% 7% mortality during early and medium term 3 rd cause of Death Detailed pre-LTx Cardiovascular evaluation is warranted. CAC (Coronary Artery Calcium) Score has been anecdotal.

Pulmonary Hypertension:

• Confirmatory right heart catheterization will be performed in patients with a RVSP >50mmHg on resting echocardiogram .

Annual Cardiovascular Assessment Patients seen by cardiology for preoperative consultation , after 12 months , will have a repeat resting ECHO and follow-up with the cardiology Patients not needed to be seen by cardiology previously, a resting ECHO

Prevelance 5-32%

Post Transplant Metabolic Syndrome

Definition of Metabolic Syndrome (MT)

• • • • • BMI >30 or waist >102 cm in men and 88 cm among women.

Fasting Plasma Glucose >100 mg/dl BP > 130/85 mmHg Triglycerides > 150 mg/dl HDL < 40 mg/dl in men and < 50 mg/dl in women

Waiting list candidates as of today 8:15pm

All Kidney Pancreas Kidney/Pancreas Liver Intestine Heart Lung Heart/Lung Based on OPTN data as of 05/06/2011

The Organ Procurement and Transplantation Network (OPTN)

111,022 88,585 1,361 2,202 16,152 264 3,133 1,772 67

Organ Bağış Oranları

(milyon nüfus başına)

Cadaveric Donor Living Donor 2010 -TR

209 70.000.000

486 70.000.000

3 1.000.000

7 1.000.000

Cadaveric Donor Living Donor 2010-US

6009 20 300.000.000 1.000.000

282 0.94

300.000.000 1.000.000

http://optn.transplant.hrsa.gov/latestdata/rptData.asp

Organ Procurement and Transplantation Network Liver Kaplan-Meier Patient Survival Rates For Transplants Performed : 1997 - 2004 Based on OPTN data as of May 6, 2011 Region U.S.

U.S.

U.S.

U.S.

U.S.

U.S.

Donor Type Cadaveric Living Cadaveric Living Cadaveric Living Years Post Transplant 1 Year 1 Year 3 Year 3 Year 5 Year 5 Year Number Functioning / Alive 13067 823 12836 1070 10424 510 Survival Rate 86.3

90.1

78.0

82.5

72.0

77.7

95% Confidence Interval (85.7, 86.8) (88.1, 92.1) (77.4, 78.6) (80.5, 84.6) (71.3, 72.7) (74.6, 80.8)

2002 2003 2004 2005 2006 2007 2008 2009 2010 TOPLAM Donör Canlı

77 33 133 200 205 264 390 364 486

2,152 1,344 Kad.

82 53 112 124 114 209 212 229 209

KARACİĞER 3,496 Toplam Sayı

159 86 245 324 319 473 602 593 695

NEGATIVE

Cardiopulmonary Exercise Testing

Stuart D. Russell, MD Associate Professor of Medicine Chief, Heart failure and Transplantation Johns Hopkins Hospital No disclosures related to this talk

Peak Exercise Tolerance as Measured by Peak Oxygen Consumption

• VO 2 •  2 diff

Masters-level Marathoner Competitive Inline Skater NFL (23 yo, All-Pro) Running Back Healthy (20 yo)- untrained Cardiac Transplant- untrained 10 Heart Failure- untrained 25 40 55 Peak VO 2 (mL/kg/min) 70

CPX and liver transplant Univariate analysis

AT ml/kg/min Blood FFP Donor age Β coefficient 1.200

-0.095

-0.006

-0.060

Odds ratio (95% CI) 3.35 (1.37-8.19) 0.91 (0.85-0.97) 0.94 (0.89-1.0) 0.94 (0.88-1.0) P value < 0.001

0.002

0.02

0.06

Multivariate analysis

AT ml/kg/min 1.344

3.84 (1.17-12.58)

Used AT > 9.1 ml/kg/min based on optimal ROC Prentis et al Liver Transpl 2012;18:152

0.02

Summary Cardiac complications are common post liver transplant and are one of the most common causes of mortality and morbidity Cardiopulmonary stress testing is a non invasive way of assessing risk of both morbidity and mortality post liver transplant Small studies have demonstrated that rehab can increase peak VO 2 in patients with liver disease Unclear if exercise training pre transplant will alter outcomes

HEH

Hepatic epithelioid hemangioendothelioma