Liver Transplantation

Section: Surgery
Effective Date: January 01, 2020
Revised Date: November 14, 2019
Last Reviewed: November 14, 2019

Description

Liver transplantation is currently the treatment for individuals with end-stage liver disease. Liver transplantation may be performed with a cadaver or living donor.  Individuals are prioritized for transplant by mortality risk and severity of illness criteria developed by the Organ Procurement and Transplantation Network and the United Network of Organ Sharing.

Criteria

A liver transplant using a cadaver or living donor may be considered medically necessary for carefully selected individuals who meet the following criteria:

  • A model of end-stage liver disease MELD score greater than 10; and
  • Are approved for transplant by the United Network for Organ Sharing (UNOS) Regional Review Board; and
  • Meets the transplanting institution’s selection criteria.
    • In the absence of the transplanting institution’s criteria, liver transplantation may be considered medically necessary for ANY ONE of the following conditions (including, but not limited to):
      • Cholestatic liver diseases
        • Biliary atresia; or
        • Primary biliary cirrhosis; or
        • Primary sclerosing cholangitis with development of secondary biliary cirrhosis; or
      • Familial amyloid polyneuropathy; or
      • Hepatocellular diseases:
    • Alcoholic liver disease; or
    • Alpha-1 antitrypsin deficiency; or
      • Cryptogenic cirrhosis; or
      • Hemochromatosis; or
    • Idiopathic autoimmune hepatitis; or
    • Non-alcoholic steatohepatitis; or
    • Protoporphyria; or
      • Viral hepatitis (either A, B, C, or non-A, non-B); or
      • Wilson’s disease; or
    • Hepatopulmonary syndrome when the following selection criteria are met:
    • Arterial hypoxemia (PaO2 less than 60 mm Hg or AaO2 gradient greater than 20 mm Hg in supine or standing position); and
    • Chronic liver disease with non-cirrhotic portal hypertension; and
    • Intrapulmonary vascular dilatation (as indicated by contrast-enhanced echocardiography, technetium-99 macroaggregated albumin perfusion scan, or pulmonary angiography); or
    • Hepato-renal Syndrome:
      • All other causes for renal failure have been excluded; and
      • GFR <40ml/min; or
    • Inborn errors of metabolism; or
    • Malignancies:
      • Cholangiocarcinoma, hilar, unresectable; or
      • Cholangiocarcinoma, intrahepatic (i.e., cholangiocarcinomas confined to the liver); or
      • Epithelioid hemangioendotheliomas (EHE) or;
      • Fibrolamellar hepatocellular, large, unresectable; or
      • Hepatoblastomas in children when all of the following criteria are met:
    • Individual is not a candidate for subtotal liver resection; and
    • Individual meets UNOS criteria for tumor size and number; and
    • There is no identifiable extrahepatic spread of tumor to surrounding lungs, abdominal organs, bone or other sites. (Note: spread of hepatoblastoma to veins and lymph nodes does not disqualify an individual for coverage of a liver transplant.); or
    • Hepatocellular carcinoma, primary, confined to the liver when all of the following criteria are met:
      • Any lung metastases have been shown to be responsive to chemotherapy; and
      • Individual is not a candidate for subtotal liver resection; and
      • Individual meets UNOS criteria for tumor size and number; and
      • There is no identifiable extrahepatic spread of tumor to surrounding lymph nodes, abdominal organs, bone or other sites; and
      • There is no macrovascular involvement; or
    • Neuroendocrine tumors, metastatic (carcinoid tumors, apudomas, gastrinomas, glucagonomas) in individuals with severe symptoms and with metastases restricted to the liver, who are unresponsive to adjuvant therapy after aggressive surgical resection including excision of the primary lesion and reduction of hepatic metastases; or
    • Polycystic disease of the liver; or
    • Portopulmonary Hypertension (pulmonary hypertension associated with liver disease or portal hypertension) in individuals with a mean pulmonary artery pressure by catheterization of less than 35 mm Hg; or
    • Toxic reactions (i.e., fulminant hepatic failure due to mushroom poisoning, acetaminophen (Tylenol) overdose, etc.); or
    • Trauma; or
    • Vascular disease
      • Budd-Chiari syndrome; or
      • Veno-occlusive disease.

In ANY of the following situations, liver transplantation is considered experimental/investigational (E/I) and therefore non-covered because the safety and and/or effectiveness of this service cannot be established by the available published peer-reviewed literature:

  • In individuals with extrahepatic cholangiocarcinoma; or
  • In individuals with intrahepatic cholangiocarcinoma; or
  • In individuals with neuroendocrine tumors metastatic to the liver who have not met the medical necessity criteria identified above.

Liver transplantation is considered not medically necessary for the following:

  • Individuals with hepatocellular carcinoma that has extended beyond the liver; or
  • Individuals with ongoing alcohol and/or drug abuse.*
    • *Note:
      • Chronic Alcoholic Liver Disease
        The following recommendations should be taken into consideration for those individuals diagnosed with “Chronic” alcoholic liver disease and are, most likely, on a liver transplant waiting list before a liver transplant is considered:
        • Abstinence of substance abuse for a minimum of six (6) months; and
        • Participation in a substance abuse/rehabilitation program, either through the facility transplant program or at a substance abuse clinic; and
        • Consistent negative results of random blood or urine drug testing.
  • Acute Alcoholic Liver Disease
    • In acute alcoholic liver disease, there will be some individuals who will not respond to or will continue to deteriorate despite medical therapy. In these cases, immediate intervention is expected to stabilize the individual, even if that intervention is immediate liver transplantation. It is also expected, that alcohol consumption will be addressed in the post liver transplant care when appropriate.

Potential Contraindications

Potential contraindications for liver transplant recipients include, but are not limited to ANY of the following:

  • Acquired immune deficiency syndrome (AIDS) diagnosis based on Center for Disease Control (CDC) definition of T cell (CD4) count, (200 cells/mm³) unless ALL of the following are noted:
    • CD4 count greater than 200 cells/mm³ for greater than six (6) months; and
    • HIV-1 ribonucleic acid (RNA) undetectable; and
    • On stable anti-retroviral therapy greater than three (3) months; and
    • No other complications from AIDS (e.g., opportunistic infection, including aspergillus, tuberculosis, coccidioidomycosis, resistant fungal infections, Kaposi’s sarcoma or other neoplasm); and
    • Meeting all other criteria for liver transplantation; or
  • Contraindications to immunosuppressive medications that could endanger the individual; or
  • End-stage disease, irreversible, other than liver disease; or
  • Infection, systemic, making immunosuppression unsafe, including chronic infection; or
  • Malignancy, current, including metastatic cancer not identified in any of the medical necessity criteria above; or
  • Malignancy, history of, with a moderate or high-risk of recurrence; or
  • Noncompliance, which places the organ at risk by not adhering to medical recommendations; or
  • Psychological conditions or chemical dependency affecting the ability to adhere to therapy; or
  • Systemic disease that could be exacerbated by immunosuppression.

Retransplantation

Liver re-transplantation may be considered medically necessary in individuals with:

  • Chronic rejection; or
  • Hepatic artery thrombosis; or
  • Ischemic type biliary lesions after donation after cardiac death; or
  • Primary graft non-function; or
  • Recurrent non-neoplastic disease-causing late graft failure.

In addition to the above criteria and subject to the discretion of the transplant center, a Hepatitis C Virus (HCV) positive donor organ maybe considered an acceptable organ option for an HCV negative adult recipient 18 years of age or older.

Liver transplantation or re-transplantation is considered not medically necessary for any other indications.

Procedure Codes

47133 47135 47140 47141 47142 47143 47144
47145 47146 47147 47399

Diagnosis Codes

Covered diagnosis for procedure codes 47133, 47135, 47140, 47141, 47142, 47143, 47144, 47145, 47146, 47147, and 47399:

B15.0 B15.9 B16.0 B16.1 B16.2 B16.9 B17.10
B17.11 B17.8 B17.9 B18.0 B18.1 B18.2 B18.8
B18.9 B19.0 B19.10 B19.11 B19.20 B19.21 B19.9
B25.1 B66.1 B66.3 C22.0 C22.1 C22.2 C22.3
C22.4 C22.7 C22.8 C22.9 E70.0 E70.1 E70.20
E70.21 E70.29 E70.30 E70.310 E70.311 E70.318 E70.319
E70.320 E70.321 E70.328 E70.329 E70.330 E70.331 E70.338
E70.339 E70.39 E70.40 E70.41 E70.49 E70.5 E70.8
E70.9 E71.0 E71.110 E71.111 E71.118 E71.120 E71.121
E71.128 E71.19 E71.2 E71.30 E71.310 E71.311 E71.312
E71.313 E71.314 E71.318 E71.32 E71.39 E71.40 E71.41
E71.42 E71.43 E71.440 E71.448 E71.50 E71.510 E71.511
E71.518 E71.520 E71.521 E71.522 E71.528 E71.529 E71.53
E71.540 E71.541 E71.542 E71.548 E72.00 E72.01 E72.02
E72.03 E72.04 E72.09 E72.10 E72.11 E72.12 E72.19
E72.20 E72.21 E72.22 E72.23 E72.29 E72.3 E72.4
E72.50 E72.51 E72.52 E72.53 E72.59 E72.81 E72.89
E72.9 E74.00 E74.01 E74.02 E74.03 E74.04 E74.09
E74.10 E74.11 E74.12 E74.19 E74.20 E74.21 E74.29
E74.31 E74.39 E74.4 E74.8 E74.9 E78.0 E78.01
E78.1 E78.2 E78.3 E78.41 E78.49 E78.5 E78.6
E78.70 E78.79 E78.81 E78.89 E78.9 E80.0 E80.1
E80.20 E80.21 E80.29 E83.00 E83.01 E83.09 E83.10
E83.110 E83.111 E83.118 E83.119 E83.19 E85.0 E85.1
E85.2 E85.3 E85.4 E85.81 E85.82 E85.89 E85.9
E88.09 E88.1 E88.2 E88.3 E88.40 E88.41 E88.42
E88.49 E88.89 E88.9 E88.01 G63 I74.8 I82.0
K70.2 K70.30 K70.31 K70.40 K70.41 K70.9 K71.0
K71.10 K71.11 K71.2 K71.3 K71.4 K71.50 K71.51
K71.6 K71.7 K71.8 K71.9 K72.00 K72.01 K72.10
K72.11 K72.90 K92.91 K73.2 K73.9 K74.0 K74.3
K74.4 K74.5 K74.60 K74.69 K75.2 K75.3 K75.4
K75.81 K76.0 K76.2 K76.3 K76.4 K76.5 K76.7
K76.89 K77 K83.0 K83.1 K83.5 K83.8 Q44.1
Q44.2 Q44.3 Q44.4 Q44.5 Q44.6 Q44.7 S36.112A
S36.112D S36.112S S36.113A S36.113D S36.113S S36.114A S36.114D
S36.114S S36.115A S36.115D S36.115S S36.116A S36.116D S36.116S
S36.118A S36.118D S36.118S S36.119A S36.119D S36.119S T86.40
T86.41 T86.42 T86.43 T86.49 Z52.6

Professional Statements and Societal Positions Guidelines

American Association for the Study of Liver Diseases et al

The American Association for the Study of Liver Diseases and the American Society of Transplantation (2013) issued joint guidelines on evaluating patients for liver transplant. These guidelines indicated liver transplantation for severe acute or advanced chronic liver disease after all effective medical treatments have been attempted. The formal evaluation should confirm the irreversible nature of the liver disease and lack of effective alternative medical therapy.

The guidelines also stated that liver transplant is indicated for the following conditions:

  • Acute liver failure complications of cirrhosis.
  • Liver-based metabolic condition with systemic manifestations:
    • α1-Antitrypsin deficiency
    • Familial amyloidosis
    • Glycogen storage disease
    • Hemochromatosis
    • Primary oxaluria
    • Wilson disease
  • Systemic complications of chronic liver disease.
  • The guidelines also included 1-A recommendations (strong recommendation with high-quality evidence) for a liver transplant that:
  • “Tobacco consumption should be prohibited in LT [liver transplant] candidates.”
  • “Patients with HIV infection are candidates for LT if immune function is adequate and the virus is expected to be undetectable by the time of LT.”
  • “LT candidates with HCV [hepatitis C virus] have the same indications for LT as for other etiologies of cirrhosis.”
  • Contraindications to liver transplant included:
  • “MELD [Model for End-stage Liver Disease] score < 15
  • Severe cardiac or pulmonary disease
  • AIDS
  • Ongoing alcohol or illicit substance abuse
  • Hepatocellular carcinoma with metastatic spread
  • Uncontrolled sepsis
  • Anatomic abnormality that precludes liver transplantation
  • Intrahepatic cholangiocarcinoma
  • Extrahepatic malignancy
  • Fulminant hepatic failure
  • Hemangiosarcoma
  • Persistent noncompliance
  • Lack of adequate social support system.”

The American Association for the Study of Liver Diseases, the American Society of Transplantation, and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition issued joint guidelines on the evaluation of the pediatric patients for liver transplant in 2014. The guidelines stated that “disease categories suitable for referral to a pediatric LT program are similar to adults: acute liver failure, autoimmune, cholestasis, metabolic or genetic, oncologic, vascular, and infectious. However, specific etiologies and outcomes differ widely from adult patients, justifying independent pediatric guidelines.” The indications listed for liver transplantation included biliary atresia, Alagille syndrome, pediatric acute liver failure, hepatic tumors, HCC, hemangioendothelioma, cystic fibrosis-associated liver disease, urea cycle disorders, immune-mediated liver disease, along with other metabolic or genetic disorders.

National Comprehensive Cancer Network

The National Comprehensive Cancer Network (NCCN) guidelines on hepatobiliary cancers (v.3.2019) recommend referral to a liver transplant center or bridge therapy for patients with HCC meeting United Network of Organ Sharing criteria of a single tumor measuring 2 to 5 cm, or 2 to 3 tumors 3 cm or less with no macrovascular involvement or extrahepatic disease. Patients should be referred to the transplant center. Patients should be referred to the transplant center before the biopsy. In patients who are ineligible for transplant and in select patients with Child-Pugh class A or B liver function with tumors that are resectable, NCCN indicates resection is the preferred treatment option; locoregional therapy may also be considered. Patients with unresectable HCC should be evaluated for liver transplantation; if the patient is a transplant candidate, then referral to a transplant center should be given or bridge therapy should be considered. NCCN guidelines on hepatobiliary cancers also indicate that. These are level 2A recommendations based on lower-level evidence and uniform consensus.

The NCCN guidelines on neuroendocrine tumors (v.3.2019) indicate that liver transplantation for neuroendocrine liver metastases is considered investigational despite “encouraging” 5-year survival rates.

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