FULMINANT HEPATIC FAILURE (FHF)

Definition: The acute onset of liver failure in a patient without a prior history of liver disease.

Key Points:

• Although there are multiple causes of FHF, the most common cause in the USA is Acetaminophen toxicity, which is the least common etiology of FHF requiring liver transplantation (as the vast majority of patients recover)

• Idiosyncratic hepatitis (Non A, B, C, D, E, G or Hepatitis “X”) is the most common cause of FHF requiring transplantation

• Predicting prognosis in FHF is critical:

  • If it is apparent based on patients clinical course or clinical parameters (Kings College Criteria) that recovery of liver function will NOT occur, liver transplantation must be considered

  • Kings College Criteria has a better “predictive value” (patients who meet criteria go on to require liver transplantation) in the setting of Acetaminophen toxicity rather than Non-Acetaminophen toxicity

• A multi-system approach needs to be adopted to maximize a successful outcome

  • Monitor mentation and if necessary, Intracranial Pressure (ICP) and Cerebral Perfusion Pressures (CPP) in patients with FHF and coma. In patients with suboptimal ICP and CPP which can not be improved with therapy, neurologic recovery following OLT is unlikely

    • Non-invasive assessment of CPP/ICP via transcranial Doppler will replace invasive monitors in the near future

  • Be vigilant in preventing/diagnosing infection! Sepsis is a frequent cause of death in patients with FHF. Prophylactic antibiotic coverage is mandatory.

  • Avoid hemodialysis is patients with FHF as this will increase ICP. Continuous hemofiltration or continuous dialysis is preferred (CVVHD)

  • N-Acetyl Cysteine may be used in patients with FHF irrespective of the cause of FHF (i.e., not only Acetaminophen toxicity) due to improvement in hemodynamics and oxygen transport, and minimal side effects.

    • May be given IV (with appropriate consent) if patients can not tolerate PO

  • “Liver Replacement therapy” by liver transplant is an accepted therapy in patients with FHF who do not exhibit recovery of liver function.

  • Future therapies/therapy undergoing investigation include

    • Bio-artificial liver (BAL)

    • Extracorporeal liver assist device (ELAD)

    • Hepatocyte transplantation

    • Xenotransplantation

  • Living donor liver transplantation, from an Adult donor to a Pediatric or Adult recipient is a potential option in cases of FHF, particularly in the setting of limited cadaveric organs. At present, this is approved for Adult to Pediatric transplants.