• Cirrhosis is the common histological outcome of chronic liver disease resulting in cell necrosis and altered liver structure and function. It is characterized by
  • Fibrosis
  • Regenerative nodules of hepatic cells
  • Impaired metabolic and synthetic function
  • Increased hepatic resistance to blood flow
• Portal hypertension is the hallmark of decompensated cirrhosis, recognizable by ascites formation, venous collateral development, and episodes of hepatic encephalopathy.

Hepatitis C 26%; alcohol related 21%; hepatitis C plus alcohol 15%; hepatitis B 15%; cryptogenic causes 18% (70% are nonalcoholic fatty liver disease [NAFLD], an increasingly recognized cause of cirrhosis); miscellaneous 5% (autoimmune hepatitis, cholestatic liver diseases, cholangitis, hemochromatosis, Wilson's disease, alpha-1 antitrypsin deficiency, drug-induced, right-sided heart failure, Budd–Chiari)

• Impaired metabolic and synthetic function
  • Albumin. Important for maintaining oncotic pressure in capillaries; affects drug distribution and elimination by its binding.
  • Coagulation factors. Vitamin K-derived factors (II, VII, IX, X), protein C, protein S, and antithrombin.
  • Cytochrome P450 enzymes. Decreased drug metabolism.
• Portal hypertension results from impaired hepatic vascular resistance together with increased mesenteric blood flow. This may lead to
  • Ascites. Describes the accumulation of fluid in the peritoneal cavity. Pathogenesis is controversial and probably different at various phases of the disease. Portal hypertension, splanchnic vasodilation, hypoalbuminemia, and increased levels of sodium retentive hormones contribute to capillary leakage.
  • Venous collateral development. Results in esophageal varices and acute bleeding as well as hypersplenism with associated leukopenia and/or thrombocytopenia.
  • Hepatic encephalopathy. Results from elevated circulating neurotoxic gut toxins (due to portocaval shunting and decreased hepatic metabolism). Patients present with depressed levels of consciousness, personality changes, and intellectual impairment.

• Maintain perfusion and oxygenation of the liver to minimize postoperative deterioration of liver function.
• Altered pharmacology can result in sensitivity to sedatives, hepatic encephalopathy, and impaired pulmonary or cardiovascular function. Additionally, altered pharmacokinetics includes an increased volume of distribution (Vd), impaired cytochrome P450, and hypoalbuminemia.
• Perioperative mortality is severely increased in cirrhotic patients.

Fatigue, nausea, decreased appetite, itching, right upper quadrant pain, abdominal distension, jaundice, dark urine, light stools, and easy bruising.

• Medical management depends on the etiology of initial liver disease. Antiviral treatment for hepatitis B and C, abstinence from alcohol, steroids and azathioprine for autoimmune hepatitis.
• Transjugular intrahepatic portosystemic shunts (TIPS) and large volume paracentesis are considered in medically refractory ascites.
• Endoscopic variceal ligation and octreotide infusion for acute bleeding.

• Ascites: Sodium restriction (first line), diuretics (second line).
• Spontaneous bacterial peritonitis: Cephalosporins usually indicated.
• Hepatic encephalopathy: Lactulose and poorly absorbable antibiotics like rifaximin, neomycin, and metronidazole.
• Hepatorenal syndrome: Splanchnic vasoconstrictors (midodrine, octreotide) and volume expansion with albumin.

• Cardiovascular (1) [A]
  • Hyperdynamic circulation: Decreased systemic vascular resistances, arterial BP, and increased heart rate lead to a high cardiac output (HCO) status. Increased CO is unequally distributed with a preferential flow toward the visceral compartment. Peripheral arteries may be dilated or constricted in advanced disease.
  • Autonomic dysregulation with blunted sympathetic and parasympathetic responses.
  • Cirrhotic cardiomyopathy: Impaired cardiac contractility, systolic and diastolic dysfunction, and ECG abnormalities (e.g., prolonged QT) may lead to ventricular arrhythmias and sudden cardiac death. Typically improves after liver transplantation.
  • Diagnostic tools include a 12 lead ECG (QTc, QT dispersion); resting or stress ECG (EF, volumes, fractional shortening, wall motion, E/A ratio); exercise ECG (exercise capacity, oxygen consumption, pressure × heart rate product).
• Hepatorenal syndrome (2) [A]
  • Acute or subacute renal failure in advanced cirrhosis is caused by vasoconstriction of renal arteries and is characterized by oliguria/anuria and a creatinine >1.5 mg/dL. Kidneys show only minimal histological changes.
  • Type 1: Rapid course in <2 weeks mandates dialysis and liver transplantation.
  • Type 2: Slowly progressing course, usually responds to volume expansion.
• Hepatopulmonary syndrome
  • Seen in advanced cirrhosis
  • Hypoxemia results from pulmonary vasodilation that causes ventilation/perfusion mismatching and intrapulmonary shunting.
  • Clinical findings include platypnea and orthodeoxia.
  • Diagnosis is via contrast ECG with the presence of bubbles in the left atrium four or more beats after the appearance in the right atrium.
  • Portopulmonary hypertension is the coexistence of portal and pulmonary hypertension. Mean survival is 15 months in the absence of a liver transplant.
• Hemostasis (3) [A]
  • Hemostatic balance is unstable and can tip either way with bleeding or thrombosis.
  • Hypocoagulability and bleeding tendencies are due to the decreased liver synthesis of coagulation factors and thrombocytopenia.
  • Hypercoagulability can also be present as suggested by frequent portal vein thrombosis.
  • Cirrhosis "rebalanced hemostasis" can result from decreased synthesis of anticoagulants like antithrombin III, Protein C and S; may result in normal thrombin generation in vivo.
  • Traditional coagulation tests such as PT, PTT, and platelet count do not accurately reflect the state of hemostasis.
  • Prophylactic correction of abnormal laboratory tests to prevent intraoperative bleeding does not have scientific evidence.

• Acute hepatitis (transaminases >10 times normal values) (4) [A]
• Child–Pugh type C
• MELD score >14

• Child–Turcotte–Pugh (CTP) and MELD classification systems were initially used for predicting mortality after portocaval shunt/TIPS placement. They are now used to predict surgical risk in patients with liver disease (4) [A].
  • Child's A (5–6 points): Postoperative mortality 10%; Child's B (7–9 points): Postoperative mortality 31%; Child's C (10–15 points): Postoperative mortality 76%.
• MELD score: Complex logarithmic formula incorporating, bilirubin, INR, and serum creatinine (scale 6–40).
  • Score <8: Postoperative mortality 5.7%; Score >20: Postoperative mortality >50%

Admission to an intensive care unit (ICU) may be indicated to monitor and support circulatory and respiratory function after intermediate/high risk procedure.

Hepatology and hematology consultation may be considered for management of coagulopathy.

• Postoperative liver failure
• Hemostatic derangements
• Postoperative delirium
• Pulmonary edema
• Pleural effusion

• Liver Transplant
• Thromboelastogram

• 571.2 Alcoholic cirrhosis of liver
• 571.5 Cirrhosis of liver without mention of alcohol

• K70.30 Alcoholic cirrhosis of liver without ascites
• K74.60 Unspecified cirrhosis of liver

Phoebe Lee , MD

andrea Vanucci , MD, DEAA