Cirrhosis

Summary
Cirrhosis is a condition caused by chronic damage to the liver, most commonly due to excessive alcohol consumption, nonalcoholic fatty liver
disease, or hepatitis C infection. Other causes may include inflammatory or metabolic diseases, such as primary biliary
cirrhosis or hemochromatosis. Cirrhosis is characterized by hepatic parenchymal necrosis and an inflammatory response to the underlying
cause. Subsequent hepatic repair mechanisms lead to fibrosis and abnormal tissue architecture, which impair liver function. Patients can
present with a range of symptoms, including ascites, hepatosplenomegaly; and skin manifestations of cirrhosis, such as jaundice, spider
angioma, and/or palmar erythema. Men may further display signs of feminization (e.g., gynecomastia, hypogonadism). In severe cases,
accumulation of toxic metabolites or involvement of further organs can lead to complications such as hepatic encephalopathy or hepatorenal
syndrome. Laboratory tests show signs of hepatocyte damage (e.g., elevated liver enzymes, hyperbilirubinemia) or impaired hepatic synthetic
function (e.g., prolonged prothrombin time, low albumin). Abdominal ultrasonography typically shows shrunken,
heterogeneous liver parenchyma with a nodular surface. A biopsy is the method of choice for confirming the diagnosis. However, it is usually
only performed if previous diagnostic modalities were inconclusive. Management consists of treatment of the underlying disease (e.g., avoiding
toxic substances, antiviral drugs), adequate caloric intake, and medication for treating complications (e.g., spironolactone for ascites). In cases
of decompensated cirrhosis, interventional procedures (e.g., paracentesis to drain ascites) may be used to alleviate symptoms or bridge the
time until liver transplantation is possible.

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Epidemiology
 Prevalence: approx. 0.27% in US adults [1]
 Sex: ♂ > ♀ (2:1) [2]
 Mortality [2]
o Responsible for approx. 1–2% of all deaths in the US (12th leading cause of death)
 o Most deaths occur in the fifth to sixth decade of life.
Epidemiological data refers to the US, unless otherwise specified.
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Etiology
 Hepatotoxicity
o Long-standing alcohol use disorder (one of the two most common causes of chronic liver disease in the US)
o Medications (e.g., acetaminophen, amiodarone, chemotherapy drugs such as methotrexate)
o Ingestion of aflatoxin (produced by Aspergillus) [3]
o Industrial chemicals such as tetrachloromethane 
 and various pesticides
 Inflammation (hepatitis)
o Chronic viral hepatitis: hepatitis B, hepatitis D, and hepatitis C (most common cause of cirrhosis in the United States)
o Primary biliary cirrhosis
o Primary sclerosing cholangitis
o Autoimmune hepatitis
o Parasitic infections (e.g., schistosomiasis, leishmaniasis, malaria) 
o IgG4 cholangiopathy
 Metabolic disorders
o Nonalcoholic steatohepatitis (NASH) 
o Hemochromatosis
o Wilson disease
o α1-antitrypsin deficiency
o Porphyrias
o Glycogen storage disease
o Cystic fibrosis
o Hereditary fructose intolerance 
 Hepatic vein congestion or vascular anomalies
o Budd-Chiari syndrome 
o Cardiac cirrhosis (congestive hepatopathy)
o Osler-Weber-Rendu syndrome 
 [4]
 Cryptogenic cirrhosis: cirrhosis of uncertain etiology despite adequate diagnostical efforts
Cryptogenic cirrhosis is a diagnosis of exclusion and should only be considered after a complete patient evaluation has ruled out all other
possible causes of cirrhosis.
Hepatitis C, alcoholic liver disease, and NASH are the most common causes of cirrhosis in the US.
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Pathophysiology
 Cirrhosis is characterized by irreversible diffuse fibrosis of the liver (the final common pathway for chronic liver diseases).
 Pathogenesis is multifactorial.
o Different liver cells and cytokines are involved in the activation and progression of liver fibrosis.
o Cytokine-mediated activation of hepatic stellate cells has been identified as a central element for developing fibrosis.
 The following three mechanisms have been described for all types of liver cirrhosis: [5]
o Degeneration and necrosis of hepatocytes
 Activated Kupffer cells destroy hepatocytes, activate hepatic stellate cells, and promote inflammation.
 Inflammatory cytokines (e.g., TGF-β, PDGF) → hepatocyte apoptosis and hepatic stellate cell activation
→ excess collagen production 
o Fibrotic tissue and regenerative nodules replace the liver parenchyma
 Hepatocyte destruction triggers repair mechanisms → excess formation of connective tissue (fibrosis)
 Excessive connective tissue in periportal zone and centrilobular zone → regenerative nodules and
fibrous septa → compression of hepatic sinusoids and venules → ↑ portal vein hydrostatic
pressure → intrasinusoidal hypertension → ↓ functional sinusoids
o Loss of liver function: sinusoidal capillarization → loss of fenestration and scar tissue formation→ impaired
substrate exchange → loss of normal liver function (exocrine and metabolic)

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Clinical features
Clinical manifestations of liver cirrhosis generally represent the severity of liver disease. [6]
 Nonspecific features
o Patients are often initially asymptomatic.
o Fatigue, malaise, anorexia, and weight loss
 Dermal features
o Pruritus
o Jaundice 
o Telangiectasia: most commonly spider angiomata 
 
o Caput medusae: paraumbilical dilation of subcutaneous veins 
o Palmar erythema (plantar erythema also possible)
o Petechiae and purpura 
o Generally dry and atrophic
o White nails with ground-glass opacity (also known as “Terry nails”) 
o Nail clubbing 
 
o Lacquered lips, smooth red tongue
 Abdominal features
o Nausea, vomiting
o Hepatomegaly (possibly causing dull RUQ pain) 
o Splenomegaly 
o Ascites (abdominal fluid buildup due to hypoalbuminemia)
 Hormonal disorders
o Hyperestrogenism [7]
 Changes in the hepatic metabolization of sex hormones cause an imbalance in the estrogen-androgen ratio, resulting in a
marked increase in systemic estrogen levels. 
 In men, increased estrogen levels cause feminization. 
 Gynecomastia
 Hypogonadism (e.g., testicular atrophy, reduced libido, erectile dysfunction, infertility)
  Decreased body hair (e.g., loss of chest hair, a female pattern of pubic hair distribution)
 In women, a massive increase in estrogen can cause amenorrhea.
 Other
o Asterixis 
o Fetor hepaticus: bad breath with a characteristic sweet, pungent smell caused by an accumulation of dimethyl sulfide 
o Dupuytren contracture
o Peripheral edema 
o See also “Clinical features” in “Portal hypertension.”
 Specific clinical features due to rare etiologies
o Hemochromatosis: dark, bronze skin color, and diabetes (bronze diabetes)
o Wilson disease
 Neurological/psychiatric symptoms (parkinsonism and personality changes)
 Indirect hyperbilirubinemia due to hemolysis.
o Alpha-1 antitrypsin deficiency: lung emphysema before 50 years of age [8]

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Diagnostics
Laboratory tests [6]
Complete blood count (CBC)
 Anemia
o Microcytic: due to chronic blood loss
o Macrocytic: due to vitamin B12 deficiency or folic acid deficiency 
 Thrombocytopenia
o Caused by the following factors: 
 ↑ Hepatic and splenic sequestration of thrombocytes (portal hypertension leads to splenomegaly with hypersplenism)
  ↓ Thrombopoietin production by the liver
Liver function tests
 Parameters of hepatocyte damage
o ↑ Transaminases (AST, ALT) 
 ALT > AST: present in most liver diseases
 AST > ALT: indicative of alcoholic liver disease
 In alcoholic hepatitis, AST generally does not exceed 500 U/L.
 AST elevation in nonalcoholic liver disease suggests progression towards cirrhosis.
 If the reference range is > 1,000 U/L, consider differential diagnoses (e.g., acetaminophen toxicity,
viral hepatitis, autoimmune hepatitis).
o Initially normal/↑ bilirubin 
o ↑ Gamma-glutamyl transpeptidase (GGT) 
o ↑ Alkaline phosphatase 
o ↑ Glutamate dehydrogenase (GDH) 
o ↑ Ammonia 
 Parameters of impaired hepatic synthesis
o ↑ Prothrombin time (↑ INR): due to decreased production of coagulation factors 
o ↓ Total protein (↓ albumin)
o ↓ Plasma cholinesterase (CHE) 
[9]
 
 A circulating enzyme produced by the liver that can hydrolyze several choline-based esters
 Involved in the breakdown of muscle relaxants (e.g., succinylcholine)
 Decreased serum levels can result from cirrhosis or organophosphate pesticide exposure (occupational or accidental).
 Other (rarer) tests
o AST-to-platelet-ratio index (APRI) 
 APRI > 1 suggests cirrhosis
 APRI < 0.5 no cirrhosis. 
o Serum protein electrophoresis 
 ↓ Albumin band
 ↑ Gamma band 
 Alpha-1, alpha-2, and beta globulin fractions are unchanged 
Other laboratory tests
Additional laboratory studies can be performed to determine the etiology of chronic liver disease.
 Hepatitis: anti-HBs, anti-HBc, HBsAg, and anti-HCV
 α1 antitrypsin deficiency: serum α1-antitrypsin
 Hemochromatosis: serum iron, ferritin, transferrin saturation
 Wilson disease: serum and urine copper, serum ceruloplasmin
 Autoimmune hepatitis: hypergammaglobulinemia in the serum protein electrophoresis, AMA, ASMA, Anti-LKM-1 antibody
 PBC: positive anti-mitochondrial antibodies (AMA or AMA-M2), ↑ alkaline phosphatase, ↑ bilirubin
 PSC: cholestasis parameters (↑GGT, ↑ alkaline phosphatase, and ↑ bilirubin) ↑ pANCA
Imaging studies
 Abdominal ultrasound
o Best initial imaging study
o Liver form and structure findings
 Nodular liver surface 
 Atrophy of the right lobe
 Loss of structural homogeneity (hyperechoic or variable increase in echogenicity) with fibrous septa 
o Liver size findings
 Initially enlarged
 Atrophies and shrinks with disease progression 
o Other possible findings
 Loss of intrahepatic portal and liver veins 
 Complications of cirrhosis such as portal hypertension 
 
 CT scan 
 
o Typical findings
 Relative hypertrophy of the left and caudate lobe
 Regenerative nodules
 Irregular liver surface
 Indirect findings: ascites, splenomegaly, portocaval collaterals
 Transient elastography
 o Ultrasound technique used to measure liver elasticity
o Allows for monitoring of patients with chronic liver disease and helps with early diagnosis of increased fibrosis and progression
to cirrhosis 

Biopsy
 Execution: usually ultrasound-guided transcutaneous approach (easily performed, but may have limited diagnostic value if only a small
tissue sample can be taken) 
 Indications: Biopsy is the gold standard for diagnosis of cirrhosis. However, it should only be considered if clinical, laboratory,
and ultrasound evidence is unclear. 
o Grading and staging: autoimmune hepatitis, PBC, PSC, hemochromatosis (measurement of iron content), Wilson disease, and
post liver transplant to evaluate for rejection
o Monitoring therapeutic success
o Evaluation of tumorous lesions
 Alternative methods
o Laparoscopic biopsy Large biopsies can be obtained.
o Transjugular biopsy (transvenous biopsy)
 For severely obese patients or patients with ascites.
 There is a lower risk of bleeding in comparison to a standard biopsy.
 A transjugular biopsy is especially useful if TIPS is planned.
Before taking a biopsy, check the patient's coagulation status as the risk of bleeding may be increased.
Monitoring the disease course
Patients diagnosed with cirrhosis should repeat laboratory tests every 6 months to recalculate the Child-Pugh score and Model for End-Stage
Liver Disease (MELD) scores (see “Prognosis” below).
 HCC screening: Patients with cirrhosis should have an abdominal ultrasound every 6 months and periodic monitoring of alpha-
fetoprotein (AFP). 
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Pathology
 Fibrosis (fibrous septa)
 Replacement of normal liver tissue with collagenous regenerative nodules (histological staging is based on the size of the regenerative
nodules) 
 [6]
 Abnormal cell activation with infiltration of inflammatory cells
 Loss of physiological vessel architecture (central vein disappearance)
MAXIMIZE TABLETABLE QUIZ
Size of the regenerative nodules Occurrence

 Following a chronic active process such as diseases with slow progression

Micronodular  1–3 mm 
 E.g., alcoholic hepatitis, chronic hepatitis B, chronic hepatitis C

 Following diffuse parenchymal necrosis with relapses or acute course

Macronodular  > 3 mm
 E.g., fulminant viral hepatitis, death cap poisoning

Both  1–3 mm and > 3 mm  Possible in every type of liver-damaging disease

 See “Alcoholic liver disease,” “Hepatitis B,” and “Hepatitis C” for specific pathological findings related to these conditions.

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Treatment
General approach [10][11]
 Provide treatment for the underlying condition (e.g., treat HCV with antiviral drugs and reduce hepatotoxic influences).
  Prevent, recognize, and treat possible complications.
 Avoidance of hepatotoxic substances (e.g., alcohol, medications such as NSAIDs) 
 Routine vaccinations: pneumococcal vaccine (PPSV23), hepatitis A vaccine, hepatitis B vaccine, influenza vaccine, tetanus vaccine 
 Balanced diet with adequate calorie intake, no protein restriction 
Pharmacotherapy
 Nonselective beta blockers (e.g., propranolol) to lower portal pressure and prevent variceal bleeding (see “Treatment” of “Portal
hypertension”).
 Spironolactone and furosemide to manage ascites and edema in patients with hypoalbuminemia
 For the treatment of specific complications related to cirrhosis, see the conditions listed in “Complications” below.
Surgical/Interventional procedures [12]
 Paracentesis: a method used to decompress abdomen due to ascites
 Transjugular intrahepatic portosystemic shunt (TIPS): a method used to lower portal pressure and manage complications
o Indications
 Refractory ascites
 Recurring esophageal varices
 Bridging time until possible liver transplant
 Surgery: A liver transplant is the only curative option in patients with decompensated cirrhosis.
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Complications
 Decompensated cirrhosis: worsening of liver function in cirrhosis characterized by the presence of jaundice, ascites, variceal
hemorrhage, or hepatic encephalopathy
o When patients develop major complications (e.g., spontaneous bacterial peritonitis, hepatorenal syndrome), they are
considered to have decompensated cirrhosis.
o Precipitating factors: infection, alcohol use, medications, bleeding, or dehydration
 Major complications [10]
o Complications caused by portal hypertension
 Ascites and subsequent spontaneous bacterial peritonitis
 Esophageal variceal hemorrhage 
o Hematologic complications: coagulopathy 
 Prolonged aPTT, INR, and PT
  This condition does not respond to vitamin K because the liver cannot utilize it for the synthesis of coagulation
factors. Fresh frozen plasma is used as treatment.
o Metabolic complications or associated organ impairment
 Jaundice
 Hepatic encephalopathy
 Hepatorenal syndrome
 Hepatopulmonary syndrome
o Tumors: hepatocellular carcinoma (HCC) 
 
 Other complications 
o Secondary hyperparathyroidism: due to limited enzymatic activation of vitamin D 
o Cirrhotic cardiomyopathy
o Hepatic hydrothorax
o Portal vein thrombosis
o Diabetes mellitus secondary to liver disease 
Cirrhosis associated ascites and edema, as well as the high risk of bleeding, increase the risk for hypovolemic shock.

We list the most important complications. The selection is not exhaustive.

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Hepatic encephalopathy
Definition [11][13]
 Fluctuations in mental status and cognitive function in the presence of severe liver disease (e.g., cirrhosis)
Pathophysiology [14]
  Cirrhosis → ↓ hepatic metabolism and portosystemic shunt → accumulation of neurotoxic metabolites,
especially ammonia (NH3) → excess glutamine and swelling produced by astrocytes → cerebral edema and ↑ intracranial
pressure → neurological deterioration 
Precipitant factors
 Increased absorption or production of ammonia
o Infections (e.g., spontaneous bacterial peritonitis)
o Gastrointestinal bleeding 
o Constipation
o Excessive protein consumption
 Decreased metabolism of ammonia
o Renal failure
o Portal vein thrombosis
o Portosystemic shunts (e.g., after TIPS placement)
o Hypovolemia and electrolyte disturbances (hypokalemia, hyponatremia) 
Clinical features
Symptoms are usually reversible. 
 Fatigue, lethargy, apathy
 Altered levels of consciousness, ranging from mild confusion to stupor or coma
 Disoriented
 Irritability
 Memory loss
 Impaired sleeping patterns
 Multiple neurological and psychiatric disturbances
 Socially aberrant behavior (e.g., urinating/defecating in public, shouting at strangers)
 Slurred speech
 Asterixis
 Muscle rigidity
Diagnostics
 Based on the patient's medical history and clinical presentation
 Assessment of mental status
 o Number connection test: test in which patients are timed while connecting numbers in order that are randomly distributed over
a piece of paper (in hepatic encephalopathy it is completed slower than the age-normalized standard or not completed)
o Psychometry-based diagnostic method (e.g., Mini-Mental State Examination) [15][16]
 Laboratory studies: elevated serum ammonia levels 
Treatment
 General measures
o Avoidance and correction of precipitating factors (e.g., hepatotoxic medication, alcohol, hypovolemia, electrolyte disturbances)
o Treatment of further complications that might aggravate the encephalopathy
 Pharmacotherapy
o Lactulose: a synthetic disaccharide laxative
 First-line treatment for hepatic encephalopathy 
 Improves hepatic encephalopathy by decreasing absorption of ammonia in the bowel: lactulose is converted to lactic
acid by intestinal flora → acidification in the gut leads to conversion of ammonia (NH3) to ammonium (NH4+) → ammonium
is excreted in the feces → decreased blood ammonia concentration
o Rifaximin: a broad-spectrum, nonabsorbable oral antibiotic
 Reduces the number of ammonia-producing intestinal bacteria
 May be added to lactulose to prevent recurrent episodes of hepatic encephalopathy after a second episode
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Hepatorenal syndrome (HRS)
Definition
 Deterioration of kidney function in patients with advanced liver disease
Pathophysiology [17][18]
 The exact pathomechanisms are not completely understood.
 Cirrhosis/portal hypertension → ↑ splanchnic vasodilators (e.g., nitric oxide) → ↓ arterial blood flow → activation
of RAAS → renal vasoconstriction → ↓ GFR
Risk factors
Generally attributed to the loss of volume
 Drainage of large volume ascites
 Gastrointestinal bleeding
 Forced diuresis
  Excess use of laxatives (lactulose)
 Post TIPS procedure
 Spontaneous bacterial peritonitis
Clinical features [17]
 For clinical features associated with advanced liver disease, see “Decompensated cirrhosis” in “Complications.”
 Ranging from oliguria up to anuria with progressive kidney failure
 Hypotension and wide pulse pressure
Diagnosis
Hepatorenal syndrome is a clinical diagnosis based on reduced glomerular filtration rate in patients with cirrhosis and no other causes for renal
failure (e.g., shock) or no detection of renal pathologies on ultrasound.
 Diagnostic criteria [19]
o Cirrhosis
o Serum creatinine ≥ 0.3 mg/dL within 48 hours or ≥ 50% from baseline
o Lack of improvement in renal function after volume expansion with albumin and at least 48 hours without diuretics
o Urinary output ≤ 0.5 ml/kg
o Decreased urine sodium levels (< 10 mmol/L) 
o No proteinuria > 500 mg/dL, no microhematuria, no acute pathological findings in the kidney ultrasound
o No exposure to nephrotoxic drugs
o Absence of shock
Treatment
 General measures: improvement of liver function if possible (e.g., cessation of alcohol use, avoid nephrotoxic drugs)
 Pharmacotherapy
o Noncritically ill individuals: a combination of midodrine, octreotide, and albumin 
o Critically ill individuals: a combination of norepinephrine and albumin
 Surgical/interventional procedures
o Renal replacement therapy (dialysis) 
o Placement of a transjugular intrahepatic portosystemic shunt (TIPS) 
o A liver transplant is the only curative option.
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Portal vein thrombosis
Definition [20]
 A complete or partial closure of the portal vein due to a thrombus
Pathophysiology
 Possibly caused by an imbalance of coagulation factors in patients with impaired hepatic synthetic function
 The closure of portal vein flow → ↓ liver blood flow → vasodilation of the hepatic artery and development of collateral hepatic veins in
an attempt to maintain liver perfusion
 Thrombotic closure of the splenic and superior mesenteric veins may extend to the portal vein → portal hypertension
Etiology
 Cirrhosis or chronic liver disease 
 Local complications of intraabdominal malignancy (e.g., HCC, pancreatic carcinoma) or inflammation (e.g., liver
abscess, pancreatitis, cholangitis)
 Thrombophilia (e.g., antiphospholipid syndrome, factor V Leiden) or general risk factors of phlebothrombosis
 Myeloproliferative disorder
 Following TIPS procedure
 Chronic mesenteric venous thrombosis
Clinical features
Depend on the extent of thrombosis and the speed of manifestation
 Acute portal vein thrombosis
o Abdominal pain (RUQ or generalized)
o Splenomegaly
o Fever
o Ascites
 Chronic portal vein thrombosis
o In cirrhotic patients, chronic PVT is often asymptomatic (incidental finding on ultrasound). 
o Abdominal pain (RUQ or generalized)
o Esophageal variceal hemorrhage
o Hepatic encephalopathy 
o Splenomegaly and hypersplenism 
o Jaundice and pruritus due to portal cholangiopathy 
Diagnostics
 Ultrasound findings
 o Echogenic or isoechoic thrombus in the portal vein 
o Dilated, coiled periportal veins
o Widening of the portal vein
o Variable portion of mostly echogenic thrombus material in the portal vein
 Color duplex ultrasound
o Confirmatory test
o Shows decreased flow velocity or complete halt of flow
 CT or MRI of the abdomen with contrast agent 
 
When PVT is detected in patients with cirrhosis, HCC must be ruled out as the cause.

Treatment [21]
The goal is to prevent precipitant factors, thrombosis extension, and achieve portal vein recanalization. There are no generalized management
recommendations for cirrhotic patients with portal vein thrombosis.
 Pharmacotherapy: anticoagulants
o Indications
 In advanced cirrhosis: determined on a case-by-case basis (candidates for a liver transplant, superior mesenteric
vein thrombosis, or individuals with a coexistent prothrombotic disorder) 
 Acute symptomatic portal vein thrombosis
 Nonmalignant portal vein thrombosis
o Given for 3–6 months
o Complete recanalization occurs in about 50% of patients.
 Surgical/Interventional procedures [22]
o Transjugular intrahepatic portosystemic shunt (TIPS)
 Can be considered for individuals with cirrhosis and chronic PVT
 Cases of uncontrollable variceal bleeding in individuals with cirrhosis
 o Percutaneous transhepatic thrombolysis with tissue plasminogen activator (tPA): individuals with cirrhosis and acute portal vein
thrombosis 
o Liver transplant: depends on the extent of thrombosis 
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Pulmonary complications of portal hypertension
Hepatopulmonary syndrome [23][24]
Definition
 A condition characterized by hypoxemia, intrapulmonary vasodilatation, and portal hypertension in the presence of cirrhosis 
Pathophysiology
 Not completely understood
 Portal hypertension and liver damage → translocation of bacterial endotoxins → changes in the production of cytokines and
pulmonary vasodilators → ↑ nitric oxide in the lung vessels → pulmonary vasodilation → hepatopulmonary syndrome
Clinical features
 Dyspnea
 Platypnea
 Orthodeoxia
Diagnostics [25]
 Medical history: diagnosis of advanced liver disease
 Arterial blood gas analysis: alveolar-arterial gradient (AaO2) ≥ 15 mmHg or ≥ 20 mmHg in patients > 64 years old while in a seating
position 
 Imaging
o Contrast echocardiogram
 Gold-standard test
 Shows intrapulmonary vasodilation
o Lung perfusion scintigraphy (Tc-99m MAA): helps diagnose and quantify intrapulmonary vasodilation
Treatment [24]
 Supportive measures: Long-term treatment with oxygen is recommended.
 Surgical procedure: liver transplant 
Portopulmonary hypertension [26][27]
Definition
  A form of pulmonary arterial hypertension (PAH) associated with portal hypertension
Pathophysiology
 Not completely understood
 High cardiac output in advanced liver disease → wall shear stress in pulmonary vasculature → ↑ vasoactive and angiogenic substances
(e.g., endothelin-1) → hypertrophy of smooth muscle cells and fibroblasts, fibrosis of intimal sheath, and microaneurysms of
pulmonary arterioles
Clinical features
 Often asymptomatic
 Clinical findings are the same as in pulmonary hypertension 
Diagnostics
 Medical history: portal hypertension or cirrhosis
 Imaging
o Transthoracic echocardiography
o Right heart catheterization (gold standard test)
 Diagnostic criteria
o Clinical signs and symptoms of portal hypertension, but not necessarily the presence of cirrhosis
o Abnormal hemodynamic measurements from right heart catheterization (mPAP > 25 mmHg at rest, PCWP < 15 mmHg,
and ↑ PVR)
Treatment
 Supportive measures: to alleviate symptoms
o Supplemental oxygen
o Diuretics: furosemide and spironolactone in cases of right heart failure due to PAH and liver cirrhosis
 Pharmacotherapy: Epoprostenol, bosentan, or sildenafil have insufficient data to make generalized recommendations.
 Surgical procedure: liver transplantation
Other pulmonary complications in cirrhosis
 Pneumonia: due to immunosuppression 
 Atelectasis: diaphragm is elevated due to massive ascites
 Lung emphysema: in α1-antitrypsin deficiency
 Hepatic hydrothorax: mostly one-sided pleural effusions (70% right, 18% left) with transudate characteristics [28]
o Pathophysiology: caused by increased permeability of the diaphragm on account of microperforations, increased lymphatic
leakage, and hypoalbuminemia
 o Clinical findings: dyspnea (at rest)
o Treatment
 Pleural tap for symptomatic relief
 In cases of recurring fluid accumulation, continuous drainage should be considered.
NOTES
FEEDBACK
Prognosis
Liver transplantation should be considered when medical treatment of cirrhosis has failed. Risk stratification for liver transplant is done by
using the Model for End-Stage Liver Disease (MELD score) and Child-Pugh score systems. [10]
Child-Pugh score
 A prognostic grading scale to assess the severity of cirrhosis, on the basis of specific laboratory markers
(e.g., bilirubin, albumin, prothrombin time), as well as ascites and encephalopathy
o Can be used as a prognostic scoring system [29]
 Child-Pugh class A: almost normal
 Child-Pugh class B: one-year survival rate of approx. 80%
 Child-Pugh class C: one-year survival rate of approx. 45%
o In patients with decompensated cirrhosis survival is poor, unless they receive liver transplantation.
MAXIMIZE TABLETABLE QUIZ
Child-Pugh score

Points 1 2 3

Serum albumin g/dL > 3.5 2.8–3.5 < 2.8

Serum bilirubin mg/dL < 2.0 2.0–3.0 > 3.0

INR < 1.7 1.7–2.3 > 2.3

Ascites None Mild Moderate

 Child-Pugh score

Points 1 2 3

Hepatic encephalopathy None Minimal Advanced

Child-Pugh class A: 5–6 points; Child-Pugh class B: 7–9 points; Child-Pugh class C: 10–15 points
CHILD's ABCDEs: Albumin, Bilirubin, Coagulation (e.g., INR), Distended abdomen (ascites), and Encephalopathy.
MELD score
 An additional model used to predict prognosis in patients with cirrhosis, in terms of three-month mortality
 Primarily used to prioritize patients needing liver transplantation
 Patients are given a score from 1–40 based on serum bilirubin, INR, and creatinine levels.
 The higher the score, the worse the prognosis.