Portal Hypertension

Portal hypertension is increased pressure in the portal venous system. This increased pressure can lead to splanchnic vasodilation, collateral blood flow through portosystemic anastomoses, and increased hydrostatic pressure. There are a number of etiologies, including cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic parenchymal necrosis and scarring (fibrosis) most commonly due to hepatitis C infection and alcoholic liver disease. Patients may present with jaundice, ascites, and hepatosplenomegaly. Cirrhosis can also cause complications such as hepatic encephalopathy, portal hypertension, portal vein thrombosis, and hepatorenal syndrome. Cirrhosis, right-sided congestive heart failure Congestive heart failure Congestive heart failure refers to the inability of the heart to supply the body with normal cardiac output to meet metabolic needs. Echocardiography can confirm the diagnosis and give information about the ejection fraction. Congestive Heart Failure, schistosomiasis Schistosomiasis Schistosomiasis is an infection caused by Schistosoma, a trematode. Schistosomiasis occurs in developing countries with poor sanitation. Freshwater snails are the intermediate host and are transmitted to humans through skin contact with contaminated fresh water. The clinical presentation occurs as a result of the host's immune response to antigens from the eggs. Schistosoma/Schistosomiasis, portal vein thrombosis, hepatitis, and Budd-Chiari syndrome Budd-Chiari syndrome Budd-Chiari syndrome is a condition resulting from the interruption of the normal outflow of blood from the liver. The primary type arises from a venous process (affecting the hepatic veins or inferior vena cava) such as thrombosis, but can also be from a lesion compressing or invading the veins (secondary type). The patient typically presents with hepatomegaly, ascites, and abdominal discomfort. Budd-Chiari Syndrome. Most individuals are asymptomatic until complications arise, including esophageal varices, portal hypertensive gastropathy, ascites Ascites Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Ascites, and hypersplenism. The diagnosis is clinical, but it can be supported by ultrasound findings (and hepatic venous pressure gradient measurement in unclear cases). Management requires treating the underlying etiology and managing the complications. This can include nonselective beta blockers to prevent bleeding from varices, diuretics and sodium restriction for ascites Ascites Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Ascites, and transjugular intrahepatic portosystemic shunt for refractory complications.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Etiology and Pathophysiology

Etiology

The etiologies of portal hypertension can be classified based on the location of increased resistance to blood flow through the liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver.

Prehepatic etiologies:

  • Portal vein thrombosis
  • Splenic vein thrombosis
  • Massive splenomegaly Splenomegaly Splenomegaly is pathologic enlargement of the spleen that is attributable to numerous causes, including infections, hemoglobinopathies, infiltrative processes, and outflow obstruction of the portal vein. Splenomegaly
  • Splanchnic arteriovenous fistula

Hepatic etiologies:

  • Presinusoidal:
    • Schistosomiasis
    • Early primary biliary cholangitis Primary Biliary Cholangitis Primary biliary cholangitis (PBC) is a chronic disease resulting in autoimmune destruction of the intrahepatic bile ducts. The typical presentation is that of a middle-aged woman with pruritus, fatigue, and right upper quadrant abdominal pain. Elevated liver enzymes and antimitochondrial antibodies (AMAs) establish the diagnosis. Primary Biliary Cholangitis
    • Granulomatous disease (e.g., sarcoidosis Sarcoidosis Sarcoidosis is a multisystem inflammatory disease that causes noncaseating granulomas. The exact etiology is unknown. Sarcoidosis usually affects the lungs and thoracic lymph nodes, but it can also affect almost every system in the body, including the skin, heart, and eyes, most commonly. Sarcoidosis)
    • Congenital hepatic fibrosis
    • Polycystic liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver disease
    • Idiopathic noncirrhotic portal hypertension
  • Sinusoidal:
    • Cirrhosis (most common cause in Western countries)
    • Acute alcoholic hepatitis
    • Viral hepatitis
    • Vitamin A intoxication
  • Postsinusoidal: hepatic veno-occlusive disease

Posthepatic etiologies:

  • Cardiac:
    • Right-sided heart failure
    • Severe tricuspid regurgitation Tricuspid regurgitation Tricuspid regurgitation (TR) is a valvular defect that allows backflow of blood from the right ventricle to the right atrium during systole. Tricuspid regurgitation can develop through a number of cardiac conditions that cause dilation of the right ventricle and tricuspid annulus. A blowing holosystolic murmur is best heard at the left lower sternal border. Tricuspid Regurgitation
    • Constrictive pericarditis Pericarditis Pericarditis is an inflammation of the pericardium, often with fluid accumulation. It can be caused by infection (often viral), myocardial infarction, drugs, malignancies, metabolic disorders, autoimmune disorders, or trauma. Acute, subacute, and chronic forms exist. Pericarditis
    • Restrictive cardiomyopathy Restrictive Cardiomyopathy Restrictive cardiomyopathy (RCM) is a fairly uncommon condition characterized by progressive stiffening of the cardiac muscle, which causes impaired relaxation and refilling of the heart during diastole, resulting in diastolic dysfunction and eventual heart failure. Restrictive Cardiomyopathy
  • Noncardiac: Budd-Chiari syndrome Budd-Chiari syndrome Budd-Chiari syndrome is a condition resulting from the interruption of the normal outflow of blood from the liver. The primary type arises from a venous process (affecting the hepatic veins or inferior vena cava) such as thrombosis, but can also be from a lesion compressing or invading the veins (secondary type). The patient typically presents with hepatomegaly, ascites, and abdominal discomfort. Budd-Chiari Syndrome

Pathophysiology

Anatomy:

  • Blood supply: the liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver has a special dual blood supply that provides a mix of oxygenated and deoxygenated (but nutrient-rich) blood. 
    • Hepatic artery proper: 
      • Supplies 25% of the liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver’s blood supply
      • Carries oxygenated blood
    • Portal vein: 
      • Supplies 75% of blood supply
      • Formed most commonly by the union of the splenic and superior mesenteric veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins
      • Carries oxygen-poor, nutrient-rich blood drained from the abdominal organs
  • Venous drainage: sinusoids → central vein of each lobule → hepatic veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins → inferior vena cava (IVC)
  • Portosystemic anastomoses: 
    • Alternative routes of circulation ensure venous drainage of the abdominal organs even if a blockage occurs in the portal system.
    • Anastomoses include:
      • Left gastric veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins and lower esophageal veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins 
      • Superior rectal veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins and inferior and middle rectal veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins
      • Paraumbilical veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins and small epigastric veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins
      • Intraparenchymal hepatic branches of right division of portal vein and retroperitoneal veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins 
      • Omental and colonic veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins with retroperitoneal veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins 
      • Ductus venosus and the IVC

Portal hypertension:

  • Pathologically elevated pressure of the portal venous system (> 5 mm Hg higher than the IVC)
  • ↑ Venous resistance (e.g., liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver disease, vascular obstruction) → ↑ portal venous pressure → complications:
    • Varices: ↑ collateral blood flow through the portosystemic anastomoses 
    • Ascites: 
      • ↑ Hydrostatic pressure in the downstream hepatic vessels →  fluid shift from intravascular space to peritoneal cavity
      • ↑ Splanchnic vasodilation and blood pooling → ↓ arterial volume → ↓ renal blood flow → RAAS activation → sodium and water retention
    • Hypersplenism: ↑ splenic vein pressure
Esophageal varices, splenomegaly, and rectal varices in portal hypertension

Esophageal varices, splenomegaly Splenomegaly Splenomegaly is pathologic enlargement of the spleen that is attributable to numerous causes, including infections, hemoglobinopathies, infiltrative processes, and outflow obstruction of the portal vein. Splenomegaly, and rectal varices resulting from backup of blood flow due to elevated pressures within the portal vein

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Clinical Presentation

Portal hypertension itself usually has no symptoms. Clinical manifestations arise as a result of the underlying etiology and/or complications.

Evidence of common complications

  • Collateral portosystemic blood flow and varices:
    • Caput medusae (engorged paraumbilical veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins)
    • Hematemesis and/or melena due to:
      • Esophageal varices (potentially life-threatening)
      • Portal hypertensive gastropathy (gastric mucosal vascular congestion)
    • Bleeding from rectal varices
  • Hypersplenism:
    • Splenomegaly
    • Pancytopenia
  • Ascites:
    • Abdominal distention
    • Fluid wave
    • Shifting dullness

Evidence of common etiologies

  • Cirrhosis:
    • Jaundice
    • Pruritus
    • Gynecomastia Gynecomastia Gynecomastia is a benign proliferation of male breast glandular ductal tissue, usually bilateral, caused by increased estrogen activity, decreased testosterone activity, or medications. The condition is common and physiological in neonates, adolescent boys, and elderly men. Gynecomastia
    • Palmar erythema
    • Spider angiomata
    • Asterixis
  • Right-sided congestive heart failure Congestive heart failure Congestive heart failure refers to the inability of the heart to supply the body with normal cardiac output to meet metabolic needs. Echocardiography can confirm the diagnosis and give information about the ejection fraction. Congestive Heart Failure
    • Peripheral edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema
    • Elevated jugular venous pressure
    • Shortness of breath
    • Paroxysmal nocturnal dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea

Diagnosis

Diagnostic evaluation

The diagnosis of portal hypertension is generally based on clinical evaluation, but it may be supported by:

  • Ultrasonography:
    • Should be performed with Doppler flow
    • Can support the diagnosis, but not sensitive
    • Potential findings suggesting portal hypertension:
      • Reversed blood flow in the portal vein
      • Portosystemic collaterals
      • Enlarged portal vein diameter
      • Ascites
      • Splenomegaly
    • Evaluates for potential etiologies:
      • Nodular/cirrhotic liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver
      • Venous thrombosis
  • Hepatic venous pressure gradient measurement (HVPG)
    • Usually done only if the diagnosis is unclear
    • Approximates the pressure gradient between the portal vein and IVC by using a transjugular catheter
    • HVPG ≥ 6 mm Hg is diagnostic.
Ultrasound of the liver identifies a large portal vein consistent with portal hypertension

Ultrasound of the liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver identifies a large portal vein, consistent with portal hypertension.

Image: “Ultrasound abdomen: ultrasound of liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver identifies a patent portal vein, 158 mm in diameter, indicative of portal hypertension in this noncirrhotic patient.” by Ratnayake S. et al. License: CC BY 3.0

Additional evaluation

Additional studies may be performed based on the clinical presentation and presence of complications.

  • Cirrhosis:
    • Liver enzymes Enzymes Enzymes are complex protein biocatalysts that accelerate chemical reactions without being consumed by them. Due to the body's constant metabolic needs, the absence of enzymes would make life unsustainable, as reactions would occur too slowly without these molecules. Basics of Enzymes (↑ bilirubin, AST, and ALT are common)
    • Coagulation studies Coagulation studies Coagulation studies are a group of hematologic laboratory studies that reflect the function of blood vessels, platelets, and coagulation factors, which all interact with one another to achieve hemostasis. Coagulation studies are usually ordered to evaluate patients with bleeding or hypercoagulation disorders. Coagulation Studies
    • ↓ Albumin
    • ↑ Ammonia 
  • Hypersplenism: CBC → evaluate for anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview, thrombocytopenia Thrombocytopenia Thrombocytopenia occurs when the platelet count is < 150,000 per microliter. The normal range for platelets is usually 150,000-450,000/µL of whole blood. Thrombocytopenia can be a result of decreased production, increased destruction, or splenic sequestration of platelets. Patients are often asymptomatic until platelet counts are < 50,000/µL. Thrombocytopenia
  • Ascites: 
    • Paracentesis can be considered.
    • Serum– ascites Ascites Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Ascites albumin gradient (SAAG): > 1.1 g/dL indicates portal hypertension
    • Ascites total protein:
      • > 2.5 g/dL, consider cardiac causes and Budd-Chiari syndrome Budd-Chiari syndrome Budd-Chiari syndrome is a condition resulting from the interruption of the normal outflow of blood from the liver. The primary type arises from a venous process (affecting the hepatic veins or inferior vena cava) such as thrombosis, but can also be from a lesion compressing or invading the veins (secondary type). The patient typically presents with hepatomegaly, ascites, and abdominal discomfort. Budd-Chiari Syndrome
      • < 2.5 g/dL, consider cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic parenchymal necrosis and scarring (fibrosis) most commonly due to hepatitis C infection and alcoholic liver disease. Patients may present with jaundice, ascites, and hepatosplenomegaly. Cirrhosis can also cause complications such as hepatic encephalopathy, portal hypertension, portal vein thrombosis, and hepatorenal syndrome. Cirrhosis
  • Varices: EGD: 
    • Can be performed to screen for esophageal varices
    • Diagnostic and therapeutic for acute bleeding from esophageal varices
Esophageal varices cirrhosis

Endoscopic view of the esophageal varices:
The red patches indicate a recent bleed.

Image: “Gastroscopy image of esophageal varices with prominent red wale spots” by Samir. License: Public Domain

Management

The focus of therapy is to prevent and treat the complications of portal vein thrombosis. It is also important to treat the underlying etiology (when possible).

Varices and portal hypertensive gastropathy management

  • Nonselective β-blockers (propranolol, nadolol): 
    • Cause splanchnic vasoconstriction and  ↓ portal inflow
    • ↓ Risk of bleeding from varices or gastropathy
  • Management for acute bleeding:
    • IV fluid resuscitation
    • Blood transfusion, as needed
    • Octreotide: causes splanchnic vasoconstriction → ↓ portal pressure
    • Antibiotic prophylaxis (ceftriaxone): ↓ risk of spontaneous bacterial peritonitis
    • Endoscopic interventions: endoscopic band ligation or sclerotherapy for varices
  • Transjugular intrahepatic portosystemic shunt: 
    • May be used for recurrent or refractory bleeding
    • Creates and stents a connection between the portal and hepatic vein → ↓ pressure in the portal vein

Ascites management

  • Conservative management
    • Sodium restriction (< 2 g/day)
    • Diuretic therapy:
      • Furosemide
      • Spironolactone
  • Invasive management:
    • Therapeutic paracentesis:
      • Rapid symptom relief
      • Used if ascites Ascites Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Ascites is refractory to conservative measures
    • Transjugular intrahepatic portosystemic shunt for refractory ascites Ascites Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Ascites

Clinical Relevance

  • Cirrhosis: late stage of hepatic necrosis and scarring. In cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic parenchymal necrosis and scarring (fibrosis) most commonly due to hepatitis C infection and alcoholic liver disease. Patients may present with jaundice, ascites, and hepatosplenomegaly. Cirrhosis can also cause complications such as hepatic encephalopathy, portal hypertension, portal vein thrombosis, and hepatorenal syndrome. Cirrhosis, chronic cellular damage causes extensive distortion of the normal hepatic architecture, which can lead to impairment of normal blood flow through the liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver and portal hypertension. Signs and symptoms are often nonspecific (e.g., fatigue, anorexia, weight loss). Decompensation manifests as jaundice Jaundice Jaundice is the abnormal yellowing of the skin and/or sclera caused by the accumulation of bilirubin. Hyperbilirubinemia is caused by either an increase in bilirubin production or a decrease in the hepatic uptake, conjugation, or excretion of bilirubin. Jaundice, ascites Ascites Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Ascites, and liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver failure. Diagnosis often requires liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver biopsy. Management is mostly supportive, with liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver transplantation being the only curative treatment.
  • Budd-Chiari syndrome Budd-Chiari syndrome Budd-Chiari syndrome is a condition resulting from the interruption of the normal outflow of blood from the liver. The primary type arises from a venous process (affecting the hepatic veins or inferior vena cava) such as thrombosis, but can also be from a lesion compressing or invading the veins (secondary type). The patient typically presents with hepatomegaly, ascites, and abdominal discomfort. Budd-Chiari Syndrome: hepatic venous outflow obstruction (from thrombosis, compression, or vascular invasion), which may affect the hepatic veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins and/or IVC. Typical presentation is with hepatomegaly, ascites Ascites Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Ascites, and abdominal discomfort. The diagnosis is confirmed by Doppler ultrasonography. Treatment involves addressing the underlying condition that caused the venous occlusion.
  • Portal vein thrombosis: can occur from a number of causes ( hypercoagulable Hypercoagulable Hypercoagulable states (also referred to as thrombophilias) are a group of hematologic diseases defined by an increased risk of clot formation (i.e., thrombosis) due to either an increase in procoagulants, a decrease in anticoagulants, or a decrease in fibrinolysis. Hypercoagulable States state, surgery, inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation, trauma, malignancy) and results in portal hypertension. Ultrasound with Doppler showing absent or diminished blood flow through the portal vein is usually diagnostic. Management includes anticoagulation, thrombolysis in specific cases, and management of complications of portal hypertension.
  • Schistosomiasis: infection by the trematode Schistosoma Schistosoma Schistosomiasis is an infection caused by Schistosoma, a trematode. Schistosomiasis occurs in developing countries with poor sanitation. Freshwater snails are the intermediate host and are transmitted to humans through skin contact with contaminated fresh water. The clinical presentation occurs as a result of the host's immune response to antigens from the eggs. Schistosoma/Schistosomiasis. Schistosomiasis occurs in developing countries with poor sanitation. The clinical presentation is a result of the host’s immune response to antigens from the eggs. Most individuals are asymptomatic, but others may develop acute schistosome dermatitis, acute schistosomiasis Schistosomiasis Schistosomiasis is an infection caused by Schistosoma, a trematode. Schistosomiasis occurs in developing countries with poor sanitation. Freshwater snails are the intermediate host and are transmitted to humans through skin contact with contaminated fresh water. The clinical presentation occurs as a result of the host's immune response to antigens from the eggs. Schistosoma/Schistosomiasis syndrome, or chronic schistosomiasis Schistosomiasis Schistosomiasis is an infection caused by Schistosoma, a trematode. Schistosomiasis occurs in developing countries with poor sanitation. Freshwater snails are the intermediate host and are transmitted to humans through skin contact with contaminated fresh water. The clinical presentation occurs as a result of the host's immune response to antigens from the eggs. Schistosoma/Schistosomiasis. The diagnosis can be made with microscopic evaluation of the urine or feces, serology, or PCR. The treatment for schistosomiasis Schistosomiasis Schistosomiasis is an infection caused by Schistosoma, a trematode. Schistosomiasis occurs in developing countries with poor sanitation. Freshwater snails are the intermediate host and are transmitted to humans through skin contact with contaminated fresh water. The clinical presentation occurs as a result of the host's immune response to antigens from the eggs. Schistosoma/Schistosomiasis is praziquantel.
  • Congestive hepatopathy: right-sided congestive heart failure Congestive heart failure Congestive heart failure refers to the inability of the heart to supply the body with normal cardiac output to meet metabolic needs. Echocardiography can confirm the diagnosis and give information about the ejection fraction. Congestive Heart Failure can lead to severe venous congestion, including within the liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver. This can progress to cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic parenchymal necrosis and scarring (fibrosis) most commonly due to hepatitis C infection and alcoholic liver disease. Patients may present with jaundice, ascites, and hepatosplenomegaly. Cirrhosis can also cause complications such as hepatic encephalopathy, portal hypertension, portal vein thrombosis, and hepatorenal syndrome. Cirrhosis. Individuals can have evidence of both heart failure and cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic parenchymal necrosis and scarring (fibrosis) most commonly due to hepatitis C infection and alcoholic liver disease. Patients may present with jaundice, ascites, and hepatosplenomegaly. Cirrhosis can also cause complications such as hepatic encephalopathy, portal hypertension, portal vein thrombosis, and hepatorenal syndrome. Cirrhosis, and the diagnosis is generally clinical. Management involves treating the underlying heart failure.

References

  1. Hou, W., Sanyal, A. J. (2009). Ascites: diagnosis and management. Med Clin North Am 93:801–817, vii. reference.medscape.com/medline/abstract/19577115
  2. Lubel, J. S., Angus, P. W. /2005). Modern management of portal hypertension. Intern Med J 35:45–49. reference.medscape.com/medline/abstract/15667468
  3. Bleibel, W., Chopra, S., Curry, M. P. (2021). Portal hypertension in adults. UpToDate. Retrieved October 3, 2021, from https://www.uptodate.com/contents/portal-hypertension-in-adults
  4. Garcia-Pagan, J. C., Pik Eu, J. C. (2021). Noncirrhotic portal hypertension. UpToDate. Retrieved October 3, 2021, from https://www.uptodate.com/contents/noncirrhotic-portal-hypertension
  5. Oliver, T. I., Sharma, B., John, S. (2021). Portal hypertension. StatPearls. Retrieved October 3, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK507718/
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  7. Tholey, D. (2021). Portal hypertension. MSD Manual Professional Version. Retrieved October 3, 2021, from https://www.msdmanuals.com/professional/hepatic-and-biliary-disorders/approach-to-the-patient-with-liver-disease/portal-hypertension

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