Portal Hypertension Syndrome – Symptoms and Treatment

Definition and Causes:

Portal hypertension syndrome is a collection of symptoms that occur when there is elevated pressure in the portal vein, which collects blood from the stomach, spleen, intestines, pancreas, and transports it to the liver. This syndrome manifests with splenomegaly (enlarged spleen), dilated veins in the esophagus, stomach, abdominal wall vessels, and hemorrhoids.

Causes of Portal Hypertension Syndrome:
The pressure in the portal vein can rise due to obstructions to blood flow in various locations within it. Often, portal hypertension syndrome is complex due to various liver diseases (such as cirrhosis, cancer, etc.) or vascular injuries, such as Budd-Chiari syndrome, among others. However, there may be other causes as well.

Causes of Portal Hypertension Syndrome include:

  1. Arteriovenous shunts – an abnormal connection between an artery and a vein, usually not present, such as the dreadful connection between the hepatic artery and portal vein. When this connection occurs, the pressure in the portal vein increases because the blood flow to it exceeds its drainage. Fistulas can be congenital or acquired (due to injury or hepatic artery enlargement or liver cancer).
  2. Vein thrombosis or occlusion (non-opening) in the portal vein, splenic vein, hepatic veins, or inferior pulmonary vein. Occlusion may result from vascular occlusive disease (small vessel occlusion in the liver), developmental defects, or penetration (tumor growth), and other conditions. Disorders in the coagulation and fibrinolysis system (such as Budd-Chiari syndrome, antithrombin III deficiency, protein C and S deficiencies) are the primary cause of vessel thrombosis in the portal vein system.
  3. Liver diseases:
    Various types of liver fibrosis (such as alcoholic, viral, primary biliary cirrhosis, etc.). In 70-80% of cases of portal hypertension, liver fibrosis is already the cause.
    Wilson’s disease – hepatic copper accumulation.
    Sarcoidosis and other granulomas (inflammation causing the formation of multiple small masses around vessels) such as tuberculosis.
    Acute hepatitis, among others.
  4. Heart diseases associated with elevated blood pressure on the right side of the heart:
    Cardiomyopathy. This is a disease of the heart muscle (myocardium), where the heart chambers dilate and the organ’s function is impaired.
    Pericarditis. This is inflammation of the heart’s covering (pericardium), leading to swelling that puts pressure on the heart muscle.
    Heart valve diseases, such as tricuspid valve incompetence, which can lead to tricuspid regurgitation (blood flow from the right ventricle to the right atrium).
  5. Cumulative diseases, such as Niemann-Pick disease type C, where fats accumulate in the brain, spinal cord, lymph nodes, spleen, and liver.
    If the cause cannot be determined, portal hypertension is termed “idiopathic.”


The prevalence of portal hypertension syndrome is estimated by assessing the prevalence of diseases causing it. For example, according to various authors, the prevalence rate of liver fibrosis in the population (based on autopsy findings) ranges from 1 to 11%, with an average ranging from 2 to 3%. This disease predominantly affects men over 40 years old. Thromboses or other variables of Budd-Chiari syndrome are detected in 4.9-9% of patients with portal hypertension not associated with tumors.

Symptoms of Portal Hypertension Syndrome:

Portal hypertension can persist for a long time (several years or even decades) without symptoms. The manifestation depends on the primary symptoms of the disease leading to portal vein pressure elevation. In the early stages of portal hypertension, regardless of the cause, heaviness, bloating, and pain in the lower right side, abdominal swelling, and weakness may occur. These symptoms coincide with those of the early stage of liver fibrosis.

  • Among the early manifestations of portal hypertension, splenomegaly (enlarged spleen) can be mentioned. Often, this occurs without symptoms, meaning the patient may not notice it. However, when splenic inflammation (splenic capsule inflammation) or splenic rupture (due to blood vessel occlusion) occurs, sharp or painful pains may appear in the lower left side.
  • Another appearance is the venous dilation in the esophagus and stomach. However, it also forms without symptoms.
  • In the late stages of portal hypertension, ascites (fluid accumulation in the abdominal cavity) develops, resulting in increased abdominal size and various digestive disorders (abdominal bloating, nausea, vomiting, watery diarrhea, etc.). Also, dilation of veins in the umbilical area in the form of “caput medusae” may also be observed in the late stages.

What Happens in the Body with Portal Hypertension:

The portal vein is formed by the merging of the splenic, superior, and inferior mesenteric veins. In the normal state, blood moves through these veins from bottom to top towards the liver. Then, the blood is transferred through the hepatic veins to the inferior vena cava, reaching the heart.

In patients with portal hypertension, there is an obstacle along the blood flow path, so the blood in the portal vein system must move in the opposite direction to reach the heart, bypassing the liver. These bypass veins are called collateral veins. Blood flow through these veins is made possible by portal venous collaterals – a vascular system that connects portal vein flows with inferior vena cava flows, providing an alternative path for blood flow bypassing the liver.

Collateral veins are smaller in size than the portal vein, as they are not intended to transport large amounts of blood. Therefore, when there is high blood pressure, these veins dilate and bend, leading to the development of varices. For example, when there is a bypass vein between the portal vein and the superior vena cava, varices of the esophagus and stomach may appear. When the venous networks of the rectum and inferior vena cava are connected (through the veins around the umbilicus and hemorrhoidal veins), hemorrhoidal nodes and/or “caput medusae” may form.

Classification and Stages of Portal Hypertension Syndrome:

  1. Suprahepatic – Blood flow takes a worse route above the liver: in the hepatic vein, inferior vena cava (due to thrombosis or other obstruction), or in the heart due to diseases raising the pressure in the right atrium, such as tricuspid valve insufficiency with grade 2-3 regurgitation, pericarditis, among others.
  2. Intrahepatic – Difficulties in blood flow occur within the liver itself. This occurs more than others – in 85-90% of cases. Portal hypertension within the liver can be:
  • Prehepatic – Develops against the background of hepatic vein branch thrombosis, bile duct inflammations, neoplastic proliferative diseases, inflammatory tumors (schistosomiasis, sarcoidosis, tuberculosis), hemochromatosis (genetic disease associated with iron deposition), Wilson’s disease, among others.
  • Intrahepatic – All forms of liver fibrosis, acute alcoholic hepatitis, severe viral hepatitis, vitamin A toxicity, may lead to this type of portal hypertension.
  • Posthepatic – Venous thrombosis or central alcoholic glassy liver fibrosis may be the cause of its appearance.
  1. Subhepatic – Blood flow below the level of the liver in the portal vein itself or in the splenic vein is difficult.
  2. Mixed – Difficulties in blood flow are present at the same time at different levels in the portal vein system and/or its upper divisions. For example, if liver fibrosis (at the liver level) is complicated by thrombosis in the portal vein (at the subhepatic level).

Some authors also distinguish the dynamic suprahepatic form of portal hypertension, caused by existing portosystemic shunts (e.g., between the splenic artery and splenic vein).

Grades of Portal Hypertension Depend on the Level of Pressure Elevation in the Portal Venous System:

  1. Grade 1 – Pressure 200-400 mm of water.
  2. Grade 2 – Pressure 400-600 mm of water.
  3. Grade 3 – More than 600 mm of water.

Complications Associated with Portal Hypertension Syndrome:

  1. Bleeding from Varices: Varices can develop anywhere in the gastrointestinal tract and are prone to injury as bleeding progresses, which can be severe. Esophageal variceal bleeding is particularly dangerous, especially when massive bleeding leads to rapid blood loss. Signs may include low blood pressure, increased heart rate, general weakness, and anemia.
  2. Hepatic Encephalopathy: With the development of collateral blood flow pathways (anastomoses), blood flow around the liver occurs without filtration, leading to the direct transfer of toxins (such as ammonia) to the brain, causing hepatic encephalopathy and brain function impairment due to toxin poisoning. Symptoms may include cognitive and motor disturbances.
  3. Loss of Consciousness: Indicates a state of impaired consciousness due to the development of hepatic encephalopathy, where problems with cognition and mobility appear.

Diagnosis of Portal Hypertension Syndrome:

When suspecting portal hypertension, it is recommended to consult a general practitioner or a gastroenterologist.

Clinical Examination:
During the examination, the doctor can detect signs of liver damage, such as spider nevi on the skin, palmar erythema, jaundice, abdominal swelling, dilated blood vessels in the umbilical area, and an enlarged liver and/or spleen, as well as signs of hepatic encephalopathy.

Diagnostic Imaging Techniques:

  • Ultrasonography: Ultrasound examinations are considered diagnostic tools for detecting any suspicion of liver disease and are widely used for diagnosing portal hypertension. Through ultrasound examination, normal changes in the liver structure and liver and spleen enlargement can be observed.
  • Ultrasound techniques also allow the determination of vessel sizes and the detection of their curvature. An increased diameter of the portal vein indicates elevated pressure when it exceeds 13 mm and/or an increase in diameter during respiration when vessels in the upper part of the portal vein and spleen vein are not adequately dilated.

Laboratory Diagnosis:

  • General and biochemical blood analysis: Elevated levels of transaminases (ALT, AST), bilirubin, gamma globulin, alkaline phosphatase, erythrocyte sedimentation rate (ESR), and leukocyte count may be observed. However, patients with portal hypertension may experience a decrease in white blood cell and platelet counts, which accumulate (sequestrate) in the enlarged spleen.

Treatment of Intrahepatic Portal Hypertension:

First Steps:

  • Treating the underlying cause of liver disease, such as avoiding alcohol consumption in alcoholic liver cirrhosis or reducing viruses in viral liver cirrhosis.
  • Providing functional rest for the liver through a diet that includes easily digestible, mechanically and chemically gentle food, and reducing protein and salt intake.
  • Using lactulose laxatives to reduce bacterial growth in the intestines and reduce intestinal absorption of toxins.

Pharmacological Treatment (Conservative):

  • Use of non-selective beta-blockers to reduce the risk of bleeding from dilated vessels by 16%, especially with medium and large-sized nodes. These drugs also shift blood flow from small vessels to large ones by 26% over 3 years.
  • Reduction of heart rate (pulse) by 25% requires continuous doses.
  • Alternatives: Nitrates can be used in case of side effects.

Emergency and Surgical Treatment:

  • Use of arterial blockers (Terlipressin, Octreotide) to reduce arterial spread in that area.
  • Use of tubes to compress dilated vessels to stop bleeding.
  • Surgery to stop acute bleeding.
  • Emergency procedures such as opening the stomach with suturing of dilated vessels.
  • Planned procedures include creating shunts (shunts) between the portal vein and other veins at different levels.
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