Portal hypertension – colloquially called portal vein hypertension – (synonyms: portal hypertension; ICD-10 K76.6: portal hypertension) with clinical relevance is said to occur when there is a permanent pressure increase of >10 mmHg in the vena portae (portal vein). Physiological (natural) is a hepatic vein pressure gradient (LVDG) of 5-10 mmHg. The portal vein collects blood from the veins of the unpaired abdominal organs (gastrointestinal tract/gastrointestinal tract and spleen) and delivers it to the liver for detoxification and metabolization. Portal hypertension is classified according to the localization of the increase in resistance as follows:
- Prehepatic block (obstruction (narrowing) is anterior to the liver) – approximately 15-25% of affected individuals suffer from this form:
- Arterio-portal venous fistulas.
- Idiopathic (with no apparent cause).
- Splenic vein thrombosis
- Portal vein thrombosis (PVT) (common).
- Intrahepatic block (obstruction is within the liver) – approximately 70-80% of affected individuals suffer from this form:
- Schistosomiasis – worm disease (tropical infectious disease) caused by trematodes (sucking worms) of the genus Schistosoma (couple flukes).
- Hepatoportal sclerosis (rare disease with sclerosis (calcification) of the intrahepatic (“located inside the liver“) portal veins).
- Congenital (congenital) fibrosis (abnormal proliferation of connective tissue).
- Myeloproliferative disorders (group of malignant (malignant) hematologic (“blood-related”) diseases).
- Primary biliary cholangitis (PBC, synonyms: nonpurulent destructive cholangitis; primary biliary cirrhosis) – relatively rare autoimmune liver disease originating in the intrahepatic (“inside the liver”) bile ducts and associated with inflammation; in the longer course, inflammation spreads to all liver tissue and eventually leads to scarring and even cirrhosis; 90% of cases involve women
- Sarcoidosis (systemic disease of connective tissue with granuloma formation (skin, lungs and lymph nodes)).
- Sinusoidal
- Chronic hepatitis
- Cirrhosis of the liver (liver shrinkage) (common).
- Steatosis hepatis (fatty liver)
- Postsinusoidal
- Hepatic vein occlusion syndrome (veno-occlusive disorders (VOD))
- Mostly toxic damage from cytostatics (drugs used in cancer).
- Posthepatic block (obstruction is located behind the liver) – about 1% of people suffer from this form.
- Budd-Chiari syndrome (thrombotic occlusion of the hepatic veins) (rare).
- Pericarditis constrictiva (thickening and calcification of the pericardium/”armored heart“).
- Right heart failure (right heart weakness) (common).
In 80% of cases, the cause of portal hypertension is liver cirrhosis. Course and prognosis: The main focus of therapy of portal hypertension is the treatment of the underlying disease and the reduction of portal pressure. The course of portal hypertension depends mainly on the course of the complications that have developed. The main complication of portal hypertension is the development of esophageal varices (esophageal varices), which in the worst case can rupture (rupture). Variceal bleeding is uncommon when portal pressure is < 12 mmHg. Consequently, reducing portal hypertension improves prognosis. Variceal bleeding is often recurrent (recurring) because the cause usually cannot be corrected. Within the first 10 days after the first bleeding, the risk of recurrent bleeding is 35%, and within one year after the first bleeding, the recurrence rate is 70%. The lethality (mortality related to the total number of people with the disease) associated with the first hemorrhage is up to 30%.In other cases, death is due to severe liver cirrhosis or pneumonia (pneumonia).
