What is portal hypertension, why does it arise and what symptoms does it have?

What is portal hypertension, why does it arise and what symptoms does it have?
Photo source: Getty images

Portal hypertension is serious for several reasons. Firstly, the causes that provoke it, and secondly, the complications that arise from it.

Characteristics

Portal hypertension is defined as a persistent increase in blood pressure in the portal bloodstream. This condition is a consequence of other diseases and pathologies in the vascular system or liver. It can even be related to heart disease.

The portal vein basin includes the veins draining into the portal vein, the portal vein itself, the hepatic veins up to the outflow of the hepatic veins into the inferior vena cava.

The portal vein or portal vein is one of the most important veins of the human body. It brings blood from the organs of the digestive system to the liver. This blood is enriched with nutrients, but also with toxins and metabolites.

The portal vein is formed by the connection of three major and large veins, namely the splenic vein (vena splenica/lienalis), the superior and inferior circumflex veins (vena mesenterica superior and/or inferior).

It reaches the liver and passes into it via the porta hepatis. In the porta hepatis it divides into two main branches, namely the right (ramus dexter) and the left (ramus sinister).

Point of entry of the portal vein into the liver = porta hepatis.
Vein leading blood to the liver = vena portae = portacele.

In addition to the main tributaries, smaller veins such as the gastric veins (venae gastricae dextra et sinistra), portal vein (vena pylorica) and pancreatic/subgastric veins (venae pancreaticae) also go to them.

In addition, the liver is also supplied with blood by the hepatic artery (arteria hepatica).

For simplicity: the portal system carries blood from the abdominal organs (spleen, stomach, pancreas, intestine) to the liver. In the liver, important chemical, nutritive substances are extracted from the blood and toxins are broken down.

Up to 80% of the blood to the liver comes through the portal system.
These two vessels are joined by the bile duct, which also goes to the liver.
Together, they form the Glisson triad.

Hypertension = elevated blood pressure. In this case it is an increase in pressure in the portal vein basin of more than 5 mmHg and in severe cases more than 10 mmHg.

This is the pressure gradient and the pressure difference between the portal and systemic circulation. It is also referred to as the portohepatic gradient. The normal value is 4-5 mmHg.

In the hepatic system, blood pressure is lower than in the systemic circulation,
similar to the pulmonary circulation.
Its elevation is caused by several disease states.

These can be present in children as early as after birth in the form of various anatomical abnormalities and congenital defects of the portal vein, liver. During life, we can acquire it due to excessive alcohol intake. One of the causes may be insufficient cardiac function.

This is only one side of portal hypertension. On the other side stand the complications that arise from it. An example is bleeding from the digestive system, which can be life-threatening, as well as fluid accumulation in the body and swelling.

This "morbid" condition is overwhelmingly the result of cirrhosis of the liver, but it can affect children, adults of all genders and the elderly.

Alcoholism is only one of the factors mentioned, albeit a significant one. We can eliminate it by our lifestyle. Abstinence from alcohol is one of the preventive measures. The causes are, of course, multiple and have a diverse basis.

From this point of view, it is important to distinguish the form of portal hypertension.

Division of portal hypertension according to the place of origin and cause:

  • prehepatic - the cause is before the liver, congenital narrowing and defects of the vein, thrombosis, oppression of the vessel
  • hepatic - the cause originates from the liver, cirrhosis, steatosis (narrowing), tumors and cysts of the liver, fibrosis, parasitic diseases
  • posthepatic - behind the liver, specifically between the hepatic veins and the inferior vena cava (heart), right-sided heart failure, vessel narrowing e.g. by tumour

Causes

Portal hypertension results from impaired outflow of blood from the portal vein. The impaired outflow of blood may be caused by an obstruction that partially or, in severe cases, completely obstructs the flow of blood through the hepatic and portal pelvic vessels.

In some cases the blood flow is normal but the blood supply is increased.

The causes are varied. They usually affect adults, but in the case of genetic predispositions and congenital defects, portal hypertension also occurs in children.

There is no significant difference in prevalence between the sexes. It affects the young as well as the elderly.

The main representative is the hepatic cause, specifically liver cirrhosis. Liver cirrhosis arises mainly as a result of excessive alcohol consumption. The functional liver tissue is replaced by fibrous tissue after damage.

Such a reconstruction of the liver parenchyma negatively affects and oppresses the blood vessels in it. Thus, it contributes to an increase in pressure in the portal circulation.

In addition, there are also cases of non-alcoholic liver damage. Hepatitis is cited as the second most common causative agent.

Hepatitis is an inflammation of the liver. The most serious types are viral hepatitis B and C. These are also important precancerous diseases. This means that over time they can lead to liver cancer. All these diseases are involved in increased blood pressure in the portal vein.

Liver failure along with portal hypertension and its complications impair health and life.

Read more in the following articles:
Cirrhosis of the liver
Liver cancer: why does it occur and how does it manifest itself? + Treatment
Steatosis of the liver: what is it, why does it occur and how does it manifest itself? How is the liver treated?
What is infectious jaundice and how is it transmitted? (Hepatitis A/B/C/D)

Sometimes, however, the cause is unknown.

Main causes of portal hypertension in table by form + risk factors

Form of PH Description
Prehepatic
  • The problem is in front of the liver
  • Congenital anomalies and defects of the portal vein and basin, e.g. absence of portal vein, etc. They are already present in young children, after birth in newborns, arterio-venous fistula (connection of an artery with a vein)
  • Cavernous transformation of the portal vein (CTPV from the English Cavernous transformation of the portal vein) is the most common cause of portal hypertension in children
  • Infectious disease of the abdominal cavity, peritonitis, rarely appendicitis or gallbladder
  • Portal vein thrombosis, splenic vein thrombosis
    • Also occur in children and young people with genetic predisposition and bleeding disorders
  • Splenomegaly (enlarged spleen) and hypersplenism (increased blood flow through the portal system)
  • Tumours in the abdominal cavity
Hepatic
  • The problem is in the liver
  • The main presenter is cirrhosis of the liver and alcoholism
  • Viral diseases of the liver - hepatitis B and C
  • Toxic liver damage, drugs, fungi, arsenic, copper
  • Fibrosis of the liver
  • Non-alcoholic steatosis of the liver - non-alcoholic fatty liver disease (NAFLD)
  • Liver cancer and extensive liver metastases
  • Autoimmune inflammation
  • Leukemia, lymphomas
  • Obesity as a risk factor
  • Metabolic syndrome
  • Thrombosis and increased risk of blood clots
  • Family predisposition
  • Recurrent liver disease
  • Gallbladder disease - primary biliary cirrhosis/cholangitis
  • Pancreatic disease - chronic pancreatitis
  • Congenital defects such as congenital fibrosis of the liver
  • Liver cysts, adenomas, angiomyolipomas, lipomas
  • Focal nodular hyperplasia - the second most common benign tumour of the liver
  • Schistosomiasis - parasitic disease
Posthepatic
  • Cause arises behind the liver, between the hepatic veins and the inferior vena cava
  • Thrombosis of the hepatic veins
  • A tumor that presses on the hepatic veins
  • Congenital defects of the vascular system
  • Compression of the inferior vena cava
  • Heart failure - right-sided heart failure
  • Inflammation of the pericardium
  • Injury
  • Budd-Chiari syndrome - thrombosis of the hepatic veins

Symptoms

Symptoms of portal hypertension are based on the restriction of blood flow through a given area - the portal vein.

1. a complication called collateral circulation = risk of severity

One of the most serious problems is the development of collateral circulation.

Simply put, this means that blood bypasses restricted and blocked blood vessels.

With collateral circulation, the blood finds a detour to continue circulating.
In this case, through collateral and collateral veins.

The blood passes through smaller vessels. These become larger. Their vascular wall is not adapted to the increased demands, leaving it vulnerable and weakened.

The result is varicose veins, or varices similar to those in the lower limbs. The risk is that they rupture and bleed acutely. This is a real threat to health and life, even in the case of a paediatric patient.

Where can these varicose veins arise?

Varicose veins in portal hypertension arise according to the basin through which the increased flow has occurred. Examples are esophageal varices and hemorrhoids.

Also known professionally as portosystemic shunts - portocaval shunts / portocaval anastomoses.
In the oesophagus, examples include the veins of the stomach (vena coronaria ventriculi) and the oesophageal veins (venae oesophageae).

In children and adults with advanced disease and abdominal swelling (ascites), a rich and prominent vascular pattern is seen on the abdomen. This prominence of the veins is due to increased flow and accumulation of blood in the collateral collateral circulation.

The prominent veins on the enlarged abdomen resemble the head of a jellyfish.
Accordingly, this symptom also bears the technical name caput medusae.
The subcutaneous veins around the umbilicus are enlarged.

Bleeding from varices is an acute condition because its extent cannot be predicted.

Bleeding from the oesophagus reveals the presence of digested blood in the stool, which is black and smelly. It is professionally referred to as melena.

In intense bleeding of a large extent, a person vomits fresh red blood.

This, together with advanced liver disease (cirrhosis) and impaired blood clotting, may be present. This is why massive haematemesis is one of the most common causes of death in people with portal hypertension.

Rectal bleeding may be present because of haemorrhoids (dilated veins of the anal opening). Professionally, this phenomenon is called haematochezia. Haematochezia means the presence of fresh bright red blood or blood clots in the stool.

The 2nd complication is enlargement of the spleen

Another complication is an enlarged spleen. Splenomegaly leads to bleeding due to thrombocytopenia (platelet disorder). In addition, anemia is associated.

A 3rd complication is the development of oedema

Ascites (swelling of the abdomen) is a symptom of problematic liver function. The same is the case with portal hypertension.

It arises due to the accumulation of blood and increase in pressure in the veins. There is leakage of blood fluid outside the blood vessels. In addition, a considerable amount of blood proteins penetrate into the abdominal cavity in addition to the fluid. This makes the whole condition worse.

Ascites also arises due to other diseases like heart failure, peritoneal cancer, pancreatitis, tuberculosis, dialysis and kidney disease.

The presence of a large and swollen abdomen exacerbates lack of appetite, indigestion - dyspepsia, flatulence (bloating), shortness of breath and difficulty moving or hernia (abdominal muscle hernia).

A major risk is the development of a bacterial infection of the peritoneum. Peritonitis is also a serious condition.

4. a complication is bleeding disorder

A bleeding disorder occurs when the function of the liver is impaired. It results from a deficiency in the production of coagulation factors necessary for normal blood clotting.

+ The situation is aggravated by splenomegaly.

A person may bleed from body orifices. The risk of bleeding from varices is exacerbated by stooling. In addition, the formation of subcutaneous clots - hematomas/bruises - is increased.

5. What are other possible complications of portal hypertension?

Other complications and symptoms:

  • Icterus - yellow discoloration of the skin and eyes (whites of the eyes).
  • spider veins
  • palmar erythema - redness of the skin of the palms of the hands
  • Encephalopathy - Hepatic encephalopathy is due to excessive amounts of toxic substances in the blood that have not passed through the liver. The blood in this case passes through the collateral shunts. It is aggravated by impaired liver function and elimination of toxins - detoxification function of the liver (excess ammonia, glutamine, phenols and others).
    • In encephalopathy, psychological problems such as behavioral changes, confusion, personality and thinking disorders occur.
    • In the severe stage, disorientation to somnolence occurs. The person is excessively drowsy but awakens when addressed. Eventually, the disturbance of consciousness passes into unconsciousness - coma.
      • Also referred to as hepatic coma
  • trembling of the limbs
  • disturbances in mobility
  • renal failure - hepatorenal syndrome
  • hyperesterism - in males, gynecomastia is accentuated - enlarged breasts and testicular atrophy
  • increased susceptibility to infections - the body is exposed to repeated inflammation
  • constipation
  • weight loss, in addition to fluid retention in the body and increasing abdominal swelling
Icterus, jaundice - yellow whites of the eyes as a sign of impaired liver function.
Icterus, i.e. jaundice and yellow whites of the eyes, is one of the symptoms of liver dysfunction. Source: Getty Images

A person may experience subjective discomforts such as:

  • Fatigue
  • malaise
  • lack of appetite
  • feeling of fullness
  • abdominal pain, especially below the right rib cage
  • bloating
  • decrease in libido

Of course, the speed of onset and extent of involvement, as well as portal vein restriction, are influential.
Acute and complete cessation of blood flow through the liver causes liver failure, shock and even death.

Diagnostics

Medical history is the basis for any health problem. A person lists his/her problems in it.

The history is followed by various methods such as laboratory tests after blood collection. Several parameters are evaluated. However, in this case, liver tests (AST, ALT), blood protein (albumin) values, haemocoagulation (blood clotting), blood count to determine anaemia, red blood cell and platelet counts are important.

The following tests are also performed:

  • imaging methods such as USG/SONO
  • + Doppler sonography to determine blood flow through the liver
  • CT or MRI
  • X-ray is performed minimally (more in the past)
  • endoscopy (gastrofibroscopy) is important to detect oesophageal and gastric varices
  • measurement of portal pressure to determine the value
  • liver biopsy
  • objective examination and abdominal examination - presence of enlarged spleen and liver
  • psychiatric examination in case of psychological problems
  • ERCP for gallbladder and biliary disorders
  • internal cardiology examination

Course

The course of the disease depends on several factors.

Slow, protracted and gradually progressive portal hypertension, with good liver function, may not develop enough to manifest itself in the full picture. For a certain period of time, it may be undiagnosed and asymptomatic - asymptomatic.

If the restriction of blood flow occurs rapidly, it is the opposite. Acute onset and severe pressure discharge means a threat to health and life.

Bleeding...

Massive bleeding from esophageal varices is similarly serious. Bleeding and sudden blood loss can lead to shock and even death.

Milder bleeding will present with black stools with associated symptoms of anaemia. Examples include pallor, weakness, lack of appetite or breathlessness and a tendency to faint.

The risk of bleeding is recurrences, i.e. repeated bleeding episodes. Recurrences may occur over the following days, weeks or months.

To what extent? No one knows. Each time, it is potentially life-threatening.
Therefore, in any case, professional examination and treatment of bleeding is necessary, as soon as possible after detection.

Insufficient blood clotting is aggravated by an enlarged spleen and platelet disorder.

The presence of varices is conditioned by increased pressure in the portal canal. Whether they occur in the oesophagus, in the subcutaneous region of the abdomen or in the region of the anus depends on the location and extent of the affection.

Abdominal swelling...

Ascites is another significant sign that something is wrong with the liver. In a small degree, one does not even notice it. However, there are cases where even 20 liters of fluid accumulate in the abdomen.

Of course, even in the case of portal hypertension, a uniform course cannot be determined. It is individual and depends on several factors. Therefore, at the appearance of any difficulties and aggravation of an already present condition, seek professional help.

Hepatic involvement...

Hemorrhagic conditions due to deficiency of hemocoagulation factors, accumulation of toxic substances and the passage of metabolites outside the portal circulation increase the severity.

Bleeding disorder, disturbances of mood, behaviour and personality, disorientation and unconsciousness (hepatic coma).

These are health and life-threatening conditions.
Why do we draw so much attention to this fact?
Do you remember what is the most common cause of portal hypertension and liver dysfunction?
Alcoholism.
And we know an effective prevention against it - ideally, we don't drink alcohol.

How it is treated: Portal hypertension

Can portal hypertension be treated? What does it all involve?

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