Portal hypertension

Overview

Portal hypertension means high blood pressure in the system of veins that carry blood from your digestive organs to your liver. This system, called the portal venous system, includes the large portal vein and smaller veins connected to it. Portal hypertension happens when blood gets blocked from or has trouble flowing through the liver.

A healthy liver filters blood, then returns it to the heart. But increased pressure in the portal venous system can force blood into other veins. This can lead to serious complications.

Portal hypertension can range from mild to severe. How severe it is depends on pressure levels in the portal venous system and the health of the liver. Mild portal hypertension may cause few or no symptoms and may be found during testing for another reason. Severe portal hypertension raises the risk of complications, such as bleeding in the digestive system, fluid buildup in the belly, and changes in thinking or alertness.

Some portal hypertension can be reversed, depending on the cause and how early it's found and treated. But most portal hypertension is caused by cirrhosis and cannot be cured or fully reversed. Ongoing treatment is needed to lower pressure and prevent and manage complications as well as treat the underlying cause.

Types

Healthcare professionals may describe portal hypertension based on where blood flow slows down or is blocked.

• Prehepatic portal hypertension. This is when blood flow slows or gets blocked before it reaches the liver. A common cause is a blood clot in the portal vein, called portal vein thrombosis.
• Intrahepatic portal hypertension. This is when blood has trouble moving through the liver itself.
• Posthepatic portal hypertension. In this type, blood flow slows down after leaving the liver.

A less common type is left-sided portal hypertension, also called sinistral portal hypertension. This rare type is high pressure that's mainly in the vein that drains the spleen, called the splenic vein. Left-sided portal hypertension is different from other portal hypertension in that the blockage usually happens because of pancreatic disease, such as pancreatitis, or surgery on the pancreas, not liver disease or cirrhosis. If left-sided portal hypertension is causing bleeding, the main treatment is to remove the spleen, called splenectomy, which usually cures the condition.

Symptoms

Portal hypertension causes no symptoms at first. Symptoms usually start when the condition leads to complications. This often is a result of blood being forced out of the portal venous system into other veins and organs.

Symptoms and visible signs that may suggest portal hypertension complications include:

• Swelling in the belly or legs. Fluid buildup in the belly, called ascites, can cause the abdomen to look or feel swollen. Fluid also can build up and cause swelling in the legs, called edema.
• Blood in vomit or bloody stool. Blood in vomit may look like blood or coffee grounds. Blood in stool can be red or darker, even black. Bloody stool or vomit can happen if you have bleeding in your digestive system.
• Bleeding that is not typical; easy or a lot of bruising; or tiny dots on the skin that may look red, purple or brown. The tiny dots are called petechiae. All are possible symptoms of thrombocytopenia, which can happen if blood builds up in the spleen causing an enlarged spleen. A healthcare professional may feel an enlarged spleen during an exam or see it on an imaging test. The condition also can cause low white blood cell counts.
• Blood vessels visible on the skin of the belly. This can happen when blood that isn't able to move through the portal venous system in the liver finds other paths to travel.
• Changes in thinking or alertness. Trouble focusing, confusion or changes in sleep may happen when the brain is affected by toxins that the liver hasn't been able to filter out. This is called hepatic encephalopathy.

It's important to watch at home for these symptoms of portal hypertension complications and seek medical care when needed.

When to seek care

Contact your healthcare professional right away for:

• New or worsening symptoms mentioned earlier.
• Weight gain or loss outside the limits of what your health professional has said is OK.
• Trouble breathing.

When to seek emergency care

Call your healthcare professional or go to the nearest emergency medical care facility right away for:

• Vomiting blood.
• Bloody, black or dark-colored stool.
• Swelling of the belly, along with a fever, belly pain or confusion. This can mean there's fluid in the belly that's infected.

Causes

Portal hypertension is high blood pressure in the portal vein, which carries blood from your digestive organs to your liver. It happens when blood flow is blocked or slowed down.

The two most common causes of portal hypertension worldwide are scarring of the liver, called cirrhosis, and a parasitic infection of the liver called schistosomiasis. In Western countries, more than 90% of portal hypertension is caused by cirrhosis. Cirrhosis makes it hard for blood to flow through the liver.

The causes of portal hypertension can be grouped by where the blockage or slowing of blood flow happens. This could be in an area before blood reaches the liver, inside the liver or after blood leaves the liver.

Before blood reaches the liver, also called prehepatic

Some causes of a blockage in the portal vein, before blood reaches the liver include:

• A blood clot in the portal vein, called portal vein thrombosis.
• A blood clot in the vein that carries blood from the spleen to the liver.

In children, blood clots can happen from:

• Infections, such as sepsis or an infection in a newborn's umbilical cord stump.
• Dehydration.
• Changes in the anatomy of the portal vein that are found at birth, such as the vein being too narrow.

Inside the liver, also called intrahepatic

This is the most common area where blockages happen, most often caused by cirrhosis.

Some causes of intrahepatic portal hypertension include:

• Cirrhosis. In children, cirrhosis can be caused by autoimmune hepatitis, biliary atresia, chronic viral hepatitis and metabolic liver diseases. In adults, causes can include long-term alcohol use disorder, hepatitis B, hepatitis C and liver diseases, such as hemochromatosis, metabolic dysfunction-associated steatotic liver disease — formerly known as nonalcoholic fatty liver disease — primary biliary cholangitis and Wilson's disease.
• Conditions such as sarcoidosis and amyloidosis, which can cause cirrhosis.
• Infections such as schistosomiasis, which can cause cirrhosis.
• Other conditions that may not cause cirrhosis but can lead to portal hypertension.

You may hear intrahepatic portal hypertension described as:

• Presinusoidal portal hypertension. This means the blood flow is blocked before blood reaches certain small blood channels inside the liver called the sinusoids. This is typically not caused by cirrhosis but by other liver conditions or infections.
• Sinusoidal portal hypertension. This means the blood flow is blocked at the point of the sinusoids in the liver. Cirrhosis is a sinusoidal cause of portal hypertension.
• Postsinusoidal portal hypertension. This means the blood flow is blocked after blood has reached the sinusoids in the liver.

After the liver, also called posthepatic

Some causes of blockages after blood leaves the liver can include:

• Budd-Chiari syndrome.
• A condition that affects the sac around the heart, called constrictive pericarditis.
• Serious heart conditions, such as cardiomyopathy.
• A blockage, such as a blood clot, in the vein that carries blood to the heart.

If a cause cannot be found, you may hear that referred to as idiopathic.

Risk factors

Different factors can increase the risk of portal hypertension. Some you may be able to change, whereas others you cannot.

Factors you can change

• Heavy drinking. This can contribute to liver damage and cirrhosis, the main cause of portal hypertension.
• Infections. Ongoing infections with hepatitis B or hepatitis C can damage the liver and lead to cirrhosis and portal hypertension.
• Conditions that contribute to liver damage. When combined, conditions such as diabetes, steatotic liver disease — formerly called fatty liver disease — high cholesterol and high blood pressure can increase the risk of liver damage and portal hypertension.

Factors you cannot change

• Inherited conditions. Liver conditions passed down in families, such as hemochromatosis and Wilson's disease, increase the risk of liver damage and portal hypertension.
• Changes in the portal vein that are found at birth. Sometimes, a person is born with portal vein anatomy that increases the risk of portal hypertension. For example, the portal vein may be too narrow.

Complications

Portal hypertension often is noticed only after complications start.

Complications can include:

• Fluid buildup in the belly, also called ascites. When the liver is damaged, such as with portal hypertension, the kidneys hold excess salt. The salt causes the body to hold onto water, and fluid builds up in the belly. This complication called ascites. With portal hypertension, fluid also may leak out of blood vessels into the belly. This happens because of the increased pressure in the portal vein system, Sometimes, ascites can become infected, called spontaneous bacterial peritonitis.

• Enlarged veins, also called varices, and bleeding. Increased blood pressure in the liver's portal vein system can force blood to seek other routes. This includes the veins of the esophagus, stomach and intestines. When pressure in those veins increases, it can cause the veins to enlarge, a condition called varices. Varices in the esophagus are called esophageal varices. Varices in the stomach are called gastric or stomach varices.

  Varices are fragile due to their thin walls. This increases the risk of bleeding. When varices bleed, blood can build up in the stomach. Larger varices are more likely to bleed. Spots on the skin over varices also suggest a higher risk of bleeding. Sometimes varices form in the rectum, where they can bleed. This is not the same as hemorrhoids.

• Changes in thinking or alertness. When the liver can't filter toxins from the blood, such as ammonia from the intestines, the toxins can build up and affect how the brain works. This is called hepatic encephalopathy. The condition can lead to confusion, changes in personality, trouble concentrating and, when severe, coma.
• Stomach and colon changes and bleeding. Pressure from portal hypertension can lead to more blood in blood vessels in the lining of the stomach and large intestine, also called the colon. This buildup of blood can cause those blood vessels to swell and bleed. The bleeding may be slow and ongoing. It's not usually a cause of a large amount of bleeding, but the bleeding can cause anemia. In the stomach, this is called portal hypertensive gastropathy. In the colon, it's called portal hypertensive colopathy.
• Enlarged spleen and low platelet count. The spleen acts as a filter for blood. When portal hypertension causes blood to back up and pool in the spleen, the spleen can swell in size, called splenomegaly. As the spleen enlarges, it can trap and destroy more blood cells, including platelets. This leads to a low platelet count, called thrombocytopenia. This condition can limit how well the blood clots and increase the risk of bleeding.
• Kidney failure. Portal hypertension is not the same as kidney failure. But high blood pressure in your liver from portal hypertension, along with fluid in the belly, called ascites, can contribute to kidney failure. This is called hepatorenal syndrome. Portal hypertension causes the blood vessels in your belly to widen. This can affect blood flow and mean your kidneys don't get enough blood. In addition, if portal hypertension leads to ascites, that fluid can become infected and trigger kidney failure.
• Low oxygen in the blood. High blood pressure in your liver from portal hypertension sometimes leads to the blood vessels in your lungs enlarging. These changes in the lungs make it hard for red blood cells to take in oxygen. Then the lungs can't send enough oxygen to the body. This condition is called hepatopulmonary syndrome.

Prevention

Taking steps to prevent or manage liver disease can help prevent complications such as portal hypertension.

• Limit or avoid alcohol. This can reduce the risk of liver disease, cirrhosis and other complications such as portal hypertension.
• Prevent infections such as hepatitis B or hepatitis C. This can reduce the risk of liver disease and cirrhosis, which can lead to portal hypertension.
• Manage existing conditions. These include liver conditions, such as hepatitis B, hepatitis C and steatotic liver disease — formerly called fatty liver disease — as well as high cholesterol and high blood pressure. Managing these conditions with lifestyle changes and medicines may help prevent complications, such as portal hypertension.

Diagnosis

Healthcare professionals often diagnose portal hypertension by asking about your health history and doing a physical exam. A physical exam can show signs such as fluid buildup in the belly or an enlarged spleen. If you have known risk factors for portal hypertension, such as cirrhosis, as well as symptoms, that may be all that's needed to make the diagnosis.

Sometimes, your health professional may order blood tests and imaging tests. If the diagnosis isn't clear, portal vein pressure may be measured directly to find out how high it is. This is not often done because the test is invasive.

Blood and lab tests

Blood tests can show changes linked to portal hypertension.

• Complete blood count (CBC). This test measures the number of different cells in your blood, including platelets. Portal hypertension can enlarge the spleen, which may lower platelet counts. Low platelet levels, called thrombocytopenia, can suggest portal hypertension.
• Liver function tests. These blood tests check how well the liver is working. Results outside the standard range may suggest a liver disease that can cause portal hypertension.
• Other tests. You may have other lab tests, for example, to find out whether fluid in the belly, called ascites, is caused by portal hypertension or something else. A measurement called the serum-ascites albumin gradient (SAAG) can help tell this by comparing the level of a protein called albumin in the blood with the level of albumin in the fluid in the belly. A gradient measurement of 1.1 grams per deciliter (g/dL) or more means the fluid is likely caused by portal hypertension.

Blood and lab test findings help guide the need for more testing, but they do not confirm portal hypertension on their own.

Imaging tests

Imaging helps show the effects of increased pressure in the portal vein system.

• Ultrasound. An ultrasound of the belly, called an abdominal ultrasound, can show an enlarged spleen, fluid in the belly and changes in the size of the portal vein. These findings may suggest portal hypertension. A special type of ultrasound called Doppler ultrasound shows the direction and speed of blood flow in the portal vein and nearby vessels. Slowed or altered blood flow patterns may suggest portal hypertension. However, ultrasound does not directly measure portal pressure.
• Upper endoscopy. This test allows a healthcare professional to look directly at the esophagus and stomach using a flexible tube with a camera. This test can find enlarged veins called varices.
• CT scan. This test shows detailed cross-sectional images, also called slices, of the liver and surrounding blood vessels. An enlarged spleen or new, enlarged or twisting veins, called collateral veins or varices, are possible signs of portal hypertension. CT also may show the underlying cause, such as scarring of the liver called cirrhosis.
• Magnetic resonance imaging (MRI). This test shows detailed images of soft tissues, including the liver and blood vessels. It also can show enlarged veins, changes in blood flow and other features linked to portal hypertension. A test called magnetic resonance elastography also might be done. This test can measure liver tissue stiffness to help show whether portal hypertension is high enough to cause complications. Portal hypertension that can cause complications also is called clinically significant.

Imaging tests show different signs that suggest portal hypertension. But they do not directly measure how high the pressure is.

Measuring portal pressure

If a diagnosis of portal hypertension isn't clear, portal pressure may be measured in one of two ways.

• Indirect method. This is done using a tube called a catheter that's placed into the veins that move blood from the liver to the heart rather than in the portal vein itself. During the test, the pressure is measured both when blood is blocked by a small balloon at the end of the catheter and when blood is not blocked by the balloon. The difference between those two numbers is called the hepatic venous pressure gradient (HVPG). It is an indirect estimate of pressure in the portal vein.

  Portal hypertension is defined as an HVPG greater than 5 millimeters of mercury (mm Hg). When the HVPG reaches 10 mm Hg or more, the risk of complications increases. An HVPG greater than 12 mm Hg is linked to a higher risk of bleeding from enlarged veins.

• Direct method. Though much less common, direct measurement of portal pressure may be done by placing a catheter into the portal vein.

How serious is portal hypertension?

Portal hypertension ranges from mild to severe. How severe it is depends on how high the pressure is and whether complications have happened. Lower pressure levels may not cause symptoms. Higher pressures, especially above 10 to 12 mm Hg, are linked to a greater risk of conditions such as bleeding or fluid buildup.

Treatment

Treating portal hypertension includes managing the underlying causes. It also includes ongoing treatment to prevent and manage complications, such as bleeding in the digestive system and fluid in the belly. This treatment is done with medicines, endoscopy procedures or both.

When medicines and endoscopy aren't enough to manage symptoms and complications, treatments may include other procedures or surgery. This may include a procedure to place a shunt that redirects blood flow in the liver or transplant surgery to replace the diseased liver with a donated liver.

Diagnosis

Can portal hypertension be cured?

If the underlying cause of portal hypertension is reversible, the liver may heal and reverse or cure the portal hypertension. For example, if viral hepatitis is the cause, treating it may resolve both conditions. If a liver transplant is done for advanced liver disease, the donated liver also could cure the underlying cause. Medicines and other procedures do not cure portal hypertension. But they can help protect the body from further damage and complications and improve some symptoms.

Treatment

Medicines

• Medicines to lower blood pressure. Beta blocker medicines can be used to reduce the risk of bleeding varices. They work to lower blood pressure in the portal vein by slowing the heart rate and widening blood vessels. They may not be safe to use if you have ascites or an infection of ascites called spontaneous bacterial peritonitis. Beta blocker medicines include propranolol (Inderal LA, Innopran XL), carvedilol (Coreg) and nadolol.
• Medicines to slow blood flow into the portal vein. Vasoconstrictor medicines such as octreotide (Sandostatin) and vasopressin (Vasostrict) taken for several days may help treat sudden, also called acute, bleeding varices. They can help by tightening widened blood vessels.
• Medicines to remove sodium and water from the body. These medicines, called diuretics, may prevent or reduce extra fluid buildup in the body called ascites. Diuretics can be given as pills or through a tube placed in a vein (IV). Your healthcare professional also may suggest limiting sodium in your diet to help prevent fluid buildup.
• Medicines to fight infection. To treat or prevent infection of ascites, called peritonitis, you may be given antibiotic medicines either in pill form or by IV.
• Medicines to treat changes in thinking and alertness. If portal hypertension causes a buildup of toxins in the brain, called hepatic encephalopathy, it can affect personality, thinking and alertness. Medicines such as lactulose and rifaximin can help prevent or reduce ammonia and other toxins from building up.

Procedures and surgery

• Endoscopy. Endoscopy involves inserting a flexible tube with a tiny camera, called an endoscope, through the mouth and down the throat. It allows a view of the esophagus, stomach and beginning of the small intestine. Endoscopy looks for enlarged veins called varices. If found, varices are measured and checked for red streaks and red spots, which usually suggest a high risk of bleeding. When bleeding risk is high or bleeding is already happening, a procedure called band ligation can help prevent and treat it.
• Endoscopic band ligation. Using an endoscope, a healthcare professional uses suction to pull the varices into a chamber at the end of the scope and wraps them with an elastic band. This essentially "strangles" the veins so they can't bleed. Endoscopic band ligation carries a small risk of complications, such as bleeding and scarring.

• Transjugular intrahepatic portosystemic shunt (TIPS). You may have a TIPS procedure to stop bleeding if it isn't stopped by medicine or endoscopy. This procedure is not a traditional surgery. Instead, it uses imaging technology and tools guided through blood vessels. The procedure reroutes — also called shunts — blood from the portal vein to a hepatic vein. Hepatic veins carry blood from the liver to the heart. This is done by placing a small hollow tube called a stent. It decreases pressure in the varices and often stops bleeding.

  TIPS can cause serious complications, including liver failure and mental confusion. These symptoms can happen when toxins that the liver typically would filter are passed through the shunt directly into the bloodstream. TIPS is mainly used when all other treatments have failed or as a temporary measure in people awaiting a liver transplant.

• Surgical shunts. Sometimes, though rarely, a shunt may be placed through traditional surgery instead of the TIPS procedure. This might be done, for example, in someone who has portal hypertension not caused by cirrhosis with bleeding that's hard to manage. It also might be done in someone who can't have a TIPS procedure due to technical reasons.
• Liver transplant. Liver transplant may be an option for some people, for example, those with severe liver disease or who have bleeding of varices again and again. Although liver transplantation is often successful, the number of people awaiting transplants far outnumbers available organs.

Portal hypertension is a serious condition. The outlook, also called prognosis, and how it affects life expectancy mainly depends on the underlying cause and how bad the liver damage is. For example, idiopathic portal hypertension that's not caused by cirrhosis has a good prognosis when complications are managed well.

However, portal hypertension usually is a result of cirrhosis or other liver disease. Because of this, the prognosis for most portal hypertension is closely tied to how the underlying liver disease progresses and is managed.

The risk of death from sudden, also called acute, bleeding varices ranges from 15% to about 40% within six weeks of the acute bleed. Treatment helps reduce the risk of further bleeding. But more than 60% of people do not survive five years beyond the first bleed. Ultimately, those with progressive liver disease and many varices need a liver transplant.

Lifestyle and home remedies

If you have portal hypertension, lifestyle changes may help protect the liver and reduce the risk of portal hypertension complications:

• Don't drink alcohol. Even a single glass of alcohol can damage your liver further. This is true for all types of alcoholic drinks.
• Eat a low-sodium diet. Extra salt can cause your body to hold on to fluids. This can worsen swelling in your belly and legs. Use herbs rather than salt to season your food. Choose prepared foods that are low in sodium.
• Protect yourself from infections. If you have cirrhosis, your body can have trouble fighting off infections. Protect yourself by avoiding people who are sick and washing your hands often. Get vaccinated, for example, for hepatitis A and B, influenza, and pneumonia.
• Use nonprescription medicines carefully. Liver damage makes it harder for your liver to process medicines. For this reason, ask a healthcare professional before taking any medicines, including those you can get without a prescription. Do not take ibuprofen (Advil, Motrin IB, others), aspirin or aspirin-containing products unless your care team tells you. A health professional may recommend that you take acetaminophen (Tylenol, others) in low doses for pain relief.
• Follow your healthcare team's instructions. Follow any diet recommendations, take medicines as prescribed, and get your blood tests and medical exams at the recommended times. Also watch for signs of complications and take action as advised by your care team.

Preparing for an appointment

If you have portal hypertension, you may be referred to a doctor who specializes in the digestive system, called a gastroenterologist, or a doctor who is an expert on the liver, called a hepatologist.

Here's some information to help you get ready for your appointment and what to expect.

What you can do

• Be aware of anything you need to do ahead of time, such as diet restrictions on the day before your appointment.
• Write down your symptoms, including when they started and how they may have changed or worsened over time.
• Take a list of all your medicines, as well as any vitamins or supplements, including the doses.
• Write down your key medical information, including other diagnosed conditions.
• Bring results of medical tests done so far, including digital copies of ultrasound, CT or MRI images and biopsy slides if you've had a liver biopsy.
• Write down key personal information, including any recent changes or stressors in your life.
• Take along a family member or friend to help you remember things.
• Write down questions to ask your healthcare professional.

Questions to ask your doctor

Preparing a list of questions can help you make the most of your time. Some basic questions to ask include:

• What's likely causing my symptoms?
• What tests do I need?
• What is most likely causing my portal hypertension?
• Is there a way to slow or stop my liver damage?
• What are my treatment options?
• What are the side effects of treatments?
• Are my symptoms likely to get worse, and what can I do to help prevent that?
• What signs and symptoms of complications should I watch for?
• I have other health conditions. How can I best manage them together?
• Are there medicines or supplements that can hurt my liver?
• What foods should I eat or avoid?
• What's the right level of physical activity for me?
• Should I see a specialist?
• How often should I schedule appointments to check on my health?

Don't hesitate to ask additional questions during your appointment.

What to expect from your doctor

Be prepared to answer questions, including:

• When did you first begin having symptoms?
• Do your symptoms happen all the time or do they come and go?
• How bad are your symptoms?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to worsen your symptoms?
• How often do you drink alcohol?
• Have you been exposed to or taken toxic drugs?
• Do you have a family history of liver disease, hemochromatosis or obesity?
• Have you ever had viral hepatitis?
• Have you ever had jaundice?
• Have you ever had a blood transfusion or used injected drugs?
• Do you have any tattoos?