Portal hypertension is a potential complication of primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis. If you’re living with PBC, it’s important to know about portal hypertension, increased blood pressure in what’s called your portal vein. Studies have found that portal hypertension can be used to predict how your PBC progresses and potential PBC complications you may experience.

Continue reading to learn more about portal hypertension, how it’s treated, and how you can prevent it.

What Is Portal Hypertension?

Portal hypertension refers to high blood pressure in your portal vein. Your portal vein is a large blood vessel in your abdomen that carries the blood from your gastrointestinal tract — that is, your digestive system, including your stomach, intestines, pancreas, and spleen — to your liver. Your liver cleans and filters this blood before it goes to your heart to be circulated around your body.

When the blood pressure in your portal vein is higher than normal, your blood has difficulty flowing to your liver. Your body may compensate for the increased pressure by redirecting blood away from your liver and into blood vessels in your esophagus and stomach. The increased blood flow may be too much for these blood vessels, causing them to stretch and weaken. These enlarged veins are known as varices.

How Can PBC Cause Portal Hypertension?

Portal hypertension can occur when something blocks or slows the blood flow through your portal vein. The most common cause of portal hypertension is cirrhosis, which is permanent advanced scarring that interferes with liver function.

In people with PBC, inflammation can damage the bile ducts found throughout your liver, causing them to die. As more liver cells die, they’re replaced with scar tissue. When your bile ducts are damaged, bile backs up into your liver instead of going to your gastrointestinal tract to help with digestion. Over time, the bile can cause more liver inflammation and fibrosis — the formation of scar tissue. Fibrosis can accumulate with time, leading to cirrhosis. This scar tissue can block the normal blood flow through the portal vein, increasing the pressure in the vein and causing blood to back up.

Although cirrhosis is the primary cause of portal hypertension, there’s evidence that some people with PBC develop portal hypertension without first having cirrhosis. A 2021 study found that out of 86 people with PBC, 82 percent had portal hypertension without cirrhosis.

People without PBC can develop portal hypertension if they have chronic liver disease that causes cirrhosis of the liver, such as nonalcoholic fatty liver disease, hepatitis C, or alcohol-induced hepatitis. Other than cirrhosis, some parasitic infections or blood clots in the liver or portal vein can also result in portal hypertension.

What Are the Signs and Symptoms of Portal Hypertension?

You may not notice any symptoms of portal hypertension in the early stages. Additionally, some symptoms may be similar to other PBC symptoms.

The first symptoms most people notice are related to the increased blood flow into new blood vessels in your stomach and esophagus. These blood vessels may not be able to handle the increased blood flow and may leak, break, or bleed, causing symptoms such as:

• Bloody vomit
• Bloody stool (poop)
• Ascites (fluid build-up in the abdomen)
• Rapid weight gain (due to fluid build-up), along with bloated stomach
• Edema (swelling in the legs and feet)

Other signs and symptoms you and your health care provider may see if you have portal hypertension include:

• Hepatomegaly (enlarged liver)
• Splenomegaly (enlarged spleen)
• Esophageal varices (enlarged veins in your esophagus)
• Gastric varices (enlarged veins in your stomach)
• Weight loss (due to malnutrition)
• Jaundice (yellowing of the skin and eyes)
• Hepatic encephalopathy (problems with mood, thinking, and coordination)
• Kidney problems
• Thrombocytopenia (low platelet count)
• Spontaneous bacterial peritonitis (a severe infection in the peritoneal cavity, located in the abdomen)
• Low blood oxygen levels

How Is Portal Hypertension Diagnosed?

If you’re living with PBC, your health care provider will likely monitor you regularly for signs of portal hypertension, especially if you have an advanced stage of PBC. If you notice any of the symptoms of portal hypertension, it’s important to talk to your health care provider right away so they can order additional tests to check for portal hypertension.

Your provider will initially use a combination of physical exam, blood tests, imaging tests, and/or endoscopy to make the diagnosis.

Blood tests can check your kidney and liver function. One of the most common signs of portal hypertension is a low platelet count.

Imaging tests, such as ultrasound or computed tomography (CT) scans, allow your provider to see how blood is moving through the veins of your liver and portal vein. Imaging tests can also detect cirrhosis, splenomegaly, varices, and ascites.

Endoscopy allows your provider to look inside your upper digestive tract. During this test, your provider will look for varices and bleeding using a tool called an endoscope. An endoscope is a flexible tube with a camera on the end that allows providers to look inside your esophagus, stomach, and the first part of your small intestine.

If, after these tests, your diagnosis remains unclear, your doctor may use a more invasive approach — measuring the pressure in your portal vein by inserting a small catheter into a vein in your neck.

How Is Portal Hypertension Treated?

Treatment for portal hypertension depends on which complications you have, but it may include lifestyle changes, medications, and medical procedures.

Lifestyle Changes

Limiting your salt intake can help you manage mild fluid buildup in your abdomen, feet, and legs. Reducing the amount of salt in your diet can be difficult. Some tips that may help you reduce your dietary salt intake include the following:

• Don’t use the salt shaker at the table.
• Avoid processed foods, including fast food.
• Try lemon juice or Mrs. Dash seasoning instead of salt.
• Use fresh herbs and spices to flavor your food.

Medications

Your provider may prescribe medication called nonselective beta-blockers to improve how well your heart and blood vessels work and to decrease portal pressure. Taking a nonselective beta-blocker can help decrease your risk of bleeding due to varices. Examples of beta-blockers you may take for portal hypertension include propranolol, nadolol (Corgard), and carvedilol (Coreg).

If you’re in the hospital and bleeding from a swollen vein, your health care provider may use a medication called octreotide (Sandostatin) to help stop the bleeding. Octreotide works by decreasing blood flow to your gastrointestinal tract.

People with ascites may take diuretics to help get rid of extra fluid through the urine.

If you have hepatic encephalopathy, your provider may prescribe a medication called lactulose. Lactulose can help you eliminate some of the waste from your body that can cause mental confusion.

If you have spontaneous bacterial peritonitis, your provider will prescribe antibiotics like ciprofloxacin (Cipro).

Medical Procedures

Several different medical procedures may help manage portal hypertension.

Banding and shunting can help prevent or stop bleeding from varices that have burst. Banding uses tiny rubber bands to tie off the bleeding varices during endoscopy. Shunting helps to open your portal vein and improve blood flow using a stent — a small, mesh tube designed to hold open blood vessels.

Shunting can be done with or without surgery. Transjugular intrahepatic portosystemic shunt (TIPS) is a nonsurgical procedure that involves using a tube to divert blood flow from the portal vein to a hepatic vein to relieve some pressure. A stent is placed to keep the vein open.

Distal splenorenal shunt (DSRS) is a surgical procedure that diverts blood flow from the liver to a kidney to help reduce pressure.

Additional medical procedures depend on your complications. If you have kidney problems, you may need dialysis — a procedure that filters your blood through an artificial kidney. You may need oxygen therapy if you have low blood oxygen levels. If you have ascites, your provider may remove excess fluid with paracentesis — a procedure that uses a needle and a plastic tube to drain excess fluid.

If you have advanced liver failure, you may be eligible for a liver transplant. During a liver transplant, your liver is replaced with another healthy liver.

Can Portal Hypertension Be Prevented?

You may not be able to prevent portal hypertension. The best way to prevent it and its complications is to follow your PBC treatment plan. Your PBC treatment may include medications such as ursodiol — also known as ursodeoxycholic acid (UDCA) — and obeticholic acid (Ocaliva). Follow up with your health care provider about the best treatment plan for you.

Find Your Team

On myPBCteam, the social network for people with primary biliary cholangitis and their loved ones, members come together to ask questions, give advice, and share their tips with others who understand life with primary biliary cholangitis.

Have you been diagnosed with portal hypertension with PBC? What treatments have you tried? Share your experiences in the comments below, or start a conversation by posting on your Activities page.