Portal Hypertension

What is portal hypertension?

Portal hypertension is elevated blood pressure in your portal vein and the smaller veins that branch off from it — your portal venous system. The portal venous system drains blood from your stomach, intestines, pancreas and spleen into your liver through the portal vein. Your liver filters the blood and then sends it back to your heart and into general circulation in your body.

When something blocks or slows the blood flow through your portal vein, it causes increased pressure throughout your portal venous system. Your body attempts to compensate for this pressure by diverting the blood flow into other veins. The extra blood flow makes these veins expand and makes their walls stretch and weaken. They may leak fluids into your abdomen, and they can also break and bleed.

Who gets portal hypertension?

In Western nations, portal hypertension is most commonly caused by cirrhosis of the liver. That’s when long-term liver disease causes scarring of your liver tissues. Scar tissue obstructs the flow of blood through the portal vein running through your liver. In other areas of the world, a parasite infection called schistosomiasis is the more common cause. Over 230 million people worldwide have this infection.

How serious is portal hypertension?

The complications of portal hypertension can be life-threatening, especially internal bleeding. Not everyone will have these complications, but the risk increases as portal hypertension increases. The greater the pressure, the more enlarged your veins become and the more likely they are to rupture. Portal hypertension is the most common cause of hospitalization and death in people with cirrhosis.

What are the 5 main symptoms of portal hypertension?

You may not have any symptoms until complications develop. Up to 90% of people with cirrhosis already have portal hypertension before they have symptoms. Up to 40% already have large varices (enlarged veins). The first noticeable symptoms of portal hypertension are usually related to new, enlarged, leaky and bleeding veins, such as:

• Blood in vomit.
• Blood in poop.
• Bloated stomach with rapid weight gain (from fluid).
• Swelling in your legs and feet (edema).
• Mental confusion or disorientation.

What are the potential complications of portal hypertension?

Complications include:

• Ascites. When fluid from your enlarged veins leaks into your abdomen, it builds up inside your peritoneal cavity. This causes uncomfortable swelling in your abdomen and can affect your appetite and digestion. In severe cases, it may extend into your chest cavity and interfere with your breathing. It’s also a risk factor for infection in your peritoneal cavity (peritonitis).
• Gastrointestinal bleeding. Enlarged veins in your gastrointestinal tract may rupture and bleed. These are most commonly esophageal varices, which are closest to the surface and have the thinnest walls. Veins in your stomach lining can also become swollen (called portal hypertensive gastropathy) and can bleed in severe cases. Bleeding may lead to anemia.
• Hypersplenism. Reduced blood flow through your spleen causes it to swell with excess blood and to create new blood vessels to accommodate the blood flow. An enlarged spleen can become overactive, removing too many blood cells from circulation. This can lead to reduced blood clotting (low platelet count) and compromised immunity (low white blood cell count).
• Low blood oxygen. In some people, portal hypertension causes blood vessels in their lungs to dilate. This interferes with their lungs’ ability to transfer oxygen to their blood. The result is hypoxemia (low blood oxygen levels). This is known as hepatopulmonary syndrome.
• Kidney failure. Dilated blood vessels resulting from portal hypertension can cause other blood vessels in your body to narrow. If this happens in your kidneys, it can cause kidney failure by restricting your blood supply. This rare complication is known as hepatorenal syndrome.
• Mild cognitive impairment. Your body adapts to portal hypertension by creating new blood vessels that bypass your liver. But when blood bypasses your liver, it doesn’t get filtered as it was supposed to. Toxins accumulate in your blood and eventually affect your mental function. You may have temporary bouts of confusion or disorientation, called hepatic encephalopathy.

What are the causes of portal hypertension?

Causes include:

• Cirrhosis of the liver. This is generally the end stage of chronic liver disease, most commonly viral hepatitis C, alcohol-induced hepatitis and nonalcoholic fatty liver disease.
• Granulomas of the liver. Granulomas are collections of inflammatory cells that accompany various infections and inflammatory diseases (such as schistosomiasis). They can obstruct the portal venous system as benign tumors and they often precede fibrosis (scarring) to come later.
• Blood clots. Thrombosis in the portal venous system can obstruct blood flow into the liver, through the liver or out of the liver. Many things can cause this, including inherited diseases.

How is portal hypertension diagnosed?

The most direct way to diagnose portal hypertension is to measure the pressure in your liver veins, which involves inserting a tiny catheter into a vein. But this is a difficult procedure and not usually necessary. Healthcare providers can recognize portal hypertension by its clinical signs and symptoms. Imaging tests and blood tests can help confirm their suspicions.

What are the clinical signs of portal hypertension?

Your healthcare provider will suspect portal hypertension if you have:

• Enlarged varices.
• Enlarged spleen.
• Gastrointestinal bleeding.
• Ascites (fluid in your abdomen).
• Signs of mild cognitive impairment.
• Low blood cell counts.
• Easy bleeding and bruising.

What tests will be done to diagnose this condition?

• Blood tests can reveal a lot about what’s going on in your organs. A comprehensive metabolic panel can show signs of kidney and liver dysfunction. A complete blood count can show if your spleen has become overactive in removing white blood cells or platelets. It can also show certain blood disorders that might cause portal hypertension or blood loss from internal bleeding.
• Imaging tests can help identify cirrhosis, ascites, enlarged organs and enlarged or new veins in your portal venous system. In particular, a Doppler ultrasound can produce images of blood moving through your veins. It can identify obstructions and abnormal widening or narrowing.
• Endoscopy. If you have signs of gastrointestinal bleeding, your healthcare provider will need to investigate with endoscopy. An upper endoscopy is a procedure in which a physician examines your esophagus, stomach and duodenum by inserting an endoscope, which is a long tube with a camera at the end. They’ll be looking for varices as well as signs of active or recent bleeding. If they find bleeding, they can usually stop it using tools inserted through the endoscope.

What are the treatment options for portal hypertension?

Portal hypertension itself may or may not be reversible, depending on its cause. If there’s an infection or a blood clot that your healthcare provider can treat with medication, portal hypertension can improve and sometimes resolve over time. However, significant scar tissue, such as in the case of cirrhosis, usually won’t reverse and leads to persistent portal hypertension. Most treatment is aimed at managing the complications of portal hypertension. Your healthcare provider will address emergencies first, then move on to longer-term solutions.

First-line treatments to stop gastrointestinal bleeding include:

• Endoscopy. Your healthcare provider will treat acute bleeding with endoscopic therapy. Endoscopic methods include sclerotherapy and banding. Sclerotherapy means injecting a solution into the bleeding varices to control the bleeding. Banding means tying off bleeding varices with tiny rubber bands.
• Medication. Your healthcare provider may prescribe medications in combination with endoscopic therapy to reduce the pressure in your varices and reduce the risk of recurrent bleeding. Beta-blockers can reduce portal pressure, while vasoconstrictors can help reduce dilated blood vessels.

Other first-line treatments for complicated portal hypertension may include:

• Oxygen therapy for hepatopulmonary syndrome.
• Dialysis for hepatorenal syndrome.
• Medication to treat hepatic encephalopathy.
• Paracentesis to remove excess fluid from ascites and test it for peritonitis.

When bleeding or other complications persist, healthcare providers may move on to second-line treatments.

Two different shunt procedures are sometimes performed to help redirect blood flow through your portal venous system and relieve pressure.

• Transjugular intrahepatic portosystemic shunt (TIPS): In this nonsurgical procedure, a physician called an interventional radiologist uses X-ray imaging to guide the placement of a stent in your liver. They use a needle to tunnel through your liver and connect the portal vein with one of your hepatic veins, diverting blood flow and relieving pressure. They place a stent to keep the tunnel open. This procedure can be highly effective, but over time there’s a small risk of the stent malfunctioning, requiring a repeat procedure to fix it.
• Distal splenorenal shunt (DSRS): This surgical procedure may be a better alternative for some people. Although you have to be in good enough health to safely undergo the surgery and recovery, it may be more effective in the long term for controlling your disease. The procedure disconnects your splenic vein from your liver and diverts it to your left kidney vein instead, selectively reducing blood flow and pressure through both your liver and your spleen.

Finally, some people with advanced liver disease may be candidates for a liver transplant.

What kind of follow-up care will I need?

All of these treatments require extensive follow-up care to keep an eye on your condition. You’ll see your healthcare provider frequently in the first year after treatment to make sure the treatment is still working and that no new complications have developed. After that, you’ll continue to see them at least twice a year for imaging tests to look at your liver and blood tests to check your liver function.

How can I prevent portal hypertension?

The best way to prevent portal hypertension is to try and stop the progress of chronic liver disease when you can. Not all causes are in your control, but some of the most common ones are — if you discover them soon enough. Liver disease caused by alcohol use or by metabolic disease can often be improved by dietary and lifestyle changes. Hepatitis C can be cured with antivirals. Make sure to get screened at least once.

What can I expect if I have this condition?

Your outlook depends on how severe your portal hypertension is and whether you have bleeding varices or other complications. The risk of death from your first episode of bleeding may be as high as 40%. If bleeding recurs after treatment, the risk of death from your next episode is close to 30%. Your risk is higher if you have more advanced cirrhosis. It’s lower if your liver disease is still early enough to control.

How do I take care of myself while living with portal hypertension?

Keep in touch with your healthcare team. Make sure to make all of your checkup appointments and take your medications as directed. Check with your provider before taking new medications, and reduce or eliminate alcohol. You may need to maintain a low-sodium diet, or a more personalized diet plan. You may also have other related conditions that you can help control with diet and lifestyle changes.

A note from Cleveland Clinic

Portal hypertension affects so many different organs that treating it can feel like putting out multiple fires. It may come as an unpleasant surprise to learn that liver disease can affect veins in your stomach, esophagus, lungs and kidneys. This is especially true if you’ve had no symptoms or signs of liver disease until now. But catching it earlier will give you the advantage in controlling portal hypertension.