Portal hypertension is an increase in the pressure within the portal vein (the vein that carries blood from the digestive organs to the liver). The increase in pressure is caused by a blockage in the blood flow through the liver.

Increased pressure in the portal vein causes large veins (varices) to develop across the esophagus and stomach to bypass the blockage. The varices become fragile and can bleed easily.

What causes portal hypertension?
The most common cause of portal hypertension is cirrhosis, or scarring of the liver. Cirrhosis results from the healing of a liver injury caused by hepatitis, alcohol abuse, or other causes of liver damage. In cirrhosis, the scar tissue blocks the flow of blood through the liver and slows its processing functions.

Portal hypertension may also be caused by thrombosis, or clotting in the portal vein.

What are the symptoms of portal hypertension?
The onset of portal hypertension may not always be associated with specific symptoms that identify what is happening in the liver. But if you have liver disease that leads to cirrhosis, the chance of developing portal hypertension is high.

The main symptoms and complications of portal hypertension include:

• Gastrointestinal bleeding; black, tarry stools or blood in the stools; or vomiting of blood due to the spontaneous rupture and hemorrhage from varices.
• Ascites, an accumulation of fluid in the abdomen.
• Encephalopathy, confusion and forgetfulness caused by poor liver function and the diversion of blood flow away from your liver.
• Reduced levels of platelets or decreased white blood cell count.

How is portal hypertension diagnosed?
Endoscopic examination, X-ray studies, and lab tests can confirm that you have variceal bleeding. Further treatment is necessary to reduce the risk of recurrent bleeding.

What are the treatment options for portal hypertension?
The effects of portal hypertension can be managed through diet, medications, endoscopic therapy, surgery, or radiology. Once the bleeding episode has been stabilized, treatment options are prescribed based on the severity of the symptoms and on how well your liver is functioning.

First level of treatment
When you are first diagnosed with variceal bleeding, you may be treated with endoscopic therapy or medications. Dietary and lifestyle changes are also important.

Endoscopic therapy consists of either sclerotherapy or banding. Sclerotherapy is a procedure performed by a gastroenterologist in which a solution is injected into the bleeding varices to stop or control the risk of bleeding. Banding is a procedure in which a gastroenterologist uses rubber bands to block the blood supply to each varix.

Medications such as beta blockers or nitrates may be prescribed alone or in combination with endoscopic therapy to reduce the pressure in your varices and further reduce the risk of recurrent bleeding.

Medications such as propranolol and isosorbide may be prescribed to lower the pressure in the portal vein and reduce the risk of recurrent bleeding.

The drug lactulose can help treat confusion and other mental changes associated with encephalopathy.

Dietary and lifestyle changes
Maintaining good nutritional habits and keeping a healthy lifestyle will help your liver function properly. Some of the things you can do to improve the function of your liver include the following:

• Do not use alcohol or street drugs.
• Do not take any over-the-counter or prescription drugs without first consulting with your physician or nurse. Some medications may make liver disease worse, and they may interfere with the positive effects of your other prescription medications.
• Follow the dietary guidelines given to you by your physician or nurse. Follow a low-sodium (salt) diet. You will probably be required to consume no more than 2 grams of sodium per day. Reduced protein intake is required only if confusion is a symptom. Your dietitian will help you create a meal plan that helps you follow these dietary guidelines.

Second level of treatment
If the first level of treatment does not successfully control your variceal bleeding, you may require one of the following decompression procedures to reduce the pressure in these veins.

• Transjugular intrahepatic portosystemic shunt (TIPS), a radiological procedure in which a stent (a tubular device) is placed in the middle of the liver.
• Distal splenorenal shunt (DSRS), a surgical procedure that connects the splenic vein to the left kidney vein in order to reduce pressure in your varices and control bleeding.

What tests are required before the TIPS and DSRS procedures?
Before receiving either of these procedures, you will have the following tests to determine the extent and severity of your portal hypertension:

• Evaluation of your medical history
• A physical examination
• Blood tests
• Angiogram
• Ultrasound
• Endoscopy

Before either the TIPS or DSRS procedure, your physician may ask you to have other pre-operative tests, which may include an electrocardiogram (also called an EKG), chest X-ray, or additional blood tests. If your physician thinks you will need additional blood products (such as plasma), they will be ordered at this time.

More about the TIPS procedure
During the TIPS procedure, a radiologist makes a tunnel through the liver with a needle, connecting the portal vein (the vein that carries blood from the digestive organs to the liver) to one of the hepatic veins (the three veins that carry blood from the liver). A metal stent is placed in this tunnel to keep the tunnel open.

The TIPS procedure reroutes blood flow in the liver and reduces pressure in all abnormal veins, not only in the stomach and esophagus, but also in the bowel and the liver.

The TIPS procedure is not a surgical procedure. The radiologist performs the procedure within the vessels under X-ray guidance. The procedure lasts 1 to 3 hours. You should expect to stay in the hospital 1 to 2 days after the procedure.

The TIPS procedure controls bleeding immediately in over 90 percent of patients. However, in about 30 percent of patients, the shunt may narrow, causing varices to bleed again at a later time.

Potential complications of the TIPS procedure
Shunt narrowing or occlusion (blockage) can occur anytime after the procedure, and most frequently within the first year. Follow-up ultrasound examinations are performed frequently after the TIPS procedure to detect these complications. The signs of occlusion include increased ascites or recurrent bleeding. This condition can be treated by a radiologist who re-expands the shunt with a balloon or repeats the procedure to place a new stent.

Encephalopathy, or mental changes caused by abnormal functioning of the brain that occur with severe liver disease. Encephalopathy can be worse when blood flow to the liver is reduced by TIPS, which may result in toxic substances reaching the brain without being metabolized first by the liver. This condition can be treated with medications, diet or by replacing the shunt.

More about the DSRS procedure
The DSRS is a surgical procedure. During the surgery, the vein from the spleen (called the splenic vein) is detached from the portal vein and attached to the left kidney (renal) vein. This surgery selectively reduces the pressure in your varices and controls the bleeding.

A general anesthetic is given to you before the surgery. The surgery lasts about 4 hours. You should expect to stay in the hospital from 7 to 10 days.

DSRS controls bleeding in over 90 percent of patients; the highest risk of any recurrent bleeding is in the first month. However, the DSRS procedure provides good long-term control of bleeding.

A potential complication of the DSRS surgery is ascites, or an accumulation of fluid in the abdomen. This can be treated with diuretics and restricted sodium intake.

Follow-up care after the TIPS or DSRS procedures
Follow-up medical care may differ from hospital to hospital. The following are some general guidelines for the scheduling of follow-up care:

• Ten days after your hospital discharge date, you will meet with your surgeon or hepatologist and nurse coordinator to evaluate your progress. Lab work will be done at this time.
• Six weeks after the TIPS procedure (and again 3 months after the procedure), you will have an ultrasound so your physician can check that the shunt is functioning properly. You will have an angiogram only if the ultrasound indicates that there is a problem. You will also have lab work done at these times and visit the surgeon or hepatologist and nurse coordinator.
• Six weeks after the DSRS procedure (and again 3 months after the procedure), you will meet with the surgeon and nurse coordinator to evaluate your progress. Lab work will be done at this time.
• Six months after either the TIPS or DSRS procedure, you will have an ultrasound to make sure the shunt is working properly. You will also visit the surgeon or hepatologist and nurse coordinator to evaluate your progress. Lab work and a galactose liver function test will also be done at this time.
• Twelve months after either procedure, you will have another ultrasound of the shunt. You will also have an angiogram so your physician can check the pressure within your veins across the shunt. You will meet with your surgeon or hepatologist and the nurse coordinator. Lab work and a galactose liver function test will be done at this time.

If the shunt is working well, every 6 months after the first year of follow-up appointments you will have an ultrasound, lab work and you will visit with your physician and nurse coordinator.

More frequent follow-up visits may be necessary, depending on your condition.

Attend all follow-up appointments as scheduled to ensure that the shunt is functioning properly. Be sure to follow the dietary recommendations that your health care providers give you.

• Liver transplant is done in cases of end-stage liver disease.
• Devascularization is a surgical procedure that removes the bleeding varices. This procedure is done when a TIPS or a surgical shunt is not possible or is unsuccessful in controlling the bleeding.
• The accumulation of fluid in the abdomen (called ascites) sometimes needs to be directly removed. This procedure is called paracentesis.

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