Portal Hypertension: Causes, Signs, Symptoms, Treatment, Diagnosis
What is Portal Hypertension?
Increase in the blood pressure in the portal venous system is known as Portal Hypertension. Portal venous system is a system of veins, which emerge from the stomach, spleen, intestine and pancreas and join the portal vein. There are smaller vessels, which branch out from the portal vein and pass through the liver. If there is blockage in the liver vessels from liver damage, then there is disturbance in the blood circulation through the liver. This causes increased pressure in the portal system and portal vein, which causes varices. Varices are the large and swollen veins present inside the stomach, esophagus, rectum or umbilical region (belly button). There can be rupture and bleeding in these varices, which can result in complications that can be life-threatening.
Causes of Portal Hypertension
- Cirrhosis of the liver is the most common cause of portal hypertension. There is scarring in the liver, which appears after healing of any liver injury occurring due to alcohol, hepatitis or other less common causes of liver damage. The scar tissue present in the cirrhosis blocks the blood flow through the liver.
- Any blockage of the vein which carries the blood to the heart from the liver can also cause portal hypertension.
- Blood clots in the portal vein can also cause portal hypertension.
- Schistosomiasis is a parasitic infection, which can also cause portal hypertension.
- Focal nodular hyperplasia is a disease where the liver has a benign tumor. This could also lead to portal hypertension.
- In some cases, the cause of the portal hypertension is unknown.
Signs & Symptoms of Portal Hypertension
Patient may not always have specific symptoms, which indicate if something is wrong with the liver. However, if the patient has liver disease, which has led to cirrhosis, then the risk of developing portal hypertension is very high. Some of the complications and primary symptoms of portal hypertension are:
- Portal hypertension patient may have symptoms of blood in the stools or black and tarry stools, which indicate gastrointestinal bleeding. Patient may vomit blood also if there is spontaneous rupture and bleeding of the varices.
- Patient with portal hypertension can have ascites where there is accumulation of fluid in the abdomen.
- There is encephalopathy where the patient experiences forgetfulness or confusion due to poor liver function.
- Decreased level of platelets or white blood cells is present.
Diagnosis of Portal Hypertension
Diagnosis of portal hypertension is often made on the basis of dilated veins or varices or ascites which can be seen when a physical exam of the abdomen or the anus is done. Lab tests, such as x-rays and endoscopic exams can also be done
Treatment of Portal Hypertension
There is no treatment for majority of the causes of portal hypertension. The aim of treatment is preventing and managing the complications, particularly if there is any bleeding from the varices. Treatment options for portal hypertension comprise of diet, medications, endoscopic therapy, radiology and surgery to treat or prevent the complications. Further treatment depends on the degree of the symptoms and the function of the liver. Treatment for portal hypertension comprises of:
- Endoscopic therapy is commonly the first line of treatment for variceal bleeding and it consists of either banding or sclerotherapy. Sclerotherapy is done when banding cannot be done. It is a procedure where a solution, which clots blood, is injected into the bleeding varices so that the bleeding stops. Banding is a procedure where rubber bands are used to compress the blood vessels in order to stop the bleeding.
- Medications, such as nonselective beta-blockers (propranolol, nadolol ) can be prescribed alone or in conjunction with endoscopic therapy to help decrease the pressure in varices and to decrease the risk of bleeding. Nonselective beta blockers can also be prescribed for preventing a first variceal hemorrhage in those patients who are at risk for bleeding. Esophageal variceal banding can also be used for this purpose, particularly those patients who cannot take beta blockers. Lactulose can be given for treating confusion and other mental changes which are related with encephalopathy.
Surgical Treatment Options for Portal Hypertension
If endoscopic therapy, medicines, dietary/lifestyle changes are not helping to control variceal bleeding, then one of the following decompression procedures need to be undertaken to reduce the pressure in the portal veins.
Transjugular Intrahepatic Portosystemic Shunt (TIPS)
In this procedure a stent is placed in the middle of the liver to connect the hepatic vein with the portal vein, which will redirect the blood flow in the liver thus relieving the pressure in the abnormal veins. In this procedure, the radiologist uses a needle to make a tunnel through the liver, which connects the portal vein to one of the liver or hepatic veins. The radiologist then puts a metal stent in the tunnel in order to keep it open. This procedure redirects the blood flow in the liver and brings down the pressure in the abnormal veins present in the stomach, esophagus, liver and bowel. This procedure is done under x-ray guidance. The duration of this procedure is 1 to 3 hours. An overnight stay s required after this procedure.
Success Rate of TIPS Procedure
In more than 90% of the patients, the bleeding and portal hypertension is controlled immediately after the TIPS procedure. In some patients, narrowing of the shunt may occur and re-bleeding of the varices may also occur.
Complications of TIPS Procedure
Narrowing or blockage of the shunt can occur in the first year following the TIPS procedure. Frequent follow-up ultrasound exams need to be done after the TIPS procedure in order to detect any complications. Increased ascites and re-bleeding are some of the signs of a blockage. Radiologist can solve this condition by re-expanding the shunt using a balloon or by re-doing the TIPS procedure and placing a new stent.
In case of severe liver disease, patient may have encephalopathy where there is abnormal functioning of the brain. After reduction of blood flow to the liver following the TIPS procedure there can be worsening of hepatic encephalopathy, as the toxic substances don't get metabolized by the liver and reach the brain as they are. This can be treated with diet, medications or by making the shunt inaccessible.
Distal Spleno-Renal Shunt (DSRS)
In this procedure, the vein from the spleen is connected to the vein from the left kidney to reduce the pressure in the varices and halt the bleeding.
DSRS is a surgical procedure in which splenic vein is separated from the portal vein and attached to the left renal vein. This surgery helps in reducing the pressure in the varices and controlling the bleeding related to portal hypertension. DSRS procedure is commonly done only in patients who have good liver function. This surgery requires general anesthesia, the effect of which lasts around 4 hours. Patient will require staying in the hospital for about a week to 10 days.
Success Rate of DSRS Surgery
Many patients (more than 90%) with portal hypertension achieve good long-term control of bleeding with DSRS procedure. There is a high risk of re-bleeding in the first month.
Complications of DSRS Surgery
There is a risk of ascites where there is build up of fluid in the abdomen with DSRS Surgery. Diuretics and reduced sodium intake is done for treating this complication.
The following Tests will need to be done before the TIPS and DSRS Procedures are performed to assess the severity and extent of the condition:
- The medical history of the patient is evaluated and physical exam is conducted.
- Blood tests are done.
- Angiogram is done to assess the blood flow in a specific artery.
- Ultrasound and endoscopy are also done.
- Electrocardiogram (EKG) is done to record the electrical activity of the heart.
- Chest x-ray and additional blood tests are also done.
Follow-Up Care after TIPS or DSRS Procedures
- Patients need to follow up with their surgeon about 10 days after the hospital discharge for evaluation of their progress. Lab tests may also be done at this time.
- An ultrasound is done about six and three months after the TIPS procedure is done, to check if the shunt is working properly. If there is any problem detected on the ultrasound, then angiogram may also be done.
- Six weeks after the DSRS procedure (and again three months after the procedure), the surgeon will evaluate your progress. Lab work may be done at these times.
- Ultrasound is again done six months after the DSRS or TIPS procedure to ensure that the shunt is functioning properly.
- The ultrasound is again repeated a year after either of the procedure. Angiogram may also be done to check the pressure inside the veins, which are present across the shunt.
- If the shunt is functioning properly, then ultrasound with lab work and consultation is done every six months after the initial year of follow-up appointments.
- Depending on the patient's condition, frequent follow-up visits may be needed.
Other Treatment Options for Portal Hypertension
- Liver transplantation can be done in patients with end-stage liver disease.
- Devascularization is a surgical procedure where bleeding varices are removed. This procedure is done when it is not possible to do a TIPS procedure or when it is not possible to place a surgical shunt. Devascularization is also done if the above mentioned procedures fail to control the bleeding.
- Paracentesis is a procedure which directly removes the ascites or accumulation of fluid in the abdomen. This procedure gives temporary relief and needs to be repeated as and when required.
Lifestyle Changes for Portal Hypertension
The following lifestyle changes will help in improving the function of the liver and preventing portal hypertension:
- Alcohol and street drugs should be avoided.
- Always consult your doctor first before starting any over-the-counter or prescription or herbal medicines, as there are some medicines which can worsen a preexisting liver disease.
- Patient needs to follow a low- salt diet. If patient is suffering from confusion, then the protein intake should also be cut down.