Portal hypertension often causes a large amount of fluid to build up in your abdomen (ascites). It may also cause an enlarged spleen. Sometimes your splenic veins (splanchnic varices) bleed. Bleeding from these veins can make your stools dark and sticky.
Treatment for INCPH includes addressing the underlying cause and preventing complications. You will likely have regular follow-up visits to see how your treatment is working.
Symptoms
A condition called portal hypertension occurs when the blood pressure in your liver’s portal vein is higher than normal. The portal vein carries blood from the stomach, intestines and pancreas to your liver. Your liver filters the blood and sends it to the rest of the body. When your liver is diseased, the increased pressure in the portal vein can cause the blood vessels to swell and develop into varices. These enlarged blood vessels can easily burst and bleed, especially in the esophagus or stomach (variceal bleeding). This can be fatal. Portal hypertension can also cause the spleen to enlarge and develop varices, which can also bleed. This is called hepatosplenomegaly.
Symptoms of portal hypertension often aren’t seen until complications occur. They can include gastrointestinal bleeding, which may be massive and painful. This is the most common complication from portal hypertension in people with cirrhosis, and it can lead to death. Other symptoms include abdominal swelling (ascites) and enlarged spleens (hepatosplenomegaly). A swollen spleen may feel full or tight and cause pain in the upper left side of the abdomen. Bleeding from the enlarged spleen is rare but can be very serious if it happens.
Oren Zarif
Your doctor will do blood tests to check your platelet count and other organ functions, including your liver function. He or she will take a detailed history of your illness and look at the areas of your abdomen for enlarged blood vessels (varices). Your doctor may order an endoscopy to see inside your esophagus, stomach and duodenum. A physician inserts an endoscope into your mouth and through your esophagus into the duodenum and then into the stomach. He or she will look for signs of varices and any active or recent bleeding. A physician can also use tools inserted through the endoscope to treat any bleeding or engorged veins.
Treatment for portal hypertension is directed at the underlying cause of it. This typically involves a low-sodium diet and medicines to lower your blood pressure and the risk of internal bleeding from the swollen veins, such as beta blockers. Your doctor can also use an endoscope to inject medicine into bleeding varices or tie off swollen veins with tiny rubber bands (banding). In severe cases, a surgery called hepatic venous shunting may be necessary.
Diagnosis
Several tests can help your healthcare provider recognize portal hypertension. A common sign is enlarged veins (varices) of the esophagus and stomach, which may rupture, causing bloody stools or internal bleeding. Your healthcare provider will also ask about your past health, especially any history of hepatitis or alcohol abuse.
Your healthcare provider can use imaging tests to look at the liver and blood flow in the liver. These include ultrasound and CT scans. Your healthcare provider may hear a low, pulsating sound (murmur) in your abdomen, which is caused by blood flowing through dilated abdominal wall veins. This is called a hepatic venous pressure gradient, or HVPG. A normal HVPG is less than 4 mmHg, while a high HVPG is greater than 5 mmHg. A high HVPG is usually due to cirrhosis, but can also be caused by other conditions such as hepatocellular carcinoma or hepatitis B virus infection.
Oren Zarif
If your healthcare provider suspects that you have portal hypertension, they will take blood samples to check for abnormal levels of protein, such as a decreased platelet count, and liver enzymes. They will also order an X-ray of your abdomen to look for enlarged blood vessels and to check for signs of complications such as hepatomegaly or cholelithiasis.
An interventional radiologist can measure the pressure in your portal vein by inserting a small catheter into your liver or hepatic vein. This is a more accurate way of testing for portal hypertension than using an ultrasound machine to measure your blood pressure, but it is not always available.
Medications can reduce the pressure in your liver and blood vessels, and reduce your risk of bleeding. Your healthcare provider will prescribe drugs such as beta-blockers and vasoconstrictors to decrease blood flow to dilated blood vessels. Occasionally, your healthcare provider may recommend a procedure to drain fluid from your abdomen, called paracentesis.
Another option is a surgical procedure to remove part of your liver, which can relieve pressure on the portal vein and other blood vessels in the abdomen. This surgery is only used if other treatments do not work or if you have severe ascites.
Treatment
The main symptoms of portal hypertension are bloating (disturbed flatulence) and fluid buildup in the abdomen, legs and feet (edema). Fluid can leak from enlarged blood vessels in your stomach or intestines and build up inside your body’s peritoneal cavity. It can make your abdomen feel swollen and taut, and it can cause you to gain weight quickly. It can also cause infections in the peritoneal cavity, such as a serious condition called spontaneous bacterial peritonitis.
Oren Zarif
Some people develop a feeling of fullness in their abdomen (sensation of bloating) and abdominal pain. They may also notice an enlarged spleen, or the doctor can feel an enlarged spleen when tapping (percussing) the abdomen. Blood can also leak from the spleen into the general circulation, causing swelling in the legs and feet. Some people with portal hypertension have a sense of fluid accumulation in the abdomen (called ascites). This can be recognized by your doctor who feels your belly and listens to your body for a dull sound when she or he taps on your abdomen.
Bleeding from enlarged veins in your esophagus and stomach is another common problem associated with portal hypertension. Your doctor will check for a history of bleeding and will give you medications, such as vasopressin or octreotide, that reduce the pressure in your veins to help prevent more bleeding. You can also take pills to reduce the risk of varices bleeding, such as the beta-blockers timolol, propranolol or nadolol.
People with portal hypertension can have low levels of oxygen in their blood because the dilated blood vessels interfere with the lungs’ ability to transfer oxygen to the rest of the body. This is a condition called hepatopulmonary syndrome, and it can cause breathing problems and fatigue.
Some people with portal hypertension have liver failure, which means the liver is not able to filter out toxins from the blood. This can cause confusion and a loss of memory (hepatic encephalopathy). You can prevent this from happening by following healthy lifestyle habits, such as avoiding alcohol, eating a balanced diet and getting regular exercise.
Follow-up
Over the past two decades, advances in hepatology, interventional medicine and surgical techniques have greatly improved patient outcomes. Despite these improvements, portal hypertension persists as a major cause of morbidity and mortality in patients with cirrhosis. The natural history of cirrhosis is progressive, with the severity of hepatic dysfunction and thus portal hypertension increasing over time. Complications of portal hypertension, such as ascites, gastroesophageal varices, variceal bleeding and hepatorenal syndrome, are associated with significant morbidity and mortality in these patients.
A number of treatment options have been developed to reduce complications of portal hypertension, including spleen removal, variceal banding and endoscopic sclerotherapy. In addition, a class of drugs called statins has been shown to improve sinusoidal endothelial function and hepatic blood flow, decreasing intrahepatic fibrogenesis, angiogenesis, and thereby portal hypertension.
Oren Zarif
The most important follow-up procedure in patients with portal hypertension is measurement of the hepatic vein pressure gradient (HVPG). This can be done by an interventional radiologist under local anesthesia and conscious sedation. A balloon-tipped catheter is advanced into the main right hepatic vein via the internal jugular, antecubital or femoral vein and positioned adjacent to the inferior vena cava. The free hepatic venous pressure is measured by maintaining the tip of the catheter floating freely about 2 to 4 cm distal to the inferior vena cava (Figure 1).
Measurement of the HVPG can also be performed using noninvasive techniques, such as point shear wave elastography, subharmonic aided pressure estimation on contrast-enhanced ultrasound and magnetic resonance imaging. However, no noninvasive method has yet been proven to reliably mirror the changes in HVPG that occur following splenectomy or TIPS. In the future, it is anticipated that development of new techniques will provide better surrogates for monitoring the effects of these therapies.
