Portal hypertension

Portal hypertension is a complex symptom complex, which is characterized by a marked increase in pressure in the portal vein due to difficult circulation. Normally, the pressure in this vein does not exceed 7 mm of mercury. If the blood pressure in the portal vein rises up to 12 - 20 mm of mercury, it begins to expand. Varicose veins are prone to rupture, which in turn leads to the development of bleeding. This pathological condition has a large number of prerequisites for development, which will be discussed below.

Syndrome of portal hypertension

Portal hypertension is a disease that is not an independent nosology. This syndrome accompanies a large number of somatic diseases. The basis of all the changes occurring in the human body is the increased pressure in the portal vein. The forms and severity of portal hypertension directly depend on the degree of obstruction to the blood flow through the vein system. Distinguish forms of the syndrome of portal hypertension, such as prehepatic or prehepatic, intrahepatic, extrahepatic, and also mixed.

The prehepatic form of portal hypertension develops due to the congenital anomalous structure of the portal vein, as well as the formation of blood clots in it. Congenital defects include veins: hypo- and aplasia, narrowing of the lumen of the vein at any site, or total constriction. The reason for the invasion of the portal vein is associated with the spread of normal obliteration occurring in the arantium duct and umbilical vein. Thrombosis and, as a consequence, blockage of the vein occurs with various septic processes in the body (suppuration in the abdominal cavity, umbilical sepsis, septicopyemia), when it is compressed by an infiltrate or cyst.

The cause of the intrahepatic form of portal hypertension in most reported cases is cirrhotic changes in the liver. Sometimes the cause for the development of portal hypertension can serve as local sclerosis in the liver tissue. According to statistics, out of five patients with portal hypertension, four suffer from an intrahepatic block.

With superhepatic portal hypertension, the outflow of blood from the veins of the liver is severely affected. The cause for problems with normal blood flow is often endophlebitis with partial or total obstruction of the vessel. This condition is called Chiari syndrome. Separately, the Buddha-Chiari disease is called. In this case, the superhepatic portal hypertension is directly related to the clotting of the thrombotic masses of the vena cava at the level of the location of the hepatic veins. Among the reasons, the abnormality of laying the inferior vena cava is not the least. Peripheral pericarditis , tumor and cystic neoplasms, as well as insufficiency of the tricuspid valve, can obstruct the flow of blood through the vessels and cause hyperepuscular portal hypertension.

The combined form of portal hypertension occurs when the portal vein is clotted with a thrombus in people suffering from cirrhosis of the liver.

With a sharp increase in pressure in the portal vein to 450 mm Hg (norm - 200) in people who suffer from portal hypertension, the blood flow is carried out through the portocaval anastomoses. These anastomoses are divided into three groups. Normal anastomoses are present in the lower third of the esophagus and the cardiac part of the stomach. From the portal vein system, blood flows through the venous plexuses of the above organs into an unpaired vein, which in turn flows into the lower vena cava. So, if a patient with portal hypertension has varicose dilated vessels in the esophagus, this can lead to massive bleeding from this organ. To promote the development of bleeding can reflux-esophagitis or ulcers of the esophagus. There are also anastomoses between the rectal veins (upper, middle and lower). In this case, the blood flows from the upper rectal veins, which are related to the portal system, continues to move through the anastomosis system, passes the internal iliac veins and flows directly into the lower vena cava.

If a person suffering from the syndrome of portal hypertension has a stable expansion of the venous plexus of the rectum, this can result in severe bleeding from the anal passage. There is also an anastomosis between the umbilical and umbilical veins (in the event that the umbilical vein has not undergone involution). This anastomosis resets the blood from the portal vein system to the umbilical. Then it enters the veins of the external abdominal wall, into the lower and upper hollow veins. If this group of anastomoses is affected in the syndrome of portal hypertension, the so-called pattern of enlarged veins of the abdominal wall will be visible on the patient's stomach. Such a picture has the name "head of a jellyfish".

Causes of portal hypertension

Etiological factors that can lead to the development of portal hypertension, a large number. The most important cause is severe damage to the liver parenchymal tissue. Parenchyma can suffer from hepatitis (chronic and acute, viral and medicinal), cirrhotic changes, malignant neoplasms of the organ, parasitic infestations, for example, schistosomes.

Portal hypertension can begin with chronic pathological changes caused by intra- and extrahepatic stasis of bile, with biliary cirrhosis (primary and secondary), neoplasms of the bile duct, and also choledocha itself. The cause can serve as calculous cholecystitis, a cancerous tumor of the head of the pancreas, a medical error during the ligation of the bile duct during surgery. A major role in the pathogenesis of portal hypertension is played by the effects of various toxins tropic to the liver parenchyma (some types of fungi, medicines).

Portal anomalies can also be caused by abnormal congenital atresia, thrombosis, stenosis or portal vein tumors, vein thrombosis in Budd Chiari disease, restrictive cardiomyopathy , namely, increased right atrial and ventricular pressure, as well as squeezing pericarditis. In certain cases, the syndrome of portal hypertension has a correlation with crises during surgical interventions, with massive burns with a high percentage of soft tissue damage, DIC syndrome, sepsis, trauma.

The direct predisposing factors that can give impetus to the development of a detailed clinical picture of portal hypertension with all the ensuing consequences are various infections, stomach and intestinal bleeding, alcoholism, predominance of animal fats over vegetable, long-term treatment with tranquilizers, diuretics, severe long-term operations recovery.

Symptoms of portal hypertension

The clinic of any form of portal hypertension directly depends on the disease that caused a persistent increase in pressure in the portal vein system. This is important to remember and use during the patient's interview and in the analysis of the picture of the disease, especially when there are already serious complications of portal hypertension (vein thrombosis, massive bleeding from the enlarged veins of the esophagus or rectum).

Portal hypertension can be characterized by the following symptoms: a sharp increase in the spleen, the presence of varicose veins in the esophagus or stomach, bleeding from these vessels, an increase in the abdomen in size due to fluid accumulation (ascites), signs of dyspepsia (tenderness in the epigastric region, lack of stool for several days, nausea, lack of appetite until the absolute refusal to eat). People with portal hypertension have characteristic vascular asterisks on the skin, in the analysis of blood - a decrease in the number of platelets, leukocytes, anemia is much less developed, changes in the coagulation system are noted (there is a tendency to hypocoagulation).

The prehepatic form of portal hypertension manifests usually in childhood, the course is favorable. In a macroscopic examination, in some patients, it can be seen that the small veined veins have been replaced by the portal vein. This education is called "cavernoma." Most of the disease manifests itself as esophageal bleeding, which is the first sign of the development of the disease in childhood, there is an increase in the size of the spleen, hypersplenism, thrombus formation in the vein system.

The intrahepatic form also has its own peculiarities, since such portal hypertension develops with cirrhosis of the liver. The nature and speed of the development of the symptom complex depends on the type of cirrhotic changes (postnecrotic cirrhosis, alcoholic, etc.), the level of compensation for the impaired function. Portal hypertension in such patients is manifested by hemorrhages, the appearance of a "jellyfish head", an increase in the spleen, and the abdomen.

Serious complication, threatening human life, is bleeding from the veins of the esophagus and stomach. It usually arises unexpectedly, it is not preceded by any pain in the epigastrium and even discomfort. A patient with this form of portal hypertension has an unexpected vomiting of blood without impurities. Vomiting with altered blood can occur if the blood from the esophagus has leaked into the stomach. This vomiting will have the color of the coffee grounds, in addition, the patient will have a stool with an admixture of blood. In this state, the phenomena of posthemorrhagic anemia grow very rapidly. The fatal outcome of the first bleeding with portal hypertension is equal to thirty percent. If there have already been bleedings in history, yellowness and an enlarged abdomen are visible, then in this case there is a neglected stage of cirrhosis, which practically does not lend itself to effective medical or surgical treatment.

The superhepatic form of portal hypertension is a consequence of Chiari's disease or Badda-Chiari syndrome. If the disease is acute, the patient suddenly experiences intense pain in the epigastrium, under the ribs to the right, the liver rapidly increases in size, persistent febrileity is noted, ascites appears. The main causes of death in this form of portal hypertension are massive loss of blood during bleeding from the esophagus, acute renal and hepatic insufficiency.

In the case of chronic course of the disease, hepato- and splenomegaly progress slowly, a collateral of small veins develops on the anterior abdominal wall, ascites symptoms develop, disturbances occur in the protein metabolism of the organism, the person looks depleted.

Signs of portal hypertension

Diagnosis of portal hypertension is based on the study of anamnesis of life and disease, on clinical manifestations and their severity, as well as on the mass of instrumental studies that can detect and indicate the signs of this syndrome.

The first thing to do if you suspect a portal hypertension patient is to conduct a thorough examination. During the examination, attention should be paid to the presence or absence of venous collaterals: veins on the abdomen, near the navel, hemorrhoids, hernia, ascites. It is necessary to carefully study the skin color, sclera, visible mucous membranes, to determine the presence of characteristic vascular asterisks. Then it is necessary to palpate the liver and spleen. Normally, in a healthy person, the liver does not protrude from under the edge of the left costal arch, and the spleen is not palpable.

A laboratory study with suspicion of portal hypertension implies a general analysis of capillary blood and urine, a coagulogram, a biochemistry of venous blood, tests for hepatitis, and antibodies to immunoglobulins A, M, and G.

In order to correctly and accurately diagnose the disease, X-ray methods are used: port and caviography, angiography of mesenteric blood vessels, celiacography and splenoportography. The totality of the data obtained from the above studies will allow us to determine the degree of blockage of the blood flow in the portal vein system and assess the chances of anastomosis. If it is necessary to determine the quality of the blood flow of the liver, it is necessary to make the patient a liver scintigraphy.

Ultrasound can help to accurately determine the increase in the spleen, liver, the presence of free fluid in the abdominal cavity. Dopplerometry of the hepatic vessels will show the size of the portal, upper mesenteric and splenic veins, and an increase in the lumen of these vessels is a direct sign of portal hypertension.

Percutaneous splenometonomy is done in order to register pressure in the portal vein system. In the splenic vein, the normal pressure does not exceed one hundred and twenty millimeters of mercury, and in portal hypertension it reaches five hundred millimeters of mercury.

Patients with portal hypertension must undergo esophagoscopy. With esophagoscopy, it is possible to identify such a sign of portal hypertension as varicose veins in the esophagus. Fibrogastroduodenoscopy is also a fairly informative way to detect signs of portal hypertension in the esophagus and cardia.

Recto-manoscopy is an instrumental method that will detect the altered veins in the rectum of the patient. In some cases, according to contraindications or refusal of the patient from instrumental research, these methods are replaced by radiography of the esophagus with the stomach.

A liver biopsy and a laparoscopic diagnostic operation are done only in cases where for some reason it did not work out to confirm the diagnosis by the above methods or if there is a suspicion of a malignant tumor.

Portal hypertension in children

In children, most often there is an extrahepatic form of portal hypertension. This is almost always due to anomalies when laying the portal vein system, expressed in a cavernous transformation. Some role among the etiological factors is played by incorrect catheterization in the early neonatal period, leading to the development of umbilical vein thrombophlebitis. This entails such consequences as thrombosis of the portal vein system.

Portal hypertension in a child can begin because of various congenital or acquired liver diseases. These diseases include fetal and viral hepatitis, a variety of cholangiopathies, expressed in a different level of damage to the bile duct (from a slight hypoplasia to complete disabling of the function). Significantly stimulates the development of symptoms of portal hypertension in children periductular fibrosis (adhesive process between ducts), accompanying all of the above processes.

To recognize extrahepatic portal hypertension in a child can be a pronounced splenomegaly. The spleen at palpation is large, practically immobile, does not hurt. There are also manifestations of hypersplenism. Often with this form of portal hypertension, there is a rapid expansion of the venous vessels of the esophagus and the cardiac part of the stomach, which leads to spontaneous massive hemorrhages.

In the overwhelming majority of patients with extrahepatic portal hypertension, hemorrhage is the first manifestation of this disease. From one bleeding to the next patient feels quite well, the disease does not bother. The increase in liver size with this pathology is not a characteristic feature, which can only develop as a consequence of thrombus formation in the portal vein due to umbilical sepsis. Ascites are also very rare. Do not undergo any changes and functional tests.

Decompensated stage of portal hypertension is observed with cirrhosis and is noted mainly in adolescents. Bleeding from the esophagus is also rare.

Treatment of portal hypertension

Conservative treatment of a disease such as portal hypertension is possible only if changes occur only at the level of intrahepatic hemodynamics. To treat portal hypertension, drugs such as beta-blockers, nitrates, glycosaminoglycans and angiotensin converting enzyme inhibitors are used.

Dosage and duration of treatment with a drug such as Nitrosorbide is selected by the attending physician individually. In most cases, the single dose ranges from ten to twenty milligrams, and the number of doses per day varies between two and five times. Thus, the daily amount of the drug is an individual indicator, but it should not exceed one hundred and eighty milligrams in twenty-four hours. Often, the course of treatment with this drug is not long, as the body gradually gets used to Nitrosorbide and the effect eventually reduces to zero.

Among beta-blockers, they have proven themselves in the therapy of portal hypertension Anaprilin and Atenolol. Atenolol in patients with portal hypertension should be administered under the supervision of a physician. Usually, this drug is taken from fifty milligrams once a day, after a few weeks the result is evaluated and, if it is insufficient, the dose is doubled. Long-term therapy with this drug is possible. Anaprilin is taken half an hour before meals once, washed down with plenty of water. They usually start with ten milligrams, gradually increase the dose every week by twenty milligrams and bring to a daily dose of eighty to one hundred and twenty milligrams. This dose is already divided into several receptions. The course of treatment is individual and usually lasts from fourteen days to two months, then a break is necessary.

Among the ACE inhibitors, ramipril (Hartil, Cardipril) showed good results in the therapy of portal hypertension. It effectively reduces the increased pressure in the dilated vessels, which is an indispensable symptom of portal hypertension. At the beginning of treatment, this drug should take 2.5 milligrams before meals at one time. If there are any undesirable effects, the dose can be divided into two. If the effect of therapy is inadequate, after two or three weeks the amount of the drug is doubled. In order to improve the antihypertensive effect of this drug, you can simultaneously appoint a diuretic. It is recommended that the first dose of the drug be taken in the presence of the treating doctor and be under its control the next eight hours, since there is a threat of an uncontrolled hypotensive reaction. During the course of treatment with ramipril in portal hypertension, you should limit the use of salty foods and do not take non-steroidal anti-inflammatory drugs without special need, since all this significantly reduces the effectiveness of the drug.

If the patient has portal hypertension already had a bleeding or there is hypersplenism or accumulation in the abdominal cavity of the fluid, the method of treatment can only be surgical.

As the first medical aid for hemorrhagia, the Sengstacken-Blackmore probe is installed from the VRVP. In terms of effectiveness, it is equal to imposing a pressure bandage on the limb. This obturator probe will not give an effect unless the bleeding from the veins has developed in the lower part of the stomach, which happens, but extremely rarely. The residence time of the probe varies from twelve hours to three days. With proper use of the probe, stopping bleeding is successful in almost 99% of all cases, but only one in two people suffering from portal hypertension can avoid relapse. The patient may develop complications such as aspiration of the lungs after applying the obturator, in rare cases, a rupture of the stomach wall by the probe may occur. Due to the presence of the device in the patient's body for more than three days, mucosal ulcers may appear in the stomach.

After bleeding due to portal hypertension, drug treatment plays an important role. From the first day appoint Vikasol (1%) in intravenous infusions of six milliliters. Duration of treatment is five days. Ten percent calcium chloride is also injected into the vein in ten cubes per day. The course of treatment, too, should not exceed five days. Every five to six hours a patient needs to do Dicinon infusions. The first injection with a dosage of 4.0, and the subsequent - 2.0. Injections do three to five days.

To reduce the severity of fibrinolysis and anticoagulant activity, the patient is dripped with a five percent aminocaproic acid per hundred milliliters four times a day. Also, bleeding due to portal hypertension requires Contrikal's injections every eight hours.

In addition to all of the above, it is necessary to neutralize the effect of histamine on capillaries and their permeability. For this purpose, prescribe diphenhydramine in a dosage of 1.0 every eight hours. Dimedrol can be replaced with Suprastin. The dose and multiplicity of administration of this drug is the same.

A very important point of successful treatment of bleeding in portal hypertension is the improvement of the rheological properties of the blood. To this end, the patient is injected with a drop of 400 milliliters of Rheopolyglucin. It is necessary to give a dropper of 0.025% solution of Strophantine (1.0 per 100 milliliters of saline and 5% glucose). You should also fight with acidification of blood. Normalize ph will help the solution of sodium bicarbonate. To this end, the patient is placed a dropper for 200 milliliters of a 4% solution.

In parallel with hemostatic treatment, it is necessary to effectively reduce blood pressure and maintain it at a level not exceeding 110 millimeters of mercury (meaning systole). Nitroglycerin handles this task well. With hemorrhage due to portal hypertension, it is prescribed in intravenous injections. A four percent solution (1.0 Nitroglycerin per 400 milliliters of saline or Ringer's solution) is used. Drip infusion is slow, no faster than twelve drops per minute.

Of drugs that have a hepatoprotective effect, it is most expedient to appoint Heptral. The first few days, it is injected with a drop of four hundred milliliters, and by the end of the first week the patient is transferred to tablets. In order to prevent multiple organ failure, a glucose solution, albumin preparations, glucocorticoids, saline solutions and vitamins should be added to the treatment of portal hypertension.

In patients with portal hypertension, the level of ammonia is increased. In order to reduce the amount of this substance, appoint Gepa-Merz. For the first intravenous infusion, the concentration of the active substance in 400 milliliters should be 60.0. All subsequent times the concentration of the drug is 30.0. Instead of Hepa-Merz, you can use Hepasol A. It is released in half-liter vials in standard dilution. Droppers with Gepasol A should be done twice a day for the first few days after bleeding.

In the treatment of portal hypertension, proton pump inhibitors (Losek, Kwamatel) are also used. Losek is administered forty milligrams intravenously every twelve hours. Kwamatel is administered twenty milligrams each. The frequency of administration of the drug is the same.

After carrying out effective treatment of bleeding, caused by portal hypertension, go to surgical removal of the cause of the disease. The only most effective method of treatment of portal hypertension is the imposition of anastomoses between the vessels. Similar operations in adults are almost always without problems, but when dealing with this method, children have some difficulties. Difficulties are due to the fact that the diameter of the child's vessels is much narrower than that of an adult. In addition, the small child lacks full-fledged anatomical structures that can be used to form an anastomosis. That is why surgery for the formation of anastomoses with portal hypertension is carried out in children who are seven to eight years old. Until that age, treatment is almost always exclusively medicated.