Pulmonary hypertension life prognosis. Pulmonary hypertension: symptoms and treatment, the dangers of the disease

Pulmonary hypertension (PH) is a disease in which pressure in the pulmonary artery increases. Normally, systolic pressure in the pulmonary circulation should be 18-25 mm Hg, diastolic - 6-10 mm Hg, and the average specific pressure - within 12-16 mm Hg. The diagnosis of "pulmonary hypertension" is made if the average specific pressure exceeds 30 mm Hg. during physical activity and 25 mm Hg. at rest.

This disease significantly worsens the patient's condition and has an unfavorable prognosis.. In some cases, it develops slowly, so patients may not be aware that they have pulmonary hypertension for a long time and start treatment at a late stage. But remember that in any case, not everything is lost: if you have been diagnosed with this disease, you can improve your condition thanks to special folk remedies. They reduce pressure in the pulmonary artery and relieve unpleasant symptoms.

Stages of pulmonary hypertension

So, we know what LH is, now it's time to talk about its stages. Based on the pressure measured in the pulmonary artery, the disease is divided into three groups:

• mild pulmonary hypertension - corresponds to an average specific pressure of 25-36 mm Hg;
• moderate pulmonary hypertension - pressure is 35-45 mm Hg;
• severe pulmonary hypertension - pressure exceeds 45 mm Hg.

Naturally, the higher the severity of the disease, the worse its prognosis.

Causes and types of pulmonary hypertension

The cause of PH is a steady increase in pressure in the pulmonary vessels. This may be due to the following factors:

• heart disease, valvular disease (mitral regurgitation) or Eisenmenger's syndrome with congenital anomalies;
• lung diseases such as pneumoconiosis (COPD), sleep apnea syndrome;
• connective tissue diseases such as systemic lupus erythematosus, etc.;
• complications after pulmonary embolism;
• other diseases that damage the pulmonary vessels (HIV infection, portal hypertension, poisoning with toxic drugs).

Pulmonary hypertension in terms of causes of occurrence is divided into 4 groups:

• pulmonary arterial hypertension - mainly caused by connective tissue diseases, HIV - infection, portal hypertension, heart defects;
• venous pulmonary hypertension - develops as a result of diseases of the left valve or left ventricle of the heart;
• pulmonary hypertension associated with respiratory diseases or hypoxia - mainly appears due to interstitial lung disease, COPD, breathing disorders during sleep, chronic mountain sickness;
• pulmonary hypertension associated with chronic thromboembolism - caused by blockage of the pulmonary arteries.

Primary pulmonary hypertension

The primary form is also sometimes referred to as idiopathic pulmonary hypertension. It occurs rarely (about 2 cases per million people), its etiology is unknown. The disease is more often diagnosed in middle-aged women. Risk factors, in addition to the above, are a family history of hypertension, infectious diseases, and portal hypertension.

Primary pulmonary hypertension occurs with vasospasm in the pulmonary circulation. In this case, excessive blood clotting is observed. The prognosis is very unfavorable. However, if you have been diagnosed with primary pulmonary hypertension, do not despair. Better pull yourself together and start treatment with folk remedies - this will help prolong life for many years.

Secondary pulmonary hypertension

It develops against the background of other diseases - bronchial asthma, heart defects, pulmonary fibrosis, hypertension, systemic lupus erythematosus, etc. Thus, in order to get rid of PH, you must first cure the primary disease, if possible.

Symptoms

The first signs of pulmonary hypertension are a feeling of continuous fatigue and difficulty breathing, especially after exercise. Patients have difficulty walking, climbing stairs, bending over, and doing normal housework.

In the later stages of the disease, the following symptoms appear:

• dry cough;
• hoarseness;
• chest pain;
• hemoptysis;
• bluish tint of lips;
• fainting;
• cold extremities.

Pulmonary hypertension in newborns is manifested by retraction of pliable places on the chest, shortness of breath and severe cyanosis of the skin. In this case, oxygen treatment does not give any results.

Forecast and consequences

Any kind of PH gives the same result: increased pulmonary vascular resistance. These conditions force the right ventricle to work harder, resulting in cardiac hypertrophy and dilatation within the right ventricle. As a result, congestive heart failure and mitral valve insufficiency develop. Excessive overload of the right ventricle due to lung disease is called cor pulmonale. These changes lead to a decrease in cardiac output, and if the disease is not treated, then to death.
The prognosis depends on the neglect of PH. If you take care of yourself, follow some recommendations and carry out treatment, you can stop the development of the disease.

Treatment

So, you already know what pulmonary hypertension is and what are its symptoms. Now it's time to consider treatment.

Palliative care if you have idiopathic (primary) pulmonary hypertension, which allows you to extend the life of the patient and improve its quality. With secondary pulmonary hypertension, the diseases that led to such a problem are eliminated.

Below we will give lifestyle advice to patients and present folk remedies that reduce pressure in the pulmonary artery.

Diet and lifestyle

Patients should avoid situations in which the symptoms of the disease worsen. Give up physical effort, do not climb to great heights, do not fly in airplanes. If the state of health deteriorates, breathing through oxygen cylinders can be used.

It is very important to try to avoid stress, as it increases blood pressure and heart rate. To combat stress, use meditation, walking in the fresh air, meeting friends, hobbies, and other appropriate ways.

When it comes to diet, the main rule here is to reduce salt intake. Replace it with other spices. Also, patients should drink no more than 1.5 liters of water per day, so as not to increase the pressure. Naturally, alcohol and cigarettes with such a disease are completely prohibited.

Garlic

A mild subspecies of pulmonary hypertension can be completely cured with garlic. The fact is that this product expands the vessels of the pulmonary circulation, removes congestion in the veins, thereby reducing pressure. It is also very good for the heart, and it is heart problems that often lead to PH.

The simplest recipe is to eat 2 cloves of garlic every morning on an empty stomach with a glass of water. If you are afraid of bad breath, chew garlic with a mint leaf.

Garlic tea helps a lot. Don't let such an exotic name scare you - such a drink is very tasty and healthy. It will help restore not only the circulatory and respiratory system, but the entire body. Ingredients per serving:

• 1 clove of garlic;
• 1 glass of water;
• a little ground ginger (about 7-8 grams);
• 1 tablespoon lemon juice;
• 1 tablespoon honey.

Put water on fire. When it begins to boil, add the pre-minced garlic clove, ginger and a spoonful of honey. Mix everything well and leave to cook on low heat for about 20 minutes. Then strain the resulting infusion. At the end, add some lemon juice. We recommend drinking this tea on an empty stomach twice a day.

Clover

Clover is very good for the cardiovascular system and will help with your disease. It can be used in many different ways.

If possible, plant clover in a pot at home, and eat the young sprouts of this plant. A tablespoon of herbs will suffice.

You can also make tea from dried clover flowers. For a glass of water, you need a teaspoon of the crushed plant. Pour boiling water over clover, leave for 15 minutes, then drink. Take 2-3 glasses of this drug per day.

Digitalis

Digitalis will save you from pain in the chest and severe shortness of breath, but you need to drink it carefully, increasing the dosage and monitoring the state of the body.

Prepare an alcohol tincture at the rate of 1 part of dry foxglove herb to 10 parts of alcohol with a strength of 70 degrees. Infuse the mixture for 2 weeks, then strain. Start with a dosage of 3 drops twice a day. The tincture should be dissolved in a small amount of water and drunk on an empty stomach. After about a week, the dosage can be increased to 5 drops, then after another week - up to 7 drops. Then move on to 10 drops of the tincture twice a day. The course of treatment lasts from 2 to 4 months.

Hawthorn

Hawthorn dilates blood vessels, helping to reduce systolic and diastolic pressure in the pulmonary artery. It can be used in several ways.

The first way is tea. To prepare a drink, pour a dessert spoon of dried hawthorn flowers and leaves into a mug and pour a glass of boiling water. Keep the glass covered for 15 minutes, then strain the drink. It should be drunk 1 glass in the morning and evening.

The second method is healing wine. You will need 50 g of ripe hawthorn fruit and 500 ml of natural red sweet wine. Crush the berries in a mortar, heat the wine to a temperature of about 80 C. Pour warm wine into a glass jar or bottle, add hawthorn, close the lid and leave for 2 weeks, then strain. Drink 25 ml of this wine every night before bed.

Method three - alcohol tincture. Combine hawthorn flowers with alcohol in a ratio of 1 to 10 and infuse in a glass container for 10 days, then strain. Drink about half a teaspoon of this tincture in the morning and evening on an empty stomach.

mistletoe

It will be much easier for the patient if he starts taking mistletoe. In the evening, pour a teaspoon of grass with a glass of cold water, let the mixture infuse overnight. Warm it up a little in the morning and drink it. This is how you should do it every day.

You can also make wine from mistletoe. You will need 50 g of the plant and 500 ml of dry white wine. Combine these ingredients and infuse for 1 week, then strain. Take 25 ml twice daily before meals.

rowan fruits

The fruits of mountain ash have a beneficial effect on the cardiovascular system. Therefore, replace ordinary tea with tea from this plant. Mash a tablespoon of fruits in a mug, add a small amount of sugar and 200 ml of boiling water. Stir and drink. It is recommended to take 3 servings of this tea per day.

Witch Doctor Collection

There is a good collection of plants recommended by experienced healers. Combine in equal parts adonis herb, valerian root, mint leaves, St. John's wort and dill seeds. At night in a thermos, brew a tablespoon of this mixture with a liter of boiling water and insist until morning. This will be your serving for the whole day, you need to drink the infusion in between meals. Continue the course of treatment for 2 months, then you need to take a break for at least 2 weeks.

Treatment with juices and smoothies

Juice is a natural healer that will help to cope with many problems, including PH. We will give some of the most effective recipes.

Juice for the heart

With pulmonary hypertension, you need to support the work of the heart. To do this, prepare this mix:

• 2 glasses of red grapes;
• 1 grapefruit;
• 1 teaspoon crushed linden flowers;
• ¼ teaspoon powdered cloves

Combine all these ingredients and mix in a blender. You need to drink smoothies immediately after preparation, it is recommended to take a glass a day. Within weeks, you will notice positive changes.

Juice to normalize pressure

Connect the following components:

• juice of 1 grapefruit;
• juice from 2 oranges;
• 6 sprigs of fresh parsley;
• 2 tablespoons of hawthorn flowers;
• 3 kiwis, peeled and chopped

Mix everything in a blender and drink immediately after preparation. Take this remedy in the morning and in the evening so that the arterial and pulmonary pressure is always normal.

Juice for shortness of breath

If shortness of breath is tortured, conduct a monthly course of treatment with this juice:

• 5-6 fresh dandelion leaves;
• 1 tablespoon of red clover flowers;
• 1 red apple;
• 1 cup shredded Brussels sprouts;
• 1 large carrot;
• 0.5 cup chopped parsley

Squeeze juice from apple, carrot and Brussels sprouts, mix juices, add parsley, red clover and finely chopped dandelion leaves. Take juice on an empty stomach, 1 glass twice a day.

Juice for general well-being

You will need:

• half a melon;
• 1 glass of fresh strawberries;
• a piece of ginger root 2.5 cm long;
• 1 orange;
• 1 grapefruit;
• ¼ cup natural yogurt (no additives)
• half a handful of wheat germ.

Squeeze juice from orange, melon, strawberry and grapefruit. Grate the ginger. Combine juices, add ginger, yogurt and wheat germ. Drink to your health!

Pulmonary hypertension is expressed in an increase in pressure inside the vessels that supply blood to the respiratory system. This entails the development of heart failure, namely, insufficiency of the right ventricle of the heart. As a result, the person dies.

Normally, the pressure in the pulmonary artery is equal to 17-23 mm. rt. Art. With pulmonary hypertension, it will exceed 25 mm at rest. rt. Art. Under load, this figure rises to 30 mm. rt. Art. and more.

Violations that occur in the body with the development of pulmonary hypertension:

Vasoconstriction, which is characterized by vasoconstriction and spasm.

Loss of elasticity of the vascular wall.

The formation of small blood clots inside the vessels of the lungs.

Growth of smooth muscle cells.

Vessel obliteration.

The growth of connective tissue inside the vessels against the background of their destruction.

All this leads to the fact that the blood is no longer able to circulate normally in the vessels of the lungs. During its passage through the arteries, there is an increase in pressure in them. This entails an increase in pressure in the right ventricle, which provokes a violation of its functioning.

A person begins to show signs of respiratory failure, and then symptoms of heart failure join. Even at the initial stages of the development of the disease, the patient's quality of life deteriorates significantly, which is caused by the impossibility of normal breathing. As the pathology progresses, a person has to more and more restrict himself in physical activity.

Pulmonary hypertension is a serious disease that claims the lives of people. If the patient does not receive adequate therapy, then he will not live longer than 2 years. At the same time, he will need outside help for life support. Pulmonary hypertension can and should be treated, but a complete recovery cannot be achieved.

The detection and treatment of pulmonary hypertension is the task of a therapist, pulmonologist, cardiologist, geneticist and infectious disease specialist, that is, doctors of several specialties. If necessary, vascular and thoracic surgeons are involved in therapy.

The disease begins to develop imperceptibly for a person. Pathological processes at the initial stage are hidden, as the body includes compensatory mechanisms. Therefore, the patient feels well.

When the pressure in the pulmonary arteries exceeds 25 mm. rt. Art., a person begins to notice deterioration in health. However, they appear only during physical activity. As the pathology progresses, the patient will experience more and more difficulties during the performance of even the most familiar activities.

The main symptoms of pulmonary hypertension are:

Shortness of breath, which occurs as the first manifestation of the disease. At first, it appears only during physical effort, it worries a person on inspiration. In the future, shortness of breath will be present on an ongoing basis, even when the patient is at rest. He does not suffer from asthma attacks.

Pain in the sternum. Their nature varies, the pains can be aching, pressing, stabbing. A person cannot accurately tell the doctor about the time of manifestation of pain, however, he notes that they become more intense during physical effort. Nitroglycerin does not eliminate the pain syndrome.

Damage to the interstitium of the lungs as a result of the growth of connective tissue or against the background of an inflammatory disease.

Thus, the following risk factors that can lead to the development of the disease can be distinguished:

Taking medications, getting into the body of toxic substances. It has been established that pulmonary hypertension may occur while taking Fenfluramine, Rapeseed oil, Aminorex, Dexfenfluramine. Scientists also suggest that substances such as amphetamine and L-tryptophan can provoke pathology.

Demographic risk factors and medical factors. It has been proven that women are more likely to suffer from pathology. It is also believed that hypertension may be associated with high blood pressure and pregnancy.

Some diseases. The relationship between PAH and HIV infection has been established. Pulmonary hypertension may develop in liver pathologies.

Degrees of pulmonary hypertension

There are four degrees of pulmonary hypertension, which determine the severity of the course of the disease:

The first degree is characterized by the absence of any symptoms.

The second degree is manifested by all the symptoms of the disease, which are described above. At the same time, their intensity is extremely low, the patient does not complain. Pathological manifestations will disturb a person only during physical activity.

The third degree of the disease is manifested by a deterioration in well-being even against the background of slight physical exertion. At rest, the patient feels normal.

The fourth degree of the disease is expressed in the fact that it is difficult for a person to perform even elementary actions. Symptoms of pulmonary hypertension do not go away during a state of complete rest.

If a person suspects that he is developing pulmonary hypertension, he should see a doctor.

The doctor will conduct a comprehensive diagnosis, which boils down to the following activities:

Examination of the patient. During the initial examination, the doctor may detect cyanosis of the skin on the upper and lower extremities. The patient's nails may be thickened, have the shape of "drum" sticks. If the patient has emphysema, then his chest will be expanded like a "barrel". The liver is often enlarged, which is noticeable on palpation. It is possible to identify ascites and pleurisy, in which fluid accumulates in the peritoneal cavity and in the pleura. The patient's legs are swollen, the veins in the neck are dilated.

Listening to the heart and lungs. Above the pulmonary artery, the noises are increased by 2 tones, characteristic single wheezing is heard. With valvular heart failure, systolic murmurs will be heard. It is possible to listen to noises that characterize heart disease, if any.

In 55% of cases, the diagnosis allows you to perform an ECG. 87% have an enlarged right ventricle with thickened walls. The load on the right parts of the heart increases significantly, which can be determined by the corresponding signs. To the right, the electrical axis of the heart deviates in 79% of people.

Vector electrocardiography can detect pulmonary hypertension in 63% of cases. As for the indicators, they change similarly to the ECG indicators.

Phonocardiography, which allows you to record heart murmurs, which increases the probability of making a correct diagnosis, at least up to 76%. At the same time, it is possible to detect an increase in pressure in the pulmonary artery, changes in the structure of the right ventricle, congestion in the pulmonary circulation, and malformations of the heart.

X-ray examination of the chest reveals bulging of the pulmonary artery, expansion of the roots of the lungs, the right heart becomes larger in size. The edges of the lungs are characterized by increased transparency.

Ultrasound of the heart or ECHOCG makes it possible to detect an increase in pressure in the trunk of the pulmonary artery, to identify abnormalities in the work of the tricuspid valve and the interventricular septum. Also, studies make it possible to detect malformations of the heart, expansion of the right heart, thickening of the walls of the right ventricle.

The introduction of the device into the heart through large veins (catheterization of the right heart). This invasive procedure allows you to determine the pressure in the pulmonary artery and in the ventricle, the level of oxygenation of the blood, disorders in the blood circulation of the ventricle of the heart and lungs. In parallel, drug tests can be performed that allow you to find out the body's response to calcium antagonists. These drugs are the main ones in the treatment of pulmonary hypertension.

If the listed research methods are not enough to make a correct diagnosis, then the doctor will refer the patient to additional examinations, including:

FVD - determination of the function of external respiration. This method allows to exclude pathologies of the respiratory system. If the pressure in the pulmonary artery system is increased, then the doctor diagnoses a decrease in the partial pressure of oxygen and carbon monoxide.

Carrying out ventilation-perfusion lung scintigraphy. During the study, the doctor injects radioactive particles into the bloodstream that reach the lungs. This makes it possible to detect thrombotic masses in the pulmonary artery system.

Multislice computed tomography of the lungs and heart with the introduction of contrast. This method allows you to create a three-dimensional model of the respiratory organs and display them on the screen. As a result, the doctor receives maximum information about the disease.

Angiopulmonography. During the study, a contrast agent is injected into the pulmonary vessels, and then a series of images are taken on x-ray machines. This study allows you to detect blood clots in the arteries.

The prognosis of pulmonary hypertension is poor. It will not be possible to achieve a complete recovery. If the patient receives treatment, then heart failure, leading to death, will still occur, but the patient will still be able to prolong life.

If the cause of pulmonary hypertension is systemic scleroderma, then the prognosis is as unfavorable as possible. When the disease occurs, the degeneration of normal organ tissue into connective tissue occurs. As a result, a person dies within the first year.

In idiopathic pulmonary hypertension, the prognosis improves slightly. Such patients can live, on average, three years after diagnosis.

If it leads to pulmonary hypertension, then the patient is sent for surgery. The five-year survival rate of such patients is equivalent to 40-44%.

If, against the background of pulmonary hypertension, heart failure rapidly increases with damage to the right ventricle of the heart, then a lethal outcome will occur within 2 years after the manifestation of the disease.

If pulmonary hypertension has an uncomplicated course and is amenable to medical correction, then about 67% of patients cross the line of 5 years.

Treatment of pulmonary hypertension can be either conservative or surgical. Full recovery does not occur. If the human body perceives the ongoing therapy, then this helps to improve its overall well-being. A person is able to perform physical work. Life expectancy can be increased by 2 times.

To alleviate the course of the disease, as well as improve the quality and life expectancy, it is necessary to adhere to the following recommendations:

Physical activity should be limited. Especially strictly this rule must be observed immediately after a meal, and also under the condition of being in a too cold or too hot room.

Physical activity should be regular, but complexes should be made only on the basis of medical indications. This allows not to burden the heart and lungs, but to maintain adequate vascular tone.

When traveling by plane, you need to use oxygen therapy.

It is necessary to prevent various diseases of the respiratory system.

It is necessary to stop taking hormonal drugs during menopause.

You should stop taking hormonal drugs to prevent unwanted pregnancy.

It is necessary to monitor the level of hemoglobin in the blood.

Medication is the main treatment for pulmonary hypertension. For therapy, several medications are used, combining them with each other.

Drugs that a person will receive throughout life:

Antiplatelet drugs that prevent the formation of blood clots. The main representative of this drug group is Aspirin.

Anticoagulant drugs, which thin the blood, make it less viscous, which is also aimed at preventing the formation of blood clots. Medicines of this group are Heparin and Warfarin.

Cardiac glycosides that contribute to the normal functioning of the heart muscle. For this purpose, the patient is prescribed Digoxin.

Calcium antagonists are drugs that are used for the main treatment of the disease. Their use is aimed at expanding the small vessels of the lungs. The main representatives of this group are: Diltiazem and Nifedipine.

Courses are also treated with the following drugs:

Prostaglandin E and Treprostinil are drugs that are aimed at preventing the formation of blood clots, as they do not allow the blood to become viscous, while expanding the lumen of the vessels.

Bosentan is an endothelial receptor antagonist. The drug is aimed at removing vascular spasm from the small arteries of the lungs, and also prevents their cells from carrying out pathological growth.

Sildenafil is a phosphodiesterase inhibitor drug. This drug helps to relieve spasm from the vessels of the lungs and reduces the load on the right ventricle of the heart.

Oxygen therapy allows you to saturate the blood with oxygen. This is especially true in the event of adverse environmental conditions, or with a sharp deterioration in the patient's well-being. It is also possible to perform inhalations with nitric oxide, which are performed in a hospital ward. This procedure allows you to expand the vessels and alleviate the patient's condition.

If medical correction does not achieve the desired effect, the patient is referred for surgery. It is indicated when the cause of pulmonary hypertension is heart disease.

Depending on the disease, various surgical techniques can be applied, including:

Atrial septostomy. This method allows you to establish communication between the atria. It is recommended for those patients who suffer from right ventricular failure. Septostomy is used as a preparation for a patient before a heart or lung transplant operation.

Thrombandarterectomy. This procedure is aimed at removing blood clots from the pulmonary arteries. The operation allows you to reduce the load on the heart, make the symptoms of the disease less pronounced. The procedure will be performed only on the condition that the thrombotic masses have not yet begun to degenerate into connective tissues.

Organ transplantation. Lung and heart transplants are possible. The complexity of the operation makes it rare.

Modern drugs

Macitentan is a drug belonging to the group of endothelial receptor antagonists. It is prescribed as an antihypertensive agent, that is, to lower blood pressure in patients with pulmonary hypertension.

The drug is recommended for the treatment of idiopathic pulmonary hypertension, for the treatment of PAH, which develops against the background of heart disease and against the background of connective tissue pathologies. The drug is aimed at relieving the symptoms of the disease and improving the quality of life of patients.

Macitentan reduces the risk of hospitalization associated with pulmonary arterial hypertension by 51.6%. The cost of the drug is quite high and is equal to 2800 euros.

Ventavis is a solution for inhalation, in which the main active ingredient is iloprost. Ventavis is a drug with antiplatelet properties. It prevents the formation of blood clots in the pulmonary arteries by inhibiting platelet aggregation.

Another effect of the use of the drug Ventavis is a vasodilating effect on the arteries of the lungs. That is, the drug expands their lumen, thereby relieving the load from the heart muscle.

Ventavis is the only inhalation drug from the prostanoid group that is available in the Russian Federation. It is inhaled 6 to 9 times a day using a nebulizer.

Ventavis therapy has a proven effectiveness, which has been confirmed in the course of scientific studies. They included 203 patients with PAH who received monotherapy with the drug for 12 weeks. This reduced dyspnea and improved NYHA functional class in 16.5% of patients. The average increase in 6-MX distance during treatment was 36.4 m.

Also, the drug can be used as a drug in the complex therapy of pulmonary arterial hypertension. The STEP study on this issue lasted 12 weeks and included 67 patients with PAH. The therapy was supplemented with Bosentan. It was found that patients had a significant increase in physical performance, and other symptoms of the disease began to decline.

The Opitiz study involved 6 people who received the drug for 5 years. At the same time, the survival rate of people increased from 32% to 49% compared with patients who did not receive similar treatment.

The AIR 1 study was also conducted, in which people received therapy for one year and three years. Survival rates were 83% at 1 year, 78% at 2 years, and 58% at 5 years.

Therefore, it can be argued that the drug not only improves the quality of life of patients with PAH, but also prolongs it. At the same time, the cost of Ventavis is about 100,000 rubles per pack.

Tracleer is a drug whose main active ingredient is bosentan. The drug is produced in the form of tablets. Its main effect is the expansion of the pulmonary arteries, by removing vascular resistance from them. This allows you to reduce the load on the heart, reduce, improve the well-being of patients and increase the body's tolerance to physical exertion.

With this drug, you can increase life expectancy in patients with idiopathic PAH, as well as with secondary PAH. Traklir is prescribed to patients with pulmonary hypertension against the background of heart defects. It can be used for both adults and children.

The cost of the drug is 200,000 rubles per package.

Revacio is a prescription drug, the main active ingredient of which is sildenafil. The leading effect of taking the drug is the removal of spasm from the pulmonary arteries, as well as the expansion of their lumen. In addition, Revatsio allows you to reduce blood pressure, reduce the load on the heart muscle. The patient's body becomes tolerant to physical exertion, even if pulmonary arterial hypertension is severe. It has been proven that the drug improves the survival of patients with PAH.

Revatio can be used in a complex treatment regimen for pulmonary hypertension. It is used to treat all forms of the disease. The average price for a package of the drug is 50,000 rubles.

Volibris is a drug from the group of endothelin receptor antagonists. Its main active ingredient is Ambrisentan. Taking the drug can reduce the clinical symptoms of pulmonary arterial hypertension, increase the level of exercise tolerance, and improve hemodynamic parameters. The effect of the use of the drug Volibris is stable during the year.

The average price for a package of a medicinal product is 60,000 -75,000 rubles.

The drug Adempas with the main active ingredient Riociguat. This drug belongs to the pharmacological group of antihypertensive drugs, guanylate cyclase stimulants.

Taking the drug Adempas can reduce pulmonary vascular resistance, improve hemodynamics, reduce the severity of shortness of breath, reduce the clinical manifestations of the disease. The drug used in complex therapy makes it possible to increase the one-year survival rate of patients up to 96%. The drug is prescribed to patients with PAH, accompanied by the formation of blood clots, with idiopathic, hereditary PAH and with PAH associated with connective tissue pathologies.

Aptravi (Selexipag)

Aptravi is a selective non-prostanoid prostacyclin receptor agonist. It should be noted that the drug is not registered in Russia. Its main effect is the expansion of spasmodic pulmonary vessels and lowering blood pressure. The drug is used in a complex treatment regimen for PAH, as well as as an independent remedy. The average cost of one package of Aptravi is equal to 5200 euros.

Pulmonary hypertension (PH) is an increase in the average pressure in the pulmonary artery up to 25 mm Hg. Art. and more.

The result is an increase in resistance in the bloodstream, malnutrition of the right ventricle of the heart and the development of terrible complications from all organs and systems due to severe hypoxia.

In 80% of cases (according to the European Society of Cardiology) a fatal outcome occurs.

Moreover, the prospects for such a scenario are characterized by a number of 3-10 years, sometimes less with an aggressive course of the main process.

The patient population is young women under the age of 40. According to statistics, the ratio of the fairer sex to men is characterized as 4-5:1. Mortality among the strong part of humanity, however, is many times higher.

The low-symptomatic course, the absence of specific manifestations lead to the fact that the diagnosis is made at a late stage, when it is almost impossible to help. And sometimes even according to the results of a pathoanatomical study.

Specific preventive measures have not been developed, as well as early screening methods.

The pathological condition is preceded by stenosis or narrowing of the lumen of blood vessels, including small branches and medium structures extending from the pulmonary artery.

The endothelium, that is, the inner lining of the vessels, thickens. This is an adaptive mechanism.

The pressure in the pulmonary artery grows progressively, rapidly, the load on the right ventricle increases, which leads to its hypertrophy (thickening).

The contractile ability falls, the phenomena of heart failure increase. A classic pathological process is being formed -.

Everyone is different, but a favorable outcome is not always found.

Risk factors

Pulmonary hypertension is a mystery to both pulmonologists and cardiologists. The pathogenesis has not been established. We can only talk about factors that increase the risk of the formation of a pathogenic process.

Taking oral contraceptives

Destroys the normal hormonal background to the ground. Estrogen-gestagenic drugs provoke an artificial drop in progesterone, which is partially responsible for adequate inhibition of proliferative processes in the body (it disrupts the division of some cells), and it is this substance that contributes to the stable regulation of vascular tone in the fairer sex.

An increase in estrogen provokes inflammatory processes, exacerbates the course of hypertension. Apparently, this is due to the greater prevalence in women.

Pronounced and prolonged increase in blood pressure

Pulmonary problems can become a complication of cardiovascular disease. This is a natural outcome, if not treated or the wrong therapy is carried out.

Doctors are partly to blame, unable to make a correct diagnosis and respond in time to the impending threat.

Family history

In most cases, it doesn't matter. Since diseases themselves are not inherited. At the same time, other generations from the previous ones receive features of the circulatory and immune systems.

Blood pressure is observed in almost all descendants of a person suffering from hypertension. It is possible to prevent the development of diseases within the framework of prevention, but few people pay attention to this issue.

Interesting:

The potential for the formation of pathologies of the cardiovascular system is determined by the number of sick relatives and gender: women are most susceptible due to genetic characteristics.

Pulmonary hypertension in such a situation becomes secondary, develops as a result of a long-term increase in blood pressure or autoimmune pathologies.

Problems with the body's defenses

Diseases such as rheumatoid arthritis, systemic lupus erythematosus, Hashimoto's thyroiditis, and other autoimmune pathologies affect the likelihood of developing pulmonary hypertension.

According to statistics, patients with the described diagnoses suffer almost three times more often than relatively healthy people. A similar correlation was noted already 20-30 years ago.

Thus, pulmonary hypertension is idiopathic. It is difficult, if not impossible, to determine the specific causes of the development of the condition.

Specific studies on this issue are still ongoing. Determining the etiology and pathogenesis will allow developing effective therapeutic tactics, early screening and prevention measures.

Despite all the above, the disease is considered to be rare and is diagnosed in 7-13% of clinical situations, which is not so much.

The main factors in the development of the disease in patients

The reasons are also not fully understood. Partially, it was possible to identify some pathologies that can lead to the development of secondary pulmonary hypertension.

Among them:

• Congestive heart failure. Violation of the work of the atria and ventricles as a result of malnutrition or other factors. Often ends in myocardial infarction.

But one does not interfere with the other. Acute circulatory disorders can be combined with increasing symptoms of pulmonary hypertension.

• . It says the same way.

• Congenital and acquired heart defects. As a result of improper operation (for example, when), the load on the arteries is unevenly distributed. Often this leads to pathological expansion of the endothelium (internal lining of the vessel), hypertrophy.

• Chronic obstructive pulmonary disease. A destructive process that sooner or later visits smokers and workers in hazardous industries.

Determined by the mass of symptoms, pulmonary hypertension is a late consequence of the disease, its especially formidable complication, at the same time the most quiet and inconspicuous.

• Chronic thrombosis of the pulmonary artery and its branches. It affects the partial blockage of the lumen by pathological objects: blood clots, air bubbles. The first is much more common.

The process should be treated urgently, since partial blockage can become complete.

Hence the violation of blood circulation, an acute change in the nature of cardiac activity and death within a few minutes.

• Hypoventilation of the lungs. As a result of a long course of pathologies of the respiratory system: from destructive bronchitis to asthma, other processes of the same kind. Treat the underlying cause.

Etiotropic therapy should not harm the cardiovascular system. Often pulmonologists and allergists thoughtlessly prescribe glucocorticoids, completely regardless of the consequences.

And these are very common. Including an increase in pressure in the pulmonary artery, aorta.

• Myocarditis. Inflammatory pathology of the heart (middle muscle layer of the organ). Occurs as a complication of infectious diseases, less often as a primary phenomenon. It is provoked by pyogenic flora.

• Cirrhosis of the liver. Acute hepatonecrosis. In other words, a destructive process affecting hepatocytes (organ cells).

Most often found in alcoholics with experience. Less common in patients with hepatitis B, C, suffering from drug intoxication.

The lungs do not suffer in the first place, this is a symptom of an advanced disease, when the destructive process reaches 40-50% of the total volume of the liver.

The compensated form has fewer risks, although it cannot be written off.

• HIV infection. In view of the development of a weakened immune system to critical levels, patients with AIDS are observed almost without exception (every third suffers). This is another factor in the early death of patients of this profile.

Clinical classification of the pathological process

Pulmonary hypertension can be subdivided on various grounds. Both typologies presented below are recognized in medical practice.

Depending on the degree of restrictions, the severity of the process, there are several classes:

• The first . It is characterized by minimal changes in organs and systems. Pulmonary hypertension of the 1st degree is the most favorable moment for treatment, but it is incredibly difficult to identify the pathology. It is necessary to look specifically, due to the absence of symptoms, it is not immediately possible to suspect a problem.
• Second . The changes are already more pronounced. There is a thickening of the heart, but insignificant. Physical activity decreases slightly, symptoms are minimal. At this stage, it is still possible to reverse the process without any special consequences for the patient's body, but complex treatment in a hospital is already required.
• Third . The symptoms are quite clear. But still non-specific, which does not allow timely diagnosis and verification. A competent professional will be able to suspect something is wrong already by sight, it is enough to conduct specialized research.
• Fourth . Extreme, terminal stage. The clinical picture is obvious, but even in such a situation there are no characteristic symptoms. One can easily confuse the process with classical hypertension. The diagnosis is made precisely at this moment, and a little more often already during the autopsy.

The generally accepted classification, however, does not give answers where the process comes from. This question is solved by the second typification, depending on the etiology.

Accordingly, allocate:

• hereditary factor. Disputes about the influence of such a moment have been going on for more than one year, and the same will continue. Apparently, genetic predisposition is of great importance.
• Idiopathic etiology. This diagnosis is made when it is impossible to understand the situation. That is, the clinic is obvious, the reasons are not clear.
• associated form. Characterized by secondary. It develops as a result of the formation of one or another pathological process that causes the disease.
• Persistent. Appears in newborns, but the factors of formation are also not understood. A connection between the perinatal period and the problem is likely.
• Drug or toxic. As a result of long-term use of corticosteroids, tonics and certain other complex drugs with devastating side effects.

Other possible drug groups: antidepressants and mood stabilizers, antipsychotics, especially of the older generation, typical, ibuprofen and anti-inflammatory non-steroidal origin, antibiotics and oral contraceptives already mentioned at the beginning of the material.

It also affects the use of psychoactive substances. Drugs such as cocaine and amphetamines hit hard on the lung structures, heart and blood vessels.

Even a short-term reception makes itself felt by adverse consequences of this kind.

Pulmonary hypertension is formed as a response to a pathological process; it is rare as a primary disease. The exact percentage is not known.

Symptoms, general and depending on the stage

Among the manifestations typical for all stages of the pathological process:

• Shortness of breath for no apparent reason. The lungs cannot function normally as a result of poor circulation.
• Hypoxia and disruption of cellular metabolism leads to inhibition of ATP synthesis. Hence muscle weakness, inability to move normally, fatigue and other manifestations of the same kind.
• Cough. Persistent, without sputum production, unproductive and dry. Always accompanies pulmonary hypertension.
• Hoarseness, inability to control the voice.
• Tachycardia as a result of a violation of the normal supply of blood and nutrients to the heart.
• Fainting.
• Ischemia of cerebral structures and, accordingly, dizziness, cephalgia, nausea, vomiting. Up to the phenomena of a stroke.
• Edema of the lower extremities.
• Pain in the right hypochondrium. They can be primary, as a result of liver damage or secondary, as a result of the involvement of the organ in the pathological process.

The absence of specific manifestations leads to the impossibility of timely diagnosis. On the other hand, a pronounced clinical picture is formed when the pressure in the vessel increases by 2 times compared to normal values.

• 1 stage. Physical activity does not change. Intensive loads lead to dizziness, cephalgia, shortness of breath, and minor tissue hypoxia.
• Stage 2. Slight decrease in physical activity. The patient can still engage in daily habitual activities. But with some restrictions. As a result, shortness of breath, palpitations, and muscle problems occur.
• 3 stage. Significant reduction in strength. Impossible, including minimal activity.
• Stage 4. Symptoms appear even in a state of complete rest.

Moderate pulmonary hypertension is the optimal time to start therapy. Catastrophic consequences have not yet arrived, and the signs are quite pronounced.

It is even better to carry out specific treatment at the very beginning of the condition. Symptoms of pulmonary hypertension develop from the lungs, blood vessels, and heart.

Diagnostics

Examination of patients with suspected pulmonary hypertension is carried out by a pulmonologist and a cardiologist, in tandem. The scheme of diagnostic measures looks like this:

• Interrogation of the patient. Usually the first complaint that people make is shortness of breath of a pronounced nature, pressing discomfort in the chest.
• Collection of anamnesis. Heredity plays a big role, as already mentioned.
• Exploration of visual data. In patients with pulmonary hypertension, there is a deformation of the distal phalanges of the fingers, nails according to a specific type.
• Listening to breath sounds. The weakening of tones, their splitting is determined.
• Electrocardiography. To assess the general condition of the heart and the intensity of its work. It is carried out at the first moment. Allows you to notice minimal deviations.
• Echocardiography. Hypertrophy of the right ventricle is revealed.
• Tomography, primarily computed. The pulmonary arteries enlarge and dilate, which is specific to the condition in question. There are changes in the heart.
• Radiography of the lungs. Not informative enough, but more accessible.
• artery catheterization. Carried out with care, allows you to quickly measure the pressure inside the anatomical structure.
• Angiopulmonography.
• Evaluation of the tonometer indicator is routine (using a household device).
• Finally, daily monitoring may be required.

The diagnostic scheme is approximately as follows. At the discretion of the leading experts, the sequence may be changed.

Medical treatment

It is carried out as a primary measure. The drugs of the following pharmaceutical groups are prescribed:

• Vasodilators. Normalize the muscle layer. But they should be used with caution, since there is a high risk of a sharp re-stenosis with an aggravation of the condition. Dosages and names are selected by a group of doctors.
• Diuretics. They allow you to "drive" excess fluid and normalize blood pressure.
• Oxygen inhalations to compensate for the lack of a substance during natural respiration.
• Statins. Allow to fight the atherosclerotic process, if any. In most cases, it is, which aggravates the already difficult situation of the patient.
• Anticoagulants. Normalize the rheological properties of blood. Used with caution due to the possibility of fatal internal bleeding.

Conservative treatment of pulmonary hypertension is effective at stages 1-2, when the disease has not yet passed to the terminal stage. Specific names of medicines are selected by the attending physicians leading the patient.

It is necessary to choose the right dosage and combination, it is better to do it in stationary conditions (pulmonology or cardiology).

Surgery

It is indicated for the ineffectiveness of conservative methods. Includes lung and heart transplants. At the moment, in the conditions of Russian reality and the realities of the CIS countries, it is almost impossible to wait in line for such an operation.

In other states, everything is also not rosy, which is due to the imperfection of medical legislation and, as a result, a small number of potential donors.

As a surgical measure for thromboembolism, pulmonary thromboendarterectomy is prescribed.(an operation to remove a blood clot from the initial branches of the pulmonary artery).

The method allows you to remove the load from the right ventricle, but it is feasible only until the thrombus begins to degenerate into connective tissue.

Forecast

The primary form of pulmonary hypertension is unfavorable, even too much. Patients live no more than 1-2 years.

Secondary is easier, especially with a favorable response to ongoing therapy. There is a chance for compensation and good survival.

With a long-term process with a steadily high pressure in the pulmonary artery, the patient dies within 5 years.

Finally

Pulmonary hypertension is a dangerous complication of many diseases. Requires a serious approach and assistance from a whole group of specialists.

You can’t delay with the diagnosis, every day counts. With an untimely start, a lethal outcome is almost guaranteed in the short term.

Pulmonary hypertension- this is a certain state of the pulmonary system, during the course of which intravascular pressure sharply increases in the bloodstream of the pulmonary artery. Interestingly, pulmonary hypertension develops due to the influence of one of two main pathological processes: both due to a sharp increase in the volume of blood flow itself and the subsequent increase in pressure due to increased blood volume, and due to an increase in intravascular pulmonary pressure itself with an unchanged volume of blood flow. It is customary to talk about the occurrence of pulmonary hypertension when the pressure in the pulmonary artery exceeds 35 mm Hg.

Pulmonary hypertension is a complex, multicomponent pathological condition. In the course of its gradual development and disclosure of all clinical signs, the cardiovascular and pulmonary systems are gradually affected and destabilized. The advanced stages of pulmonary hypertension and its individual forms, characterized by high activity (for example, forms of idiopathic pulmonary hypertension or pulmonary hypertension with certain autoimmune lesions), may result in the development of respiratory and cardiovascular failure and subsequent death.

It must be understood that the survival of patients with pulmonary hypertension directly depends on the timely diagnosis and drug therapy of the disease. Therefore, it is necessary to clearly define the first key signs of pulmonary hypertension and the links of its pathogenesis in order to prescribe timely therapy.

Primary or idiopathic (of unknown cause) pulmonary hypertension is currently the most poorly understood subtype of pulmonary hypertension. Its main causes of development are based on genetic disorders that manifest themselves during the embryonic laying of future vessels that provide the pulmonary system. In addition, due to the same effect of genome defects, the body will experience a lack of synthesis of certain substances that can constrict or, on the contrary, expand blood vessels: endothelial factor, serotonin and a special factor angiotensin 2. In addition to the two factors described above, which are prerequisites for primary pulmonary hypertension, there is also another prerequisite factor: excessive platelet aggregation activity. As a result, many small vessels in the pulmonary circulatory system will be clogged with blood clots.

As a consequence, the intravascular pressure in the pulmonary circulatory system will rise sharply, and this pressure will affect the walls of the pulmonary artery. Since the arteries have a more reinforced muscular layer, in order to cope with the increasing pressure in their bloodstream and “push” the required amount of blood further through the vessels, the muscular part of the pulmonary artery wall will increase - its compensatory hypertrophy will develop.

In addition to hypertrophy and small thrombosis of the pulmonary arterioles, the phenomenon of concentric pulmonary artery fibrosis may also be involved in the development of primary pulmonary hypertension. During this, the lumen of the pulmonary artery itself will narrow and, as a result, the pressure of the blood flow in it will increase.

As a result of high blood pressure, the inability of normal pulmonary vessels to support the advancement of blood flow with an already higher pressure than normal, or the failure of pathologically altered vessels to promote the advancement of blood flow with normal pressure indicators, another compensatory mechanism will develop in the pulmonary circulatory system - the so-called " detours”, namely, arteriovenous shunts will open. By passing blood through these shunts, the body will try to reduce the high pressure in the pulmonary artery. But since the muscle wall of arterioles is much weaker, very soon these shunts will fail and multiple sections will form, which will also increase the value of pressure in the pulmonary artery system in pulmonary hypertension. In addition, such shunts interfere with the proper flow of blood around the circulation. In the course of this, the processes of oxygenation of the blood and the supply of oxygen to tissues are disrupted.

With secondary hypertension, the course of the disease is slightly different. Secondary pulmonary hypertension is caused by a huge number of diseases: chronic obstructive lesions of the pulmonary system (for example, COPD), congenital heart disease, thrombotic lesions of the pulmonary artery, hypoxic conditions (Pickwick's syndrome) and, of course, cardiovascular diseases. Moreover, cardiac diseases that can lead to the development of secondary pulmonary hypertension are usually divided into two subclasses: diseases that cause failure of the left ventricular function, and those diseases that will lead to an increase in pressure in the left atrial chamber.

The primary causes of pulmonary hypertension that accompany the development of left ventricular failure include ischemic damage to the left ventricular myocardium, its cardiomyopathic and myocardial damage, defects in the aortic valve system, coarctation of the aorta, and the effect of arterial hypertension on the left ventricle. Diseases that cause an increase in pressure in the left atrial chamber and the subsequent development of pulmonary hypertension include mitral stenosis, a tumor lesion of the left atrium and developmental anomalies: a three-atrial abnormal heart or the development of a pathological fibrous ring located above the mitaral valve ("supravalvular mitral ring").

During the development of secondary pulmonary hypertension, the following main pathogenetic links can be distinguished. Usually they are divided into functional and anatomical. The functional mechanisms of pulmonary hypertension develop as a result of a violation of normal or the emergence of new pathological functional features. It is on their elimination or correction that subsequent drug therapy will be directed. Anatomical mechanisms for the development of pulmonary hypertension arise from certain anatomical defects in the pulmonary artery itself or the pulmonary circulation system. These changes are almost impossible to cure with medical therapy, some of these defects can be corrected with the help of certain surgical aids.

The functional mechanisms of the development of pulmonary hypertension include the pathological Savitsky reflex, an increase in the minute volume of blood, the impact on the pulmonary artery of biologically active substances and an increase in the level of intrathoracic pressure, an increase in blood viscosity and exposure to frequent bronchopulmonary infections.

The pathological Savitsky reflex develops in response to an obstructive lesion of the bronchi. With bronchial obstruction, spastic compression (constriction) of the branches of the pulmonary artery occurs. As a result, intravascular pressure and resistance to blood flow in the pulmonary circulation significantly increase in the pulmonary artery. As a result, the normal blood flow through these vessels is disturbed, slowed down, and the tissues do not receive oxygen and nutrients in full, during which hypoxia develops. In addition, pulmonary hypertension causes hypertrophy of the muscular layer of the pulmonary artery itself (as discussed above) as well as hypertrophy and dilatation of the right heart.

The minute volume of blood in pulmonary hypertension occurs as a response to the hypoxic consequences of an increase in intravascular pressure in the pulmonary artery. Low oxygen in the blood affects certain receptors that are located in the aortic-carotid zone. During this exposure, the amount of blood that the heart can pump through itself in one minute (minute volume of blood) automatically increases. At first, this mechanism is compensatory and reduces the development of hypoxia in patients with pulmonary hypertension, but very quickly, the increased volume of blood that will pass through the narrowed arteries will lead to an even greater development and aggravation of pulmonary hypertension.

Biologically active substances are also produced due to the development of hypoxia. They cause spasm of the pulmonary artery and an increase in aortic-pulmonary pressure. The main biologically active substances that can narrow the pulmonary artery are histamines, endothelin, thromboxane, lactic acid and serotonin.

Intrathoracic pressure most often occurs with broncho-obstructive lesions of the pulmonary system. During these lesions, it rises sharply, compresses the alveolar capillaries and contributes to an increase in pressure in the pulmonary artery and the development of pulmonary hypertension.

With an increase in blood viscosity, the ability of platelets to settle and form blood clots increases. As a result, changes develop similar to those in the pathogenesis of primary hypertension.

Frequent bronchopulmonary infections have two pathways of action to exacerbate pulmonary hypertension. The first way is a violation of pulmonary ventilation and the development of hypoxia. The second is the toxic effect directly on the myocardium and the possible development of myocardial lesions of the left ventricle.

The anatomical mechanisms of the development of pulmonary hypertension include the development of the so-called reduction (reduction in the number) of the vessels of the pulmonary circulation. This is due to thrombosis and sclerosis of small vessels of the pulmonary circulation.

Thus, it is possible to distinguish the following main stages in the development of pulmonary hypertension: an increase in the level of pressure in the pulmonary artery system; malnutrition of tissues and organs and the development of their hypoxic lesions; hypertrophy and dilatation of the right heart and the development of "cor pulmonale".

Pulmonary hypertension symptoms

Since pulmonary hypertension is inherently a rather complex disease and develops in the course of the action of certain factors, its clinical signs and syndromes will be very diverse. It is important to understand that the first clinical signs of pulmonary hypertension will appear when the pressure in the bloodstream of the pulmonary artery is 2 or more times higher than normal.

The first signs of pulmonary hypertension are the appearance of shortness of breath and hypoxic organ damage. Dyspnea will be associated with a gradual decrease in the respiratory function of the lungs, due to high intra-aortic pressure and reduced blood flow in the pulmonary circulation. Shortness of breath with pulmonary hypertension develops quite early. At first, it occurs only as a result of physical exertion, but very soon it begins to appear independently of them and becomes permanent.

In addition to shortness of breath, hemoptysis also very often develops. Patients may notice the release of a small amount of sputum with streaks of blood when coughing. Hemoptysis appears due to the fact that as a result of exposure to pulmonary hypertension, blood stagnation occurs in the pulmonary circulation. As a result, part of the plasma and erythrocytes will sweat through the vessel and the appearance of individual blood streaks in the sputum.

When examining patients with pulmonary hypertension, one can detect cyanosis of the skin and a characteristic change in the phalanges of the fingers and nail plates - “drum sticks” and “hour glasses”. These changes occur due to malnutrition of tissues and the development of gradual dystrophic changes. In addition, "drumsticks" and "watch glasses" are a clear sign of bronchial obstruction, which can also be an indirect sign of the development of pulmonary hypertension.

Auscultation can determine the increase in pressure in the pulmonary artery. This will be evidenced by the amplification of 2 tones heard through the stethophonendoscope in the 2nd intercostal space on the left - the point where the pulmonary valve is usually auscultated. During diastole, blood passing through the pulmonary valve meets high blood pressure in the pulmonary artery and the sound that is heard will be much louder than normal.

But one of the most important clinical signs of the development of pulmonary hypertension will be the development of the so-called cor pulmonale. Cor pulmonale is a hypertrophic change in the right heart that develops in response to high blood pressure in the pulmonary artery. This syndrome is accompanied by a number of objective and subjective symptoms. Subjective signs of cor pulmonale syndrome in pulmonary hypertension will be the presence of constant pain in the region of the heart (cardialgia). These pains will disappear with inhalation of oxygen. The main reason for such a clinical manifestation of cor pulmonale is hypoxic myocardial damage, in the course of impaired oxygen transport to it due to high pressure in the pulmonary circulation and high resistance to normal blood flow. In addition to pain in pulmonary hypertension, strong and intermittent palpitations and general weakness can also be noted.

In addition to subjective signs, by which it is impossible to fully assess the presence or absence of a developed cor pulmonale syndrome in a patient with pulmonary hypertension, there are also objective signs. Percussion of the heart area can determine the displacement of its left border. This is due to an increase in the right ventricle and the displacement of the left sections beyond the normal boundaries of percussion. Also, an increase in the right ventricle due to its hypertrophy will lead to the fact that it will be possible to determine the pulsation or the so-called cardiac impulse along the left border of the heart.

With cor pulmonale decompensation, signs of liver enlargement will develop and the jugular veins will swell. In addition, a positive symptom of Plesh will be a characteristic indicator of cor pulmonale decompensation - when pressing on the enlarged liver, a simultaneous swelling of the jugular veins will appear.

Pulmonary hypertension degree

Pulmonary hypertension is classified according to many different features. The main features of the classification of pulmonary hypertension by stages are the degree of development of the cor pulmonale, ventilation disorders, the degree of hypoxic tissue damage, hemodynamic disturbances, radiological, electrocardiographic signs.

It is customary to distinguish 3 degrees of pulmonary hypertension: transient, stable and stable with severe circulatory failure.

Grade 1 (transient degree of pulmonary hypertension) is characterized by the absence of clinical and radiological signs. At this stage, primary and minor signs of insufficiency of external respiration will be observed.

Grade 2 pulmonary hypertension (stable stage pulmonary hypertension) will be accompanied by the development of shortness of breath, which will occur with previously habitual physical activity. In addition to shortness of breath, acrocyanosis will be observed at this stage. Objectively, an enhanced apex beat will be determined, which will indicate the beginning formation of a cor pulmonale. Auscultatory at the 2nd degree of pulmonary hypertension, it will already be possible to listen to the first signs of increased pressure in the pulmonary artery - the accent of 2 tones described above on the auscultation point of the pulmonary artery.

On a general radiograph of the thoracic region, one can see a bulging of the contour of the pulmonary artery (due to high pressure in it), expansion of the roots of the lungs (also due to high pressure in the vessels of the small pulmonary circulation). On the electrocardiogram, signs of overload of the right heart will already be determined. When examining the function of external respiration, there will be trends towards the development of arterial hypoxemia (a decrease in the amount of oxygen).

In the third stage of pulmonary hypertension, diffuse cyanosis will be added to the clinical signs described above. Cyanosis will be a characteristic shade - gray, "warm" type of cyanosis. There will also be swelling, painful enlargement of the liver and swelling of the jugular veins.

Radiologically, to the signs inherent in stage 2, the expansion of the right ventricle visible on the radiograph will also be added. On the electrocardiogram, there will be an increase in signs of overload of the right heart and hypertrophy of the right ventricle. When examining the function of external respiration, marked hypercapnia and hypoxemia will be observed, and metabolic acidosis may also occur.

Pulmonary hypertension in newborns

Pulmonary hypertension can develop not only in adulthood, but also in newborns. The reason for the occurrence of this condition lies in the features of the pulmonary system of a newborn child. At his birth, a sharp jump in intravascular pressure occurs in the pulmonary artery system. This jump is due to blood flow to the expanded lungs and the start of pulmonary circulation. It is this sharp jump in pressure in the pulmonary artery that is the primary cause of the development of pulmonary hypertension in a newborn child. With it, the circulatory system is not able to reduce and stabilize the spontaneous increase in intravascular pressure, at the first breath of the child. As a result, decompensation of pulmonary circulation occurs and characteristic changes in pulmonary hypertension occur in the body.

But pulmonary hypertension can also occur after a sharp jump in pressure in the pulmonary circulatory system. If, after such a jump, the pulmonary vascular system of the newborn is unadapted to the new physiological level of intravascular pressure in it, then this can also lead to pulmonary hypertension.

As a result of these reasons, a special compensation mechanism is launched in the body, during which it tries to reduce the pressure that is too high for it. This mechanism is similar to that in the appearance of shunts in adult pulmonary hypertension. Since the embryonic blood flow pathways have not yet been fused in a newborn child, a large shunt is automatically launched with this type of pulmonary hypertension - blood is discharged through an opening that has not yet been overgrown, through which the fetus was supplied with oxygen from the mother - the embryonic ductus arteriosus.

It is customary to talk about the presence of severe pulmonary hypertension in a newborn child when an increase in the value of intra-arterial pulmonary pressure over 37 mm is observed. rt st.

Clinically, this type of arterial hypertension will be characterized by the rapid development of cyanosis, impaired respiratory function of the child. In addition, the appearance of severe shortness of breath will come to the fore. It is important to note that this type of pulmonary hypertension in a newborn child is an extremely life-threatening condition - in the absence of prompt treatment, the death of a newborn can occur within hours of the first manifestations of the disease.

Pulmonary hypertension treatment

Treatment of pulmonary hypertension is aimed at eliminating the following factors: high intra-arterial pulmonary pressure, prevention of thrombosis, relief of hypoxia and unloading of the right heart.

One of the most effective treatments for pulmonary hypertension is the use of calcium channel blockers. The most commonly used drugs from this line of drugs are Nifedipine and Amlodipine. It is important to note that in 50% of patients with pulmonary hypertension during long-term therapy with these drugs, there is a significant reduction in clinical symptoms and improvement in general condition. Therapy with calcium channel blockers begins at first with low doses, and then gradually increases to a high daily dose (about 15 mg per day). When prescribing this therapy, it is important to periodically monitor the average level of arterial pressure in the pulmonary artery in order to adjust therapy.

When choosing a calcium channel blocker, it is also important to consider the patient's heart rate. If bradycardia (less than 60 beats per minute) is diagnosed, then Nifedipine is prescribed to treat pulmonary hypertension. If tachycardia of 100 or more beats per minute is diagnosed, then Diltiazem is the optimal drug for the treatment of pulmonary hypertension.

If pulmonary hypertension does not respond to therapy with calcium channel blockers, then treatment with prostaglandins is prescribed. These drugs trigger the expansion of narrowed pulmonary vessels and prevent platelet aggregation and the subsequent development of thrombosis in pulmonary hypertension.

In addition, patients with pulmonary hypertension are periodically prescribed oxygen therapy procedures. They are carried out with a decrease in the partial pressure of oxygen in the blood below 60-59 mm Hg.

In order to unload the right heart, diuretics are prescribed. They reduce the volume overload of the right ventricle and reduce the stagnation of venous blood in the systemic circulation.

It is also important to periodically carry out therapy with anticoagulants. More often than others, the drug Warfarin is used for these purposes. It is an indirect anticoagulant and prevents thrombosis. But when prescribing Warfarin, it is necessary to control the so-called international normal ratio - the ratio of the patient's prothrombin time to the established norm. For the use of Warfarin in pulmonary hypertension, the INR should be in the range of 2-2.5. If this index is lower, then the risk of developing massive bleeding is extremely high.

Pulmonary hypertension prognosis

The prognosis of pulmonary hypertension is generally unfavorable. Approximately 20% of reported cases of pulmonary hypertension are fatal. The type of pulmonary hypertension is also an important prognostic sign. So, with secondary pulmonary hypertension resulting from autoimmune processes, the worst prognosis of the outcome of the disease is observed: about 15% of all patients with this form die within a few years after diagnosis from gradually developing lung failure.

An important factor that can determine the life expectancy of a patient with pulmonary hypertension is also the average pressure in the pulmonary artery. With an increase in this indicator over 30 mm Hg and with its high stability (lack of response to appropriate therapy), the average life expectancy of the patient will be only 5 years.

In addition, the time of onset of signs of cardiac insufficiency plays an important role in the prognosis of the disease. With identified signs of class 3 or 4 heart failure and signs of the development of right ventricular failure, the prognosis of pulmonary hypertension is also considered extremely unfavorable.

Idiopathic (primary) pulmonary insufficiency is also characterized by poor survival. It is extremely difficult to treat and with this form of pulmonary hypertension it is almost impossible to influence the therapy on the factor that directly causes a sharp increase in pressure in the bloodstream of the pulmonary artery. The average life expectancy of such patients will be only 2.5 years (on average).

But besides the large number of negative prognostic indicators for pulmonary hypertension, there are also several positive ones. One of them is that if the signs of the disease gradually disappear during the treatment of pulmonary hypertension with calcium channel blockers (that is, the disease responds to this therapy), then the survival of patients in 95% of cases will exceed the five-year threshold.

Hypertension - what is it? Pulmonary hypertension is often diagnosed in the elderly. Among the diseases of the vascular system, this pathology takes 3rd place.

First of all, it is worth noting that pulmonary hypertension is not an independent disease, but a consequence of pathologies in the pulmonary vessels that led to an increase in blood volume. As a result, blood pressure in the pulmonary artery rises.

What causes primary hypertension?

Despite the fact that in most cases these are the consequences of another disease, the pathology can be congenital. It is this form that is diagnosed in children. It is called primary.

Primary pulmonary hypertension is currently poorly understood. Its main cause is genetic disorders in the embryo that occurred during the formation of the vascular system. This leads not only to an increase in pressure in the pulmonary artery, but also causes malfunctions in the production of certain substances in the body, such as serotonin.

Primary pulmonary hypertension can also result from elevated platelets. Blood clots clog blood vessels, resulting in increased blood pressure. In addition to thrombosis, idiopathic pulmonary hypertension occurs due to narrowing of the lumen in the vessels. This phenomenon is called pulmonary fibrosis.

If the disease is not diagnosed in time, then the lack of treatment will lead to even greater problems. The body, trying to relieve pressure in the pulmonary artery, throws excess blood to the arteriovenous shunts. Opening a "workaround" helps buy time. However, shunts are much weaker than blood vessels, wear out quickly, which leads to an even greater increase in pressure.

In addition, primary pulmonary hypertension in this case can cause circulatory disorders, which will cause other organs and tissues of the human body to suffer.

Secondary form of the disease

Violations of the secondary type arise as a result of many chronic diseases of the respiratory or cardiovascular system.

The most common causes of pulmonary hypertension are:

• tuberculosis;
• bronchial asthma;
• vasculitis;
• congenital or acquired heart defects;
• heart failure;
• pulmonary embolism;
• metabolic disease;
• long stay in the mountains.

In addition, factors that cause secondary hypertension can be:

• taking certain medications in large quantities;
• toxins;
• cirrhosis;
• obesity;
• hyperthyroidism;
• neoplasms;
• heredity;
• pregnancy.

The prognosis of survival depends on the form of pathology, stage and nature of the underlying cause. So, during the first year, about 15% of patients die from pulmonary hypertension.

In the second year of the disease, 32% of patients die, and the prognosis for survival in the third year is 50%. Less than 35 percent of 100 survive the fourth year of illness.

But these are general statistics. The individual prognosis depends on the course of the disease, namely:

• the rate of development of symptoms;
• effectiveness of treatment;
• forms of the disease.

The worst prognosis is observed in the primary form and a moderate course.

Symptoms and stages of the disease

The main symptom of the pathology, as with most diseases of the respiratory system, is shortness of breath.

However, there are special differences:

• shortness of breath is always present, even at rest;
• does not stop in a sitting position.

Signs of pulmonary hypertension common to most patients:

• weakness;
• fast fatiguability;
• persistent cough (no phlegm);
• swelling of the lower extremities;
• enlargement of the liver provokes the occurrence of pain in this area;
• chest pain can sometimes cause fainting;
• expansion of the pulmonary artery leads to infringement of the laryngeal nerve, because of this, the voice can become hoarse.

It is not uncommon for a patient to begin to lose weight regardless of their diet. Not only the physical, but also the psychological state worsens, apathy appears.

Depending on how pronounced the symptoms are, pulmonary hypertension can be divided into 4 stages.

1. At the first stage, there are no symptoms.
2. A decrease in physical activity indicates the beginning of the second stage of the disease. Shortness of breath, weakness, and dizziness may occur. However, in a state of rest, all unpleasant sensations go away.
3. The third stage is characterized by the presence of all symptoms that can persist even during rest.
4. In the fourth stage, the symptoms are pronounced, shortness of breath and weakness are constantly present.

Moderate pulmonary hypertension is considered the most dangerous. The weak severity of symptoms does not allow to establish an accurate diagnosis, which leads to incorrect treatment and the development of complications.

Methods of diagnosis and treatment

It is quite difficult to diagnose the disease, especially if it is idiopathic hypertension, the treatment of which should be timely. Comprehensive diagnostics is needed, including such methods;

• examination by a cardiologist and pulmonologist;
• electrocardiogram;
• echocardiography;
• CT scan;
• Ultrasound of the heart;
• general and biochemical blood test;
• measurement of pressure in the artery of the lung.

Diagnosis of hypertension is a complex process. But only according to its results, the doctor can make a diagnosis and begin to treat the pathology. The basis of any therapy is pressure reduction. Treatment can be medical, non-pharmacological or surgical.

Treatment with folk remedies is not welcome. Some doctors may supplement therapy with traditional medicine, but this is rare. In any case, it is necessary to give preference to the advice of a specialist.

Drug treatment consists in taking such drugs:

• diuretics;
• anticoagulants;
• prostaglandins;
• antibiotics (if necessary);
• nitric oxide inhalation.

This video talks about pulmonary hypertension:

With the ineffectiveness of medications, surgical treatment is prescribed. Typically, the following methods are used:

1. Atrial septostomy. An opening is created between the atria, which reduces pressure in the lung artery.
2. In the presence of blood clots, thromboendarterectomy is performed.
3. Lung and/or heart transplantation is used in the most severe cases.

Non-drug method:

• drinking at least 1.5 liters of fluid daily;
• blood oxygen saturation;
• bed rest.

Treatment with folk remedies is most often combined with drug therapy.

This video talks about the treatment of pulmonary hypertension:

Hypertension is a serious disease, often fatal. The best prevention is regular examinations, this is the only way to identify pathology at the beginning of development.

Among the diseases of the cardiovascular system, there are many that can lead to serious complications and death of a person, and sometimes in the early stages from the onset of development. Progressive, deadly pathology of pulmonary hypertension can occur in newborns, older children, adults, causes an increase in blood pressure in the pulmonary artery and ends in death. It is extremely important to start treating pulmonary hypertension early, which will help improve the prognosis and prolong a person's life.

What is pulmonary hypertension

Pulmonary hypertension, or pulmonary hypertension (PH) is a group of pathologies in which there is a progressive increase in pulmonary vascular resistance, which provokes right ventricular failure and premature death of a person. This disease is a severe type of cardiovascular disease, covering the pulmonary circulation, because sooner or later it leads to a strong drop in physical endurance and the development of heart failure. Such a pathology as cor pulmonale has a close relationship with pulmonary hypertension and occurs in conjunction with it.

The mechanism of the development of the disease is as follows. The inner layer of the vessels of the lungs (endothelium) grows, reduces the lumen of the arterioles, thereby disturbing the blood flow. The resistance in the vessels grows, the right ventricle has to contract strongly in order to normally push blood into the lungs, for which it is completely unsuited. As a compensatory reaction of the body, the myocardium of the ventricle thickens, the right parts of the heart hypertrophy, but then there is a sharp drop in the strength of the contractions of the heart, death occurs.

In the syndrome of pulmonary hypertension in humans, the average pressure in the pulmonary artery is 30 mm Hg. and higher. Primary PH usually occurs in children from birth, and later the resulting disease is recognized as secondary and occurs much more often. The prevalence of secondary PH is higher than the incidence of primary PH due to the presence of a large number of cases of chronic cardiovascular diseases and lower respiratory tract lesions.

Approximately 20 people per 1 million population per year develop this pathology, and in those suffering from chronic pulmonary diseases with hypoventilation of the lungs, it occurs in literally 50% of clinical cases.

Primary PH has a very poor prognosis for survival; in the secondary form, it is possible to increase life expectancy through timely treatment.

Disease classification

Pulmonary hypertension is primarily divided into primary and secondary. In turn, primary hypertension (Aerz's disease) was divided into obliterating, arterial reticular, thromboembolic forms. A more in-depth classification, including an understanding of the mechanisms of the disease, includes the following types of disease:

1. pulmonary arterial hypertension (more common than other types);
2. venous hypertension;
3. pulmonary capillary hemangiomatosis;
4. hypertension with damage to the left chambers of the heart, including systolic dysfunction of the right or left ventricle, damage to the valves of the left parts of the heart;
5. pulmonary hypertension against the background of diseases of the respiratory system (COPD, interstitial diseases, night breathing disorders, high-altitude pulmonary hypertension, malformations of the lungs);
6. chronic thromboembolic (post-embolic) pulmonary hypertension; Familiarize yourself with the Thromboembolic Complications Risk Scale
7. pulmonary hypertension with an unclear mechanism of development.

The most common form of arterial hypertension of the lungs, which is divided into forms:

• idiopathic;
• hereditary (caused by mutation of the second type receptor gene for bone morphogenesis protein, or provoked by a mutation of the activin-like kinase-1 gene, or other unknown mutations);
• drug and toxic;
• associated with connective tissue diseases, CHD (congenital heart disease), HIV and AIDS, chronic hemolytic anemia, schistosomiasis, etc.;
• persistent neonatal hypertension.

According to the degree of functional disorders, the disease is divided into classes:

1. the first is normal physical activity, good exercise tolerance, first-degree heart failure (mild or borderline PH);
2. the second - physical activity is reduced, the patient feels comfortable only without exertion, and with simple tension, characteristic symptoms appear - shortness of breath, chest pain, etc. (moderate PH);
3. the third - unpleasant symptoms occur at the smallest loads (high degree of PH, worse prognosis);
4. the fourth - intolerance to any load, all the symptoms of the disease are expressed even at rest, there are severe symptoms of stagnation in the lungs, hypertensive crises, abdominal dropsy, etc. join.

The classification according to the size of the discharge of blood is as follows:

1. discharge not higher than 30% of the minute volume of the pulmonary circulation;
2. reset rises to 50%;
3. more than 70% blood loss.

Differentiation of the disease by pressure is as follows:

1. the first group - the pressure in the pulmonary artery is less than 30 mm Hg;
2. the second group - pressure 30-50 mm Hg;
3. the third group - pressure 50-70 mm Hg;
4. the fourth group - pressure above 70 mm Hg.

Causes

Primary pulmonary hypertension, or Aerz's disease, is a very rare disease whose causes are not yet clear. It is assumed that other autoimmune diseases and disorders of the homeostasis system (especially high platelet activity) can somehow affect the development of primary PH. Pathology leads to a primary lesion of the vascular endothelium against the background of an increase in the production of the vasoconstrictor substance endothelin, to fibrosis and necrosis of the walls of the branches of the pulmonary artery, which causes an increase in pressure and general pulmonary resistance.

As for other forms of primary hypertension, they can be caused by burdened heredity or gene mutation at the time of conception. The mechanism of the development of the disease in this case is similar: an imbalance in the exchange of nitrogen compounds - a change in vascular tone - inflammation - proliferation of the endothelium - a decrease in the internal caliber of the arteries.

Secondary pulmonary hypertension allows you to track its etiology, which can be very diverse. There are two mechanisms for the development of the disease:

1. Functional - the normal operation of certain parts of the body is disturbed, therefore, all the changes characteristic of PH occur. Treatment is aimed at eliminating the pathological influence and can be quite successful.
2. Anatomical. PH is associated with the presence of a defect in the structure of the lungs or the pulmonary circulation. Usually this type of disease does not respond to medications and can only be corrected surgically, but not always.

Most often, PH is caused by pathology of the heart and lungs. Heart diseases that can lead to the development of pulmonary hypertension include:

• CHD (atrial, ventricular septal defect, open ductus arteriosus, mitral valve stenosis, etc.);
• severe hypertension;
• cardiomyopathy;
• cardiac ischemia;
• complications after surgery on the heart and coronary vessels;
• chronic or acute pulmonary thrombosis;
• atrial tumors.

No less often, the causes of PH are reduced to the presence of chronic diseases of the lower respiratory tract, which lead to changes in the structure of lung tissues and to alveolar hypoxia:

• bronchiectasis - the formation of cavities in the lungs and their suppuration;
• obstructive bronchitis with the closure of part of the airways;
• fibrosis of the lung tissue and its replacement with connective tissue cells;
• lung tumor that compresses blood vessels.

The following risk factors can contribute to the development of the disease:

• poisoning with poisons, toxins, chemicals;
• taking drugs;
• excessive consumption of anorectics, antidepressants;
• pregnancy, especially multiple;
• living in a high mountain area;
• HIV infection;
• cirrhosis of the liver;
• tumor diseases of the blood;
• increased pressure in the portal vein (portal hypertension);
• chest deformity;
• pronounced obesity;
• thyrotoxicosis;
• some rare hereditary diseases.

Other, less common causes, the mechanism of action of which on this area of ​​the body is not always clear, can also cause secondary hypertension. These include myeloproliferative diseases, removal of the spleen, vasculitis, sarcoidosis, lymphangioleiomyomatosis, neurofibromatosis, Gaucher disease, pathologies of glycogen accumulation, hemodialysis, etc.

Symptoms of manifestation

At the very beginning of its development, the disease is compensated, therefore it proceeds without symptoms. The norm of pressure in the artery is 30 mm Hg. by systolic pressure, 15 mm Hg - diastolic. When this norm is exceeded by 1.5-2 times, the clinic of the disease becomes apparent. Sometimes secondary pulmonary hypertension is diagnosed only when the stage is already running, changes in the body are irreversible.

Symptoms of pulmonary hypertension are most often non-specific, and even a doctor can confuse them with other cardiac pathologies if a thorough examination is not carried out. However, the main symptom - shortness of breath - still has a number of characteristic features. Shortness of breath can also appear at rest, increases even with little physical exertion, does not stop in a sitting position, while shortness of breath with other heart diseases subsides under such conditions.

The most initial symptoms of PH during the development of uncompensated or partially compensated stages are as follows:

• weight loss when taking into account normal nutrition;
• weakness, loss of strength, depressed mood, general poor health;
• hoarseness, hoarseness of voice;
• frequent coughing, coughing;
• a feeling of bloating, fullness in the abdomen due to the onset of stagnation in the portal vein system;
• nausea, dizziness;
• fainting;
• increased heart rate;
• stronger pulsation of the jugular veins than usual.

In the future, without adequate treatment, the patient's condition is greatly aggravated. Other clinical signs of PH are also added - sputum with blood, hemoptysis, angina attacks with chest pain, fear of death. Different types of arrhythmias develop, more often - atrial fibrillation. By this stage, the liver is already seriously enlarged in size, its capsule is stretched, so the person is worried about pain in the right hypochondrium, a sharp increase in the abdomen. Due to the developed heart failure, edema also appears on the legs in the region of the legs and feet.

In the terminal stage, suffocation increases, blood clots appear in the lungs, some tissues die due to lack of blood supply. There are hypertensive crises, bouts of pulmonary edema. During a nocturnal attack, the patient may die of suffocation. The attack is accompanied by a lack of air, a strong cough, the release of blood from the lungs, blue skin, severe swelling of the veins in the neck. Uncontrolled excretion of feces and urine is possible. A hypertensive crisis can also be fatal, but most often, patients with pulmonary hypertension die from acute heart failure or PE.

Complications and their prevention

The most common complication of the disease is atrial fibrillation. This disease in itself is dangerous by the development of ventricular fibrillation, which, in fact, is a clinical death from cardiac arrest. Also, an inevitable and dangerous complication is pulmonary edema and a hypertensive crisis, after which a person's condition, as a rule, deteriorates sharply, and in the future he is assigned a disability. The consequence of advanced pulmonary hypertension is right ventricular failure, hypertrophy and dilatation of the right heart, thrombosis of the pulmonary arterioles. A lethal outcome is possible both from a combination of all these complications, which differ in a progressive course, and from pulmonary embolism - acute blockage of a vessel by a thrombus and circulatory arrest through it.

Diagnosis of pathology

Since primary pulmonary hypertension is very rare, a detailed and very thorough examination should be carried out to find the cause of PH, which is more often secondary. For this purpose, as well as to assess the severity of the pathology, the following examination is carried out:

1. External examination, physical examination. The doctor pays attention to cyanosis of the skin, swelling of the legs and abdomen, deformity of the distal phalanges, a change in the shape of the nails like watch glasses, and shortness of breath. During auscultation of the heart, an accent of the second tone is heard, its splitting in the region of the pulmonary artery. With percussion, an expansion of the cardiac boundaries is noticeable.
2. ECG. There are signs of overload of the right ventricle against the background of its expansion and thickening. Often there are objective data on the presence of extrasystole, atrial fibrillation, atrial fibrillation.
3. Chest X-ray. Radiographic signs of PH - an increase in the size of the heart, an increase in the peripheral transparency of the lung fields, an increase in the roots of the lungs, a shift in the borders of the heart to the right.
4. Echocardiography (ultrasound of the heart). Determines the size of the heart, hypertrophy and stretching of the right cavities of the heart, allows you to calculate the amount of pressure in the pulmonary artery, and also detects heart defects and other pathologies.
5. Functional breathing tests, blood gas analysis. They will help clarify the diagnosis, the degree of respiratory failure.
6. Scintigraphy, CT, MRI. Necessary to study the state of small pulmonary vessels, to search for blood clots.
7. Cardiac catheterization. Required for direct measurement of pulmonary artery pressure.

To assess the severity of the disease and the degree of violations from other organs, the patient may be given recommendations to do spirometry, abdominal ultrasound, complete blood count, urine test for the study of kidney function, etc.

Methods of treatment Conservative treatment

The goal of conservative therapy is the elimination of etiological factors, or their correction, reduction of pressure in the pulmonary artery, prevention of complications, especially thrombosis. Treatment is most often carried out in the hospital, after removing the exacerbation - at home. For this, the patient is prescribed to take various medications:

1. Vasodilators (calcium channel blockers) - Nifedipine, Prazosin. They are especially effective in the early stages of pathology, when there are no pronounced disorders in the arterioles yet.
2. Disaggregants - Aspirin, Cardiomagnyl. Essential for blood thinning.
3. At the level of hemoglobin with LH above 170 g/l, as well as with bulging of the cervical veins, bloodletting of 200-500 ml should be performed More about cervical thrombus
4. Diuretics - Lasix, Furosemide. They are used in the development of right ventricular failure.
5. Cardiac glycosides - Digoxin. They are prescribed only in the presence of atrial fibrillation in a patient to reduce the heart rate.
6. Anticoagulant drugs - Warfarin, Heparin. They are prescribed for a tendency to form blood clots.
7. Prostaglandins, prostaglandin analogues - Epoprostenol, Treprostinil. Reduce pressure in the pulmonary artery, slow down the pathological transformation of the pulmonary vessels.
8. Endothelin receptor antagonists - Bosentan. Help reduce the rate of endothelin production and slow down the progression of PH.
9. Drugs to improve tissue metabolism - Riboxin, Potassium Orotat, vitamins.
10. Various drugs for the treatment of underlying pulmonary and cardiac diseases, other pathologies that caused the development of pulmonary hypertension.

All patients with pulmonary hypertension are shown ozone therapy, oxygen therapy - oxygen inhalation. A positive effect is achieved after a course of oxygen treatment, so it is recommended up to several times a year.

Surgical treatments

In some situations, surgery helps to reduce the progression of the disease and increase life expectancy. In people with pulmonary hypertension, the following surgical procedures may be used:

1. Interatrial shunting or balloon atrial septostomy. When an artificial opening (open oval window) is created between the atria, high pulmonary hypertension becomes lower, thereby improving the prognosis.
2. Lung transplant. For a pronounced reduction in pressure, transplantation of only one lung is sufficient. However, within 5 years after such an operation, bronchiolitis obliterans develops in half of the patients as a reaction of rejection of a new organ, and therefore long-term survival is in doubt.
3. Heart and lung transplant. It is possible only in the last stages of the disease, which is provoked by CHD or cardiomyopathy. If an operation is performed at an early stage of the pathology, life expectancy does not increase.

Folk remedies and nutrition

It is impossible to completely cure pulmonary hypertension without eliminating its causes even by traditional methods, not to mention the treatment of folk remedies. But still, the advice of traditional healers will help reduce the symptoms of the disease and in the complex of therapy they can participate in it:

• Brew a tablespoon of red rowan fruits with 250 ml of boiling water, leave for an hour. Drink half a glass 3 times a day in courses for a month.
• Pour 250 ml of boiling water over a teaspoon of herbs and flowers of spring adonis, let it brew, take 2 tablespoons of the infusion on an empty stomach three times a day for 21 days.
• Drink 100 ml of fresh pumpkin juice daily, which will help get rid of arrhythmias in PH.

Nutrition in this pathology limits salt, animal fats, the amount of fluid consumed. In general, the emphasis in the diet should be on plant foods, and animal products should be eaten in moderation, and only healthy, low-fat foods. There are some lifestyle recommendations for patients with pulmonary hypertension:

1. Vaccination against all possible infectious pathologies - influenza, rubella. This will help to avoid exacerbation of existing autoimmune diseases, if the patient has them.
2. Dosed physical exercises. For any cardiac pathology, a person is prescribed a special exercise therapy, and only in the last stages of PH should classes be limited or excluded.
3. Prevention or termination of pregnancy. An increase in the load on the heart in women with pulmonary hypertension can lead to death, so pregnancy with this pathology is strongly discouraged.
4. Visiting a psychologist Usually, people with PH develop depressive states, neuropsychic balance is disturbed, therefore, if necessary, they need to visit a specialist to improve their emotional state.

Features of treatment in children and newborns

In childhood, secondary PH most often occurs due to hypoxia or pathology of the respiratory system. Treatment should be based on the class of disease severity and is generally similar to that in adults. The child immediately after the end of the diagnosis is hospitalized in a specialized center, in the children's department. Without fail, to maintain the normal state of the muscles, the child must perform daily dosed physical activity that does not cause any complaints. It is extremely important to prevent infectious diseases, hypothermia.

Cardiac glycosides are given to children only in short courses, diuretics are selected taking into account maintaining the balance of electrolytes. The use of anticoagulants in children is a controversial issue, since their complete safety at an early age has not yet been proven. The only possible drug for use is Warfarin, which is taken in the form of tablets if necessary. Without fail, vasodilators are prescribed that lower the pressure in the pulmonary artery, which are initially introduced into the course of therapy at a minimum dose, and then it is adjusted.

If there is no effect from treatment with calcium channel blockers - the simplest type of vasodilators - other drugs of the same action are prescribed - prostaglandins, phosphodiesterase-5 inhibitors, endothelin receptor antagonists (they are a priority in childhood), etc. In pediatrics, the most effective specific drug for pulmonary hypertension is Bosentan, which is used from the age of 2-3 years. In addition, the child is prescribed massage, exercise therapy, spa treatment. In newborns, predominantly, only primary pulmonary hypertension occurs, or pathology against the background of severe CHD, which is treated in a similar way, but has an unfavorable prognosis.

Forecast and life expectancy

The prognosis depends on the cause of the disease, as well as on the level of pressure in the artery. If the response to therapy is positive, the prognosis improves. The most unfavorable situation is for those patients who have a consistently high level of pressure in the pulmonary artery. With a decompensated degree of the disease, people usually live no more than 5 years. The prognosis for primary pulmonary hypertension is extremely unfavorable - the survival rate after a year is 68%, after 5 years - only 30%.

Preventive measures

The main measures to prevent the disease:

• to give up smoking;
• regular physical activity, but without excesses;
• proper nutrition, refusal to abuse salt;
• timely therapy of the main pulmonary, cardiac pathology, which is provoked by PH;
• early start of dispensary observation of persons with COPD and other lung diseases;
• exclusion of stress.

Pulmonary hypertension in COPD. The development of pulmonary hypertension is considered one of the most important prognostic factors for patients with COPD. Numerous studies have shown the predictive value of parameters such as RV dysfunction, mean pulmonary artery pressure, and pulmonary vascular resistance (PVR). The results of Burrows' 7-year follow-up of 50 patients with COPD showed that pulmonary vascular resistance is one of the most important predictors of patient survival. None of the patients with a value of this parameter above 550 dinhshems5 lived for more than 3 years.

According to the data obtained on the basis of several long-term studies, the mortality of patients with COPD is closely related to the degree of pulmonary hypertension. At an average pressure level in the pulmonary artery of 20-30 mm Hg. The 5-year survival rate of patients is 70-90%, with values ​​of this indicator of 30-50 mm Hg. - 30%, and in severe pulmonary hypertension (mean pressure in the pulmonary artery more than 50 mm Hg), the 5-year survival rate of patients is almost equal to a bullet. Similar data were obtained in a recently published study of the Strasbourg group: the authors compared the survival of COPD patients with a pressure in the pulmonary artery less than 20 mm Hg, 20-40 mm Hg. and more than 40 mm p I I g. The highest mortality was observed in patients with severe pulmonary hypertension (Fig. 1).

Rice. 1. Survival of COPD patients depending on the severity of pulmonary hypertension

The level of pressure in the pulmonary artery is considered not only a prognostic factor, but also a predictor of hospitalization in patients with COPD. In the Kessier study, which included 64 patients with COPD, an increase in pressure in the pulmonary artery at rest of more than 18 mm Hg. turned out to be the strongest independent risk factor for hospitalization of patients (Fig. 2). This relationship indicates the possibility of identifying the group of the most vulnerable patients in need of aggressive therapy. Thus, active correction of pulmonary hypertension in COPD patients can significantly improve their functional status and reduce the frequency of hospitalization.

Rice. 2. A branch of the pulmonary artery (arteriole) in a patient with COPD: intimal hyperplasia, moderate hypertrophy of the media. Stained with hematoxylin and eosin. SW. x 200.

Pulmonary hypertension in idiopathic pulmonary fibrosis. Pulmonary hypertension is considered an unfavorable prognostic factor in patients with idiopathic pulmonary fibrosis. According to Lettieri, mortality during the first year among patients with idiopathic pulmonary fibrosis with pulmonary hypertension was 28%, and among patients with this pathology, but without pulmonary hypertension - 5.5%. According to data obtained at the Mauo Clinic, the median survival of patients with systolic pressure in the pulmonary artery more than 50 mm Hg. (according to the results of EchoCG)) was 8.5 months, and patients with systolic pressure in the pulmonary artery less than 50 mm Hg. - 4 years.

Avdeev S.N.

Secondary pulmonary hypertension

A pathological condition, the course of which is characterized by an increase in pressure in the pulmonary artery, is called pulmonary hypertension in medicine. In terms of frequency, the disease ranks third worldwide among vascular diseases characteristic of the elderly.

Causes of Pulmonary Hypertension

Pulmonary hypertension can be either a congenital anomaly, that is, primary, or acquired, which is called secondary.

The following factors may be the reason for the increase in pressure in the arteries of the lungs:

• heart failure;
• vasculitis;
• heart defects of various origins;
• chronic lung diseases, including tuberculosis, bronchial asthma, etc.;
• PE or other lesions of the pulmonary vessels;
• metabolic disorders;
• location in high mountain regions.

In cases where it is not possible to determine the exact causes of hypertension, the doctor makes the diagnosis of primary hypertension. As a disease state of unknown origin, primary pulmonary hypertension can be triggered by various contraceptives or result from an autoimmune disease.

Secondary pulmonary disease can be caused by pathologies of the heart muscle, lungs or blood vessels.

Disease classification

Depending on the severity of the course of the disease state, physicians distinguish 4 classes of patients:

1. The first stage, not accompanied by loss of physical activity. A patient with pulmonary insufficiency can withstand normal activities without the appearance of weakness, dizziness, chest pain or shortness of breath.
2. At the second stage disease, the patient's physical activity is limited. At rest, there are no complaints, but the usual load causes severe shortness of breath, dizziness and severe weakness.
3. Third stage of hypertension causes the above symptoms with the slightest physical activity of a person suffering from this disease.
4. Pulmonary hypertension stage IV marked by pronounced signs of weakness, shortness of breath and pain, even when a person is in a state of absolute rest.

Symptoms of lung failure

The main symptom of the disease is shortness of breath, which has several specific features that make it possible to distinguish it from the symptoms of other diseases:

schematic structure of the vessels of the lungs

• observed at rest;
• its intensity increases with minimal physical activity;
• in a sitting position, shortness of breath does not stop, unlike shortness of breath of cardiac origin.

Other signs of pulmonary hypertension are also characteristic of most patients:

• weakness and fatigue;
• persistent dry cough;
• swelling of the legs;
• pain in the liver area caused by its enlargement;
• pain in the sternum due to the fact that the pulmonary artery expands;
• in some cases, a hoarse voice is observed. This is due to the fact that the artery, when expanded, infringes on the laryngeal nerve.

Thus, pulmonary hypertension, the symptoms of which are often not specific, does not always allow a correct diagnosis to be made without a whole range of examinations.

Diagnosis of the disease

As a rule, patients come to doctors with complaints of severe shortness of breath, which interferes with their daily life. Since primary pulmonary hypertension does not have specific symptoms that allow a diagnosis to be made with confidence at the first visit to the doctor, the diagnosis should be carried out with the participation of a cardiologist and pulmonologist.

The complex of procedures involved in the process of making a diagnosis includes the following methods:

• medical examination and history taking. Often the disease has hereditary causes, so it is extremely important to collect information about family ailments;
• clarification of the current lifestyle of the patient. Smoking, refraining from physical activity, taking various medications - all this is important in finding out the causes of shortness of breath;
• general inspection. On it, the doctor has the opportunity to identify the physical condition of the veins in the neck, skin color (blue in case of hypertension), enlargement of the liver, the occurrence of edema in the legs, thickening of the fingers;
• electrocardiogram. The procedure allows you to identify changes in the right side of the heart;
• echocardiography helps to determine the rate of passage of blood and the general condition of the vessels;
• will show, using a layered image, an increase in the pulmonary artery, as well as possible concomitant ailments of the lungs and heart;
• radiography of the lungs will allow you to observe the condition of the artery, its expansion and narrowing;
• the catheterization method is used to reliably measure the pressure inside the pulmonary artery. Doctors consider this procedure not only the most informative for obtaining pressure values, but also associated with minimal risks of complications;
• the 6-minute walk test helps determine the patient's physical response to stress and establish the class of hypertension;
• blood test: biochemical and general;
• angiopulmonography allows, by introducing a special contrast agent into the vessels, to obtain a complete picture of the vessels in the region of the pulmonary artery. The method must be used with extreme caution, since its use can provoke a hypertensive crisis in the patient.

Thus, pulmonary hypertension should be diagnosed only after a thorough comprehensive study of the state of the patient's vessels in order to exclude an erroneous diagnosis.

Reason to see a doctor

The patient should consult a doctor if he feels the following signs of malaise:

• the occurrence or increase in shortness of breath when performing normal daily activities;
• the appearance of pain of undiagnosed origin in the chest;
• if the patient has an inexplicable and persistent feeling of fatigue;
• the appearance or increase in the degree of swelling.

Treatment of pulmonary insufficiency

In most cases, primary pulmonary hypertension is treatable. The main guidelines for choosing a treatment method are:

• identification and elimination of the cause of the patient's condition;
• decrease in pressure in the artery of the lung;
• preventing the formation of blood clots in the patient's vascular system.

When prescribing treatment, the doctor may prescribe the following drugs:

• medicines that act relaxing on the muscular layer of blood vessels. This group of drugs is very effective in the initial stages of the disease. Pulmonary hypertension, the treatment of which was started even before the vascular changes become pronounced and irreversible, has a very good chance of completely getting rid of the patient's symptoms;
• medicines designed to lower the viscosity of the blood. If the blood clotting is very severe, the doctor may decide to bleed. The hemoglobin level in such patients should not exceed 170 g/l;
• with severe shortness of breath and hypoxia, oxygen inhalations are indicated as a means of relieving unpleasant physiological symptoms;
• Your doctor may recommend reducing your salt intake and reducing your fluid intake to 1.5 liters of clean water per day.
• a strict ban on intense physical activity. Only such activity is allowed in which the patient does not feel discomfort and painful manifestations;
• if pulmonary hypertension is accompanied by a complication in the form of insufficiency of the right ventricle of the heart, the doctor prescribes regular intake of diuretic drugs;
• in the most advanced cases of the disease, they resort to a heart and lung transplant procedure. The technique has not yet been sufficiently developed in practical conditions, but the statistics of such transplantations testify to their effectiveness.

Possible Complications

Among the negative consequences of the disease, the main ones can be distinguished:

• exacerbation of heart failure. The right parts of the heart can no longer cope with their current load, which further worsens the patient's situation;
• a condition called PE is a thrombosis of an artery in the lung when a blood clot blocks a vessel. This disease is not only dangerous, but directly threatens the life of the patient;
• associated with pulmonary edema.

As a rule, the disease significantly reduces the patient's standard of living and in most cases leads to premature death.

At the same time, pulmonary hypertension causes chronic or acute forms of heart and lung failure, life-threatening for the patient.

Prevention

In order to minimize the risk of this disease, the following measures must be taken:

• adhere to the principles of a healthy lifestyle. In particular, it is necessary to categorically give up smoking and do physical exercises daily;
• it is necessary to timely identify and treat the underlying diseases that entail hypertension. This is possible in the case of regular preventive visits to the doctor;
• with established diseases of the bronchi and lungs of a chronic nature, you need to carefully monitor the course of the disease. Dispensary observation will avoid complications of the disease and facilitate its course;
• diagnosed pulmonary hypertension is not a ban on any physical activity. On the contrary, such patients are shown a moderate exercise in the fresh air. Activity should be regular, but never more intense;
• all situations that entail a stressful state must be excluded. Conflicts at work, at home or in transport can exacerbate the disease.

Thus, the sooner the patient turns to the doctor for advice and the more carefully he follows his instructions, the more likely it is to stop the course of the disease and not transfer it to a more severe stage that is less treatable.