Guidelines for pulmonary hypertension. Pulmonary hypertension. Lung or heart-lung transplant

An increase in pressure in the pulmonary capillary system (pulmonary hypertension, hypertension) is most often a secondary disease that is not directly related to vascular damage. The primary conditions are not well understood, but the role of the vasoconstrictor mechanism, thickening of the arterial wall, fibrosis (tissue thickening) has been proven.

In accordance with the ICD-10 (International Classification of Diseases), only the primary form of pathology is coded as I27.0. All secondary signs are added as complications to the underlying chronic disease.

Some features of the blood supply to the lungs

The lungs have a double blood supply: the system of arterioles, capillaries and venules is included in gas exchange. And the tissue itself receives nutrition from the bronchial arteries.

The pulmonary artery is divided into the right and left trunk, then into branches and lobar vessels of large, medium and small caliber. The smallest arterioles (part of the capillary network) have a diameter 6–7 times greater than in the systemic circulation. Their powerful muscles are able to narrow, completely close or expand the arterial bed.

With narrowing, resistance to blood flow increases and internal pressure in the vessels increases, expansion reduces pressure, reduces the resistance force. The occurrence of pulmonary hypertension depends on this mechanism. The total network of pulmonary capillaries covers an area of ​​140 m2.

The veins of the pulmonary circulation are wider and shorter than in the peripheral circulation. But they also have a strong muscle layer, are able to influence the pumping of blood towards the left atrium.

How is pressure in the pulmonary vessels regulated?

The value of arterial pressure in the pulmonary vessels is regulated by:

• pressor receptors in the vascular wall;
• branches of the vagus nerve;
• sympathetic nerve.

Extensive receptor zones are located in large and medium-sized arteries, in places of branching, in veins. Spasm of the arteries leads to impaired oxygen saturation of the blood. And tissue hypoxia contributes to the release into the blood of substances that increase tone and cause pulmonary hypertension.

Irritation of the vagus nerve fibers increases blood flow through the lung tissue. The sympathetic nerve, on the contrary, causes a vasoconstrictor effect. Under normal conditions, their interaction is balanced.

The following indicators of pressure in the pulmonary artery are taken as the norm:

• systolic (upper level) - from 23 to 26 mm Hg;
• diastolic - from 7 to 9.

Pulmonary arterial hypertension, according to international experts, starts from the upper level - 30 mm Hg. Art.

Factors causing hypertension in the small circle

The main factors of pathology, according to the classification of V. Parin, are divided into 2 subspecies. Functional factors include:

• constriction of arterioles in response to low oxygen content and high concentration of carbon dioxide in the inhaled air;
• increase in the minute volume of passing blood;
• increased intrabronchial pressure;
• increase in blood viscosity;
• left ventricular failure.

The anatomical factors include:

• complete obliteration (overlapping of the lumen) of vessels by a thrombus or embolism;
• disturbed outflow from zonal veins due to their compression in case of aneurysm, tumor, mitral stenosis;
• change in blood circulation after removal of the lung by surgery.

What causes secondary pulmonary hypertension?

Secondary pulmonary hypertension appears due to known chronic diseases of the lungs and heart. These include:

• chronic inflammatory diseases of the bronchi and lung tissue (pneumosclerosis, emphysema, tuberculosis, sarcoidosis);
• thoracogenic pathology in violation of the structure of the chest and spine (Bekhterev's disease, the consequences of thoracoplasty, kyphoscoliosis, Pickwick's syndrome in obese people);
• mitral stenosis;
• congenital heart defects (for example, non-closure of the ductus arteriosus, "windows" in the interatrial and interventricular septum);
• tumors of the heart and lungs;
• diseases accompanied by thromboembolism;
• vasculitis in the area of ​​the pulmonary artery.

What causes primary hypertension?

Primary pulmonary hypertension is also called idiopathic, isolated. The prevalence of pathology is 2 people per 1 million inhabitants. The ultimate reasons remain unclear.

It is established that women make up 60% of patients. Pathology is detected both in childhood and in old age, but the average age of identified patients is 35 years.

In the development of pathology, 4 factors are important:

• primary atherosclerotic process in the pulmonary artery;
• congenital inferiority of the wall of small vessels;
• increased tone of the sympathetic nerve;
• pulmonary vasculitis.

The role of the mutating bone protein gene, angioproteins, their influence on the synthesis of serotonin, increased blood clotting due to blocking of anticoagulant factors has been established.

A special role is given to infection with the herpes virus of the eighth type, which causes metabolic changes that lead to the destruction of the walls of the arteries.

The result is hypertrophy, then expansion of the cavity, loss of right ventricular tone and development of insufficiency.

Other Causes and Factors of Hypertension

There are many causes and lesions that can cause hypertension in the pulmonary circulation. Some of them deserve special mention.

Among acute diseases:

• respiratory distress syndrome in adults and newborns (toxic or autoimmune damage to the membranes of the respiratory lobules of the lung tissue, causing a lack of a surfactant substance on its surface);
• severe diffuse inflammation (pneumonitis) associated with the development of a massive allergic reaction to inhaled odors of paint, perfume, flowers.

In this case, pulmonary hypertension can be caused by food, drugs and folk remedies.

Pulmonary hypertension in newborns can be caused by:

• the continued circulation of the fetus;
• meconium aspiration;
• diaphragmatic hernia;
• general hypoxia.

In children, hypertension is promoted by enlarged palatine tonsils.

Classification by the nature of the flow

It is convenient for clinicians to divide hypertension in the pulmonary vessels according to the timing of development into acute and chronic forms. Such a classification helps to "combine" the most common causes and clinical course.

Acute hypertension occurs due to:

• thromboembolism of the pulmonary artery;
• severe asthmatic status;
• respiratory distress syndrome;
• sudden left ventricular failure (due to myocardial infarction, hypertensive crisis).

To the chronic course of pulmonary hypertension lead:

• increased pulmonary blood flow;
• increase in resistance in small vessels;
• increased pressure in the left atrium.

A similar development mechanism is typical for:

• defects of the interventricular and interatrial septum;
• open ductus arteriosus;
• mitral valve defect;
• proliferation of myxoma or thrombus in the left atrium;
• gradual decompensation of chronic left ventricular failure, for example, in ischemic disease or cardiomyopathies.

Diseases leading to chronic pulmonary hypertension:

• hypoxic nature - all obstructive diseases of the bronchi and lungs, prolonged oxygen deficiency at altitude, hypoventilation syndrome associated with chest injuries, apparatus breathing;
• mechanical (obstructive) origin associated with narrowing of the arteries - a reaction to drugs, all variants of primary pulmonary hypertension, recurrent thromboembolism, connective tissue diseases, vasculitis.

Clinical picture

Symptoms of pulmonary hypertension appear when the pressure in the pulmonary artery is increased by 2 times or more. Patients with hypertension in the pulmonary circle notice:

• shortness of breath, aggravated by physical exertion (may develop paroxysmal);
• general weakness;
• rarely loss of consciousness (in contrast to neurological causes without seizures and involuntary urination);
• paroxysmal retrosternal pain, similar to angina pectoris, but accompanied by an increase in shortness of breath (scientists explain them by a reflex connection between the pulmonary and coronary vessels);
• the admixture of blood in the sputum when coughing is characteristic of a significantly increased pressure (associated with the release of red blood cells into the interstitial space);
• hoarseness of voice is determined in 8% of patients (caused by mechanical compression of the left recurrent nerve by the dilated pulmonary artery).

The development of decompensation as a result of pulmonary heart failure is accompanied by pain in the right hypochondrium (liver distension), edema in the feet and legs.

When examining a patient, the doctor pays attention to the following:

• a blue tint of the lips, fingers, ears, which intensifies as shortness of breath worsens;
• the symptom of "drum" fingers is detected only with prolonged inflammatory diseases, defects;
• the pulse is weak, arrhythmias are rare;
• arterial pressure is normal, with a tendency to decrease;
• palpation in the epigastric zone allows you to determine the increased shocks of the hypertrophied right ventricle;
• an accentuating second tone on the pulmonary artery is auscultated, a diastolic murmur is possible.

The relationship of pulmonary hypertension with permanent causes and certain diseases allows us to distinguish variants in the clinical course.

Portopulmonary hypertension

Pulmonary hypertension leads to a simultaneous increase in pressure in the portal vein. The patient may or may not have cirrhosis of the liver. It accompanies chronic liver disease in 3–12% of cases. Symptoms do not differ from those listed. More pronounced swelling and heaviness in the hypochondrium on the right.

Pulmonary hypertension with mitral stenosis and atherosclerosis

The disease is characterized by the severity of the course. Mitral stenosis contributes to the occurrence of atherosclerotic lesions of the pulmonary artery in 40% of patients due to increased pressure on the vessel wall. Functional and organic mechanisms of hypertension are combined.

The narrowed left atrioventricular passage in the heart is the "first barrier" to blood flow. In the presence of narrowing or blockage of small vessels, a "second barrier" is formed. This explains the ineffectiveness of the operation to eliminate stenosis in the treatment of heart disease.

By catheterization of the chambers of the heart, high pressure is detected inside the pulmonary artery (150 mm Hg and above).

Vascular changes progress and become irreversible. Atherosclerotic plaques do not grow to large sizes, but they are enough to narrow small branches.

Pulmonary heart

The term "cor pulmonale" includes a symptom complex caused by damage to lung tissue (pulmonary form) or pulmonary artery (vascular form).

There are flow options:

1. acute - typical for pulmonary embolization;
2. subacute - develops with bronchial asthma, lung carcinomatosis;
3. chronic - caused by emphysema, a functional spasm of the arteries, turning into an organic narrowing of the channel, characteristic of chronic bronchitis, pulmonary tuberculosis, bronchiectasis, frequent pneumonia.

The increase in resistance in the vessels gives a pronounced load on the right heart. The general lack of oxygen also affects the myocardium. The thickness of the right ventricle increases with the transition to dystrophy and dilation (persistent expansion of the cavity). Clinical signs of pulmonary hypertension gradually increase.

Hypertensive crises in the vessels of the "small circle"

The crisis course often accompanies pulmonary hypertension associated with heart defects. A sharp deterioration in the condition due to a sudden increase in pressure in the pulmonary vessels is possible once a month or more.

Patients note:

• increased shortness of breath in the evening;
• feeling of external compression of the chest;
• severe cough, sometimes with hemoptysis;
• pain in the interscapular region with irradiation to the anterior sections and sternum;
• cardiopalmus.

On examination, the following are revealed:

• excited state of the patient;
• inability to lie in bed due to shortness of breath;
• severe cyanosis;
• weak frequent pulse;
• visible pulsation in the area of ​​the pulmonary artery;
• swollen and pulsating neck veins;
• excretion of copious amounts of light urine;
• possible involuntary defecation.

Diagnostics

Diagnosis of hypertension in the pulmonary circulation is based on the identification of its signs. These include:

• hypertrophy of the right parts of the heart;
• determination of increased pressure in the pulmonary artery according to the results of measurements using catheterization.

Russian scientists F. Uglov and A. Popov proposed to distinguish between 4 elevated levels of hypertension in the pulmonary artery:

• I degree (mild) - from 25 to 40 mm Hg. Art.;
• II degree (moderate) - from 42 to 65;
• III - from 76 to 110;
• IV - above 110.

Examination methods used in the diagnosis of hypertrophy of the right chambers of the heart:

1. Radiography - indicates the expansion of the right borders of the cardiac shadow, an increase in the arch of the pulmonary artery, reveals its aneurysm.
2. Ultrasound methods (ultrasound) - allow you to accurately determine the size of the chambers of the heart, the thickness of the walls. A variety of ultrasound - dopplerography - shows a violation of blood flow, flow velocity, the presence of obstacles.
3. Electrocardiography - reveals early signs of hypertrophy of the right ventricle and atrium by a characteristic deviation to the right of the electrical axis, an enlarged atrial "P" wave.
4. Spirography - a method of studying the possibility of breathing, determines the degree and type of respiratory failure.
5. In order to detect the causes of pulmonary hypertension, pulmonary tomography is performed with X-ray slices of different depths or in a more modern way - computed tomography.

More complex methods (radionuclide scintigraphy, angiopulmonography). A biopsy to study the state of lung tissue and vascular changes is used only in specialized clinics.

During catheterization of the cavities of the heart, not only pressure is measured, but also measurements of blood oxygen saturation are made. This helps in identifying the causes of secondary hypertension. During the procedure, they resort to the introduction of vasodilators and check the reaction of the arteries, which is necessary in the choice of treatment.

How is the treatment carried out?

Treatment of pulmonary hypertension is aimed at eliminating the underlying pathology that caused the increase in pressure.

At the initial stage, help is provided by anti-asthma drugs, vasodilators. Folk remedies can further strengthen the allergic mood of the body.

If the patient has chronic embolization, then the only remedy is the surgical removal of the thrombus (embolectomy) by excising it from the pulmonary trunk. The operation is carried out in specialized centers, it is necessary to switch to artificial blood circulation. Mortality reaches 10%.

Primary pulmonary hypertension is treated with calcium channel blockers. Their effectiveness leads to a decrease in pressure in the pulmonary arteries in 10–15% of patients, accompanied by a good response from seriously ill patients. This is considered an auspicious sign.

Epoprostenol, an analogue of Prostacyclin, is administered intravenously through a subclavian catheter. Inhalation forms of medicines (Iloprost), Beraprost tablets inside are used. The effect of subcutaneous administration of a drug such as Treprostinil is being studied.

Bosentan is used to block receptors that cause vasospasm.

At the same time, patients need drugs to compensate for heart failure, diuretics, anticoagulants.

A temporary effect is provided by the use of solutions of Eufillin, No-shpy.

Are there folk remedies?

It is impossible to cure pulmonary hypertension with folk remedies. Very carefully apply recommendations on the use of diuretic fees, cough suppressants.

Do not get involved in healing with this pathology. Lost time in diagnosis and initiation of therapy can be lost forever.

Forecast

Without treatment, the average survival time of patients is 2.5 years. Epoprostenol treatment increases the duration to five years in 54% of patients. The prognosis of pulmonary hypertension is unfavorable. Patients die from progressive right ventricular failure or thromboembolism.

Patients with pulmonary hypertension on the background of heart disease and arterial sclerosis live up to 32–35 years of age. The crisis current aggravates the patient's condition, is regarded as an unfavorable prognosis.

The complexity of the pathology requires maximum attention to cases of frequent pneumonia, bronchitis. Prevention of pulmonary hypertension is to prevent the development of pneumosclerosis, emphysema, early detection and surgical treatment of congenital malformations.

Clinic, diagnosis and treatment of rheumatic heart disease

Rheumatic heart disease is an acquired pathology. It is usually classified as a vascular disease in which damage is directed against the tissues of the heart, causing malformations. At the same time, joints and nerve fibers are affected in the body.

The inflammatory response is triggered predominantly by group A hemolytic streptococcus, which causes upper respiratory infections (tonsillitis). Mortality and hemodynamic disturbances occur due to damage to the heart valves. Most often, chronic rheumatic processes cause lesions of the mitral valve, less often - the aortic valve.

Mitral valve lesions

Acute rheumatic fever leads to the development of mitral stenosis 3 years after the onset of the disease. It has been established that every fourth patient with rheumatic heart disease has isolated mitral valve stenosis. In 40% of cases, a combined valve lesion develops. According to statistics, mitral stenosis is more common in women.

Inflammation leads to damage to the edge of the valve leaflets. After the acute period, there is a thickening and fibrosis of the edges of the valves. When tendon cords and muscles are involved in the inflammatory process, they shorten and scar. As a result, fibrosis and calcification lead to a change in the structure of the valve, which becomes rigid and immobile.

Rheumatic damage leads to a decrease in the opening of the valve by half. Higher pressure is now needed to push blood through the narrow opening from the left atrium into the left ventricle. An increase in pressure in the left atrium leads to "jamming" in the pulmonary capillaries. Clinically, this process is manifested by shortness of breath during exercise.

Patients with this pathology do not tolerate increased heart rate very well. Functional insufficiency of the mitral valve can cause fibrillation and pulmonary edema. This development can occur in patients who have never noticed symptoms of the disease.

Clinical features

Rheumatic heart disease with mitral valve disease is manifested in patients with symptoms:

• dyspnea;
• coughing and wheezing during an attack.

At the beginning of the disease, the patient may not pay attention to the symptoms, as they do not have a pronounced manifestation. Only during exercise pathological processes are exacerbated. As the disease progresses, the patient is unable to breathe normally when lying down (orthopnea). Only taking a forced sitting position, the patient breathes. In some cases, severe dyspnea occurs at night with attacks of suffocation, which forces the patient to be in a sitting position.

Patients can withstand moderate exercise. However, they are at risk for pulmonary edema, which can be triggered by:

• pneumonia;
• stress
• pregnancy
• sexual intercourse;
• atrial fibrillation.

With a fit of coughing, hemoptysis may occur. The causes of complications are associated with rupture of bronchial veins. Such profuse bleeding rarely poses a threat to life. During suffocation, blood-stained sputum may appear. With a long course of the disease, against the background of heart failure, pulmonary infarction may occur.

Thromboembolism poses a threat to life. During atrial fibrillation, a detached blood clot can travel through the bloodstream to the kidneys, arteries of the heart, the aortic bifurcation area, or the brain.

Symptoms include:

• chest pain;
• hoarseness of voice (with compression of the laryngeal nerve);
• ascites;
• liver enlargement;
• swelling.

Diagnostics

In order to make a diagnosis, a series of examinations is carried out. The doctor examines the pulse, pressure, interrogates the patient. In the case when pulmonary hypertension has not yet developed, the pulse and pressure are normal. In severe pulmonary hypertension, a change in the heart rhythm occurs. During auscultation, a change in heart sounds is detected, and the severity of stenosis is assessed.

Instrumental examination methods include:

1. Chest X-ray.
2. Echocardiography.
3. Dopplerography.
4. Cardiac catheterization.
5. coronary angiography.

ECG is one of the least sensitive research methods, which allows you to identify signs only in the presence of a severe degree of stenosis. X-ray allows you to assess the degree of enlargement of the left atrium. Echocardiography confirms the diagnosis. The method allows you to evaluate the thickening, degree of calcification and mobility of the valve leaflets.

Dopplerography reveals the severity of stenosis and blood flow velocity. If the patient is scheduled to undergo surgery for valve replacement, cardiac catheterization is included in the examination.

Treatment

Chronic rheumatic heart disease is treated conservatively and promptly. Conservative treatment includes:

• Lifestyle change.
• Prevention of recurrence of rheumatic fever.
• Antibiotic therapy for endocarditis (if any).
• Appointment of anticoagulants (Warfarin).
• Diuretics (Furosemide, Lasix, etc.).
• Nitrates (when there is chronic valve insufficiency).
• Beta blockers.

The choice of surgery depends on the severity of the patient's condition. To alleviate the condition, carry out:

• closed or open mitral commissurotomy (separation of the valve leaflets, cleaning them from calcifications and blood clots during the operation);
• mitral valve replacement;
• percutaneous balloon valvuloplasty.

Balloon plasty is performed on patients whose valve leaflets are sufficiently flexible and mobile. The catheter is inserted through the femoral vein into the atrial septum. A balloon is installed at the site of the stenosis of the orifice and inflated. Thanks to this procedure, stenosis is reduced. The operation allows you to delay valve replacement. The risk of balloon plastic surgery is minimal, which allows the operation to be performed by women who are expecting a baby.

If the patient has a severe degree of calcification, pronounced changes in the valve, valve replacement surgery is indicated. It should be borne in mind that rheumatic processes in the heart will sooner or later lead to serious consequences. Medications only provide temporary relief. After valve replacement, treatment with anticoagulants (Warfarin) under the control of blood clotting is important. With inadequate therapy after prosthetics, there is a risk of thromboembolism.

Doctors cannot predict the exact time of development of stenosis. With successful prevention of rheumatic fevers and commissurotomy, patients can live for a long time without signs of valve stenosis.

Rheumatic aortic valve disease

Rarely, rheumatic heart disease can lead to aortic stenosis. Rarely, such a pathology is isolated. In most cases, a combined lesion of the valves is detected. Damage to the leaflets leads to fibrosis, rigidity, and severe stenosis.

With attacks of rheumatism, valvulitis (inflammation of the valves) develops. This leads to gluing of the edges of the valve leaflets, scarring, thickening and shortening of the leaflets. As a result, the normal tricuspid valve becomes confluent, with a small orifice.

Patients adapt to pathological changes due to chronic processes. Myocardial hypertrophy maintains cardiac output for a long time without the onset of symptoms and valve dilatation. The disease is characterized by a long asymptomatic period. The patient may complain of angina attacks after exertion.

Rheumatic valve inflammation can lead to leaflet sagging. As a result of prolapse, blood from the aorta is thrown into the left ventricle. The patient develops heart failure. Complete exhaustion of the heart occurs 15 years after the onset of the disease.

The development of pathology leads to shortness of breath, dizziness, suffocation in the supine position (orthopnea). During the examination, the doctor reveals a pulse of small filling, a violation of heart sounds, a rough systolic murmur of ejection into the aorta. Additionally, the doctor prescribes echocardiography.

Treatment includes:

• prevention of infective endocarditis;
• prevention of rheumatic attacks;
• lifestyle changes;
• correction of physical activity.

For the relief of angina attacks, patients are prescribed long-acting nitrates. Treatment includes the appointment of cardiac glycosides and diuretics. Progression of the disease worsens the prognosis, so valve replacement is indicated in patients with advanced valvular stenosis, since drug treatment does not improve the condition.

Prevention

Chronic rheumatic pathology is prevented by timely treatment of laryngitis, pharyngitis caused by hemolytic streptococcus A. Diseases are treated with penicillin antibiotics or erythromycin in case of allergy to penicillins.

Secondary prevention is to prevent rheumatic attacks and fever. Patients are prescribed antibiotics on an individual basis. Patients with signs of carditis continue to receive a course of antibiotics for ten years after a rheumatic attack. It is worth noting that the neglect of primary prevention leads to the risk of developing defects after rheumatism. Conservative treatment of defects helps to slow down the progression of pathology and increases the survival of patients.

Signs, grades and treatment of pulmonary hypertension

Pulmonary hypertension is a pathology in which a persistent increase in blood pressure is observed in the vascular bed of an artery. This disease is considered progressive, and eventually leads to the death of a person. Symptoms of pulmonary hypertension manifest themselves depending on the severity of the disease. It is very important to identify it in time and start timely treatment.

• The reasons
• Classification
• Primary pulmonary hypertension
• Secondary hypertension
• Symptoms
• Diagnostics
• Treatment
• Effects
• Prevention

This disease is sometimes found in children. In pulmonary hypertension in newborns, there is no pulmonary circulation to maintain or reduce the already reduced pulmonary vascular resistance at birth. Usually this condition is observed in post-term or premature babies.

The reasons

There are a lot of reasons and risk factors leading to the disease. The main ailments against which the syndrome develops are lung diseases. Most often they are bronchopulmonary diseases, in which the structure of the lung tissue is disturbed and alveolar hypoxia occurs. In addition, the disease can develop against the background of other diseases of the pulmonary system:

• Bronchiectasis. The main symptom of this disease is considered to be the formation of cavities in the lower part of the lungs and suppuration.
• Obstructive chronic bronchitis. In this case, the lung tissue gradually changes, and the airways are closed.
• Fibrosis of lung tissue. This condition is characterized by a change in lung tissue when connective tissue replaces normal cells.

Normal lung and with bronchiectasis

Pulmonary hypertension can also be caused by heart disease. Among them, importance is attached to congenital malformations, such as patent ductus arteriosus, septal defects, and patent foramen ovale. A prerequisite may be diseases in which the functionality of the heart muscle is impaired, contributing to blood stagnation in the pulmonary circulation. Such ailments include cardiomyopathy, coronary artery disease and hypertension.

There are several ways in which pulmonary arterial hypertension develops:

1. Alveolar hypoxia is the main cause of the development of the disease. With it, the alveoli receive an insufficient amount of oxygen. This is observed with uneven pulmonary ventilation, which gradually increases. If a reduced amount of oxygen enters the lung tissue, the vessels of the bloodstream of the pulmonary system narrow.
2. Changes in the structure of lung tissue when connective tissue grows.
3. Increase in the number of erythrocytes. This condition is due to constant hypoxia and tachycardia. Microthrombi appear as a result of vasospasm and increased adhesion of blood cells. They clog the lumen of the pulmonary vessels.

Primary pulmonary hypertension in children develops for unknown reasons. Diagnosis of children showed that the basis of the disease is neurohumoral instability, hereditary predisposition, pathology of the homeostasis system and damage to the vessels of the pulmonary circulation of an autoimmune nature.

Several other factors may contribute to the development of pulmonary hypertension. This may be taking certain drugs that affect the lung tissue: antidepressants, cocaine, amphetamines, anorexigens. Toxins can also influence the development of the disease. These include poisons of biological origin. There are certain demographic and medical factors that can lead to hypertension. These include pregnancy, female sex, hypertension. Cirrhosis of the liver, HIV infection, blood disorders, hyperthyroidism, hereditary disorders, portal hypertension, and other rare diseases can help develop pulmonary hypertension. The impact can be exerted by compression of the pulmonary vessels by a tumor, the consequences of obesity and a deformed chest, as well as climbing in the highlands.

Classification

There are two important forms of the disease, primary and secondary.

Primary pulmonary hypertension

With this form, there is a persistent increase in pressure in the artery, however, not against the background of diseases of the cardiovascular and respiratory systems. There is no thoraco-diaphragmatic pathology. This type of disease is considered hereditary. It is usually transmitted in an autosomal recessive manner. Sometimes development occurs according to the dominant type.

A prerequisite for the development of this form may be a strong aggregation of platelet activity. This leads to the fact that a large number of small vessels located in the circulatory pulmonary system are clogged with blood clots. Because of this, there is a sharp increase in the system of intravascular pressure, which acts on the walls of the artery of the lungs. In order to cope with this and push the right amount of blood further, the muscular part of the arterial wall increases. This is how its compensatory hypertrophy develops.

Primary hypertension may develop against the background of concentric pulmonary fibrosis. This leads to a narrowing of its lumen and an increase in blood flow pressure. As a result of this, and also due to the inability of healthy pulmonary vessels to support the movement of blood with high pressure or the inability of the altered vessels to maintain the movement of blood with normal pressure, a compensatory mechanism develops. It is based on the emergence of bypass routes, which are open arteriovenous shunts. The body tries to lower high blood pressure levels by transferring blood through them. However, the muscle wall of the arterioles is also weak, so the shunts quickly fail. This forms areas that also increase the pressure value. Shunts disrupt proper blood flow, which leads to disruption of blood oxygenation and oxygen supply to tissues. Despite the knowledge of all these factors, primary pulmonary hypertension is still poorly understood.

Secondary hypertension

The course of this type of disease is slightly different. It is caused by many diseases - hypoxic conditions, congenital heart defects, and so on. Cardiac diseases that contribute to the development of a secondary form:

• Diseases that cause insufficiency of LV function. The ailments that are the root cause of hypertension and accompany diseases of this group include: ischemic myocardial damage, aortic valve defects, myocardial and cardiomyopathic damage to the left ventricle.
• Diseases leading to an increase in pressure in the left atrial chamber: developmental anomalies, tumor lesions of the atrium and mitral stenosis.

The development of pulmonary hypertension can be divided into two parts:

• functional mechanisms. Their development is due to the violation of normal and or the formation of new functional pathological features. Drug therapy is aimed precisely at their correction and elimination. The functional links include an increase in blood volume per minute, an increase in blood viscosity, a pathological Savitsky reflex, the influence of frequent bronchopulmonary infections and the effect of biologically active elements on the artery.
• anatomical mechanisms. Their occurrence is preceded by certain anatomical defects in the pulmonary artery or pulmonary circulation system. Medical therapy in this case does not bring practically any benefit. Some defects can be corrected with surgery.

Depending on the severity of hypertension, there are four degrees.

1. Pulmonary hypertension 1 degree. This form proceeds without disturbing the activity of the physical plane. Ordinary exercise does not cause shortness of breath, dizziness, weakness, or chest pain.
2. 2 degree. The disease causes a slight impairment of activity. Habitual exercise is accompanied by shortness of breath, weakness, chest pain and dizziness. At rest, there are no such symptoms.
3. Grade 3 is characterized by a significant impairment of physical activity. Light physical activity causes shortness of breath and other symptoms listed above.
4. 4 degree is accompanied by the mentioned signs at the slightest load and at rest.

There are two more forms of the disease:

1. Chronic thromboembolic hypertension. It develops rapidly as a result of thromboembolism of the trunk and large branches of the artery. Characteristic features are an acute onset, rapid progression, development of pancreatic insufficiency, hypoxia, and a drop in blood pressure.
2. Pulmonary hypertension due to unclear mechanisms. Suspected causes include sarcoidosis, tumors, and fibrosing mediastinitis.

Depending on the pressure, three more three types of disease are distinguished:

1. Light form, when the pressure is from 25 to 36 mm Hg;
2. Moderate pulmonary hypertension, pressure from 35 to 45 mm Hg;
3. Severe form with pressure over 45 mm Hg.

Symptoms

The disease can proceed without symptoms in the stage of compensation. In this regard, it is most often detected when the severe form has begun to develop. Initial manifestations are noted when the pressure in the pulmonary artery system is increased two or more times compared to the norm. With the development of the disease, symptoms such as weight loss, shortness of breath, fatigue, hoarseness, cough and palpitations appear. One cannot explain them. At an early stage of the disease, fainting may occur due to acute cerebral hypoxia and heart rhythm disturbances, as well as dizziness.

Since the signs of pulmonary hypertension are not specific, it is difficult to make an accurate diagnosis based on subjective complaints. Therefore, it is very important to conduct a thorough diagnosis and pay attention to all the symptoms that somehow indicate problems with the pulmonary artery or other systems in the body, a failure in which can lead to the development of hypertension.

Diagnostics

Since a disease of a secondary nature is a complication of other diseases, it is important to identify the underlying disease during diagnosis. This is possible thanks to the following measures:

• Examination of the medical history. This includes gathering information about when the dyspnea, chest pain, and other symptoms began, what the patient attributes these conditions to, and how they were treated.
• Lifestyle analysis. This is information about the patient's bad habits, similar diseases in relatives, working and living conditions, the presence of congenital pathological conditions and previous surgeries.
• Visual examination of the patient. The doctor should pay attention to the presence of such external signs as blue skin, changes in the shape of the fingers, enlarged liver, swelling of the lower extremities, pulsation of the veins of the neck. The lungs and heart are also auscultated with a phonendoscope.
• ECG. Allows you to see signs of enlargement of the right heart.
• An x-ray of the chest organs helps to detect an increase in the size of the heart.
• Ultrasound of the heart. Helps to estimate the size of the heart and indirectly determine the pressure in the arteries of the lungs.
• artery catheterization. Using this method, you can determine the pressure in it.

Such data will help determine whether primary pulmonary hypertension in humans or secondary, treatment tactics and prognosis. In order to establish the class and type of disease, as well as assess exercise tolerance, spirometry, chest CT scan, diffuse lung capacity assessment, abdominal ultrasound, blood test, and so on are performed.

Treatment

Treatment of pulmonary hypertension is based on several methods.

1. Non-drug treatment. It includes drinking no more than 1.5 liters of fluid per day, as well as reducing the amount of table salt consumed. Oxygen therapy is effective, as it helps to eliminate acidosis and restore the functions of the central nervous system. It is important for patients to avoid situations that cause shortness of breath and other symptoms, so avoiding physical exertion is a good recommendation.
2. Drug therapy: diuretics, calcium antagonists, nitrates, ACE inhibitors, antiplatelet agents, antibiotics, prostaglandins, and so on.
3. Surgical treatment of pulmonary hypertension: thromboendarectomy, atrial septostomy.
4. folk methods. Alternative treatment can be used only on the recommendation of a doctor.

Effects

A frequent complication of the disease is heart failure of the pancreas. It is accompanied by a violation of the heart rhythm, which is manifested by atrial fibrillation. For severe stages of hypertension, the development of thrombosis of arterioles of the lungs is characteristic. In addition, hypertensive crises can develop in the bloodstream of the vessels, which are manifested by bouts of pulmonary edema. The most dangerous complication of hypertension is death, which usually occurs due to the development of arterial thromboembolism or cardiopulmonary failure.

In the severe stage of the disease, thrombosis of arterioles of the lungs is possible.

In order to avoid such complications, it is necessary to start treatment of the disease as early as possible. Therefore, at the first signs, you need to rush to the doctor and undergo a full examination. In the process of treatment, you must follow the recommendations of the doctor.

Prevention

To prevent this terrible disease, you can use some measures that are aimed at improving the quality of life. It is necessary to give up bad habits and avoid psycho-emotional stress. Any diseases should be treated promptly, especially those that can lead to the development of pulmonary hypertension.

Taking care of yourself in moderation, you can avoid many diseases that reduce life expectancy. Let's remember that our health often depends on ourselves!

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LECTURE ON INTERNAL DISEASES.

TOPIC: PULMONARY HEART.

Relevance of the topic: Diseases of the bronchopulmonary system, chest are of great importance in the defeat of the heart. The defeat of the cardiovascular system in diseases of the bronchopulmonary apparatus, most authors refer to the term cor pulmonale.

Chronic cor pulmonale develops in approximately 3% of patients suffering from chronic lung diseases, and in the overall structure of mortality from congestive heart failure, chronic cor pulmonale accounts for 30% of cases.

Cor pulmonale is hypertrophy and dilatation or only dilatation of the right ventricle resulting from hypertension of the pulmonary circulation, developed as a result of diseases of the bronchi and lungs, chest deformity, or primary damage to the pulmonary arteries. (WHO 1961).

Hypertrophy of the right ventricle and its dilatation with changes as a result of a primary lesion of the heart, or congenital malformations do not belong to the concept of cor pulmonale.

Recently, clinicians have noticed that hypertrophy and dilatation of the right ventricle are already late manifestations of cor pulmonale, when it is no longer possible to rationally treat such patients, so a new definition of cor pulmonale was proposed:

Cor pulmonale is a complex of hemodynamic disorders in the pulmonary circulation, which develops as a result of diseases of the bronchopulmonary apparatus, chest deformities, and primary lesions of the pulmonary arteries, which at the final stage manifested by right ventricular hypertrophy and progressive circulatory failure.

ETIOLOGY OF THE PULMONARY HEART.

Cor pulmonale is a consequence of diseases of three groups:

Diseases of the bronchi and lungs, primarily affecting the passage of air and alveoli. This group includes approximately 69 diseases. They are the cause of cor pulmonale in 80% of cases.

chronic obstructive bronchitis

pneumosclerosis of any etiology

pneumoconiosis

tuberculosis, not by itself, as post-tuberculosis outcomes

SLE, Boeck's sarcoidosis, fibrosing alveolitis (endo- and exogenous)

Diseases that primarily affect the chest, diaphragm with limitation of their mobility:

kyphoscoliosis

multiple rib injuries

pickwick syndrome in obesity

ankylosing spondylitis

pleural suppuration after pleurisy

Diseases primarily affecting the pulmonary vessels

primary arterial hypertension (Ayerza`s disease)

recurrent pulmonary embolism (PE)

compression of the pulmonary artery from the veins (aneurysm, tumors, etc.).

Diseases of the second and third groups are the cause of the development of cor pulmonale in 20% of cases. That is why they say that, depending on the etiological factor, there are three forms of cor pulmonale:

bronchopulmonary

thoracophrenic

vascular

Norms of values ​​characterizing the hemodynamics of the pulmonary circulation.

The systolic pressure in the pulmonary artery is about five times less than the systolic pressure in the systemic circulation.

Pulmonary hypertension is said to be if the systolic pressure in the pulmonary artery at rest is greater than 30 mm Hg, the diastolic pressure is greater than 15, and the mean pressure is greater than 22 mm Hg.

PATHOGENESIS.

The basis of the pathogenesis of cor pulmonale is pulmonary hypertension. Since cor pulmonale most often develops in bronchopulmonary diseases, we will start with this. All diseases, and in particular chronic obstructive bronchitis, will primarily lead to respiratory (lung) failure. Pulmonary insufficiency is a condition in which normal blood gases are disturbed.

This is a state of the body in which either the normal gas composition of the blood is not maintained, or the latter is achieved by abnormal operation of the external respiration apparatus, leading to a decrease in the functional capabilities of the body.

There are 3 stages of lung failure.

Arterial hypoxemia underlies the pathogenesis of chronic heart disease, especially in chronic obstructive bronchitis.

All these diseases lead to respiratory failure. Arterial hypoxemia will lead to alveolar hypoxia at the same time due to the development of pneumofibrosis, emphysema of the lungs, intra-alveolar pressure increases. Under conditions of arterial hypoxemia, the non-respiratory function of the lungs is disturbed - biologically active substances begin to be produced, which have not only a bronchospastic, but also a vasospastic effect. At the same time, when this occurs, a violation of the vascular architectonics of the lungs occurs - some of the vessels die, some expand, etc. Arterial hypoxemia leads to tissue hypoxia.

The second stage of pathogenesis: arterial hypoxemia will lead to a restructuring of central hemodynamics - in particular, an increase in the amount of circulating blood, polycythemia, polyglobulia, and an increase in blood viscosity. Alveolar hypoxia will lead to hypoxemic vasoconstriction in a reflex way, with the help of a reflex called the Euler-Liestrand reflex. Alveolar hypoxia led to hypoxemic vasoconstriction, an increase in intra-arterial pressure, which leads to an increase in hydrostatic pressure in the capillaries. Violation of the non-respiratory function of the lungs leads to the release of serotonin, histamine, prostaglandins, catecholamines, but most importantly, under conditions of tissue and alveolar hypoxia, the interstitium begins to produce more angiotensin-converting enzyme. The lungs are the main organ where this enzyme is formed. It converts angiotensin 1 into angiotensin 2. Hypoxemic vasoconstriction, the release of biologically active substances under conditions of restructuring of central hemodynamics will lead not only to an increase in pressure in the pulmonary artery, but to a persistent increase in it (above 30 mm Hg), that is, to the development of pulmonary hypertension. If the processes continue further, if the underlying disease is not treated, then naturally part of the vessels in the pulmonary artery system dies due to pneumosclerosis, and the pressure steadily rises in the pulmonary artery. At the same time, persistent secondary pulmonary hypertension will open shunts between the pulmonary artery and bronchial arteries, and non-oxygenated blood enters the systemic circulation through the bronchial veins and also contributes to an increase in the work of the right ventricle.

So, the third stage is persistent pulmonary hypertension, the development of venous shunts, which enhance the work of the right ventricle. The right ventricle is not powerful in itself, and hypertrophy with elements of dilation rapidly develops in it.

The fourth stage is hypertrophy or dilatation of the right ventricle. Myocardial dystrophy of the right ventricle will contribute as well as tissue hypoxia.

So, arterial hypoxemia led to secondary pulmonary hypertension and right ventricular hypertrophy, to its dilatation and development of predominantly right ventricular circulatory failure.

The pathogenesis of the development of cor pulmonale in thoracophrenic form: in this form, hypoventilation of the lungs due to kyphoscoliosis, pleural suppuration, spinal deformities, or obesity is the leading one, in which the diaphragm rises high. Hypoventilation of the lungs will primarily lead to a restrictive type of respiratory failure, in contrast to the obstructive type that is caused by chronic cor pulmonale. And then the mechanism is the same - a restrictive type of respiratory failure will lead to arterial hypoxemia, alveolar hypoxemia, etc.

The pathogenesis of the development of cor pulmonale in the vascular form lies in the fact that with thrombosis of the main branches of the pulmonary arteries, the blood supply to the lung tissue decreases sharply, since along with thrombosis of the main branches, friendly reflex narrowing of the small branches occurs. In addition, in the vascular form, in particular in primary pulmonary hypertension, the development of cor pulmonale is facilitated by pronounced humoral changes, that is, a noticeable increase in the amount of serotonin, prostaglandins, catecholamines, the release of convertase, angiotensin-converting enzyme.

The pathogenesis of cor pulmonale is a multi-stage, multi-stage, in some cases not completely clear.

CLASSIFICATION OF THE PULMONARY HEART.

There is no single classification of cor pulmonale, but the first international classification is mainly etiological (WHO, 1960):

bronchopulmonary heart

thoracophrenic

vascular

A domestic classification of the cor pulmonale is proposed, which provides for the division of the cor pulmonale according to the rate of development:

• subacute

  chronic

Acute cor pulmonale develops within a few hours, minutes, maximum days. Subacute cor pulmonale develops over several weeks or months. Chronic cor pulmonale develops over several years (5-20 years).

This classification provides for compensation, but acute cor pulmonale is always decompensated, that is, it requires immediate assistance. Subacute can be compensated and decompensated mainly according to the right ventricular type. Chronic cor pulmonale can be compensated, subcompensated, decompensated.

By genesis, acute cor pulmonale develops in vascular and bronchopulmonary forms. Subacute and chronic cor pulmonale can be vascular, bronchopulmonary, thoracophrenic.

Acute cor pulmonale develops primarily:

with embolism - not only with thromboembolism, but also with gas, tumor, fat, etc.,

with pneumothorax (especially valvular),

with an attack of bronchial asthma (especially with asthmatic status - a qualitatively new condition in patients with bronchial asthma, with complete blockade of beta2-adrenergic receptors, and with acute cor pulmonale);

with acute confluent pneumonia

right-sided total pleurisy

A practical example of subacute cor pulmonale is recurrent thromboembolism of small branches of the pulmonary arteries during an attack of bronchial asthma. A classic example is cancerous lymphangitis, especially in chorionepithelioma, in peripheral lung cancer. The thoracodiphragmatic form develops with hypoventilation of central or peripheral origin - myasthenia gravis, botulism, poliomyelitis, etc.

To distinguish at what stage the cor pulmonale from the stage of respiratory failure passes into the stage of heart failure, another classification was proposed. Cor pulmonale is divided into three stages:

hidden latent insufficiency - there is a violation of the function of external respiration - the VC / CL decreases to 40%, but there are no changes in the gas composition of the blood, that is, this stage characterizes respiratory failure of 1-2 stages.

stage of severe pulmonary insufficiency - the development of hypoxemia, hypercapnia, but without signs of heart failure in the periphery. There is shortness of breath at rest, which cannot be attributed to heart damage.

stage of pulmonary heart failure of varying degrees (edema in the limbs, an increase in the abdomen, etc.).

Chronic cor pulmonale according to the level of pulmonary insufficiency, saturation of arterial blood with oxygen, hypertrophy of the right ventricle and circulatory failure is divided into 4 stages:

the first stage - pulmonary insufficiency of the 1st degree - VC / CL decreases to 20%, the gas composition is not disturbed. Right ventricular hypertrophy is absent on the ECG, but there is hypertrophy on the echocardiogram. There is no circulatory failure at this stage.

pulmonary insufficiency 2 - VC / CL up to 40%, oxygen saturation up to 80%, the first indirect signs of right ventricular hypertrophy appear, circulatory failure +/-, that is, only shortness of breath at rest.

the third stage - pulmonary insufficiency 3 - VC / CL less than 40%, saturation of arterial blood up to 50%, there are signs of right ventricular hypertrophy on the ECG in the form of direct signs. Circulatory failure 2A.

fourth stage - pulmonary insufficiency 3. Blood oxygen saturation less than 50%, right ventricular hypertrophy with dilatation, circulatory failure 2B (dystrophic, refractory).

CLINIC OF ACUTE PULMONARY HEART.

The most common cause of development is PE, an acute increase in intrathoracic pressure due to an attack of bronchial asthma. Arterial precapillary hypertension in acute cor pulmonale, as well as in the vascular form of chronic cor pulmonale, is accompanied by an increase in pulmonary resistance. Next comes the rapid development of dilatation of the right ventricle. Acute right ventricular failure is manifested by severe shortness of breath turning into inspiratory suffocation, rapidly increasing cyanosis, pain behind the sternum of a different nature, shock or collapse, the size of the liver rapidly increases, edema in the legs appears, ascites, epigastric pulsation, tachycardia (120-140), hard breathing , in some places weakened vesicular; wet, various rales are heard, especially in the lower parts of the lungs. Of great importance in the development of acute pulmonary heart are additional research methods, especially ECG: a sharp deviation of the electrical axis to the right (R 3>R 2>R 1, S 1>S 2>S 3), P-pulmonale appears - a pointed P wave, in the second , third standard leads. The blockade of the right leg of the bundle of His is complete or incomplete, ST inversion (usually rise), S in the first lead is deep, Q in the third lead is deep. Negative S wave in leads 2 and 3. These same signs can also occur in acute myocardial infarction of the posterior wall.

Emergency care depends on the cause of acute cor pulmonale. If there was PE, then painkillers, fibrinolytic and anticoagulant drugs (heparin, fibrinolysin), streptodecase, streptokinase are prescribed, up to surgical treatment.

With asthmatic status - large doses of glucocorticoids intravenously, bronchodilators through a bronchoscope, transfer to mechanical ventilation and bronchial lavage. If this is not done, the patient dies.

With valvular pneumothorax - surgical treatment. With confluent pneumonia, along with antibiotic treatment, diuretics and cardiac glycosides are required.

CLINIC OF CHRONIC PULMONARY HEART.

Patients are concerned about shortness of breath, the nature of which depends on the pathological process in the lungs, the type of respiratory failure (obstructive, restrictive, mixed). With obstructive processes, dyspnea of ​​an expiratory nature with an unchanged respiratory rate, with restrictive processes, the duration of exhalation decreases, and the respiratory rate increases. An objective examination, along with signs of the underlying disease, cyanosis appears, most often diffuse, warm due to the preservation of peripheral blood flow, in contrast to patients with heart failure. In some patients, cyanosis is so pronounced that the skin acquires a cast-iron color. Swollen neck veins, swelling of the lower extremities, ascites. The pulse is quickened, the borders of the heart expand to the right, and then to the left, muffled tones due to emphysema, the accent of the second tone over the pulmonary artery. Systolic murmur at the xiphoid process due to dilatation of the right ventricle and relative insufficiency of the right tricuspid valve. In some cases, with severe heart failure, you can listen to a diastolic murmur on the pulmonary artery - Graham-Still murmur, which is associated with relative insufficiency of the pulmonary valve. Above the lungs, percussion, the sound is boxy, breathing is vesicular, hard. In the lower parts of the lungs there are congestive, inaudible moist rales. On palpation of the abdomen - an increase in the liver (one of the reliable, but not early signs of cor pulmonale, since the liver can be displaced due to emphysema). The severity of symptoms depends on the stage.

The first stage: against the background of the underlying disease, shortness of breath increases, cyanosis appears in the form of acrocyanosis, but the right border of the heart is not expanded, the liver is not enlarged, in the lungs the physical data depend on the underlying disease.

The second stage - shortness of breath turns into attacks of suffocation, with difficulty breathing, cyanosis becomes diffuse, from the data of an objective study: a pulsation appears in the epigastric region, muffled tones, the accent of the second tone over the pulmonary artery is not constant. The liver is not enlarged, may be omitted.

The third stage - signs of right ventricular failure join - an increase in the right border of cardiac dullness, an increase in the size of the liver. Persistent swelling in the lower extremities.

The fourth stage is shortness of breath at rest, a forced position, often accompanied by respiratory rhythm disorders such as Cheyne-Stokes and Biot. The edema is constant, not amenable to treatment, the pulse is weak and frequent, the heart of a bull, the tones are deaf, the systolic murmur at the xiphoid process. Lots of moist rales in the lungs. The liver is of considerable size, does not shrink under the action of glycosides and diuretics, as fibrosis develops. Patients are constantly dozing.

Diagnosis of thoracodiaphragmatic heart is often difficult, one must always remember about the possibility of its development in kyphoscoliosis, Bechterew's disease, etc. The most important sign is the early onset of cyanosis, and a noticeable increase in shortness of breath without asthma attacks. Pickwick's syndrome is characterized by a triad of symptoms - obesity, drowsiness, severe cyanosis. This syndrome was first described by Dickens in The Posthumous Papers of the Pickwick Club. Associated with traumatic brain injury, obesity is accompanied by thirst, bulimia, arterial hypertension. Diabetes mellitus often develops.

Chronic cor pulmonale in primary pulmonary hypertension is called Ayerz's disease (described in 1901). A polyetiological disease of unknown origin, predominantly affects women from 20 to 40 years old. Pathological studies have established that in primary pulmonary hypertension there is a thickening of the intima of the precapillary arteries, that is, thickening of the media is noted in the muscle-type arteries, and fibrinoid necrosis develops, followed by sclerosis and the rapid development of pulmonary hypertension. Symptoms are varied, usually complaints of weakness, fatigue, pain in the heart or joints, 1/3 of patients may experience fainting, dizziness, Raynaud's syndrome. And in the future, shortness of breath increases, which is the sign that indicates that primary pulmonary hypertension is moving into a stable final stage. Cyanosis is rapidly growing, which is expressed to the degree of a cast-iron hue, becomes permanent, edema rapidly increases. The diagnosis of primary pulmonary hypertension is established by exclusion. Most often this diagnosis is pathological. In these patients, the entire clinic progresses without a background in the form of obstructive or restrictive respiratory failure. With echocardiography, the pressure in the pulmonary artery reaches its maximum values. Treatment is ineffective, death occurs from thromboembolism.

Additional research methods for cor pulmonale: in a chronic process in the lungs - leukocytosis, an increase in the number of erythrocytes (polycythemia associated with increased erythropoiesis due to arterial hypoxemia). X-ray data: appear very late. One of the early symptoms is a bulging of the pulmonary artery on x-ray. The pulmonary artery bulges, often flattening the waist of the heart, and this heart is mistaken by many physicians for a mitral configuration of the heart.

ECG: indirect and direct signs of right ventricular hypertrophy appear:

deviation of the electrical axis of the heart to the right - R 3 > R 2 > R 1, S 1 > S 2 > S 3, the angle is greater than 120 degrees. The most basic indirect sign is an increase in the interval of the R wave in V 1 greater than 7 mm.

direct signs - blockade of the right leg of the bundle of His, the amplitude of the R wave in V 1 more than 10 mm with complete blockade of the right leg of the bundle of His. The appearance of a negative T wave with a displacement of the wave below the isoline in the third, second standard lead, V1-V3.

Of great importance is spirography, which reveals the type and degree of respiratory failure. On ECG, signs of right ventricular hypertrophy appear very late, and if only deviations of the electrical axis to the right appear, then they already speak of pronounced hypertrophy. The most basic diagnosis is dopplercardiography, echocardiography - an increase in the right heart, increased pressure in the pulmonary artery.

PRINCIPLES OF TREATMENT OF PULMONARY HEART.

Treatment of cor pulmonale is to treat the underlying disease. With exacerbation of obstructive diseases, bronchodilators, expectorants are prescribed. With Pickwick's syndrome - treatment of obesity, etc.

Reduce pressure in the pulmonary artery with calcium antagonists (nifedipine, verapamil), peripheral vasodilators that reduce preload (nitrates, corvaton, sodium nitroprusside). Of greatest importance is sodium nitroprusside in combination with angiotensin-converting enzyme inhibitors. Nitroprusside 50-100 mg intravenously, capoten 25 mg 2-3 times a day, or enalapril (second generation, 10 mg per day). Treatment with prostaglandin E, antiserotonin drugs, etc. are also used. But all these drugs are effective only at the very beginning of the disease.

Treatment of heart failure: diuretics, glycosides, oxygen therapy.

Anticoagulant, antiaggregant therapy - heparin, trental, etc. Due to tissue hypoxia, myocardial dystrophy develops rapidly, therefore, cardioprotectors are prescribed (potassium orotate, panangin, riboxin). Cardiac glycosides are prescribed very carefully.

PREVENTION.

Primary - prevention of chronic bronchitis. Secondary - treatment of chronic bronchitis.

RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical Protocols of the Ministry of Health of the Republic of Kazakhstan - 2014

Primary pulmonary hypertension (I27.0)

Cardiology

general information

Short description

Approved

At the Expert Commission on Health Development

Ministry of Health of the Republic of Kazakhstan

Pulmonary hypertension- hemodynamic and pathophysiological state, defined by an increase in mean pulmonary arterial pressure (MPAP) > 25 mm Hg. at rest, as assessed by right heart catheterization. .

I. INTRODUCTION:

Name: Pulmonary hypertension

Protocol code:

MBK-10 code:

I27.0 Primary pulmonary hypertension

Abbreviations used in the protocol:

ALAH associated pulmonary arterial hypertension
ANA antinuclear antibodies
AE endothelin receptor antagonists
HIV human immunodeficiency virus
WHO World Health Organization
congenital heart defects

PLA pulmonary artery pressure

DZLK wedge pressure in pulmonary capillaries
ASD
VSD ventricular septal defect
RAP right atrial pressure
D-EchoCG doppler echocardiography
CTD connective tissue disease
IPAH idiopathic pulmonary arterial hypertension
CT computed tomography

CAG coronary angiography
PAH pulmonary arterial hypertension
LA pulmonary artery

PH pulmonary hypertension
DZLK wedge pressure in pulmonary capillaries

PVR pulmonary vascular resistance
SPPA mean pulmonary artery pressure

RV systolic pressure in the right ventricle
PDE-5 phosphodiesterase type 5 inhibitors
COPD chronic obstructive pulmonary disease
CTEPH chronic thromboembolic pulmonary hypertension
PE-EchoCG transesophageal echocardiography
HR heart rate
echocardiography echocardiography

BNP brain natriuretic peptide

ESC European Society of Cardiology
NYHA New York Heart Association
INR international normalized ratio

TAPSE systolic range of motion of the tricuspid annulus

V/Q ventilation-perfusion index

Protocol development date: year 2014

Protocol Users: cardiologists (adults, children, including interventional ones), cardiac surgeons, general practitioners, pediatricians, therapists, rheumatologists, oncologists (chemotherapy, mammology), phthisiatricians, pulmonologists, infectious disease specialists.

This protocol uses the following classes of recommendations and levels of evidence (Appendix 1).

Classification

Classification :

Pathophysiological classification:

1. Precapillary: mean pressure in LA ≥25 mm Hg, DZLK ≤15 mm Hg, CO normal/low.

Clinical groups:

- PH lung diseases;

− CTELPH;

- PH with a multifactorial etiological factor.

2. Post-capillary: SDLA ≥25 mm Hg, DZLK > 15 mm Hg, SD normal/reduced.

Clinical groups:

- PH in diseases of the left heart.

Clinical classification:

1. Pulmonary arterial hypertension:

1.2 Hereditary:

1.2.2 ALK1, ENG, SMAD9, CAV1, KCNK3

1.2.3 Unknown

1.3 Induced by drugs and toxins

1.4 Associated with:

1.4.1 Connective tissue diseases

1.4.2 HIV infection

1.4.3 Portal hypertension

1.4.5 Schistosomiasis

1.5 Persistent pulmonary arterial hypertension of the newborn

2. Pulmonary hypertension due to diseases of the left heart:

2.1 Systolic dysfunction

2.2 Diastolic dysfunction

2.3 Valvular heart disease

2.4 Congenital/acquired obstruction of the outflow tract of the left ventricle.

3. Pulmonary hypertension due to lung disease and/or hypoxemia:

3.2 Interstitial lung diseases

3.3 Other lung diseases with a mixed restrictive and obstructive component

3.4 Breathing disorders during sleep

3.5 Alveolar hypoventilation

3.6 Chronic high altitude exposure

3.7 Lung malformations

4. HTELG

5. Pulmonary hypertension with unclear and / or multifactorial mechanisms:

5.1 Hematological disorders: chronic hemolytic anemia. myeloproliferative disorders, splenectomy.

5.2 Systemic diseases: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis

5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, metabolic disorders associated with thyroid diseases

5.4 Other: tumor obstruction, fibrosing mediastinitis, chronic renal failure, segmental pulmonary hypertension.

Table 1. Modified functional classification of PH (NYHA). Agreed by WHO:

Diagnostics

II. METHODS, APPROACHES AND PROCEDURES FOR DIAGNOSIS AND TREATMENT

List of basic and additional diagnostic measures

The rationale for the use of basic and additional diagnostic methods is presented in the tables (Appendices 2.3)

Basic (mandatory) diagnostic examinations performed at the outpatient level for dynamic control:

(1 time per semester)

2. ECG (1 time per quarter)

3. Echocardiography (every 3-6 months)

4. X-ray of the chest in 2 projections (direct, left lateral) (1 time per year and according to clinical indications)

Additional diagnostic examinations performed at the outpatient level for dynamic control:

1. MRI of the chest and mediastinum

2. Duplex scanning of peripheral vessels of the extremities

3. Blood test for pro - BNP level (every 3-6 months)

The minimum list of examinations that must be carried out when referring to planned hospitalization:

1. Complete blood count 6 parameters

2. Precipitation microreaction with cardiolipin antigen

3. ELISA for HIV, hepatitis B, C.

6. X-ray of the chest organs in 2 projections (direct, left lateral).

Basic (mandatory) diagnostic examinations carried out at the hospital level(in case of emergency hospitalization, diagnostic examinations are performed that were not performed at the outpatient level):

1. Complete blood count 6 parameters

2. Blood test for pro level - BNP

5. X-ray of the chest organs direct and lateral projections with contrasting of the esophagus

6. Six Minute Walk Test

7. Catheterization of the right heart with angiopulmonography

8. Spirography

9. CT angiopulmonography

Additional diagnostic examinations carried out at the hospital level(in case of emergency hospitalization, diagnostic examinations are carried out that were not performed at the outpatient level:

1. Urinalysis

2. Blood electrolytes

3. Determination of CRP in blood serum

4. Total protein and fractions

5. Blood urea

6. Blood creatinine and glomerular filtration rate

7. Determination of AST, ALT, bilirubin (total, direct)

8. Determination of the international normalized ratio of the prothrombin complex in plasma

9. Coagulogram

10. Blood test for D-dimer

11. Immunogram

12. Tumor markers in the blood

13. PCR for tuberculosis from blood

14. Antinuclear antibodies

15. Rheumatoid factor

16. Thyroid hormones

17. Procalcitonin test

18. Sputum analysis for Mycobacterium tuberculosis by bacterioscopy

19. PE EchoCG

20. Ultrasound of the abdominal organs

21. Ultrasound of the thyroid gland

22. Ventilation - perfusion scintigraphy

Diagnostic measures taken at the stage of emergency care:

2. Pulse oximetry

Diagnostic criteria

Complaints:
- fatigue
- weakness
- anginal chest pain
- syncope

Presence in history:
- deep vein thrombosis
- HIV infection
- liver disease
- diseases of the left side of the heart
- lung disease

hereditary diseases
- intake of drugs and toxins (table 2)

table 2 Risk level of drugs and toxins that can cause PH

Physical examination:
- peripheral cyanosis
- Harsh breathing on lung auscultation
- increased heart sounds along the left parasternal line
- strengthening of the pulmonary component of the II tone
- pansystolic murmur of tricuspid regurgitation
- diastolic murmur of insufficiency of pulmonary valves
- right ventricular III tone
- organic noise of congenital heart defects

Physical tolerance(Table 1)
An objective assessment of exercise tolerance in patients with PH is an important way to establish the severity of the disease and the effectiveness of treatment. For PH, a 6-minute walk test (6MT) is used to assess gas exchange parameters.

Laboratory research
- Determination of the BNP index in order to confirm the diagnosis of heart failure (primarily left ventricular dysfunction), clarify the causes of acute dyspnea, assess the condition of patients with heart failure and control treatment. Normative indicators: BNP 100-400 pg/ml, NT-proBNP 400-2000 pg/ml.

General clinical laboratory examinations are carried out in order to identify the primary cause of the development of PH (Appendices 2.3).

Instrumental Research

echocardiography
Echocardiography is an important study in the diagnosis of PH, since, in addition to an indicative diagnosis, it allows you to fix the primary disorders that caused PH (CHD with bypass, disruption of the left heart, possible cardiac complications).
Criteria for establishing the diagnosis by Doppler echocardiography (Table 3).

Table 3 Doppler echocardiographic diagnosis of PH

Note:

1. Doppler echocardiography stress testing is not recommended for screening for PH (recommendation class III, level of evidence C).

2. signs of PH: dilatation of the right side of the heart, valve and trunk of the pulmonary artery, abnormal movement and function of the interventricular septum, increased wall thickness

of the right ventricle, an increase in the rate of regurgitation on the pulmonary valve, a shortening of the time of acceleration of ejection from the right ventricle into the LA.

3. SRV = 4v2+ DPP

4. DPP - calculated according to the parameters of the inferior vena cava or the size of the expansion of the jugular vein

Right heart catheterization and vasoreactive tests.
Right heart catheterization with tonometry and a vasoreactive test is a mandatory study to establish the diagnosis of PAH.
Carrying out CAG is necessary to diagnose the disease of the left parts of the heart.
The minimum volume of parameters that must be recorded during catheterization of the right heart:
- Pressure in the pulmonary artery (systolic, diastolic and mean);
- Pressure in the right atrium, in the right ventricle;
- Cardiac output;
- Oxygen saturation in the inferior and superior vena cava, pulmonary artery, right heart and in the systemic circulation;
- LSS;
- DZLK;
- Presence/absence of pathological shunts
- Reaction to vasoreactive test. The result of the vasoreactivity test is considered positive if the PSAP falls > 10 mmHg. Art. and/or reaches an absolute value< 40 мм рт. ст. при условии неизменной величины сердечного выброса (больные с положительной острой реакцией).

The use of drugs for conducting a vasoreactive test is carried out in accordance with Table 4

Table 4 Use of drugs for the vasoreactive test

Chest X-ray

Chest x-ray can reliably rule out PH-related moderate to severe lung disease and pulmonary venous hypertension due to pathology of the left heart. However, a normal chest x-ray does not rule out mild postcapillary pulmonary hypertension secondary to left heart disease.

In patients with PH at the time of diagnosis, there are changes on the chest x-ray:

- expansion of the pulmonary artery, which, when contrasting, "loses" peripheral branches.

− enlargement of the right atrium and ventricle

Ventilation-perfusion (V/Q) lung scan is an additional diagnostic method:

With PH, V/Q scanning may be completely normal.

The V/Q ratio will be altered in the presence of small peripheral non-segmental perfusion defects that are normally ventilated.

In CTEPH, perfusion defects are usually located at the lobar and segmental level, which is reflected by segmental perfusion defects in its graphical representation. Since these areas are ventilated normally, perfusion defects do not coincide with ventilation defects.

In patients with parenchymal lung diseases, perfusion defects coincide with ventilation defects.

Indications for consultation of narrow specialists:

− Cardiologist (adult, pediatric, including interventional): exclusion of diseases of the left heart, congenital heart defects, determination of tactics for the treatment of right ventricular failure, the state of the peripheral vascular system, determination of the degree of involvement of the cardiovascular system in the pathological process

− Rheumatologist: for the purpose of differential diagnosis of systemic connective tissue disease

− Pulmonologist: for the purpose of diagnosing a primary lesion of the lungs

− Cardiac surgeon: in order to diagnose the primary disease (CHD, left ventricular outflow obstruction).

- TB doctor: if you have symptoms that are suspicious of TB.

- Oncologist: if you have symptoms that are suspicious of cancer.

- Nephrologist: if symptoms are suspected of kidney disease.

− Infectionist: if present if symptoms suspected of schistosomiasis

− Geneticist: if hereditary PAH is suspected.

Differential Diagnosis

Differential Diagnosis: Table 5

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Treatment

Treatment goals:

1. Control over the course of the underlying disease

2. Prevention of complications

Treatment tactics

Non-drug treatment

Diet - table number 10. Mode - 1.2

Medical treatment

The list of main and additional drugs for the treatment of PAH is presented in Table 6. The probability of using the main drugs is based on the results of the study (vasoreactive test), individual sensitivity.

Table 6. Medical therapy

Indications for specific therapy are presented in Table 7

Table 7. Indications for specific therapy

*Initial combination therapy includes specific and complementary therapies

**Agreed combination therapy, used in the absence of clinical effect, (IIa-B):

Antagonists of endothelin receptors AER + PDE-5 inhibitors of phosphodiesterase 5;

Antagonists of endothelin receptors AE + prostanoids;
-phosphodiesterase 5 inhibitors PDE-5 + prostanoids

Indications for specific therapy with a negative vasoreactive test are presented in Table 8

Table 8 Indications for specific therapy with a negative vasoreactive test

Indications for additional therapy are presented in Table 9

Table 9 Indications for additional therapy

Table 10 Therapy of PH associated with congenital heart disease with left-to-right shunting

Medical treatment provided on an outpatient basis :

List of essential medicines:

− Sildenafil

− Iloprost

− Bosentan

− Amlodipine

− Nifedipine

− Diltiazem

List of additional medicines:

− Furosemide

− Veroshpiron

− Captopril

− Enalapril

− Warfarin

− Digoxin

Treatment at the outpatient level provides for the continuation of permanent therapy selected in a hospital setting. The appointment of drugs is carried out according to the recommendations presented in table 6. Correction of doses and treatment regimens is carried out under the control of the patient's condition and functional indicators.

Medical treatment provided at the inpatient level :

The selection of drug treatment in a hospital is carried out according to the recommendations presented in tables 6-9.

Drug treatment provided at the stage of emergency emergency care with an established diagnosis of PH:

- Iloprost inhalation (the drug is prescribed according to the recommendations presented in Table 6).

− Oxygen therapy under the control of oxygen saturation below 8 kPa (60 mm Hg)

Other types of treatment: not provided.

Surgical intervention provided in a hospital: in the absence of a clinical effect of combination therapy, balloon atrial septostomy (I-C) and / or lung transplantation (I-C) is recommended.

Preventive actions:

Prevention of the development of pulmonary hypertension and its complications by correcting removable etiological factors.

Prevention of progression of PH: conducting adequate medical maintenance therapy.

Further management

The timing and frequency of examination of patients is carried out according to the recommendations presented in Table 11.

Table 11 Timing and frequency of examination of patients with PH

Indicators of the effectiveness of treatment and the safety of diagnostic methods.

Evaluation of the effectiveness of treatment and determination of the patient's objective condition is carried out taking into account the prognostic criteria presented in tables 12 and 13.

Table 12 Prognostic criteria for PH treatment

*TAPSE and pericardial effusion can be measured in almost all patients, so these criteria are presented for predicting PH.

Table 13 Determination of the objective state of the patient

Treatment is assessed as ineffective if the condition of patients with baseline FC II-III is defined as "stable and unsatisfactory", as well as "unstable and deteriorating".

For patients with baseline FC IV, in the absence of progression to FC III or higher, as well as the definition of the condition as "stable and unsatisfactory", treatment is assessed as ineffective.

Drugs (active substances) used in the treatment

Hospitalization

Indications for hospitalization

The diagnosis of pulmonary hypertension is established only in stationary conditions.

emergency hospitalization(up to 2 hours):

Clinic of pulmonary hypertension crisis: sharply increasing shortness of breath, severe cyanosis, cold extremities, hypotension, syncope, chest pain, dizziness).

Minutes of the meetings of the Expert Commission on Health Development of the Ministry of Health of the Republic of Kazakhstan, 2014

1. 1. Galie, N et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation ( ISHLT). Eur Heart J 2009;30:2493–2537. 2. Revised Classification of Pulmonary HTN, Nice, France 2013. 3. Mukerjee D, et al. Rheumatology 2004; 43:461-6. 4. Robyn J Barst A review of pulmonary arterial hypertension: the role of ambrisentan Vasc Health Risk Manag. February 2007; 3 (1): 11–22. PMCID: PMC1994051; 5. Frumkin LR. The Pharmacological Treatment of Pulmonary Arterial Hypertension. Pharmacol Rev 2012;1. 6. Simonneau G et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH): A Phase III long-term extension study (CHEST-2). 5th World Symposium of Pulmonary Hypertension (WSPH) 2013, Nice, France. Poster

Information

III. ORGANIZATIONAL ASPECTS OF PROTOCOL IMPLEMENTATION

List of developers:

Abzalieva S.A. - Candidate of Medical Sciences, Director of the Department of Clinical Activities of AGIUV

Kulembayeva A.B. - Candidate of Medical Sciences, Deputy Chief Physician of the PKP on the REM of the BSNP in Almaty

Axis deviation right (+150)

qR complex in holes. V1, R:S ratio in otv. V6<1

Visualization of changes in the structure of pulmonary blood flow.

Immunogram

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3.2 Medical treatment.

3,2,1 Maintenance therapy.

3,2,2 Specific therapy.

Cor pulmonale (PC) is hypertrophy and/or dilatation of the right ventricle (RV) resulting from pulmonary arterial hypertension caused by diseases that affect the function and/or structure of the lungs and are not associated with a primary pathology of the left heart or congenital heart defects. LS is formed due to diseases of the bronchi and lungs, thoracophrenic lesions or pathology of the pulmonary vessels. The development of chronic cor pulmonale (CHP) is most often due to chronic pulmonary insufficiency (CLF), and the main cause of the formation of CLP is alveolar hypoxia, which causes spasm of the pulmonary arterioles.

Diagnostic search is aimed at identifying the underlying disease that led to the development of CHL, as well as assessing CRF, pulmonary hypertension, and the condition of the pancreas.

Treatment of CHLS is the treatment of the underlying disease that is the cause of CHLS (chronic obstructive bronchitis, bronchial asthma, etc.), elimination of alveolar hypoxia and hypoxemia with a decrease in pulmonary arterial hypertension (training of the respiratory muscles, electrical stimulation of the diaphragm, normalization of the oxygen transport function of the blood (heparin, erythrocytapheresis, hemosorption), long-term oxygen therapy (VCT), almitrin), as well as correction of right ventricular heart failure (ACE inhibitors, diuretics, aldosterone blockers, angiothesin II receptor antagonists). VCT is the most effective treatment for CLN and CHLS, which can increase the life expectancy of patients.

Keywords: cor pulmonale, pulmonary hypertension, chronic pulmonary insufficiency, chronic cor pulmonale, right ventricular heart failure.

DEFINITION

Pulmonary heart- this is hypertrophy and / or dilatation of the right ventricle, resulting from pulmonary arterial hypertension caused by diseases that affect the function and / or structure of the lungs and are not associated with a primary pathology of the left heart or congenital heart defects.

Pulmonary heart (PC) is formed on the basis of pathological changes in the lung itself, violations of extrapulmonary respiratory mechanisms that provide ventilation of the lung (damage to the respiratory muscles, violation of the central regulation of respiration, elasticity of the bone and cartilage formations of the chest, or conduction of a nerve impulse along n. diaphragmicus, obesity), as well as damage to the pulmonary vessels.

CLASSIFICATION

In our country, the classification of cor pulmonale proposed by B.E. Votchalom in 1964 (Table 7.1).

Acute LS is associated with a sharp increase in pulmonary arterial pressure (PAP) with the development of right ventricular failure and is most often caused by thromboembolism of the main trunk or large branches of the pulmonary artery (PE). However, the doctor sometimes encounters a similar condition when large areas of lung tissue are turned off from the circulation (bilateral extensive pneumonia, status asthmaticus, valve pneumothorax).

Subacute cor pulmonale (PLC) is most often the result of recurrent thromboembolism of small branches of the pulmonary artery. The leading clinical symptom is increasing dyspnea with rapidly developing (within months) right ventricular failure. Other causes of PLS ​​include neuromuscular diseases (myasthenia gravis, poliomyelitis, damage to the phrenic nerve), exclusion of a significant part of the respiratory section of the lung from the act of breathing (severe bronchial asthma, miliary pulmonary tuberculosis). A common cause of PLS ​​are oncological diseases of the lungs, gastrointestinal tract, breast and other localization, due to lung carcinomatosis, as well as compression of the lung vessels by a germinating tumor, followed by thrombosis.

Chronic cor pulmonale (CHP) in 80% of cases occurs with damage to the bronchopulmonary apparatus (most often with COPD) and is associated with a slow and gradual increase in pressure in the pulmonary artery over many years.

The development of CLS is directly related to chronic pulmonary insufficiency (CLF). In clinical practice, the classification of CRF based on the presence of dyspnea is used. There are 3 degrees of CLN: the appearance of shortness of breath with previously available efforts - I degree, shortness of breath during normal exertion - II degree, shortness of breath at rest - III degree. It is sometimes appropriate to supplement the above classification with data on the gas composition of the blood and pathophysiological mechanisms for the development of pulmonary insufficiency (Table 7.2), which makes it possible to select pathogenetically substantiated therapeutic measures.

Classification of cor pulmonale (according to Votchal B.E., 1964)

Table 7.1.

The end of the table. 7.1.

Note. The diagnosis of cor pulmonale is made after the diagnosis of the underlying disease: when formulating the diagnosis, only the first two columns of the classification are used. Columns 3 and 4 contribute to an in-depth understanding of the essence of the process and the choice of therapeutic tactics

Table 7.2.

Clinical and pathophysiological classification of chronic pulmonary insufficiency

(Aleksandrov O.V., 1986)

In the presented classification of CLN, the diagnosis of CLN with a high probability can be made at stages II and III of the process. In stage I CLN (latent), rises in PAP are detected, usually in response to physical activity and during an exacerbation of the disease in the absence of signs of RV hypertrophy. This circumstance made it possible to express the opinion (N.R. Paleev) that in order to diagnose the initial manifestations of CLS, it is necessary to use not the presence or absence of RV myocardial hypertrophy, but an increase in PAP. However, in clinical practice, direct measurement of PAP in this group of patients is not sufficiently substantiated.

Over time, the development of decompensated HLS is possible. In the absence of a special classification of RV failure, the well-known classification of heart failure (HF) according to V.Kh. Vasilenko and N.D. Strazhesko, which is usually used for heart failure, which has developed as a result of damage to the left ventricle (LV) or both ventricles. The presence of left ventricular HF in patients with CLS is most often due to two reasons: 1) CHL in people over 50 years of age is often combined with coronary artery disease, 2) systemic arterial hypoxemia in patients with CLS leads to dystrophic processes in the LV myocardium, to its moderate hypertrophy and contractile insufficiency.

Chronic obstructive pulmonary disease is the main cause of chronic cor pulmonale.

PATHOGENESIS

The development of chronic LS is based on the gradual formation of pulmonary arterial hypertension due to several pathogenetic mechanisms. The main cause of PH in patients with bronchopulmonary and thoracophrenic forms of CLS is alveolar hypoxia, the role of which in the development of pulmonary vasoconstriction was first shown in 1946 by U. Von Euler and G. Lijestrand. The development of the Euler-Liljestrand reflex is explained by several mechanisms: the effect of hypoxia is associated with the development of depolarization of vascular smooth muscle cells and their contraction due to changes in the function of potassium channels of cell membranes.

wounds, exposure to the vascular wall of endogenous vasoconstrictor mediators, such as leukotrienes, histamine, serotonin, angiotensin II and catecholamines, the production of which increases significantly under hypoxic conditions.

Hypercapnia also contributes to the development of pulmonary hypertension. However, a high concentration of CO 2, apparently, does not act directly on the tone of the pulmonary vessels, but indirectly - mainly through the acidosis caused by it. In addition, CO 2 retention contributes to a decrease in the sensitivity of the respiratory center to CO 2, which further reduces lung ventilation and contributes to pulmonary vasoconstriction.

Of particular importance in the genesis of PH is endothelial dysfunction, manifested by a decrease in the synthesis of vasodilating antiproliferative mediators (NO, prostacyclin, prostaglandin E 2) and an increase in the level of vasoconstrictors (angiotensin, endothelin-1). Pulmonary endothelial dysfunction in COPD patients is associated with hypoxemia, inflammation, and exposure to cigarette smoke.

Structural changes in the vascular bed occur in CLS patients - remodeling of the pulmonary vessels, characterized by thickening of the intima due to the proliferation of smooth muscle cells, deposition of elastic and collagen fibers, hypertrophy of the muscular layer of the arteries with a decrease in the inner diameter of the vessels. In patients with COPD, due to emphysema, there is a reduction in the capillary bed, compression of the pulmonary vessels.

In addition to chronic hypoxia, along with structural changes in the vessels of the lungs, a number of other factors also affect the increase in pulmonary pressure: polycythemia with a change in the rheological properties of blood, impaired metabolism of vasoactive substances in the lungs, an increase in minute blood volume due to tachycardia and hypervolemia. One of the possible causes of hypervolemia is hypercapnia and hypoxemia, which increase the concentration of aldosterone in the blood and, accordingly, Na + and water retention.

In patients with severe obesity, Pickwick's syndrome (named after the work of Charles Dickens) develops, which is manifested by hypoventilation with hypercapnia, which is associated with a decrease in the sensitivity of the respiratory center to CO 2, as well as impaired ventilation due to mechanical limitation by adipose tissue with dysfunction (fatigue) respiratory muscles.

Elevated blood pressure in the pulmonary artery may initially contribute to an increase in the volume of perfusion of the pulmonary capillaries, however, over time, hypertrophy of the myocardium of the pancreas develops, followed by its contractile insufficiency. Indicators of pressure in the pulmonary circulation are presented in table. 7.3.

Table 7.3

Indicators of pulmonary hemodynamics

The criterion for pulmonary hypertension is the level of mean pressure in the pulmonary artery at rest, exceeding 20 mm Hg.

CLINIC

The clinical picture consists of the manifestations of the underlying disease, leading to the development of CHLS and damage to the pancreas. In clinical practice, chronic obstructive pulmonary disease (COPD) is most often found among the causative pulmonary diseases, i.e. bronchial asthma or chronic obstructive bronchitis and emphysema. The CLS clinic is inextricably linked with the manifestation of CHLN itself.

A characteristic complaint of patients is shortness of breath. Initially, during exercise (stage I of CRF), and then at rest (stage III of CRF). It has an expiratory or mixed character. A long course (years) of COPD dulls the patient's attention and forces him to consult a doctor when shortness of breath appears during mild exertion or at rest, that is, already in stage II-III CRF, when the presence of CHL is indisputable.

Unlike dyspnea associated with left ventricular failure and venous congestion in the lungs, dyspnea in pulmonary hypertension does not increase in the horizontal position of the patient and does not

decreases in the sitting position. Patients may even prefer a horizontal position of the body, in which the diaphragm takes a greater part in intrathoracic hemodynamics, which facilitates the process of breathing.

Tachycardia is a frequent complaint of patients with CHL and appears even at the stage of development of CRF in response to arterial hypoxemia. Heart rhythm disorder is rare. The presence of atrial fibrillation, especially in people over 50 years of age, is usually associated with concomitant coronary artery disease.

Half of the patients with CLS experience pain in the heart area, often of an indeterminate nature, without irradiation, as a rule, not associated with physical activity and not relieved by nitroglycerin. The most common view on the mechanism of pain is relative coronary insufficiency due to a significant increase in the muscle mass of the pancreas, as well as a decrease in the filling of the coronary arteries with an increase in end-diastolic pressure in the pancreatic cavity, myocardial hypoxia against the background of general arterial hypoxemia (“blue angina pectoris”) and reflex narrowing right coronary artery (pulmocoronary reflex). A possible cause of cardialgia may be stretching of the pulmonary artery with a sharp increase in pressure in it.

With decompensation of the pulmonary heart, edema may appear in the legs, which first occur most often during an exacerbation of a bronchopulmonary disease and are first localized in the area of ​​​​the feet and ankles. As right ventricular failure progresses, edema spreads to the area of ​​the legs and thighs, and rarely, in severe cases of right ventricular failure, there is an increase in the abdomen in volume due to emerging ascites.

A less specific symptom of cor pulmonale is loss of voice, which is associated with compression of the recurrent nerve by a dilated trunk of the pulmonary artery.

Patients with CLN and CHLS may develop encephalopathy due to chronic hypercapnia and cerebral hypoxia, as well as impaired vascular permeability. With severe encephalopathy, some patients experience increased excitability, aggressiveness, euphoria, and even psychosis, while other patients experience lethargy, depression, drowsiness during the day and insomnia at night, and headaches. Rarely, syncope occurs during physical exertion as a result of severe hypoxia.

A common symptom of CLN is a diffuse "grayish-blue", warm cyanosis. When right ventricular failure occurs in patients with CLS, cyanosis often acquires a mixed character: against the background of diffuse bluish staining of the skin, cyanosis of the lips, tip of the nose, chin, ears, fingertips and toes appears, and the extremities in most cases remain warm, possibly due to peripheral vasodilation due to hypercapnia. Swelling of the cervical veins is characteristic (including on inspiration - Kussmaul's symptom). Some patients may develop a painful blush on the cheeks and an increase in the number of vessels on the skin and conjunctiva (“rabbit or frog eyes” due to hypercapnia), Plesh’s symptom (swelling of the neck veins when pressing the palm of the hand on the enlarged liver), Corvisar’s face, cardiac cachexia, signs of the main diseases (emphysematous chest, kyphoscoliosis of the thoracic spine, etc.).

On palpation of the region of the heart, a pronounced diffuse cardiac impulse, epigastric pulsation (due to hypertrophy and dilatation of the pancreas) can be detected, and with percussion, an expansion of the right border of the heart to the right. However, these symptoms lose their diagnostic value due to the often developing emphysema, in which the percussion dimensions of the heart can even be reduced (“drip heart”). The most common auscultatory symptom in CHLS is the emphasis of the second tone over the pulmonary artery, which can be combined with splitting of the second tone, right ventricular IV heart sound, diastolic murmur of pulmonary valve insufficiency (Graham-Still murmur) and systolic murmur of tricuspid insufficiency, with the intensity of both murmurs increasing by inspiratory height (Rivero-Corvalho symptom).

Arterial pressure in patients with compensated CHLS is often increased, and in decompensated patients it is reduced.

Hepatomegaly is detected in almost all patients with decompensated LS. The liver is enlarged, compacted on palpation, painful, the edge of the liver is rounded. With severe heart failure, ascites appears. In general, such severe manifestations of right ventricular heart failure in CLS are rare, because the very presence of severe CRF or the addition of an infectious process in the lung leads to a tragic ending in the patient earlier than it occurs due to heart failure.

The clinic of chronic cor pulmonale is determined by the severity of pulmonary pathology, as well as pulmonary and right ventricular heart failure.

INSTRUMENTAL DIAGNOSIS

The X-ray picture of CLS depends on the stage of CRF. Against the background of radiological manifestations of a pulmonary disease (pneumosclerosis, emphysema, increased vascular pattern, etc.), at first there is only a slight decrease in the shadow of the heart, then a moderate bulging of the cone of the pulmonary artery appears in the direct and right oblique projection. Normally, in direct projection, the right heart contour is formed by the right atrium, and in CHLS with an increase in the RV, it becomes edge-forming, and with significant hypertrophy of the RV, it can form both the right and left edges of the heart, pushing the left ventricle back. In the final decompensated stage of HLS, the right edge of the heart can be formed by a significantly dilated right atrium. Nevertheless, this "evolution" takes place against the background of a relatively small shadow of the heart ("drip" or "hanging").

Electrocardiographic diagnosis of CLS is reduced to the detection of pancreatic hypertrophy. The main (“direct”) ECG criteria for RV hypertrophy include: 1) R in V1>7mm; 2) S in V5-6 > 7 mm; 3) RV1 + SV5 or RV1 + SV6 > 10.5 mm; 4) RaVR > 4 mm; 5) SV1,V2 =s2 mm; 6) RV5,V6<5 мм; 7) отношение R/SV1 >one; 8) complete blockade of the right leg of the bundle of His with RV1>15 mm; 9) incomplete blockade of the right leg of the bundle of His with RV1>10 mm; 10) negative TVl and decrease in STVl, V2 with RVl>5 mm and no coronary insufficiency. In the presence of 2 or more "direct" ECG signs, the diagnosis of RV hypertrophy is considered reliable.

Indirect ECG signs of RV hypertrophy suggest RV hypertrophy: 1) rotation of the heart around the longitudinal axis clockwise (shift of the transition zone to the left, to leads V5-V6 and the appearance in leads V5, V6 of the QRS type RS complex; SV5-6 is deep, and RV1-2 - normal amplitude); 2) SV5-6 > RV5-6; 3) RaVR > Q(S)aVR; 4) deviation of the electrical axis of the heart to the right, especially if α>110; 5) electric axis heart type

SI-SII-SIII; 6) complete or incomplete blockade of the right leg of the bundle of His; 7) electrocardiographic signs of right atrial hypertrophy (P-pulmonale in leads II, III, aVF); 8) an increase in the activation time of the right ventricle in V1 by more than 0.03 s. There are three types of ECG changes in CHLS:

1. rSR "-type ECG is characterized by the presence of a split QRS complex of the rSR type in lead V1 and is usually detected with severe RV hypertrophy;

2. The R-type ECG is characterized by the presence of a QRS complex of the Rs or qR type in lead V1 and is usually detected with severe RV hypertrophy (Fig. 7.1).

3. S-type ECG is often detected in COPD patients with emphysema. It is associated with a posterior displacement of the hypertrophied heart, which is caused by pulmonary emphysema. The ECG looks like rS, RS or Rs with a pronounced S wave in both the right and left chest leads

Rice. 7.1. ECG of a patient with COPD and CHLS. Sinus tachycardia. Pronounced hypertrophy of the right ventricle (RV1 = 10 mm, SV1 is absent, SV5-6 = 12 mm, a sharp EOS deviation to the right (α = +155°), negative TV1-2 and a decrease in the STV1-2 segment). Right atrial hypertrophy (P-pulmonale in V2-4)

Electrocardiographic criteria for RV hypertrophy are not sufficiently specific. They are less clear-cut than in LV hypertrophy and can lead to false positive and false negative diagnoses. A normal ECG does not exclude the presence of CHLS, especially in patients with COPD, so ECG changes should be compared with the clinical picture of the disease and echocardiography data.

Echocardiography (EchoCG) is the leading non-invasive method for assessing pulmonary hemodynamics and diagnosing LS. Ultrasound diagnosis of LS is based on the identification of signs of damage to the myocardium of the pancreas, which are given below.

1. Change in the size of the right ventricle, which is assessed in two positions: in the parasternal position along the long axis (normally less than 30 mm) and in the apical four-chamber position. To detect dilatation of the pancreas, measurement of its diameter (normally less than 36 mm) and area at the end of diastole along the long axis in the apical four-chamber position is more often used. In order to more accurately assess the severity of RV dilatation, it is recommended to use the ratio of the RV end-diastolic area to the LV end-diastolic area, thereby excluding individual differences in heart size. An increase in this indicator of more than 0.6 indicates a significant dilatation of the pancreas, and if it becomes equal to or greater than 1.0, then a conclusion is made about a pronounced dilatation of the pancreas. With dilatation of the RV in the apical four-chamber position, the shape of the RV changes from crescent-shaped to oval, and the apex of the heart may be occupied not by the LV, as is normal, but by the RV. Dilatation of the pancreas may be accompanied by dilatation of the trunk (more than 30 mm) and branches of the pulmonary artery. With massive thrombosis of the pulmonary artery, its significant dilatation (up to 50-80 mm) can be determined, and the lumen of the artery becomes oval.

2. With hypertrophy of the pancreas, the thickness of its anterior wall, measured in diastole in the subcostal four-chamber position in the B- or M-mode, exceeds 5 mm. In patients with CHLS, as a rule, not only the anterior wall of the pancreas is hypertrophied, but also the interventricular septum.

3. Tricuspid regurgitation of varying degrees, which in turn causes dilatation of the right atrium and inferior vena cava, the decrease in inspiratory collapse of which indicates increased pressure in the right atrium.

4. Evaluation of the diastolic function of the pancreas is performed on the basis of the transtricuspid diastolic flow in the mode of pulsed

wave Doppler and color M-modal Doppler. In patients with CHLS, a decrease in the diastolic function of the pancreas is found, which is manifested by a decrease in the ratio of peaks E and A.

5. Reduced contractility of the pancreas in patients with LS is manifested by hypokinesia of the pancreas with a decrease in its ejection fraction. An echocardiographic study determines such indicators of RV function as end-diastolic and end-systolic volumes, ejection fraction, which normally is at least 50%.

These changes have different severity depending on the severity of the development of drugs. So, in acute LS, dilatation of the pancreas will be detected, and in chronic LS, signs of hypertrophy, diastolic and systolic dysfunction of the pancreas will be added to it.

Another group of signs is associated with the development of pulmonary hypertension in LS. The degree of their severity is most significant in acute and subacute LS, as well as in patients with primary pulmonary hypertension. CHLS is characterized by a moderate increase in systolic pressure in the pulmonary artery, which rarely reaches 50 mm Hg. Assessment of the pulmonary trunk and flow in the outflow tract of the pancreas is performed from the left parasternal and subcostal short-axis approach. In patients with pulmonary pathology, due to the limitation of the ultrasound window, the subcostal position may be the only possible access to visualize the outflow tract of the pancreas. Using pulsed wave Doppler, you can measure the average pressure in the pulmonary artery (Ppa), for which the formula proposed by A. Kitabatake et al. is usually used. (1983): Log10(Pra) = - 2.8 (AT/ET) + 2.4, where AT is the acceleration time of the flow in the outflow tract of the pancreas, ET is the ejection time (or the time of expulsion of blood from the pancreas). The Ppa value obtained using this method in patients with COPD correlates well with the data of an invasive examination, and the possibility of obtaining a reliable signal from the pulmonary valve exceeds 90%.

The most important for the detection of pulmonary hypertension is the severity of tricuspid regurgitation. The use of a jet of tricuspid regurgitation is the basis of the most accurate non-invasive method for determining systolic pressure in the pulmonary artery. Measurements are carried out in the continuous-wave Doppler mode in the apical four-chamber or subcostal position, preferably with the simultaneous use of color Doppler

whom mapping. To calculate the pressure in the pulmonary artery, it is necessary to add the pressure in the right atrium to the pressure gradient across the tricuspid valve. Measurement of the transtricuspid gradient can be performed in more than 75% of patients with COPD. There are qualitative signs of pulmonary hypertension:

1. With PH, the nature of the movement of the posterior cusp of the pulmonary valve changes, which is determined in the M-mode: a characteristic indicator of PH is the presence of an average systolic tooth due to partial overlap of the valve, which forms a W-shaped movement of the valve in systole.

2. In patients with pulmonary hypertension, due to increased pressure in the right ventricle, the interventricular septum (IVS) is flattened, and the left ventricle resembles the letter D (D-shaped left ventricle) along the short axis. With a high degree of PH, the IVS becomes, as it were, the wall of the pancreas and moves paradoxically towards the left ventricle in diastole. When the pressure in the pulmonary artery and the right ventricle becomes more than 80 mm Hg, the left ventricle decreases in volume, is compressed by the dilated right ventricle and takes on the shape of a crescent.

3. Possible regurgitation on the pulmonary valve (regurgitation of the first degree is normal in young people). With a constant-wave Doppler study, it is possible to measure the rate of pulmonary regurgitation with a further calculation of the magnitude of the end-diastolic pressure gradient of the LA-RV.

4. Change in the shape of the blood flow in the outflow tract of the pancreas and at the mouth of the LA valve. At normal pressure in the LA, the flow has an isosceles shape, the peak of the flow is located in the middle of systole; in pulmonary hypertension, the peak flow shifts to the first half of systole.

However, in patients with COPD, their pulmonary emphysema often makes it difficult to clearly visualize the structures of the heart and narrows the echocardiogram window, making the study informative in no more than 60-80% of patients. In recent years, a more accurate and informative method of ultrasound examination of the heart has appeared - transesophageal echocardiography (TEE). TEE in patients with COPD is the preferred method for accurate measurements and direct visual assessment of the structures of the pancreas, due to the higher resolution of the transesophageal probe and the stability of the ultrasound window, and is of particular importance in emphysema and pneumosclerosis.

Catheterization of the right heart and pulmonary arteries

Right heart and pulmonary artery catheterization is the gold standard for diagnosing PH. This procedure allows you to directly measure the pressure in the right atrium and RV, pressure in the pulmonary artery, calculate cardiac output and pulmonary vascular resistance, determine the level of oxygenation of mixed venous blood. Catheterization of the right heart due to its invasiveness cannot be recommended for widespread use in the diagnosis of CHL. Indications are: severe pulmonary hypertension, frequent episodes of decompensated right ventricular failure, and selection of candidates for lung transplantation.

Radionuclide ventriculography (RVG)

RVG measures the right ventricular ejection fraction (REF). EFVC is considered abnormal below 40-45%, but the EFVC itself is not a good indicator of right ventricular function. It allows you to evaluate the systolic function of the right ventricle, which is highly dependent on afterload, decreasing with an increase in the latter. Therefore, a decrease in EFVC is recorded in many patients with COPD, and is not an indicator of true right ventricular dysfunction.

Magnetic resonance imaging (MRI)

MRI is a promising method for assessing pulmonary hypertension and changes in the structure and function of the right ventricle. An MRI-measured right pulmonary artery diameter greater than 28 mm is a highly specific sign of PH. However, the MRI method is quite expensive and is available only in specialized centers.

The presence of a chronic lung disease (as a cause of CLS) requires a special study of the function of external respiration. The doctor is faced with the task of clarifying the type of ventilation insufficiency: obstructive (impaired passage of air through the bronchi) or restrictive (decrease in the area of ​​gas exchange). In the first case, chronic obstructive bronchitis, bronchial asthma can be cited as an example, and in the second - pneumosclerosis, lung resection, etc.

TREATMENT

CLS occurs most often after the onset of CLN. Therapeutic measures are complex in nature and are aimed mainly at correcting these two syndromes, which can be represented as follows:

1) treatment and prevention of the underlying disease - most often exacerbations of chronic pulmonary pathology (basic therapy);

2) treatment of CLN and PH;

3) treatment of right ventricular heart failure. Basic therapeutic and preventive measures include

prevention of acute viral respiratory diseases (vaccination) and exclusion of smoking. With the development of chronic pulmonary pathology of an inflammatory nature, it is necessary to treat exacerbations with antibiotics, mucoregulatory drugs and immunocorrectors.

The main thing in the treatment of chronic pulmonary heart is the improvement of the function of external respiration (elimination of inflammation, broncho-obstructive syndrome, improvement of the respiratory muscles).

The most common cause of CLN is broncho-obstructive syndrome, the cause of which is the contraction of the smooth muscles of the bronchi, the accumulation of viscous inflammatory secretions, and edema of the bronchial mucosa. These changes require the use of beta-2-agonists (fenoterol, formoterol, salbutamol), M-anticholinergics (ipratropium bromide, tiotropium bromide), and in some cases inhaled glucocorticosteroid drugs in the form of inhalations using a nebulizer or an individual inhaler. It is possible to use methylxanthines (eufillin and prolonged theophyllines (teolong, teotard, etc.)). Therapy with expectorants is very individual and requires various combinations and selection of herbal remedies (coltsfoot, wild rosemary, thyme, etc.), and chemical production (acetylcysteine, ambroxol, etc.).

If necessary, exercise therapy and postural drainage of the lungs are prescribed. Breathing with positive expiratory pressure (no more than 20 cm of water column) is shown using both simple devices

in the form of "whistles" with a movable diaphragm, and complex devices that control the pressure on exhalation and inhalation. This method reduces the air flow inside the bronchus (which has a bronchodilator effect) and increases the pressure inside the bronchi in relation to the surrounding lung tissue.

The extrapulmonary mechanisms of CRF development include a decrease in the contractile function of the respiratory muscles and diaphragm. The possibilities for correcting these disorders are still limited: exercise therapy or electrical stimulation of the diaphragm in stage II. HLN.

In CLN, erythrocytes undergo a significant functional and morphological reorganization (echinocytosis, stomatocytosis, etc.), which significantly reduces their oxygen transport function. In this situation, it is desirable to remove erythrocytes with lost function from the bloodstream and stimulate the release of young (functionally more capable). For this purpose, it is possible to use erythrocytepheresis, extracorporeal blood oxygenation, hemosorption.

Due to the increase in the aggregation properties of erythrocytes, blood viscosity increases, which requires the appointment of antiplatelet agents (chimes, reopoliglyukin) and heparin (preferably the use of low molecular weight heparins - fraxiparin, etc.).

In patients with hypoventilation associated with reduced activity of the respiratory center, drugs that increase central inspiratory activity - respiratory stimulants - can be used as auxiliary methods of therapy. They should be used for moderate respiratory depression that does not require the use of O 2 or mechanical ventilation (sleep apnea syndrome, obesity-hypoventilation syndrome), or when oxygen therapy is not possible. The few drugs that increase arterial blood oxygenation include nikethamide, acetosalamide, doxapram, and medroxyprogesterone, but all of these drugs have a large number of side effects with long-term use and therefore can only be used for a short time, such as during an exacerbation of the disease.

Currently, almitrina bismesilate is one of the drugs that can correct hypoxemia in patients with COPD for a long time. Almitrin is a specific ago-

nistome of peripheral chemoreceptors of the carotid node, the stimulation of which leads to an increase in hypoxic vasoconstriction in poorly ventilated regions of the lungs with an improvement in ventilation-perfusion ratios. The ability of almitrin at a dose of 100 mg / day has been proven. in patients with COPD, lead to a significant increase in paCO2 (by 5-12 mm Hg) and a decrease in paCO2 (by 3-7 mmHg) with an improvement in clinical symptoms and a decrease in the frequency of exacerbations of the disease, which is capable of several years to delay the appointment of long-term 0 2 therapy. Unfortunately, 20-30% of COPD patients do not respond to therapy, and widespread use is limited by the possibility of developing peripheral neuropathy and other side effects. Currently, the main indication for the appointment of almitrin is moderate hypoxemia in patients with COPD (pa0 2 56-70 mm Hg or Sa0 2 89-93%), as well as its use in combination with VCT, especially against the background of hypercapnia.

Vasodilators

In order to reduce the degree of PAH, peripheral vasodilators are included in the complex therapy of patients with cor pulmonale. The most commonly used calcium channel antagonists and nitrates. Two calcium antagonists currently recommended are nifedipine and diltiazem. The choice in favor of one of them depends on the initial heart rate. Patients with relative bradycardia should be recommended nifedipine, with relative tachycardia - diltiazem. The daily doses of these drugs, which have proven effective, are quite high: for nifedipine 120-240 mg, for diltiazem 240-720 mg. Favorable clinical and prognostic effects of calcium antagonists used in high doses in patients with primary PH (especially those with a previous positive acute test) have been shown. III generation dihydropyridine calcium antagonists - amlodipine, felodipine, etc. - are also effective in this group of patients with LS.

However, calcium channel antagonists are not recommended for COPD-associated pulmonary hypertension, despite their ability to reduce Ppa and increase cardiac output in this group of patients. This is due to the aggravation of arterial hypoxemia caused by dilatation of the pulmonary vessels in

poorly ventilated areas of the lungs with deterioration in ventilation-perfusion ratios. In addition, with long-term therapy with calcium antagonists (more than 6 months), the beneficial effect on the parameters of pulmonary hemodynamics is leveled.

A similar situation in patients with COPD occurs with the appointment of nitrates: acute samples demonstrate a deterioration in gas exchange, and long-term studies show the absence of a positive effect of drugs on pulmonary hemodynamics.

Synthetic prostacyclin and its analogues. Prostacyclin is a powerful endogenous vasodilator with antiaggregatory, antiproliferative and cytoprotective effects that are aimed at preventing pulmonary vascular remodeling (reducing endothelial cell damage and hypercoagulability). The mechanism of action of prostacyclin is associated with relaxation of smooth muscle cells, inhibition of platelet aggregation, improvement of endothelial function, inhibition of vascular cell proliferation, as well as a direct inotropic effect, positive changes in hemodynamics, and an increase in oxygen utilization in skeletal muscles. The clinical use of prostacyclin in patients with PH is associated with the synthesis of its stable analogues. To date, the greatest experience in the world has been accumulated for epoprostenol.

Epoprostenol is a form of intravenous prostacyclin (prostaglandin I 2). Favorable results were obtained in patients with vascular form of LS - with primary PH in systemic connective tissue diseases. The drug increases cardiac output and reduces pulmonary vascular resistance, and with long-term use improves the quality of life of patients with LS, increasing exercise tolerance. The optimal dose for most patients is 20-40 ng/kg/min. An analog of epoprostenol, treprostinil, is also used.

Oral formulations of a prostacyclin analogue have now been developed. (beraprost, iloprost) and clinical trials are being conducted in the treatment of patients with a vascular form of LS developed as a result of pulmonary embolism, primary pulmonary hypertension, and systemic connective tissue diseases.

In Russia, from the group of prostanoids for the treatment of patients with LS, only prostaglandin E 1 (vazaprostan) is currently available, which is prescribed intravenously

growth 5-30 ng/kg/min. Course treatment with the drug is carried out at a daily dose of 60-80 mcg for 2-3 weeks against the background of long-term therapy with calcium antagonists.

Endothelin receptor antagonists

Activation of the endothelin system in patients with PH was the rationale for the use of endothelin receptor antagonists. The effectiveness of two drugs of this class (bosentan and sitaczentan) in the treatment of patients with chronic respiratory disease, which developed against the background of primary PH or against the background of systemic connective tissue diseases, has been proven.

Phosphodiesterase type 5 inhibitors

Sildenafil is a powerful selective inhibitor of cGMP-dependent phosphodiesterase (type 5), preventing the degradation of cGMP, causes a decrease in pulmonary vascular resistance and right ventricular overload. To date, there are data on the effectiveness of sildenafil in patients with LS of various etiologies. When using sildenafil in doses of 25-100 mg 2-3 times a day, it caused an improvement in hemodynamics and exercise tolerance in patients with LS. Its use is recommended when other drug therapy is ineffective.

Long-term oxygen therapy

In patients with bronchopulmonary and thoracophrenic form of CLS, the main role in the development and progression of the disease belongs to alveolar hypoxia, therefore, oxygen therapy is the most pathogenetically substantiated method of treating these patients. The use of oxygen in patients with chronic hypoxemia is critical and must be continuous, long-term, and usually administered at home, hence this form of therapy is called long-term oxygen therapy (LTOT). The task of VCT is to correct hypoxemia with the achievement of pO 2 values ​​>60 mm Hg. and Sa0 2 >90%. It is considered optimal to maintain paO 2 within 60-65 mm Hg, and exceeding these values ​​leads only to a slight increase in Sa0 2 and oxygen content in arterial blood, however, it may be accompanied by CO 2 retention, especially during sleep, which has negative

effects on the function of the heart, brain and respiratory muscles. Therefore, VCT is not indicated for patients with moderate hypoxemia. Indications for VCT: raO 2<55 мм рт.ст. или Sa0 2 < 88% в покое, а также раО 2 56-59 мм рт.ст. или Sa0 2 89% при наличии легочного сердца или полицитемии (гематокрит >55%). For most patients with COPD, an O 2 flow of 1–2 l/min is sufficient, and in the most severe patients, the flow can be increased to 4–5 l/min. The oxygen concentration should be 28-34% vol. VCT is recommended for at least 15 hours per day (15-19 hours per day). The maximum breaks between oxygen therapy sessions should not exceed 2 hours in a row, because. breaks of more than 2-3 hours significantly increase pulmonary hypertension. Oxygen concentrators, liquid oxygen tanks and compressed gas cylinders can be used for VCT. The most commonly used concentrators (permeators) that release oxygen from the air by removing nitrogen. VCT increases the life expectancy of patients with CRF and CLS by an average of 5 years.

Thus, despite the presence of a large arsenal of modern pharmacological agents, VCT is the most effective method of treating most forms of CLS, so the treatment of patients with CLS is primarily the task of a pulmonologist.

Long-term oxygen therapy is the most effective method of treating CLN and HLS, increasing the life expectancy of patients by an average of 5 years.

Long-term home ventilation

In the terminal stages of pulmonary diseases, due to a decrease in the ventilation reserve, hypercapnia may develop, requiring respiratory support, which should be carried out for a long time, on an ongoing basis, at home.

NO inhalation therapy

Inhalation therapy with NO, whose action is similar to the endothelium-relaxing factor, has a positive effect in patients with CLS. Its vasodilating effect is based on the activation of guanylate cyclase in the smooth muscle cells of the pulmonary vessels, which leads to an increase in the level of cyclo-GMP and a decrease in the intracellular calcium content. Inhalation N0 region

gives a selective effect on the vessels of the lungs, and it causes vasodilation mainly in well-ventilated regions of the lungs, improving gas exchange. With the course application of NO in patients with chronic respiratory disease, there is a decrease in pressure in the pulmonary artery, an increase in the partial pressure of oxygen in the blood. In addition to its hemodynamic effects, NO prevents and reverses pulmonary vascular and pancreatic remodeling. The optimal doses of inhaled NO are concentrations of 2-10 ppm, and high concentrations of NO (more than 20 ppm) can cause excessive vasodilation of the pulmonary vessels and lead to a deterioration in the ventilation-perfusion balance with increased hypoxemia. The addition of NO inhalations to VCT in patients with COPD enhances the positive effect on gas exchange, reducing the level of pulmonary hypertension and increasing cardiac output.

CPAP therapy

Continuous Positive Airway Pressure Therapy (continuous positive airway pressure- CPAP) is used as a method of treatment for CRF and CLS in patients with obstructive sleep apnea syndrome, preventing the development of airway collapse. The proven effects of CPAP are the prevention and resolution of atelectasis, an increase in lung volumes, a decrease in ventilation-perfusion imbalance, an increase in oxygenation, lung compliance, and redistribution of fluid in lung tissue.

cardiac glycosides

Cardiac glycosides in patients with COPD and cor pulmonale are effective only in the presence of left ventricular heart failure, and may also be useful in the development of atrial fibrillation. Moreover, it has been shown that cardiac glycosides can induce pulmonary vasoconstriction, and the presence of hypercapnia and acidosis increases the likelihood of glycoside intoxication.

Diuretics

In the treatment of patients with decompensated CHLS with edematous syndrome, diuretic therapy, including antagonists, is used.

aldosterone (aldactone). Diuretics should be given cautiously, at low doses, because in RV failure, cardiac output is more dependent on preload and, therefore, an excessive reduction in intravascular fluid volume can lead to a decrease in RV filling volume and a decrease in cardiac output, as well as an increase in blood viscosity. and a sharp decrease in pressure in the pulmonary artery, thereby worsening the diffusion of gases. Another serious side effect of diuretic therapy is metabolic alkalosis, which in patients with COPD with respiratory failure can lead to inhibition of the activity of the respiratory center and deterioration of gas exchange.

Angiotensin-converting enzyme inhibitors

In the treatment of patients with decompensated cor pulmonale in recent years, angiotensin-converting enzyme inhibitors (ACE inhibitors) have come to the fore. ACE inhibitor therapy in patients with CHLS leads to a decrease in pulmonary hypertension and an increase in cardiac output. In order to select an effective therapy for CLS in patients with COPD, it is recommended to determine the polymorphism of the ACE gene, because only in patients with subtypes of the ACE II and ID gene, a pronounced positive hemodynamic effect of ACE inhibitors is observed. The use of ACE inhibitors in minimal therapeutic doses is recommended. In addition to the hemodynamic effect, there is a positive effect of ACE inhibitors on the size of the heart chambers, remodeling processes, exercise tolerance and increased life expectancy in patients with heart failure.

Angiotensin II receptor antagonists

In recent years, data have been obtained on the successful use of this group of drugs in the treatment of CLS in patients with COPD, which was manifested by an improvement in hemodynamics and gas exchange. The appointment of these drugs is most indicated in patients with CLS with intolerance to ACE inhibitors (due to dry cough).

Atrial septostomy

Recently, in the treatment of patients with right ventricular heart failure that developed against the background of primary PH, there have been

use an atrial septostomy, i.e. creation of a small perforation in the interatrial septum. Creating a right-to-left shunt allows you to reduce the average pressure in the right atrium, unload the right ventricle, increase the left ventricular preload and cardiac output. Atrial septostomy is indicated when all types of medical treatment of right ventricular heart failure are ineffective, especially in combination with frequent syncope, or as a preparatory stage before lung transplantation. As a result of the intervention, there is a decrease in syncope, an increase in exercise tolerance, but the risk of developing life-threatening arterial hypoxemia increases. The mortality rate of patients during atrial septostomy is 5-15%.

Lung or heart-lung transplant

From the end of the 80s. In the 20th century, after the introduction of the immunosuppressive drug cyclosporine A, lung transplantation began to be successfully used in the treatment of end-stage pulmonary insufficiency. In patients with CLN and LS, transplantation of one or both lungs, the heart-lung complex is performed. It was shown that 3 and 5-year survival after transplantation of one or both lungs, heart-lung complex in patients with LS was 55 and 45%, respectively. Most centers prefer to perform bilateral lung transplantation due to fewer postoperative complications.