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Pulmonary arterial hypertension clinical guidelines. Chronic cor pulmonale: clinical signs and treatment recommendations

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LECTURE ON INTERNAL DISEASES.

TOPIC: HEART PULMONARY.

Relevance of the topic: Diseases of the bronchopulmonary system and chest are of great importance in affecting the heart. Damage to the cardiovascular system in diseases of the bronchopulmonary apparatus is referred to by most authors as 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, which developed as a result of diseases of the bronchi and lungs, deformation of the chest, or primary damage to the pulmonary arteries. (WHO 1961).

Hypertrophy of the right ventricle and its dilatation due to changes as a result of primary heart damage or congenital defects 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:

“Pulmonary heart is a complex of hemodynamic disorders in the pulmonary circulation, developing as a result of diseases of the bronchopulmonary apparatus, deformations of the chest, and primary damage to the pulmonary arteries, which at the final stage manifests itself as right ventricular hypertrophy and progressive circulatory failure.”

ETIOLOGY OF HEART PULMONARY.

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 cause the development 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 and diaphragm with limitation of their mobility:

kyphoscoliosis

multiple rib injuries

Pickwickian 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, tumor, etc.).

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

bronchopulmonary

thoradiaphragmatic

vascular

Standards for values characterizing the hemodynamics of the pulmonary circulation.

Systolic pressure in the pulmonary artery is approximately five times less than systolic pressure in the systemic circulation.

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

PATHOGENESIS.

The pathogenesis of cor pulmonale is based on pulmonary hypertension. Since cor pulmonale most often develops in bronchopulmonary diseases, we’ll start with that. All diseases, and in particular chronic obstructive bronchitis, will primarily lead to respiratory (pulmonary) failure. Pulmonary insufficiency is a condition in which the normal gas composition of the blood is disrupted.

This is a state of the body in which either the maintenance of normal blood gas composition is not ensured, 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 pulmonary failure.

Arterial hypoxemia underlies the pathogenesis of chronic heart diseases, especially 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, pulmonary emphysema, and intra-alveolar pressure increases. Under conditions of arterial hypoxemia, the non-respiratory function of the lungs is disrupted - biological active substances begin to be produced, which have not only a bronchospastic, but also a vasospastic effect. At the same time, a violation of the vascular architecture 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 increased blood viscosity. Alveolar hypoxia will lead to hypoxemic vasoconstriction through a reflex called the Euler-Liestrand reflex. Alveolar hypoxia led to hypoxemic vasoconstriction, increased intra-arterial pressure, which leads to increased hydrostatic pressure in the capillaries. Impaired non-respiratory function of the lungs leads to the release of serotonin, histamine, prostaglandins, catecholamines, but the most important thing is that under conditions of tissue and alveolar hypoxia, the interstitium begins to produce angiotensin converting enzyme in greater quantities. 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 in conditions of restructuring of central hemodynamics will lead not just to an increase in pressure in the pulmonary artery, but to a persistent increase in it (above 30 mmHg), that is, to the development of pulmonary hypertension. If the processes continue further, if the underlying disease is not treated, then naturally some of the vessels in the pulmonary artery system die due to pneumosclerosis, and the pressure persistently increases in the pulmonary artery. At the same time, persistent secondary pulmonary hypertension will lead to the fact that the shunts between the pulmonary artery and the bronchial arteries open and unoxygenated 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 dilatation quickly develops in it.

The fourth stage is hypertrophy or dilatation of the right ventricle. Dystrophy of the right ventricular myocardium will contribute as well as tissue hypoxia.

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

Pathogenesis of the development of cor pulmonale in the thoracodiaphragmatic form: in this form, the leading factor is hypoventilation of the lungs due to kyphoscoliosis, pleural suppuration, spinal deformities, or obesity 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 pulmonary heart disease. 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 is that with thrombosis of the main branches of the pulmonary arteries, the blood supply to the pulmonary tissue sharply decreases, since along with thrombosis of the main branches, there is a concomitant reflex narrowing of the small branches. 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 sertonin, prostaglandins, catecholamines, the release of convertase, angiotensin-converting enzyme.

The pathogenesis of cor pulmonale is multistage, multistage, and in some cases not entirely clear.

CLASSIFICATION OF HEART PULMONARY.

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

bronchopulmonary heart

thoradiaphragmatic

vascular

A domestic classification of the pulmonary heart has been proposed, which provides for the division of the pulmonary heart according to the rate of development:

subacute
chronic

Acute cor pulmonale develops over a period of hours, minutes, or 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, or decompensated.

According to its genesis, acute cor pulmonale develops in vascular and bronchopulmonary forms. Subacute and chronic cor pulmonale can be vascular, bronchopulmonary, or thoradiaphragmatic.

Acute cor pulmonale develops primarily:

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

with pneumothorax (especially valvular),

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

for 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 with chorionepitheliomas and peripheral lung cancer. The thoracodiaphragmatic form develops with hypoventilation of central or peripheral origin - myasthenia gravis, botulism, poliomyelitis, etc.

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

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

stage of severe pulmonary failure - 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 cardiac damage.

stage of pulmonary heart failure of varying degrees (swelling in the extremities, enlarged abdomen, etc.).

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

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

pulmonary failure 2 - VC/BVC 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.

third stage - pulmonary failure 3 - VC/CVC less than 40%, arterial blood saturation up to 50%, signs of right ventricular hypertrophy appear on the ECG as direct signs. Circulatory failure 2A.

fourth stage - pulmonary failure 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 pulmonary embolism, an acute increase in intrathoracic pressure due to an attack of bronchial asthma. Arterial precapillary hypertension in acute cor pulmonale, as in the vascular form of chronic cor pulmonale, is accompanied by an increase in pulmonary resistance. Next comes the rapid development of right ventricular dilatation. Acute right ventricular failure is manifested by severe shortness of breath turning into inspiratory suffocation, rapidly increasing cyanosis, chest pain of various types, shock or collapse, rapidly increasing liver size, swelling in the legs, ascites, epigastric pulsation, tachycardia (120-140), harsh breathing , in some places weakened vesicular; Moist, varied rales are heard, especially in the lower parts of the lungs. Additional research methods, especially ECG, are of great importance in the development of acute pulmonary heart disease: 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. Right bundle branch block is complete or incomplete, ST inversion (usually elevation), S in the first lead is deep, Q in the third lead is deep. Negative S wave in the second and third leads. The same signs may also occur in acute myocardial infarction of the posterior wall.

Emergency care depends on the cause of acute cor pulmonale. If there was a pulmonary embolism, painkillers, fibrinolytic and anticoagulant drugs (heparin, fibrinolysin), streptodecase, streptokinase) are prescribed, including surgical treatment.

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

For valvular pneumothorax - surgical treatment. In case of confluent pneumonia, along with antibiotic treatment, diuretics and cardiac glycosides are necessarily prescribed.

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, shortness of breath of an expiratory nature with an unchanged respiratory rate, with restrictive processes, the duration of exhalation decreases and the respiratory rate increases. Upon 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, edema of the lower extremities, ascites. The pulse is increased, the boundaries of the heart expand to the right, and then to the left, the tones are dull due to emphysema, the accent of the second tone is 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 - a Graham-Still murmur, which is associated with relative insufficiency of the pulmonary valve. Above the lungs percussion there is a box sound, breathing is vesicular and harsh. In the lower parts of the lungs there are congestive, silent moist rales. When palpating the abdomen, there is an enlarged 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.

First stage: against the background of the underlying disease, shortness of breath intensifies, cyanosis appears in the form of acrocyanosis, but the right border of the heart is not enlarged, the liver is not enlarged, physical findings in the lungs depend on the underlying disease.

The second stage - shortness of breath turns into attacks of suffocation, with difficulty in breathing, cyanosis becomes diffuse, from the data of an objective study: 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 and may be prolapsed.

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

The fourth stage is shortness of breath at rest, forced position, often accompanied by respiratory rhythm disorders such as Cheyne-Stokes and Biot. The swelling is constant, cannot be treated, the pulse is weak and frequent, the heart is bullish, the sounds are muffled, the systolic murmur at the xiphoid process. There is a lot of moist rales in the lungs. The liver is of considerable size and does not contract under the influence of glycosides and diuretics as fibrosis develops. Patients are constantly dozing.

Diagnosis of thoradiaphragmatic heart is often difficult; one must always remember about the possibility of its development in kyphoscoliosis, ankylosing spondylitis, etc. The most important sign is the early appearance of cyanosis, and a noticeable increase in shortness of breath without attacks of suffocation. 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, and arterial hypertension. Diabetes mellitus often develops.

Chronic cor pulmonale in primary pulmonary hypertension is called Aerz's disease (described in 1901). A polyetiological disease of unknown origin, it mainly affects women from 20 to 40 years old. Pathomorphological studies have established that with primary pulmonary hypertension, thickening of the intima of the precapillary arteries occurs, that is, in muscular-type arteries, thickening of the media is noted, 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, and Raynaud's syndrome. And then shortness of breath increases, which is a sign that indicates that primary pulmonary hypertension is entering a stable final stage. Cyanosis quickly increases, which is expressed to the degree of a cast-iron tint, becomes permanent, and swelling quickly increases. The diagnosis of primary pulmonary hypertension is established by exclusion. Most often this diagnosis is pathological. In these patients, the entire clinical picture progresses without any background in the form of obstructive or restrictive breathing disorders. 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: for a chronic process in the lungs - leukocytosis, an increase in the number of red blood cells (polycythemia associated with increased erythropoiesis due to arterial hypoxemia). X-ray findings: appear very late. One of the early symptoms is bulging of the pulmonary artery trunk on x-ray. The pulmonary artery bulges, often flattening the waist of the heart, and this heart is mistaken by many doctors for the 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, angle greater than 120 degrees. The most basic indirect sign is an increase in the interval of the R wave in V1 by more than 7 mm.

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

Spirography, which reveals the type and degree of respiratory failure, is of great importance. On the 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 diagnostics are Doppler cardiography, echocardiography - enlargement of the right side of the heart, increased pressure in the pulmonary artery.

PRINCIPLES OF TREATMENT OF HEART PULMONARY.

Treatment of cor pulmonale involves treating the underlying disease. In case of exacerbation of obstructive diseases, bronchodilators and expectorants are prescribed. For 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). Sodium nitroprusside is of greatest importance 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, antiplatelet therapy - heparin, trental, etc. Due to tissue hypoxia, myocardial dystrophy quickly develops, so cardioprotectors are prescribed (potassium orotate, panangin, riboxin). Cardiac glycosides are prescribed very carefully.

PREVENTION.

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

PULMONARY HEART.

Hypertrophy of the right ventricle and its dilatation due to changes as a result of primary heart damage or congenital defects do not belong to the concept of cor pulmonale.

ETIOLOGY OF HEART PULMONARY.

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 cause the development 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 and diaphragm with limitation of their mobility:

kyphoscoliosis

multiple rib injuries

Pickwickian 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, tumor, etc.).

bronchopulmonary

thoradiaphragmatic

vascular

Standards for values characterizing the hemodynamics of the pulmonary circulation.

Systolic pressure in the pulmonary artery is approximately five times less than systolic pressure in the systemic circulation.

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

PATHOGENESIS.

There are 3 stages of pulmonary failure.

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

The fourth stage is hypertrophy or dilatation of the right ventricle. Dystrophy of the right ventricular myocardium will contribute as well as tissue hypoxia.

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

The pathogenesis of cor pulmonale is multistage, multistage, and in some cases not entirely clear.

CLASSIFICATION OF HEART PULMONARY.

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

bronchopulmonary heart

thoradiaphragmatic

vascular

A domestic classification of the pulmonary heart has been proposed, which provides for the division of the pulmonary heart according to the rate of development:

subacute
chronic

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

According to its genesis, acute cor pulmonale develops in vascular and bronchopulmonary forms. Subacute and chronic cor pulmonale can be vascular, bronchopulmonary, or thoradiaphragmatic.

Acute cor pulmonale develops primarily:

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

with pneumothorax (especially valvular),

for acute confluent pneumonia

right-sided total pleurisy

stage of pulmonary heart failure of varying degrees (swelling in the extremities, enlarged abdomen, etc.).

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

third stage - pulmonary failure 3 - VC/CVC less than 40%, arterial blood saturation up to 50%, signs of right ventricular hypertrophy appear on the ECG as direct signs. Circulatory failure 2A.

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

CLINIC OF ACUTE PULMONARY HEART.

For valvular pneumothorax - surgical treatment. In case of confluent pneumonia, along with antibiotic treatment, diuretics and cardiac glycosides are necessarily prescribed.

CLINIC OF CHRONIC PULMONARY HEART.

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

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

PRINCIPLES OF TREATMENT OF HEART PULMONARY.

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

PREVENTION.

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

Methodological recommendations are given on the clinical picture, diagnosis and treatment of pulmonary heart disease. Recommendations are addressed to students of 4-6 years. The electronic version of the publication is posted on the website of St. Petersburg State Medical University (http://www.spb-gmu.ru).

Methodological recommendations are addressed to students of 4-6 years Chronic cor pulmonale Under chronic cor pulmonale

Ministry of Health and Social Development

Russian Federation

G OU VPO "ST. PETERSBURG STATE"

MEDICAL UNIVERSITY

NAMED AFTER ACADEMICIAN I.P. PAVLOV"

Associate Professor V.N.Yablonskaya

Associate Professor O.A. Ivanova

assistant Zh.A. Mironova

Editor: Head Department of Hospital Therapy, St. Petersburg State Medical University named after. acad. I.P. Pavlova Professor V.I. Trofimov

Reviewer: Professor of the Department of Propaedeutics of Internal Diseases

St. Petersburg State Medical University named after. acad. I.P. Pavlova B.G. Lukichev

Chronic cor pulmonale

Under chronic cor pulmonale (HLS) understand right ventricular hypertrophy (RV), or a combination of hypertrophy with dilatation and/or right ventricular heart failure (RVH) due to diseases primarily affecting the function or structure of the lungs, or both, and not associated with primary failure of the left heart or congenital and acquired heart defects.

This definition of the WHO expert committee (1961), according to a number of experts, currently needs correction due to the introduction into practice of modern diagnostic methods and the accumulation of new knowledge about the pathogenesis of CHL. In particular, CHL is proposed to be considered as pulmonary hypertension in combination with hypertrophy. dilatation of the right ventricle, dysfunction of both ventricles of the heart associated with primary structural and functional changes in the lungs.

Pulmonary hypertension (PH) is said to occur when the pressure in the pulmonary artery (PA) exceeds established normal values:

Systolic – 26 – 30 mm Hg.

Diastolic – 8 – 9 mm Hg.

Average – 13 – 20 mm Hg.

Chronic cor pulmonale is not an independent nosological form, but it complicates many diseases that affect the airways and alveoli, the chest with limited mobility, as well as the pulmonary vessels. Essentially all diseases that can lead to the development of respiratory failure and pulmonary hypertension (there are more than 100 of them) can cause chronic cor pulmonale. However, in 70-80% of cases of CHL, chronic obstructive pulmonary disease (COPD) occurs. Currently, chronic pulmonary heart disease is observed in 10-30% of pulmonary patients hospitalized in a hospital. It is 4-6 times more common in men. Being a severe complication of chronic obstructive pulmonary disease (COPD), CLP determines the clinical picture, course and prognosis of this disease, leads to early disability of patients and is often the cause of death. Moreover, the mortality rate in patients with CHL has doubled over the past 20 years.

ETIOLOGY AND PATHOGENESIS OF CHRONIC PULMONARY HEART.

Since chronic cor pulmonale is a condition that occurs secondary and is essentially a complication of a number of respiratory diseases, in accordance with the primary causes, it is customary to distinguish the following types of CHL:

1.Bronchopulmonary:

The cause is diseases affecting the airways and alveoli:

Obstructive diseases (chronic obstructive pulmonary disease (COPD), primary pulmonary emphysema, severe bronchial asthma with significant irreversible obstruction)

Diseases occurring with severe pulmonary fibrosis (tuberculosis, bronchiectasis, pneumoconiosis, repeated pneumonia, radiation injury)

Interstitial lung diseases (idiopathic fibrosing alveolitis, pulmonary sarcoidosis, etc.), collagenosis, pulmonary carcinomatosis

2. Thoradiaphragmatic:

The reason is diseases that affect the chest (bones, muscles, pleura) and affect the mobility of the chest:

Chronic cor pulmonale: the view of cardiologists

Prepared by Maxim Gvozdik | 03/27/2015

The prevalence of chronic obstructive pulmonary disease (COPD) is rapidly increasing worldwide: if

in 1990 they were in twelfth place in the structure of morbidity, then according to WHO experts, by 2020 they will move into the top five after pathologies such as coronary heart disease (CHD), depression, injuries due to road accidents and cerebrovascular diseases. It is also predicted that by 2020, COPD will take third place in the structure of causes of death. IHD, arterial hypertension and obstructive pulmonary diseases are quite often combined, which gives rise to a number of problems both in pulmonology and cardiology. November 30, 2006

A scientific and practical conference “Features of diagnosis and treatment of obstructive pulmonary diseases with concomitant pathology” was held at the F.G. Yanovsky Institute of Phthisiology and Pulmonology of the Academy of Medical Sciences of Ukraine

cardiovascular system", during which much attention was paid to general problems of cardiology

and pulmonology.

The report “Heart failure in chronic cor pulmonale: a cardiologist’s view” was made by

Corresponding Member of the Academy of Medical Sciences of Ukraine, Doctor of Medical Sciences, Professor Ekaterina Nikolaevna Amosova .

– In modern cardiology and pulmonology there are a number of common problems regarding which it is necessary to reach a common opinion and unify approaches. One of them is chronic pulmonary heart disease. Suffice it to say that dissertations on this topic are equally often defended in both cardiology and pulmonology councils; it is included in the list of problems dealt with by both branches of medicine, but unfortunately, a unified approach to this pathology has not yet been developed. We should not forget about general practitioners and family doctors who find it difficult to understand the contradictory information and information published in the pulmonology and cardiology literature.

The definition of chronic cor pulmonale in a WHO document dates back to 1963. Unfortunately, since that time, WHO recommendations on this important issue have not been clarified or re-affirmed, which, in fact, has led to discussions and contradictions. Today, in foreign cardiological literature there are practically no publications about chronic cor pulmonale, although there is a lot of talk about pulmonary hypertension; moreover, the recommendations of the European Society of Cardiology regarding pulmonary hypertension have recently been revised and approved.

The concept of “pulmonary heart” includes extremely heterogeneous diseases; they differ in etiology, mechanism of development of myocardial dysfunction, its severity, and have different approaches to treatment. Chronic cor pulmonale is based on hypertrophy, dilatation, and dysfunction of the right ventricle, which, by definition, are associated with pulmonary hypertension. The heterogeneity of these diseases is even more obvious when considering the degree of increase in pulmonary artery pressure in pulmonary hypertension. In addition, its very presence has completely different meanings for different etiological factors of chronic pulmonary heart disease. For example, in vascular forms of pulmonary hypertension, this is the basis that requires treatment, and only a decrease in pulmonary hypertension can improve the patient’s condition; in COPD, pulmonary hypertension is not so pronounced and does not require treatment, as evidenced by Western sources. Moreover, a decrease in pressure in the pulmonary artery in COPD does not lead to relief, but worsens the patient’s condition, as there is a decrease in blood oxygenation. Thus, pulmonary hypertension is an important condition for the development of chronic cor pulmonale, but its importance should not be taken into account.

Often this pathology becomes the cause of chronic heart failure. And if we talk about it in cor pulmonale, it is worth recalling the criteria for diagnosing heart failure (HF), which are reflected in the recommendations of the European Society of Cardiology. To make a diagnosis, there must be: firstly, symptoms and clinical signs of heart failure, and secondly, objective signs of systolic or diastolic myocardial dysfunction. That is, the presence of dysfunction (changes in myocardial function at rest) is mandatory for making a diagnosis.

The second question is the clinical symptoms of chronic pulmonary heart disease. In the cardiology audience, it is necessary to talk about the fact that edema does not correspond to the presence of right ventricular failure. Unfortunately, cardiologists are very little aware of the role of extracardiac factors in the origin of clinical signs of venous stagnation in the systemic circulation. Edema in such patients is often perceived as a manifestation of heart failure; they begin to actively treat it, but to no avail. This situation is well known to pulmonologists.

The pathogenetic mechanisms of the development of chronic pulmonary heart disease also include extracardiac factors of blood deposition. Of course, these factors are important, but they should not be overestimated and everything should be associated only with them. And finally, we talk little, in fact, we have just begun, about the role of hyperactivation of the renin-angiotensin-aldosterone system and its significance in the development of edema and hypervolemia.

In addition to the listed factors, it is worth mentioning the role of myocardiopathy. In the development of chronic pulmonary heart disease, a large role is played by damage to the myocardium not only of the right ventricle, but also of the left, which occurs under the influence of a complex of factors, including toxic ones, which are associated with bacterial agents; in addition, this is a hypoxic factor that causes dystrophy of the myocardium of the ventricles of the heart .

Our studies established that there is practically no correlation between systolic pressure in the pulmonary artery and the size of the right ventricle in patients with chronic cor pulmonale. There is some correlation between the severity of COPD and dysfunction of the right ventricle; in relation to the left ventricle, these differences are less pronounced. When analyzing the systolic function of the left ventricle, it was noted to deteriorate in patients with severe COPD. It is extremely difficult to correctly assess the contractility of the myocardium, even of the left ventricle, because the indices that we use in clinical practice are very rough and depend on pre- and afterload.

As for the indicators of diastolic function of the right ventricle, all patients were diagnosed with a hypertrophic type of diastolic dysfunction. The indicators from the right ventricle were expected, but from the left, we somewhat unexpectedly received signs of impaired diastolic relaxation, which increased depending on the severity of COPD.

Indicators of ventricular systolic function in patients with COPD and idiopathic pulmonary hypertension are different. Of course, changes in the right ventricle are more pronounced in idiopathic pulmonary hypertension, while at the same time the systolic function of the left ventricle is more altered in COPD, which is associated with the effect of unfavorable factors of infection and hypoxemia on the myocardium of the left ventricle, and then it makes sense to talk about cardiopathy in that broad sense understanding that is present in cardiology today.

In our study, type I disturbances in the diastolic function of the left ventricle were registered in all patients; peak indices were more pronounced in the right ventricle in patients with idiopathic pulmonary hypertension; diastolic disturbances in patients with COPD. It is worth emphasizing that these are relative indicators, because we took into account the different ages of the patients.

In all patients, during echocardiography, the diameter of the inferior vena cava was measured and the degree of its collapse during inspiration was determined. It was found that in moderate COPD, the diameter of the inferior vena cava is not increased; it increased only in severe COPD, when FEV1 is less than 50%. This allows us to raise the question that the role of extracardiac factors should not be absolutized. At the same time, the collapse of the inferior vena cava during inspiration was already impaired in moderate COPD (this indicator reflects an increase in pressure in the left atrium).

We also analyzed heart rate variability. It should be noted that cardiologists consider a decrease in heart rate variability to be a marker of activation of the sympathoadrenal system and the presence of heart failure, that is, prognostically unfavorable. We found a decrease in variability in moderate COPD, the severity of which increased in accordance with obstructive disorders of the ventilation function of the lungs. Moreover, we found a significant correlation between the severity of heart rate variability disturbances and right ventricular systolic function. This suggests that heart rate variability in COPD appears quite early and can serve as a marker of myocardial damage.

When diagnosing chronic pulmonary heart disease, especially in pulmonary patients, great attention must be paid to the instrumental study of myocardial dysfunction. In this regard, the most convenient study in clinical practice is echocardiography, although there are limitations for its use in patients with COPD, in whom radionuclide ventriculography of the right ventricle, which combines relatively low invasiveness and very high accuracy, should ideally be used.

Of course, it is not news to anyone that chronic cor pulmonale in COPD and idiopathic pulmonary hypertension is very heterogeneous in terms of the morphofunctional state of the ventricles, prognosis and a number of other reasons. The existing European classification of heart failure, which was included practically unchanged in the document of the Ukrainian Society of Cardiology, does not reflect the difference in the mechanisms of development of this disease. If these classifications were convenient in clinical practice, we would not be discussing this topic. It seems logical to us to leave the term “chronic pulmonary heart” for bronchopulmonary pathology, emphasizing decompensated, subcompensated and compensated. This approach will avoid the use of the terms FC and SN. In vascular forms of chronic pulmonary heart disease (idiopathic, postthromboembolic pulmonary hypertension), it is advisable to use an approved gradation of heart failure. However, it seems to us appropriate, by analogy with cardiological practice, to indicate the presence of right ventricular systolic dysfunction in the diagnosis, because this is important for chronic pulmonary heart disease associated with COPD. If the patient does not have dysfunction, this is one situation in prognostic and treatment plans; if there is, the situation is significantly different.

Cardiologists in Ukraine have been using the Strazhesko-Vasilenko classification for several years when diagnosing chronic heart failure, always indicating whether the systolic function of the left ventricle is preserved or reduced. So why not use this in relation to chronic pulmonary heart disease?

Doctor of Medical Sciences, Professor Yuri Nikolaevich Sirenko dedicated his speech to the peculiarities of treatment of patients with coronary artery disease and arterial hypertension in combination with COPD.

– In preparation for the conference, I tried to find on the Internet references over the past 10 years to pulmonary arterial hypertension, a nosology that often appeared in the USSR. I managed to find approximately 5 thousand references to arterial hypertension in chronic obstructive pulmonary diseases, but the problem of pulmonary arterial hypertension does not exist anywhere in the world except in the countries of the post-Soviet space. Today there are several positions regarding the diagnosis of so-called pulmonary arterial hypertension. They were developed in the early 1980s, when more or less reliable functional research methods appeared.

The first position is the development of pulmonary arterial hypertension 5-7 years after the onset of chronic lung disease; the second is the connection between increased blood pressure and exacerbation of COPD; third – increased blood pressure due to increased bronchial obstruction; fourth, daily monitoring reveals a connection between increased blood pressure and inhalation of sympathomimetics; fifth – high variability of blood pressure throughout the day with a relatively low average level.

I managed to find a very serious work by Moscow academician E.M. Tareev “Does pulmonary arterial hypertension exist?”, in which the author makes a mathematical assessment of the possible connection between the above factors in patients with arterial hypertension and COPD. And no dependency was found! The research results did not confirm the existence of independent pulmonary arterial hypertension. Moreover, E.M. Tareev believes that systemic arterial hypertension in patients with COPD should be considered as hypertension.

After this categorical conclusion, I looked at the world's recommendations. There is not a single line about COPD in the current recommendations of the European Society of Cardiology, and the American ones (seven recommendations of the National Joint Committee) also say nothing on this topic. It was possible to find only in the American recommendations of 1996 (in six editions) information that non-selective beta-blockers should not be used in patients with COPD, and if there is a cough, ACE inhibitors should be replaced with angiotensin receptor blockers. That is, such a problem really does not exist in the world!

Then I reviewed the statistics. It turned out that they began to talk about pulmonary arterial hypertension after it was established that approximately 35% of COPD patients have high blood pressure. Today, Ukrainian epidemiology gives the following figures: among the adult rural population, blood pressure is elevated in 35%, in the urban population – in 32%. We cannot say that COPD increases the incidence of arterial hypertension, so we should not talk about pulmonary arterial hypertension, but about some specifics of the treatment of arterial hypertension in COPD.

Unfortunately, in our country, sleep apnea syndrome, except for the Institute of Phthisiology and Pulmonology named after. F.G. Yanovsky AMS of Ukraine, are practically not studied anywhere. This is due to the lack of equipment, money and desire for specialists. And this question is very important and represents another problem where cardiac pathology intersects with respiratory tract pathology and there is a very high percentage of the risk of developing cardiovascular complications and death. Pulmonary hypertension, cardiac and respiratory failure complicate and worsen the course of arterial hypertension and, most importantly, worsen the treatment options for patients.

I would like to start the conversation about the treatment of arterial hypertension with a simple algorithm, which is the basis for cardiologists and therapists. A doctor who encounters a patient with hypertension is faced with questions: what form of arterial hypertension does the patient have - primary or secondary - and are there signs of target organ damage and cardiovascular risk factors? By answering these questions, the doctor knows the patient’s treatment tactics.

To date, there is not a single randomized clinical trial that was specifically designed to clarify the treatment of arterial hypertension in COPD, so modern recommendations are based on three very unreliable factors: retrospective analysis, expert opinion and the doctor’s own experience.

Where should treatment begin? Of course, with first-line antihypertensive drugs. The first and main group of them are beta-blockers. Many questions arise regarding their selectivity, but there are already drugs with fairly high selectivity, confirmed in experiment and clinic, which are safer than the drugs we used before.

When assessing airway patency in healthy people after taking atenolol, a worsening response to salbutamol and minor changes when taking more modern drugs were found. Although, unfortunately, such studies have not been conducted with the participation of patients, the categorical ban on the use of beta-blockers in patients with COPD should still be lifted. They should be prescribed if the patient tolerates them well; it is advisable to use them in the treatment of arterial hypertension, especially in combination with coronary artery disease.

The next group of drugs are calcium antagonists; they are almost ideal for treating such patients, but we must remember that non-dihydropyridine drugs (diltiazem, verapamil) should not be used for high blood pressure in the pulmonary artery system. They have been shown to worsen the course of pulmonary hypertension. That leaves dihydropyridines, which are known to improve bronchial patency and may therefore reduce the need for bronchodilators.

Today, all experts agree that ACE inhibitors do not worsen the airway, do not cause cough in patients with COPD, and if it occurs, patients should be switched to angiotensin receptor blockers. We have not conducted special studies, but based on the literature data and our own observations, it can be argued that the experts are a little disingenuous, since a number of patients with COPD react with a dry cough to ACE inhibitors, and there is a serious pathogenetic basis for this.

Unfortunately, the following picture can very often be observed: a patient with high blood pressure goes to a cardiologist and is prescribed ACE inhibitors; After some time, the patient begins to cough, goes to a pulmonologist, who cancels ACE inhibitors, but does not prescribe angiotensin receptor blockers. The patient goes back to the cardiologist, and everything starts all over again. The reason for this situation is the lack of control over appointments. It is necessary to move away from this practice; therapists and cardiologists must take a comprehensive approach to treating the patient.

Another very important point in the treatment of patients, which reduces the possibility of side effects, is the use of lower doses. Modern European recommendations give the right to choose between low doses of one or two drugs. Today, the combination of different drugs has been proven to be highly effective, affecting various stages of pathogenesis and mutually reinforcing the effect of drugs. I believe that combination therapy for patients with COPD is the treatment of choice for hypertension.

– pathology of the right heart, characterized by enlargement (hypertrophy) and expansion (dilatation) of the right atrium and ventricle, as well as circulatory failure, developing as a result of hypertension of the pulmonary circulation. The formation of the pulmonary heart is facilitated by pathological processes of the bronchopulmonary system, pulmonary vessels, and chest. Clinical manifestations of acute cor pulmonale include shortness of breath, chest pain, increased skin cyanosis and tachycardia, psychomotor agitation, and hepatomegaly. The examination reveals an increase in the borders of the heart to the right, a gallop rhythm, pathological pulsation, signs of overload of the right parts of the heart on the ECG. Additionally, chest X-ray, ultrasound of the heart, pulmonary function test, and blood gas analysis are performed.

ICD-10

I27.9 Pulmonary heart failure, unspecified

General information

The acute form of cor pulmonale develops quickly, over several minutes, hours or days; chronic – over several months or years. Almost 3% of patients with chronic bronchopulmonary diseases gradually develop cor pulmonale. Cor pulmonale significantly aggravates the course of cardiopathologies, ranking 4th among the causes of mortality in cardiovascular diseases.

Reasons for the development of cor pulmonale

The bronchopulmonary form of cor pulmonale develops with primary lesions of the bronchi and lungs as a result of chronic obstructive bronchitis, bronchial asthma, bronchiolitis, emphysema, diffuse pneumosclerosis of various origins, polycystic lung disease, bronchiectasis, tuberculosis, sarcoidosis, pneumoconiosis, Hammen-Rich syndrome, etc. This form can cause about 70 bronchopulmonary diseases, contributing to the formation of cor pulmonale in 80% of cases.

The emergence of the thoradiaphragmatic form of cor pulmonale is facilitated by primary lesions of the chest, diaphragm, limitation of their mobility, which significantly impairs ventilation and hemodynamics in the lungs. These include diseases that deform the chest (kyphoscoliosis, ankylosing spondylitis, etc.), neuromuscular diseases (poliomyelitis), pathologies of the pleura, diaphragm (after thoracoplasty, with pneumosclerosis, paresis of the diaphragm, Pickwick syndrome in obesity, etc. ).

The vascular form of cor pulmonale develops with primary lesions of the pulmonary vessels: primary pulmonary hypertension, pulmonary vasculitis, thromboembolism of the branches of the pulmonary artery (PE), compression of the pulmonary trunk by an aortic aneurysm, atherosclerosis of the pulmonary artery, mediastinal tumors.

The main causes of acute cor pulmonale are massive pulmonary embolism, severe attacks of bronchial asthma, valvular pneumothorax, and acute pneumonia. Pulmonary heart of subacute course develops with repeated pulmonary embolism, cancerous lymphangitis of the lungs, in cases of chronic hypoventilation associated with poliomyelitis, botulism, myasthenia gravis.

Mechanism of development of cor pulmonale

Arterial pulmonary hypertension plays a leading role in the development of cor pulmonale. At the initial stage, it is also associated with a reflex increase in cardiac output in response to increased respiratory function and tissue hypoxia that occurs during respiratory failure. With the vascular form of cor pulmonale, the resistance to blood flow in the arteries of the pulmonary circulation increases mainly due to the organic narrowing of the lumen of the pulmonary vessels when they are blocked by emboli (in the case of thromboembolism), with inflammatory or tumor infiltration of the walls, or overgrowth of their lumen (in the case of systemic vasculitis). In bronchopulmonary and thoracodiaphragmatic forms of cor pulmonale, narrowing of the lumen of the pulmonary vessels occurs due to their microthrombosis, overgrowth with connective tissue or compression in areas of inflammation, tumor process or sclerosis, as well as when the ability of the lungs to stretch and collapse of blood vessels in altered segments of the lungs is weakened. But in most cases, the leading role is played by the functional mechanisms of the development of pulmonary arterial hypertension, which are associated with impaired respiratory function, pulmonary ventilation and hypoxia.

Arterial hypertension of the pulmonary circulation leads to overload of the right parts of the heart. As the disease develops, a shift in acid-base balance occurs, which may initially be compensated, but later decompensation of disorders may occur. With cor pulmonale, there is an increase in the size of the right ventricle and hypertrophy of the muscular membrane of large vessels of the pulmonary circulation, narrowing of their lumen with further sclerosis. Small vessels are often affected by multiple blood clots. Gradually, dystrophy and necrotic processes develop in the heart muscle.

Classification of the pulmonary heart

Based on the rate of increase in clinical manifestations, several variants of the course of cor pulmonale are distinguished: acute (develops over several hours or days), subacute (develops over weeks and months) and chronic (occurs gradually over a number of months or years against the background of prolonged respiratory failure).

The process of formation of chronic pulmonary heart goes through the following stages:

preclinical – manifested by transient pulmonary hypertension and signs of hard work of the right ventricle; are detected only during instrumental research;
compensated – characterized by right ventricular hypertrophy and stable pulmonary hypertension without symptoms of circulatory failure;
decompensated (cardiopulmonary failure) - symptoms of right ventricular failure appear.

There are three etiological forms of cor pulmonale: bronchopulmonary, thoracodiaphragmatic and vascular.

Based on compensation, chronic cor pulmonale can be compensated or decompensated.

Symptoms of cor pulmonale

The clinical picture of cor pulmonale is characterized by the development of heart failure against the background of pulmonary hypertension. The development of acute cor pulmonale is characterized by the appearance of sudden pain in the chest, severe shortness of breath; a decrease in blood pressure, up to the development of collapse, cyanosis of the skin, swelling of the neck veins, increasing tachycardia; progressive enlargement of the liver with pain in the right hypochondrium, psychomotor agitation. Characterized by increased pathological pulsations (precordial and epigastric), expansion of the border of the heart to the right, gallop rhythm in the area of the xiphoid process, ECG signs of overload of the right atrium.

With massive pulmonary embolism, a state of shock and pulmonary edema develops within a few minutes. Acute coronary insufficiency is often associated, accompanied by rhythm disturbances and pain. Sudden death occurs in 30-35% of cases. Subacute cor pulmonale is manifested by sudden moderate pain, shortness of breath and tachycardia, brief fainting, hemoptysis, and signs of pleuropneumonia.

In the compensation phase of chronic pulmonary heart disease, symptoms of the underlying disease are observed with gradual manifestations of hyperfunction, and then hypertrophy of the right heart, which are usually not clearly expressed. Some patients experience pulsation in the upper abdomen caused by enlargement of the right ventricle.

In the stage of decompensation, right ventricular failure develops. The main manifestation is shortness of breath, which worsens with physical activity, inhalation of cold air, and in a lying position. Pain in the heart area, cyanosis (warm and cold cyanosis), rapid heartbeat, swelling of the neck veins that persists during inspiration, liver enlargement, and peripheral edema that are resistant to treatment appear.

When examining the heart, muffled heart sounds are revealed. Blood pressure is normal or low, arterial hypertension is characteristic of congestive heart failure. Symptoms of cor pulmonale become more pronounced with exacerbation of the inflammatory process in the lungs. In the late stage, swelling increases, liver enlargement progresses (hepatomegaly), neurological disorders appear (dizziness, headaches, apathy, drowsiness), and diuresis decreases.

Diagnosis of pulmonary heart

Diagnostic criteria for cor pulmonale include the presence of diseases - causative factors of cor pulmonale, pulmonary hypertension, enlargement and expansion of the right ventricle, right ventricular heart failure. Such patients need consultation with a pulmonologist and cardiologist. When examining the patient, pay attention to signs of breathing problems, bluishness of the skin, pain in the heart, etc. The ECG determines direct and indirect signs of right ventricular hypertrophy.

Forecast and prevention of cor pulmonale

In cases of decompensation of the pulmonary heart, the prognosis for work capacity, quality and life expectancy is unsatisfactory. Typically, the ability to work in patients with cor pulmonale suffers already in the early stages of the disease, which dictates the need for rational employment and resolving the issue of assigning a disability group. Early initiation of complex therapy can significantly improve labor prognosis and increase life expectancy.

To prevent pulmonary heart disease, prevention, timely and effective treatment of the diseases leading to it is required. First of all, this concerns chronic bronchopulmonary processes, the need to prevent their exacerbations and the development of respiratory failure. To prevent the processes of decompensation of the pulmonary heart, it is recommended to adhere to moderate physical activity.

RCHR (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

Brief description

Approved

At the Expert Commission on Health Development Issues

Ministry of Health of the Republic of Kazakhstan

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

I. INTRODUCTORY PART:

Name: Pulmonary hypertension

Protocol code:

Code according to MBK-10:

I27.0 - Primary pulmonary hypertension

Abbreviations used in the protocol:

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

PAP pressure in the pulmonary artery

DZLK wedge pressure in pulmonary capillaries
ASD atrial septal defect
VSD ventricular septal defect
DPP pressure in the right atrium
D-EchoCG Doppler echocardiography
CTD connective tissue diseases
IPAH idiopathic pulmonary arterial hypertension
CT computed tomography

CAG coronary angiography
PAH pulmonary arterial hypertension
PA pulmonary artery

PH pulmonary hypertension
DZLK wedge pressure in pulmonary capillaries

PVR pulmonary vascular resistance
MPAP mean pressure in the pulmonary artery

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
Heart rate heart rate
EchoCG 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 valve annulus

V/Q ventilation-perfusion index

Date of development of the protocol: 2014

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

The following grades of recommendation and levels of evidence are used in this protocol (Appendix 1).

Classification

Classification :

Pathophysiological classification:

1. Precapillary: mean pressure in the PA ≥25mm.Hg, PAWP ≤15mm.Hg, CO normal/reduced.

Clinical groups:

− PH of lung diseases;

− CTEPH;

− PH with a multifactorial etiological factor.

2. Post-capillary: MPAP ≥25mmHg, PCWP >15mmHg, CO 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 newborns

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 left ventricular outflow tract.

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

3.2 Interstitial lung diseases

3.3 Other lung diseases with mixed restrictive and obstructive components

3.4 Breathing disorders during sleep

3.5 Alveolar hypoventilation

3.6 Chronic exposure to high altitude

3.7 Lung malformations

4. CTEPH

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 disease

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

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

Class	Description
Class I	Patients with PH, but without restrictions on physical activity. Standard exercise does not cause shortness of breath, fatigue, chest pain, or syncope.
Class II	Patients with PH with slight limitation of physical activity. Feel comfortable at rest. Standard exercise causes minor shortness of breath, fatigue, chest pain, and syncope.
Class III	Patients with PH with significant limitation of physical activity. Feel comfortable at rest. A load less than standard causes shortness of breath, fatigue, chest pain, and syncope.
Class IV	Patients with PH who are unable to perform any physical activity without symptoms. These patients have signs of heart failure of the right ventricular type. At rest, shortness of breath and/or fatigue may occur. Discomfort occurs at the slightest physical exertion.

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 on an outpatient basis for dynamic control:

(once every six months)

2. ECG (once per quarter)

3. EchoCG (every 3-6 months)

4. X-ray of the chest organs in 2 projections (direct, left lateral) (once a year and according to clinical indications)

Additional diagnostic examinations performed on an outpatient basis for dynamic control:

1. MRI of the chest and mediastinum

2. Duplex scanning of peripheral vessels of the extremities

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

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

1. General blood test 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 carried out that were not carried out at the outpatient level):

1. General blood test 6 parameters

2. Blood test for pro level - BNP

5. X-ray of the chest organs, direct and lateral projections with contrast 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 not performed at the outpatient level are carried out:

1. General urine test

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. Analysis of sputum for Mycobacterium tuberculosis by bacterioscopy

19. Emergency EchoCG

20. Ultrasound of the abdominal organs

21. Ultrasound of the thyroid gland

22. Ventilation-perfusion scintigraphy

Diagnostic measures carried out at the stage of emergency care:

2. Pulse oximetry

Diagnostic criteria

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

History of:
- deep vein thrombosis
- HIV infection
- liver diseases
- diseases of the left heart
- lung diseases

Hereditary diseases
- taking drugs and toxins (Table 2)

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

Definite

Aminorex

Fenfluramine

Dexfenfluramine

Toxic rapeseed oil

Benfluorex

Possible

Cocaine

Phenylpropanolamine

St. John's wort

Chemotherapy drugs

Selective serotonin reuptake inhibitor

Pergolide

Likely

Amphetamines

L - tryptophan

Methamphetamines

Unlikely

Oral contraceptives

Estrogens

Smoking

Physical examination:
- peripheral cyanosis
- hard breathing during auscultation of the lungs
- increased heart sounds along the left parasternal line
- strengthening of the pulmonary component of tone II
- pansystolic murmur of tricuspid regurgitation
- diastolic murmur of pulmonary valve insufficiency
- right ventricular III tone
- organic murmur of congenital heart defects

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

Laboratory research
- Determination of the BNP indicator in order to confirm the diagnosis of heart failure (primarily left ventricular dysfunction), clarify the causes of acute shortness of breath, assess the condition of patients with heart failure and monitor treatment. Standard values: BNP 100–400 pg/ml, NT-proBNP 400–2000 pg/ml.

General clinical laboratory examinations are carried out to identify the primary cause of the development of PH (Appendices 2,3).

Instrumental studies

Echocardiography
Echocardiography is an important study in the diagnosis of PH, since in addition to the tentative diagnosis, it allows us to record the primary disorders that caused PH (CHD with shunting, dysfunction of the left side of the heart, possible cardiac complications).
Criteria for establishing a diagnosis using Doppler echocardiography (Table 3).

Table 3 Doppler echocardiographic diagnosis of PH

EchoCG signs:	No LH	PH possible		PH probable
Velocity of tricuspid regurgitation	≤2.8m/s	≤2.8m/s	2.9 - 3.4m/s	>3.4m/s
SDLA	≤36mmHg	≤36mmHg	37-50mmHg	>50mmHg
Additional EchoCG signs of PH**	No	There is	No/is	No/is
Recommendation class	I	IIa	IIa	I
Level of evidence	B	C	C	B

Note:

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

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

Right ventricle, increased rate of regurgitation on the pulmonary valve, shortened acceleration time of ejection from the RV to the PA.

3. SDPZh = 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.
Catheterization of the right heart with tonometry and vasoreactive test is a mandatory study to establish the diagnosis of PAH.
To diagnose disease of the left heart, a coronary angiography is necessary.
The minimum volume of parameters that must be recorded during catheterization of the right heart:
- Pulmonary artery pressure (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 systemic circulation;
- LSS;
- DZLK;
- Presence/absence of pathological shunts
- Reaction to vasoreactive test. The vasoreactivity test result is considered positive if the MPAP decreases > 10 mmHg. Art. and/or reaches absolute value< 40 мм рт. ст. при условии неизменной величины сердечного выброса (больные с положительной острой реакцией).

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

Table 4 Use of drugs to perform a vasoreactive test

Preparation	Method of administration	Half-lifeleniya (T ½)	General dose	Initial dose	Duration of administration
Epoprostenol	intravenous	3min	2-12 ng / kg -1 / min -1	2 ng / kg -1 /min -1	10 min
Adenosine	intravenous	5-10s	50-350 mcg/kg -1 /min -1	50 µg/kg -1 /min -1	2 min
Nitric oxide	inhalation	15-30s	10-20 ml/min		5 min
Iloprost	inhalation	3min	2.5-5µg/kg	2.5 µg	2 min

X-ray of the chest organs

Chest X-ray allows one to reliably exclude moderate and severe lung diseases associated with PH and pulmonary venous hypertension caused by pathology of the left heart. However, a normal chest x-ray does not exclude mild post-capillary pulmonary hypertension due to diseases of the left heart.

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

− expansion of the pulmonary artery, which, when contrasted, “loses” its peripheral branches.

− enlargement of the right atrium and ventricle

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

In PH, the V/Q scan 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 levels, which is reflected by segmental perfusion defects when depicted graphically. 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 with specialists:

− Cardiologist (adult, pediatric, including interventional): exclusion of diseases of the left side of the heart, congenital heart defects, determination of treatment tactics for right ventricular failure, 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 primary lung damage

− Cardiac surgeon: for the purpose of diagnosing the primary disease (CHD, LV outflow obstruction).

− Phthisiatrician: in the presence of symptoms suspicious for tuberculosis.

− Oncologist: in the presence of symptoms suspicious for cancer.

− Nephrologist: if there are symptoms suspicious for kidney disease.

− Infectious disease specialist: if there are symptoms suspicious for schistosomiasis

− Geneticist: if hereditary PAH is suspected.

Differential diagnosis

Differential diagnosis: Table 5

Differential diagnosis	Diagnostic procedures	Diagnostic criteria
Hereditary PAH	Karyotyping with cytogenetic study	BNPR2; ALK1, ENG, SMAD9, CAV1, KCNK3
Drug- and toxin-induced PAH	History, blood test for toxins.	Identification of taking drugs from the list (Table 2)
PAH associated with congenital heart disease	EchoCG, catheterization of POS	Diagnosis of congenital heart disease with left-to-right blood shunting.
HIV-associated PAH	Immunological studies	HIV diagnosis
PAH associated with CTD	SRB, ASLO, RF, ANA, AFLA.	Diagnosis of systemic connective tissue disease.
PAH associated with portal hypertension	Biochemical blood test with determination of liver enzymes, bilirubin with fractions. Ultrasound of the abdominal organs, FEGDS.	Diagnosis of portal hypertension.
PH associated with left heart disease	ECG, EchoCG, CAG, ACG.	Diagnosis of systolic/diastolic dysfunction of the left ventricle, valve defects of the left heart, congenital/acquired obstruction of the left ventricle.
PH associated with lung diseases.	Chest X-ray, breathing tests, spirography	Diagnosis of COPD, interstitial lung diseases, other lung diseases with a mixed restrictive and obstructive component, sleep breathing disorders, alveolar hypoventilation, chronic exposure to high altitudes, lung malformations
XTELG	Ventilation-perfusion scintigraphy, angiopulmonography, echocardiography.	Diagnosis of defects in pulmonary perfusion and ventilation, detection of CTEPH.

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Treatment

Treatment goals:

1. Monitoring the course of the underlying disease

2. Prevention of complications

Treatment tactics

Non-drug treatment

Diet - table No. 10. Mode - 1.2

Drug treatment

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

Table 6. Drug therapy

Pharmacotherapeutic group	International generic Name	Unit (tablets, ampoules, capsule)	Single dose of medications	Frequency of application (number of times per day)
1	2	3	5	6
Basic
Calcium channel blockers
	Amlodipine	Tab.	0.05-0.2 mg/kg (adults 2.5-10 mg)	1
	Nifedipine	Caps.	0.25-0.5 mg/kg (adult 10-20 mg)	3
	Nifedipine	Tab.	0.5-1 mg/kg (adult 20-40 mg)	2
	Diltiazem	Tab.	90mg (adult)	3
PDE-5
	Sildenafil	Tab.	90mg (adult)	2
AER
	Bosentan	Tab.	1.5 - 2 mg/kg (therapeutic dose for adults 62.5 - 125 mg, for children 31.25 mg)	2
Prostanoids (antiplatelet agents)
	Iloprost (inhalation)	amp.	2.5-5 mcg	4-6
Additional
Diuretics
	Furosemide	Tab.	1-3mg/kg	2
	Furosemide	amp.	1-3mg/kg	2
	Veroshpiron	Tab.	3mg/kg	2
Indirect anticoagulants
	Warfarin	Tab.	Std. Scheme (INR)	1
ACE inhibitors
	Captopril	Tab.	0.1 mg/kg	3
	Enalapril	Tab.	0.1 mg/kg	2
Cardiac glycosides
	Digoxin	Tab.	12.5 mg	1

Indications for specific therapy are presented in Table 7

Table 7. Indications for specific therapy

Drugs		Class of recommendation - level of evidence
Drugs		WHO FC II	WHO FC III	WHO FC IV
Calcium channel blockers		I-C	I-C	-
AER	Bosentan	I-A	I-A	IIa-C
PDE-5	Sildenafil	I-A	I-A	IIa-C
Prostanoids	Iloprost (inhalation)	-	I-A	IIa-C
Initial combination therapy*		-	-	IIa-C
Concerted combination therapy**		IIa-C	IIa-B	IIa-B
Balloon atrioseptostomy		-	I-C	I-C
Lung transplantation		-	I-C	I-C

*Initial combination therapy includes specific and adjunctive therapy

**Concerted combination therapy used in case of lack of clinical effect (IIa-B):

Endothelin receptor antagonists AER + PDE-5 phosphodiesterase 5 inhibitors;

Endothelin receptor antagonists AER + prostanoids;
- phosphodiesterase 5 inhibitors PDE-5 + prostanoids

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

Table 8 Indications for specific therapy for a negative vasoreactive test

Indications for additional therapy are presented in Table 9

Table 9 Indications for additional therapy

Group of drugs	Indications	Class of recommendations, level of evidence
Diuretics	Signs of pancreatic failure, edema.	I-C
Oxygen therapy	When PO2 in arterial blood is less than 8 kPa (60 mmHg)	I-C
Oral anticoagulants	IPAH, hereditary PAH, PAH due to anorexigens, ALAH.	IIa-C
Digoxin	With the development of atrial tachyarrhythmia, in order to slow the heart rate	IIb-C

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

Patient group	Drugs	Recommendation class	Level of evidence
Eisemenger syndrome, WHO FC III	Bosentan	I	B
	Sildenafil	IIa	C
	Iloprost	IIa	C
	Combination therapy	IIb	C
	Ca channel blockers	IIa	C
Signs of heart failure, pulmonary thrombosis, in the absence of hemoptysis.	Oral anticoagulants	IIa	C

Drug 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 involves the continuation of permanent therapy selected in a hospital setting. The prescription 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.

Drug treatment provided at the inpatient level :

The selection of drug treatment in inpatient settings is carried out according to the recommendations presented in Tables 6-9.

Drug treatment provided at the emergency stage with a diagnosis of PH:

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

− Oxygen therapy under control of oxygen saturation below 8 kPa (60 mmHg)

Other types of treatment: not provided.

Surgical intervention provided in an inpatient setting: in the absence of clinical effect from combination therapy, balloon atrioseptostomy (I-C) and/or lung transplantation (I-C) is recommended.

Preventive measures:

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

Prevention of progression of PH: adequate drug 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

	Before starting therapy	Every 3-6 months	3-4 months after the start/correction of therapy	In case of clinical deterioration
Clinical assessment of WHO FC	+	+	+	+
6 minute walk test	+	+	+	+
Caldiopulmonary stress test	+		+	+
BNP/NT-proBNP	+	+	+	+
EchoCG	+	+	+	+
Right heart catheterization	+		+	+

Indicators of treatment effectiveness and 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 the treatment of PH

Prognostic criterion	Favorable prognosis	Poor prognosis
Clinical signs of pancreatic failure	No	Eat
Rate of symptom progression	Slow	Fast
Syncopations	No	Eat
WHO FC	I, II	IV
6 minute walk test	More than 500 m	Less than 300m
Plasma BNP/NT-proBNP levels	Normal or slightly elevated	Significantly increased
EchoCG examination	No pericardial effusion, TAPSE* greater than 2.0 cm	Pericardial effusion, TAPSE less than 1.5 cm
Hemodynamics	DPP less than 8 mm Hg, Cardiac index ≥2.5 l/min/m 2	DPP more than 15 mm Hg, Cardiac index ≤2.0 l/min/m 2

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

Table 13. Determination of the patient’s objective condition

Treatment is assessed as ineffective if the condition of patients with initial FC II - III is determined as “stable and unsatisfactory”, as well as “unstable and worsening”.

For patients with initial FC IV, in the absence of dynamics to FC III or higher, and the condition is defined as “stable and unsatisfactory,” treatment is assessed as ineffective.

Drugs (active ingredients) used in treatment

Hospitalization

Indications for hospitalization

The diagnosis of pulmonary hypertension is established only in inpatient settings.

Emergency hospitalization(up to 2 hours):

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

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

1. Galiè, 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: 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

Kulembaeva A.B. - Candidate of Medical Sciences, Deputy Chief Physician of the State Clinical Hospital at the PCV BSNP in Almaty

Recommendation class Level of evidence Rationale General blood test I IN Brain natriuretic peptide (BNP) I IN Confirming the diagnosis of heart failure (primarily left ventricular dysfunction), clarifying the causes of acute shortness of breath, assessing the condition of patients with heart failure and monitoring treatment ECG I IN

Axis deviation to the right (+150)

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

Functional class of pulmonary hypertension Ventilation-perfusion scintigraphy I WITH Detection of segmental perfusion defects, exclusion of pulmonary embolism, diagnosis of CTEPH Catheterization of the right heart with angiopulmonography I WITH Confirmation of the diagnosis of PH, the degree of damage to the pulmonary vessels, control of the treatment. Spirography I WITH Functional state of the lungs and severity of PAH. CT angiopulmonography I WITH

Visualization of changes in the structure of pulmonary blood flow.

It is possible to diagnose primary pathology (connective tissue diseases, lung diseases, infectious lesions, etc.) Total protein and fractions I C Blood urea I C Signs of primary diseases Blood creatinine and glomerular filtration rate I C Determination of AST, ALT, bilirubin, total, direct I C Signs of primary diseases or complications of PH INR I C Monitoring the intake of indirect anticoagulants (warfarin) Coagulogram I C complications of hemostasis, signs of a systemic inflammatory response during drug treatment D-dimer I C Diagnosis of pulmonary embolism

Immunogram

I C signs of immunodeficiency Tumor markers in the blood I C Symptoms of cancer pathology PCR for tuberculosis from blood I C Symptoms of tuberculosis Antinuclear antibodies I C Rheumatoid factor I C Signs of systemic connective tissue disease Thyroid hormones I C Symptoms of thyroid disease Procalcitonin test I C Diff. diagnosis of infectious and non-infectious disease, early diagnosis of sepsis Sputum analysis for Mycobacterium tuberculosis I C Symptoms of tuberculosis Urine analysis for Mycobacterium tuberculosis I C Symptoms of tuberculosis Emergency echocardiography I C Diagnosis of primary/secondary anatomical and functional pathology of the heart, identification of complications. Ultrasound of the abdominal organs I C Screening for portal hypertension Ultrasound of the thyroid gland I C Etiological diagnosis

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