Chronic pulmonary heart disease recommendations. Pulmonary hypertension in the new recommendations of the European Society of Cardiology (2015) Chronic pulmonary heart disease year of 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 heart vascular system for diseases of the bronchopulmonary apparatus, most authors use 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 the share of chronic pulmonary heart 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 thromboembolism pulmonary artery(TELA)

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.

ABOUT pulmonary hypertension they say if the systolic pressure in the pulmonary artery at rest is more than 30 mmHg, the diastolic pressure is more than 15, and the average 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 internal blood pressure, which leads to an increase in 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 thoradiaphragmatic form: in this form, the leading one 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 cor pulmonale has been proposed, which provides for the division of the cor pulmonale 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 bronchial asthma(especially in 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 nature, shock or collapse, the size of the liver rapidly increases, swelling appears in the legs, ascites, epigastric pulsation, tachycardia (120-140), harsh breathing, weakened vesicular in some places; 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, then 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. On palpation of the abdomen - enlarged liver (one of the reliable, but not early signs pulmonary heart, 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. Swelling is constant, cannot be treated, the pulse is weak, frequent, bullish heart, muffled sounds, systolic murmur in 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 a 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 early symptoms is a bulging of the pulmonary artery trunk on a radiograph. 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.

Of great importance is spirography, which reveals the type and degree of respiratory failure. 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.

Cor pulmonale (CP) is hypertrophy and/or dilatation of the right ventricle (RV) resulting from pulmonary arterial hypertension caused by diseases affecting the function and/or structure of the lungs and not associated with primary pathology of the left heart or congenital heart defects. LS is formed as a result of diseases of the bronchi and lungs, thoracodiaphragmatic lesions or pathology of the pulmonary vessels. The development of chronic pulmonary heart disease (CPP) is most often caused by chronic pulmonary failure (CPF), and the main reason for the formation of CPP is alveolar hypoxia, causing spasm of the pulmonary arterioles.

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

Treatment of CHL is therapy of the underlying disease that causes CHL (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 (LCT), almitrin), as well as correction of right ventricular heart failure (ACE inhibitors, diuretics, aldosterone blockers, angiotesin II receptor antagonists). VCT is the most effective method treatment of CLN and CHL, which can increase the life expectancy of patients.

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

DEFINITION

Pulmonary heart- is hypertrophy and/or dilatation of the right ventricle resulting from pulmonary arterial hypertension caused by diseases affecting the function and/or structure of the lungs and not associated with primary pathology of the left heart or birth defects hearts.

The pulmonary heart (CP) is formed on the basis of pathological changes in the lung itself, violations of the extrapulmonary respiratory mechanisms that provide ventilation of the lung (damage to the respiratory muscles, disruption of the central regulation of breathing, elasticity of the osteochondral formations of the chest or conduction of nerve impulses along n. diaphragmicus, obesity), as well as pulmonary vascular damage.

CLASSIFICATION

In our country, the classification of cor pulmonale proposed by B.E. is most widespread. 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 excluded from the circulation (bilateral extensive pneumonia, status asthmaticus, valve pneumothorax).

Subacute cor pulmonale (CPP) most often results from recurrent thromboembolism of small branches of the pulmonary artery. Leading clinical symptom is increasing shortness of breath with rapidly developing (over 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 part of the lung from the act of breathing (severe bronchial asthma, miliary pulmonary tuberculosis). Common cause PLS are oncological diseases of the lungs, gastrointestinal tract, mammary gland and other localizations, due to lung carcinomatosis, as well as compression of the lung vessels by a growing tumor, followed by thrombosis.

Chronic cor pulmonale (CHP) in 80% of cases occurs when the bronchopulmonary apparatus is damaged (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 CHL is directly related to chronic pulmonary failure (CPF). In clinical practice, a classification of CLN is used based on the presence of shortness of breath. There are 3 degrees of CLN: the appearance of shortness of breath with previously available efforts - I degree, shortness of breath with 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 the pathophysiological mechanisms of the development of pulmonary failure (Table 7.2), which allows the selection of pathogenetically based therapeutic measures.

Classification of the pulmonary heart (according to Votchal B.E., 1964)

Table 7.1.

Character of the current	Compensation status	Predominant pathogenesis	Features of the clinical picture
pulmonary development in several hours, days	Decompensated	Vascular	Massive pulmonary embolism
		Bronchopulmonary	Valvular pneumothorax, pneumomediastinum. Bronchial asthma, prolonged attack. Pneumonia with a large area affected. Exudative pleurisy with massive effusion
Subacute pulmonary development in several	Compensated. Decompensated	Vascular
		Bronchopulmonary	Repeated prolonged attacks of bronchial asthma. Cancerous lymphangitis of the lungs
		Thoradiaphragmatic	Chronic hypoventilation of central and peripheral origin in botulism, poliomyelitis, myasthenia, etc.

End of 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 failure

(Alexandrov O.V., 1986)

Stage of chronic pulmonary failure	Presence of clinical signs	Instrumental diagnostic data	Therapeutic measures
I. Ventilation violations (hidden)	Clinical manifestations are absent or minimally expressed	Absence or presence of only ventilation disorders (obstructive type, restrictive type, mixed type) when assessing respiratory function	Basic therapy for a chronic disease - antibiotics, bronchodilators, stimulation drainage function lung Exercise therapy, electrical stimulation of the diaphragm, aeroionotherapy
P. Ventilation-hemodynamic and ventilation-hemic disorders	Clinical manifestations: shortness of breath, cyanosis	Violations of respiratory function include ECG, echocardiographic and radiographic signs of overload and hypertrophy of the right heart, changes in blood gas composition, as well as erythrocytosis, increased blood viscosity, morphological changes in red blood cells	Supplemented with long-term oxygen therapy (if paO 2<60мм рт.ст.), альмитрином, ЛФК, кардиологическими средствами
III. Metabolic disorders	Clinical manifestations are pronounced	Intensification of the violations described above. Metabolic acidosis. Hypoxemia, hypercapnia	Complemented by extracorporeal treatment methods (erythrocytepheresis, hemosorption, plasmapheresis, extracorporeal membrane oxygenation)

In the presented classification of CLN, the diagnosis of CLN can most likely be made at stages II and III of the process. In stage I CLN (latent), elevations in LBP are detected, usually in response to physical activity and during exacerbation of the disease in the absence of signs of pancreatic hypertrophy. This circumstance allowed us to express the opinion (N.R. Paleev) that 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 LBP. However, in clinical practice, direct measurement of PAP in this group of patients is not sufficiently justified.

Over time, decompensation of CHL may develop. 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 that develops as a result of damage to the left ventricle (LV) or both ventricles. The presence of left ventricular HF in patients with CHL is most often due to two reasons: 1) CHL in people over 50 years of age is often combined with coronary heart disease, 2) systemic arterial hypoxemia in patients with CHL leads to degenerative processes in the LV myocardium, to its moderate hypertrophy and contractile insufficiency.

The main cause of the development of chronic pulmonary heart disease is chronic obstructive pulmonary disease.

PATHOGENESIS

The development of chronic drugs is based on the gradual formation of pulmonary arterial hypertension, caused by several pathogenetic mechanisms. The main cause of PH in patients with bronchopulmonary and thoracodiaphragmatic forms of CHL 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-Lillestrand 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 helps to reduce the sensitivity of the respiratory center to CO 2, which further reduces ventilation and promotes 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). Dysfunction of the pulmonary vascular endothelium in patients with COPD is associated with hypoxemia, inflammation, and exposure to cigarette smoke.

In patients with CLS, structural changes in the vascular bed occur - 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 internal diameter of the vessels. In patients with COPD, due to emphysema, there is a reduction in the capillary bed and compression of the pulmonary vessels.

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

Patients with severe obesity develop Pickwick's syndrome (named after the work of Charles Dickens), 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 restriction by adipose tissue with dysfunction (fatigue) respiratory muscles.

Increased blood pressure in the pulmonary artery may initially contribute to an increase in the volume of perfusion of the pulmonary capillaries, but over time, hypertrophy of the RV myocardium develops, followed by its contractile failure. Pressure indicators in the pulmonary circulation are presented in table. 7.3.

Table 7.3

Pulmonary hemodynamic parameters

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

CLINIC

The clinical picture consists of manifestations of the underlying disease, leading to the development of CLS 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 clinical picture of CHL is inextricably linked with the manifestation of CHL itself.

A characteristic complaint of patients is shortness of breath. Initially, when physical activity(stage I CLN), and then at rest (stage III CLN). It is expiratory or mixed in nature. A long course (years) of COPD dulls the patient’s attention and forces him to consult a doctor when shortness of breath appears during light physical exertion or at rest, that is, already in stage II-III chronic pulmonary disease, when the presence of chronic pulmonary disease is indisputable.

Unlike shortness of breath associated with left ventricular failure and venous stagnation of blood in the lungs, shortness of breath with pulmonary hypertension does not increase in the horizontal position of the patient and does not

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

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

Half of patients with CLS experience pain in the heart area, often of an uncertain nature, without irradiation, usually not associated with physical activity and not relieved by nitroglycerin. The most common view on the mechanism of pain is relative coronary insufficiency, caused by 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 cavity of the pancreas, myocardial hypoxia against the background of general arterial hypoxemia (“blue angina”) 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 cor pulmonale, swelling may appear in the legs, which first appears most often during an exacerbation of bronchopulmonary disease and is 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, an increase in the volume of the abdomen due to the formation of ascites is noted.

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

In patients with CLN and CLS, encephalopathy may develop 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. Fainting rarely occurs during exercise as a result of severe hypoxia.

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

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

Blood pressure in patients with compensated CHL is often increased, and in decompensated patients it is decreased.

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

The clinical picture of chronic pulmonary heart disease is determined by the severity of pulmonary pathology, as well as pulmonary and right ventricular heart failure.

INSTRUMENTAL DIAGNOSTICS

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

Electrocardiographic diagnosis of CHL comes down to identifying RV hypertrophy. The main (“direct”) ECG criteria for pancreatic 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 >1; 8) complete blockade of the right bundle branch with RV1>15 mm; 9) incomplete blockade of the right bundle branch with RV1>10 mm; 10) negative TVl and decreased STVl,V2 with RVl>5 mm and the absence of coronary insufficiency. If there are 2 or more “direct” ECG signs, the diagnosis of pancreatic 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 appearance in leads V5, V6 QRS complex type RS; SV5-6 is deep, and RV1-2 is of 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) electrical axis of the heart type

SI-SII-SIII; 6) complete or incomplete blockade of the right bundle branch; 7) electrocardiographic signs of hypertrophy of the right atrium (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 CLS:

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

2. 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 pulmonary 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 precordial leads

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

Electrocardiographic criteria for RV hypertrophy are not specific enough. They are less clear than with LV hypertrophy and can lead to false-positive and false-negative diagnoses. Normal ECG does not exclude the presence of CHL, especially in patients with COPD, therefore ECG changes must 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 pulmonary disease. Ultrasound diagnostics LS is based on identifying signs of damage to the pancreas myocardium, which are given below.

1. Change in the size of the right ventricle, which is assessed in two positions: in the parasternal long-axis position (normally less than 30 mm) and in the apical four-chamber position. To detect pancreatic dilatation, 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 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 by more than 0.6 indicates significant dilatation of the pancreas, and if it becomes equal to or greater than 1.0, then a conclusion is made about 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 pancreatic hypertrophy, the thickness of its anterior wall, measured in diastole in the subcostal four-chamber position in B- or M-mode, exceeds 5 mm. In patients with CLS, as a rule, not only the anterior wall of the pancreas hypertrophies, but also the interventricular septum.

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

4. RV diastolic function is assessed using transtricuspid diastolic flow in pulse-mode

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

5. A decrease in the 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 is normally at least 50%.

These changes have different severity depending on the severity of drug development. Thus, 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 patients with LS. The degree of their severity is most significant in acute and subacute LS, as well as in patients with primary pulmonary hypertension. CPS is characterized by a moderate increase in systolic pressure in the pulmonary artery, which rarely reaches 50 mmHg. Assessment of the pulmonary trunk and flow in the outflow tract of the pancreas is carried out from the left parasternal and subcostal short-axis approach. In patients with pulmonary pathology, due to the limited ultrasound window, the subcostal position may be the only possible access for visualizing the outflow tract of the pancreas. Using pulsed wave Doppler, the mean pulmonary artery pressure (Ppa) can be measured, for which the formula proposed by A. Kitabatake et al. (1983): Log10(Pra) = - 2.8 (AT/ET) + 2.4, where AT is the time of acceleration of 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 invasive examination, and the possibility of obtaining a reliable signal from the pulmonary valve exceeds 90%.

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

whom mapping. To calculate pulmonary artery pressure, 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 COPD patients. There are qualitative signs of pulmonary hypertension:

1. In PH, the pattern of movement of the posterior leaflet of the pulmonary artery valve changes, which is determined in M-mode: a characteristic indicator of PH is the presence of a mid-systolic wave due to partial closure 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 along its short axis (D-shaped left ventricle). At high degree The LH of the IVS becomes like a wall of the RV and moves paradoxically in diastole towards the left ventricle. When the pressure in the pulmonary artery and right ventricle becomes more than 80 mm Hg, the left ventricle decreases in volume, is compressed by the dilated right ventricle and takes on a crescent shape.

3. Possible regurgitation on the pulmonary valve (normally, first-degree regurgitation is possible in young people). With a continuous wave Doppler study, it is possible to measure the velocity of pulmonary regurgitation with further calculation of the value of the end-diastolic pressure gradient of the PA-RV.

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

However, in patients with COPD, the existing pulmonary emphysema often makes it difficult to clearly visualize the structures of the heart and narrows the “window” of echocardiography, 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 more preferable method for accurate measurements and direct visual assessment of pancreatic structures, which is due to the higher resolution of the transesophageal sensor and the stability of the ultrasound window, and is of particular importance in pulmonary emphysema and pneumosclerosis.

Catheterization of the right heart and pulmonary artery

Catheterization of the right heart and pulmonary artery is the “gold standard” method for diagnosing PH. This procedure allows you to directly measure right atrium and RV pressure, pulmonary artery pressure, calculate cardiac output and pulmonary vascular resistance, and 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 right ventricular ejection fraction (RVEF). RVEF is considered abnormal if it is below 40-45%, but RVEF itself is not a good indicator of right ventricular function. It allows you to assess the systolic function of the right ventricle, which is highly dependent on afterload, decreasing as the latter increases. Therefore, a decrease in RVF is recorded in many patients with COPD, not being an indicator of true right ventricular dysfunction.

Magnetic resonance imaging (MRI)

MRI is a promising modality for assessing pulmonary hypertension and changes in right ventricular structure and function. A right pulmonary artery diameter measured on MRI 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 chronic pulmonary disease (as the cause of chronic pulmonary disease) requires a special study of the function of external respiration. The doctor is tasked with clarifying the type of ventilation failure: obstructive (impaired passage of air through the bronchi) or restrictive (reduced gas exchange area). In the first case, examples include chronic obstructive bronchitis, bronchial asthma, and in the second case, pneumosclerosis, lung resection, etc.

TREATMENT

CHL occurs most often after the appearance of CLN. Therapeutic measures are complex and 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 chronic pulmonary hypertension and pulmonary hypertension;

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

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

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

The most common cause of CLN is broncho-obstructive syndrome, the cause of which is the contraction of bronchial smooth muscles, the accumulation of viscous inflammatory secretion, and swelling 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 inhalation using a nebulizer or personal inhaler. It is possible to use methylxanthines (aminophylline and prolonged theophyllines (theolong, theotard, etc.)). Therapy with expectorants is very individual and requires various combinations and selection of herbal products (coltsfoot, wild rosemary, thyme, etc.) and chemical production (acetylcysteine, ambroxol, etc.).

If necessary, exercise therapy and postural pulmonary drainage are prescribed. Breathing shown positive pressure on exhalation (no more than 20 cm of water column) using 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 speed of air flow inside the bronchus (which has a bronchodilator effect) and increases the pressure inside the bronchi relative to the surrounding lung tissue.

Extrapulmonary mechanisms for the development of CLN 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.

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

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

In patients with hypoventilation associated with reduced activity of the respiratory center, medications that increase central inspiratory activity - respiratory stimulants - can be used as auxiliary methods of therapy. They should be used in cases of moderate respiratory depression that do not require the use of O2 or mechanical ventilation (sleep apnea syndrome, obesity-hypoventilation syndrome), or when oxygen therapy is not possible. A few drugs that increase arterial oxygenation include nicetamide, acetozalamide, doxapram and medroxyprogesterone, but all of these drugs have a large number of side effects when used over a long period of time and therefore can only be used for a short time, for example during an exacerbation of the disease.

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

nistome of peripheral chemoreceptors of the carotid ganglion, stimulation of which leads to increased hypoxic vasoconstriction in poorly ventilated regions of the lungs with improved 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 pa0 2 (by 5-12 mm Hg) and a decrease in paCO 2 (by 3-7 mm Hg) 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 prescribing 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 drugs are calcium channel antagonists and nitrates. The currently recommended calcium antagonists include nifedipine and diltiazem. The choice in favor of one of them depends on the initial heart rate. In patients with relative bradycardia, nifedipine should be recommended; in patients with relative tachycardia, diltiazem should be recommended. The daily doses of these drugs, which have proven effectiveness, 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 drugs.

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

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

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

Synthetic prostacyclin and its analogues. Prostacyclin is a powerful endogenous vasodilator with antiaggregation, antiproliferative and cytoprotective effects that are aimed at preventing pulmonary vascular remodeling (reducing damage to endothelial cells and hypercoagulation). 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 increased 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 an intravenous form of prostacyclin (prostaglandin I 2). Favorable results were obtained in patients with the vascular form of HL - with primary PH in systemic diseases connective tissue. The drug increases cardiac output and reduces pulmonary vascular resistance, and when long-term use improves the quality of life of patients with drugs, increasing tolerance to physical activity. The optimal dose for most patients is 20-40 ng/kg/min. An analogue of epoprostenol, treprostinil, is also used.

Currently, oral forms of a prostacyclin analogue have been developed (beraprost, iloprost) and clinical trials are being conducted in the treatment of patients with the vascular form of LS that 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 drugs, only prostaglandin E 1 (vasaprostan) is currently available, which is prescribed intravenously by drip at a rapid rate.

growth 5-30 ng/kg/min. Course treatment with the drug is carried out in 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 served as a rationale for the use of endothelin receptor antagonists. The effectiveness of two drugs of this class (bosentan and sitaxentan) in the treatment of patients with CHL that 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, causing a decrease in pulmonary vascular resistance and right ventricular overload. To date, there is data on the effectiveness of sildenafil in patients with drugs 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 drugs. Its use is recommended when other drug therapy is ineffective.

Long-term oxygen therapy

In patients with bronchopulmonary and thoracodiaphragmatic forms of CHL, 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 constant, long-term, and usually carried out at home, which is why this form of therapy is called long-term oxygen therapy (LOT). The goal of DCT is to correct hypoxemia to achieve paO 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 to only a slight increase in Sa0 2 and oxygen content in arterial blood, but may be accompanied by CO 2 retention, especially during sleep, which has negative consequences.

effects on the function of the heart, brain and respiratory muscles. Therefore, VCT is not indicated for patients with moderate hypoxemia. Indications for DCT: 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 O2 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. It is recommended to conduct VCT at least 15 hours a day (15-19 hours/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 to perform VCT. The most commonly used are concentrators (permeators), which release oxygen from the air by removing nitrogen. VCT increases the life expectancy of patients with CLN and CHL 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 CHL, therefore the treatment of patients with CHL is primarily the task of a pulmonologist.

Long-term oxygen therapy is the most effective method of treating chronic pulmonary insufficiency and congestive heart disease, 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 must be provided for a long time, on an ongoing basis at home.

NO inhalation therapy

Inhalation therapy N0, which has an effect similar to endothelium relaxing factor, has positive effect in patients with CHL. 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 intracellular calcium levels. Inhalation NO 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 a course of use of NO in patients with CHL, a decrease in pressure in the pulmonary artery and an increase in the partial pressure of oxygen in the blood are observed. In addition to its hemodynamic effects, NO helps prevent and reverse pulmonary vascular and pancreatic remodeling. 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 inhaled NO 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 method (continuous positive airway pressure- CPAP) is used as a treatment method for chronic respiratory failure and chronic pulmonary hypertension in patients with obstructive sleep apnea syndrome, preventing the development of airway collapse. The proven effects of CPAP are the prevention and straightening of atelectasis, increasing lung volumes, reducing ventilation-perfusion imbalance, increasing oxygenation, lung compliance, and redistributing fluid in the 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 CHL with edematous syndrome, therapy with diuretics, including antagonists, is used

aldosterone (aldactone). Diuretics should be prescribed cautiously, with small doses, since with the development of RV failure, cardiac output is more dependent on preload, and, therefore, 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. Other serious side effect 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 rates.

Angiotensin-converting enzyme inhibitors

In the treatment of patients with decompensated cor pulmonale in last years Angiotensin-converting enzyme inhibitors (ACEIs) came into first place. ACEI therapy in patients with CHL leads to a decrease in pulmonary hypertension and an increase in cardiac output. In order to select effective therapy for CHL in patients with COPD, it is recommended to determine the polymorphism of the ACE gene, because Only patients with ACE II and ID gene subtypes exhibit a pronounced positive hemodynamic effect of ACE inhibitors. It is recommended to use ACE inhibitors in minimal therapeutic doses. 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 an increase in life expectancy in patients with heart failure.

Angiotensin II receptor antagonists

In recent years, data have been obtained on successful application of this group of drugs in the treatment of CHL in patients with COPD, which was manifested by an improvement in hemodynamics and gas exchange. The most indicated use of these drugs is in patients with CHL who are intolerant 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,

use atrial septostomy, i.e. creation of a small perforation in the interatrial septum. Creating a right-to-left shunt allows one to reduce mean pressure in the right atrium, unload the right ventricle, and increase left ventricular preload and cardiac output. Atrial septostomy is indicated when all types of drug treatment for right ventricular heart failure are ineffective, especially in combination with frequent syncope, or as a preparatory step before lung transplantation. As a result of the intervention, a decrease in syncope and an increase in exercise tolerance are observed, 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 transplantation

Since the late 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 failure. In patients with CLN and LS, transplantation of one or both lungs or the heart-lung complex is performed. It was shown that 3 and 5-year survival rates after transplantations of one or both lungs and the heart-lung complex in patients with LS were 55 and 45%, respectively. Most centers prefer to perform bilateral lung transplantation due to fewer postoperative complications.

Given guidelines, dedicated to the clinic, 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 (RV) hypertrophy, 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 its implementation in practice modern methods diagnostics and 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, according to the primary causes, it is customary to distinguish the following types of CHL:

1.Bronchopulmonary:

The reason 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 quite often combine, 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. Moreover, its very presence has absolutely different meaning with various 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 pulmonary heart disease, but its significance should not be absolute.

Often this pathology causes 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 stasis in big circle blood 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 cor pulmonale 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 big role is attributed to 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, it 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 the 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 different ages 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 instrumental research 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 in the clinic, which are safer than the drugs we used before.

When assessing airway patency in healthy people after taking atenolol, a deterioration in the reaction to salbutamol and minor changes were established when taking more modern drugs. Although, unfortunately, such studies have not been conducted with patients, the categorical ban on the use of beta-blockers in patients with COPD must 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, a combination of different drugs has been proven to be highly effective, affecting various stages of pathogenesis and mutually reinforcing the effect. medicines. 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

– 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.

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 bronchial asthma, valvular pneumothorax, 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. On 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 the acid-base balance occurs, which may initially be compensated, but later decompensation of the 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

By slew rate clinical manifestations There are several variants of the course of cor pulmonale: acute (develops over a few 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; decreased blood pressure, up to the development of collapse, cyanosis 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 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 manifests itself with sudden moderate painful sensations, shortness of breath and tachycardia, brief fainting, hemoptysis, 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, or 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 during exacerbation inflammatory process in the lungs. IN late stage swelling increases, liver enlargement progresses (hepatomegaly), neurological disorders(dizziness, headaches, apathy, drowsiness) 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 start complex therapy can significantly improve labor prognosis and increase life expectancy.

Prevention of cor pulmonale requires warning, timely and effective treatment diseases leading to it. 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 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 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 CT scan

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: year 2014

Protocol users: cardiologists (adults, children, including interventional), cardiac surgeons, doctors general practice, 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 diseases thyroid gland

5.4 Other: tumor obstruction, fibrosing mediastinitis, chronic kidney 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 analysis blood 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 factions

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 organs abdominal cavity

21. Ultrasound of the thyroid gland

22. Ventilation-perfusion scintigraphy

Diagnostic measures carried out at the emergency stage emergency care:

2. Pulse oximetry

Diagnostic criteria

Complaints:
- fatigue
- weakness
- anginal pain in 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 inhibitor serotonin reuptake 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 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 primary cause 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 a vasoreactive test is a mandatory study to establish a 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

A drug	Method of administration	Half-lifeleniya (T ½)	General dose	Initial dose	Duration of administration
Epoprostenol	intravenous	3 min	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 minutes
Nitric oxide	inhalation	15-30s	10-20 ml/min		5 minutes
Iloprost	inhalation	3 min	2.5-5 µg/kg	2.5 µg	2 minutes

Chest X-ray

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 analysis blood 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 medicines	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
		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

**Harmonized 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 :

Selection of drug treatment in inpatient conditions 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 actions:

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 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 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 the 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. 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 analysis 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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