State educational institution of higher professional education "Moscow State Medical and Dental University of Roszdrav"

Faculty of Medicine

Martynov A.I., Maychuk E.Yu., Panchenkova L.A., Khamidova H.A.,

Yurkova T.E., Pak L.S., Zavyalova A.I.

Chronic cor pulmonale

Educational and methodological manual for practical training in hospital therapy

Moscow 2012

Reviewers: Doctor of Medical Sciences Professor of the Department of Emergency Conditions at the Clinic of Internal Medicine of the Faculty of Postgraduate Education of the First Moscow State Medical University named after N.M. Sechenova Shilov A.M.

Doctor of Medical Sciences Professor of the Department of Hospital Therapy No. 2 of the State Educational Institution of Higher Professional Education MGMSU, Makoeva L.D.

Maychuk E.Yu., Martynov A.I., Panchenkova L.A., Khamidova H.A., Yurkova T.E., Pak L.S., Zavyalova A.I. Textbook for medical students. M.: MGMSU, 2012, 25 p.

The textbook describes in detail modern ideas about the classification, clinical picture, principles of diagnosis and treatment of chronic pulmonary heart disease. The manual contains a work plan for a practical lesson, questions to prepare for the lesson, an algorithm for substantiating a clinical diagnosis; final test lessons are included, intended for students to independently assess their knowledge, as well as situational tasks on the topic.

This training manual has been prepared in accordance with the working curriculum for the discipline “Hospital Therapy”, approved in 2008 at the Moscow State University of Medicine and Dentistry on the basis of sample curricula of the Ministry of Health and Social Development of the Russian Federation and the State educational standard for higher professional education in the specialty “060101-General Medicine” "

The manual is intended for teachers and students of medical universities, as well as clinical residents and interns.

Department of Hospital Therapy No. 1

(Head of the department – ​​Doctor of Medical Sciences, Professor Maychuk E.Yu.)

Authors: professor, doctor of medical sciences Maychuk E.Yu., academician, doctor of medical sciences Martynov A.I., professor, doctor of medical sciences Panchenkova L.A., assistant, Ph.D. Khamidova Kh.A., assistant, Ph.D. Yurkova T.E., professor, doctor of medical sciences Pak L.S., associate professor, candidate of medical sciences, Zavyalova A.I.

MGMSU, 2012

Department of Hospital Therapy No. 1, 2012

Definition and theoretical basis of topic 4

Motivational characteristics of topic 14

Stages of diagnostic search 15

Clinical tasks 18

Test tasks 23

Literature 28

DEFINITION AND THEORETICAL ISSUES OF THE TOPIC

Chronic cor pulmonale (CHP)- hypertrophy and/or dilatation of the right ventricle in combination with pulmonary hypertension, occurring against the background of various diseases that impair the structure and/or function of the lungs, with the exception of cases where changes in the lungs themselves are the result of primary damage to the left side of the heart or congenital heart defects and large blood vessels.

ETIOLOGY

According to the etiological classification developed by the WHO committee (1961), there are 3 groups of pathological processes leading to the formation of CHL:

diseases leading to primary disruption of the passage of air in the bronchi and alveoli (chronic obstructive pulmonary disease, bronchial asthma, emphysema, bronchiectasis, pulmonary tuberculosis, silicosis, pulmonary fibrosis, pulmonary granulomatosis of various etiologies, pulmonary resection and others);

diseases leading to restriction of chest movement (kyphoscoliosis, obesity, pleural fibrosis, ossification of the costal joints, consequences of thoracoplasty, myasthenia gravis, etc.);

diseases accompanied by damage to the pulmonary vessels (primary pulmonary hypertension, vasculitis in systemic diseases, recurrent thromboembolism of the pulmonary arteries).

The main cause is chronic obstructive pulmonary disease (COPD), which accounts for 70 - 80% of all cases of chronic pulmonary disease.

CLASSIFICATION OF CHRONIC PULMONARY HEART:

By degree of compensation:

compensated;

decompensated.

By origin:

vascular genesis;

bronchopulmonary origin;

thoracodiaphragmatic genesis.

PATHOGENESIS OF CHRONIC PULMONARY HEART

There are 3 stages in the development of CHL:

precapillary hypertension in the pulmonary circulation;

right ventricular hypertrophy;

right ventricular heart failure.

The pathogenesis of CLS is based on the development of pulmonary hypertension.

Main pathogenetic mechanisms:

Lung disease, damage to the chest, spine, diaphragm. Violations of ventilation and respiratory mechanics. Impaired bronchial conduction (obstruction). Reduced respiratory surface (restriction).

Generalized hypoxic vasoconstriction due to alveolar hypoventilation (generalized Euler-Lillestrand reflex), i.e. a generalized increase in the tone of small pulmonary vessels occurs and pulmonary arterial hypertension develops.

Hypertensive influence of humoral factors (leukotrienes, PgF 2 α, thromboxane, serotonin, lactic acid).

Reduction of the vascular bed, sclerotic and atherosclerotic changes in the branches of the pulmonary artery and pulmonary trunk.

Increased blood viscosity due to erythrocytosis, which develops in response to chronic hypoxemia.

Development of bronchopulmonary anastomoses.

Increased intraalveolar pressure in obstructive bronchitis.

In the early stages of the formation of CHL, compensatory-adaptive reactions predominate, however, a prolonged increase in pressure in the pulmonary artery leads to hypertrophy over time, with repeated exacerbations of bronchopulmonary infection, increasing obstruction - to dilatation and failure of the right ventricle.

CLINICAL PICTURE

The clinical picture includes symptoms:

the underlying disease that led to the development of CHL;

respiratory failure;

cardiac (right ventricular) failure;

Complaints

Shortness of breath, worsening with physical activity. Unlike patients with left ventricular failure, with decompensated cor pulmonale, body position does not affect the degree of shortness of breath - patients can freely lie on their back or side. Orthopnea is atypical for them, since there is no congestion in the lungs, there is no “obstruction” of the small circle, as with failure of the left heart. Dyspnea for a long time is caused mainly by respiratory failure, it is not affected by the use of cardiac glycosides, it decreases with the use of bronchodilators and oxygen. The severity of shortness of breath (tachypnea) is often not related to the degree of arterial hypoxemia, and therefore has an organic diagnostic value.

Persistent tachycardia.

Cardialgia, the development of which is associated with metabolic disorders (hypoxia, infectious-toxic effects), insufficient development of collaterals, reflex narrowing of the right coronary artery (pulmonary-coronary reflex), decreased filling of the coronary arteries with an increase in end-diastolic pressure in the cavity of the right ventricle.

Arrhythmias are more common during exacerbation of COPD, in the presence of decompensation of the cor pulmonale in patients suffering from concomitant coronary heart disease, arterial hypertension, and obesity.

Neurological symptoms (cranialgia, dizziness, drowsiness, darkening and double vision, speech impairment, poor concentration of thoughts, loss of consciousness) are associated with cerebral circulatory disorders.

Objective signs

Diffuse “warm” cyanosis (the distal parts of the extremities are warm due to the vasodilating effect of carbon dioxide accumulating in the blood);

Swelling of the neck veins due to obstructed blood outflow to the right atrium (the neck veins swell only during exhalation, especially in patients with obstructive pulmonary lesions; when heart failure occurs, they remain swollen during inhalation).

Thickening of the terminal phalanges (“drumsticks”) and nails (“watch glasses”).

Edema of the lower extremities, as a rule, is less pronounced and does not reach the same degree as in primary heart diseases.

Enlarged liver, ascites, positive venous pulse, positive Plesch's sign (hepatojugular symptom - when you press on the edge of the liver, swelling of the veins of the neck becomes obvious).

Systolic precordial and epigastric pulsation (due to right ventricular hypertrophy).

Percussion determines the expansion of absolute and relative cardiac dullness of the right border of the heart; the percussion sound above the manubrium of the sternum has a tympanic tint, and above the xiphoid process it becomes dull-tympanic or completely deaf.

Deafness of heart sounds.

The accent of the second tone is over the pulmonary artery (with an increase in pressure in it by more than 2 times).

Increased systolic murmur over the xiphoid process or to the left of the sternum with the development of relative valve insufficiency.

DIAGNOSIS OF CHRONIC PULMONARY HEART

Laboratory data

In a clinical blood test, erythrocytosis, high hematocrit, and slow ESR are determined in patients with CHL.

In a biochemical blood test, with the development of decompensation of the right ventricular type, increases in residual nitrogen, bilirubin, hypoalbuminemia, and hyperglobulinemia are possible.

X-ray signs

Normal or enlarged heart shadow in lateral projection

Relative increase in the RV arch in the left (second) oblique position.

Dilation of the common trunk of the pulmonary artery in the right (first) oblique position.

Dilation of the main branch of the pulmonary artery more than 15 mm in the lateral projection.

An increase in the difference between the width of the shadow of the main segmental and subsegmental branches of the pulmonary artery.

Kerley's lines are horizontal narrow shadows over the costophrenic sinus. It is believed that they arise due to the expansion of lymphatic vessels at the thickening of the interlobular fissures. In the presence of Kerley's line, the pulmonary capillary pressure exceeds 20 mmHg. Art. (normal – 5 – 7 mm Hg).

Electrocardiographic signs

Signs of hypertrophy and overload of the right heart are observed.

Direct signs of hypertrophy:

R wave in V1 more than 7 mm;

R/S ratio in V1 is more than 1;

own deviation V1 - 0.03 – 0.05 s;

qR shape in V1;

incomplete blockade of the right bundle branch, if R is more than 10 mm;

complete blockade of the right bundle branch, if R is more than 15 mm;

picture of right ventricular overload in V1 – V2.

Indirect signs of hypertrophy:

chest leads:

The R wave in V5 is less than 5 mm;

The S wave in V5 is more than 7 mm;

The R/S ratio in V5 is less than 1;

The S wave in V1 is less than 2 mm;

Complete right bundle branch block if R is less than 15 mm;

Incomplete right bundle branch block, if R is less than 10 mm;

standard leads:

P-pulmonale in standard ECG leads II and III;

EOS deviation to the right;

type S1, S2, S3.

Echocardiographic signs

Hypertrophy of the right ventricle (the thickness of its anterior wall exceeds 0.5 cm).

Dilatation of the right heart (the end-diastolic size of the right ventricle is more than 2.5 cm).

Paradoxical movement of the interventricular septum in diastole towards the left.

“D”-shaped right ventricle.

Tricuspid regurgitation.

Systolic pressure in the pulmonary artery, determined by echocardiography, is normally 26–30 mm Hg. There are degrees of pulmonary hypertension:

I – 31 – 50 mmHg;

II – 51 – 75 mmHg;

III – 75 mm Hg. Art. and higher.

TREATMENT OF CHRONIC PULMONARY HEART

Basic principles of treatment of patients with CHL:

Prevention and treatment of underlying lung diseases.

Drug reduction of pulmonary hypertension. However, a sharp drug reduction of pulmonary hypertension can lead to a deterioration in gas exchange function of the lungs and an increase in venous blood shunt, since moderate pulmonary hypertension in patients with chronic pulmonary hypertension is a compensatory mechanism of ventilation-perfusion dysfunction.

Treatment of right ventricular failure.

The main goal of treatment of patients with CHL is to improve oxygen transport to reduce the level of hypoxemia and improve the contractile ability of the myocardium of the right heart, which is achieved by reducing the resistance and vasoconstriction of the pulmonary vessels.

Treatment and prevention underlying disease, for example, anticholinergics, bronchodilators - anticholinergic drugs (Atrovent, Berodual), selective β2 - antagonists (Berotec, Salbutomol), methylxanthines, mucolytics. In case of exacerbation of the process - antibacterial drugs, if necessary - corticosteroids.

At all stages of the course of CLS pathogenetic method of treatment Long-term oxygen therapy is used - inhalation of air enriched with oxygen (30 - 40% oxygen) through a nasal catheter. The oxygen flow rate is 2 - 3 liters per minute at rest and 5 liters per minute during exercise. Criteria for prescribing long-term oxygen therapy: PAO2 less than 55 mm Hg. and oxygen saturation (erythrocyte oxygen saturation, SAO2) less than 90%. Long-term oxygenation should be prescribed as early as possible in order to correct disturbances in blood gas composition, reduce arterial hypoxemia and prevent hemodynamic disturbances in the pulmonary circulation, which allows stopping the progression of pulmonary hypertension and remodeling of pulmonary vessels, increasing survival and improving the quality of life of patients.

Calcium antagonists cause dilatation of blood vessels in the pulmonary and systemic circulation, and therefore are classified as direct vasodilators. Tactics for prescribing calcium antagonists: treatment begins with small doses of the drug, gradually increasing the daily dose, bringing it to the maximum tolerated; nifedipine is prescribed - 20 - 40 mg/day, adalat - 30 mg/day, diltiazem from 30 - 60 mg/day to 120 - 180 mg/day, isradin - 2.5 - 5.0 mg/day, verapamil - from 80 to 120 – 240 mg/day, etc. The course of therapy ranges from 3 – 4 weeks to 3 – 12 months. The dose of the drug is selected taking into account the level of pressure in the pulmonary artery and a differentiated approach to side effects that occur when prescribing calcium antagonists. An immediate effect should not be expected when prescribing calcium antagonists.

Nitrates cause dilatation of the arteries of the pulmonary circulation; reduce the afterload on the right ventricle due to cardiodilation, reduce the afterload on the right ventricle due to a decrease in hypoxic vasoconstriction of the PA; reduce pressure in the left atrium, reduce post-capillary pulmonary hypertension by reducing end-diastolic pressure in the left ventricle. Average therapeutic dose: nitrosorbide – 20 mg 2 times a day.

ACE inhibitors (ACEIs) significantly improve survival and life prognosis in patients with congestive heart failure, including in patients with chronic heart failure, since the result of the use of ACE inhibitors is a decrease in arterial and venous tone, a decrease in venous return of blood to the heart, a decrease in diastolic pressure in the pulmonary artery and right atrium, increase in cardiac output. Captopril (Capoten) is prescribed in a daily dose of 75–100 mg, ramipril – 2.5–5 mg/day, etc., the dose depends on the initial blood pressure level. If side effects or ACEI intolerance develop, AT II receptor antagonists (losartan, valsartan, etc.) can be prescribed.

Prostaglandins– a group of drugs that can successfully reduce pressure in the pulmonary artery with minimal impact on the systemic blood flow. A limitation to their use is the duration of intravenous administration, since prostaglandin E1 has a short half-life. For long-term infusion, a special portable pump is used, connected to a Hickman catheter, which is installed in the jugular or subclavian vein. The dose of the drug varies from 5 ng/kg per minute to 100 ng/kg per minute.

Nitric oxide acts similarly to endothelium relaxing factor. With a course of inhaled use of NO in patients with CHL, a decrease in pressure in the pulmonary artery, an increase in the partial pressure of oxygen in the blood, and a decrease in pulmonary vascular resistance are observed. However, we cannot forget about the toxic effect of NO on the human body, which requires adherence to a strict dosage regimen.

Prostacyclin(or its analogue - iloprost) is used as a vasodilator.

Diuretics prescribed when edema appears, combining them with limited fluid and salt intake (furosemide, Lasix, potassium-sparing diuretics - triamterene, combination drugs). It should be borne in mind that diuretics can cause dryness of the bronchial mucosa, reduce the mucosal index of the lungs and worsen the rheological properties of the blood. At the initial stages of the development of CLS with fluid retention in the body due to hyperaldosteronism, caused by the stimulating effect of hypercapnia on the zona glomerulosa of the adrenal cortex, it is advisable to administer aldosterone antagonists in isolation (veroshpiron - 50 - 100 in the morning daily or every other day).

Question about the feasibility of using cardiac glycosides in the treatment of patients with CHL remains controversial. It is believed that cardiac glycosides, having a positive inotropic effect, lead to more complete emptying of the ventricles, increasing cardiac output. However, in this category of patients without concomitant heart pathology, cardiac glycosides do not increase hemodynamic parameters. When taking cardiac glycosides, patients with CLS more often experience symptoms of digitalis intoxication.

An important component of treatment is the correction of hemorheological disorders.

Use anticoagulants for the purpose of treatment and prevention of thrombosis, thromboembolic complications. In hospital settings, heparin is mainly used in a daily dose of 5000 - 20,000 units subcutaneously under the control of laboratory parameters (blood clotting time, activated partial thromboplastin time). Among oral anticoagulants, preference is given to warfarin, which is prescribed in an individually selected dose under the control of INR.

Antiplatelet agents (acetylsalicylic acid, chimes) and hirudotherapy are also used.

Preventive measures should be aimed at observing the work and rest regime. It is necessary to completely stop smoking (including passive smoking), avoid hypothermia if possible, and prevent acute respiratory viral infections.

FORECAST

The duration of pulmonary hypertension (from its onset to death) is approximately 8–10 years or more. 30 - 37% of patients with circulatory failure and 12.6% of all patients with cardiovascular diseases die from decompensation of CHL.

MOTIVATIONAL CHARACTERISTICS OF THE TOPIC

Knowledge of the topic is necessary to develop students’ skills and abilities in the diagnosis and treatment of chronic pulmonary heart disease. To study the topic, it is necessary to repeat sections of the course of normal anatomy and physiology of the respiratory system, the course of pathology of the respiratory system, propaedeutics of internal diseases, and clinical pharmacology.

Purpose of the lesson: study the etiology, pathogenesis, clinical manifestations, diagnostic methods, approaches to the treatment of chronic pulmonary heart disease.

The student must know:

Questions to prepare for the lesson:

A) Definition of the concept “Chronic pulmonary heart”.

B) Etiological factors of chronic pulmonary heart disease.

C) The main pathophysiological mechanisms of the development of chronic pulmonary heart disease.

D) Classification of chronic pulmonary heart disease.

D) Laboratory and instrumental diagnostics of chronic pulmonary heart disease.

E) Modern approaches to the treatment of chronic pulmonary heart disease

  PH is a serious chronic disease with an unfavorable prognosis; it is advisable to recommend rational daily activity to patients. For all patients, general recommendations are important, compliance with which can reduce the risk of possible worsening of the disease.
  Epidural anesthesia is recommended as the method of choice for surgical interventions in patients with PH.

  Comments. Elective surgery in patients with PAH is considered to be high risk. Currently, the most preferred method of anesthesia is epidural anesthesia. In patients receiving oral PAH-specific therapy, during the preparation and conduct of surgical interventions, it is possible to consider the issue of inhaled and/or intravenous administration of drugs.

3.2 Drug treatment.

  There are two sections of drug therapy in patients with PH: maintenance therapy (oral anticoagulants and antiplatelet agents, diuretics, cardiac glycosides, oxygen therapy) and specific therapy, including calcium antagonists, prostanoids, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors.

3.2.1 Maintenance therapy.

  Anticoagulants and antiplatelet agents.
  It is recommended to prescribe warfarin to patients with IPH, inherited PAH, associated PAH while taking anorectics.

  Comments. The target INR level for PAH is 1.5-2.5. In other forms of PH, the decision to prescribe anticoagulants should be made individually in each case based on an assessment of the risk/effectiveness ratio. In particular, patients with portopulmonary PH have a high risk of developing bleeding from dilated veins of the esophagus.
  It is recommended to prescribe warfarin to patients with CTEPH.

  Comments. In CTEPH, target INR levels during warfarin** therapy are 2.5 - 3.5.
  It is recommended to prescribe low molecular weight heparins as an alternative to warfarin in patients with pulmonary hypertension with an increased risk of bleeding or in case of intolerance to the latter.
  Strength of recommendation level I (level of evidence certainty C).
  Comments. The most available low molecular weight heparins are nadroparin and enoxaparin**. During the 1st month of therapy, doses of nadroparin 15000 UAXaIC 2 times a day or enoxaparin 1 mg/kg body weight 2 times a day are used, subsequently lower prophylactic doses are used: nadroparin 7500 UAXaIC 1-2 times a day and enoxaparin 20-40 mgx1-2 times .
  The administration of antiplatelet agents is recommended for patients with PAH who have a positive test for vasoreactivity and are intolerant of oral anticoagulants.
  Level of strength of recommendation IIb (Level of evidence C).
  Comments. The use of acetylsalicylic acid 75-150 mg does not require laboratory control.
  For severe climacteric symptoms, postmenopausal patients with PH are recommended to undergo hormone replacement therapy, provided that adequate hypocoagulation is achieved using anticoagulant therapy.
  Level of strength of recommendation IIa (Level of evidence C).
  Comments. There are still unresolved questions about hormone replacement therapy in patients with PAH during menopause. This type of therapy can probably be discussed in cases of severe menopausal symptoms.
  Diuretics.
  It is recommended to prescribe diuretics in all cases of pancreatic decompensation in patients with PH.
  Strength of recommendation level I (level of evidence certainty C).
  Comments. Doses of diuretics should be carefully titrated to avoid a sharp decrease in circulating blood volume and a decrease in blood pressure. Loop diuretics are used: furosemide** 20-120 mg/day, ethacrynic acid 50-100 mg/day, torasemide 5-20 mg/day. It is advisable to add adosterone antagonists: veroshpiron** 25-150 mg, eplerenone 20 mg.
  In all cases of prescribing diuretics, it is recommended to carefully monitor blood electrolyte levels, as well as the state of renal function. .
  Strength of recommendation level I (level of evidence certainty C).
  Oxygen therapy.
  Oxygen therapy is recommended for patients with PH due to chronic obstructive pulmonary disease (COPD) for at least 15 hours a day to achieve a partial pressure of O2 in arterial blood of more than 8 kPa. .
  Strength of recommendation level I (level of evidence certainty C).
  Comments. It is important to maintain O2 saturation at 90% or higher at all times.
  In an outpatient setting, oxygen therapy is recommended to improve clinical symptoms and correct desaturation during physical activity. .
  Level of strength of recommendation IIb (Level of evidence C).
  Cardiac glycosides and inotropic drugs.
  The administration of digoxin** 0.25 mg/day is recommended to slow down the ventricular rate in case of supraventricular tachyarrhythmias in patients with PH. .
  Strength of recommendation level I (level of evidence certainty C).
  Cardiac glycosides are recommended for the progression of CHF in patients with PH. .
  Level of strength of recommendation IIb (Level of evidence C).
  Dobutamine in patients with PH is recommended in the terminal stage of the disease as inotropic support.
  Strength of recommendation level I (level of evidence certainty C).
  Other cardiovascular drugs.
  Treatment of anemia/iron deficiency in patients with PH is recommended. .
  Level of strength of recommendation IIb (Level of evidence C).
  Comments. Iron deficiency is determined in 43% of patients with IPH, 46% of patients with PAH due to systemic scleroderma and 56% of patients with Eisenmenger syndrome. In these categories of patients, it has been shown that iron deficiency can lead to poor exercise capacity and possibly increased mortality, regardless of the severity of the anemia. Regular monitoring of the state of iron metabolism in all patients with PAH is required for timely determination of iron deficiency and prescription of therapy with iron-containing drugs. Several studies have shown that iron absorption is impaired in PAH, so intravenous administration may be preferable, although no controlled studies have been conducted in this area.
  It is not recommended to prescribe angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, β-blockers, or ivabradine in patients with PAH in the absence of concomitant pathology.

  Comments. Currently, there is no convincing data in favor of the effectiveness and safety of angiotensin-converting enzyme inhibitors and sartans, β-blockers and ivabradine in patients with PAH. The use of these drugs is recommended only in the presence of concomitant cardiovascular diseases, such as arterial hypertension, coronary heart disease, left ventricular heart failure.

3.2.2 Specific therapy.

  Calcium antagonists.
  Calcium antagonists are recommended in high doses for patients with IPH, inherited PAH, or PAH due to medication with positive APP.
  Strength of recommendation level I (level of evidence certainty C).
  Comments. It is possible to use dihydropyridine AKs and diltiazem. Patients with a resting heart rate less than 80 beats/min. Nifedipine in prolonged forms or other dihydropyridine AKs of the third generation are recommended. For relative tachycardia (heart rate at rest more than 80 beats per minute), diltiazem is recommended at a dose of 240-720 mg.
  Amlodipine is recommended as the drug of choice for patients with PH with symptoms of right ventricular heart failure.
  Level of strength of recommendation IIa (Level of evidence C).
  Comments. Daily doses of AA that have shown effectiveness are quite high - for nifedipine** - 120-240 mg, for amlodipine** - up to 10-15 mg. It is recommended to gradually titrate the dose of the drug gradually over several weeks to the maximum tolerated dose.
  Patients with idiopathic/hereditary PAH, PAH due to medication, receiving AA in high doses require careful dynamic monitoring with a follow-up visit after 3-4 months. After initiation of therapy.
  Strength of recommendation level I (level of evidence certainty C).
  Comments. It is necessary to monitor the stability of the clinical effect of AK. In patients with IPH with positive OFP after 3-4 months of continuous therapy with AK, it is recommended to evaluate the effectiveness of therapy with mandatory CPOS after 3-4 months. If the response is inadequate - failure to achieve FC I or II (WHO), lack of significant improvement/almost normalization of hemodynamic parameters - treatment adjustment is required. In some cases, it is necessary to combine AKs with other PAH-specific drugs, since withdrawal of the former leads to clinical deterioration.
  Continuation of treatment with AA in high doses is recommended for patients with idiopathic/hereditary PAH, PAH due to medication in FC I-II and significant improvement in hemodynamics close to normalization. .
  Strength of recommendation level I (level of evidence certainty C).
  It is recommended to add PAH-specific therapy in patients with idiopathic/hereditary PAH, PAH due to medication in FC III-IV without significant improvement as a result of treatment with AA in high doses).
  Strength of recommendation level I (Level of certainty of evidence C.
  Prescribing AA in high doses is not recommended for patients with PAH without undergoing AFP or in the case of negative AFP, with the exception of clinical situations when the prescription of standard doses of drugs is due to other indications. .
  Strength of recommendation level III (Level of certainty of evidence C).
  Comments. Prescribing AC without carrying out a physical phase or a negative physical phase is fraught with the development of severe side effects - hypotension, syncope, right ventricular heart failure.
  Prostaglandins/prostanoids.
  Prostaglandins. This is a group of lipid compounds with a unique structure formed from a single substrate, arachidonic acid. . Prostaglandin E1 (PGE1) is a vasodilating prostaglandin with antiaggregation and antiproliferative effects. Due to the short half-life (3-5 minutes), it is possible to quickly titrate the dose to the maximum value, and, if necessary, quickly stop the effect of the drug. 90% of PGE1 is inactivated in the lungs, therefore, when administered intravenously, its entry into the systemic circulation is extremely small, as a result of which no pronounced systemic hypotension is observed.
  It is recommended to use intravenous PGE1 up to 30ng/kg/min for performing general physical therapy during CPOS.
  Level of strength of recommendation IIb (Level of evidence C).
  Comments. Previously, PGE1 was used in the form of intravenous intravenous infusions for 2-3 weeks during long-term therapy with calcium antagonists. Currently, due to the advent of inhaled iloprost and oral drugs for PAH-specific therapy, it is rational to use it exclusively for testing vasoreactivity in AFP.
  The use of intravenous PGE1 for chronic therapy is not recommended. .
  Strength of recommendation level III (Level of certainty of evidence C).
  Prostacyclin (prostaglandin I2). A powerful endogenous vasodilator with a whole range of additional effects - antiaggregation, antiproliferative and cytoprotective, which are aimed at preventing pulmonary vascular remodeling - reducing damage to endothelial cells and hypercoagulation. In patients with PH of various etiologies, a violation of prostacyclin production has been proven, as evidenced by a decrease in the expression of prostacyclin synthase in the pulmonary arteries and a decrease in the excretion of prostacyclin metabolites in the urine. From the class of prostanoids, which have different pharmacokinetic characteristics and similar pharmacodynamic effects, the only drug recommended in our country is iloprost in inhalation form.
  Iloprost. A chemically stable analogue of prostacyclin in aerosol form for inhalation is used in patients with PH in the form of mono- and combined PAH-specific therapy. The effectiveness of inhaled iloprost was assessed in the randomized, placebo-controlled study AIR-1 in patients with PAH and inoperable forms of CTEPH with class III-IV (NYHA). Iloprost/placebo inhalations were carried out 6-9 times at 2.5-5 mcg per inhalation during the day (average 30 mcg per day). Iloprost improved clinical symptoms, exercise tolerance, affected PVR, and the frequency of clinical events.
  Iloprost in inhalation form is recommended for the treatment of moderate and severe forms of PH: IPH, inherited PAH, PAH due to CTD, PAH while taking medications, inoperable forms of CTEPH.
  Recommendation strength level I (evidence level B) for patients with FC III.

  Comments. In the STEP study in 60 patients previously treated with bosentan, the addition of inhaled iloprost to therapy led to an increase in D6MQ (p< 0,051) по сравнению с плацебо . Илопрост отличается хорошей переносимостью. Наиболее частыми побочными эффектами были приливы и боли в челюсти.
  Endothelin receptor antagonists.
  Endothelin-1 (ET-1). This is a peptide of endothelial origin, characterized by powerful vasoconstrictor and mitogenic properties against smooth muscle cells. Activation of the endothelin system in patients with PAH is indicated by assessing its plasma and tissue concentrations. This is the rationale for the use of AREs that block type A receptors (ETA) or simultaneously both types of receptors - ETA and ETB. Activation of ETA and ETB receptors of smooth muscle cells causes a vasoconstrictor and mitogenic effect. Stimulation of ETB receptors promotes the clearance of ET-1 in the lungs and increases the production of NO and prostacyclin. However, in PAH there is an obvious deficiency of ETB receptors in the endothelium. Three large randomized clinical trials (RCTs) have been conducted with ARE. It has now been shown that, despite differences in activity towards different receptors, the effectiveness of dual and selective AREs in patients with PAH is comparable.
  Ambrisentan. Nonsulfonamide ARE, propanoic acid derivative, selective ETA receptor antagonist. The drug was studied in a pilot and two placebo-controlled studies. Two 12-week placebo-controlled RCTs, ARIES-1 (n=202) and ARIES-2 (n=192), studied the effectiveness and safety of ambrisentan used in different dose regimens - 2.5 mg or 5 mg in ARIES-1; 5mg or 10mg in ARIES-2. Both RCTs included patients over the age of 18 years with PAH of various etiologies (IPH, PAH due to anorectics, PAH-CTD or PAH-HIV), with any FC. However, most patients had FC II (ARIES-1: 32%; ARIES-2: 45%) or III (ARIES-1: 58%; ARIES-2: 52%), with a small proportion of FC I (ARIES-1: 2 .5%; ARIES-2: 1.5%) and IV (ARIES-1: 7%; ARIES-2: 2%). The mean placebo-adjusted increase in D6MX at week 12 of treatment (primary endpoint) in ARIES-1 was +31m (p=0.008) and +51m (p=0.001) in the 5mg and 10mg treatment groups, respectively; in ARIES-2 +32m (p=0.02) and +59m (p=0.001) in the 2.5 and 5 mg ambrisentan treatment groups, respectively. In 280 patients who completed 48 weeks of ambrisentan monotherapy, the improvement in D6MQ was +39m compared to baseline. In 3 groups of different dose regimens of therapy, the increase in D6MX varied from +31 to +59m.
  Compared with placebo, ambrisentan did not affect the risk of death or the need for hospitalization. In the ARIES-1/2 RCT, the incidence of death and the need for hospitalization due to progression of PAH was not significantly different between the ambrisentan and placebo groups.
  Ambrisentan is recommended for the treatment of patients with PAH to improve exercise tolerance and slow the progression of clinical symptoms (Table 9).

  Level of strength of recommendation IIb (Level of evidence C) for patients with FC IV.
  Comments. In RCTs, the effectiveness of the drug was established in patients with IPH, inherited PAH, PAH due to CTD with FC II-III (WHO). The recommended dose is 5 mg 1 time per day with a possible increase to 10 mg. The incidence of liver dysfunction is 0.8 to 3%, which requires monthly monitoring. During therapy with ambrisentan, peripheral edema occurs more often than with other AREs.
  Bosentan. An ERA that blocks both types of receptors was evaluated in PAH (IPH, PAH-CTD, Eisenmenger syndrome) in 6 RCTs (Study-351, BREATHE-1, BREATHE-2, BREATHE-5, EARLY, COMPASS-2). It has demonstrated the ability to improve exercise capacity and physical fitness, hemodynamic and echocardiographic parameters, and increase the time to clinical deterioration in patients with PAH compared with placebo.
  Bosentan** is recommended in patients with IPH, PAH associated with CTD, and Eisenmenger syndrome to improve exercise tolerance and slow the progression of the disease.
  Level of strength of recommendations I (Level of certainty of evidence A) for patients with FC II-III.
  Recommendation strength level IIb (Evidence Level C) for FC IV patients (Table 9).
  It is recommended to prescribe bosentan at a starting dose of 62.5 mg twice daily, followed by an increase in dose to 125 mg twice daily under close monthly monitoring of liver enzymes.

  Comments. In a pilot 12-week study of 351 in 32 patients with IPH and PAH-CTD FC III-IV placebo, the adjusted increase in D6MQ in the bosentan group was +76m (95% CI, 12-139; p = 0.021). In the BREATHE-1 RCT, 213 patients with IPH and PAH-MCTD were randomized 1:1:1 to receive 62.5 mg bosentan or placebo twice daily for 4 weeks, followed by 125 mg or 250 mg twice daily for 12 weeks. weeks Bosentan, compared with placebo, provided an increase in D6MQ by 44m (95% CI, 21-67m; p=0.001). In the BREATHE-5 RCT in patients with Eisenmenger syndrome FC III, bosentan compared with placebo for 16 weeks provided a decrease in the PVR index by -472.0 dynes/sec/cm 5 (p = 0.04), LAP. - by -5.5 mm, p=0.04) and increased D6MX by +53.1m (p=0.008). In the EARLY RCT using bosentan in patients with PAH with class II (WHO) (IPH, hereditary PAH, PAH-CTD, PAH-HIV, PAH-anorectics, PAH-CHD), there was a significant improvement in hemodynamics and an increase in the time to progression of PAH. When assessing hemodynamic parameters by 6 months. Treatment was observed to reduce PVR -22.6% (95% CI, -33.5 -10.0), as well as reduce the risk of clinical deterioration -77% (p = 0.01) at 24 weeks. The placebo-corrected increase in D6MQ in the bosentan group was +19m (95% CI, -33.6-10; p=0.07).
  It is recommended to monitor the level of transaminases in the blood monthly in patients with PAH during therapy with bosentan.
  Strength of Recommendation Level I (Level of Evidence A).
  Comments. Side effects of bosentan identified in RCTs include impaired liver function with increased transaminase levels, peripheral edema, palpitations, and chest pain. Monthly monitoring of blood transaminase levels is recommended in patients receiving bosentan. An increase in transaminase levels is observed in approximately 10% of patients, dose-dependent and reversible after dose reduction or drug discontinuation. The most likely mechanism of action of bosentan on liver enzyme levels is dose-dependent competition with bile salts, which leads to their retention in hepatocytes.
  It is recommended to monitor hemoglobin and blood hematocrit levels in patients with PAH receiving bosentan.
  Strength of Recommendation Level I (Level of Evidence A).
  Comments. In patients with PH, bosentan may cause anemia.
  Macitentan is recommended in patients with PAH to prevent disease progression (death, need for parenteral prostanoids, clinical worsening of PAH (decrease in D6M, worsening of clinical symptoms, need for additional PAH-specific therapy) (Table 9).
  Recommendation strength level I (evidence level B) for patients with FC II-III.
  Level of strength of recommendation IIb (Level of evidence C) for patients with FC IV.
  Comments. Macitentan is a dual ERA that was studied in the long-term, multicenter, double-blind, placebo-controlled clinical trial SERAPHIN to evaluate the effect of therapy on morbidity and mortality in patients with PAH. 742 patients with IPH or hereditary PAH, PAH-CTD, PAH after surgical correction of congenital heart disease-systemic pulmonary shunts, PAH-HIV, or due to drugs/toxins were randomized 1:1:1 to receive macitentan 3 mg ( n=250) and 10 mg (n=242) or placebo (n=250) once daily for approximately 100 weeks. The composite primary endpoint was the time until the first clinical event associated with worsening PAH (disease progression, initiation of parenteral prostanoid therapy, lung transplantation, atrial septostomy) or fatal outcome. Progression of PAH was established when a combination of three criteria was achieved: a decrease in D6MQ by 15% or more compared to the initial value (the result was confirmed in two tests performed on different days during a 2-week period), worsening of the clinical symptoms of PAH (deterioration of FC, appearance of signs pancreatic decompensation without significant dynamics when using oral diuretics), the need for additional therapy. Use of macitentan at doses of 3 mg (RR 0.70 (97.5% CI, 0.52-0.96; p=0.01) and 10 mg (RR 0.55 (97.5% CI, 0.39- 0.76; p=0.001) compared with placebo contributed to a reduction in the risk of morbidity and mortality in PAH by 30% and 45%, respectively. The effect of therapy did not depend on whether patients initially received concomitant PAH-specific therapy with PDE5, oral or inhaled prostanoids. By the 6th month of observation, in the placebo group, D6MX decreased by 9.4 m; in the macitentan treatment groups, the increase in distance was +7.4 m when a dose of 3 mg was prescribed (therapy effect +16.8 m compared with placebo (97.5% CI). , 2.7-3.4; p=0.01) and +12.5 m when prescribed 10 mg (therapy effect +22.0 m compared with placebo 97.5% CI, 3.2-40.8 ; p = 0.008). FC improved compared to baseline by the 6th month of treatment in 13% of patients in the placebo group, 20% in the macitentan 3 mg group (p = 0.04) and 22% in the 10 mg group (p = 0.006). Compared with the placebo group, therapy with macitentan caused a significant decrease in PVR and an increase in CI. Therapy with macitentan is characterized by a favorable tolerability profile. The frequency of a more than 3-fold increase in transaminases and the development of peripheral edema did not differ between treatment groups, which indicates the absence of hepatotoxicity of the drug. When prescribing macitentan, anemia was observed significantly more often than placebo. A decrease in hemoglobin ≤ 8 g/dl was observed in 4.3% of patients taking macitentan 10 mg/day.
  In women taking ERA, adequate contraception is recommended, taking into account the possible teratogenic effect.
  Strength of Recommendation Level I (Level of Evidence A).
  Phosphodiesterase type 5 inhibitors.
  Inhibitors of cGMP-dependent phosphodiesterase (type 5) prevent the degradation of cGMP, which leads to vasodilation by influencing the NO/cGMP system and causes a decrease in PVR and RV congestion. Sildenafil. Potent selective inhibitor of phosphodiesterase type 5 (PDE5) for oral administration. Four RCTs in patients with PAH have proven the positive effects of sildenafil in the form of improved exercise tolerance, clinical symptoms and/or hemodynamics.
  Sildenafil is recommended for PAH to improve exercise tolerance (Table 9).
  Level of strength of recommendations I (Level of certainty of evidence A) for patients with FC II-III.
  Level of strength of recommendation IIb (Level of evidence C) for patients with FC IV.

– 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 of bronchial asthma, valvular pneumothorax, and acute pneumonia. Pulmonary heart of subacute course develops with repeated pulmonary embolism, cancerous lymphangitis of the lungs, in cases of chronic hypoventilation associated with poliomyelitis, botulism, myasthenia gravis.

Mechanism of development of cor pulmonale

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

Arterial hypertension of the pulmonary circulation leads to overload of the right parts of the heart. As the disease develops, a shift in 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

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

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

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

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

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

Symptoms of cor pulmonale

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

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

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

In the stage of decompensation, right ventricular failure develops. The main manifestation is shortness of breath, which worsens with physical activity, inhalation of cold air, 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 with exacerbation of the inflammatory process in the lungs. In the late stage, swelling increases, liver enlargement progresses (hepatomegaly), neurological disorders appear (dizziness, headaches, apathy, drowsiness), and diuresis decreases.

Diagnosis of pulmonary heart

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

Forecast and prevention of cor pulmonale

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

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

PULMONARY HEART.

Relevance of the topic: Diseases of the bronchopulmonary system and chest are of great importance in affecting the heart. Damage to the cardiovascular system in diseases of the bronchopulmonary apparatus is referred to by most authors as cor pulmonale.

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

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

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

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

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

ETIOLOGY OF HEART PULMONARY.

Cor pulmonale is a consequence of diseases of three groups:

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

chronic obstructive bronchitis

pneumosclerosis of any etiology

pneumoconiosis

tuberculosis, not by itself, as post-tuberculosis outcomes

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

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

kyphoscoliosis

multiple rib injuries

Pickwickian syndrome in obesity

ankylosing spondylitis

pleural suppuration after pleurisy

Diseases primarily affecting the pulmonary vessels

primary arterial hypertension (Ayerza's disease)

recurrent pulmonary embolism (PE)

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

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

bronchopulmonary

thoradiaphragmatic

vascular

Standards for values ​​characterizing the hemodynamics of the pulmonary circulation.

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

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

PATHOGENESIS.

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

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

There are 3 stages of pulmonary failure.

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

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

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

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

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

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

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

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

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

CLASSIFICATION OF HEART PULMONARY.

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

bronchopulmonary heart

thoradiaphragmatic

vascular

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

• subacute

  chronic

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

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

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

Acute cor pulmonale develops primarily:

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

with pneumothorax (especially valvular),

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

for acute confluent pneumonia

right-sided total pleurisy

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

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

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

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

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

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

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

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

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

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

CLINIC OF ACUTE PULMONARY HEART.

The most common cause of development is pulmonary embolism, an acute increase in intrathoracic pressure due to an attack of bronchial asthma. Arterial precapillary hypertension in acute cor pulmonale, as in the vascular form of chronic cor pulmonale, is accompanied by an increase in pulmonary resistance. Next comes the rapid development of right ventricular dilatation. Acute right ventricular failure is manifested by severe shortness of breath turning into inspiratory suffocation, rapidly increasing cyanosis, chest pain of various types, shock or collapse, rapidly increasing liver size, swelling in the legs, ascites, epigastric pulsation, tachycardia (120-140), harsh breathing , in some places weakened vesicular; Moist, varied rales are heard, especially in the lower parts of the lungs. Additional research methods, especially ECG, are of great importance in the development of acute pulmonary heart disease: a sharp deviation of the electrical axis to the right (R 3 >R 2 >R 1, S 1 >S 2 >S 3), P-pulmonale appears - a pointed P wave, in the second , third standard leads. Right bundle branch block is complete or incomplete, ST inversion (usually elevation), S in the first lead is deep, Q in the third lead is deep. Negative S wave in the second and third leads. The same signs may also occur in acute myocardial infarction of the posterior wall.

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

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

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

CLINIC OF CHRONIC PULMONARY HEART.

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

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

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

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

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

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

Chronic cor pulmonale in primary pulmonary hypertension is called Aerz's disease (described in 1901). A polyetiological disease of unknown origin, it mainly affects women from 20 to 40 years old. Pathomorphological studies have established that with primary pulmonary hypertension, thickening of the intima of the precapillary arteries occurs, that is, in muscular-type arteries, thickening of the media is noted, and fibrinoid necrosis develops, followed by sclerosis and the rapid development of pulmonary hypertension. Symptoms are varied, usually complaints of weakness, fatigue, pain in the heart or joints, 1/3 of patients may experience fainting, dizziness, and Raynaud's syndrome. And then shortness of breath increases, which is a sign that indicates that primary pulmonary hypertension is entering a stable final stage. Cyanosis quickly increases, which is expressed to the degree of a cast-iron tint, becomes permanent, and swelling quickly increases. The diagnosis of primary pulmonary hypertension is established by exclusion. Most often this diagnosis is pathological. In these patients, the entire clinical picture progresses without any background in the form of obstructive or restrictive breathing disorders. With echocardiography, the pressure in the pulmonary artery reaches its maximum values. Treatment is ineffective, death occurs from thromboembolism.

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

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

deviation of the electrical axis of the heart to the right - R 3 >R 2 >R 1, S 1 >S 2 >S 3, angle greater than 120 degrees. The most basic indirect sign is an increase in the interval of the R wave in V1 by more than 7 mm.

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

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 of treating chronic pulmonary insufficiency and congestive heart disease, which can increase the life expectancy of patients.

Key words: 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 congenital heart defects.

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. The 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). A common cause of PLS ​​is cancer of the lungs, gastrointestinal tract, breast 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 gravis, 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 of the drainage function of the 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 artery 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 cellular membranes;

wounds, the effect on 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. First, during physical activity (stage I of CLN), and then at rest (stage III of 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 mild physical exertion or at rest, that is, already in stage II-III chronic pulmonary disease, when the presence of chronic pulmonary disease is undeniable.

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 CHL 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 the appearance in leads V5, V6 of a QRS complex of the RS type; SV5-6 is deep, and RV1-2 - normal amplitude); 2) SV5-6 > RV5-6; 3) RaVR > Q(S)aVR; 4) deviation of the electrical axis of the heart to the right, especially if α>110; 5) 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 of a split QRS complex of the rSR" type in lead V1 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. A 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 EchoCG data.

Echocardiography (EchoCG) is the leading non-invasive method for assessing pulmonary hemodynamics and diagnosing pulmonary disease. Ultrasound diagnosis of drugs 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 of 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 dilation 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). With a high degree of PH, 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 include: 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 of the 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 a reduction in the smooth muscles of the bronchi, the accumulation of a 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 with positive expiratory pressure (no more than 20 cm of water column) is indicated 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 endothelial cell damage 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 LS - with primary PH in systemic connective tissue diseases. The drug increases cardiac output and reduces pulmonary vascular resistance, and with long-term use improves the quality of life of patients with 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. A course of 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. To perform VCT, oxygen concentrators, liquid oxygen tanks and compressed gas cylinders can be used. 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 NO, the effect of which is similar to endothelium-relaxing factor, has a 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. Another serious side effect of diuretic therapy is metabolic alkalosis, which in patients with COPD with respiratory failure can lead to depression of the activity of the respiratory center and deterioration of gas exchange rates.

Angiotensin-converting enzyme inhibitors

In recent years, angiotensin-converting enzyme inhibitors (ACEIs) have come into first place in the treatment of patients with decompensated cor pulmonale. 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 the successful use 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 of 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.