Chronic cor pulmonale recommendations. Pulmonary hypertension in the new recommendations of the European Society of Cardiology (2015) Causes of cor pulmonale
PULMONARY HEART.
Relevance of the topic: Diseases of the bronchopulmonary system, chest are of great importance in the defeat of the heart. The defeat of the cardiovascular system in diseases of the bronchopulmonary apparatus, most authors refer to the term cor pulmonale.
Chronic cor pulmonale develops in approximately 3% of patients suffering from chronic lung diseases, and in the overall structure of mortality from congestive heart failure, chronic cor pulmonale accounts for 30% of cases.
Cor pulmonale is hypertrophy and dilatation or only dilatation of the right ventricle resulting from hypertension of the pulmonary circulation, developed as a result of diseases of the bronchi and lungs, chest deformity, or primary damage to the pulmonary arteries. (WHO 1961).
Hypertrophy of the right ventricle and its dilatation with changes as a result of a primary lesion of the heart, or congenital malformations do not belong to the concept of cor pulmonale.
Recently, clinicians have noticed that hypertrophy and dilatation of the right ventricle are already late manifestations of cor pulmonale, when it is no longer possible to rationally treat such patients, so a new definition of cor pulmonale was proposed:
Cor pulmonale is a complex of hemodynamic disorders in the pulmonary circulation, which develops as a result of diseases of the bronchopulmonary apparatus, chest deformities, and primary lesions of the pulmonary arteries, which at the final stage manifested by right ventricular hypertrophy and progressive circulatory failure.
ETIOLOGY OF THE PULMONARY HEART.
Cor pulmonale is a consequence of diseases of three groups:
Diseases of the bronchi and lungs, primarily affecting the passage of air and alveoli. This group includes approximately 69 diseases. They are the cause of cor pulmonale in 80% of cases.
chronic obstructive bronchitis
pneumosclerosis of any etiology
pneumoconiosis
tuberculosis, not by itself, as post-tuberculosis outcomes
SLE, Boeck's sarcoidosis, fibrosing alveolitis (endo- and exogenous)
Diseases that primarily affect chest, diaphragm with limitation of their mobility:
kyphoscoliosis
multiple rib injuries
pickwick syndrome in obesity
ankylosing spondylitis
pleural suppuration after pleurisy
Diseases primarily affecting the pulmonary vessels
primary arterial hypertension(Ayerza`s disease, disease Ayerza`s)
recurrent thromboembolism pulmonary artery(TELA)
compression of the pulmonary artery from the veins (aneurysm, tumors, etc.).
Diseases of the second and third groups are the cause of the development of cor pulmonale in 20% of cases. That is why they say that, depending on the etiological factor, there are three forms of cor pulmonale:
bronchopulmonary
thoracophrenic
vascular
Norms of values characterizing the hemodynamics of the pulmonary circulation.
The systolic pressure in the pulmonary artery is about five times less than the systolic pressure in the systemic circulation.
Pulmonary hypertension is said to be if the systolic pressure in the pulmonary artery at rest is greater than 30 mm Hg, the diastolic pressure is greater than 15, and the mean pressure is greater than 22 mm Hg.
PATHOGENESIS.
The basis of the pathogenesis of cor pulmonale is pulmonary hypertension. Since cor pulmonale most often develops in bronchopulmonary diseases, we will start with this. All diseases, and in particular chronic obstructive bronchitis, will primarily lead to respiratory (lung) failure. Pulmonary insufficiency is a condition in which normal blood gases are disturbed.
This is a state of the body in which either the normal gas composition of the blood is not maintained, or the latter is achieved by abnormal operation of the external respiration apparatus, leading to a decrease in the functional capabilities of the body.
There are 3 stages lung failure.
Arterial hypoxemia underlies the pathogenesis of chronic heart disease, especially in chronic obstructive bronchitis.
All these diseases lead to respiratory failure. Arterial hypoxemia will lead to alveolar hypoxia at the same time due to the development of pneumofibrosis, emphysema of the lungs, intra-alveolar pressure increases. Under conditions of arterial hypoxemia, the non-respiratory function of the lungs is disturbed - biologically active substances begin to be produced, which have not only a bronchospastic, but also a vasospastic effect. At the same time, when this occurs, a violation of the vascular architectonics of the lungs occurs - some of the vessels die, some expand, etc. Arterial hypoxemia leads to tissue hypoxia.
The second stage of pathogenesis: arterial hypoxemia will lead to a restructuring of central hemodynamics - in particular, an increase in the amount of circulating blood, polycythemia, polyglobulia, and an increase in blood viscosity. Alveolar hypoxia will lead to hypoxemic vasoconstriction in a reflex way, with the help of a reflex called the Euler-Liestrand reflex. Alveolar hypoxia led to hypoxemic vasoconstriction, an increase in blood pressure, which leads to an increase in hydrostatic pressure in the capillaries. Violation of the non-respiratory function of the lungs leads to the release of serotonin, histamine, prostaglandins, catecholamines, but most importantly, under conditions of tissue and alveolar hypoxia, the interstitium begins to produce more angiotensin-converting enzyme. The lungs are the main organ where this enzyme is formed. It converts angiotensin 1 into angiotensin 2. Hypoxemic vasoconstriction, the release of biologically active substances under conditions of restructuring of central hemodynamics will lead not only to an increase in pressure in the pulmonary artery, but to a persistent increase in it (above 30 mm Hg), that is, to the development pulmonary hypertension. If the processes continue further, if the underlying disease is not treated, then naturally part of the vessels in the pulmonary artery system dies due to pneumosclerosis, and the pressure steadily rises in the pulmonary artery. At the same time, persistent secondary pulmonary hypertension will open shunts between the pulmonary artery and bronchial arteries, and non-oxygenated blood enters the systemic circulation through the bronchial veins and also contributes to an increase in the work of the right ventricle.
So, the third stage is persistent pulmonary hypertension, the development of venous shunts, which enhance the work of the right ventricle. The right ventricle is not powerful in itself, and hypertrophy with elements of dilation rapidly develops in it.
The fourth stage is hypertrophy or dilatation of the right ventricle. Myocardial dystrophy of the right ventricle will contribute as well as tissue hypoxia.
So, arterial hypoxemia led to secondary pulmonary hypertension and right ventricular hypertrophy, to its dilatation and development of predominantly right ventricular circulatory failure.
The pathogenesis of the development of cor pulmonale in thoracophrenic form: in this form, hypoventilation of the lungs due to kyphoscoliosis, pleural suppuration, spinal deformities, or obesity is the leading one, in which the diaphragm rises high. Hypoventilation of the lungs will primarily lead to a restrictive type of respiratory failure, in contrast to the obstructive type that is caused by chronic cor pulmonale. And then the mechanism is the same - a restrictive type of respiratory failure will lead to arterial hypoxemia, alveolar hypoxemia, etc.
The pathogenesis of the development of cor pulmonale in the vascular form lies in the fact that with thrombosis of the main branches of the pulmonary arteries, the blood supply to the lung tissue decreases sharply, since along with thrombosis of the main branches, friendly reflex narrowing of the small branches occurs. In addition, in the vascular form, in particular in primary pulmonary hypertension, pronounced humoral changes contribute to the development of cor pulmonale, that is, a noticeable increase in the amount of serotonin, prostaglandins, catecholamines, the release of convertase, angiotensin-converting enzyme.
The pathogenesis of cor pulmonale is a multi-stage, multi-stage, in some cases not completely clear.
CLASSIFICATION OF THE PULMONARY HEART.
There is no single classification of cor pulmonale, but the first international classification mostly etiological (WHO, 1960):
bronchopulmonary heart
thoracophrenic
vascular
A domestic classification of the cor pulmonale is proposed, which provides for the division of the cor pulmonale according to the rate of development:
subacute
chronic
Acute cor pulmonale develops within a few hours, minutes, maximum days. Subacute cor pulmonale develops over several weeks or months. Chronic cor pulmonale develops over several years (5-20 years).
This classification provides for compensation, but acute cor pulmonale is always decompensated, that is, it requires immediate assistance. Subacute can be compensated and decompensated mainly according to the right ventricular type. Chronic cor pulmonale can be compensated, subcompensated, decompensated.
By genesis, acute cor pulmonale develops in vascular and bronchopulmonary forms. Subacute and chronic cor pulmonale can be vascular, bronchopulmonary, thoracophrenic.
Acute cor pulmonale develops primarily:
with embolism - not only with thromboembolism, but also with gas, tumor, fat, etc.,
with pneumothorax (especially valvular),
with an attack of bronchial asthma (especially with asthmatic status - a qualitatively new condition in patients with bronchial asthma, with complete blockade of beta2-adrenergic receptors, and with acute cor pulmonale);
with acute confluent pneumonia
right-sided total pleurisy
A practical example of subacute cor pulmonale is recurrent thromboembolism of small branches of the pulmonary arteries during an attack of bronchial asthma. A classic example is cancerous lymphangitis, especially in chorionepithelioma, in peripheral lung cancer. The thoracodiphragmatic form develops with hypoventilation of central or peripheral origin - myasthenia gravis, botulism, poliomyelitis, etc.
To distinguish at what stage the cor pulmonale from the stage of respiratory failure passes into the stage of heart failure, another classification was proposed. Cor pulmonale is divided into three stages:
hidden latent insufficiency - there is a violation of the function of external respiration - the VC / CL decreases to 40%, but there are no changes in the gas composition of the blood, that is, this stage characterizes respiratory failure of 1-2 stages.
stage of severe pulmonary insufficiency - the development of hypoxemia, hypercapnia, but without signs of heart failure in the periphery. There is shortness of breath at rest, which cannot be attributed to heart damage.
stage of pulmonary heart failure of varying degrees (edema in the limbs, an increase in the abdomen, etc.).
Chronic cor pulmonale according to the level of pulmonary insufficiency, saturation of arterial blood with oxygen, right ventricular hypertrophy and circulatory failure is divided into 4 stages:
the first stage - pulmonary insufficiency of the 1st degree - VC / CL decreases to 20%, the gas composition is not disturbed. Right ventricular hypertrophy is absent on the ECG, but there is hypertrophy on the echocardiogram. There is no circulatory failure at this stage.
pulmonary insufficiency 2 - VC / CL up to 40%, oxygen saturation up to 80%, the first indirect signs of right ventricular hypertrophy appear, circulatory failure +/-, that is, only shortness of breath at rest.
the third stage - pulmonary insufficiency 3 - VC / CL less than 40%, saturation of arterial blood up to 50%, there are signs of right ventricular hypertrophy on the ECG in the form of direct signs. Circulatory failure 2A.
fourth stage - pulmonary insufficiency 3. Blood oxygen saturation less than 50%, right ventricular hypertrophy with dilatation, circulatory failure 2B (dystrophic, refractory).
CLINIC OF ACUTE PULMONARY HEART.
The most common cause of development is PE, an acute increase in intrathoracic pressure due to an attack of bronchial asthma. Arterial precapillary hypertension in acute cor pulmonale, as well as in the vascular form of chronic cor pulmonale, is accompanied by an increase in pulmonary resistance. Next comes the rapid development of dilatation of the right ventricle. Acute right ventricular failure is manifested by severe shortness of breath turning into inspiratory suffocation, rapidly increasing cyanosis, pain behind the sternum of a different nature, shock or collapse, the size of the liver rapidly increases, edema in the legs appears, ascites, epigastric pulsation, tachycardia (120-140), hard breathing , in some places weakened vesicular; wet, various rales are heard, especially in the lower parts of the lungs. Of great importance in the development of acute pulmonary heart are additional research methods, especially ECG: a sharp deviation of the electrical axis to the right (R 3>R 2>R 1, S 1>S 2>S 3), P-pulmonale appears - a pointed P wave, in the second , third standard leads. The blockade of the right leg of the bundle of His is complete or incomplete, ST inversion (usually rise), S in the first lead is deep, Q in the third lead is deep. Negative S wave in leads 2 and 3. These same signs can also occur in acute myocardial infarction of the posterior wall.
Emergency care depends on the cause of acute cor pulmonale. If there was PE, then painkillers, fibrinolytic and anticoagulant drugs (heparin, fibrinolysin), streptodecas, streptokinase are prescribed, up to surgical treatment.
With asthmatic status - large doses of glucocorticoids intravenously, bronchodilators through a bronchoscope, transfer to mechanical ventilation and bronchial lavage. If this is not done, the patient dies.
With valvular pneumothorax - surgical treatment. With confluent pneumonia, along with antibiotic treatment, diuretics and cardiac glycosides are required.
CLINIC OF CHRONIC PULMONARY HEART.
Patients are concerned about shortness of breath, the nature of which depends on the pathological process in the lungs, the type of respiratory failure (obstructive, restrictive, mixed). With obstructive processes, dyspnea of an expiratory nature with an unchanged respiratory rate, with restrictive processes, the duration of exhalation decreases, and the respiratory rate increases. An objective examination, along with signs of the underlying disease, cyanosis appears, most often diffuse, warm due to the preservation of peripheral blood flow, in contrast to patients with heart failure. In some patients, cyanosis is so pronounced that the skin acquires a cast-iron color. Swollen neck veins, swelling of the lower extremities, ascites. The pulse is quickened, the borders of the heart expand to the right, and then to the left, muffled tones due to emphysema, the accent of the second tone over the pulmonary artery. Systolic murmur at the xiphoid process due to dilatation of the right ventricle and relative insufficiency of the right tricuspid valve. In some cases, with severe heart failure, you can listen to a diastolic murmur on the pulmonary artery - Graham-Still murmur, which is associated with relative insufficiency of the pulmonary valve. Above the lungs, percussion, the sound is boxy, breathing is vesicular, hard. In the lower parts of the lungs there are congestive, inaudible moist rales. On palpation of the abdomen - an increase in the liver (one of the reliable, but not early signs cor pulmonale, since the liver can be displaced due to emphysema). The severity of symptoms depends on the stage.
The first stage: against the background of the underlying disease, shortness of breath increases, cyanosis appears in the form of acrocyanosis, but the right border of the heart is not expanded, the liver is not enlarged, in the lungs the physical data depend on the underlying disease.
The second stage - shortness of breath turns into attacks of suffocation, with difficulty breathing, cyanosis becomes diffuse, from the data of an objective study: a pulsation appears in the epigastric region, muffled tones, the accent of the second tone over the pulmonary artery is not constant. The liver is not enlarged, may be omitted.
The third stage - signs of right ventricular failure join - an increase in the right border of cardiac dullness, an increase in the size of the liver. Persistent swelling in the lower extremities.
The fourth stage is shortness of breath at rest, a forced position, often accompanied by respiratory rhythm disorders such as Cheyne-Stokes and Biot. The edema is constant, not amenable to treatment, the pulse is weak and frequent, the heart of a bull, the tones are deaf, the systolic murmur at the xiphoid process. Lots of moist rales in the lungs. The liver is of considerable size, does not shrink under the action of glycosides and diuretics, as fibrosis develops. Patients are constantly dozing.
Diagnosis of thoracodiaphragmatic heart is often difficult, one must always remember about the possibility of its development in kyphoscoliosis, Bechterew's disease, etc. The most important sign is the early onset of cyanosis, and a noticeable increase in shortness of breath without asthma attacks. Pickwick's syndrome is characterized by a triad of symptoms - obesity, drowsiness, severe cyanosis. This syndrome was first described by Dickens in The Posthumous Papers of the Pickwick Club. Associated with traumatic brain injury, obesity is accompanied by thirst, bulimia, arterial hypertension. Diabetes mellitus often develops.
Chronic cor pulmonale in primary pulmonary hypertension is called Ayerz's disease (described in 1901). A polyetiological disease of unknown origin, predominantly affects women from 20 to 40 years old. Pathological studies have established that in primary pulmonary hypertension, there is a thickening of the intima of the precapillary arteries, that is, in the arteries muscular type 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, Raynaud's syndrome. And in the future, shortness of breath increases, which is the sign that indicates that primary pulmonary hypertension is moving into a stable final stage. Cyanosis is rapidly growing, which is expressed to the degree of a cast-iron hue, becomes permanent, edema rapidly increases. The diagnosis of primary pulmonary hypertension is established by exclusion. Most often this diagnosis is pathological. In these patients, the entire clinic progresses without a background in the form of obstructive or restrictive respiratory failure. With echocardiography, the pressure in the pulmonary artery reaches its maximum values. Treatment is ineffective, death occurs from thromboembolism.
Additional research methods for cor pulmonale: in a chronic process in the lungs - leukocytosis, an increase in the number of red blood cells (polycythemia associated with increased erythropoiesis due to arterial hypoxemia). X-ray data: appear very late. One of the early symptoms is a bulging of the pulmonary artery on x-ray. The pulmonary artery bulges, often flattening the waist of the heart, and this heart is mistaken by many physicians for a mitral configuration of the heart.
ECG: indirect and direct signs of right ventricular hypertrophy appear:
deviation of the electrical axis of the heart to the right - R 3 > R 2 > R 1 , S 1 > S 2 > S 3, the angle is greater than 120 degrees. The most basic indirect sign is an increase in the interval of the R wave in V 1 greater than 7 mm.
direct signs - blockade of the right leg of the bundle of His, the amplitude of the R wave in V 1 more than 10 mm with complete blockade of the right leg of the bundle of His. The appearance of a negative T wave with a displacement of the wave below the isoline in the third, second standard lead, V1-V3.
Of great importance is spirography, which reveals the type and degree of respiratory failure. On the ecg signs hypertrophy of the right ventricle appears very late, and if only deviations of the electrical axis to the right appear, then they already speak of pronounced hypertrophy. The most basic diagnosis is dopplercardiography, echocardiography - an increase in the right heart, increased pressure in the pulmonary artery.
PRINCIPLES OF TREATMENT OF PULMONARY HEART.
Treatment of cor pulmonale is to treat the underlying disease. With exacerbation of obstructive diseases, bronchodilators, expectorants are prescribed. With Pickwick's syndrome - treatment of obesity, etc.
Reduce pressure in the pulmonary artery with calcium antagonists (nifedipine, verapamil), peripheral vasodilators that reduce preload (nitrates, corvaton, sodium nitroprusside). Of greatest importance is sodium nitroprusside in combination with angiotensin-converting enzyme inhibitors. Nitroprusside 50-100 mg intravenously, capoten 25 mg 2-3 times a day, or enalapril (second generation, 10 mg per day). Treatment with prostaglandin E, antiserotonin drugs, etc. are also used. But all these drugs are effective only at the very beginning of the disease.
Treatment of heart failure: diuretics, glycosides, oxygen therapy.
Anticoagulant, antiaggregant therapy - heparin, trental, etc. Due to tissue hypoxia, myocardial dystrophy develops rapidly, therefore, cardioprotectors are prescribed (potassium orotate, panangin, riboxin). Cardiac glycosides are prescribed very carefully.
PREVENTION.
Primary - prevention of chronic bronchitis. Secondary - treatment of chronic bronchitis.
Cor pulmonale (PC) is hypertrophy and/or dilatation of the right ventricle (RV) resulting from pulmonary arterial hypertension caused by diseases that affect the function and / or structure of the lungs, and are not associated with a primary pathology of the left heart or congenital heart defects. LS is formed due to diseases of the bronchi and lungs, thoracophrenic lesions or pathology of the pulmonary vessels. The development of chronic cor pulmonale (CHP) is most often due to chronic pulmonary insufficiency (CLF), and the main cause of the formation of CLP is alveolar hypoxia, which causes spasm of the pulmonary arterioles.
Diagnostic search is aimed at identifying the underlying disease that led to the development of CHL, as well as assessing CRF, pulmonary hypertension, and the condition of the pancreas.
Treatment of CHLS is the treatment of the underlying disease that is the cause of CHLS (chronic obstructive bronchitis, bronchial asthma, etc.), elimination of alveolar hypoxia and hypoxemia with a decrease in pulmonary arterial hypertension (training of the respiratory muscles, electrical stimulation of the diaphragm, normalization of the oxygen transport function of the blood (heparin, erythrocytapheresis, hemosorption), long-term oxygen therapy (VCT), almitrin), as well as correction of right ventricular heart failure (ACE inhibitors, diuretics, aldosterone blockers, angiothesin II receptor antagonists). VCT is the most effective method treatment of CLN and HLS, which can increase the life expectancy of patients.
Keywords: cor pulmonale, pulmonary hypertension, chronic pulmonary insufficiency, chronic cor pulmonale, right ventricular heart failure.
DEFINITION
Pulmonary heart- this is hypertrophy and / or dilatation of the right ventricle, resulting from pulmonary arterial hypertension caused by diseases that affect the function and / or structure of the lungs and are not associated with a primary pathology of the left heart or congenital heart defects.
Pulmonary heart (PC) is formed on the basis of pathological changes of the lung itself, violations of extrapulmonary respiratory mechanisms that provide ventilation of the lung (damage to the respiratory muscles, violation of the central regulation of respiration, elasticity of the bone and cartilage formations of the chest or conduction of a nerve impulse along n. diaphragmicus, obesity), as well as damage to the pulmonary vessels.
CLASSIFICATION
In our country, the classification of cor pulmonale proposed by B.E. Votchalom in 1964 (Table 7.1).
Acute LS is associated with a sharp increase in pulmonary arterial pressure (PAP) with the development of right ventricular failure and is most often caused by thromboembolism of the main trunk or large branches of the pulmonary artery (PE). However, the doctor sometimes encounters a similar condition when large areas of lung tissue are turned off from the circulation (bilateral extensive pneumonia, status asthmaticus, valve pneumothorax).
Subacute cor pulmonale (PLC) is most often the result of recurrent thromboembolism of small branches of the pulmonary artery. The leading clinical symptom is increasing dyspnea with rapidly developing (within months) right ventricular failure. Other causes of PLS include neuromuscular diseases (myasthenia gravis, poliomyelitis, damage to the phrenic nerve), exclusion of a significant part of the respiratory section of the lung from the act of breathing (severe bronchial asthma, miliary pulmonary tuberculosis). A common cause of PLS is lung cancer, gastrointestinal tract, mammary gland and other localization, due to lung carcinomatosis, as well as compression of the vessels of the lungs by a germinating tumor, followed by thrombosis.
Chronic cor pulmonale (CHP) in 80% of cases occurs with damage to the bronchopulmonary apparatus (most often with COPD) and is associated with a slow and gradual increase in pressure in the pulmonary artery over many years.
The development of CLS is directly related to chronic pulmonary insufficiency (CLF). IN clinical practice use the classification of CLN based on the presence of dyspnea. There are 3 degrees of CLN: the appearance of shortness of breath with previously available efforts - I degree, shortness of breath during normal exertion - II degree, shortness of breath at rest - III degree. It is sometimes appropriate to supplement the above classification with data on the gas composition of the blood and pathophysiological mechanisms for the development of pulmonary insufficiency (Table 7.2), which makes it possible to select pathogenetically substantiated therapeutic measures.
Classification of cor pulmonale (according to Votchal B.E., 1964)
Table 7.1.
The nature of the flow | Compensation Status | Preferential 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 protracted attacks of bronchial asthma. Cancer lymphangitis of the lungs |
||
Thoracodiaphragmatic | Chronic hypoventilation of central and peripheral origin in botulism, poliomyelitis, myasthenia gravis, etc. |
The end of the table. 7.1.
Note. The diagnosis of cor pulmonale is made after the diagnosis of the underlying disease: when formulating the diagnosis, only the first two columns of the classification are used. Columns 3 and 4 contribute to an in-depth understanding of the essence of the process and the choice of therapeutic tactics
Table 7.2.
Clinical and pathophysiological classification of chronic pulmonary insufficiency
(Aleksandrov O.V., 1986)
Stage of chronic pulmonary insufficiency | Availability 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) in the assessment of respiratory function | Basic therapy 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 | ECG, echocardiographic and radiographic signs of overload and hypertrophy of the right heart, changes in the gas composition of the blood, as well as erythrocytosis, increased blood viscosity, morphological changes in erythrocytes join the violations of the respiratory function. | Supplemented with long-term oxygen therapy (if pO 2<60мм рт.ст.), альмитрином, ЛФК, кардиологическими средствами |
III. Metabolic disorders | Clinical manifestations are pronounced | Strengthening of the violations described above. metabolic acidosis. Hypoxemia, hypercapnia | Complemented by extracorporeal methods of treatment (erythrocytapheresis, hemosorption, plasmapheresis, extracorporeal membrane oxygenation) |
In the presented classification of CLN, the diagnosis of CLN with a high probability can be made at stages II and III of the process. In stage I CLN (latent), rises in PAP are detected, usually in response to physical activity and during an exacerbation of the disease in the absence of signs of RV hypertrophy. This circumstance made it possible to express the opinion (N.R. Paleev) that for the diagnosis of 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 substantiated.
Over time, the development of decompensated HLS is possible. In the absence of a special classification of RV failure, the well-known classification of heart failure (HF) according to V.Kh. Vasilenko and N.D. Strazhesko, which is usually used for heart failure, which has developed as a result of damage to the left ventricle (LV) or both ventricles. The presence of left ventricular HF in patients with CLS is most often due to two reasons: 1) CHL in people over 50 years of age is often combined with coronary artery disease, 2) systemic arterial hypoxemia in patients with CLS leads to dystrophic processes in the LV myocardium, to its moderate hypertrophy and contractile insufficiency.
Chronic obstructive pulmonary disease is the main cause of chronic cor pulmonale.
PATHOGENESIS
The development of chronic LS is based on the gradual formation of pulmonary arterial hypertension due to several pathogenetic mechanisms. The main cause of PH in patients with bronchopulmonary and thoracophrenic forms of CLS is alveolar hypoxia, the role of which in the development of pulmonary vasoconstriction was first shown in 1946 by U. Von Euler and G. Lijestrand. The development of the Euler-Liljestrand reflex is explained by several mechanisms: the effect of hypoxia is associated with the development of depolarization of vascular smooth muscle cells and their contraction due to changes in the function of potassium channels of cell membranes.
wounds, exposure to the vascular wall of endogenous vasoconstrictor mediators, such as leukotrienes, histamine, serotonin, angiotensin II and catecholamines, the production of which increases significantly under hypoxic conditions.
Hypercapnia also contributes to the development of pulmonary hypertension. However, a high concentration of CO 2, apparently, does not act directly on the tone of the pulmonary vessels, but indirectly - mainly through the acidosis caused by it. In addition, CO 2 retention contributes to a decrease in the sensitivity of the respiratory center to CO 2, which further reduces lung ventilation and contributes to pulmonary vasoconstriction.
Of particular importance in the genesis of PH is endothelial dysfunction, manifested by a decrease in the synthesis of vasodilating antiproliferative mediators (NO, prostacyclin, prostaglandin E 2) and an increase in the level of vasoconstrictors (angiotensin, endothelin-1). Pulmonary endothelial dysfunction in COPD patients is associated with hypoxemia, inflammation, and exposure to cigarette smoke.
Structural changes in the vascular bed occur in CLS patients - remodeling of the pulmonary vessels, characterized by thickening of the intima due to the proliferation of smooth muscle cells, deposition of elastic and collagen fibers, hypertrophy of the muscular layer of the arteries with a decrease in the inner diameter of the vessels. In patients with COPD, due to emphysema, there is a reduction in the capillary bed, compression of the pulmonary vessels.
In addition to chronic hypoxia, along with structural changes in the vessels of the lungs, a number of other factors also affect the increase in pulmonary pressure: polycythemia with a change in the rheological properties of blood, impaired metabolism of vasoactive substances in the lungs, an increase in minute blood volume due to tachycardia and hypervolemia. One of the possible causes of hypervolemia is hypercapnia and hypoxemia, which increase the concentration of aldosterone in the blood and, accordingly, Na + and water retention.
In patients with severe obesity, Pickwick's syndrome (named after the work of Charles Dickens) develops, which is manifested by hypoventilation with hypercapnia, which is associated with a decrease in the sensitivity of the respiratory center to CO 2, as well as impaired ventilation due to mechanical limitation by adipose tissue with dysfunction (fatigue) respiratory muscles.
Elevated blood pressure in the pulmonary artery may initially contribute to an increase in the volume of perfusion of the pulmonary capillaries, however, over time, hypertrophy of the myocardium of the pancreas develops, followed by its contractile insufficiency. Indicators of pressure in the pulmonary circulation are presented in table. 7.3.
Table 7.3
Indicators of pulmonary hemodynamics
The criterion for pulmonary hypertension is the level of mean pressure in the pulmonary artery at rest, exceeding 20 mm Hg.
CLINIC
The clinical picture consists of the manifestations of the underlying disease, leading to the development of CHLS and damage to the pancreas. In clinical practice, most often among the causative lung diseases chronic obstructive pulmonary disease (COPD) is detected, i.e. bronchial asthma or chronic obstructive bronchitis and emphysema. The CLS clinic is inextricably linked with the manifestation of CHLN itself.
A characteristic complaint of patients is shortness of breath. Initially, during exercise (stage I of CRF), and then at rest (stage III of CRF). It has an expiratory or mixed character. A long course (years) of COPD dulls the patient's attention and forces him to consult a doctor when shortness of breath appears during mild exertion or at rest, that is, already in stage II-III CRF, when the presence of CHL is indisputable.
Unlike dyspnea associated with left ventricular failure and venous stasis of blood in the lungs, dyspnea in pulmonary hypertension does not increase in the horizontal position of the patient and does not
decreases in the sitting position. Patients may even prefer a horizontal position of the body, in which the diaphragm takes a greater part in intrathoracic hemodynamics than facilitates the breathing process.
Tachycardia is a frequent complaint of patients with CHL and appears even at the stage of development of CRF in response to arterial hypoxemia. Heart rhythm disorder is rare. The presence of atrial fibrillation, especially in people over 50 years of age, is usually associated with concomitant coronary artery disease.
Half of the patients with CLS experience pain in the region of the heart, often of an indeterminate nature, without irradiation, as a rule, not associated with physical activity and not relieved by nitroglycerin. The most common view on the mechanism of pain is relative coronary insufficiency due to a significant increase in the muscle mass of the pancreas, as well as a decrease in the filling of the coronary arteries with an increase in end-diastolic pressure in the pancreatic cavity, myocardial hypoxia against the background of general arterial hypoxemia (“blue angina pectoris”) and reflex narrowing right coronary artery (pulmocoronary reflex). A possible cause of cardialgia may be stretching of the pulmonary artery with a sharp increase in pressure in it.
With decompensation of the pulmonary heart, edema may appear on the legs, which first occur most often during an exacerbation of a bronchopulmonary disease and are first localized in the area of the feet and ankles. As right ventricular failure progresses, edema spreads to the area of the legs and thighs, and rarely, in severe cases of right ventricular failure, there is an increase in the abdomen in volume due to emerging ascites.
A less specific symptom of cor pulmonale is loss of voice, which is associated with compression of the recurrent nerve by a dilated trunk of the pulmonary artery.
Patients with CRF and CLS may develop encephalopathy due to chronic hypercapnia and hypoxia of the brain, as well as impaired vascular permeability. With severe encephalopathy, some patients experience increased excitability, aggressiveness, euphoria, and even psychosis, while other patients experience lethargy, depression, drowsiness during the day and insomnia at night, and headaches. Rarely, syncope occurs during physical exertion as a result of severe hypoxia.
A common symptom of CLN is a diffuse "grayish-blue", warm cyanosis. When right ventricular failure occurs in patients with CLS, cyanosis often acquires a mixed character: against the background of diffuse bluish staining of the skin, cyanosis of the lips, tip of the nose, chin, ears, fingertips and toes appears, and the limbs in most cases remain warm, possibly due to peripheral vasodilation due to hypercapnia. Swelling of the cervical veins is characteristic (including on inspiration - Kussmaul's symptom). Some patients may develop a painful blush on the cheeks and an increase in the number of vessels on the skin and conjunctiva (“rabbit or frog eyes” due to hypercapnia), Plesh’s symptom (swelling of the neck veins when pressing the palm of the hand on the enlarged liver), Corvisar’s face, cardiac cachexia, signs of the main diseases (emphysematous chest, kyphoscoliosis of the thoracic spine, etc.).
On palpation of the region of the heart, a pronounced diffuse cardiac impulse, epigastric pulsation (due to hypertrophy and dilatation of the pancreas) can be detected, and with percussion, an expansion of the right border of the heart to the right. However, these symptoms lose their diagnostic value due to the often developing pulmonary emphysema, in which the percussion dimensions of the heart can even be reduced (“drip heart”). The most common auscultatory symptom in CHLS is the emphasis of the second tone over the pulmonary artery, which can be combined with splitting of the second tone, right ventricular IV heart sound, diastolic murmur of pulmonary valve insufficiency (Graham-Still murmur) and systolic murmur of tricuspid insufficiency, with the intensity of both murmurs increasing by inspiratory height (Rivero-Corvalho symptom).
Arterial pressure in patients with compensated CHLS is often increased, and in decompensated patients it is reduced.
Hepatomegaly is detected in almost all patients with decompensated LS. The liver is enlarged, compacted on palpation, painful, the edge of the liver is rounded. With severe heart failure, ascites appears. In general, such severe manifestations of right ventricular heart failure in CLS are rare, because the very presence of severe CRF or the addition of an infectious process in the lung leads to a tragic ending in the patient earlier than it occurs due to heart failure.
The clinic of chronic cor pulmonale is determined by the severity of pulmonary pathology, as well as pulmonary and right ventricular heart failure.
INSTRUMENTAL DIAGNOSIS
The X-ray picture of CLS depends on the stage of CRF. Against the background of radiological manifestations of a pulmonary disease (pneumosclerosis, emphysema, increased vascular pattern, etc.), at first there is only a slight decrease in the shadow of the heart, then a moderate bulging of the cone of the pulmonary artery appears in the direct and right oblique projection. Normally, in direct projection, the right heart contour is formed by the right atrium, and in CHLS with an increase in the RV, it becomes edge-forming, and with significant hypertrophy of the RV, it can form both the right and left edges of the heart, pushing the left ventricle back. In the final decompensated stage of HLS, the right edge of the heart can be formed by a significantly dilated right atrium. Nevertheless, this "evolution" takes place against the background of a relatively small shadow of the heart ("drip" or "hanging").
Electrocardiographic diagnosis of CLS is reduced to the detection of pancreatic hypertrophy. The main (“direct”) ECG criteria for RV hypertrophy include: 1) R in V1>7mm; 2) S in V5-6 > 7 mm; 3) RV1 + SV5 or RV1 + SV6 > 10.5 mm; 4) RaVR > 4 mm; 5) SV1,V2 =s2 mm; 6) RV5,V6<5 мм; 7) отношение R/SV1 >1; 8) complete blockade the right leg of the bundle of His with RV1>15 mm; 9) incomplete blockade of the right leg of the bundle of His with RV1>10 mm; 10) negative TVl and decrease in STVl, V2 with RVl>5 mm and no coronary insufficiency. In the presence of 2 or more "direct" ECG signs, the diagnosis of RV hypertrophy is considered reliable.
Indirect ECG signs of RV hypertrophy suggest RV hypertrophy: 1) rotation of the heart around the longitudinal axis clockwise (shift of the transition zone to the left, to leads V5-V6 and the appearance in leads V5, V6 of the QRS type RS complex; SV5-6 is deep, and RV1-2 - normal amplitude); 2) SV5-6 > RV5-6; 3) RaVR > Q(S)aVR; 4) deviation of the electrical axis of the heart to the right, especially if α>110; 5) electric axis heart type
SI-SII-SIII; 6) complete or incomplete blockade of the right leg of the bundle of His; 7) electrocardiographic signs of right atrial hypertrophy (P-pulmonale in leads II, III, aVF); 8) an increase in the activation time of the right ventricle in V1 by more than 0.03 s. There are three types of ECG changes in CHLS:
1. rSR "-type ECG is characterized by the presence of a split QRS complex of the rSR type in lead V1 and is usually detected with severe RV hypertrophy;
2. The R-type ECG is characterized by the presence of a QRS complex of the Rs or qR type in lead V1 and is usually detected with severe RV hypertrophy (Fig. 7.1).
3. S-type ECG is often detected in COPD patients with emphysema. It is associated with a posterior displacement of the hypertrophied heart, which is caused by pulmonary emphysema. The ECG looks like rS, RS or Rs with a pronounced S wave in both the right and left chest leads
Rice. 7.1. ECG of a patient with COPD and CHLS. Sinus tachycardia. Pronounced hypertrophy of the right ventricle (RV1 = 10 mm, SV1 is absent, SV5-6 = 12 mm, a sharp EOS deviation to the right (α = +155°), negative TV1-2 and a decrease in the STV1-2 segment). Right atrial hypertrophy (P-pulmonale in V2-4)
Electrocardiographic criteria for RV hypertrophy are not sufficiently specific. They are less clear-cut than in LV hypertrophy and can lead to false positive and false negative diagnoses. A normal ECG does not exclude the presence of CHLS, especially in patients with COPD, so ECG changes should be compared with the clinical picture of the disease and echocardiography data.
Echocardiography (EchoCG) is the leading non-invasive method for assessing pulmonary hemodynamics and diagnosing LS. Ultrasound diagnostics LS is based on the identification of signs of damage to the myocardium of the pancreas, which are given below.
1. Change in the size of the right ventricle, which is assessed in two positions: in the parasternal position along the long axis (normally less than 30 mm) and in the apical four-chamber position. To detect dilatation of the pancreas, measurement of its diameter (normally less than 36 mm) and area at the end of diastole along the long axis in the apical four-chamber position is more often used. In order to more accurately assess the severity of RV dilatation, it is recommended to use the ratio of the RV end-diastolic area to the LV end-diastolic area, thereby excluding individual differences in heart size. An increase in this indicator over 0.6 indicates a significant dilatation of the pancreas, and if it becomes equal to or greater than 1.0, then a conclusion is made about a pronounced dilatation of the pancreas. With dilatation of the RV in the apical four-chamber position, the shape of the RV changes from crescent-shaped to oval, and the apex of the heart may be occupied not by the LV, as is normal, but by the RV. Dilatation of the pancreas may be accompanied by dilatation of the trunk (more than 30 mm) and branches of the pulmonary artery. With massive thrombosis of the pulmonary artery, its significant dilatation (up to 50-80 mm) can be determined, and the lumen of the artery becomes oval.
2. With hypertrophy of the pancreas, the thickness of its anterior wall, measured in diastole in the subcostal four-chamber position in B- or M-mode, exceeds 5 mm. In patients with CHLS, as a rule, not only the anterior wall of the pancreas is hypertrophied, but also the interventricular septum.
3. Tricuspid regurgitation of varying degrees, which in turn causes dilatation of the right atrium and inferior vena cava, the decrease in inspiratory collapse of which indicates increased pressure in the right atrium.
4. Evaluation of the diastolic function of the pancreas is performed on the basis of the transtricuspid diastolic flow in the mode of pulsed
wave Doppler and color M-modal Doppler. In patients with CHLS, a decrease in the diastolic function of the pancreas is found, which is manifested by a decrease in the ratio of peaks E and A.
5. Reduced contractility of the pancreas in patients with LS is manifested by hypokinesia of the pancreas with a decrease in its ejection fraction. An echocardiographic study determines such indicators of RV function as end-diastolic and end-systolic volumes, ejection fraction, which normally is at least 50%.
These changes have different severity depending on the severity of the development of drugs. So, in acute LS, dilatation of the pancreas will be detected, and in chronic LS, signs of hypertrophy, diastolic and systolic dysfunction of the pancreas will be added to it.
Another group of signs is associated with the development of pulmonary hypertension in LS. The degree of their severity is most significant in acute and subacute LS, as well as in patients with primary pulmonary hypertension. CHLS is characterized by a moderate increase in systolic pressure in the pulmonary artery, which rarely reaches 50 mm Hg. Assessment of the pulmonary trunk and flow in the outflow tract of the pancreas is performed from the left parasternal and subcostal short-axis approach. In patients with pulmonary pathology, due to the limitation of the ultrasound window, the subcostal position may be the only possible access to visualize the outflow tract of the pancreas. Using pulsed wave Doppler, you can measure the average pressure in the pulmonary artery (Ppa), for which the formula proposed by A. Kitabatake et al. is usually used. (1983): Log10(Pra) = - 2.8 (AT/ET) + 2.4, where AT is the acceleration time of the flow in the outflow tract of the pancreas, ET is the ejection time (or the time of expulsion of blood from the pancreas). The Ppa value obtained using this method in patients with COPD correlates well with the data of an invasive examination, and the possibility of obtaining a reliable signal from the pulmonary valve exceeds 90%.
The most important for the detection of pulmonary hypertension is the severity of tricuspid regurgitation. The use of a jet of tricuspid regurgitation is the basis of the most accurate non-invasive method for determining systolic pressure in the pulmonary artery. Measurements are carried out in the continuous-wave Doppler mode in the apical four-chamber or subcostal position, preferably with the simultaneous use of color Doppler
whom mapping. To calculate the pressure in the pulmonary artery, it is necessary to add the pressure in the right atrium to the pressure gradient across the tricuspid valve. Measurement of the transtricuspid gradient can be performed in more than 75% of patients with COPD. There are qualitative signs of pulmonary hypertension:
1. With PH, the nature of the movement of the posterior cusp of the pulmonary valve changes, which is determined in the M-mode: a characteristic indicator of PH is the presence of an average systolic tooth due to partial overlap of the valve, which forms a W-shaped movement of the valve in systole.
2. In patients with pulmonary hypertension, due to increased pressure in the right ventricle, the interventricular septum (IVS) flattens, and the left ventricle resembles the letter D (D-shaped left ventricle) along the short axis. With a high degree of PH, the IVS becomes, as it were, the wall of the pancreas and moves paradoxically towards the left ventricle in diastole. When the pressure in the pulmonary artery and the right ventricle becomes more than 80 mm Hg, the left ventricle decreases in volume, is compressed by the dilated right ventricle and takes on the shape of a crescent.
3. Possible regurgitation on the pulmonary valve (regurgitation of the first degree is normal in young people). With a constant-wave Doppler study, it is possible to measure the rate of pulmonary regurgitation with a further calculation of the magnitude of the end-diastolic pressure gradient of the LA-RV.
4. Change in the shape of the blood flow in the outflow tract of the pancreas and at the mouth of the LA valve. At normal pressure in the LA, the flow has an isosceles shape, the peak of the flow is located in the middle of systole; in pulmonary hypertension, the peak flow shifts to the first half of systole.
However, in patients with COPD, their pulmonary emphysema often makes it difficult to clearly visualize the structures of the heart and narrows the echocardiogram window, making the study informative in no more than 60-80% of patients. In recent years, a more accurate and informative method of ultrasound examination of the heart has appeared - transesophageal echocardiography (TEE). TEE in patients with COPD is the preferred method for accurate measurements and direct visual assessment of the structures of the pancreas, due to the higher resolution of the transesophageal probe and the stability of the ultrasound window, and is of particular importance in emphysema and pneumosclerosis.
Catheterization of the right heart and pulmonary arteries
Right heart and pulmonary artery catheterization is the gold standard for diagnosing PH. This procedure allows you to directly measure right atrial and RV pressure, pulmonary artery pressure, calculate cardiac output and pulmonary vascular resistance, determine the level of mixed oxygenation venous blood. Catheterization of the right heart due to its invasiveness cannot be recommended for widespread use in the diagnosis of CHL. Indications are: severe pulmonary hypertension, frequent episodes of decompensated right ventricular failure, and selection of candidates for lung transplantation.
Radionuclide ventriculography (RVG)
RVG measures the right ventricular ejection fraction (REF). EFVC is considered abnormal below 40-45%, but the EFVC itself is not a good indicator of right ventricular function. It allows you to evaluate the systolic function of the right ventricle, which is highly dependent on afterload, decreasing with an increase in the latter. Therefore, a decrease in EFVC is recorded in many patients with COPD, and is not an indicator of true right ventricular dysfunction.
Magnetic resonance imaging (MRI)
MRI is a promising method for assessing pulmonary hypertension and changes in the structure and function of the right ventricle. An MRI-measured right pulmonary artery diameter greater than 28 mm is a highly specific sign of PH. However, the MRI method is quite expensive and is available only in specialized centers.
The presence of a chronic lung disease (as a cause of CLS) requires a special study of the function of external respiration. The doctor is faced with the task of clarifying the type of ventilation insufficiency: obstructive (impaired passage of air through the bronchi) or restrictive (decrease in the area of gas exchange). In the first case, chronic obstructive bronchitis, bronchial asthma can be cited as an example, and in the second - pneumosclerosis, lung resection, etc.
TREATMENT
CLS occurs most often after the onset of CLN. Therapeutic measures are complex in nature and are aimed mainly at correcting these two syndromes, which can be represented as follows:
1) treatment and prevention of the underlying disease - most often exacerbations of chronic pulmonary pathology (basic therapy);
2) treatment of CLN and PH;
3) treatment of right ventricular heart failure. Basic therapeutic and preventive measures include
prevention of acute viral diseases respiratory tract(vaccination) and smoking cessation. With the development of chronic pulmonary pathology of an inflammatory nature, it is necessary to treat exacerbations with antibiotics, mucoregulatory drugs and immunocorrectors.
The main thing in the treatment of chronic pulmonary heart is the improvement of the function of external respiration (elimination of inflammation, broncho-obstructive syndrome, improvement of the respiratory muscles).
The most common cause of CLN is broncho-obstructive syndrome, the cause of which is the contraction of the smooth muscles of the bronchi, the accumulation of viscous inflammatory secretions, and edema of the bronchial mucosa. These changes require the use of beta-2-agonists (fenoterol, formoterol, salbutamol), M-anticholinergics (ipratropium bromide, tiotropium bromide), and in some cases inhaled glucocorticosteroid drugs in the form of inhalations using a nebulizer or individual inhaler. It is possible to use methylxanthines (eufillin and prolonged theophyllines (teolong, teotard, etc.)). Therapy with expectorants is very individual and requires various combinations and selection of herbal remedies (coltsfoot, wild rosemary, thyme, etc.), and chemical production (acetylcysteine, ambroxol, etc.).
If necessary, exercise therapy and postural drainage of the lungs are prescribed. Breathing with positive expiratory pressure (no more than 20 cm of water column) is shown using both simple devices
in the form of "whistles" with a movable diaphragm, and complex devices that control the pressure on exhalation and inhalation. This method reduces the air flow inside the bronchus (which has a bronchodilator effect) and increases the pressure inside the bronchi in relation to the surrounding lung tissue.
The extrapulmonary mechanisms of CRF development include a decrease in contractile function respiratory muscles and diaphragm. The possibilities for correcting these disorders are still limited: exercise therapy or electrical stimulation of the diaphragm in stage II. HLN.
In CLN, erythrocytes undergo a significant functional and morphological reorganization (echinocytosis, stomatocytosis, etc.), which significantly reduces their oxygen transport function. In this situation, it is desirable to remove erythrocytes with lost function from the bloodstream and stimulate the release of young (functionally more capable). For this purpose, it is possible to use erythrocytepheresis, extracorporeal blood oxygenation, hemosorption.
Due to the increase in the aggregation properties of erythrocytes, blood viscosity increases, which requires the appointment of antiplatelet agents (chimes, reopoliglyukin) and heparin (preferably the use of low molecular weight heparins - fraxiparin, etc.).
In patients with hypoventilation associated with reduced activity of the respiratory center, drugs that increase central inspiratory activity - respiratory stimulants - can be used as auxiliary methods of therapy. They should be used for moderate respiratory depression that does not require the use of O 2 or mechanical ventilation (sleep apnea syndrome, obesity-hypoventilation syndrome), or when oxygen therapy is not possible. The few drugs that increase arterial blood oxygenation include nikethamide, acetosalamide, doxapram, and medroxyprogesterone, but all of these drugs have a large number of side effects with long-term use and therefore can only be used for a short time, such as during an exacerbation of the disease.
Almitrina bismesylate is currently among the drugs capable of correcting hypoxemia in patients with COPD for a long time. Almitrin is a specific ago-
nistome of peripheral chemoreceptors of the carotid node, the stimulation of which leads to an increase in hypoxic vasoconstriction in poorly ventilated regions of the lungs with an improvement in ventilation-perfusion ratios. The ability of almitrin at a dose of 100 mg / day has been proven. in patients with COPD, lead to a significant increase in paCO2 (by 5-12 mm Hg) and a decrease in paCO2 (by 3-7 mmHg) with an improvement in clinical symptoms and a decrease in the frequency of exacerbations of the disease, which is capable of several years to delay the appointment of long-term 0 2 therapy. Unfortunately, 20-30% of COPD patients do not respond to therapy, and widespread use is limited by the possibility of developing peripheral neuropathy and other side effects. Currently, the main indication for the appointment of almitrin is moderate hypoxemia in patients with COPD (pa0 2 56-70 mm Hg or Sa0 2 89-93%), as well as its use in combination with VCT, especially against the background of hypercapnia.
Vasodilators
In order to reduce the degree of PAH, peripheral vasodilators are included in the complex therapy of patients with cor pulmonale. The most commonly used calcium channel antagonists and nitrates. Two calcium antagonists currently recommended are nifedipine and diltiazem. The choice in favor of one of them depends on the initial heart rate. Patients with relative bradycardia should be recommended nifedipine, with relative tachycardia - diltiazem. The daily doses of these drugs, which have proven effective, are quite high: for nifedipine 120-240 mg, for diltiazem 240-720 mg. Favorable clinical and prognostic effects of calcium antagonists used in high doses in patients with primary PH (especially those with a previous positive acute test) have been shown. III generation dihydropyridine calcium antagonists - amlodipine, felodipine, etc. - are also effective in this group of patients with LS.
However, calcium channel antagonists are not recommended for COPD-associated pulmonary hypertension, despite their ability to reduce Ppa and increase cardiac output in this group of patients. This is due to the aggravation of arterial hypoxemia caused by dilatation of the pulmonary vessels in
poorly ventilated areas of the lungs with deterioration in ventilation-perfusion ratios. In addition, with long-term therapy with calcium antagonists (more than 6 months), the beneficial effect on the parameters of pulmonary hemodynamics is leveled.
A similar situation in patients with COPD occurs with the appointment of nitrates: acute samples demonstrate a deterioration in gas exchange, and long-term studies show the absence of a positive effect of drugs on pulmonary hemodynamics.
Synthetic prostacyclin and its analogues. Prostacyclin is a powerful endogenous vasodilator with antiaggregatory, antiproliferative and cytoprotective effects that are aimed at preventing pulmonary vascular remodeling (reducing endothelial cell damage and hypercoagulability). The mechanism of action of prostacyclin is associated with relaxation of smooth muscle cells, inhibition of platelet aggregation, improvement of endothelial function, inhibition of proliferation of vascular cells, as well as a direct inotropic effect, positive changes in hemodynamics, an increase in oxygen utilization in skeletal muscles. The clinical use of prostacyclin in patients with PH is associated with the synthesis of its stable analogues. To date, the greatest experience in the world has been accumulated for epoprostenol.
Epoprostenol is a form of intravenous prostacyclin (prostaglandin I 2). Favorable results were obtained in patients with vascular form of LS - with primary PH in systemic connective tissue diseases. The drug increases cardiac output and reduces pulmonary vascular resistance, and with long-term use improves the quality of life of patients with LS, increasing exercise tolerance. The optimal dose for most patients is 20-40 ng/kg/min. An analog of epoprostenol, treprostinil, is also used.
Oral formulations of a prostacyclin analogue have now been developed. (beraprost, iloprost) and clinical trials are being conducted in the treatment of patients with a vascular form of LS developed as a result of pulmonary embolism, primary pulmonary hypertension, and systemic connective tissue diseases.
In Russia, from the group of prostanoids for the treatment of patients with LS, only prostaglandin E 1 (vazaprostan) is currently available, which is prescribed intravenously
growth 5-30 ng/kg/min. Course treatment with the drug is carried out at a daily dose of 60-80 mcg for 2-3 weeks against the background of long-term therapy with calcium antagonists.
Endothelin receptor antagonists
Activation of the endothelin system in patients with PH was the rationale for the use of endothelin receptor antagonists. The effectiveness of two drugs of this class (bosentan and sitaczentan) in the treatment of patients with CPS, which developed against the background of primary PH or against the background of systemic connective tissue diseases, has been proven.
Phosphodiesterase type 5 inhibitors
Sildenafil is a powerful selective inhibitor of cGMP-dependent phosphodiesterase (type 5), preventing the degradation of cGMP, causes a decrease in pulmonary vascular resistance and right ventricular overload. To date, there are data on the effectiveness of sildenafil in patients with LS of various etiologies. When using sildenafil in doses of 25-100 mg 2-3 times a day, it caused an improvement in hemodynamics and exercise tolerance in patients with LS. Its use is recommended when other drug therapy is ineffective.
Long-term oxygen therapy
In patients with bronchopulmonary and thoracophrenic form of CLS, the main role in the development and progression of the disease belongs to alveolar hypoxia, therefore, oxygen therapy is the most pathogenetically substantiated method of treating these patients. The use of oxygen in patients with chronic hypoxemia is critical and must be continuous, long-term, and usually administered at home, hence this form of therapy is called long-term oxygen therapy (LTOT). The task of VCT is to correct hypoxemia with the achievement of pO 2 values >60 mm Hg. and Sa0 2 >90%. It is considered optimal to maintain paO 2 within 60-65 mm Hg, and exceeding these values leads only to a slight increase in Sa0 2 and oxygen content in arterial blood, however, it may be accompanied by CO 2 retention, especially during sleep, which has negative
effects on the function of the heart, brain and respiratory muscles. Therefore, VCT is not indicated for patients with moderate hypoxemia. Indications for VCT: raO 2<55 мм рт.ст. или Sa0 2 < 88% в покое, а также раО 2 56-59 мм рт.ст. или Sa0 2 89% при наличии легочного сердца или полицитемии (гематокрит >55%). For most patients with COPD, an O 2 flow of 1–2 l/min is sufficient, and in the most severe patients, the flow can be increased to 4–5 l/min. The oxygen concentration should be 28-34% vol. VCT is recommended for at least 15 hours per day (15-19 hours per day). The maximum breaks between oxygen therapy sessions should not exceed 2 hours in a row, because. breaks of more than 2-3 hours significantly increase pulmonary hypertension. Oxygen concentrators, liquid oxygen tanks and compressed gas cylinders can be used for VCT. The most commonly used concentrators (permeators) that release oxygen from the air by removing nitrogen. VCT increases the life expectancy of patients with CRF and CLS by an average of 5 years.
Thus, despite the presence of a large arsenal of modern pharmacological agents, VCT is the most effective method of treating most forms of CLS, so the treatment of patients with CLS is primarily the task of a pulmonologist.
Long-term oxygen therapy is the most effective method of treating CLN and HLS, increasing the life expectancy of patients by an average of 5 years.
Long-term home ventilation
In the terminal stages of pulmonary diseases, due to a decrease in the ventilation reserve, hypercapnia may develop, requiring respiratory support, which should be carried out for a long time, on an ongoing basis, at home.
NO inhalation therapy
Inhalation therapy with NO, whose action is similar to the endothelium-relaxing factor, has a positive effect in patients with CHD. Its vasodilating effect is based on the activation of guanylate cyclase in the smooth muscle cells of the pulmonary vessels, which leads to an increase in the level of cyclo-GMP and a decrease in the intracellular calcium content. Inhalation N0 region
gives a selective effect on the vessels of the lungs, and it causes vasodilation mainly in well-ventilated regions of the lungs, improving gas exchange. With the course application of NO in patients with chronic respiratory disease, there is a decrease in pressure in the pulmonary artery, an increase in the partial pressure of oxygen in the blood. In addition to its hemodynamic effects, NO prevents and reverses pulmonary vascular and pancreatic remodeling. The optimal doses of inhaled NO are concentrations of 2-10 ppm, and high concentrations of NO (more than 20 ppm) can cause excessive vasodilation of the pulmonary vessels and lead to a deterioration in the ventilation-perfusion balance with increased hypoxemia. The addition of NO inhalations to VCT in patients with COPD enhances the positive effect on gas exchange, reducing the level of pulmonary hypertension and increasing cardiac output.
CPAP therapy
Continuous Positive Airway Pressure Therapy (continuous positive airway pressure- CPAP) is used as a method of treatment for CRF and CLS in patients with obstructive sleep apnea syndrome, preventing the development of airway collapse. The proven effects of CPAP are the prevention and resolution of atelectasis, an increase in lung volumes, a decrease in ventilation-perfusion imbalance, an increase in oxygenation, lung compliance, and redistribution of fluid in lung tissue.
cardiac glycosides
Cardiac glycosides in patients with COPD and cor pulmonale are effective only in the presence of left ventricular heart failure, and may also be useful in the development of atrial fibrillation. Moreover, it has been shown that cardiac glycosides can induce pulmonary vasoconstriction, and the presence of hypercapnia and acidosis increases the likelihood of glycoside intoxication.
Diuretics
In the treatment of patients with decompensated CHLS with edematous syndrome, diuretic therapy, including antagonists, is used.
aldosterone (aldactone). Diuretics should be administered cautiously, at low doses, because in the development of RV failure, cardiac output is more dependent on preload, and, therefore, an excessive reduction in intravascular fluid volume can lead to a decrease in RV filling volume and a decrease in cardiac output, as well as an increase in blood viscosity. and a sharp decrease in pressure in the pulmonary artery, thereby worsening the diffusion of gases. Another serious side effect of diuretic therapy is metabolic alkalosis, which in patients with COPD with respiratory failure can lead to inhibition of the activity of the respiratory center and deterioration of gas exchange.
Angiotensin-converting enzyme inhibitors
In the treatment of patients with decompensated cor pulmonale in recent years, angiotensin-converting enzyme inhibitors (ACE inhibitors) have come to the fore. ACE inhibitor therapy in patients with CHLS leads to a decrease in pulmonary hypertension and an increase in cardiac output. In order to select an effective therapy for CLS in patients with COPD, it is recommended to determine the polymorphism of the ACE gene, because only in patients with subtypes of the ACE II and ID gene, a pronounced positive hemodynamic effect of ACE inhibitors is observed. The use of ACE inhibitors in minimal therapeutic doses is recommended. In addition to the hemodynamic effect, there is a positive effect of ACE inhibitors on the size of the heart chambers, remodeling processes, exercise tolerance and increased life expectancy in patients with heart failure.
Angiotensin II receptor antagonists
In recent years, data have been obtained on the successful use of this group of drugs in the treatment of CLS in patients with COPD, which was manifested by an improvement in hemodynamics and gas exchange. The appointment of these drugs is most indicated in patients with CLS with intolerance to ACE inhibitors (due to dry cough).
Atrial septostomy
Recently, in the treatment of patients with right ventricular heart failure that developed against the background of primary PH, there have been
use an atrial septostomy, i.e. creation of a small perforation in the interatrial septum. Creating a right-to-left shunt allows you to reduce the average pressure in the right atrium, unload the right ventricle, increase the left ventricular preload and cardiac output. Atrial septostomy is indicated when all types of medical treatment of right ventricular heart failure are ineffective, especially in combination with frequent syncope, or as a preparatory stage before lung transplantation. As a result of the intervention, there is a decrease in syncope, an increase in exercise tolerance, but the risk of developing life-threatening arterial hypoxemia increases. The mortality rate of patients during atrial septostomy is 5-15%.
Lung or heart-lung transplant
From the end of the 80s. In the 20th century, after the introduction of the immunosuppressive drug cyclosporine A, lung transplantation began to be successfully used in the treatment of end-stage pulmonary insufficiency. In patients with CLN and LS, transplantation of one or both lungs, the heart-lung complex is performed. It was shown that 3 and 5-year survival after transplantation of one or both lungs, heart-lung complex in patients with LS was 55 and 45%, respectively. Most centers prefer to perform bilateral lung transplantation due to fewer postoperative complications.
Guidelines are given on the clinic, diagnosis and treatment of cor pulmonale. Recommendations are addressed to students of 4-6 courses. The electronic version of the publication is available on the SPbGMU website (http://www.spb-gmu.ru).
Guidelines are addressed to students of 4-6 courses Chronic cor pulmonale Under chronic cor pulmonale
Ministry of Health and Social Development
Russian Federation
G OU HPE "SAINT PETERSBURG STATE
MEDICAL UNIVERSITY
NAMED AFTER ACADEMICIAN I.P. PAVLOV"
Associate Professor V.N. Yablonskaya
Associate Professor O.A. Ivanova
assistant Zh.A. Mironova
Editor: Head Department of Hospital Therapy, St. Petersburg State Medical University. acad. I.P. Pavlova Professor V.I. Trofimov
Reviewer: Professor of the Department of Propaedeutics of Internal Diseases
SPbGMU im. acad. I.P. Pavlova B.G. Lukichev
Chronic cor pulmonale
Under chronic cor pulmonale (HLS) understand right ventricular (RV) hypertrophy, or a combination of hypertrophy with dilatation and/or right ventricular heart failure (RVF) due to diseases that primarily affect lung function or structure, or both, and not associated with primary left heart failure or congenital or acquired heart defects.
This definition of the WHO expert committee (1961), according to a number of experts, currently needs to be corrected due to the introduction into practice modern methods diagnostics and accumulation of new knowledge about the pathogenesis of CLS. In particular, CHLS is proposed to be considered as pulmonary hypertension in combination with hypertrophy. dilatation of the right ventricle, dysfunction of both ventricles of the heart associated with primary structural and functional changes in the lungs.
Pulmonary hypertension (PH) is said to be when the pressure in the pulmonary artery (PA) exceeds established normal values:
Systolic - 26 - 30 mm Hg.
Diastolic - 8 - 9 mm Hg.
Average - 13 - 20 mm Hg.St.
Chronic cor pulmonale is not an independent nosological form, but it complicates many diseases that affect the airways and alveoli, the chest with limited mobility, and pulmonary vessels. In essence, all diseases that can lead to the development of respiratory failure and pulmonary hypertension (there are more than 100 of them) can cause chronic cor pulmonale. At the same time, in 70-80% of cases, chronic obstructive pulmonary disease (COPD) accounts for CLS. Currently, chronic cor pulmonale is observed in 10-30% of pulmonological patients hospitalized in a hospital. It is 4-6 times more common in men. Being a severe complication of chronic obstructive pulmonary disease (COPD), HLS determines the clinic, course and prognosis of this disease, leads to early disability of patients and often serves as a cause deaths. Moreover, the mortality rate in patients with CLS over the past 20 years has increased by 2 times.
ETIOLOGY AND PATHOGENESIS OF CHRONIC PULMONARY HEART.
Since chronic cor pulmonale is a condition that occurs secondarily and is essentially a complication of a number of respiratory diseases, the following types of CHLS are usually distinguished in accordance with the primary causes:
1. Bronchopulmonary:
The reason is diseases that affect the airways and alveoli:
Obstructive diseases (chronic obstructive pulmonary disease (COPD), primary pulmonary emphysema, bronchial asthma severe course with significant irreversible obstruction)
Diseases that occur with severe pulmonary fibrosis (tuberculosis, bronchiectasis, pneumoconiosis, repeated pneumonia, radiation damage)
Interstitial lung diseases (idiopathic fibrosing alveolitis, lung sarcoidosis, etc.), collagenosis, lung carcinomatosis
2. Thoracodiaphragmatic:
The reason is diseases that affect the chest (bones, muscles, pleura) and affect the mobility of the chest:
Chronic cor pulmonale: the view of cardiologists
Prepared by Maxim Gvozdyk | 03/27/2015
The prevalence of chronic obstructive pulmonary disease (COPD) is rapidly increasing worldwide: if
in 1990 they were in twelfth place in the structure of morbidity, then according to WHO experts, by 2020 they will move into the top five after such pathologies as coronary heart disease (CHD), depression, injuries due to traffic accidents and cerebrovascular disease. It is also predicted that by 2020 COPD will take the third place in the structure of causes of death. Coronary artery disease, arterial hypertension and obstructive pulmonary disease are often combined, which gives rise to a number of problems in both pulmonology and cardiology. November 30, 2006
at the Institute of Phthisiology and Pulmonology named after F.G. Yanovsky of the Academy of Medical Sciences of Ukraine, a scientific-practical conference “Peculiarities of diagnosis and treatment of obstructive pulmonary diseases with concomitant pathology” was held
cardiovascular system”, during which much attention was paid to the general problems of cardiology
and pulmonology.
The report "Heart failure in chronic cor pulmonale: a cardiologist's point of view" was presented by
Corresponding Member of the Academy of Medical Sciences of Ukraine, Doctor of Medical Sciences, Professor Ekaterina Nikolaevna Amosova .
- In modern cardiology and pulmonology, there are a number of common problems in respect of which it is necessary to reach a consensus and unify approaches. One of them is chronic cor pulmonale. Suffice it to say that dissertations on this topic are equally often defended both in cardiological and pulmonological councils, it is included in the list of problems dealt with by both branches of medicine, but unfortunately, a unified approach to this pathology has not yet been developed. Let's not forget general practitioners and family doctors who find it difficult to understand the contradictory information and information printed in the pulmonological and cardiology literature.
The definition of chronic cor pulmonale in a WHO document dates back to 1963. Unfortunately, since that time, WHO recommendations on this important issue have not been clarified or reaffirmed, which, in fact, led to discussions and contradictions. Today, there are practically no publications on chronic cor pulmonale in the foreign cardiological literature, although there is a lot of talk about pulmonary hypertension, moreover, the recommendations of the European Society of Cardiology regarding pulmonary hypertension have recently been revised and approved.
The concept of "cor pulmonale" includes extremely heterogeneous diseases, they differ in etiology, the mechanism of development of myocardial dysfunction, its severity, and have different approaches to treatment. Chronic cor pulmonale is based on both hypertrophy, dilatation, and dysfunction of the right ventricle, which, by definition, are associated with pulmonary hypertension. The heterogeneity of these diseases is even more obvious if we consider the degree of increase in pressure in the pulmonary artery in pulmonary hypertension. In addition, its very presence has a completely different meaning for various etiological factors of chronic pulmonary heart disease. So, for example, in vascular forms of pulmonary hypertension, this is the basis that requires treatment, and only a decrease in pulmonary hypertension can improve the patient's condition; in COPD - pulmonary hypertension is not so pronounced and does not need treatment, as evidenced by Western sources. Moreover, a decrease in pressure in the pulmonary artery in COPD does not lead to relief, but worsens the patient's condition, as there is a decrease in blood oxygenation. Thus, pulmonary hypertension is an important condition for the development of chronic cor pulmonale, but its importance should not be absolute.
Often this pathology becomes the cause of chronic heart failure. And if we talk about it with cor pulmonale, then it is worth recalling the criteria for diagnosing heart failure (HF), which are reflected in the recommendations of the European Society of Cardiology. To make a diagnosis, there must be: firstly, the symptoms and clinical signs of heart failure, and secondly, objective signs of systolic or diastolic myocardial dysfunction. That is, the presence of dysfunction (changes in myocardial function at rest) is mandatory for diagnosis.
The second question is the clinical symptoms of chronic cor pulmonale. In the cardiology audience, it is necessary to say that edema does not correspond to the fact of the presence of right ventricular failure. Unfortunately, cardiologists are very little aware of the role of non-cardiac factors in the origin of clinical signs of venous congestion in big circle circulation. Edema in such patients is often perceived as a manifestation of heart failure, it is actively treated, but to no avail. This situation is well known to pulmonologists.
The pathogenetic mechanisms of the development of chronic cor pulmonale also include non-cardiac factors of blood deposition. Of course, these factors are important, but you should not overestimate them and associate everything only with them. And finally, we talk little, in essence, just started, about the role of hyperactivation of the renin-angiotensin-aldosterone system and its significance in the development of edema and hypervolemia.
In addition to these factors, it is worth mentioning the role of myocardiopathy. In the development of chronic pulmonary heart, a large role is played by myocardial damage not only of the right ventricle, but also of the left, which occurs under the influence of a complex of factors, including toxic, which is associated with bacterial agents, in addition, it is a hypoxic factor that causes dystrophy of the myocardium of the ventricles of the heart .
In the course of our studies, it was found that there is practically no correlation between systolic pressure in the pulmonary artery and the size of the right ventricle in patients with chronic cor pulmonale. There is some correlation between the severity of COPD and impaired right ventricular function, with respect to the left ventricle, these differences are less pronounced. When analyzing the systolic function of the left ventricle, its deterioration was noted in patients with severe COPD. The contractility of the myocardium, even of the left ventricle, is extremely difficult to correctly assess, because the indices that we use in clinical practice are very rough and depend on pre- and afterload.
As for the indicators of diastolic function of the right ventricle, all patients were diagnosed with a hypertrophic type of diastolic dysfunction. The indicators from the right ventricle are expected, but from the left side, we somewhat unexpectedly received signs of impaired diastolic relaxation, which increased depending on the severity of COPD.
Indicators of ventricular systolic function in patients with COPD and idiopathic pulmonary hypertension are different. Of course, changes in the right ventricle are more pronounced in idiopathic pulmonary hypertension, while the systolic function of the left ventricle is more changed in COPD, which is associated with the effect of adverse factors of infection and hypoxemia on the myocardium of the left ventricle, and then it makes sense to talk about cardiopathy in that broad sense present in cardiology today.
In our study, all patients had type I disorders of diastolic function of the left ventricle, peak rates were more pronounced in the right ventricle in patients with idiopathic pulmonary hypertension, diastolic disorders in patients with COPD. It is worth emphasizing that these are relative indicators, because we took into account the different ages of patients.
Echocardiography of all patients measured the diameter of the inferior vena cava and determined the degree of its collapse during inspiration. It was found that in moderate COPD, the diameter of the inferior vena cava is not increased, it increased only in severe COPD, when FEV1 is less than 50%. This allows us to raise the question that the role of extracardiac factors should not be absolutized. At the same time, the collapse of the inferior vena cava on inspiration was already disturbed in moderate COPD (this indicator reflects an increase in pressure in the left atrium).
We also analyzed heart rate variability. It should be noted that cardiologists consider a decrease in heart rate variability to be a marker of activation of the sympathoadrenal system, the presence of heart failure, that is, a poor prognostic indicator. We found a decrease in variability in moderate COPD, the severity of which increased in accordance with obstructive disorders of the ventilation function of the lungs. Moreover, we found a significant correlation between the severity of heart rate variability disorder and right ventricular systolic function. This suggests that heart rate variability in COPD appears quite early and can serve as a marker of myocardial damage.
When diagnosing chronic cor pulmonale, especially in pulmonary patients, it is necessary to pay great attention to the instrumental study of myocardial dysfunction. In this regard, echocardiography is the most convenient study in clinical practice, although there are limitations for its use in patients with COPD, in which, ideally, radionuclide ventriculography of the right ventricle should be used, which combines relatively low invasiveness and very high accuracy.
Of course, it is not news to anyone that chronic cor pulmonale in COPD and idiopathic pulmonary hypertension is very heterogeneous in terms of the morphofunctional state of the ventricles, prognosis and a number of other reasons. The existing European classification of heart failure, which was included in the document of the Ukrainian Society of Cardiology almost unchanged, does not reflect the difference in the mechanisms of development of this disease. If these classifications were convenient in clinical practice, we would not be discussing this topic. It seems logical to us to leave the term "chronic pulmonary heart" for bronchopulmonary pathology, to emphasize - decompensated, subcompensated and compensated. This approach will avoid the use of the terms FK and CH. In vascular forms of chronic pulmonary heart (idiopathic, post-thromboembolic pulmonary hypertension), it is advisable to use the approved HF gradation. However, it seems to us appropriate, by analogy with cardiology practice, to indicate the presence of right ventricular systolic dysfunction in the diagnosis, because this is important for chronic cor pulmonale associated with COPD. If the patient does not have dysfunction, this is one situation in the prognostic and treatment plans, if there is, the situation is significantly different.
Cardiologists of Ukraine have been using the Strazhesko-Vasilenko classification when making a diagnosis of chronic heart failure for several years, necessarily indicating whether the systolic function of the left ventricle is preserved or reduced. So why not use it for chronic cor pulmonale?
Doctor of Medical Sciences, Professor Yuri Nikolaevich Sirenko devoted his speech to the peculiarities of the treatment of patients with coronary artery disease and arterial hypertension in combination with COPD.
– In preparation for the conference, I tried to find on the Internet references over the past 10 years to pulmonogenic arterial hypertension, a nosology that often appeared in the USSR. I managed to find about 5 thousand references to arterial hypertension in chronic obstructive pulmonary diseases, but the problem of pulmonogenic arterial hypertension does not exist anywhere in the world, except for the post-Soviet countries. To date, there are several positions regarding the diagnosis of the so-called pulmonogenic arterial hypertension. They were developed in the early 1980s, when more or less reliable functional research methods appeared.
The first position is the development of pulmonary arterial hypertension 5-7 years after the onset of chronic lung disease; the second is the relationship between an increase in blood pressure and an exacerbation of COPD; the third is an increase in blood pressure due to increased bronchial obstruction; fourth - with daily monitoring, a relationship is revealed between an increase in blood pressure and inhalation of sympathomimetics; fifth - high variability of blood pressure during the day with a relatively low average level.
I managed to find a very serious work of the Moscow Academician E.M. Tareeva "Does pulmonary arterial hypertension exist?", in which the author makes a mathematical assessment of the possible relationship of the above factors in patients with arterial hypertension and COPD. And no dependency found! The results of the studies did not confirm the existence of independent pulmonogenic arterial hypertension. Moreover, E.M. Tareev believes that systemic arterial hypertension in patients with COPD should be considered as hypertension.
After such a categorical conclusion, I looked at the world's recommendations. In the modern recommendations of the European Society of Cardiology there is not a single line about COPD, the American ones (seven recommendations of the National Joint Committee) also do not say anything on this topic. It was possible to find only in the American recommendations of 1996 (in six editions) information that non-selective beta-blockers should not be used in patients with COPD, and if there is a cough, ACE inhibitors should be replaced with angiotensin receptor blockers. That is, there really is no such problem in the world!
Then I reviewed the statistics. It turned out that they started talking about pulmonary arterial hypertension after it was established that approximately 35% of patients with COPD have high blood pressure. Today, Ukrainian epidemiology gives the following figures: among the adult rural population, blood pressure is increased in 35%, in urban - in 32%. We cannot say that COPD increases the incidence of arterial hypertension, so we should not talk about pulmonogenic arterial hypertension, but about some specifics of the treatment of arterial hypertension in COPD.
Unfortunately, in our country, sleep apnea syndrome, in addition to the Institute of Phthisiology and Pulmonology named after. F.G. Yanovsky of the Academy of Medical Sciences of Ukraine, practically nowhere are they engaged. This is due to the lack of equipment, money and the desire of specialists. And this question is very important and represents another problem where cardiac pathology intersects with the pathology of the respiratory tract and there is a very high percentage of the risk of developing cardiovascular complications and death. Pulmonary hypertension, heart and respiratory failure complicate and worsen the course of arterial hypertension and, most importantly, worsen the ability to treat patients.
I would like to start a conversation about the treatment of arterial hypertension with a simple algorithm, which is the basis for cardiologists and therapists. Before a doctor who meets a patient with hypertension, questions arise: what form of arterial hypertension does the patient have - primary or secondary - and are there signs of target organ damage and cardiovascular risk factors? By answering these questions, the doctor knows the tactics of treating the patient.
To date, there are no randomized clinical trial, which was specifically organized to clarify the tactics of treating arterial hypertension in COPD, so current recommendations are based on three very unreliable factors: retrospective analysis, expert opinion and the doctor's own experience.
Where should treatment begin? Of course, with first-line antihypertensive drugs. The first and main group of them is beta-blockers. Many questions arise regarding their selectivity, but there are already drugs with a fairly high selectivity, confirmed in the experiment and the clinic, which are safer than the drugs that we used before.
When assessing the patency of the respiratory tract in healthy people after taking atenolol, a deterioration in the response to salbutamol and minor changes when taking more modern drugs. Although, unfortunately, such studies have not been conducted with the participation of patients, the categorical ban on the use of beta-blockers in patients with COPD should still be lifted. They should be prescribed if the patient tolerates them well, it is advisable to use them in the treatment of arterial hypertension, especially in combination with coronary artery disease.
The next group of drugs is calcium antagonists, they are almost ideal for the treatment of such patients, but it must be remembered that non-dihydropyridine drugs (diltiazem, verapamil) should not be used for high blood pressure in the pulmonary artery system. They have been shown to worsen the course of pulmonary hypertension. The remaining dihydropyridines are known to improve bronchial patency and thus may reduce the need for bronchodilators.
Today, all experts agree that ACE inhibitors do not impair airway patency, do not cause cough in patients with COPD, and if it occurs, patients should be transferred to angiotensin receptor blockers. We did not conduct special studies, but based on the literature data and our own observations, it can be argued that the experts are a little cunning, since a certain number of patients with COPD react with a dry cough to ACE inhibitors, and there is a serious pathogenetic reason for this.
Unfortunately, very often one can observe the following picture: a patient with high blood pressure goes to a cardiologist, he is prescribed ACE inhibitors; after some time, the patient begins to cough, goes to a pulmonologist, who cancels ACE inhibitors, but does not prescribe angiotensin receptor blockers. The patient again gets to the cardiologist, and everything starts all over again. The reason for this situation is the lack of control over appointments. It is necessary to move away from this practice, therapists and cardiologists should take a comprehensive approach to treating the patient.
Another very important point in the treatment of patients, which allows to reduce the possibility of side effects, is the use of lower doses. Modern European guidelines give the right to choose between low doses of one or two drugs. Today, the great effectiveness of a combination of different drugs has been proven, which affects various links in pathogenesis, mutually reinforcing the effect medicines. I think that's exactly combination therapy for patients with COPD is the choice in the treatment of arterial hypertension.
Belarusian State Medical University
Trisvetova E.L.
Belarusian State Medical University, Minsk, Belarus
Pulmonary hypertension in the new (2015)
Recommendations European Society of Cardiology
Summary. The ESC/ERS 2015 guidelines, based on an analysis of studies performed since the publication of the previous edition, highlight the main points of pulmonary hypertension from the standpoint of evidence-based medicine: an improved classification of physician strategy in a specific clinical situation, taking into account the patient's disease outcome, the risk-benefit ratio of diagnostic procedures and medicinal agents.
Keywords: pulmonary hypertension, pulmonary arterial hypertension, classification, diagnosis, treatment.
summary. The recommendations of the ESC/ERS 2015 based on the analysis of studies carried out since the publication of the previous edition, highlights the key provisions of pulmonary hypertension with evidence-based medicine: an improved classification strategy by the physician in a particular clinical situation, taking into account the outcome of the disease in a patient, the risks and benefits of diagnostic procedures and medical means.
keywords: pulmonary hypertension, pulmonary arterial hypertension, classification, diagnosis, treatment.
The European Society of Cardiology (ESC) Congress, held from August 29 to September 2, 2015 in London, was packed with events and reports on the results of scientific research, approved five new recommendations for clinical practice: prevention, diagnosis and treatment of infective endocarditis; ventricular arrhythmias and prevention of sudden cardiac death; diagnosis and treatment of diseases of the pericardium; treatment of patients with acute coronary syndrome without displacement and elevation of the ST segment; diagnosis and treatment of pulmonary hypertension.
In the recommendations (2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension), covering the problems of pulmonary hypertension, based on an analysis of studies performed since the publication of the previousthe next edition (Guidelines for the diagnosis and treatment of pulmonary hypertension ESC, ERS, ISHLT, 2009), from the standpoint of evidence-based medicine, the doctor's strategy in a specific clinical situation is presented, taking into account the patient's disease outcome, the risk-benefit ratio of diagnostic procedures and therapeutic agents.
Pulmonary hypertension (PH) is a pathophysiological condition that complicates many cardiovascular and respiratory diseases.
Definition and classification
PH is diagnosed with an increase in meanpulmonary artery pressure (PAPm) ≥25 mmHg Art. at rest according to the results of right heart catheterization. Normal PAPm at rest is 14±3 mmHg. Art. with an upper limit of 20 mm Hg. Art. The clinical value of PAPm in the range of 21-24 mm Hg. Art. undefined. In the absence of obvious clinical signs of diseases accompanied by PH, patients with PAPm values in the indicated interval should be monitored.
The term "pulmonary arterial hypertension" nzia (PAH) is used to characterize groups of patients with hemodynamic disorders in the form of precapillary PH, which are characterized by wedge pressure (PAWP) ≤15 mm Hg. Art. and pulmonary vascular resistance (PVR) >3 units. Wood in the absence of other causes (lung disease, chronic thromboembolic PH, rare diseases, etc.).
According to a combination of PAP, PAWP, cardiac output, diastolic pressure gradient, and PVR measured at rest, PH is classified by hemodynamic parameters (Table 1).
Table 1. Hemodynamic classification of pulmonary hypertension
|
Name (definition) |
Characteristic |
|
|
PAPm ≥25 mmHg Art. |
||
|
Precapillary LH |
PAPm ≥25 mmHg Art. PAWR ≤15mmHg Art. |
1. Pulmonary arterial hypertension 3. PH due to lung disease 4. Chronic thromboembolic PH 5. PH of unknown origin or with multifactorial mechanisms |
|
Postcapillary LH Isolated postcapillary PH Combined pre- and post-capillary LH |
PAPm ≥25 mmHg Art. PAWR ≤15mmHg Art. DPG<7 мм рт. ст. и/или PVR ≤3 units Wood DPG<7 мм рт. ст. и/или PVR ≤3 units Wood |
2. PH caused by diseases of the left heart 5. PH of unknown origin or with multifactorial mechanisms |
Note: PAPm - mean pulmonary artery pressure, PAWP - wedge pressure, PVR - pulmonary vascular resistance, DPG - diastolic pressure gradient (diastolic pressure in the pulmonary artery - mean pressure in the pulmonary artery).
The clinical classification of PH includes five groups of conditions united by similar pathophysiological, clinical, hemodynamic characteristics and treatment strategies (Table 2).
Table 2. Clinical classification of pulmonary hypertension
|
1. Pulmonary arterial hypertension |
|
1.1. idiopathic 1.2. Family 1.2.1. BMPR2-mutations 1.2.2. Other mutations 1.3. Induced by drugs or toxins 1.4. Associated with: 1.4.1. Connective tissue diseases 1.4.2. HIV infection 1.4.3. portal hypertension 1.4.4. Congenital heart defects 1.4.5. Schistosomiasis |
|
1 ?. Pulmonary veno-occlusive disease with/without pulmonary capillary hemangiomatosis |
|
1?.1. idiopathic 1?.2. congenital 1?.2.1.EIF2AK4- mutations 1?.2.2. Other mutations 1?.3. Induced by drugs, toxins or radiation 1?.4. Associated with: 1?.4.1. Connective tissue diseases one?. 4.2. HIV infection |
|
1 ??. Persistent pulmonary hypertension of the newborn |
|
2. Pulmonary hypertension due to diseases of the left heart |
|
2.1. Systolic dysfunction of the left ventricle 2.2. Left ventricular diastolic dysfunction 2.3. Valvular disease 2.4. Congenital/acquired left ventricular inflow/outflow tract obstruction and congenital cardiomyopathy 2.5. Congenital/acquired pulmonary vein stenosis |
|
3. Pulmonary hypertension associated with lung disease and/or hypoxemia |
|
3.1. Chronic obstructive pulmonary disease (COPD) 3.2. Interstitial lung disease 3.3. Other pulmonary diseases with mixed restrictive and obstructive disorders 3.4. Breathing disorders during sleep 3.5. Diseases with alveolar hypoventilation 3.6. Long stay in the highlands 3.7. Development-related lung disease |
|
4. Chronic thromboembolic pulmonary hypertension or other pulmonary arterial obstruction |
|
4.1. Chronic thromboembolic pulmonary hypertension 4.2. Other pulmonary arterial obstruction 4.2.1. Angiosarcoma 4.2.2. Other intravascular tumors 4.2.3. Arteritis 4.2.4. Congenital pulmonary arterial stenosis |
|
5. Pulmonary hypertension with unknown cause or multifactorial mechanisms |
|
5.1. Blood diseases: chronic hemolytic anemia, myeloproliferative diseases, splenectomy 5.2. Systemic diseases: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis 5.3. Metabolic diseases: glycogen storage diseases, Gaucher disease, diseases thyroid gland 5.4. Others: pulmonary tumor obstructive microangiopathy, fibrosing mediastinitis, chronic renal failure (with/without hemodialysis), segmental pulmonary hypertension |
Note: BMPR2 - bone morphogenetic protein receptor, type 2, receptors for bone morphogenetic protein; EIF2AK4 - eukaryotic. translation initiation factor-2-alpha-kinase-4 is a family of kinases that phosphorylate the alpha subunit of the eukaryotic translation initiation factor-2.
Epidemiologyand risk factors for PH
Data on the prevalence of PH are inconclusive. In the UK, there are 97 cases of PH per 1 million people; 1.8 times more among women than among men. In the United States, the age-standardized mortality rate for patients with PH ranges from 4.5 to 12.3 per 100,000 population. Comparative epidemiological studies of various groups of patients with PH have shown that the disease is not widespread, the most common group 2 is non-severe PH due to diseases of the left heart.
RAS (Group 1) is rare: according to research results, in Europe there are 15-60 cases per 1 million population, the incidence during the year is 5-10 cases per 1 million population. In registries, 50% of patients are diagnosed with idiopathic, hereditary, or drug-induced RAS. Among connective tissue diseases with associated RAS, the main cause is systemic sclerosis. Idiopathic RAS refers to sporadic disease without a family history of PH or a known trigger. It is more often diagnosed in the elderly, the average age of patients is 50-65 years (in the register of 1981, the average age of diagnosis is 36 years). The predominance of women in old age, according to research, is doubtful.
Table 3. Risk factors for the development of pulmonary hypertension
Note: * - increased risk of persistent PH in newborns from mothers who used selective serotonin reuptake inhibitors; ** - alkylating agents as a possible cause of pulmonary veno-occlusive disease.
The prevalence of PH in patients of the 2nd group increases with the appearance and progression of signs of heart failure. An increase in pulmonary artery pressure is determined in 60% of patients with severe left ventricular systolic dysfunction and in 70% of patients with heart failure and preserved left ventricular ejection fraction.
In diseases of the lungs and / or hypoxemia (group 3), mild, rarely - severe PH is common, mainly with a combination of emphysema and widespread fibrosis.
The prevalence of PH in chronic thromboembolism is 3.2 cases per 1 million population. In survivors of acute pulmonary embolism, PH is diagnosed in 0.5-3.8% of cases.
Diagnosis of PH
The diagnosis of PH is based on clinical findings, including history, symptom development, and physical examination. An important role in confirming the diagnosis of PH is given to the results of instrumental research methods, interpreted by experienced specialists. The diagnostic algorithm is built on the results confirming or excluding diseases accompanied by the development of PH (groups 2-5).
Clinical signs
Clinical symptoms PH is nonspecific and is mainly due to progressive dysfunction of the right ventricle. Initial symptoms: shortness of breath, fatigue, weakness, angina pectoris pain in the heart area, syncope - are associated with stress, later they occur at rest. An increase in the abdomen, swelling of the ankles indicate the development of right ventricular heart failure.
Some patients develop symptoms (hemoptysis, hoarseness, remote wheezing, angina pectoris) associated with mechanical complications resulting from abnormal redistribution of blood flow in the pulmonary vascular bed.
Physical signs: pulsation to the left of the sternum along the parasternal line in the fourth intercostal space, an increase in the right ventricle, with auscultation of the heart - an increase in the second tone in the second intercostal space on the left, pansystolic murmur in case of tricuspid insufficiency, Graham-Still murmur. An increase in venous pressure is manifested by a pulsation of the cervical veins, there are signs of right ventricular failure - hepatomegaly, peripheral edema, ascites. A clinical study will reveal the disease that caused PH: COPD - “barrel-shaped” chest, changes in the distal phalanges of the fingers - “drum sticks” and “watch glasses”; with interstitial lung diseases - "cellophane" wheezing during auscultation of the lungs; with hereditary hemorrhagic telangiectasia and systemic sclerosis - telangiectasia on the skin and mucous membranes, digital ulcers and/or sclerodactyly; with liver diseases - palmar erythema, testicular atrophy, telangiectasia, etc.
Instrumental research methods
The results of electrocardiography confirm the diagnosis, but do not exclude it, in the absence of pathological changes on the ECG.In severe PH, there is a deviation of the electrical axis of the heart to the right, "pulmonary" R, signs of right ventricular hypertrophy (sensitivity - 55%, specificity - 70%), blockade of the right bundle branch block, prolongation of the QT interval. Often, cardiac arrhythmias (supraventricular extrasystole, flutter or atrial fibrillation) are noted, exacerbating hemodynamic disorders and contributing to the progression of heart failure.
On chest x-ray in 90% of cases of idiopathic pulmonary arterial hypertension characteristic changes are revealed: expansion of the main branches of the pulmonary artery, contrasting with the depletion of the peripheral pulmonary pattern, an increase in the right heart (late stages). X-ray examination helps in differential diagnosis PH, since there are signs of lung diseases (group 3), characteristic of arterial and venous hypertension. The degree of PH does not correlate with the degree of radiological changes.
When examining the function of external respiration Anemia and gas composition of arterial blood determine the contribution of diseases of the respiratory tract and lung parenchyma to the development of PH. Patients with pulmonary arterial hypertension have a mild or moderate decrease in lung volumes, depending on the severity of the disease, normal or slightly reduced diffusive capacity of the lungs for carbon monoxide (DLCO). Low DLCO (<45% от должного) свидетельствуют о плохом прогнозе. При ЛГ, обусловленной ХОБЛ, выявляют признаки необратимой обструкции, увеличение остаточного объема легких и снижение показателя DLCO.
In COPD, interstitial lung diseases, changes in the gas composition of arterial blood include a decrease in PaO 2, an increase in PaCO 2. With a combination of pulmonary emphysema and pulmonary fibrosis, it is possible to obtain pseudo-normal spirometry indicators, a decrease in DLCO indicators will indicate a violation of the functional state of the lungs.
Given the significant prevalence of PH (70-80%) in nocturnal hypoxemia and central obstructive sleep apnea, oximetry or polysomnography is necessary to clarify the diagnosis.
Transthoracic echocardio method-graphs evaluate the state of the heart muscle and heart chambers to detect hypertrophy and dilatation of the right heart, diagnose the pathology of the myocardium and valvular apparatus, and hemodynamic disorders (Table 4). Evaluation of tricuspid regurgitation and changes in the diameter of the inferior vena cava during respiratory maneuvers is carried out with a Doppler study to calculate the mean systolic pressure in the pulmonary artery. Transthoracic echocardiography is not enough to judge mild or asymptomatic PH due to methodological inaccuracies of the study and the individual characteristics of patients. In a clinical context, the results of an echocardiographic study are essential to the decision to perform cardiac catheterization.
Table 4. Echocardiographic features suggestive of PH (in addition to changes in tricuspid regurgitation rate)
|
Ventricles of the heart |
pulmonary artery |
inferior vena cava and right atrium |
|
Right ventricular/left ventricular basal diameter ratio >1.0 |
Acceleration of outflow from the right ventricle< 105 мс and/or midsystolic notching |
Inferior vena cava diameter >21 mm with reduced inspiratory collapse |
|
Displacement of the interventricular septum (left ventricular eccentricity index >1.1 in systole and/or diastole) |
speed early diastolic pulmonary regurgitation >2.2 m/s |
Square right atrial >18 cm 2 |
|
Pulmonary artery diameter >25 mm |
To obtain a more detailed understanding of the structural changes in the heart and hemodynamic disturbances, transesophageal echocardiography is performed, occasionally with contrast.
In the case of suspected thromboembolic PH, ventilation-perfusion scanning of the lungs is necessary (sensitivity - 90-100%, specificity - 94-100%). In the case of RAS, ventilation-perfusion scan results may be normal or with small non-segmental peripheral perfusion defects, which also occur in pulmonary veno-occlusive disease. New research technologies have emerged, such as three-dimensional magnetic resonance imaging, in which the study of perfusion is as informative as in ventilation-perfusion scanning of the lungs.
Method computed tomography high resolution with contrasting of the vessels of the lungs allows you to get important information on the state of the parenchyma and vascular bed of the lungs, heart and mediastinum. The assumption of PH will arise if highly specific signs are detected: expansion of the diameter of the pulmonary artery ≥29 mm, the ratio of the diameter of the pulmonary artery to the ascending aorta ≥1.0, the ratio of segmental bronchial arteries >1:1 in three to four lobes. The method is informative in differential d diagnosing PH that has developed with damage to the lung parenchyma caused by emphysema, with interstitial lung diseases, for choosing the tactics of surgical treatment for chronic thromboembolism, vasculitis and arteriovenous malformations.
Magnetic resonance imaging of the heart is accurate and well reproduced and is used for non-invasive assessment of the right ventricle, its morphology and function, blood flow status, including stroke volume, pulmonary artery distensibility, and right ventricular mass. In patients with presumed PH, late accumulation of gadolinium, reduced pulmonary artery compliance and retrograde blood flow have a high predictive value in diagnosis.
To identify diseases that cause the formation of PH, it is recommended to perform ultrasound abdominal organs. Ultrasound findings may confirm the presence of portal hypertension associated with PH.
Right heart catheterization is performed in specialized hospitals to confirm the diagnosis of pulmonary arterial hypertension, congenital heart shunts, left heart diseases accompanied by PH, chronic thromboembolic PH, assess the severity of hemodynamic disorders, conduct tests for vasoreactivity, and monitor the effectiveness of treatment. The study is performed after receiving the results of routine methods, indicating the alleged PH.
Vasoreactivity tests during right heart catheterization to identify patients who may respond to high doses of calcium blockersductus canal (BCC) is recommended for suspected idiopathic, hereditary, or drug-associated RAS. With other forms of RAS and PH, the results of the test are often doubtful. To perform a vasoreactivity test, use nitric oxide or alternative means- epoprostenol, adenosine, iloprost. A positive result is assessed in the case of a decrease in mean pulmonary arterial pressure ≥10 mm Hg. Art., until the absolute value of the mean pulmonary arterial pressure ≤40 mm Hg. Art. with/without increased cardiac output. The use of oral or intravenous CCBs when performing a vasoreactivity test is not recommended.
Laboratory research
The study of blood and urine is useful for verifying diseases in some forms of PH and assessing the condition internal organs. Liver function tests may change with high hepatic venous pressure, liver disease, treatment with endothelin receptor antagonists. Serological studies performed to diagnose viral diseases (including HIV). The study of thyroid function, violations of which occur in RAS, is carried out with a deterioration in the course of the disease, immunological studies are necessary for the diagnosis of systemic sclerosis, antiphospholipid syndrome etc.
Investigation of the level of N-terminal brain natriuretic peptide (NT-proBNP) is necessary because it is considered as an independent risk factor in patients with PH.
Molecular genetic diagnosis is performed in case of suspected sporadic or familial form of pulmonary arterial hypertension (group 1).
The diagnostic algorithm for PH (figure) consists of several stages of research, including methods that confirm the assumption of PH (history, physical findings, echocardiographic findings), followed by methods that clarify the severity of PH and possible diseases that caused an increase in pressure in the pulmonary artery. In the absence of signs of diseases of groups 2-4, a diagnostic search for diseases of group 1 is carried out.
Clinical assessment remains key in diagnosing the condition of a patient with PH. For an objective assessment of the functional ability of patients with PH, a 6-minute walk test (MX) and an assessment of dyspnea according to G. Borg (1982), as well as cardiopulmonary exercise tests with an assessment of gas exchange, are used. The functional classification of PH is carried out according to the modified version of the classification (NYHA) of heart failure (WHO, 1998). Deterioration in functional class (FC) is an alarming indicator of disease progression, prompting further investigation to clarify the causes of clinical deterioration.
Risk assessment for pulmonary arterial hypertension (high, moderate, low) is based on the results of a comprehensive examination of the patient (Table 5).
Table 5. Qualitative and quantitative clinical, instrumental and laboratory parameters for risk assessment in pulmonary arterial hypertension
|
Prognostic signs (in mortality assessment within 1 year) |
Low risk<5% |
Moderate risk, 5-10% |
High risk, >10% |
|
Clinical symptoms right ventricular heart failure |
Missing |
Missing |
Present |
|
Progression of symptoms |
Slow |
||
|
Rare episodes |
Recurring episodes |
||
|
Cardiopulmonary load tests |
Peak VO2 >15 ml/min/kg (>65% predicted) VE/V CO 2 slope<36 |
Peak VO 2 11-15 ml/min/kg (35-65% should) VE/VCO 2 slope 36-44.9 |
Peak VO2<11 мл/мин/кг (<35% долж.) |
|
Plasma NT-proBNP level |
BNP<50 нг/л NT-proBNP<300 нг/л |
BNP 50-300 ng/l NT-proBNP 300-1400 ng/l |
BNP >300 ng/l NT-proBNP >1400 ng/l |
|
Imaging results (EchoCG, MRI) |
PP area<18 см 2 No pericardial effusion |
PP area 18-26 cm 2 No or minimal pericardial effusion |
Area PP 18 >26 cm 2 Effusion in the pericardium |
|
Hemodynamics |
RAP<8 мм рт. ст. CI ≥2.5 l/min/m2 |
RAP 8-14 mmHg Art. CI 2.0-2.4 l/min/m2 |
RAP >14 mmHg Art. CI<2,0 л/мин/м 2 |
Note: 6MX - 6-minute walk test, RP - right atrium, BNP - atrial natriuretic peptide, NT-proBNP - N-terminal pro-brain natriuretic peptide, VE/VCO 2 - CO ventilatory equivalent 2 , RAP - pressure in the right atrium, CI - cardiac index, SvO 2 - saturation of venous blood with oxygen.
Thus, in pulmonary arterial hypertension, depending on the results of the recommended studies, the patient may have a low, moderate, high risk of clinical deterioration or death. Undoubtedly, other factors not included in the table can influence the course and outcome of the disease. At the same time, at low risk (mortality within a year is less than 5%), patients are diagnosed with a non-progressive course of the disease with low FC, a 6MX test > 440 m, without clinically significant signs of right ventricular dysfunction. At a moderate (intermediate) risk (mortality within 1 year 5-10%), FC III and moderate violations of exercise tolerance, signs of right ventricular dysfunction are detected. At high risk (mortality >10%), progression of the disease and signs of severe dysfunction and insufficiency of the right ventricle with FC IV, dysfunction of other organs are diagnosed.
Treatment
The modern strategy for the treatment of patients with pulmonary arterial hypertension consists of three stages, including the following activities:
General (physical activity, supervised rehabilitation, planning and control during pregnancy, in the postmenopausal period, infection prevention, psychosocial support), maintenance therapy (oral anticoagulants, oxygen therapy, digoxin, diuretics) (Table 6);
|
Class |
Level evidence |
|
|
Continuous long-term oxygen therapy is recommended for patients with RAS when arterial oxygen pressure is less than 8 kPa (60 mmHg) |
||
|
Oral anticoagulants are considered in patients with RAS (idiopathic and hereditary) |
||
|
Need to correct anemia or iron metabolism in patients with RAS |
||
|
The use of ACE inhibitors, ARBs, beta-blockers, and ivabradine is not recommended except in cases where these drug groups are required (hypertension, coronary artery disease, left ventricular heart failure) |
.? initial therapy with high doses of CCB in patients who respond positively to the vasoreactivity test, or drugs recommended for the treatment of pulmonary arterial hypertension, with a negative test for vasoreactivity;
In case of treatment failure - a combination of recommended drugs, lung transplantation.
Oral anticoagulants are prescribed due to the high risk of vascular thrombotic complications in patients with RAS. Evidence of the effectiveness of the application received in one center. The place of new oral anticoagulants in RAS is uncertain.
Diuretics are indicated for decompensated heart failure with fluid retention in a patient with RAS. Randomized clinical trials on the use of diuretics in RAS have not been conducted, however, the appointment of drugs of this group, as well as aldosterone antagonists, is carried out according to the recommendations for the treatment of heart failure.
Oxygen therapy is necessary for patients with arterial hypoxemia at rest.
Digoxin increases cardiac outputoc when administered as a bolus in patients with idiopathic RAS, its long-term efficacy is unknown. Undoubtedly, digoxin is useful in reducing the heart rate in supraventricular tachyarrhythmias.
With regard to ACE inhibitors, ARBs, beta-blockers, and ivabradine, there are no convincing data on the need for their prescription and safety in RAS.
Iron deficiency occurs in 43% of patients with idiopathic RAS, 46% of patients with systemic sclerosis and RAS, and 56% of patients with Eisenmenger's syndrome. Preliminary results indicate that iron deficiency is associated with decreased exercise tolerance, possibly with higher mortality, independent of the presence and severity of anemia. An examination to identify the causes of iron deficiency and replacement therapy (preferably intravenous) in patients with RAS is recommended.
Specific medicinal T therapy(Table 7)
|
Drug, route of administration |
Class, level of evidence |
|||||||
|
FC III |
||||||||
|
Calcium channel blockers |
||||||||
|
Endothelin receptor antagonists |
||||||||
|
Inhibitors phosphodiesterase-5 |
||||||||
|
Stimulant guanylate cyclase |
||||||||
|
prostacyclin |
||||||||
|
Receptor agonist prostacyclin |
||||||||
A small proportion of patients with idiopathic RAS who test positive for vasoreactivity during right heart catheterization have a beneficial effect on CCB with long-term treatment. Published studies have predominantly used nifedipine, diltiazem and, with less clinical benefit, amlodipine. The choice of drug is based on the patient's baseline heart rate, in the case of relative bradycardia use nifedipine or amlodipine, with tachycardia - diltiazem. Daily doses of CCB in idiopathic RAS are high: nifedipine 120-240 mg, diltiazem 240-720 mg, amlodipine 20 mg. Treatment is started with small doses, gradually titrated to the tolerated maximum recommended dose of the drug, monitoring the effectiveness of therapy after 3-4 months.
The vasodilating effect of CCB does not have a favorable long-term effect in RAS caused by connective tissue diseases, HIV, portopulmonary hypertension, veno-occlusive disease.
Endothelin receptor blockers are prescribed in connection with a certain activation of the endothelin system in patients with RAS, despite the fact that it is not yet known whether the cause or effect of the disease is an increase in endothelin activity. Drugs (ambrisentan, bosentan, macitentan) have a vasoconstrictive and mitogenic effect by binding to two receptor isoforms in pulmonary vascular smooth muscle cells, endothelin type A and B receptors.
Phosphodiesterase-5 (PDE-5) inhibitors (sildenafil, tadalafil, vardenafil) and guanylate cyclase stimulators (riociguat) exhibit vasodilating and antiproliferative effects, have a positive effect on hemodynamics, increase exercise tolerance in long-term treatment of patients with RAS.
The use of prostacyclin analogues and prostacyclin receptor agonists (beroprost, epoprostenolol, iloprost, treprostenil, selexipag) is based on the current understanding of the mechanisms of RAS development. Beneficial effect of ana logs of prostacyclin due to inhibition of platelet aggregation, cytoprotective and antiproliferative action. Preparations of the prostacyclin analogue group improve exercise tolerance (beroprost); improve the course of the disease, increase exercise tolerance, affect hemodynamics in idiopathic RA and PH associated with systemic sclerosis, and reduce mortality in idiopathic RA (epoprostenolol, treprostenil).
In case of ineffectiveness of RAS monotherapy, a combination of representatives of two or more classes of specific drugs that affect different pathogenetic links in the development of the disease is used simultaneously. Combination therapy, depending on the patient's condition and the PH group, is used at the beginning of treatment or sequentially, prescribing drugs one after the other.
In addition to general recommendations, supportive and specific therapy, the treatment of patients with RAS with the ineffectiveness of medical methods is carried out by surgical methods (balloon atrial septostomy, veno-arterial extracorporeal membrane oxygenation, transplantation of the lungs or the heart-lung complex).
Treatment of complications arising from RAS is carried out according to recommendations developed for specific situations.
Summarizing a brief review of new recommendations for the diagnosis and treatment of pulmonary hypertension, compared with the previous edition (2009), we can note the simplification of clinical classification, the introduction of new parameters of hemodynamics and pulmonary vascular resistance in the definition of postcapillary PH and RAS, advances in the genetic diagnosis of conditions, another systematization of risk factors, improved diagnostic algorithm, presented developments in assessing the severity and risk of RAS, new algorithms for treatment.
References
1.Gali e , N. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension / N. Gali e , M. Humbert, J.-L. Vachiery. - Available at: http://dx.doi.org/10.1093/eurheartj/ehv317
International Reviews: Clinical Practice and Health. - 2016. - No. 2. - P.52-68.
Attention ! The article is addressed to medical specialists. Reprinting this article or its fragments on the Internet without a hyperlink to the original source is considered a copyright infringement.
For citation: Vertkin A.L., Topolyansky A.V. Cor pulmonale: diagnosis and treatment // BC. 2005. No. 19. S. 1272
Cor pulmonale - an increase in the right ventricle of the heart in diseases that violate the structure and (or) function of the lungs (with the exception of cases of primary damage to the left heart, congenital heart defects).
The following diseases lead to its development:
- Primarily affecting the passage of air in the lungs and alveoli (chronic bronchitis, bronchial asthma, pulmonary emphysema, tuberculosis, pneumoconiosis, bronchiectasis, sarcoidosis, etc.);
- primarily affecting the mobility of the chest (kyphoscoliosis and other deformities of the chest, neuromuscular diseases - for example, polio, obesity - Pickwick's syndrome, sleep apnea);
- Primarily affecting the vessels of the lungs (primary pulmonary hypertension, arteritis, thrombosis and embolism of the vessels of the lungs, compression of the trunk of the pulmonary artery and pulmonary veins by a tumor, aneurysm, etc.).
In the pathogenesis of cor pulmonale, the main role is played by a decrease in the total cross section of the vessels of the lungs. In diseases that primarily affect the passage of air in the lungs and the mobility of the chest, alveolar hypoxia leads to spasm of the small pulmonary arteries; in diseases affecting the vessels of the lungs, an increase in resistance to blood flow is due to narrowing or blockage of the lumen of the pulmonary arteries. An increase in pressure in the pulmonary circulation leads to hypertrophy of the smooth muscles of the pulmonary arteries, which become more rigid. Overloading the right ventricle with pressure causes its hypertrophy, dilatation, and later - right ventricular heart failure.
Acute cor pulmonale develops with pulmonary embolism, spontaneous pneumothorax, a severe attack of bronchial asthma, severe pneumonia in a few hours or days. It is manifested by sudden pressing pain behind the sternum, severe shortness of breath, cyanosis, arterial hypotension, tachycardia, amplification and accent of the II heart sound over the pulmonary trunk; deviation of the electrical axis of the heart to the right and electrocardiographic signs of overload of the right atrium; rapidly increasing signs of right ventricular failure - swelling of the cervical veins, enlargement and tenderness of the liver.
Chronic cor pulmonale is formed over a number of years in chronic obstructive pulmonary disease, kyphoscoliosis, obesity, recurrent pulmonary embolism, primary pulmonary hypertension. There are three stages in its development: I (preclinical) - diagnosed only with instrumental examination; II - with the development of right ventricular hypertrophy and pulmonary hypertension without signs of heart failure; III (decompensated cor pulmonale) - when symptoms of right ventricular failure appear.
Clinical signs of chronic cor pulmonale are shortness of breath, aggravated by physical exertion, fatigue, palpitations, chest pain, fainting. When the recurrent nerve is compressed by the dilated trunk of the pulmonary artery, hoarseness occurs. On examination, objective signs of pulmonary hypertension can be detected - accent II tone on the pulmonary artery, Graham-Still diastolic murmur (noise of relative pulmonary valve insufficiency). An increase in the right ventricle may be indicated by a pulsation behind the xiphoid process, which increases on inspiration, an expansion of the boundaries of the relative dullness of the heart to the right. With significant dilatation of the right ventricle, relative tricuspid insufficiency develops, manifested by systolic murmur at the base of the xiphoid process, pulsation of the cervical veins and liver. In the stage of decompensation, signs of right ventricular failure appear: liver enlargement, peripheral edema.
The ECG reveals hypertrophy of the right atrium (spiky high P waves in leads II, III, aVF) and the right ventricle (deviation of the electrical axis of the heart to the right, an increase in the amplitude of the R wave in the right chest leads, blockade of the right leg of the His bundle, the appearance of a deep S wave in I and Q wave in III standard leads).
Radiologically acute and subacute pulmonary heart is manifested by an increase in the right ventricle, expansion of the arch of the pulmonary artery, expansion of the lung root; chronic cor pulmonale - hypertrophy of the right ventricle, signs of hypertension in the pulmonary circulation, expansion of the superior vena cava.
Echocardiography may show right ventricular wall hypertrophy, dilatation of the right heart chambers, dilatation of the pulmonary artery and superior vena cava, pulmonary hypertension, and tricuspid insufficiency.
In a blood test in patients with chronic cor pulmonale, polycythemia is usually detected.
With the development of acute pulmonary heart, treatment of the underlying disease is indicated (elimination of pneumothorax; heparin therapy, thrombolysis or surgical intervention for pulmonary embolism; adequate therapy of bronchial asthma, etc.).
Treatment of cor pulmonale proper is mainly aimed at reducing pulmonary hypertension, and with the development of decompensation, it includes correction of heart failure (Table 1). Pulmonary hypertension decreases with the use of calcium antagonists - nifedipine at a dose of 40–180 mg per day (preferably the use of long-acting forms of the drug), diltiazem at a dose of 120–360 mg per day [Chazova I.E., 2000], and amlodipine (Amlovas ) at a dose of 10 mg per day. So, according to Franz I.W. et al. (2002), during therapy with amlodipine at a dose of 10 mg per day for 18 days in 20 COPD patients with pulmonary hypertension, a significant decrease in pulmonary vascular resistance and pressure in the pulmonary artery was noted, while changes in gas exchange parameters in the lungs were not observed. According to the results of a crossover randomized study conducted by Sajkov D. et al. (1997), equivalent doses of amlodipine and felodipine equally reduced pulmonary artery pressure, but side effects (headache and edematous syndrome) developed less frequently during amlodipine therapy.
The effect of therapy with calcium antagonists usually appears after 3-4 weeks. It has been shown that a decrease in pulmonary pressure during calcium antagonist therapy significantly improves the prognosis of these patients, however, only a third of patients respond to calcium antagonist therapy in this way. Patients with severe right ventricular failure usually respond poorly to calcium antagonist therapy.
In clinical practice, in patients with signs of cor pulmonale, theophylline preparations (intravenous drip, prolonged oral preparations) are widely used, which reduce pulmonary vascular resistance, increase cardiac output and improve the well-being of these patients. At the same time, there appears to be no evidence base for the use of theophylline preparations in pulmonary hypertension.
Effectively reduces pressure in the pulmonary artery by intravenous infusion of prostacyclin (PGI2), which has antiproliferative and antiaggregant effects; the drug increases exercise tolerance, improves the quality of life and reduces mortality in these patients. Its disadvantages include often developing side effects (dizziness, arterial hypotension, cardialgia, nausea, abdominalgia, diarrhea, rash, pain in the extremities), the need for constant (long-term) intravenous infusions, as well as the high cost of treatment. The efficacy and safety of prostacyclin analogues, iloprost, used in the form of inhalation and beraprost, used orally, as well as treprostinil, administered both intravenously and subcutaneously, are being studied.
The possibility of using the endothelin receptor antagonist bosentan, which effectively reduces pressure in the pulmonary artery, is being studied, but the pronounced systemic side effects limit the intravenous use of this group of drugs.
Inhalation of nitric oxide (NO) for several weeks also reduces pulmonary hypertension, but this therapy is not available to all medical institutions. In recent years, attempts have been made to use PDE5 inhibitors in pulmonary hypertension, in particular, sildenafil citrate. Charan N.B. in 2001, described two patients who noted an improvement in the course of COPD while taking sildenafil, which they took for erectile dysfunction. Today, the bronchodilatory, anti-inflammatory effect of sildenafil and its ability to reduce pressure in the pulmonary artery has been shown both in experimental and clinical studies. According to the data obtained, PDE5 inhibitors in pulmonary hypertension significantly improve exercise tolerance, increase the cardiac index, improve the quality of life of patients with pulmonary hypertension, including primary. Long-term multicentre studies are needed to definitively resolve the issue of the effectiveness of this class of drugs in COPD. In addition, the high cost of treatment certainly hinders the widespread introduction of these drugs into clinical practice.
In the formation of chronic cor pulmonale in patients with chronic obstructive pulmonary diseases (bronchial asthma, chronic bronchitis, pulmonary emphysema), long-term oxygen therapy is indicated to correct hypoxia. With polycythemia (in the case of an increase in hematocrit above 65–70%), bloodletting is used (usually a single one), which allows to reduce pressure in the pulmonary artery, increase the patient's tolerance to physical activity and improve his well-being. The amount of blood removed is 200-300 ml (depending on the level of blood pressure and the patient's well-being).
With the development of right ventricular failure, diuretics are indicated, incl. spironolactone; it should be borne in mind that diuretics do not always help to reduce shortness of breath in pulmonary hypertension. ACE inhibitors (captopril, enalapril, etc.) are also used. The use of digoxin in the absence of left ventricular failure is ineffective and unsafe, since hypoxemia and hypokalemia developing against the background of diuretic therapy increase the risk of developing glycoside intoxication.
Considering the high probability of thromboembolic complications in heart failure and the need for active diuretic therapy, prolonged bed rest, the appearance of signs of phlebothrombosis, preventive anticoagulant therapy is indicated (usually subcutaneous administration of heparin 5000 IU 2 times a day or low molecular weight heparin 1 time per day). In patients with primary pulmonary hypertension, indirect anticoagulants (warfarin) are used under the control of INR. Warfarin increases the survival of patients, but does not affect their general condition.
Thus, in modern clinical practice, drug treatment of cor pulmonale is reduced to the treatment of heart failure (diuretics, ACE inhibitors), as well as the use of calcium antagonists and theophylline drugs to reduce pulmonary hypertension. A good effect on calcium antagonist therapy significantly improves the prognosis of these patients, and the lack of an effect requires the use of drugs of other classes, which is limited by the complexity of their use, the high likelihood of side effects, the high cost of treatment, and in some cases, insufficient knowledge of the issue.
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