What is significant pulmonary hypertension. Causes of pulmonary hypertension

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Pulmonary hypertension (PH) is characteristic of diseases that are completely different both in the reasons for their occurrence and in their defining characteristics. LH is associated with the endothelium (inner layer) of the pulmonary vessels: as it grows, it reduces the lumen of arterioles and disrupts blood flow. The disease is rare, with only 15 cases per 1,000,000 people, but survival rate is very low, especially in the primary form of PH.

Resistance in the pulmonary circulation increases, the right ventricle of the heart is forced to increase contractions in order to push blood into the lungs. However, it is anatomically not adapted to long-term pressure load, and with PH in the pulmonary artery system it rises above 25 mm Hg. at rest and 30 mmHg during physical stress. First, during a short period of compensation, thickening of the myocardium and an enlargement of the right chambers of the heart are observed, and then a sharp decrease in the force of contractions (dysfunction). The result is premature death.

The reasons for the development of PH have not yet been fully determined. For example, in the 60s in Europe there was an increase in the number of cases associated with excessive use of contraceptives and weight loss products. Spain, 1981: complications in the form of muscle damage that began after the popularization of rapeseed oil. Almost 2.5% of the 20,000 cases were diagnosed with pulmonary arterial hypertension. The root of the evil turned out to be tryptophan (an amino acid) present in the oil; this was scientifically proven much later.

Impaired function (dysfunction) of the pulmonary vascular endothelium: the cause may be a hereditary predisposition or the influence of external damaging factors. In any case, the normal balance of nitric oxide metabolism changes, vascular tone changes towards spasm, then inflammation, the endothelium begins to grow and the lumen of the arteries decreases.

Impaired synthesis or availability of nitric oxide (NO), decreased production of prostacyclin, additional excretion of potassium ions - all deviations from the norm lead to arterial spasm, proliferation of the muscular wall of blood vessels and endothelium. In any case, the final development is a violation of blood flow in the pulmonary artery system.

Moderate pulmonary hypertension does not produce any significant symptoms, This is the main danger. Signs of severe pulmonary hypertension are determined only in later periods its development, when pulmonary arterial pressure rises, compared to the norm, two or more times. Normal pressure in the pulmonary artery: systolic 30 mmHg, diastolic 15 mmHg.

Initial symptoms of pulmonary hypertension:

• Unexplained shortness of breath, even with little physical activity or at rest;
• Gradual loss of body weight even with normal, nutritious nutrition;
• Asthenia, a constant feeling of weakness and powerlessness, depressed mood - regardless of the season, weather and time of day;
• Constant dry cough, hoarse voice;
• Discomfort in the abdominal area, a feeling of heaviness and “bloating”: the beginning of stagnation of blood in the portal vein system, which carries venous blood from the intestines to the liver;
• Dizziness, fainting – manifestations of oxygen starvation (hypoxia) of the brain;
• Rapid heartbeat, over time, pulsation of the jugular vein becomes noticeable in the neck.

Later manifestations of PH:

1. Sputum streaked with blood and hemoptysis: signal increasing pulmonary edema;
2. Angina attacks (chest pain, cold sweat, feeling of fear of death) is a sign of myocardial ischemia;
3. Arrhythmias (heart rhythm disturbances) of the atrial fibrillation type.

Pain in the hypochondrium on the right: in the development of venous stagnation, a large circle of blood circulation is already involved, the liver has enlarged and its membrane (capsule) has stretched - therefore pain has appeared (the liver itself does not have pain receptors, they are located only in the capsule)

Swelling of the legs, legs and feet. Accumulation of fluid in the abdomen (ascites): manifestation of heart failure, peripheral blood stagnation, decompensation phase - a direct danger to the patient’s life.

End stage PH:

• Blood clots in the arterioles of the lungs lead to death (infarction) of active tissue and increased suffocation.

Hypertensive crises and attacks of acute pulmonary edema: most often occur at night or in the morning. Begins with a feeling of sudden lack of air, then joins coughing, bloody sputum is released. The skin takes on a bluish tint (cyanosis), and the veins in the neck pulsate. The patient is excited and frightened, loses self-control, and may move chaotically. At best, the crisis will end with copious discharge of light-colored urine and uncontrolled passage of feces; at worst, death. The cause of death may be blockage of a pulmonary artery by a blood clot (thromboembolism) and subsequent acute heart failure.

Systemic diseases connective tissue– scleroderma, rheumatoid arthritis, systemic lupus erythematosus.

Congenital heart defects (with blood shunting from left to right) in newborns, occurring in 1% of cases. After corrective blood flow surgery, the survival rate of this category of patients is higher than that of children with other forms of PH.

Late stages of liver dysfunction, pulmonary-hepatic vascular pathologies in 20% give a complication in the form of PH.

HIV infection: PH is diagnosed in 0.5% of cases, survival rate is within three years falls to 21% compared to the first year – 58%.

Intoxication: amphetamines, cocaine. The risk increases three dozen times if these substances were used more than three months in a row.

Blood diseases: with some types of anemia, PH is diagnosed in 20–40%, which increases mortality among patients.

Chronic obstructive pulmonary disease (COPD), caused by prolonged inhalation of particles of coal, asbestos, shale and toxic gases. It often occurs as an occupational disease among miners and workers in hazardous industries.

Sleep apnea syndrome: partial cessation of breathing during sleep. Dangerous, detected in 15% of adults. The consequence may be PH, stroke, arrhythmias, and arterial hypertension.

Chronic thrombosis: noted in 60% after survey of patients with pulmonary hypertension.

Lesions of the heart, its left half: acquired defects, coronary disease, hypertension. About 30% is associated with pulmonary hypertension.

Diagnosis of precapillary PH (associated with COPD, arterial pulmonary hypertension, chronic thrombosis:

• Pulmonary artery pressure: average ≥ 25 mm Hg at rest, more than 30 mm Hg during exertion;
• Increased pulmonary artery wedge pressure, blood pressure inside the left atrium, end-diastolic ≥15 mm, pulmonary vascular resistance ≥ 3 units. Wood.

Postcapillary PH (for diseases of the left half of the heart):

1. Pulmonary artery pressure: average ≥25 (mm Hg)
2. Initial: >15 mm
3. Difference ≥12 mm (passive PH) or >12 mm (reactive).

ECG: overload on the right: enlargement of the ventricle, expansion and thickening of the atrium. Extrasystole (extraordinary contractions of the heart), fibrillation (chaotic contraction of muscle fibers) of both atria.

X-ray examination: increased peripheral transparency of the lung fields, the roots of the lungs are enlarged, the borders of the heart are shifted to the right, the shadow of the arch of the dilated pulmonary artery is visible on the left along the cardiac contour.

photo: pulmonary hypertension on x-ray

Functional breathing tests, qualitative and quantitative analysis the composition of gases in the blood: the level of respiratory failure and the severity of the disease are revealed.

Echo-cardiography: the method is very informative - it allows you to calculate the average pressure in the pulmonary artery (MPAP) and diagnose almost all heart defects. PH is recognized already in the initial stages, with MPAP ≥ 36 – 50 mm.

Scintigraphy: for PH with blocking of the lumen of the pulmonary artery by a thrombus (thromboembolism). The sensitivity of the method is 90 - 100%, specific for thromboembolism is 94 - 100%.

Computer (CT) and magnetic resonance imaging (MRI): at high resolution, in combination with the use of a contrast agent (with CT), they allow you to assess the condition of the lungs, large and small arteries, walls and cavities of the heart.

Inserting a catheter into the cavity of the “right” heart, testing the vascular response: determining the degree of PH, blood flow problems, assessing the effectiveness and relevance of treatment.

Treatment of pulmonary hypertension is possible only in combination, combining general recommendations to reduce the risks of exacerbations; adequate treatment of the underlying disease; symptomatic means of influencing the general manifestations of PH; surgical methods; treatment with folk remedies and non-traditional methods - only as auxiliary.

Vaccination (influenza, pneumococcal infections): for patients with autoimmune systemic diseases - rheumatism, systemic lupus erythematosus, etc., to prevent exacerbations.

Nutrition control and dosed physical activity: for diagnosed cardiovascular failure of any origin (origin), in accordance with the functional stage of the disease.

Preventing pregnancy (or, according to indications, even terminating it): the circulatory system of the mother and child are linked together, increasing the load on the heart and blood vessels of a pregnant woman with PH can lead to death. According to the laws of medicine, priority in saving lives always belongs to the mother if it is not possible to save both at once.

Psychological support: all people with chronic diseases constantly experience stress, the balance of the nervous system is disturbed. Depression, a feeling of uselessness and burdensomeness for others, irritability over trifles - a typical psychological portrait of any “chronic” patient. This condition worsens the prognosis for any diagnosis: a person must definitely want to live, otherwise medicine will not be able to help him. Conversations with a psychotherapist, an activity you enjoy, active communication with fellow sufferers and healthy people are an excellent basis for getting a taste for life.

Surgical methods for treating PH

Balloon atrial septostomy: performed to facilitate the shunting of oxygen-rich blood inside the heart, from left to right, due to the difference in systolic pressure. A catheter with a balloon and blade is inserted into the left atrium. The blade cuts the septum between the atria, and the inflated balloon widens the opening.

Lung transplantation (or lung-heart complex): performed for health reasons, only in specialized medical centers. The operation was first performed in 1963, but by 2009 more than 3,000 successful lung transplants were performed annually. The main problem is the shortage of donor organs. Lungs are taken from only 15%, hearts from 33%, and livers and kidneys from 88% of donors. Absolute contraindications for transplantation: chronic renal and liver failure, HIV infection, malignant tumors, hepatitis C, the presence of the HBs antigen, as well as smoking, drug and alcohol use within six months before surgery.

They are used only in combination, as aids for general improvement of well-being. No self-medication!

1. Red rowan fruits: a tablespoon per glass of boiling water, ½ glass three times a day. Amygdalin contained in berries reduces the sensitivity of cells to hypoxia (decreased oxygen concentration), swelling is reduced due to its diuretic effect, and the vitamin and mineral set has a beneficial effect on the entire body.
2. Adonis (spring), herb: teaspoon per glass of boiling water, leave for 2 hours, up to 2 tablespoons on an empty stomach, 2-3 times a day. Used as a diuretic and analgesic.
3. Fresh pumpkin juice: half a glass per day. Contains a lot of potassium, useful for certain types of arrhythmia.

The classification is based on the principle functional disorders in PH, the variant is modified and is associated with manifestations of heart failure (WHO, 1998):

• Class I: PH with normal physis. activity. Standard loads are well tolerated, mild PH, grade 1 failure.
• Class II: LH plus decreased activity. Comfort in a quiet position, but dizziness, shortness of breath and chest pain begin even with normal exertion. Moderate pulmonary hypertension, increasing symptoms.
• Class III: PH with decreased initiative. Problems even at low loads. High degree of blood flow disturbances, worsening prognosis.
• Class IV: PH with minimal activity intolerance. Shortness of breath and fatigue are felt even at complete rest. Signs of high circulatory failure are congestive manifestations in the form of ascites, hypertensive crises, pulmonary edema.

The prognosis will be more favorable if:

1. The rate of development of PH symptoms is low;
2. The treatment improves the patient's condition;
3. The pressure in the pulmonary artery system decreases.

Poor prognosis:

1. Symptoms of PH develop dynamically;
2. Signs of decompensation of the circulatory system (pulmonary edema, ascites) are increasing;
3. Pressure level: in the pulmonary artery more than 50 mmHg;
4. With primary idiopathic PH.

The overall prognosis for pulmonary arterial hypertension is related to the form of PH and the phase of the prevailing disease. Mortality per year, with current treatment methods, is 15%. Idiopathic PH: patient survival after a year is 68%, after 3 years - 48%, after 5 years - only 35%.

In this article you will learn: what is pulmonary hypertension. The causes of the development of the disease, types of increased pressure in the vessels of the lung, and how the pathology manifests itself. Features of diagnosis, treatment and prognosis.

• Classification of pathology
• Causes and risk factors for development
• Manifestations of pathology, classes of disease
• Diagnostics
• Treatment methods
• Forecast

Pulmonary hypertension is a pathological condition in which there is a gradual increase in pressure in the pulmonary vascular system, which leads to increasing insufficiency of the right ventricle and ultimately ends in the premature death of a person.

When the disease occurs in the circulatory system of the lungs, the following pathological changes occur:

In order for blood to pass through the changed vessels, the pressure in the trunk of the pulmonary artery increases. This leads to increased pressure in the cavity of the right ventricle and leads to disruption of its function.

Such changes in blood flow manifest themselves as increasing respiratory failure in the early stages and severe heart failure in the final stages of the disease. From the very beginning, the inability to breathe normally imposes significant restrictions on the patients’ daily life, forcing them to limit themselves in exercise. The decrease in resistance to physical labor worsens as the disease progresses.

Pulmonary hypertension is considered a very serious disease - without treatment, patients live less than 2 years, and most of this time they require help with personal care (cooking, cleaning the room, shopping for groceries, etc.). With therapy, the prognosis improves somewhat, but the disease cannot be completely cured.

Click on photo to enlarge

The problem of diagnosing, treating and monitoring people with pulmonary hypertension is dealt with by doctors of many specialties, depending on the cause of the disease, these can be: therapists, pulmonologists, cardiologists, infectious disease specialists and geneticists. If surgical correction is necessary, vascular and thoracic surgeons are involved.

Pulmonary hypertension is a primary, independent disease in only 6 cases per 1 million population; this form includes the causeless and hereditary form of the disease. In other cases, changes in the vascular bed of the lungs are associated with any primary pathology of an organ or organ system.

Created on this basis clinical classification increased pressure in the pulmonary artery system:

Pulmonary arterial hypertension	Idiopathic or without primary cause
	Familial (caused by mutations in certain genes)
	Related or associated with: An increase in pressure in the circulatory system of the lungs due to a violation of the structure of the heart is noted with defects associated with the discharge of arterial blood into venous system: defects of the septa between the atria and ventricles, patent ductus arteriosus. The following reasons may influence the increase in pressure in the pulmonary arteries: The primary stage of changes in the vascular system of the lungs proceeds unnoticed - compensatory, replacement mechanisms cope with the emerging pathology, and this is enough for the patient’s normal condition. When the level of average pressure in the pulmonary artery reaches a critical level of 25–30 mm Hg. Art. The first symptoms of pulmonary hypertension appear. At this stage, the patient experiences deterioration only during physical overexertion and lives a full life. As changes in the blood vessels progress, tolerance to stress decreases; as a result, even household chores may require outside help. Dyspnea Difficulty occurs only when inhaling The first manifestation of pathology Occurs only during physical activity at first In later stages, present at rest No asthma attacks Chest pain Can be anything: aching, squeezing, stabbing or pressing There is no clear onset of pain Duration from a few seconds to a day Intensifies with physical stress There is no effect from taking Nitroglycerin Dizziness, fainting Always load related Duration from 2–5 to 20–25 minutes Interruptions in heart function, palpitations According to ECG data, sinus tachycardia is recorded Cough Occurs in 1/3 of patients In most cases dry (if there is no concomitant disease respiratory system) Blood in the sputum (hemoptysis) Only 10% of patients have it Occurs once Rarely persists for several days Associated with the formation of clots in small vessels In order to assess the severity of the disease, depending on the severity of manifestations, pulmonary hypertension is divided into classes. Having decided what kind of disease pulmonary hypertension is, how it manifests itself and why it occurs, we can move on to the specifics of making a diagnosis. The table presents methods for examining the patient and pathological changes characteristic specifically of increased pressure in the circulatory system of the lungs. Signs of the disease that caused the pathology (if any) are not indicated in this article. Initial examination of the patient Bluish skin on the arms and legs (acrocyanosis) Changes in the shape of fingertips and nails (thickening, expansion like “drumsticks” and “watch glasses”) Increased chest size in patients with emphysema (“barrel chest”) Enlarged liver (hepatomegaly) Accumulation of fluid in the abdominal and chest cavities (ascites, pleurisy) Swelling in the legs Enlargement and swelling of the veins in the neck Auscultation or listening to the lungs and heart Increased tone 2 above the pulmonary artery Systolic murmur of valve insufficiency between the right atrium and ventricle Dry, isolated wheezing over the surface of the lungs Other murmurs characteristic of heart disease, if it is the cause of the pathology ECG (electrocardiography) is 55% effective. Thickening and expansion of the right ventricle (hypertrophy and dilatation) - detected in 87% of patients Signs of overload of the right side of the heart - present in less than 60% of cases Deviation of the electrical axis of the heart to the right – in 79% VKG (vectorelectrocardiography) – assessment cardiac cycle not on the line (ECG), but on the plane. 63% effective. The same changes as on the ECG FCG (phonocardiography) – registration of noise that occurs during myocardial activity. Diagnostic efficiency increases to 76–81% The initial stage of increased pressure in the pulmonary artery Primary changes in the structure of the right ventricle before a decrease in its function occurs Stagnation of blood in the pulmonary circulation Changes in tricuspid valve function Diagnosis of cardiac muscle malformations Chest X-ray Pulmonary artery bulge Expansion of the roots of the lungs Enlargement of the right side of the heart muscle High transparency along the edge of the lung tissue Echocardiography or ultrasound of the heart (ultrasonography) through the chest Increased pressure in the pulmonary artery trunk Degree of tricuspid valve insufficiency Paradoxical movement of the interventricular septum (during contraction it deviates into the cavity of the right ventricle) Presence of heart defects with blood discharge into the venous system Expansion of the cavities of the right parts of the heart muscle Thickening of the anterior wall of the right ventricle Right heart catheterization is an invasive procedure for inserting a probe into the heart through a system of large veins. Exact numbers of increased pressure in the trunk of the pulmonary artery, ventricular cavity Change in the volume of blood flowing from the ventricle into the artery during myocardial contraction Increased vascular resistance in the pulmonary circulation Level of oxygen saturation of arterial and venous blood Carrying out drug tests during cardiac catheterization Reaction to administration of calcium antagonists (essential drugs for treatment) Used to clarify the cause of increased pressure in the pulmonary artery system and to carry out clarifying diagnostics if there is no certainty about the exact disease. These studies are also necessary to establish the clinical class of pulmonary hypertension. Any treatment for pulmonary hypertension only provides temporary effect. It is impossible to completely rid the patient of the disease, regardless of the cause of the increase in pressure in the blood vessels of the lungs. With a good response to treatment, the general condition of the patient improves, the ability to perform physical activity improves, and life expectancy more than doubles. If the pathology is not treated, death occurs within 2 years after detection of the disease. Medicines Drug therapy is the main method of correcting pulmonary hypertension. Several groups of drugs are used in treatment, most often in combination with each other.
	Diuretics – reducing the load on the heart	Furosemide Spironolactone
	Cardiac glycosides – improve myocardial function under conditions of stress and impaired function of the right side	Digoxin
Calcium antagonists are the main drugs of basic therapy; they dilate the vessels of the small capillary network of the lungs	Nifedipine Diltiazem

Treprostinil

Endothelin receptor antagonists - relax the smooth muscles of the capillary network of the lung tissue, suppress the processes of vascular cell division Bosentan Phosphodiesterase inhibitors - reduce vascular resistance in the lungs and the load on the right ventricle Sildenafil

Oxygen therapy is a highly effective way to increase blood saturation levels when the condition worsens or in unfavorable environmental conditions.

Nitric oxide - has a high vasodilating effect, is used for inhalation for several hours only when hospitalized in a hospital.

Used in cases of low effectiveness of drug treatment and in cases of malformations of the heart muscle.

Atrial septostomy - creating a communication between the atria	With severe insufficiency of right ventricular function, such as preparatory stage before heart and lung transplantation
Thrombendarterectomy – removal of blood clots from arteries	Clots are removed from the initial sections of the branches of the pulmonary artery to reduce the load on the right ventricle and reduce the manifestations of heart failure. Can only be carried out if the blood clot has not begun to degenerate into connective tissue (the first 5–10 days)
Heart or lung-heart complex transplantation	Carry out in patients with primary, idiopathic pulmonary hypertension in last stage heart failure For severe heart defects that cause disruption of blood flow in the lungs

Once a diagnosis of pulmonary hypertension is made, the prognosis for complete recovery is poor. The life span of patients, even with treatment, is limited. In any form of the disease, there is a rapid or slow increase in the insufficiency of the right half of the heart muscle, which causes the death of the patient.

• The most unfavorable course is pulmonary hypertension against the background of systemic scleroderma (degeneration of organ cells into connective tissue) - patients live no more than a year.
• The prognosis for the primary form of increased pressure in the blood vessels of the lungs is slightly better: the average lifespan is up to 3 years.
• The development of cardiac and pulmonary failure due to cardiac malformations is an indication for surgical treatment (organ transplantation). 40–44% of such patients survive the five-year period.
• An extremely unfavorable factor is the rapid increase in insufficiency of right ventricular function and its extreme forms of manifestation. Treatment in such cases has no effect, and patients die within 2 years.
• With a good response to drug treatment (with a “mild” form of pulmonary hypertension), more than 67% of patients survive the five-year period.

Pulmonary hypertension- this is a certain state of the pulmonary system, during which intravascular pressure sharply increases in the blood flow of the pulmonary artery. It is interesting that pulmonary hypertension develops due to the influence of one of two main pathological processes: both due to a sharp increase in the volume of the blood flow itself and a subsequent increase in pressure due to the increased blood volume, and due to an increase in the intravascular pulmonary pressure itself with an unchanged blood flow volume. It is customary to speak of the occurrence of pulmonary hypertension when the pressure in the pulmonary artery exceeds 35 mmHg.

Pulmonary hypertension is a complex, multicomponent pathological condition. During its gradual development and the disclosure of all clinical signs, the cardiovascular and pulmonary systems are gradually affected and destabilized. Advanced stages of pulmonary hypertension and its individual forms, characterized by high activity (for example, forms of idiopathic pulmonary hypertension or pulmonary hypertension in certain autoimmune lesions), can result in the development of respiratory and cardiovascular dysfunction and subsequent death.

You need to understand that the survival of patients with pulmonary hypertension directly depends on timely diagnosis and drug therapy of the disease. Therefore, it is necessary to clearly define the first key signs of pulmonary hypertension and the links in its pathogenesis in order to prescribe timely therapy.

Pulmonary hypertension can manifest itself either as a solo (primary) disease or as a result of a specific underlying cause.

Primary or idiopathic (of unknown origin) pulmonary hypertension is currently the most poorly studied subtype of pulmonary hypertension. Its main causes of development are based on genetic disorders that manifest themselves during the embryonic formation of future vessels supplying the pulmonary system. In addition, due to the same effect of genome defects, the body will experience insufficient synthesis of certain substances that can narrow or, on the contrary, dilate blood vessels: endothelial factor, serotonin and the special factor angiotensin 2. In addition to the two factors described above, which are prerequisites for primary pulmonary hypertension, There is also another prerequisite factor: excessive platelet aggregation activity. As a result, many small vessels in the pulmonary circulatory system will be clogged with blood clots.

As a result, the intravascular pressure in the pulmonary circulatory system will sharply increase, and this pressure will act on the walls of the pulmonary artery. Since the arteries have a more reinforced muscle layer, in order to cope with the increasing pressure in their bloodstream and “push” the required amount of blood further through the vessels, the muscular part of the wall of the pulmonary artery will increase - its compensatory hypertrophy will develop.

In addition to hypertrophy and minor thrombosis of the pulmonary arterioles, the development of primary pulmonary hypertension may also involve a phenomenon such as concentric fibrosis of the pulmonary artery. During this, the lumen of the pulmonary artery itself will narrow and, as a result, the pressure of the blood flow in it will increase.

As a consequence of high blood pressure, the inability of normal pulmonary vessels to support the advancement of blood flow with an already higher than normal pressure or the inability of pathologically altered vessels to promote the advancement of blood flow with normal pressure indicators, another compensatory mechanism will develop in the pulmonary circulatory system - so-called “bypass paths” will arise, and it is the arteriovenous shunts that will open. By transferring blood through these shunts, the body will try to reduce the high level of pressure in the pulmonary artery. But since arterioles muscle wall much weaker, then very soon these shunts will fail and multiple areas will form, which will also increase the pressure value in the pulmonary artery system during pulmonary hypertension. In addition, such shunts disrupt the proper flow of blood through the circulation. During this process, the processes of blood oxygenation and oxygen supply to tissues are disrupted.

With secondary hypertension, the course of the disease is slightly different. Secondary pulmonary hypertension is caused by a huge number of diseases: chronic obstructive lesions of the pulmonary system (for example COPD), congenital heart defects, thrombotic lesions of the pulmonary artery, hypoxic conditions (Pickwick syndrome) and, of course, cardiovascular diseases. Moreover cardiac diseases, which can lead to the development of secondary pulmonary hypertension are usually divided into two subclasses: diseases that cause insufficiency of left ventricular function, and those diseases that will lead to increased pressure in the chamber of the left atrium.

The underlying causes of pulmonary hypertension that accompany the development of left ventricular failure include ischemic damage to the left ventricular myocardium, its cardiomyopathic and myocardial damage, defects of the aortic valve system, coarctation of the aorta, and the effect of arterial hypertension on the left ventricle. Diseases that cause increased pressure in the left atrium chamber and the subsequent development of pulmonary hypertension include mitral stenosis, tumor lesions of the left atrium, and developmental anomalies: triatrial anomalous heart or the development of a pathological fibrous ring located above the mitral valve (“supravalvular mitral annulus”).

During the development of secondary pulmonary hypertension, the following main pathogenetic links can be distinguished. They are usually divided into functional and anatomical. The functional mechanisms of pulmonary hypertension develop due to disruption of normal or the emergence of new pathological functional features. Subsequent drug therapy will be aimed at their elimination or correction. The anatomical mechanisms for the development of pulmonary hypertension arise due to certain anatomical defects in the pulmonary artery itself or the pulmonary circulatory system. It is almost impossible to cure these changes with drug therapy; some of these defects can be corrected through certain surgical procedures.

The functional mechanisms of the development of pulmonary hypertension include the pathological Savitsky reflex, an increase in minute blood volume, the effect of biologically active substances on the pulmonary artery and an increase in the level of intrathoracic pressure, an increase in blood viscosity and the impact of frequent bronchopulmonary infections.

Savitsky's pathological reflex develops in response to obstructive bronchial lesions. With bronchial obstruction, spastic compression (constriction) of the branches of the pulmonary artery occurs. As a result, intravascular pressure and resistance to blood flow in the pulmonary circulation increases significantly in the pulmonary artery. As a result, the normal flow of blood through these vessels is disrupted, slows down, and the tissues do not receive the full amount of oxygen and nutrients, resulting in hypoxia. In addition, pulmonary hypertension causes hypertrophy of the muscular layer of the pulmonary artery itself (as discussed above) as well as hypertrophy and dilatation of the right heart.

Minute blood volume in pulmonary hypertension occurs as a response to the hypoxic consequences of increased intravascular pressure in the pulmonary artery. Low oxygen levels in the blood affect certain receptors that are located in the aortic-carotid area. During this effect, the amount of blood that the heart can pump through itself in one minute (minute blood volume) automatically increases. At first, this mechanism is compensatory and reduces the development of hypoxia in patients with pulmonary hypertension, but very quickly the increased volume of blood that will pass through the narrowed arteries will lead to an even greater development and aggravation of pulmonary hypertension.

Biologically active substances are also produced due to the development of hypoxia. They cause spasm of the pulmonary artery and an increase in aortic-pulmonary pressure. The main biologically active substances that can narrow the pulmonary artery are histamines, endothelin, thromboxane, lactic acid and serotonin.

Intrathoracic pressure most often occurs with broncho-obstructive lesions of the pulmonary system. During these lesions, it increases sharply, compresses the alveolar capillaries and contributes to an increase in pressure in the pulmonary artery and the development of pulmonary hypertension.

As blood viscosity increases, the ability of platelets to settle and form blood clots increases. As a result of this, changes similar to those in the pathogenesis of primary hypertension develop.

Frequent bronchopulmonary infections have two effects on the aggravation of pulmonary hypertension. The first way is a violation of pulmonary ventilation and the development of hypoxia. The second is the toxic effect directly on the myocardium and the possible development of myocardial lesions of the left ventricle.

The anatomical mechanisms of the development of pulmonary hypertension include the development of the so-called reduction (decrease in the number) of vessels of the pulmonary circulation. This occurs due to thrombosis and sclerosis of small vessels of the pulmonary circulation.

Thus, we can distinguish the following main stages in the development of pulmonary hypertension: an increase in the level of pressure in the pulmonary artery system; disruption of nutrition of tissues and organs and the development of their hypoxic lesions; hypertrophy and dilatation of the right heart and the development of “pulmonary heart”.

Since pulmonary hypertension is by its nature a rather complex disease and develops during the action of certain factors, its clinical signs and syndromes will be very diverse. It is important to understand that the first clinical signs of pulmonary hypertension will appear when the pressure in the bloodstream of the pulmonary artery is 2 or more times higher than normal values.

The first signs of pulmonary hypertension are the appearance of shortness of breath and hypoxic organ damage. Shortness of breath will be associated with a gradual decrease in the respiratory function of the lungs, due to high intra-aortic pressure and reduced blood flow in the pulmonary circulation. Dyspnea with pulmonary hypertension develops quite early. At first it occurs only as a result of exposure to physical activity, but very soon it begins to appear independently of them and becomes permanent.

In addition to shortness of breath, hemoptysis also very often develops. Patients may notice the release of a small amount of sputum streaked with blood when coughing. Hemoptysis occurs due to the fact that, as a result of the effects of pulmonary hypertension, blood stagnation occurs in the pulmonary circulation. As a result, some of the plasma and red blood cells will sweat through the vessel and the appearance of individual blood streaks in the sputum.

When examining patients with pulmonary hypertension, cyanosis may be detected skin and a characteristic change in the phalanges of the fingers and nail plates - “drumsticks” and “watch glasses”. These changes occur due to insufficient tissue nutrition and the development of gradual degenerative changes. In addition, "drumsticks" and "watch glasses" are a clear sign bronchial obstruction, which can also be an indirect sign of the development of pulmonary hypertension.

Auscultation can detect increased pressure in the pulmonary artery. This will be indicated by an increase in 2 tones heard through a stethoscope in the 2nd intercostal space on the left - the point where the pulmonary valve is usually heard. During diastole, blood passing through the pulmonary valve encounters high blood pressure in the pulmonary artery and the sound that is heard will be much louder than normal.

But one of the most important clinical signs of the development of pulmonary hypertension will be the development of the so-called pulmonary heart. Cor pulmonale is a hypertrophic change in the right heart that develops in response to increased blood pressure in the pulmonary artery. This syndrome is accompanied by a number of objective and subjective signs. Subjective signs of cor pulmonale syndrome in pulmonary hypertension will be the presence constant pain in the area of ​​the heart (cardialgia). These pains will disappear with oxygen inhalation. The main reason for this clinical manifestation of cor pulmonale is hypoxic damage to the myocardium, during the disruption of oxygen transport to it due to high pressure in the pulmonary circulatory system and high resistance to normal blood flow. In addition to pain with pulmonary hypertension, strong and periodic palpitations and general weakness may also be noted.

In addition to subjective signs, which cannot be used to fully assess the presence or absence of pulmonary heart syndrome in a patient with pulmonary hypertension, there are also objective signs. By percussion of the heart area, a displacement of its left border can be determined. This occurs due to the enlargement of the right ventricle and its displacement of the left parts beyond the normal boundaries of percussion. Also, an increase in the right ventricle due to its hypertrophy will lead to the fact that it will be possible to determine the pulsation or the so-called cardiac impulse along the left border of the heart.

With decompensation of the pulmonary heart, signs of liver enlargement will develop and the neck veins will swell. In addition, a characteristic indicator of decompensation of the pulmonary heart will be a positive Plesh symptom - when pressing on the enlarged liver, simultaneous swelling of the neck veins will appear.

Pulmonary hypertension is classified according to many different criteria. The main signs of the classification of pulmonary hypertension by stages are the degree of development of the cor pulmonale, ventilation disturbances, the degree of hypoxic tissue damage, hemodynamic disturbances, radiological and electrocardiographic signs.

It is customary to distinguish 3 degrees of pulmonary hypertension: transient, stable and stable with severe circulatory failure.

Degree 1 ( transient degree pulmonary hypertension) is characterized by the absence of clinical and radiological signs. At this stage, primary and minor signs of deficiency will be observed. external respiration.

Stage 2 pulmonary hypertension (stable stage of pulmonary hypertension) will be accompanied by the development of shortness of breath, which will occur with previously habitual physical activity. In addition to shortness of breath, acrocyanosis will be observed at this stage. Objectively, an enhanced apical cardiac impulse will be determined, which will indicate the beginning formation of the pulmonary heart. Auscultation with 2nd degree pulmonary hypertension will already be possible to listen to the first signs of increased pressure in the pulmonary artery - the above-described emphasis of the 2nd tone at the point of auscultation of the pulmonary artery.

On a general radiograph thoracic it will be possible to see a bulging of the contour of the pulmonary artery (due to high pressure in it), expansion of the roots of the lungs (also due to the effect of high pressure in the vessels of the pulmonary circulation). The electrocardiogram will already show signs of overload on the right side of the heart. When examining the function of external respiration, a tendency towards the development of arterial hypoxemia (a decrease in the amount of oxygen) will be observed.

In the third stage of pulmonary hypertension, diffuse cyanosis will be added to the clinical signs described above. Cyanosis will be a characteristic shade - gray, “warm” type of cyanosis. Swelling, painful enlargement of the liver and swelling of the neck veins will also appear.

Radiologically, in addition to the signs inherent in stage 2, the expansion of the right ventricle visible on the radiograph will also be added. The electrocardiogram will show increased signs of right heart overload and right ventricular hypertrophy. When examining respiratory function, severe hypercapnia and hypoxemia will be observed, and metabolic acidosis may also occur.

Pulmonary hypertension can develop not only in adulthood, but also in newborns. The cause of this condition lies in the characteristics of the pulmonary system of a newborn child. At his birth, a sharp jump in intravascular pressure occurs in the pulmonary artery system. This jump occurs due to blood flow to the opened lungs and the start of pulmonary circulation. It is this sharp jump in pressure in the pulmonary artery that is the primary cause of the development of pulmonary hypertension in a newborn child. With it, the circulatory system is not able to reduce and stabilize the spontaneous increase in intravascular pressure when the child takes his first breath. As a result, decompensation of the pulmonary circulation occurs and characteristic changes of pulmonary hypertension occur in the body.

But pulmonary hypertension can also occur after a sharp rise in pressure in the pulmonary circulatory system. If, after such a jump, the vascular pulmonary system of the newborn turns out to be unadapted to the new physiological level of intravascular pressure in it, then this can also lead to pulmonary hypertension.

As a result of these reasons, a special compensation mechanism is launched in the body, during which it tries to reduce the pressure that is too high for it. This mechanism is similar to that of shunts in adult pulmonary hypertension. Since the fetal blood flow tracts have not yet closed in the newborn child, automatically with this type of pulmonary hypertension a large shunt is triggered - the blood is discharged through the not yet closed opening through which the fetus was supplied with oxygen from the mother - the fetal ductus arteriosus.

It is customary to speak of the presence of severe pulmonary hypertension in a newborn child when there is an increase in intra-arterial pulmonary pressure above 37 mm. RT Art.

Clinically, this type of arterial hypertension will be characterized by the rapid development of cyanosis and impaired respiratory function of the child. In addition, the appearance of severe shortness of breath will come to the fore. It is important to note that this type of pulmonary hypertension in a newborn child is an extremely life-threatening condition - in the absence of prompt treatment, the death of a newborn can occur within a matter of hours from the moment of the first manifestations of the disease.

Treatment of pulmonary hypertension is aimed at eliminating the following factors: high intra-arterial pulmonary pressure, prevention of thrombosis, relief of hypoxia and unloading of the right side of the heart.

One of the most effective treatments for pulmonary hypertension is the use of calcium channel blockers. The most commonly used drugs from this line of drugs are Nifedipine and Amlodipine. It is important to note that 50% of patients with pulmonary hypertension with long-term therapy with these drugs experience a significant reduction in clinical symptoms and improvement in general condition. Therapy with calcium channel blockers is started initially with low doses and then gradually increased to high doses. daily dose(about 15 mg per day). When prescribing this therapy, it is important to periodically monitor the average level of blood pressure in the pulmonary artery in order to adjust the therapy.

When choosing a calcium channel blocker, it is also important to consider the patient's heart rate. If bradycardia is diagnosed (less than 60 beats per minute), then Nifedipine is prescribed to treat pulmonary hypertension. If tachycardia of 100 beats per minute or higher is diagnosed, then the optimal drug for the treatment of pulmonary hypertension is Diltiazem.

If pulmonary hypertension does not respond to therapy with calcium channel blockers, then treatment with prostaglandins is prescribed. These drugs trigger the expansion of narrowed pulmonary vessels and prevent platelet aggregation and the subsequent development of thrombosis in pulmonary hypertension.

In addition, patients with pulmonary hypertension are periodically prescribed oxygen therapy procedures. They are carried out when the partial pressure of oxygen in the blood decreases below 60-59 mmHg.

In order to unload the right side of the heart, diuretics are prescribed. They reduce the volume overload of the right ventricle and reduce the stagnation of venous blood in the systemic circulation.

It is also important to periodically administer anticoagulant therapy. The drug Warfarin is used most often for these purposes. It is an indirect anticoagulant and prevents thrombus formation. But when prescribing Warfarin, it is necessary to monitor the so-called international normal ratio - the ratio of the patient’s prothrombin time to the established norm. To use Warfarin for pulmonary hypertension, INR levels should be in the range of 2-2.5. If this index is lower, then the risk of developing massive bleeding is extremely high.

The prognosis of pulmonary hypertension is generally unfavorable. Approximately 20% of reported cases of pulmonary hypertension are fatal. The type of pulmonary hypertension is also an important prognostic sign. Thus, with secondary pulmonary hypertension, which arises as a result of autoimmune processes, the worst prognosis for the outcome of the disease is observed: about 15% of all patients with this form die within a few years after diagnosis from gradually developing insufficiency of pulmonary function.

An important factor, which can determine the life expectancy of a patient with pulmonary hypertension, are also indicators of the average pressure in the pulmonary artery. If this indicator increases above 30 mmHg and if it is highly stable (lack of response to appropriate therapy), the average life expectancy of the patient will be only 5 years.

In addition, the time of onset of signs of cardiac failure also plays an important role in the prognosis of the disease. If signs of class 3 or 4 heart failure are detected and signs of development of right ventricular failure, the prognosis of pulmonary hypertension is also considered extremely unfavorable.

Idiopathic (primary) pulmonary failure also has poor survival. It is extremely difficult to treat, and with this form of pulmonary hypertension it is almost impossible to influence with therapy the factor that directly causes a sharp increase in pressure in the bloodstream of the pulmonary artery. The average life expectancy of such patients will be only 2.5 years (on average).

But in addition to the large number of negative prognostic indicators for pulmonary hypertension, there are also several positive ones. One of them is that if, when treating pulmonary hypertension with calcium channel blockers, the signs of the disease gradually disappear (that is, the disease responds to this therapy), then the survival rate of patients in 95% of cases will exceed the five-year threshold.

Pulmonary hypertension (PH) is a syndrome of different diseases, united by a common characteristic symptom - an increase in blood pressure in the pulmonary artery and an increase in the load in the right cardiac ventricle. The inner layer of blood vessels grows and interferes with normal blood flow. To move blood into the lungs, the ventricle is forced to contract intensely.

Since the organ is anatomically not adapted to such a load (with PH, the pressure in the pulmonary artery system rises to 25-30 mm Hg), it provokes its hypertrophy (growth), followed by decompensation, a sharp decrease in the force of contractions and premature death.

According to the generally accepted classification for coding medical diagnoses developed by the World Health Organization, the disease code according to ICD-10 (latest revision) is I27.0 - primary pulmonary hypertension.

The disease progresses severely, with a pronounced decrease in physical capabilities, heart and pulmonary failure. PH is a rare disease (only 15 cases per million people), but survival is unlikely, especially in the primary form in the later stages, when a person dies, like from cancer, in just six months.

Such rare diseases are called “orphan”: treatment is expensive, there are few drugs (it is not economically profitable to produce them if consumers are less than 1% of the population). But these statistics are of little comfort if the misfortune has affected a loved one.

It is curious that a terrible disease develops under the influence of one of two pathologies: with a sharp increase in blood volumes with a subsequent increase in pressure, or with pressure drops in vessels with a constant volume of blood flow.

The diagnosis of “pulmonary hypertension” is established when the pressure parameters in the pulmonary vessels during exercise reach 35 mmHg. Art. Normally, blood pressure in the lungs is 5 times lower than in the body as a whole. This is necessary so that the blood has time to become saturated with oxygen and freed from carbon dioxide. When the pressure in the vessels of the lungs increases, it does not have time to receive oxygen, and the brain simply starves and shuts down.

PH is a complex, multivariate pathology. During the manifestation of all its clinical symptoms, damage and destabilization of the cardiovascular and pulmonary systems occurs. Particularly active and advanced forms (idiopathic PH, PH with autoimmune damage) lead to system dysfunction with inevitable premature death.

It is important to understand that the chance of survival of patients with such a serious diagnosis is directly proportional to the time of diagnosis. Therefore, it is necessary to clearly understand the first signs and links of pathogenesis in order to develop adequate and timely therapy.

PH can be a solo (primary) form of the disease, or develop after exposure to another underlying cause.

The causes of PH are not fully understood. For example, in the 60s of the last century in Europe there was an increase in the disease caused by the uncontrolled use of oral contraceptives and diet pills.

In Spain, a similar surge was noted due to the popularization of rapeseed oil. Of the 20 thousand cases, 2.5% were diagnosed with PH. Later cause boom, scientists named the amino acid tryptophan.

If endothelial function is impaired, the prerequisite may be a genetic predisposition or exposure to external aggressive factors. In each case, this leads to a disruption of the metabolic processes of nitric oxide, a change vascular tone(the appearance of spasms, inflammation), proliferation of the internal walls of blood vessels with a simultaneous reduction in their lumen.

The increased concentration of endothelin (a substance that constricts blood vessels) is explained either by increased secretion in the endothelium or by a decrease in its breakdown in the lungs. The sign is characteristic of idiopathic PH, congenital childhood heart defects, and systemic diseases.

The production or availability of nitric oxide is disrupted, the synthesis of prostacyclin is reduced, the excretion of potassium is increased - any deviation provokes arterial spasm, proliferation of arterial walls, and impaired blood flow in the pulmonary artery.

The following factors can also increase pressure in the pulmonary arteries:

• Cardiac pathologies of various origins;
• Chronic lung diseases (such as tuberculosis or bronchial asthma);
• Vasculitis;
• Metabolic disorders;
• PE and other pulmonary vascular problems;
• Long stay in high mountains.

If the exact cause of PH is not established, the disease is diagnosed as primary (congenital).

Based on severity, there are 4 stages of PH:

1. The first stage is not accompanied by loss of muscle activity. Hypertensive patients maintain the usual rhythm of life without feeling dizziness, fainting, weakness, painful sensations in the sternum, severe shortness of breath.
2. At the next stage, the patient's physical capabilities are limited. Calm state There are no complaints, but with a standard load, shortness of breath, loss of strength, and loss of coordination appear.
3. At the third stage of disease development, everything indicated symptoms appear in hypertensive patients even with little activity.
4. The last stage is characterized by severe symptoms of shortness of breath, pain, and weakness even in a calm state.

The first clinical symptoms of PH appear only after the pressure in the pulmonary vessels has doubled. The key sign of the disease is shortness of breath, with its own characteristics that make it possible to distinguish it from signs of other diseases:

• Manifests itself even in a calm state;
• With any load, the intensity increases;
• In a sitting position, the attack does not stop (if compared with cardiac dyspnea).

The remaining symptoms of PH are also common to most patients:

• Fatigue and weakness;
• Fainting and heart rhythm disturbances;
• Endless dry cough;
• Swelling of the legs;
• Painful sensations in the liver associated with its growth;
• Chest pain from dilated artery;
• Hoarse notes in the voice associated with pinched laryngeal nerve.

As you can see, the signs of PH are not so specific that an accurate diagnosis can be made without a comprehensive examination.

Most often, hypertensive patients come for consultation with complaints of constant shortness of breath, which complicates their usual life. Since primary PH does not have any special signs that make it possible to diagnose PH during the initial examination, the examination is carried out comprehensively - with the participation of a pulmonologist, cardiologist and therapist.

Methods for diagnosing PH:

• Primary medical examination with recording of anamnesis. Pulmonary hypertension is also due to a hereditary predisposition, so it is important to collect all the information about the family history of the disease.
• Analysis of the patient's lifestyle. Bad habits, lack of physical activity, taking certain medications - all play a role in determining the causes of shortness of breath.
• Physical examination allows you to assess the condition of the neck veins, skin tone (in case of hypertension - bluish), liver size (in this case, enlarged), the presence of swelling and thickening of the fingers.
• ECG carried out to detect changes in the right half of the heart.
• Echocardiography Helps determine blood flow rate and changes in arteries.
• CT using layer-by-layer images will allow you to see the expansion of the pulmonary artery and concomitant diseases of the heart and lungs.
• Catheterization used for accurate measurement of pressure in blood vessels. A special catheter is passed through a puncture in the thigh to the heart, and then to the pulmonary artery. This method is not only the most informative, it is characterized by the minimum number of side effects.
• Testing “6 min. walking" shows the patient's response to additional stress to establish the class of hypertension.
• Blood analysis(biochemical and general).
• Angiopulmonography By injecting contrast markers into the vessels, it allows you to see their exact pattern in the area of ​​the pulmonary artery. The technique requires great caution, since manipulations can provoke a hypertensive crisis.

To avoid mistakes, PH is diagnosed only as a result of studying data from a comprehensive vascular diagnostics. Reasons for visiting the clinic may be:

• The appearance of shortness of breath with habitual exertion.
• Pain in the sternum of unknown origin.
• Constant feeling of persistent fatigue.
• Increasing swelling of the lower extremities.

In the first stages, the disease responds to the proposed therapy. Key guidelines when choosing a treatment regimen should be:

• Identification and elimination of the cause feeling unwell patient;
• Reduced pressure in the blood vessels of the lungs;
• Prevention of blood clots in the arteries.

• Drugs that relax vascular muscles are especially effective in the early stages of PH. If treatment is started before irreversible processes occur in the vessels, the prognosis will be favorable.
• Blood thinning medications. If the blood viscosity is strong, the doctor may prescribe bloodletting. Hemoglobin in such patients should be at a level of up to 170 g/l.
• Oxygen inhalations, which relieve symptoms, are prescribed for severe shortness of breath.
• Recommendations for eating low-salt foods and water intake up to 1.5 l/day.
• Control of physical activity - loads that do not cause discomfort are allowed.
• Diuretics are prescribed when PH is complicated by pathology of the right ventricle.
• When the disease is advanced, drastic measures are resorted to - lung and heart transplantation. The method of such an operation for pulmonary hypertension is only being mastered in practice, but transplantation statistics convince us of their effectiveness.
• The only drug in Russia for the treatment of PH is Tracleer, which reduces pressure in the pulmonary arteries by inhibiting the activity of indothelin-1, a powerful vasoactive substance that provokes vasoconstriction. Oxygen saturation in the lungs is restored, the threat of a sharp oxygen deficiency with loss of consciousness disappears.

From negative consequences need to mark:

• Heart failure. The right half of the heart does not compensate for the resulting load, this aggravates the patient’s situation.
• PE – pulmonary artery thrombosis, when the vessels are blocked by blood clots. This is not just a critical condition - there is a real threat to life.
• A hypertensive crisis and a complication in the form of pulmonary edema significantly reduces the patient’s quality of life and often leads to death. LH provokes acute and chronic form failure of the heart and lungs, life-threatening for hypertensive patients.

PH can develop in either mature age, and in infants. This is explained by the characteristics of the lungs of a newborn. When it is born, a strong pressure drop occurs in the arteries of the lungs, caused by the opening of the lungs and the flow of blood.

This factor is a prerequisite for PH in newborns. If the circulatory system does not reduce the pressure in the vessels with the first breath, decompensation of pulmonary blood flow occurs with changes characteristic of PH.

The diagnosis of “pulmonary hypertension” is established in an infant if the pressure in its vessels reaches 37 mm Hg. Art. Clinically, this type of PH is characterized by the rapid development of cyanosis and severe shortness of breath. For a newborn, this is a critical condition: death usually occurs within a few hours.

There are 3 stages in the development of PH in children:

1. At the first stage, increased pressure in the pulmonary arteries is the only anomaly; the child has no clear symptoms. Shortness of breath may appear during exertion, but even the pediatrician does not always pay attention to this, explaining the problem by the detraining of the modern child’s body.
2. At the second stage, cardiac output decreases, demonstrating extensive clinical symptoms: hypoxemia, shortness of breath, syncope. The pressure in the vessels of the lungs is consistently high.
3. After the onset of right gastric failure, the disease enters the third stage. Despite high arterial pressure, cardiac output, accompanied by venous congestion and peripheral edema, falls sharply.

Each stage can last from 6 months to 6 years - from minimal hemodynamic changes to death. But still curative measures for young patients are more effective than for adults, since the processes of pulmonary vascular remodeling in children can be prevented and even reversed.

The prognosis for the treatment of pulmonary hypertension in most cases is unfavorable: 20% of recorded cases of PH resulted in premature death. The type of pulmonary hypertension is also an important factor.

In the secondary form, which develops as a result of autoimmune failures, the statistics are the worst: 15% of patients die due to deficiency within a few years after diagnosis. The life expectancy of this category of hypertensive patients is influenced by the average blood pressure in the lungs. If it is kept at 30 mmHg. Art. and higher and does not respond to treatment measures, life expectancy is reduced to 5 years.

An important circumstance will be the time when cardiac failure also joins pulmonary failure. Idiopathic (primary) pulmonary hypertension has poor survival. It is extremely difficult to treat, and the average life expectancy for this category of patients is 2.5 years.

With so many negative prognoses, there is also a positive moment: when treating PH with calcium channel blockers, the symptoms of the disease gradually disappear. If PH responds adequately to the proposed treatment, survival in 95% of cases exceeds the five-year mark.

Simple measures will help minimize the risk of developing such a formidable disease:

• Adhere to the principles of a healthy lifestyle by quitting smoking and regular physical activity.
• It is important to promptly identify and effectively treat diseases that cause hypertension. This is quite possible with regular preventive medical examinations.
• If you have chronic diseases of the lungs and bronchi, you must pay close attention to the course of the disease. Clinical observation will help prevent complications.
• The diagnosis of “pulmonary hypertension” does not prohibit physical activity; on the contrary, systematic exercise is recommended for hypertensive patients. It is only important to observe the measure.
• Situations that provoke stress should be avoided. Participation in conflicts can exacerbate the problem.

PH today is a serious and difficult to diagnose disease, since the symptoms of pulmonary hypertension are not much different from many other, less dangerous diseases. It is all the more important to be attentive to your health and the well-being of your loved ones.

If your child faints at school or your grandmother has unusual shortness of breath, do not delay visiting a doctor. Modern drugs and treatment methods can significantly reduce clinical manifestations diseases, improve the quality of life, increase its duration. The earlier the disease is detected, the more carefully all the doctor’s instructions are followed, the greater the chance of defeating the disease.

Pulmonary hypertension is a dangerous disease that requires the patient to take his health very seriously. Timely treatment will help avoid a negative scenario, while unattended pathology can eventually lead to the death of the patient.

What is the disease

Pulmonary hypertension is a condition in which there is a persistent increase in blood pressure in the pulmonary artery.

Increased pressure in the pulmonary arteries leads to enlargement of the right ventricle and, as a consequence, heart failure

The phenomenon of increasing pressure in the pulmonary artery can be caused by increased resistance in the bloodstream of the lungs or sharp increase volume of blood passing through the lungs circulatory system. This process occurs gradually and, in the end, if the patient is not provided with timely qualified medical care, it can result in death, the immediate cause of which is right ventricular heart failure.

Pulmonary hypertension should not be confused with arterial hypertension, also called hypertension, in which total blood pressure periodically or constantly increases. These pathologies differ in the nature of their occurrence and health risks.

Pulmonary hypertension affects people at the most active age - 30–40 years, and in women the pathology is registered four times more often than in men. The particular danger of the disease is that for a long period of time it can be practically asymptomatic, and therefore, by the time the diagnosis is made, it can sometimes be very difficult, if not impossible, to help the patient.

Classification and forms of pathology

Experts divide pulmonary hypertension into two main types - primary (idiopathic) and secondary.

1. The idiopathic form of the pathology is relatively rare, has an unclear nature and in most cases depends on hereditary factors and autoimmune diseases. Such hypertension can be transmitted not only to the next generation, but also through generations. It is characterized by an increase in the size of the right ventricle and sclerotic transformations of the pulmonary artery and is characterized by narrowing of the pulmonary vessels.
   

   The primary form of pulmonary hypertension is characterized by rapid progression. In most cases, it causes the patient to become unable to work, and sometimes leads to death.

2. The secondary form of pulmonary hypertension is often associated with connective tissue pathologies, for example, scleroderma, congenital and acquired heart defects, and immunodeficiency virus (HIV). In addition, it can result from pulmonary artery and other diseases that cause insufficiency of left ventricular function. Sometimes it develops with lung diseases, but in such cases it does not take severe forms.

Thromboembolic and post-embolic hypertension are also distinguished.

Depending on the pressure parameters in the pulmonary artery, three degrees of hypertension are distinguished:

• I (mild) - 25–45 mm Hg;
• II (moderate) - 45–65 mm Hg;
• III (severe) - over 65 mm Hg.

Reasons for the development of pathology

Pulmonary hypertension can be triggered by a number of factors, including:

Medicinal and chemical substances that can cause the development of pulmonary hypertension - table

Symptoms and signs

The symptomatic picture of pulmonary hypertension depends on the severity of the disease.

Classification of patients depending on the severity (degree) of the disease - table

Class sick	Symptoms
I	Physical activity is not impaired, normal loads are tolerated satisfactorily, without causing shortness of breath or weakness.
II	Physical activity is slightly impaired. The patient feels normal only at rest, experiencing dizziness, shortness of breath and chest pain during physical activity.
III	Physical activity is significantly impaired. Even minor exertion causes weakness, chest pain, shortness of breath and dizziness.
IV	Feeling dizzy, chest pain, weakness and shortness of breath even at rest.

In the compensated variant, the disease may not be detected for a long time. The first signs appear when the pressure in the pulmonary artery increases twice as much as the accepted norm.

As the disease progresses, the symptomatic picture worsens, tachycardia joins shortness of breath and weakness, atrial fibrillation, cough, hoarse voice. Over time, the patient begins to suffer from frequent fainting due to heart failure and oxygen starvation (hypoxia) of the brain.

At severe stages pulmonary hypertension additionally develops:

• hemoptysis;
• severe pain in the chest;
• swelling of the lower extremities;
• liver dysfunction;
• thrombosis of pulmonary vessels.

Pulmonary hypertension in the later stages is characterized by the presence of attacks - hypertensive crises, during which pulmonary edema may occur with increasing asphyxia. The following symptoms are also present:

• hacking cough with sputum;
• cyanosis (blue discoloration) of the skin;
• psychomotor agitation;
• strong pulsation of the neck veins;
• increased secretion of low-concentrated urine and involuntary defecation after the end of the crisis.

Exists high risk death due to the development of pulmonary heart failure and possible pulmonary embolism.

Basic diagnostic methods

The first factor that patients pay attention to is shortness of breath, and they rush to see a doctor with complaints about it. Already upon examination of the patient, the specialist identifies symptoms that allow a preliminary conclusion to be made about the presence of pulmonary hypertension. External signs of pathology may be:

• bluish tint to the skin;
• a specific shape of the fingers, the outer phalanges of which are thickened like drum sticks;
• convex nails, shaped like a watch glass.

The diagnosis of pulmonary hypertension usually involves a pulmonologist and a cardiologist, each of whom conducts a number of examinations to make an accurate diagnosis:

How to treat pulmonary hypertension

The main goal of therapy in this case is to eliminate the causes causing disease, combating the formation of blood clots and lowering pressure in the pulmonary artery. To achieve this, complex measures are taken, including medications, physiotherapy, and sometimes surgical treatments.

Important! As for treatment using alternative (traditional) medicine methods, this is strictly contraindicated for pulmonary hypertension.

Conservative therapy

1. Patients are recommended to take drugs that relax the smooth muscles of blood vessels (Prazosin, Nifedipine). These measures give good effect in the early stages of the disease, until the vessels have undergone obliteration.
2. If treatment is necessary, patients may be prescribed Sildenafil, better known as Viagra.
   

   It is curious that Sildenafil (Viagra) was originally invented as a treatment for coronary heart disease. However, as it later turned out, the use of this drug causes an active rush of blood to the pelvic organs. So side effect exceeded the main effect.

3. If the patient is diagnosed with right heart failure, diuretics are used.
4. To combat thromboembolism, the use of anticoagulants and medications that reduce blood viscosity (Cardioaspirin, Dipyridamole) is indicated.
5. If the blood is too thick, you have to resort to bloodletting.
6. In case of severe shortness of breath and oxygen starvation, the patient is given oxygen therapy - saturating the body with oxygen. The patient is also recommended to have a quiet lifestyle with the exception of any physical activity.

Diet

Proper nutrition, excluding foods high in cholesterol, is an important component of the treatment of pulmonary hypertension.

The following should be excluded from the diet:

• fat meat;
• sweets;
• salty foods;
• caffeine;
• alcoholic drinks, etc.

Can be used:

• vegetables;
• fruits;
• cereals;
• nuts;
• low-fat lactic acid products;
• vegetable oils;
• poultry;
• fish.

Surgical intervention

In severe forms of pulmonary hypertension, surgery cannot be avoided. The types of operations differ significantly depending on the cause that needs to be eliminated.

1. Embolectomy and thromboendarterectomy. If pulmonary hypertension is the result of a blood clot blocking the artery, the patient undergoes an emergency embolectomy, which is the removal of the clot blocking the artery. When arterial thrombosis becomes chronic, a thromboendarterectomy is performed - a thrombus located in the blood canal is excised along its entire length using a longitudinal incision in the artery. This operation is quite complex, but it helps in most cases to restore pulmonary function.

   A contraindication to embolectomy is elderly age coupled with severe concomitant diseases

2. Balloon atrial septostomy. Surgery aimed at eliminating the consequences birth defect heart, when there is a need to expand an existing cardiac septal defect or even artificially create it. Such measures make it possible to optimize the discharge of oxygen-rich blood into the right half of the heart. The surgical technique consists of widening the hole in the heart septum using a special balloon filled with air.
3. Lung and heart-lung complex transplantation. This is a very complex and expensive operation that requires special equipment and the highest qualifications of surgeons, but in some cases only it can save the patient’s life. Such a surgical intervention carries many risks, among which the main one is the likelihood of donor tissue rejection. In addition, it is very difficult to find a suitable donor. Such operations are prescribed in exceptional cases and only for patients with stage IV pathology, when they are practically confined to a bed or wheelchair.

Women diagnosed with pulmonary hypertension are advised to avoid pregnancy. If the pathology has already developed while the child is expecting, an abortion is performed for medical reasons to save the patient’s life, after which adequate treatment is prescribed.

Babies born with pulmonary hypertension need resuscitation measures - they are given oxygen therapy to stop the progression of the disease. Oxygen saturation is carried out both through ventilation of the lungs and at the cellular level. At the same time, the newborn is cultured to check for infection and treated with antibacterial drugs until the results are obtained.

Prognosis and complications

The prognosis for the course of pulmonary hypertension will depend on the reason for which the pathology developed. If treatment is started in a timely manner, the outcome will be more favorable.

Medical statistics show that the average survival rate of patients with pulmonary hypertension who have not received treatment does not exceed 2.5 years. With drug therapy, the survival rate over five years is 53–55%, and with a positive reaction of the body to the drugs, it reaches 90%.

The disease has an unfavorable prognosis with a significant increase in pressure in the pulmonary artery - more than 50–55 mm Hg. Art. Within a few years, such patients die.

Prevention measures

Prevention of pulmonary hypertension consists in timely identification of the causes that cause the disease, in the setting accurate diagnosis and effective treatment.

It will not be superfluous to follow nutritional rules in which:

• reduced amount of salt;
• the daily volume of liquid consumed is limited to 1.5 liters;
• the diet contains sufficient quantities of vitamins and microelements - especially magnesium and potassium;
• High cholesterol foods are excluded from the menu.

Fans of mountaineering who have prerequisites for the development of pathology should give up their hobby.

Elena Malysheva about pulmonary hypertension in adults and children - video

Pulmonary hypertension is an extremely serious disease, which, fortunately, is quite rare, but this should not lull vigilance against this dangerous pathology. The appearance of the first symptoms is a reason to contact a specialist.

Pulmonary hypertension (PH) is an increase in the average pressure in the pulmonary artery to 25 mm Hg. Art. and more.

The result is an increase in resistance in the bloodstream, impaired nutrition of the right ventricle of the heart and the development of serious complications in all organs and systems due to severe hypoxia.

In 80% of cases (data from the European Society of Cardiology), death occurs.

Moreover, the prospects for such a scenario are characterized by a number of 3-10 years, sometimes less if the main process is aggressive.

The patient population is young women under 40 years of age. According to statistics, the ratio of the fairer sex to men is characterized as 4-5:1. The mortality rate among the strong part of humanity, however, is several times higher.

The asymptomatic course and lack of specific manifestations lead to the diagnosis being made at a late stage, when it is almost impossible to help. And sometimes even based on the results of a pathological examination.

Specific preventive measures have not been developed, as well as early screening methods.

The pathological condition is preceded by stenosis or narrowing of the lumen of blood vessels, including small branches and medium-sized structures extending from the pulmonary artery.

The endothelium, that is, the inner lining of the blood vessels, thickens. This is an adaptive mechanism.

The pressure in the pulmonary artery increases progressively, rapidly, the load on the right ventricle increases, which leads to its hypertrophy (thickening).

Contractility decreases, and symptoms of heart failure increase. A classic pathological process is formed -.

It happens differently for everyone, but favorable outcome does not always occur.

Risk factors

Pulmonary hypertension is a mystery for both pulmonologists and cardiologists. Pathogenesis has not been established. We can only talk about factors that increase the risk of a pathogenic process.

Taking oral contraceptives

Destroys normal hormonal levels to the core. Estrogen-gestagen drugs provoke an artificial drop in progesterone, which is partly responsible for the adequate inhibition of proliferative processes in the body (disturbs the division of some cells); it is also this substance that contributes to the stable regulation of vascular tone in the fairer sex.

An increase in estrogen provokes inflammatory processes and aggravates the course of hypertension. Apparently, this is due to the higher prevalence in women.

Severe and prolonged increase in blood pressure

Pulmonary problems can become a complication of cardiovascular disease. This is a natural outcome if left untreated or given the wrong therapy.

Doctors are partly to blame for their inability to make a correct diagnosis and react in time to the impending threat.

Family history

In most cases it does not matter. Because diseases themselves are not inherited. At the same time, other generations receive features of the circulatory and immune systems from previous ones.

Blood pressure is observed in almost all descendants of a person suffering from hypertension. It is possible to prevent the development of diseases as part of prevention, but few people pay attention to this issue.

Interesting:

The potential for the formation of pathologies of the cardiovascular system is determined by the number of sick relatives and gender: women are most susceptible due to genetic characteristics.

Pulmonary hypertension in such a situation becomes secondary, developing as a result of a long-term increase in blood pressure or autoimmune pathologies.

Problems with the body's defenses

Diseases such as rheumatoid arthritis, systemic lupus erythematosus, Hashimoto's thyroiditis, and other autoimmune pathologies affect the likelihood of developing pulmonary hypertension.

According to statistics, patients with the described diagnoses suffer almost three times more often than relatively healthy people. A similar correlation was noted 20-30 years ago.

Thus, pulmonary hypertension is idiopathic. It is difficult, if not impossible, to determine the specific reasons for the development of the condition.

Specific research on this issue is still underway. Determining the etiology and pathogenesis will allow us to develop effective therapeutic tactics, early screening and prevention measures.

Despite all that has been said, the disease is considered low-common and is diagnosed in 7-13% of clinical situations, which is not so much.

The main factors in the development of the disease in patients

The reasons are also not fully understood. It was partially possible to identify some pathologies that could lead to the development of secondary pulmonary hypertension.

Among them:

• Congestive heart failure. Disruption of the atria and ventricles as a result of poor nutrition or other factors. Often ends in myocardial infarction.

But one does not interfere with the other. Acute circulatory disorders can be combined with increasing symptoms of pulmonary hypertension.

• . It says the same thing.

• Congenital and acquired heart defects. As a result of improper operation (for example, with), the load on the arteries is distributed unevenly. This often leads to pathological expansion of the endotheolium (internal lining of the vessel), hypertrophy.

• Chronic obstructive pulmonary disease. A destructive process that sooner or later comes to smokers and workers in hazardous industries.

Determined by a mass of symptoms, pulmonary hypertension is a late consequence of the disease, a particularly formidable complication, at the same time the quietest and most unnoticeable.

• Chronic thrombosis of the pulmonary artery and its branches. This is due to partial blockage of the lumen by pathological objects: blood clots, air bubbles. The first is much more common.

The process must be treated urgently, since partial blockage can become complete.

Hence the circulatory disorder, acute change nature of cardiac activity and death within a few minutes.

• Hypoventilation of the lungs. As a result of a long course of pathologies of the respiratory system: from destructive bronchitis to asthma, and other processes of the same kind. The underlying cause is being treated.

Etiotropic therapy should not harm the cardiovascular system. Often, pulmonologists and allergists thoughtlessly prescribe glucocorticoids, completely regardless of the consequences.

And similar ones are very common. Including an increase in pressure in the pulmonary artery and aorta.

• Myocarditis. Inflammatory pathology of the heart (middle muscular layer of the organ). Occurs as a complication infectious diseases, less often as a primary phenomenon. It is provoked by pyogenic flora.

• Cirrhosis of the liver. Acute hepatonecrosis. In other words, a destructive process affecting hepatocytes (organ cells).

Most often found in experienced alcoholics. Less common in patients with hepatitis B, C, suffering from drug intoxication.

The lungs are not the first to suffer; this is a symptom of a developed disease, when the destructive process reaches 40-50% of the total liver volume.

The compensated form has lower risks, although it cannot be written off.

• HIV infection. Due to the development of weakened immunity to critical levels, almost all patients with AIDS are observed (one in three suffers). This is another factor early death patients of similar profile.

Clinical classification of the pathological process

Pulmonary hypertension can be subdivided on different grounds. Both typologies presented below are recognized in medical practice.

Depending on the degree of restrictions and severity of the process, several classes are distinguished:

• First . Characterized by minimal changes in organs and systems. Pulmonary hypertension of the 1st degree is the most favorable moment for treatment, but identifying the pathology is incredibly difficult. You need to look specifically, because due to the absence of symptoms, it is not immediately possible to suspect a problem.
• Second . The changes are already more pronounced. The heart thickens, but only slightly. Physical activity decreases slightly, symptoms are minimal. At this stage, it is still possible to reverse the process without any special consequences for the patient’s body, but complex treatment in a hospital setting is already required.
• Third . The symptoms are quite obvious. But it is still nonspecific, which prevents timely diagnosis and verification. A competent professional will be able to suspect something is wrong just by sight; it is enough to conduct specialized research.
• Fourth . Extreme, terminal stage. The clinical picture is obvious, but even in this situation there are no characteristic symptoms. The process can easily be confused with classic hypertension. The diagnosis is made precisely at this moment, and a little more often during the autopsy.

The generally accepted classification, however, does not answer where the process comes from. This issue is resolved by the second typification, depending on the etiology.

Accordingly, they distinguish:

• Hereditary factor. Disputes about the impact of such a moment have been going on for many years, and will continue for just as long. Genetic predisposition appears to be of great importance.
• Idiopathic etiology. This diagnosis is made when it is impossible to understand the situation. That is, the clinic is obvious, the reasons are not clear.
• Associated form. Characterized by secondary nature. It develops as a result of the formation of one or another pathological process that causes the disease.
• Persistent. Appears in newborns, but the formation factors are also not clear. There is a likely connection between the perinatal period and the problem.
• Medicinal or toxic. As a result of long-term use of corticosteroids, tonics and certain other drugs complex action with devastating side effects.

Other possible groups of drugs: antidepressants and mood stabilizers, antipsychotics, especially the older generation, typical ones, ibuprofen and anti-inflammatory drugs of non-steroidal origin, antibiotics and, already mentioned at the beginning of the material, oral contraceptives.

Taking psychoactive substances also has an effect. Drugs such as cocaine and amphetamine hit the lung structures, heart and blood vessels hard.

Even short-term use makes itself felt by adverse effects of this kind.

Pulmonary hypertension is formed as a response to a pathological process; it is rare as a primary disease. The exact percentage is not known.

Symptoms, general and depending on stage

Among the manifestations typical for all stages of the pathological process:

• Shortness of breath for no apparent reason. The lungs cannot function properly as a result of poor circulation.
• Hypoxia and disruption of cellular metabolism leads to inhibition of ATP synthesis. Hence muscle weakness, inability to move normally, fatigue and other manifestations of a similar kind.
• Cough. Persistent, without mucus production, unproductive and dry. Always accompanies pulmonary hypertension.
• Hoarseness, inability to control the voice.
• Tachycardia as a result of disruption of the normal supply of blood and nutrients to the heart.
• Fainting.
• Ischemia of cerebral structures and, accordingly, dizziness, cephalalgia, nausea, vomiting. Up to the phenomenon of stroke.
• Edema of the lower extremities.
• Pain in the right hypochondrium. They can be primary, as a result of liver damage, or secondary, as a result of the involvement of the organ in the pathological process.

The absence of specific manifestations leads to the impossibility of timely diagnosis. On the other hand, a pronounced clinical picture is formed when the pressure in the vessel increases by 2 times compared to normal values.

• Stage 1. Physical activity does not change. Intense loads lead to dizziness, cephalalgia, shortness of breath, and minor tissue hypoxia.
• Stage 2. Slight decrease in physical activity. The patient can still carry out normal daily activities. But with some restrictions. The result is shortness of breath, palpitations, and muscle problems.
• Stage 3. Significant reduction in strength. Minimum activity is also impossible.
• Stage 4. Symptoms appear even in a state of complete rest.

Moderate pulmonary hypertension is the optimal time to start therapy. Catastrophic consequences have not yet arrived, and the signs are quite pronounced.

It is even better to carry out specific treatment at the very beginning of the condition. Symptoms of pulmonary hypertension develop in the lungs, blood vessels, and heart.

Diagnostics

Patients with suspected pulmonary hypertension are examined by a pulmonologist and a cardiologist in tandem. The diagram of diagnostic measures looks like this:

• Questioning the patient. Usually the first complaint that people make is severe shortness of breath, pressing discomfort in the chest.
• Anamnesis collection. Heredity plays a big role, as already mentioned.
• Visual data exploration. Deformity is observed in patients with pulmonary hypertension distal phalanges fingers, nails according to a specific type.
• Listening to breathing sounds. The weakening of tones and their splitting are determined.
• Electrocardiography. To assess the general condition of the heart and the intensity of its work. It is carried out at the very first moment. Allows you to notice minimal deviations.
• Echocardiography. Right ventricular hypertrophy is detected.
• Tomography, primarily computed tomography. The pulmonary arteries become enlarged and dilated, which is specific to the condition in question. Changes in the heart are detected.
• X-ray of the lungs. Not informative enough, but more accessible.
• Arterial catheterization. Performed with care, it allows you to quickly measure the pressure inside an anatomical structure.
• Angiopulmonography.
• Assessment of the tonometer indicator is routine (using a household device).
• Finally, 24-hour monitoring may be required.

The diagnostic scheme is approximately as follows. The order may be changed at the discretion of leading specialists.

Treatment is medicinal

Carried out as a primary measure. The following pharmaceutical groups are prescribed:

• Vasodilators. Normalize muscle layer. But they must be used with caution, since there is a high risk of sudden re-stenosis with worsening of the condition. Dosages and names are selected by a group of doctors.
• Diuretics. They allow you to “drive away” excess fluid and normalize blood pressure.
• Oxygen inhalations to compensate for the lack of substance during natural breathing.
• Statins. Allows you to fight the atherosclerotic process, if any. In most cases it is, which aggravates the already difficult situation of the patient.
• Anticoagulants. Normalizes the rheological properties of blood. Used with caution due to the possibility of fatal internal bleeding.

Conservative treatment of pulmonary hypertension is effective at stages 1-2, when the disease has not yet reached the terminal stage. Specific names of medications are selected by the attending physicians caring for the patient.

Need to pick up correct dosage and combination, it is better to do this in a hospital setting (pulmonology or cardiology).

Surgery

Indicated when conservative methods are ineffective. Consists of lung and heart transplantation. At the moment, in the conditions of Russian reality and the realities of the CIS countries, it is almost impossible to wait in line for such an operation.

In other states, everything is also not rosy, which is due to the imperfection of medical legislation and, as a result, a small number of potential donors.

Pulmonary thromboendarterectomy is prescribed as a surgical measure for thromboembolism.(an operation to remove a blood clot from the initial branches of the pulmonary artery).

The method allows you to remove the load from the right ventricle, but it is only feasible until the blood clot begins to degenerate into connective tissue.

Forecast

The primary form of pulmonary hypertension is unfavorable, even too unfavorable. Patients live no more than 1-2 years.

Secondary is easier, especially with a favorable response to the therapy. There is a chance for compensation and good survival.

With a long-term process with persistently high pressure in the pulmonary artery, the patient dies within 5 years.

Finally

Pulmonary hypertension is dangerous complication many diseases. It requires a serious approach and assistance from a whole group of specialists.

You can’t delay diagnosis; every day counts. If not started promptly, death is almost guaranteed in the short term.

Pulmonary hypertension is a dangerous and progressive condition in which there is a persistent increase in pressure in the vascular bed of the pulmonary artery. Only in 6-10% of cases this pathological condition can be idiopathic (or primary) and arise due to genetic mutations or other causes. In the rest of the patients, pulmonary hypertension is secondary, and its development is due to the complicated course of various diseases.

Normally, at rest, the average pressure in the pulmonary artery is 9-15 mm Hg. Art. To make a diagnosis of pulmonary hypertension, specialists rely on the following criteria: an increase in pressure above 25 mmHg. Art. at rest or more than 50 mm Hg. Art. under load.

In this pathological condition, the pressure in the pulmonary artery bed increases gradually and progressively. Ultimately, pulmonary hypertension causes the development of right ventricular failure, which can subsequently lead to death. According to statistics, this pathological condition is detected 4 times more often in women than in men. The average age of patients is usually from 30 to 40 years, but increased pressure in the blood vessels of the lungs can be detected in both childhood and old age.

In this article we will introduce you to the main causes, manifestations, methods of identifying and treating pulmonary hypertension. This information will help you suspect the development of the disease in time, and you will make the right decision about the need to see a doctor.

Pulmonary hypertension in most cases develops secondary to other heart diseases

So far, scientists have not been able to establish the reasons for the development of such rare disease as idiopathic pulmonary hypertension. It is assumed that its occurrence may be caused by gene mutations, autoimmune diseases or taking oral contraceptives.

Secondary pulmonary hypertension is a pathological condition that can be provoked by various chronic diseases of the lungs, blood vessels, heart and some other systems and organs. Most often, this complication is provoked by the following ailments:

• and (, septal defects, etc.);
• diseases accompanied by;
• neoplasms of the lungs and heart;
• chronic inflammatory and obstructive processes in the lungs and bronchi (tuberculosis, pneumosclerosis, emphysema, sarcoidosis);
• vasculitis localized in the pulmonary artery area;
• pulmonary-hepatic vascular pathologies and late stages of liver dysfunction;
• disorders of the chest structure and spinal column(kyphoscoliosis, ankylosing spondylitis, Pickwick syndrome in obesity, errors about thoracoplasty);
• blood pathologies: chronic hemolytic anemia, myeloproliferative diseases, condition after splenectomy.

In addition, pulmonary hypertension can be provoked by a number of acute diseases and conditions:

• respiratory distress syndrome caused by autoimmune or toxic damage and leading to an insufficient amount of surfactant on the lobules of lung tissue;
• severe diffuse pneumonitis accompanied by severe allergic reaction on inhaled odors of perfumes, paints, flowers, etc.;
• taking certain medications and exposure to toxins (toxic rapeseed oil, Aminorex, amphetamines, Fenfluramine, L-tryptophan, cocaine, cytostatics, etc.);
• acceptance of funds traditional medicine or food products.

Statistics indicate that those most likely to develop pulmonary hypertension are HIV-infected people, drug addicts, people taking medications to suppress appetite, and patients with arterial hypertension and pregnant women.

Pulmonary hypertension in newborns can be caused by the following conditions:

• general hypoxia;
• diaphragmatic hernia;
• meconium aspiration;
• continued fetal circulation.

Development mechanism

The development of pulmonary hypertension is provoked by a gradual narrowing of the lumen of the vessels of the pulmonary artery bed - capillaries and arterioles. Such changes are caused by thickening of the inner layer of blood vessels - the endothelium. In a complicated course, this process may be accompanied by inflammation and destruction of the muscular layer of the pulmonary artery.

The narrowing of the lumen of blood vessels causes constant sedimentation of blood clots in it and leads to its obliteration. Subsequently, the patient's pressure in the pulmonary artery system increases and pulmonary hypertension develops. This process increases the load on the right ventricle of the heart. Initially, its walls hypertrophy, trying to compensate for hemodynamic disturbances. Subsequently, due to constant loads, they contractility the stage of decompensation decreases and develops, expressed in right ventricular failure.

Classification of pulmonary hypertension by class

Depending on the severity of symptoms and the severity of the patient’s condition, there are four classes of pulmonary hypertension:

• I – the patient’s physical activity does not suffer, after usual exercise he does not experience dizziness, weakness, shortness of breath or chest pain;
• II – the patient’s physical activity is slightly impaired, at rest the state of health does not change in any way, but after habitual exercise he develops dizziness, weakness, shortness of breath and chest pain;
• III – the patient’s physical activity is significantly impaired, even minor physical exertion causes dizziness, weakness, shortness of breath and chest pain;
• IV – even minimal exercise causes dizziness, weakness, shortness of breath and chest pain; often the same symptoms occur at rest.

Symptoms

Seemingly inexplicable, unrelated shortness of breath may turn out to be a manifestation of pulmonary hypertension

The main insidiousness of pulmonary hypertension is that during the compensation stage it can be completely asymptomatic and is detected already in advanced forms. Its first signs begin to appear only when pulmonary arterial pressure exceeds the norm by 2 times or more.

Typically, the first symptoms of pulmonary hypertension are:

• the appearance of unexplained shortness of breath even with minor exertion or with complete rest;
• unexplained loss of body weight with the usual diet;
• hoarse voice;
• episodes of dizziness or fainting;
• cardiopalmus;
• noticeable pulsation jugular vein on the neck;
• a feeling of discomfort and heaviness in the liver area.

Later, the patient appears and. In addition, streaks of blood may be detected in the sputum. With increasing pulmonary edema, the patient develops hemoptysis.

The patient complains of pain in the right hypochondrium, which is provoked by the development of venous stagnation. When palpating the abdomen, the doctor determines the expansion of the borders of the liver and pain. Hemodynamic disturbances in the systemic circulation lead to the appearance of swelling in the legs (in the area of ​​​​the feet and legs) and accumulation of fluid in abdominal cavity(ascites).

At the terminal stage of development of pulmonary hypertension, hypertensive crises occur in the vascular bed, provoking the development of pulmonary edema. They begin with attacks of lack of air, then the patient begins to have a severe cough with the release of bloody sputum, fear and anxiety appear. The skin becomes bluish, the patient develops psychomotor agitation, feces may be released uncontrollably, and light-colored urine may be released profusely. In severe cases, pulmonary edema ends in the development of acute heart failure and pulmonary embolism, which lead to death.

Diagnostics

The most common complaint of patients with pulmonary hypertension with which they come to see a doctor is shortness of breath. When examining the patient, cyanosis is revealed, and when listening to heart sounds, a splitting of the second sound in the projection of the pulmonary artery and its accent are revealed.

To clarify the diagnosis of pulmonary hypertension and obtain a complete clinical picture, the following types of studies are prescribed:

• X-ray of the lungs;
• functional breathing tests;
• qualitative and quantitative analysis of blood gases;
• catheterization of the “right” heart and pulmonary artery;
• angiopulmonography;
• scintigraphy.

Treatment

Treatment of pulmonary hypertension should always begin as early as possible and be comprehensive. It includes recommendations for reducing the risks of aggravation and complications of the pathological condition, treatment of the underlying disease and symptomatic drug therapy. As a supplement, folk or non-traditional methods can be used. If conservative therapy is ineffective, surgical treatment is recommended for the patient.

To reduce existing risks of pulmonary hypertension, patients are recommended to:

• vaccination against influenza and pneumococcal infections that aggravate the course of the pathology;
• regular and dosed physical activity;
• diet for heart failure;
• prevention of pregnancy.

Relatives and relatives of patients with pulmonary hypertension should provide them with constant psychological support. Such patients often develop depression, they feel unnecessary, are often irritated by little things and do not want to be a burden to their environment. Such a dejected state negatively affects the course of pulmonary hypertension, and to eliminate it, constant psychological work on the existing problem is necessary. If necessary, patients may be recommended to consult a psychotherapist.

Drug therapy

Drug treatment includes a range of medicines, affecting the cause or links in the pathogenesis of the disease, alleviating one or another symptom

To eliminate the manifestations and consequences of pulmonary hypertension, the patient is prescribed the following groups of drugs:

• – reduce the load on the heart, reduce swelling;
• and – reduce the load on the heart, prevent the development of thrombosis and thromboembolism;
• – improve heart function, eliminate arrhythmias and vascular spasms, reduce shortness of breath and swelling;
• vasodilators - reduce pressure in the pulmonary bed;
• prostacyclins and endothelin receptor antagonists - block endothelial growth, eliminate vascular and bronchial spasm, and prevent thrombus formation.

To eliminate severe hypoxia, patients are shown courses of oxygen therapy.

For idiopathic pulmonary hypertension, patients are prescribed PDE type 5 inhibitors (Sildenafil) and nitric oxide. Their action reduces vascular resistance, eliminates hypertension and facilitates blood flow.

ethnoscience

As additional means for the treatment of pulmonary hypertension, the doctor may recommend taking medicinal herbs:

• infusion of red rowan fruits;
• freshly squeezed pumpkin juice;
• infusion of spring Adonis, etc.

Patients with pulmonary hypertension should know that treatment of this pathology is impossible only with the help of folk remedies. In such cases, medicinal herbs can only be used in the form of mild diuretics and preparations to calm cough.

Surgery

To facilitate the work of the heart, the patient may be recommended to perform a balloon atrial septostomy. The essence of this intervention is to insert into the left atrium a catheter with a balloon and a blade, which cuts the septum between the atria. Using a balloon, the doctor widens the incision and thus facilitates the discharge of oxygenated blood from the left half of the heart to the right.

In extremely severe cases, lung and/or heart transplantation may be performed. Such interventions are performed in specialized surgical centers and are often unavailable due to the shortage and high cost of donor organs. In some cases, transplantation may be absolutely contraindicated: malignant neoplasms, HIV infection, smoking, the presence of HBs antigen, hepatitis C and drug or alcohol use in the last 6 months.

Forecasts

With secondary pulmonary hypertension, the prognosis is favorable if the rate of development of the pathological condition remains small, the prescribed therapy is effective and leads to a decrease in pressure in the pulmonary bed. With rapid progression of symptoms, an increase in pressure more than 50 mm Hg. Art. and poor effectiveness of treatment, the prognosis worsens. In such cases, most of the patients die within 5 years.

Idiopathic pulmonary hypertension always has an extremely poor prognosis. During the first year of the disease, only 68% of patients remain alive, after 3 - 38%, and after 5 years - only 35%.

Pulmonary hypertension refers to severe and dangerous pathological conditions, which over time can cause the patient’s death from pulmonary embolism or constantly increasing right ventricular heart failure. Its treatment should begin as early as possible, and the patient needs constant monitoring by a pulmonologist and cardiologist.

Channel One, program “Live Healthy!” with Elena Malysheva, in the “About Medicine” section, a conversation about pulmonary hypertension (see from 34:20 min.):

Scientific report by Tsareva N.A. on the topic “New in the treatment of pulmonary hypertension”:

D.M.Sc. Konopleva L.F. talks about the treatment of pulmonary hypertension:

Pulmonary hypertension is a complex, multicomponent pathological condition. During its gradual development and disclosure of all clinical signs, the cardiovascular and pulmonary systems are gradually affected and destabilized. Advanced stages of pulmonary hypertension and its individual forms can be fatal.

Description of the disease

Pulmonary hypertension is a medical term meaning increased pressure in the pulmonary artery system (at rest - 25 mmHg, during exercise - 30), which delivers venous blood to the lungs to saturate it with oxygen. This form of hypertension is fundamentally different from essential hypertension, which appears in the human body with age and is characterized by increased blood pressure in the vessels of the systemic circulation (measured using a tonometer and, as a rule, has two numbers: systolic (synonymous with “upper”) and diastolic (synonymous with “lower”). Normally, it usually does not exceed 140 and 90 millimeters of mercury).

Pulmonary hypertension (hypertension) is also measured in millimeters of mercury, but this figure can only be determined by undergoing an ultrasound examination of the heart or a pulmonary artery catheterization procedure.

PH is considered a very common pathology (millions of people live in the world with a mild degree of severity, hundreds of thousands with a moderate degree, and thousands of people with a severe degree). The incidence is 65 cases per million population, and the number of patients with primary arterial pulmonary hypertension does not exceed 20.

Causes of pulmonary hypertension

PH is divided into two fundamentally different types:

1. Primary hypertension (synonyms - idiopathic, congenital) is an independent pathology, the reason for its development is unknown.
2. Secondary PH is a component of other diseases.

Both forms are characterized by a long course.

Causes of secondary pulmonary hypertension:

• heart diseases: congenital and acquired defects, ischemic disease, arterial hypertension. In this case, the basis is a dysfunction of the heart muscle, valves, and partitions;
• pulmonary pathology: developmental anomalies, pulmonary embolism, proliferation of connective tissue. The mechanism for the development of pathology in such a situation is stagnation of blood in the vessels of the lungs, including the main pulmonary arteries. If the cause of the disease was blockage of a vessel by a blood clot, hypertension is called thromboembolic or post-embolic;
• diseases that cause disturbances in the structure of connective tissue - fibrosis, systemic lupus erythematosus and others;
• diseases that cause changes in blood composition (hematological);
• harmful effects of drugs and toxins;

Of all the causes of pulmonary hypertension, primary makes up 3.5% of cases, with diseases of the left ventricle (coronary heart disease, arterial hypertension) - 78% of cases, with lung disease - 10%, thromboembolism makes up 1.5%, the rest - 7%.

Pulmonary stage hypertension	Systolic pressure in the pulmonary artery	Morphological changes
1 (minor)	25–50 mm Hg. Art.	thickening of the middle muscular layer of the pulmonary vessels, the patency of the arteries is not impaired
2 (moderate)	51–75 mm Hg. Art.	thickening along with the tunica media and the inner tunica (intima)
3 (pronounced)	76–110 mm Hg. Art.	proliferation of connective tissue in the lining of the pulmonary vessels (sclerosis), their lumen decreases
4 (sharply expressed)	76–110 mm Hg. Art.	Irreversible changes are formed in the lining of blood vessels, which significantly disrupts the exchange of oxygen between the alveoli of the lungs and venous blood (Eisenmenger syndrome)

Symptoms of the disease in adults and children

Symptoms of pulmonary hypertension include:

• shortness of breath from slight to severe, especially during physical activity;
• pain in the heart area;
• swelling of the legs;
• low ability to perform physical activity;
• fast fatiguability;
• cyanosis of the skin;
• signs of concomitant diseases;
• poor appetite and pallor.

The appearance of symptoms indicates a long course of the disease, damage to most pulmonary vessels and an unfavorable prognosis, so timely diagnosis is important.

Pregnancy with high pulmonary hypertension is associated with a great risk for the mother and fetus, since during this period the volume of circulating blood and the need for oxygen in both organisms sharply increases. Such patients are advised to refrain from conceiving.

In newborns, PH with complex heart defects sharply comes to the fore after the transition from the intrauterine type of blood circulation to a full one in two circles. Immediately after birth, cyanosis of the skin, shortness of breath, and impaired gas exchange appear, which may require emergency intervention.

Diagnostic methods (X-ray, ultrasound, ECG)

The doctor diagnoses pulmonary hypertension only after undergoing a set of procedures

To detect the disease at an early stage, the following measures are necessary:

• examination by a doctor, carefully identifying all the details of the disease (time of occurrence, initial manifestations, stages of development);
• electrocardiography. When analyzing the ECG, symptoms of overload and hypertrophy of the right ventricle (the initial part of the pulmonary circulation) are determined;
• X-ray. The chest X-ray shows symptoms such as expansion of the diameter of the heart, enlargement of the artery arch, and increased pulmonary pattern;
• Ultrasound of the heart. During the examination, its dimensions, wall thickness are determined, systolic pressure indicators are calculated, etc.;
• Catheterization is the gold standard for diagnosing PH. It is characterized by measuring pressure in the pulmonary artery in a direct way, by inserting a catheter there through a puncture of the subclavian according to the Seldinger technique, and also includes conducting pharmacological tests with drugs that relax the muscular lining of the pulmonary vessels.

After diagnosing pulmonary hypertension and its degree, methods follow to determine the nature of the disease:

• computed tomography of the chest (lung pathology, systemic connective tissue diseases);
• Ultrasound of the abdominal organs (increased pressure in the system main vein liver - portal);
• biochemical tests to detect HIV infection, toxins;
• blood tests (determination of anemia);
• spirography (impaired lung function).

After determining the nature of the disease, the doctor creates a treatment plan for the patient.

Video: pulmonary artery catheterization

Treatment

It is possible to treat pulmonary hypertension only in a complex manner, combining general recommendations to reduce the risks of exacerbations, adequate therapy for the underlying disease, symptomatic means of influencing the general manifestations of PH, and surgical methods.

Drug treatment (list of drugs)

When primary pulmonary hypertension is diagnosed, the patient is prescribed the following drugs:

In case of pulmonary hypertension associated with other diseases, it is necessary first of all to treat the underlying pathology.

Non-drug therapy

Non-drug treatment includes following the following recommendations:

1. It is forbidden to engage in heavy physical activity.
2. Colds must be avoided.
3. It is not recommended to stay in high altitude conditions. Other climatic zones (including northern latitudes) do not affect the course of pulmonary hypertension.
4. Oxygen therapy (breathing with humidified oxygen) should be performed.

Patients with high pulmonary hypertension should avoid pregnancy, but the use of oral contraceptives is not recommended. It is advisable to discuss other methods of protection with your doctor.

Diet and folk remedies have not shown sufficient effectiveness in correcting pulmonary hypertension, so their use is inappropriate.

Surgery

As for the surgical treatment of pulmonary hypertension, it is indicated for congenital and acquired heart defects that lead to hemodynamic impairment.

In severe forms of pulmonary hypertension, surgery cannot be avoided

In adult patients, the following methods for correcting congenital and acquired defects are used:

• suturing septal defects;
• correction of defects using a patch made of various materials (plastic);
• intravascular installation of occluders (devices that stop blood access through septal defects);
• replacing your own malfunctioning heart valve with a prosthetic one (prosthetics);
• valve correction using suture material while maintaining its natural structure (plastic);

Separately, it is worth highlighting a group of newborn children in whom, due to severe life-threatening cardiac anomalies (transposition of the great vessels, single ventricle), measures are initially taken aimed at temporarily improving their condition:

1. Potts anastomosis between the descending aorta and the left pulmonary artery. The goal is to reduce the load on the right side of the heart. Indications:
   • childhood, due to which it is impossible to perform atrioseptostomy - an operation to connect the atria through an opening in the septum;
   • severe insufficient ability of the right ventricle to pump blood;
   • sharply increased pressure in the pulmonary artery.
2. Atrioseptostomy - creation of communication between the atria, including the intravascular method.

In the presence of high pulmonary hypertension in children and adults, the only radical way to resolve the disease is a lung transplant or a heart-lung complex.

Contraindications

The main contraindication for surgical treatment- irreversible damage to the pulmonary vessels with high degrees of pulmonary hypertension (Eisenmenger syndrome).

Prohibitions for planned interventions: acute respiratory diseases, abnormal laboratory tests, other significant impairment of renal function and external respiration.

Prevention

Prevention measures of this disease include:

• timely diagnosis, including diseases that cause the development of pulmonary hypertension;
• treatment of underlying diseases;
• timely surgical treatment of heart defects.

Complications and prognosis

The main complication of this disease is the progression of chronic heart failure, which is the logical outcome of pulmonary hypertension. Without proper treatment, the mortality rate is 22–38%.

Pulmonary hypertension is a common symptom complex observed not only in heart disease, but also in many other conditions. At timely diagnosis and treatment, patients can count on a good quality of life with a normal life expectancy.