ICD code for high pulmonary hypertension. Pulmonary hypertension secondary Pulmonary hypertension ICD code

at rest and more than 30 mm Hg. Art. under load.

Secondary pulmonary hypertension: Causes

Etiology

Pathogenesis

Secondary pulmonary hypertension: Signs, symptoms

Clinical manifestations

Secondary pulmonary hypertension: Diagnosis

Diagnostics

X-ray of the chest organs. Important for identifying parenchymal diseases of the lungs. Allows to identify dilation of the trunk of the pulmonary artery and the roots of the lungs. Dilation of the right descending branch of the pulmonary artery is more than 16–20 mm.

ECG A normal ECG does not exclude pulmonary hypertension P - pulmonale Deviation of the EOS to the right Signs of right ventricular hypertrophy (high R waves in leads V1–3 and deep S waves in leads V5–6) Signs of blockade of the right bundle branch.

Ventilation-perfusion lung scintigraphy is important for diagnosing pulmonary embolism.

Jugular venography High-amplitude wave A with compensated hypertrophy of the right ventricle High-amplitude wave V with right ventricular failure.

Pulmonary function examination Decreased functional capacity of the lungs Changes in the ventilation/perfusion ratio suggest embolism of the proximal pulmonary arteries.

Cardiac catheterization is important for the diagnosis of left ventricular dysfunction and heart defects, as well as for direct measurement of pressure in the pulmonary vessels. Pulmonary artery pressure (PAP) is increased Pulmonary artery wedge pressure (PAP) is normal or increased Pulmonary vascular resistance (PVR) is calculated using Wood's formula: [PVR = (PAPavg - PAP)/CO], where CO is cardiac output. The SLS determines the severity of pulmonary hypertension: mild - 2-5 units, moderate - 5-10 units, severe - more than 10 units.

Liver biopsy - if cirrhosis is suspected.

Secondary pulmonary hypertension: Treatment methods

Treatment

ICD-10. I28. 8 Other specified pulmonary vascular diseases

Did this article help you? Yes - 1 No - 0 If the article contains an error Click here 1850 Rating:

Click here to add a comment to: Secondary pulmonary hypertension (Diseases, description, symptoms, folk recipes and treatment)

Other forms of pulmonary heart failure (I27)

To indicate the underlying disease, use an additional code, if necessary.

Excludes: Eisenmenger’s defect (Q21.8)

Chronic heart disease of pulmonary origin

Cor pulmonale (chronic) NOS

In Russia, the International Classification of Diseases, 10th revision (ICD-10) has been adopted as a single normative document for recording morbidity, reasons for the population's visits to medical institutions of all departments, and causes of death.

ICD-10 was introduced into healthcare practice throughout the Russian Federation in 1999 by order of the Russian Ministry of Health dated May 27, 1997. No. 170

The release of a new revision (ICD-11) is planned by WHO in 2017-2018.

With changes and additions from WHO.

Processing and translation of changes © mkb-10.com

Other secondary pulmonary hypertension

Definition and general information [edit]

Secondary pulmonary hypertension

Etiology and pathogenesis[edit]

Etiology: congenital heart defects, collagenosis, undissolved thrombus in the pulmonary artery, prolonged increase in pressure in the left atrium, hypoxemia, COPD.

Pathological anatomy: the same as for primary pulmonary hypertension. Thrombosis and other changes in the pulmonary veins occur.

Pathogenesis: the same as for primary pulmonary hypertension. In COPD, a significant role is played by a decrease in the volume of the pulmonary parenchyma. With collagenosis - vasculitis of the pulmonary arteries.

Clinical manifestations[edit]

Clinical manifestations: the same as for primary pulmonary hypertension. With PE, the main reason for the progression of pulmonary hypertension is the growth of an undissolved thrombus, and not repeated PE.

Other secondary pulmonary hypertension: Diagnosis[edit]

To diagnose parenchymal lung diseases - radiography or CT scan of the chest, functional studies of the lungs. To diagnose pulmonary embolism, ventilation-perfusion lung scintigraphy is used. To diagnose left ventricular dysfunction, left-to-right shunt, mitral stenosis - EchoCG and cardiac catheterization. To exclude liver cirrhosis, which can lead to secondary pulmonary hypertension, liver function is determined.

Differential diagnosis[edit]

Other secondary pulmonary hypertension: Treatment[edit]

COPD. Annual vaccination against influenza and pneumococcal infection, active treatment of pulmonary infections. The following drugs are also prescribed:

A. The basis of treatment is oxygen and diuretics. For bronchospasm - bronchodilators, sometimes in combination with corticosteroids;

b. the effectiveness of anticoagulants has not been proven;

V. due to the risk of arrhythmogenic effects, digoxin is usually not used, except in cases of decompensated right ventricular failure;

d. empiric use of vasodilators should be avoided. Calcium antagonists can suppress hypoxic vasoconstriction of pulmonary arterioles, exacerbating ventilation-perfusion imbalances and thereby increasing hypoxemia.

Other reasons: collagenosis; congenital heart defects; TELA; interstitial lung diseases. General measures are the same as for COPD (see Chapter 18, paragraph III.B.2). First of all, the underlying disease is treated (immunosuppressants for collagenosis, valvuloplasty or valve replacement for mitral stenosis, embolectomy for thromboembolism of large branches of the pulmonary artery, surgical correction for congenital heart defects). Drug treatment:

A. anticoagulants, digoxin, diuretics, oxygen - as for primary pulmonary hypertension. In congenital heart defects with right-to-left shunting, oxygen is ineffective;

b. positive test results for the reversibility of pulmonary hypertension (see Chapter 18, paragraph III.B.1.e) occur in less than 5% of cases.

Course of the disease in the absence of treatment

Right ventricular failure is progressing. Life expectancy depends both on the severity of the underlying disease and on hemodynamics.

Pulmonary hypertension secondary ICD code 10

Pulmonary hypertension syndrome

Increased pressure in the pulmonary capillary system (pulmonary hypertension, hypertension) is most often a secondary disease not directly related to vascular damage. The primary conditions have not been sufficiently studied, but the role of the vasoconstrictor mechanism, thickening of the arterial wall, and fibrosis (tissue compaction) has been proven.

In accordance with ICD-10 (International Classification of Diseases), only the primary form of pathology is coded as I27.0. All secondary symptoms are added as complications to the underlying chronic disease.

Some features of the blood supply to the lungs

The lungs have a double blood supply: a system of arterioles, capillaries and venules is included in gas exchange. And the tissue itself receives nutrition from the bronchial arteries.

The pulmonary artery is divided into right and left trunks, then into branches and lobar vessels of large, medium and small caliber. The smallest arterioles (part of the capillary network) have a diameter 6–7 times larger than in the systemic circulation. Their powerful muscles are capable of narrowing, completely closing or expanding the arterial bed.

With narrowing, resistance to blood flow increases and internal pressure in the vessels increases; expansion reduces pressure and reduces the force of resistance. The occurrence of pulmonary hypertension depends on this mechanism. The total network of pulmonary capillaries covers an area of ​​140 m2.

The veins of the pulmonary circle are wider and shorter than those in the peripheral circulation. But they also have a strong muscle layer and are able to influence the pumping of blood towards the left atrium.

How is pressure in the pulmonary vessels regulated?

The amount of blood pressure in the pulmonary vessels is regulated by:

• pressor receptors in the vascular wall;
• branches of the vagus nerve;
• sympathetic nerve.

Extensive receptor zones are located in large and medium-sized arteries, at branching points, and in veins. Arterial spasm leads to impaired oxygen saturation of the blood. And tissue hypoxia promotes the release of substances into the blood that increase tone and cause pulmonary hypertension.

Irritation of the vagus nerve fibers increases blood flow through the lung tissue. The sympathetic nerve, on the contrary, causes a vasoconstrictor effect. Under normal conditions, their interaction is balanced.

The following indicators of pressure in the pulmonary artery are accepted as the norm:

• systolic (upper level) - from 23 to 26 mm Hg;
• diastolic - from 7 to 9.

Pulmonary arterial hypertension, according to international experts, begins at the upper level – 30 mmHg. Art.

Factors causing hypertension in the pulmonary circulation

The main factors of pathology, according to V. Parin’s classification, are divided into 2 subtypes. Functional factors include:

• constriction of arterioles in response to low oxygen levels and high concentrations of carbon dioxide in the inhaled air;
• increase in minute volume of passing blood;
• increased intrabronchial pressure;
• increased blood viscosity;
• left ventricular failure.

Anatomical factors include:

• complete obliteration (blocking of the lumen) of blood vessels by a thrombus or embolus;
• impaired outflow from the zonal veins due to their compression due to aneurysm, tumor, mitral stenosis;
• changes in blood circulation after surgical removal of a lung.

What causes secondary pulmonary hypertension?

Secondary pulmonary hypertension occurs due to known chronic diseases of the lungs and heart. These include:

• chronic inflammatory diseases of the bronchi and lung tissue (pneumosclerosis, emphysema, tuberculosis, sarcoidosis);
• thoracogenic pathology in violation of the structure of the chest and spine (Bechterew's disease, consequences of thoracoplasty, kyphoscoliosis, Pickwick's syndrome in obese people);
• mitral stenosis;
• congenital heart defects (for example, patent ductus arteriosus, “windows” in the interatrial and interventricular septum);
• tumors of the heart and lungs;
• diseases accompanied by thromboembolism;
• vasculitis in the area of ​​the pulmonary arteries.

What causes primary hypertension?

Primary pulmonary hypertension is also called idiopathic, isolated. The prevalence of the pathology is 2 people per 1 million inhabitants. The definitive reasons remain unclear.

It has been established that women make up 60% of patients. Pathology is detected both in childhood and in old age, but the average age of identified patients is 35 years.

In the development of pathology, 4 factors are important:

The role of the mutating bone protein gene, angioproteins, their influence on the synthesis of serotonin, and increased blood clotting due to the blocking of anticoagulant factors has been established.

A special role is given to infection with the herpes virus type eight, which causes metabolic changes leading to the destruction of arterial walls.

The result is hypertrophy, then expansion of the cavity, loss of right ventricular tone and the development of failure.

Other causes and factors of hypertension

There are many causes and lesions that can cause hypertension in the pulmonary circle. Some of them need to be highlighted.

Among acute diseases:

• respiratory distress syndrome in adults and newborns (toxic or autoimmune damage to the membranes of the respiratory lobes of the lung tissue, causing a lack of surfactant substance on its surface);
• severe diffuse inflammation (pneumonitis), associated with the development of a massive allergic reaction to inhaled odors of paint, perfume, and flowers.

In this case, pulmonary hypertension can be caused by food, drugs and folk remedies.

Pulmonary hypertension in newborns can be caused by:

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

In children, hypertension is promoted by enlarged tonsils.

Classification according to the nature of the flow

It is convenient for clinicians to divide hypertension in the pulmonary vessels according to the timing of development into acute and chronic forms. Such a classification helps to “combine” the most common causes and clinical course.

Acute hypertension occurs due to:

• pulmonary embolism;
• severe status asthmaticus;
• respiratory distress syndrome;
• sudden left ventricular failure (due to myocardial infarction, hypertensive crisis).

The chronic course of pulmonary hypertension is caused by:

• increased pulmonary blood flow;
• increase in resistance in small vessels;
• increased pressure in the left atrium.

A similar development mechanism is typical for:

• defects of the interventricular and interatrial septum;
• patent ductus arteriosus;
• mitral valve disease;
• proliferation of myxoma or thrombus in the left atrium;
• gradual decompensation of chronic left ventricular failure, for example, with ischemic disease or cardiomyopathies.

The following diseases lead to chronic pulmonary hypertension:

• hypoxic nature - all obstructive diseases of the bronchi and lungs, prolonged oxygen deficiency at altitude, hypoventilation syndrome associated with chest injuries, mechanical breathing;
• mechanical (obstructive) origin, associated with narrowing of the arteries - reaction to drugs, all variants of primary pulmonary hypertension, recurrent thromboembolism, connective tissue diseases, vasculitis.

Clinical picture

Symptoms of pulmonary hypertension appear if the pressure in the pulmonary artery is increased by 2 times or more. Patients with hypertension in the pulmonary circle notice:

• shortness of breath, which worsens with physical activity (can develop in paroxysms);
• general weakness;
• rarely loss of consciousness (as opposed to neurological causes without convulsions and involuntary urination);
• paroxysmal chest pains, similar to angina pectoris, but accompanied by an increase in shortness of breath (scientists explain them by a reflex connection between the pulmonary and coronary vessels);
• the admixture of blood in the sputum when coughing is characteristic of significantly increased pressure (associated with the release of red blood cells into the interstitial space);
• hoarseness is detected in 8% of patients (caused by mechanical compression of the recurrent nerve on the left by the dilated pulmonary artery).

The development of decompensation as a result of pulmonary heart failure is accompanied by pain in the right hypochondrium (liver stretching), swelling in the feet and legs.

When examining a patient, the doctor pays attention to the following:

• a blue tint to the lips, fingers, ears, which intensifies as shortness of breath becomes more severe;
• the symptom of “drum” fingers is detected only in long-term inflammatory diseases and defects;
• the pulse is weak, arrhythmias are rare;
• blood pressure is normal, with a tendency to decrease;
• palpation in the epigastric zone makes it possible to determine increased impulses of the hypertrophied right ventricle;
• On auscultation, an accentuating second sound on the pulmonary artery is heard, and a diastolic murmur is possible.

The relationship of pulmonary hypertension with persistent causes and certain diseases allows us to distinguish variants in the clinical course.

Portopulmonary hypertension

Pulmonary hypertension leads to a simultaneous increase in portal vein pressure. In this case, the patient may or may not have cirrhosis of the liver. It accompanies chronic liver diseases in 3–12% of cases. The symptoms are no different from those listed. Swelling and heaviness in the hypochondrium on the right are more pronounced.

Pulmonary hypertension with mitral stenosis and atherosclerosis

The disease varies in severity. Mitral stenosis contributes to the occurrence of atherosclerotic lesions of the pulmonary artery in 40% of patients due to increased pressure on the vessel wall. Functional and organic mechanisms of hypertension are combined.

The narrowed left atrioventricular passage in the heart is the “first barrier” to blood flow. If there is narrowing or blockage of small vessels, a “second barrier” is formed. This explains the failure of surgery to eliminate stenosis in the treatment of heart disease.

By catheterization of the heart chambers, high pressure inside the pulmonary artery is detected (150 mm Hg and above).

Vascular changes progress and become irreversible. Atherosclerotic plaques do not grow to large sizes, but they are sufficient to narrow small branches.

Pulmonary heart

The term “cor pulmonale” includes a symptom complex caused by damage to the lung tissue (pulmonary form) or pulmonary artery (vascular form).

There are flow options:

1. acute - typical for pulmonary artery embolization;
2. subacute - develops with bronchial asthma, lung carcinomatosis;
3. chronic - caused by emphysema, a functional spasm of the arteries, turning into an organic narrowing of the artery, characteristic of chronic bronchitis, pulmonary tuberculosis, bronchiectasis, frequent pneumonia.

An increase in resistance in the vessels puts a pronounced load on the right heart. The general lack of oxygen also affects the myocardium. The thickness of the right ventricle increases with the transition to dystrophy and dilatation (persistent expansion of the cavity). Clinical signs of pulmonary hypertension gradually increase.

Hypertensive crises in the vessels of the “small circle”

A crisis course often accompanies pulmonary hypertension associated with heart defects. A sharp deterioration in the condition due to a sudden increase in pressure in the pulmonary vessels is possible once a month or more often.

• increased shortness of breath in the evening;
• feeling of external compression of the chest;
• severe cough, sometimes with hemoptysis;
• pain in the interscapular region radiating to the anterior sections and sternum;
• cardiopalmus.

Upon examination, the following is revealed:

• patient's agitated state;
• inability to lie in bed due to shortness of breath;
• pronounced cyanosis;
• weak rapid pulse;
• visible pulsation in the area of ​​the pulmonary artery;
• swollen and pulsating neck veins;
• excretion of copious amounts of light-colored urine;
• involuntary defecation is possible.

Diagnostics

Diagnosis of hypertension in the pulmonary circulation is based on identifying its signs. These include:

• hypertrophy of the right heart;
• determination of increased pressure in the pulmonary artery based on the results of measurements using catheterization.

Russian scientists F. Uglov and A. Popov proposed to distinguish between 4 elevated levels of hypertension in the pulmonary artery:

• I degree (mild) – from 25 to 40 mm Hg. Art.;
• II degree (moderate) – from 42 to 65;
• III - from 76 to 110;
• IV - above 110.

Examination methods used in the diagnosis of hypertrophy of the right chambers of the heart:

1. X-ray - indicates an expansion of the right borders of the cardiac shadow, an increase in the arch of the pulmonary artery, and reveals its aneurysm.
2. Ultrasound methods (ultrasound) - allow you to accurately determine the size of the heart chambers and the thickness of the walls. A type of ultrasound - Dopplerography - shows disturbances in blood flow, flow speed, and the presence of obstacles.
3. Electrocardiography - reveals early signs of hypertrophy of the right ventricle and atrium by a characteristic deviation to the right of the electrical axis, an enlarged atrial “P” wave.
4. Spirography is a method of studying the possibility of breathing; it determines the degree and type of respiratory failure.
5. In order to detect the causes of pulmonary hypertension, pulmonary tomography is performed using x-ray sections of different depths or in a more modern way - computed tomography.

More complex methods (radionuclide scintigraphy, angiopulmonography). A biopsy to study the condition of lung tissue and vascular changes is used only in specialized clinics.

When catheterizing the cavities of the heart, not only the pressure is measured, but also the oxygen saturation of the blood is measured. This helps in identifying the causes of secondary hypertension. During the procedure, vasodilators are administered and the reaction of the arteries is checked, which is necessary in the choice of treatment.

How is the treatment carried out?

Treatment of pulmonary hypertension is aimed at excluding the underlying pathology that caused the increase in pressure.

At the initial stage, anti-asthmatic drugs and vasodilators provide assistance. Folk remedies can further enhance the allergic mood of the body.

If a patient has chronic embolization, the only remedy is surgical removal of the thrombus (embolectomy) by excision from the pulmonary trunk. The operation is carried out in specialized centers; a transition to artificial blood circulation is necessary. Mortality reaches 10%.

Primary pulmonary hypertension is treated with calcium channel blockers. Their effectiveness leads to a decrease in pressure in the pulmonary arteries in 10–15% of patients, accompanied by good response from seriously ill patients. This is considered a favorable sign.

An analogue of Prostacyclin, Epoprostenol, is administered intravenously through a subclavian catheter. Inhaled forms of medications (Iloprost), Beraprost tablets orally are used. The effect of subcutaneous administration of a drug such as Treprostinil is being studied.

Bosentan is used to block receptors that cause vasospasm.

At the same time, patients need drugs to compensate for heart failure, diuretics, and anticoagulants.

The use of solutions of Eufillin and No-shpa has a temporary effect.

Are there any folk remedies?

It is impossible to cure pulmonary hypertension with folk remedies. Recommendations on the use of diuretics and cough suppressants are used very carefully.

You should not get carried away with healing for this pathology. Lost time in diagnosis and initiation of therapy may be lost forever.

Forecast

Without treatment, the average survival time for patients is 2.5 years. Treatment with Epoprostenol increases the lifespan to five years in 54% of patients. The prognosis of pulmonary hypertension is unfavorable. Patients die from increasing right ventricular failure or thromboembolism.

Patients with pulmonary hypertension due to heart disease and arterial sclerosis live up to 32–35 years of age. The crisis course aggravates the patient’s condition and is regarded as an unfavorable prognosis.

The complexity of the pathology requires maximum attention to cases of frequent pneumonia and bronchitis. Prevention of pulmonary hypertension consists of preventing the development of pneumosclerosis, emphysema, early detection and surgical treatment of congenital defects.

Why is pulmonary hypertension dangerous and how to treat it?

1. Description of the disease
2. Causes of pulmonary hypertension
3. Classification of the disease
4. Signs of PH
5. Treatment of PH
6. Potential Complications
7. Pulmonary hypertension in children
8. PH prognosis
9. Recommendations for the prevention of PH

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 by a dozen Hg), it provokes its hypertrophy (growth) with subsequent decompensation, a sharp decrease in the force of contractions and premature death.

Description of the disease

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.

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.

Causes of pulmonary hypertension

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.

If endothelial function is impaired, the prerequisite may be a genetic predisposition or exposure to external aggressive factors. In each case, this leads to disruption of the metabolic processes of nitric oxide, changes in 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).

Classification of the disease

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 dizzy, fainting, weakness, painful sensations in the sternum, or severe shortness of breath.
2. At the next stage, the patient's physical capabilities are limited. The calm state does not cause any complaints, but with a standard load, shortness of breath, loss of strength, and loss of coordination appear.
3. At the third stage of the development of the disease, all of these 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.

Signs of PH

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.

Diagnosis of pulmonary hypertension

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 medical history. 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 - everything plays a role in establishing the causes of shortness of breath.
• A physical examination allows you to assess the condition of the neck veins, skin tone (cyanotic in case of hypertension), liver size (in this case, enlarged), the presence of swelling and thickening of the fingers.
• An ECG is performed to detect changes in the right side of the heart.
• Echocardiography helps determine the speed of blood flow and changes in the arteries.
• A CT scan 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 is used to accurately measure 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 test (biochemical and general).
• Pulmonary angiography by injecting contrast markers into the vessels 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.

Treatment of PH

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 of the patient’s poor health;
• 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.

Potential Complications

Among the negative consequences it should be noted:

• 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. PH provokes acute and chronic forms of heart and lung failure, which threaten the life of hypertensive patients.

Pulmonary hypertension in children

PH can develop both in adulthood 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. And yet, therapeutic 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.

PH prognosis

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.

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.

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 the clinical manifestations of the disease, improve the quality of life, and 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.

Chronic thromboembolic pulmonary hypertension

• Russian Medical Society on Arterial Hypertension

Keywords

• chronic thromboembolic pulmonary hypertension
• pulmonary hypertension
• pulmonary arterial hypertension
• idiopathic pulmonary hypertension
• pulmonary artery pressure
• phosphodiesterase type 5 inhibitors
• Nitric oxide
• endothelin receptor antagonists
• prostaglandin E1
• stimulators of soluble guanylate cyclase
• right heart catheterization
• thromboendarterectomy
• pulmonary embolism
• balloon angioplasty

List of abbreviations

Endothelin receptor antagonists

Activated partial thromboplastin time

Pulmonary artery wedge pressure

Pulmonary artery pressure/mean pulmonary artery pressure

Right atrial pressure

Cardiac ischemia

Phosphodiesterase type 5 inhibitors

Right heart catheterization

Pulmonary arterial hypertension/pulmonary hypertension

Pulmonary vascular resistance

Inferior vena cava

Acute pharmacological test

Randomized clinical trial

Pulmonary artery systolic pressure

6 Minute Walk Test

Pulmonary embolism

Chronic thromboembolic pulmonary hypertension

Extracorporeal membrane oxygenation

Oxygen/carbon dioxide partial pressure

N-terminal fragment of brain natriuretic propeptide

Terms and Definitions

Chronic thromboembolic pulmonary hypertension (CTEPH) is a precapillary form of pulmonary hypertension, in which chronic obstruction of the large and medium branches of the pulmonary arteries, as well as secondary changes in the microvasculature of the lungs, lead to a progressive increase in pulmonary vascular resistance and pressure in the pulmonary artery with the development of severe dysfunction of the right sections heart and heart failure.

1. Brief information

1.1. Definition

Chronic thromboembolic pulmonary hypertension (CTEPH) is a precapillary form of pulmonary hypertension, in which chronic obstruction of the large and medium branches of the pulmonary arteries, as well as secondary changes in the microvasculature of the lungs, lead to a progressive increase in pulmonary vascular resistance and pressure in the pulmonary artery with the development of severe dysfunction of the right sections heart and heart failure. CTEPH is a unique form of pulmonary hypertension (PH) because it is potentially curable with surgery.

The diagnosis of CTEPH is established if the criteria for precapillary PH are met according to right heart catheterization (RHC): mean pulmonary artery pressure (MPAP) ? 25 mmHg; pulmonary artery wedge pressure (PAWP)? 15 mmHg; pulmonary vascular resistance (PVR) > 2 units. Wood (all hemodynamic parameters should be measured at rest); detection of chronic/organized thrombi/emboli in the pulmonary arteries of the elastic type (pulmonary trunk, lobar, segmental, subsegmental pulmonary arteries) with effective anticoagulant therapy for at least 3 months.

1.2.Etiology and pathogenesis

Despite the fact that the prevalence of venous thrombosis is quite high, and the risk of acute venous thrombosis during a person’s lifetime reaches 5%, CTEPH is a rare disease. Acute pulmonary embolism (PE) is an undoubted trigger for the development and progression of CTEPH, however, anamnestic indications of previous episode occurs in no more than half of patients with a verified diagnosis. Foreign studies have carefully studied factors associated with the development of CTEPH. Independent risk factors for the development of CTEPH include: previous splenectomy, ventriculovenous shunts for the treatment of hydrocephalus, installation of central intravenous catheters or pacemaker leads, thyroid hormone replacement therapy, cancer and chronic inflammatory diseases (Table 1).

Provoking factors for the development of acute pulmonary embolism are severe injuries, surgical interventions, fractures of the lower extremities and joints, and the use of oral contraceptives. Blood transfusions and erythropoiesis-stimulating drugs are also associated with an increased risk of PE.

Table 1 - Risk factors for chronic thromboembolic pulmonary hypertension

Diseases and conditions associated with CTEPH

Risk factors for the development of CTEPH identified during the diagnosis of acute PE

• Splenectomy
• Ventriculoatrial shunts (to treat hydrocephalus)
• Central intravenous catheters and pacemaker leads
• Chronic inflammatory diseases (osteomyelitis, inflammatory bowel disease)
• Cancerous diseases
• Hormone replacement therapy for hypothyroidism

• Young age
• Previous pulmonary embolism
• Idiopathic pulmonary embolism (no precipitating factors)
• Major perfusion defect
• Repeated pulmonary embolism

Risk factors identified

during the diagnosis of CTEPH

Plasma risk factors,

associated with CTEPH

• Blood group II,III,IV
• Thrombophilia
• Major perfusion defect

• Antiphospholipid syndrome
• Hemoglobinopathies
• Factor V mutations
• Elevated levels of factor VIII

elevated lipoprotein(a) levels

In the blood of patients with CTEPH, lupus anticoagulant (10% of patients), antiphospholipid antibodies and/or lupus anticoagulant (20%) are more often detected. Increased activity of factor VIII, a protein associated with the development of venous thrombosis, is found in 39% of patients with CTEPH. Patients with CTEPH are characterized by the presence of blood group II, III or IV, in which, as a rule, increased levels of von Willebrand factor are detected. Fibrinolysis disorders are not typical. If the traditional risk factors for venous thromboembolism are deficiency of antithrombin III, protein C and S, deficiency of factor V and plasminogen, then when studying these factors in patients with CTEPH compared with patients with idiopathic PH and healthy volunteers, no differences between groups could be identified. Elevated levels of total cholesterol and lipoprotein(a) are often found in the plasma of patients with CTEPH. The latter competes with plasminogen, suppressing fibrinolysis, and may be involved in damage to the vascular wall.

When analyzing risk factors for the development of CTEPH during the diagnosis of acute PE, the predictors of long-term complications were: young age of patients and a large perfusion defect according to lung scintigraphy during the period of acute PE. Hereditary thrombophilia or anamnestic indications of venous thrombosis were not associated with the formation of CTEPH. Insufficient anticoagulant therapy, a large mass of thrombotic masses, residual thrombi and recurrent pulmonary embolism contribute to the development of CTEPH. However, when studying independent predictors of CTEPH 12 months after PE, the type of therapy (thrombolytics or heparins) did not affect the incidence of the disease in subsequent years.

Figure 1. Pathogenesis of chronic thromboembolic pulmonary hypertension

The complex and complex pathogenesis of CTEPH remains incompletely understood to date. When studying the possible genetic substrate, no specific mutations responsible for the development of the disease were identified. The basis of the pathobiological processes is the formation of thrombotic masses that have not undergone lysis, which later become fibrotic, which leads to mechanical obstruction of the large and medium branches of the pulmonary arteries (Fig. 1). The initial increase in pulmonary artery pressure (PAP) causes the development of secondary vascular changes. Remodeling of the pulmonary microvasculature contributes to the progression of PH even in the absence of recurrent thromboembolic events. The final outcome of the disease, as with other forms of PH, is severe right ventricular (RV) dysfunction and heart failure.

PE is considered to be an acute episode with apparent reversibility if thrombolysis is effective. However, during prospective observation of patients who have suffered acute pulmonary embolism, ventilation-perfusion scintigraphy and computed tomography (CT) of the lungs reveal significant perfusion abnormalities in 57% and 52% of patients, respectively. When screening for CTEPH using echocardiography (EchoCG), 2-44% of patients show signs of increased PAP and/or RV overload. 12 months after PE, the disease more often developed in patients with pulmonary artery systolic pressure (SPAP) > 50 mm Hg. during an acute episode. This fact indicates the likelihood of developing CTEPH in patients who have suffered acute PE with persistent obstructive lesions of the pulmonary vascular bed. Embolic material is not completely lysed, is replaced by connective tissue, and deforms the lumen of blood vessels. If the overall degree of stenosis of the pulmonary vascular bed reaches 50-60%, CTEPH may develop.

Unlike acute pulmonary embolism, in CTEPH there is no linear relationship between the degree of increase in PVR and the severity of obstructive lesions of the pulmonary vessels. The morphological substrate of CTEPH is also: generalized spasm of arterioles of the pulmonary circulation due to the release of vasoconstrictive substances from platelets and endothelium, secondary thrombus formation, progressive remodeling of small pulmonary arteries and arterioles. Pulmonary embolism or in situ thrombosis can occur due to disturbances in the blood coagulation cascade, including including dysfunction of endothelial cells and platelets. Platelet pathology and procoagulative changes may play a potential role in the formation of local thrombosis in CTEPH. In most cases, it remains unclear whether thrombosis and platelet dysfunction are a cause or a consequence of the disease. Inflammatory infiltrates, as a rule, are found in the morphological material obtained during thromboendarterectomy. Frequent detection of pathology of the immune system, cancer, chronic infections indicates the participation of inflammatory and immune mechanisms in the pathogenesis of CTEPH.

Obstructive lesions observed in the distal pulmonary arteries may be associated with various factors, such as shear stress due to increased pressure, inflammatory processes, and the release of cytokines and mediators that promote cell proliferation. In these cases, the disease is likely initiated by thrombotic or inflammatory damage to the pulmonary vessels. There are hypotheses that CTEPH may develop as a result of in-situ thrombus formation in altered arteries without a previous episode of PE, but this concept requires confirmation.

Thus, changes in the pulmonary arteries in CTEPH can be divided into two components. The first component includes changes in large vessels to the level of subsegmental branches. Pathological lesions are presented in the form of organized blood clots, tightly attached to the vascular wall of the pulmonary arteries of the elastic type. They can completely close the lumen or form varying degrees of stenosis, networks and cords. The second component is changes in small pulmonary vessels and microvascular bed. In non-occluded areas, arteriopathy indistinguishable from pulmonary arterial hypertension (PAH), including the formation of plexiform lesions, may develop. The severity of these components may vary. With a predominance of changes in large arteries, there is a “classical” technically resectable form of CTEPH. The dominance of the second component is characteristic of the distal form of the disease, which is considered inoperable.

CTEPH is characterized by the development of collateral blood flow from the vessels of the systemic circulation up to reperfusion, at least partial, in areas distal to the occluded lesions. The role of collaterals is performed by the bronchial arteries, the arteries of the diaphragm and the posterior mediastinum. In some cases, the adhesive process is extremely pronounced with partial revascularization through formed adhesions. Bronchial arteries most often arise from the aortic arch and are clearly visible on CT scans. Detection of large collateral arteries confirms the diagnosis of CTEPH.

1.3. Epidemiology

Accurate epidemiological data on the prevalence of CTEPH in the world and in our country are currently lacking. According to foreign observations, in the spectrum of various forms of PH, the share of patients with CTEPH accounts for about 1.5-3%. CTEPH is a fairly rare disease with a prevalence of 8-40 cases per 1 million population, incidence - 5-10 cases per 1 million population per year. According to the Spanish PH registry, the prevalence and incidence of CTEPH were 3.2 cases per 1 million population and 0.9 cases per 1 million per year in the population, respectively. In the UK, the incidence of CTEPH is estimated at 1-1.75 cases per 1 million population per year. In an epidemiological study in Switzerland, the diagnosis of CTEPH was confirmed in 29% of patients with detected PH.

Venous thrombosis is a common clinical event with an incidence, according to various sources, from 20 to 70 cases per 100 thousand. population per year, CTEPH is a long-term complication of acute pulmonary embolism with an incidence of 0.1-9.1% during the first two years after the episode.

It is believed that the true prevalence of CTEPH may be significantly higher. Indeed, 50-60% of patients with CTEPH have no history of acute pulmonary embolism or deep vein thrombosis of the lower extremities, which makes it difficult to timely diagnose the disease and assess the true incidence and prevalence. In most studies, the incidence of CTEPH was assessed during the first months after acute PE, although the onset of the disease may be delayed for many years due to the gradual development of distal vasculopathy.

The diagnosis of CTEPH is often made at older ages. The average age of Russian patients at the time of diagnosis, according to the National Register, is 45.8±13.7 years. It is believed that the prevalence of pathology in the population among women and men is equal. In the Japanese study, CTEPH was more often diagnosed in women, which coincides with Russian data: in the CTEPH registry, the proportion of women with a verified diagnosis reaches 75%. This pathology is rare in children.

In the absence of treatment, the prognosis of CTEPH is unfavorable and depends on the degree of PH. According to foreign studies, the ten-year survival rate of patients with inoperable CTEPH with PAP is in the range of mmHg. is 50%; at DLAsr. from 41 to 50 mmHg. -20%; at DLAsr. more than 50 mmHg - 5%. According to the international registry of CTEPH, the 3-year survival rate of patients after successful thromboendarterectomy (TEE) was 89%, while for inoperable forms it was 70%.

1.4.Coding according to ICD

I27.8 - Other specified forms of pulmonary heart failure

1.5. Classification

In the modern clinical classification of PH, created with the aim of standardizing diagnostic and therapeutic measures based on the similarity of pathogenetic and clinical features, approaches to diagnosis and treatment, five categories or groups of PH are distinguished. The clinical classification of PH is presented in the clinical recommendations “Pulmonary hypertension”. CTEPH, along with with other pulmonary artery obstructions (tumors, vascular anomalies, emboli), belongs to group IV (Table 2).

Table 2. Clinical classification of CTEPH (group IV)

2. Diagnostics

The diagnostic strategy for CTEPH involves conducting a comprehensive examination to establish a diagnosis of PH and confirm the clinical group, as well as assessing the functional and hemodynamic status of patients to determine treatment tactics and, above all, resolve the issue of potential operability. The stages of the diagnostic and differential diagnostic process are presented in Figure 2.

Figure 2. Algorithm for diagnosing chronic thromboembolic pulmonary hypertension

CTEPH occurs in almost all age groups, more often in middle-aged and elderly patients.

• In all patients with PH of unknown origin, it is recommended to exclude the thromboembolic nature of the disease.

The primary conditions have not been sufficiently studied, but the role of the vasoconstrictor mechanism, thickening of the arterial wall, and fibrosis (tissue compaction) has been proven.

In accordance with ICD-10 (International Classification of Diseases), only the primary form of pathology is coded as I27.0. All secondary symptoms are added as complications to the underlying chronic disease.

Some features of the blood supply to the lungs

The lungs have a double blood supply: a system of arterioles, capillaries and venules is included in gas exchange. And the tissue itself receives nutrition from the bronchial arteries.

The pulmonary artery is divided into right and left trunks, then into branches and lobar vessels of large, medium and small caliber. The smallest arterioles (part of the capillary network) have a diameter 6–7 times larger than in the systemic circulation. Their powerful muscles are capable of narrowing, completely closing or expanding the arterial bed.

With narrowing, resistance to blood flow increases and internal pressure in the vessels increases; expansion reduces pressure and reduces the force of resistance. The occurrence of pulmonary hypertension depends on this mechanism. The total network of pulmonary capillaries covers an area of ​​140 m2.

The veins of the pulmonary circle are wider and shorter than those in the peripheral circulation. But they also have a strong muscle layer and are able to influence the pumping of blood towards the left atrium.

How is pressure in the pulmonary vessels regulated?

The amount of blood pressure in the pulmonary vessels is regulated by:

• pressor receptors in the vascular wall;
• branches of the vagus nerve;
• sympathetic nerve.

Extensive receptor zones are located in large and medium-sized arteries, at branching points, and in veins. Arterial spasm leads to impaired oxygen saturation of the blood. And tissue hypoxia promotes the release of substances into the blood that increase tone and cause pulmonary hypertension.

Irritation of the vagus nerve fibers increases blood flow through the lung tissue. The sympathetic nerve, on the contrary, causes a vasoconstrictor effect. Under normal conditions, their interaction is balanced.

The following indicators of pressure in the pulmonary artery are accepted as the norm:

• systolic (upper level) - from 23 to 26 mm Hg;
• diastolic - from 7 to 9.

Pulmonary arterial hypertension, according to international experts, begins at the upper level – 30 mmHg. Art.

Factors causing hypertension in the pulmonary circulation

The main factors of pathology, according to V. Parin’s classification, are divided into 2 subtypes. Functional factors include:

• constriction of arterioles in response to low oxygen levels and high concentrations of carbon dioxide in the inhaled air;
• increase in minute volume of passing blood;
• increased intrabronchial pressure;
• increased blood viscosity;
• left ventricular failure.

Anatomical factors include:

• complete obliteration (blocking of the lumen) of blood vessels by a thrombus or embolus;
• impaired outflow from the zonal veins due to their compression due to aneurysm, tumor, mitral stenosis;
• changes in blood circulation after surgical removal of a lung.

What causes secondary pulmonary hypertension?

Secondary pulmonary hypertension occurs due to known chronic diseases of the lungs and heart. These include:

• chronic inflammatory diseases of the bronchi and lung tissue (pneumosclerosis, emphysema, tuberculosis, sarcoidosis);
• thoracogenic pathology in violation of the structure of the chest and spine (Bechterew's disease, consequences of thoracoplasty, kyphoscoliosis, Pickwick's syndrome in obese people);
• mitral stenosis;
• congenital heart defects (for example, patent ductus arteriosus, “windows” in the interatrial and interventricular septum);
• tumors of the heart and lungs;
• diseases accompanied by thromboembolism;
• vasculitis in the area of ​​the pulmonary arteries.

What causes primary hypertension?

Primary pulmonary hypertension is also called idiopathic, isolated. The prevalence of the pathology is 2 people per 1 million inhabitants. The definitive reasons remain unclear.

It has been established that women make up 60% of patients. Pathology is detected both in childhood and in old age, but the average age of identified patients is 35 years.

In the development of pathology, 4 factors are important:

• primary atherosclerotic process in the pulmonary artery;
• congenital inferiority of the wall of small vessels;
• increased tone of the sympathetic nerve;
• vasculitis of the pulmonary vessels.

The role of the mutating bone protein gene, angioproteins, their influence on the synthesis of serotonin, and increased blood clotting due to the blocking of anticoagulant factors has been established.

A special role is given to infection with the herpes virus type eight, which causes metabolic changes leading to the destruction of arterial walls.

The result is hypertrophy, then expansion of the cavity, loss of right ventricular tone and the development of failure.

Other causes and factors of hypertension

There are many causes and lesions that can cause hypertension in the pulmonary circle. Some of them need to be highlighted.

Among acute diseases:

• respiratory distress syndrome in adults and newborns (toxic or autoimmune damage to the membranes of the respiratory lobes of the lung tissue, causing a lack of surfactant substance on its surface);
• severe diffuse inflammation (pneumonitis), associated with the development of a massive allergic reaction to inhaled odors of paint, perfume, and flowers.

In this case, pulmonary hypertension can be caused by food, drugs and folk remedies.

Pulmonary hypertension in newborns can be caused by:

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

In children, hypertension is promoted by enlarged tonsils.

Classification according to the nature of the flow

It is convenient for clinicians to divide hypertension in the pulmonary vessels according to the timing of development into acute and chronic forms. Such a classification helps to “combine” the most common causes and clinical course.

Acute hypertension occurs due to:

• pulmonary embolism;
• severe status asthmaticus;
• respiratory distress syndrome;
• sudden left ventricular failure (due to myocardial infarction, hypertensive crisis).

The chronic course of pulmonary hypertension is caused by:

• increased pulmonary blood flow;
• increase in resistance in small vessels;
• increased pressure in the left atrium.

A similar development mechanism is typical for:

• defects of the interventricular and interatrial septum;
• patent ductus arteriosus;
• mitral valve disease;
• proliferation of myxoma or thrombus in the left atrium;
• gradual decompensation of chronic left ventricular failure, for example, with ischemic disease or cardiomyopathies.

The following diseases lead to chronic pulmonary hypertension:

• hypoxic nature - all obstructive diseases of the bronchi and lungs, prolonged oxygen deficiency at altitude, hypoventilation syndrome associated with chest injuries, mechanical breathing;
• mechanical (obstructive) origin, associated with narrowing of the arteries - reaction to drugs, all variants of primary pulmonary hypertension, recurrent thromboembolism, connective tissue diseases, vasculitis.

Clinical picture

Symptoms of pulmonary hypertension appear if the pressure in the pulmonary artery is increased by 2 times or more. Patients with hypertension in the pulmonary circle notice:

• shortness of breath, which worsens with physical activity (can develop in paroxysms);
• general weakness;
• rarely loss of consciousness (as opposed to neurological causes without convulsions and involuntary urination);
• paroxysmal chest pains, similar to angina pectoris, but accompanied by an increase in shortness of breath (scientists explain them by a reflex connection between the pulmonary and coronary vessels);
• the admixture of blood in the sputum when coughing is characteristic of significantly increased pressure (associated with the release of red blood cells into the interstitial space);
• hoarseness is detected in 8% of patients (caused by mechanical compression of the recurrent nerve on the left by the dilated pulmonary artery).

The development of decompensation as a result of pulmonary heart failure is accompanied by pain in the right hypochondrium (liver stretching), swelling in the feet and legs.

When examining a patient, the doctor pays attention to the following:

• a blue tint to the lips, fingers, ears, which intensifies as shortness of breath becomes more severe;
• the symptom of “drum” fingers is detected only in long-term inflammatory diseases and defects;
• the pulse is weak, arrhythmias are rare;
• blood pressure is normal, with a tendency to decrease;
• palpation in the epigastric zone makes it possible to determine increased impulses of the hypertrophied right ventricle;
• On auscultation, an accentuating second sound on the pulmonary artery is heard, and a diastolic murmur is possible.

The relationship of pulmonary hypertension with persistent causes and certain diseases allows us to distinguish variants in the clinical course.

Portopulmonary hypertension

Pulmonary hypertension leads to a simultaneous increase in portal vein pressure. In this case, the patient may or may not have cirrhosis of the liver. It accompanies chronic liver diseases in 3–12% of cases. The symptoms are no different from those listed. Swelling and heaviness in the hypochondrium on the right are more pronounced.

Pulmonary hypertension with mitral stenosis and atherosclerosis

The disease varies in severity. Mitral stenosis contributes to the occurrence of atherosclerotic lesions of the pulmonary artery in 40% of patients due to increased pressure on the vessel wall. Functional and organic mechanisms of hypertension are combined.

The narrowed left atrioventricular passage in the heart is the “first barrier” to blood flow. If there is narrowing or blockage of small vessels, a “second barrier” is formed. This explains the failure of surgery to eliminate stenosis in the treatment of heart disease.

By catheterization of the heart chambers, high pressure inside the pulmonary artery is detected (150 mm Hg and above).

Vascular changes progress and become irreversible. Atherosclerotic plaques do not grow to large sizes, but they are sufficient to narrow small branches.

Pulmonary heart

The term “cor pulmonale” includes a symptom complex caused by damage to the lung tissue (pulmonary form) or pulmonary artery (vascular form).

There are flow options:

1. acute - typical for pulmonary artery embolization;
2. subacute - develops with bronchial asthma, lung carcinomatosis;
3. chronic - caused by emphysema, a functional spasm of the arteries, turning into an organic narrowing of the artery, characteristic of chronic bronchitis, pulmonary tuberculosis, bronchiectasis, frequent pneumonia.

An increase in resistance in the vessels puts a pronounced load on the right heart. The general lack of oxygen also affects the myocardium. The thickness of the right ventricle increases with the transition to dystrophy and dilatation (persistent expansion of the cavity). Clinical signs of pulmonary hypertension gradually increase.

Hypertensive crises in the vessels of the “small circle”

A crisis course often accompanies pulmonary hypertension associated with heart defects. A sharp deterioration in the condition due to a sudden increase in pressure in the pulmonary vessels is possible once a month or more often.

• increased shortness of breath in the evening;
• feeling of external compression of the chest;
• severe cough, sometimes with hemoptysis;
• pain in the interscapular region radiating to the anterior sections and sternum;
• cardiopalmus.

Upon examination, the following is revealed:

• patient's agitated state;
• inability to lie in bed due to shortness of breath;
• pronounced cyanosis;
• weak rapid pulse;
• visible pulsation in the area of ​​the pulmonary artery;
• swollen and pulsating neck veins;
• excretion of copious amounts of light-colored urine;
• involuntary defecation is possible.

Diagnostics

Diagnosis of hypertension in the pulmonary circulation is based on identifying its signs. These include:

• hypertrophy of the right heart;
• determination of increased pressure in the pulmonary artery based on the results of measurements using catheterization.

Russian scientists F. Uglov and A. Popov proposed to distinguish between 4 elevated levels of hypertension in the pulmonary artery:

• I degree (mild) – from 25 to 40 mm Hg. Art.;
• II degree (moderate) – from 42 to 65;
• III - from 76 to 110;
• IV - above 110.

Examination methods used in the diagnosis of hypertrophy of the right chambers of the heart:

1. X-ray - indicates an expansion of the right borders of the cardiac shadow, an increase in the arch of the pulmonary artery, and reveals its aneurysm.
2. Ultrasound methods (ultrasound) - allow you to accurately determine the size of the heart chambers and the thickness of the walls. A type of ultrasound - Dopplerography - shows disturbances in blood flow, flow speed, and the presence of obstacles.
3. Electrocardiography - reveals early signs of hypertrophy of the right ventricle and atrium by a characteristic deviation to the right of the electrical axis, an enlarged atrial “P” wave.
4. Spirography is a method of studying the possibility of breathing; it determines the degree and type of respiratory failure.
5. In order to detect the causes of pulmonary hypertension, pulmonary tomography is performed using x-ray sections of different depths or in a more modern way - computed tomography.

More complex methods (radionuclide scintigraphy, angiopulmonography). A biopsy to study the condition of lung tissue and vascular changes is used only in specialized clinics.

When catheterizing the cavities of the heart, not only the pressure is measured, but also the oxygen saturation of the blood is measured. This helps in identifying the causes of secondary hypertension. During the procedure, vasodilators are administered and the reaction of the arteries is checked, which is necessary in the choice of treatment.

How is the treatment carried out?

Treatment of pulmonary hypertension is aimed at excluding the underlying pathology that caused the increase in pressure.

At the initial stage, anti-asthmatic drugs and vasodilators provide assistance. Folk remedies can further enhance the allergic mood of the body.

If a patient has chronic embolization, the only remedy is surgical removal of the thrombus (embolectomy) by excision from the pulmonary trunk. The operation is carried out in specialized centers; a transition to artificial blood circulation is necessary. Mortality reaches 10%.

Primary pulmonary hypertension is treated with calcium channel blockers. Their effectiveness leads to a decrease in pressure in the pulmonary arteries in 10–15% of patients, accompanied by good response from seriously ill patients. This is considered a favorable sign.

An analogue of Prostacyclin, Epoprostenol, is administered intravenously through a subclavian catheter. Inhaled forms of medications (Iloprost), Beraprost tablets orally are used. The effect of subcutaneous administration of a drug such as Treprostinil is being studied.

Bosentan is used to block receptors that cause vasospasm.

At the same time, patients need drugs to compensate for heart failure, diuretics, and anticoagulants.

The use of solutions of Eufillin and No-shpa has a temporary effect.

Are there any folk remedies?

It is impossible to cure pulmonary hypertension with folk remedies. Recommendations on the use of diuretics and cough suppressants are used very carefully.

You should not get carried away with healing for this pathology. Lost time in diagnosis and initiation of therapy may be lost forever.

Forecast

Without treatment, the average survival time for patients is 2.5 years. Treatment with Epoprostenol increases the lifespan to five years in 54% of patients. The prognosis of pulmonary hypertension is unfavorable. Patients die from increasing right ventricular failure or thromboembolism.

Patients with pulmonary hypertension due to heart disease and arterial sclerosis live up to 32–35 years of age. The crisis course aggravates the patient’s condition and is regarded as an unfavorable prognosis.

The complexity of the pathology requires maximum attention to cases of frequent pneumonia and bronchitis. Prevention of pulmonary hypertension consists of preventing the development of pneumosclerosis, emphysema, early detection and surgical treatment of congenital defects.

Why is pulmonary hypertension dangerous and how to treat it?

1. Description of the disease
2. Causes of pulmonary hypertension
3. Classification of the disease
4. Signs of PH
5. Treatment of PH
6. Potential Complications
7. Pulmonary hypertension in children
8. PH prognosis
9. Recommendations for the prevention of PH

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 by a dozen Hg), it provokes its hypertrophy (growth) with subsequent decompensation, a sharp decrease in the force of contractions and premature death.

Description of the disease

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.

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.

Causes of pulmonary hypertension

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.

If endothelial function is impaired, the prerequisite may be a genetic predisposition or exposure to external aggressive factors. In each case, this leads to disruption of the metabolic processes of nitric oxide, changes in 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).

Classification of the disease

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 dizzy, fainting, weakness, painful sensations in the sternum, or severe shortness of breath.
2. At the next stage, the patient's physical capabilities are limited. The calm state does not cause any complaints, but with a standard load, shortness of breath, loss of strength, and loss of coordination appear.
3. At the third stage of the development of the disease, all of these 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.

Signs of PH

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.

Diagnosis of pulmonary hypertension

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 medical history. 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 - everything plays a role in establishing the causes of shortness of breath.
• A physical examination allows you to assess the condition of the neck veins, skin tone (cyanotic in case of hypertension), liver size (in this case, enlarged), the presence of swelling and thickening of the fingers.
• An ECG is performed to detect changes in the right side of the heart.
• Echocardiography helps determine the speed of blood flow and changes in the arteries.
• A CT scan 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 is used to accurately measure 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 test (biochemical and general).
• Pulmonary angiography by injecting contrast markers into the vessels 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.

Treatment of PH

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 of the patient’s poor health;
• 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.

Potential Complications

Among the negative consequences it should be noted:

• 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. PH provokes acute and chronic forms of heart and lung failure, which threaten the life of hypertensive patients.

Pulmonary hypertension in children

PH can develop both in adulthood 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. And yet, therapeutic 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.

PH prognosis

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.

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.

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 the clinical manifestations of the disease, improve the quality of life, and 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.

Please note that all information posted on the site is for reference only and

not intended for self-diagnosis and treatment of diseases!

Copying of materials is permitted only with an active link to the source.

Millions of people around the world suffer from diseases of the cardiovascular system, which have firmly taken the lead among ailments leading to death. These include pulmonary hypertension (PH). What is stage 1 pulmonary hypertension, what are the symptoms of the disease, how to minimize the negative impact on the body, what diagnostic and treatment methods are used by modern medicine? The answers to these questions will help to create a real picture of an insidious and difficult-to-treat disease and improve the patient’s quality of life.

What it is?

Pulmonary hypertension is a type of hypertension, the characteristic symptom of which is increased pressure in the arteries of the lungs. The result of the pathology is a narrowing of the lumen in the lung vessel due to the abnormal structure of the arteries of the respiratory organs. It is more typical for women of the middle age group. Men are three times less likely to be diagnosed with this pathology.

The disease manifests itself almost asymptomatically in the initial stages of its course. A person may not even be aware of its presence until the disease manifests itself with symptoms such as hypertensive crisis, pulmonary edema, and hemoptysis. That is, when the degree of development of the disease is high, the patient seeks medical help, which significantly complicates treatment.

With pulmonary hypertension of the 1st degree, there is no shortness of breath and pain in the chest, there are no unpleasant sensations during normal walking, and there are none during other moderately active actions of the body. Excessive fatigue and weakness are not observed.

Hypertension is classified according to several parameters:

• Current
• Severity of the disease

At the diagnostic stage, the main factor indicating the presence of this pathology is increased pulmonary pressure in a calm and active state of the body. It is measured in millimeters of mercury. The parameters are established by ICD-10 (International Classification of Diseases) and should be no more than 25 (at rest) and 35 (during certain physical activities). The ICD 10 code for pulmonary hypertension (127.0) is established only for diseases of the primary type.

The primary and secondary types of the disease can only be determined by exclusion. Secondary pulmonary hypertension appears as a result of acquired diseases, that is, it is the result of the negative impact of other diseases on the circulatory system.

Causes of hypertension:

• Chronic inflammatory processes such as tuberculosis, liver cirrhosis, pneumosclerosis
• Neoplasms in the heart muscle or respiratory tract
• Blood clots
• Pathologies in the structure of the spine or chest
• Congenital heart defect

The list of causes of idiopathic pulmonary hypertension includes:

Most often, specialists find it difficult to determine the mechanism of development of primary pulmonary hypertension. Let us recall that ICD 10 assigns code 127.0 to primary pulmonary hypertension.

Symptoms of the primary type of hypertension include:

• Shortness of breath of a different nature - from minimal to significant, appearing during heavy exertion or at rest. As a rule, there are no asthma attacks.
• Fainting and dizziness, lasting up to 5 minutes. Appear during significant physical exertion.
• Painful sensations of various types in the chest area. Duration ranges from a few minutes to several hours. Typically, taking medications that have a positive effect on vasodilation does not relieve pain.
• Dry cough, which can appear both during exercise and at rest.
• Interruptions in the functioning of the heart muscle.
• Sputum with blood during expectoration (single or continuous).

Depending on the severity of the course, several degrees of development are distinguished. At each stage, the disease manifests itself more clearly, limiting the patient in certain actions.

First degree (average systolic pressure in the range of 25-45 mm Hg) - subtle symptoms with preservation of physical activity. Absence of dizziness and shortness of breath with a traditional lifestyle.

Second degree (average systolic pressure 45-75 mm Hg) - there is respiratory failure with persistently elevated blood pressure. Chest pain and dizziness, the first signs of cardiac ventricular hypertrophy, are diagnosed.

The third degree (average systolic pressure from 76 to 110 mm Hg) is an irreversible stage with formed vascular atherosclerosis and complete hypertrophy of the heart ventricle. Previously identified manifestations include hemoptysis and an obsessive, prolonged cough. Small loads cause severe shortness of breath, and significant swelling appears in the legs and in the area of ​​the lymph nodes.

The fourth degree (average systolic pressure above 110 Hg) is high pulmonary hypertension, in which a person loses his ability to work and cannot partially or fully take care of himself without outside help. There is significant swelling of the lower extremities, a number of concomitant diseases appear, for example, the liver increases in size, diffuse cyanosis. Pain in the chest area and severe shortness of breath are present at rest.

According to the course, pulmonary hypertension is divided into acute and chronic. Provocateurs of the acute type are hypertensive crisis, myocardial infarction, pulmonary embolism, etc. Increased blood pressure in the left atrium, obstructive respiratory diseases, and other causes of narrowing of the pulmonary arteries are provocateurs of the development of the chronic type.

Diagnostics

It is important to know!

Vessels become dirty very quickly, especially in older people. To do this, you don’t need to eat burgers or fries all day long. It is enough to eat one sausage or scrambled egg for some amount of cholesterol to be deposited in the blood vessels. Over time, pollution accumulates...

Pulmonary hypertension is diagnosed by a number of clinical and laboratory tests. When seeing a patient with high pressure in the pulmonary artery, the doctor collects an anamnesis and conducts a survey.

To determine an accurate diagnosis and draw up a treatment program, the following procedures are necessary:

Depending on the patient’s condition, this disease may require a number of additional hardware tests. Their appointment is within the competence of the treating doctor.

To get a comprehensive answer to the question of how stage 1 pulmonary hypertension manifests itself - what it is, and how to cope with the disease, you need to seriously listen to the doctor’s recommendations and follow them exactly.

Experts say that only a responsible approach to diagnosis will make it possible to get a real picture of the disease and begin effective treatment. Unfortunately, self-medication will not give any results and can only cause harm.

Traditional medicine methods do not work for this pathology. They should be abandoned immediately so as not to worsen the disease. According to statistics, hypertension in the early stages can be easily eliminated with medications.

The specialist draws up a treatment protocol, according to which the patient is prescribed medications from the following groups:

• diuretics;
• anticoagulants for blood thinning;
• inotropic drugs and cardiac glycosides to increase the power of heart contractions;
• endothelin receptor antagonists to block receptors for substances that cause vasoconstriction;
• calcium antagonists to block slow calcium channels in cells;
• prostaglandins to regulate vascular tone;
• nitrogen oxides for vasodilation;

In addition to medication, surgical treatment methods are used. This could be a lung and heart transplant or just a respiratory transplant. Atrial septostomy, which involves creating a hole in the interatrial septum, is also used as a surgical treatment.

An important point in the treatment of the disease are such measures as:

• Limiting salt intake and drinking fluids (maximum 1.5 liters per day).
• Regular prevention of ARVI is vaccination, which can reduce the risk of contracting colds.
• Depending on the degree of idiopathic pulmonary hypertension according to ICD 10, the doctor has the right to limit or completely prohibit any physical activity.
• Procedures using oxygen.
• To avoid complications of the disease, which can be caused by pregnancy and subsequent labor, women are prescribed the use of contraceptives.

The International Classification of Diseases System of 2016, in its latest edition (ICD 10), assigns the status of a disease to primary pulmonary hypertension, due to which the patient may become disabled. To receive it, the patient must undergo a full medical examination confirming the presence of the disease, as well as the degree of its severity. There are disabilities of groups 1, 2 and 3.

Main disability criteria:

• Disability group I - restriction of movement of the third degree, loss of the ability to care for oneself, without partial or complete assistance, progressive course of hypertension.
• Disability group II is assigned to patients who have limited ability to work and motor functions. The treatment provided practically does not give the desired effect or the effect is limited to a certain period. Such patients can work from home if there are special conditions, for example, beading or knitting, or making souvenirs.
• Disability group III is given to patients with limited ability not only to work, but also to move and self-care. They cannot work in professions associated with heavy physical or mental stress, where there is constant speech load during the working day. This degree of disability involves transferring the patient to light work, for example, clerical work, repairing radio equipment in appropriate production conditions.

The disability group is determined by a special commission (ITU). Depending on the severity of the disease, regular confirmation is required.