Causes, symptoms and treatment of pulmonary hypertension

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

Contents of the article:

  • Classification of pathology
  • Causes and risk factors for development of
  • Manifestations of pathology, disease classes
  • Diagnosis
  • Methods of treatment
  • Forecast

Pulmonary hypertension is a pathological condition in which there is a gradual increase in pressure in the vascular system of the lungs, which leads to increasing insufficiencythe work of the right ventricle and eventually ends with the premature death of a person.

Normal mean pulmonary arterial pressure( mmHg) Mean pressure for pulmonary hypertension( mmHg)
17-23 Over 25 at rest

Over 30 - with

load When a disease in the circulatory system of the lungsthe following pathological changes occur:

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  1. Narrowing or spasm of blood vessels( vasoconstriction).
  2. Decreased capacity of the vascular wall to stretch( elasticity).
  3. Formation of small blood clots.
  4. Spread of smooth muscle cells.
  5. Closure of the lumen of blood vessels due to blood clots and thickened walls( obliteration).
  6. Destruction of vascular structures and their replacement with connective tissue( reduction).

In order for blood to pass through altered vessels, there is an increase in pressure in the pulmonary artery trunk. This leads to an increase in pressure in the cavity of the right ventricle and leads to a disruption of its function.

Such changes in the blood flow show themselves increasing respiratory insufficiency in the first stages and severe heart failure - in the final disease. From the very beginning, the inability to breathe normally places significant restrictions on the patients' habitual life, forcing them to limit themselves to stresses. The reduction in resistance to manual labor is exacerbated as the disease progresses.

Pulmonary hypertension is considered a very serious disease - without treatment, patients live less than 2 years, and most of this time they need help in caring for themselves( cooking, cleaning the premises, purchasing products, etc.). When the therapy is carried out, the prognosis is somewhat improved, but it is impossible to completely cure the disease.

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The doctors of many specialties are engaged in diagnostics, treatment and supervision of people with pulmonary hypertension, depending on the cause of the disease development: it can be: therapists, pulmonologists, cardiologists, infectious disease specialists and genetics. If surgical correction is necessary, vascular and thoracic surgeons join.

Classification of pathology

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

This is the basis for the clinical classification of pressure increase in the pulmonary arterial system:

Basic group Subgroups of the disease
Pulmonary arterial hypertension Idiopathic or without a primary cause
Family( caused by mutations in certain genes)
Associated, or associated, with:
  • vascular collagen deposition( systemic lupus erythematosus, scleroderma);
  • congenital heart disease;
  • by hepatic hypertension;
  • with HIV infection;
  • by drug or toxic damage;
  • with thyroid disorders;
  • by Gosher's disease( deposition in the tissues of glucocerebroside);
  • with Rundu-Osler disease;
  • by glycogen storage disease;
  • pathologies of the structure of the hemoglobin molecule( hemoglobinopathy);
  • by removal of the spleen;
  • diseases, accompanied by increased activity of red bone marrow( myeloproliferative).
Associated with a large lesion of capillaries and veins:
  • pulmonary veno-occlusive disease( formation of thrombi in the venous system of the lungs),
  • pulmonary capillary hemangiomatosis( proliferation of small vessels with the formation of benign formations - hemangiomas).
Persistent long-term pulmonary hypertension of newborns
Pulmonary hypertension associated with lesion of the left heart Left ventricular lesion
Lesion of left ventricular valves
Hypertension associated with respiratory system diseases and / or low oxygen content in the blood Disturbance of lung developmenthereditary and congenital forms of underdevelopment)
Highland( associated with low air pressure in the mountains)
Pathology of breathing during sleep( nighttime syndrome
Dysfunction of the alveoli - the main active lung tissue units
Chronic obstructive pulmonary disease
Lesion of the intercellular space of the lung tissue( interstitium) - inflammation, proliferation of connective tissue
Pulmonary hypertension in the background of thromboembolism or embolism Blocking of the terminal branches of the pulmonary arteries
Overlapping the luminal opening of the primaryvascular branches
Non-clotting thrombosis:
  • by tumor masses;
  • by parasites;
  • foreign bodies.
Mixed forms of Sarcoidosis - formation of granulomas from connective tissue in the lung tissue
Histiocytosis - formation of foci of histiocyte accumulation in the lungs
Lymphangiomatosis - benign lymphatic vessel tumors
Depression of pulmonary vessels from the outside:
  • enlarged hilar lymph nodes( lymphadenopathy);
  • inflammation of the mediastinum.

Increased pressure in the circulatory system of the lungs due to a violation of the structure of the heart is noted for defects associated with the discharge of arterial blood into the venous system: defects of the partitions between the atria and ventricles, open arterial duct.

Causes and risk factors for the development of

The following causes can influence the increase in pressure in the pulmonary arteries:

Group factors proved Probable
Drugs and toxins aminorex Amphetamine
Rapeseed oil L-tryptophan
fenfluramine
dexfenfluramine
Medical and demographic Female sex Pregnancy
Hypertension
Diseases HIV Liver disease is replacement cellconnective tissue

Manifestations of pathology, disease classes

The primary stage of changes in the vascular system of the lungs is imperceptibleo - compensatory, replacement mechanisms cope with the developed pathology, and this is sufficient for the patient's normal state.

When the level of average pulmonary artery pressure reaches a critical value of 25-30 mmHg, Art.the first symptoms appear in pulmonary hypertension. At this stage, the ill person experiences a worsening of the state only with physical overstrain and lives a full life.

As the changes in the vessels progress, exercise tolerance decreases, and even household chores may require outside help.

Symptoms of the disease Their characteristic features
Shortness of breath Difficulty occurs only on inhalation

First manifestation of

pathology First occurs only with physical exertion

Late stages are present and at rest

No asthma attacks

Chest pains Can be any:compressive, pricking or pressing

No clear onset of pain

Duration from a few seconds to a day

Strengthened with physical stress

No efiction from taking Nitroglycerin

Dizziness, syncope Always associated with the load

Duration from 2-5 to 20-25 minutes

Cardiac abnormalities, heartbeat According to the ECG, sinus tachycardia is recorded
Cough Occurs in 1/3 of the diseased

In most casesof cases dry( if there is no concomitant disease of the respiratory system)

Sputum blood( hemoptysis) Only in 10% of patients

Appears once

Rarely persists for several days

Svyatzan with the formation of clots in small vessels

To assess the severity of the disease, depending on the severity of the manifestations, the division of pulmonary hypertension into classes is used.

A slight physical strain leads to a worsening of the

state At rest, the condition is satisfactory

The fourth Impossibility to perform any loads

Symptom manifestation even at rest

Diagnostics

Having decided that the disease for pulmonary hypertension, how it manifests itself and why it occurs, you can proceed to the specifics of the diagnosis. The table shows the methods of examining the patient and the pathological changes that are typical for increasing pressure in the circulatory system of the lungs. Symptoms of the disease that caused the pathology( if any) are not indicated in this article.

Diagnosis method

Change in the shape of fingertips and nails( thickening, "drumsticks" and "watch glass")

Increase in the size of the breastcells in patients with emphysema( "barrel")

Liver enlargement( hepatomegaly)

Fluidity accumulation in the abdominal and breast cavities( ascites, pleurisy)

Edema on the legs

Increase and swelling of veins on the neck

Auscultation andListening to the lungs and heart Strengthening 2 tones above the pulmonary artery

Systolic valve failure between the right atrium and ventricle

Dry, single wheezing above the lung surface

Other noises typical for heart disease if it causes a pathology

ECG( electrocardiography) -is effective in 55%. Right ventricular thickening and dilatation( hypertrophy and dilatation) - is detected in 87% of cases

Symptoms of right heart congestion are present in less than 60% of cases

Deviation of the electric heart axis to the right in 79%

VCG( vector electrocardiography) - cardiac cycle evaluationnot on the line( ECG), but on the plane. It is effective in 63%. Same changes as ECG
FKG( phonocardiography) - registration of noises that occur during myocardial work. Diagnostic efficiency rises to 76-81% Initial stage of pulmonary artery pressure increase

Primary changes in the structure of the right ventricle prior to attachment of a decrease in its function

Blood congestion in the small circle of the blood

Tricuspid valve function change

Diagnosis of heart muscle defects

Chest X-ray Swelling of the pulmonary artery

Extension of the roots of the lungs

Increase of the right heart muscle parts

High transparency of the craniumth lung tissue

Echocardiography or ultrasound of the heart( ultrasonography) through the chest Increased level of pressure in the pulmonary artery trunk

Degree of failure of the tricuspid valve

Paradoxical movement of the interventricular septum( with contraction deflects into the right ventricular cavity)

Presence of cardiac malformations with discharge of blood into the venoussystem

Expansion of cavities of the right heart muscle parts

Thickening of the anterior wall of the right ventricle

Right heart catheterizationInvasive procedure for insertion of the sensor into the heart through the large vein system Accurate figures of increased pressure in the pulmonary artery trunk, ventricular cavity

Change in the volume of blood flowing from the ventricle into the artery with contraction of the myocardium

Increase of vascular resistance in the small circulation circle

The oxygen saturation level of the arterial andvenous blood

Conducting medical samples for cardiac catheterization Reaction to the introduction of calcium antagonists( essential drugs fortreatment)

Additional research methods

Used to clarify the cause of pressure increase in the pulmonary arterial system and conduct refining diagnostics if there is no certainty of the exact disease. These studies are also necessary to establish a clinical class of pulmonary hypertension.

  1. FVD( function of external respiration).It is necessary to evaluate to exclude the pathology of the respiratory system. At an elevated pressure in the circulatory system of the lungs, a moderate decrease in the partial pressure of oxygen and carbon monoxide( the main blood gases) is noted.
  2. Ventilation-perfusion lung scintigraphy is the study of lungs with the introduction of radioactive particles into the blood. Conducted to exclude clogging with clots of branches of the arteries of the lungs, as the main cause of breathing problems.
  3. MSCT( multispiral computed tomography) of the heart and lungs with contrast enhancement is an x-ray method for creating a three-dimensional image of an organ using a computer program. It allows you to pinpoint the cause of the pressure increase if it is associated with the cardiovascular or respiratory systems, and also assess the degree of dysfunction of the heart muscle and the level of replacement of the connective tissue of the lung vessels.
  4. APG( angiopulmonografiya) - the introduction of contrast material in the vessels of the lungs and the fixation of the resulting picture using X-rays. It is indicated if there is a suspicion of thromboembolism, which could not be detected in other studies.

Treatment methods

Any treatment for pulmonary hypertension has only a temporary effect. It is impossible to completely relieve the patient of the disease, regardless of the cause of the increase in pressure in the vessels of the lungs. With a good response to treatment, the general condition of the ill person improves, the ability to perform physical exertion and more than twice the life expectancy. If the pathology is not treated, then the lethal outcome occurs within 2 years after the detection of the disease.

General recommendations for patients with a high level of pressure in the vessels of the lungs, allowing to significantly reduce the risk of deterioration of the general condition and course of the disease:

  • avoid physical exertion after eating and under unfavorable temperature conditions( very hot or cold);
  • perform daily dosed physical exercises that do not cause symptoms and maintain a good vascular tone;
  • use oxygen therapy for air travel;
  • to avoid and prevent inflammatory diseases of the bronchi and lungs;
  • does not use hormone replacement therapy in menopause;
  • if possible, abstain from pregnancy and childbirth;
  • does not use hormonal methods of protection from pregnancy;
  • should not be allowed to lower hemoglobin( anemia).

Medications

Drug therapy is the main way to correct pulmonary hypertension. In the treatment, several groups of drugs are used, most often in combination with each other.

1. Basic treatment - the patient receives the medication on an ongoing basis

Drug group, purpose of Key representatives
Disaggregants - prevention of clot formation in the lumen of the vessels Aspirin
Anticoagulants - decrease in blood viscosity, improve its fluidity and prevent thrombogenesis Warfarin

Heparin

Diuretics - reduced heart load Furosemide

Spironolactone

Cardiac glycosides - improve myocardial function in conditionsload and disrupted function of the right divisions Digoxin
Calcium antagonists - the basic drugs of basic therapy, dilate the vessels of the small capillary lung network Nifedipine

Diltiazem

2. Course treatment - use several times a year or as necessary

Drug Group, Purpose of Key Representatives of
Prostaglandins - dilate blood vessels, dilute blood and prevent clotting Prostaglandin E

Treprostinil

Endothelin receptor antagonists - relax the smooth muscles of the capillary network of lung tissue, inhibit the division of the vascular cells Boszentan
Inhibitorsphosphodiesterase - reduce vascular resistance in the lungs and load on the right ventricle Sildenafil

Oxygen therapy - highly effectivesu- method of increasing blood level of saturation with a deterioration state or in adverse environmental conditions.

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

Surgical methods of treatment

Applied in case of low effectiveness of conducted medical treatment and with malformations of the heart muscle.

Method When using
Atrial septostomy - creating a message between the atria With severe right ventricular failure, as a preparatory stage before heart and lung transplantation
Thrombenderterectomy - removal of blood clots from arteries Remove clots from the initial sections of pulmonary artery branches to reduceload on the right ventricle and reduce manifestations of heart failure.

Can only be performed if the thrombus does not begin to degenerate into connective tissue( the first 5-10 days)

Heart or lung-to-heart transplantation Conducted in patients with primary, idiopathic pulmonary hypertension in the last stage of heart failure

In severeheart diseases that caused the violation of blood flow in the lungs

Forecast

When the diagnosis of pulmonary hypertension is established, the prognosis for complete recovery is unfavorable. The life expectancy of patients, even with the provision of treatment, is limited. With any form of the disease, fast or slow increase in the failure of the right half of the heart muscle occurs, against which the death of the patient occurs.

  • The most unfavorable trend is pulmonary hypertension in the background of systemic scleroderma( the degeneration of the cells of the organs into the connective tissue) - patients live no more than a year.
  • The prognosis for the primary form of increasing pressure in the blood vessels of the lungs is slightly better: the average life-span is up to 3 years.
  • Development of cardiac and pulmonary insufficiency in heart defects is an indication for surgical treatment( organ transplantation).A five-year period is experienced by 40-44% of such patients.
  • The extremely unfavorable factor is a rapid increase in the failure of the function of the right ventricle and its extreme forms of manifestation. Treatment in such cases does not have an effect, the diseased die within 2 years.
  • With a good response to ongoing drug treatment( with the "soft" form of pulmonary hypertension), more than 67% of patients experience a five-year period.

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