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:
- Narrowing or spasm of blood vessels( vasoconstriction).
- Decreased capacity of the vascular wall to stretch( elasticity).
- Formation of small blood clots.
- Spread of smooth muscle cells.
- Closure of the lumen of blood vessels due to blood clots and thickened walls( obliteration).
- 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.
Click on the picture to enlarge 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:
| |
Associated with a large lesion of capillaries and veins:
| |
| 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:
| |
| 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:
|
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.
- 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.
- 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.
- 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.
- 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.
