Pulmonary hypertension (LH) is a group of diseases that is characterized by a pathological condition of the respiratory system of the body caused by an increase in pressure in the pulmonary artery. The disease has a complex etiology and pathogenesis, which makes it difficult to timely diagnosis and treatment.
The pulmonary artery is the largest and most important paired vessel of the pulmonary circulation, ensuring the transport of venous blood to the lungs. The proper functioning of the respiratory system depends on its condition.
Normally, the average pressure in the pulmonary artery should be no more than 25 mm Hg. Art. Under the influence of various factors (physical activity, age, taking certain medications), these values may vary slightly up or down. This physiological increase or decrease does not pose a threat to health. But if the average pressure exceeds 25 mm Hg. pillar alone, it is a sign of pulmonary hypertension.
The disease affects mainly women. Pulmonary hypertension is also diagnosed in children, this is due to the presence of heart defects.
The disease is rare. Its idiopathic form is recorded 5 - 6 cases per 1 million population.
Pulmonary hypertension is in ICD-10 in the group with code I27, under which are marked forms of pulmonary heart disease, not listed in the other classes.
Primary pulmonary hypertension is encrypted I27.0 and is characterized by the occurrence of the disease without a clear etiology.
The various forms of secondary pulmonary hypertension that occur as a consequence of other diseases are indicated by code I27.2.
Classification of pathology
In medical practice, the following types of pulmonary hypertension are distinguished:
- Primary Pulmonary Hypertension. It is rare and is caused by a hereditary factor associated with mutations at the gene level. Separately allocated idiopathic form of primary pulmonary hypertension, the causes of which can not be determined.
- Secondary pulmonary hypertension. This form of the disease develops on the background of a different pathology or under the influence of negative external causes. Often the disease develops due to congenital heart defects, connective tissue diseases, infectious (HIV), parasitic (schistosomiasis) and systemic pathologies (for example, in hemolytic anemia). Also, pulmonary hypertension can develop when taking drugs (antibiotics). There is evidence linking the occurrence of pathology with contact with aggressive chemical compounds, in particular, asbestos.
- Thromboembolic pulmonary hypertension. The cause of its occurrence is a thrombus, which clogs the lumen of the pulmonary artery during its movement through the bloodstream. This form of the disease has a rapid course with the development of acute respiratory failure and orthostatic collapse.
- Mixed pulmonary hypertension. The emergence of this form of the disease is associated with a history of other diseases (renal failure, cancer pathology, vasculitis, etc.). The causal relationship between them and the level of pressure in the pulmonary artery is not fully defined.
Causes and risk factors
The causes of the development of primary pulmonary hypertension are not known for certain. There is a theory that its occurrence can be correlated with genetic as well as autoimmune processes.
Cases have also been reported when pulmonary hypertension occurred in members of the same family with heart defects along the descending line, indicating a significant role of the hereditary factor.
The pathogenesis of the disease is due to the gradual narrowing of the lumen in the bed of the pulmonary artery, as well as the pulmonary vessels and the pulmonary circulation in general. This is not connected with atherosclerotic changes, which is typical for patients suffering from arterial hypertension, but with pathological thickening of the walls of veins and capillaries, which leads to a loss of their elasticity.
The consequence of these processes is an increase in blood pressure in the pulmonary artery and vascular grid of the lungs. Owing to this pathology, the phenomena of general hypoxia and insufficiency of tissue nutrition are observed.
In severe cases of pulmonary hypertension, inflammatory processes involving the inner lining of the vessels, the endothelium, are possible. This factor increases the risk of pulmonary thrombosis and other complications.
Risk factors for pulmonary hypertension:
- age from 20 to 40 years
- infectious diseases (HIV, hepatitis),
- portal hypertension
- medication (oral contraceptives, antidepressants),
- heart diseases,
- conditions associated with arterial hypertension.
There are separate studies proving a high risk of developing pulmonary hypertension with frequent consumption of rapeseed oil, but the causal relationship between this is not completely understood.
Symptoms of pathology, classes of disease
Pulmonary arterial hypertension, with the exception of its thromboembolic form, has a slow development. Clinical symptoms increase gradually and weakly manifest themselves. Perhaps their complete absence.
The leading symptom of pulmonary hypertension is shortness of breath, which is initially present only during increased physical exertion, but as the pathological process progresses, it begins to occur at rest. In the latter stages of the disease, the severity of dyspnea is so strong that the patient is not able to perform basic household activities on his own.
A characteristic difference in dyspnea in pulmonary hypertension from a similar symptom in heart failure associated with the formation of heart defects is that it does not disappear when the patient's body position changes.
In addition to shortness of breath, characteristic symptoms of the disease are:
- dry cough,
- enlarged liver and its soreness
- swelling of the lower extremities,
- chest pains
- increased fatigue and constant weakness
- hoarseness (not all patients).
Pulmonary hypertension is a disease characterized by a gradual increase in pathological symptoms that disrupt the heart. Depending on its severity, it is customary to distinguish 4 classes (stages) of the disease.
- Class I. Hypertension grade 1 - the initial form of the disease. The patient does not feel pathological changes. Physical activity is transferred easily, physical activity does not suffer.
- II class. At stage 2, physical exertion is transferred as usual, however, with their high intensity, the patient notes the appearance of severe shortness of breath and increased fatigue. The severity of morphological changes in blood vessels and internal organs is negligible.
- Class III. At 3 stages, the usual physical exertion is poorly tolerated. Moderate activity is accompanied by most of the symptoms listed above. On ECG, right ventricular hypertrophy is noted.
- Class IV. The manifestation of the characteristic symptoms observed in the absence of stress. It is noted the impossibility of performing everyday tasks. The patient needs constant care. At stage 4, join the symptoms of heart failure.
Complications of pulmonary hypertension
Pulmonary hypertension associated with an increase in pressure in the pulmonary artery is accompanied by serious complications of the cardiovascular system. In the process of development of pathological processes associated with pulmonary arterial hypertension, hypertrophy of the right ventricle of the heart muscle occurs. This is due to the increased load on this chamber, as a result of which its contractile capacity deteriorates, leading to the development of heart failure.
Hypertension grade 1 is characterized by the fact that patients may experience irregularities in the work of the heart, expressed in cardiac tachycardia and atrial fibrillation.
As the vascular walls change, blood clots form in the veins - blood clots. They narrow the lumen of blood vessels, which leads to increased severity of symptoms. A detached thrombus can block the blood flow, which leads to pulmonary embolism, which in the absence of timely medical care ends in the death of the patient.
With a history of arterial hypertension, pulmonary hypertension increases the frequency and severity of crises that are fraught with the development of a heart attack or stroke.
A serious complication of the disease is death, which occurs either as a result of respiratory failure, or as a result of abnormalities in the heart.
Diagnosis of pulmonary hypertension begins with a history taking. It clarifies the nature and duration of symptoms, the presence of chronic diseases, the lifestyle of patients and associated risk factors.
At direct inspection the following characteristic features are revealed:
- blueness of the skin,
- enlarged liver
- swelling of the neck veins,
- changes in the heart and lungs when listening to them with a phonendoscope.
The leading role in the diagnosis of pulmonary hypertension belongs to instrumental methods.
Catheterization of the pulmonary artery. Through this method, pulmonary artery pressure is measured. The essence of the study lies in the fact that after the puncture of a vein, a catheter is inserted into its lumen, to which a device for measuring blood pressure is connected. The method is informative in identifying any form of hemodynamic disorders. And is included in the standard of research for suspected pulmonary hypertension. Catheterization is not shown to all patients, in the presence of heart failure or tachyarrhythmia, the possibility of its implementation is assessed after consulting a cardiologist.
ECG. With the help of electrocardiography revealed pathology associated with impaired electrical activity of the heart. Also, this method allows to evaluate the morphological structure of the myocardium. Indirect signs of pulmonary hypertension on ECG are dilatations of the right ventricle of the heart and EOS shift to the right.
Ultrasound of the heart. Ultrasound allows you to assess the structure of the heart muscle, the size and structure of its chambers, the state of the valves and coronary vessels. Changes associated with thickening of the walls of the atria and right ventricle indirectly indicate pulmonary hypertension.
Spirometry Such testing allows to evaluate the following functional features of the bronchopulmonary system:
- lung capacity,
- total lung capacity
- expiratory volume
- exhalation rate
- breathing rate.
Deviations of the above indicators from the norm indicate pathologies of respiratory function.
Angiopulmonography. The technique consists in the introduction of a contrast agent into the vessels of the lungs in order to conduct an X-ray study to identify pathologies of the morphological structure of the pulmonary artery. Angiopulmonography is performed when other diagnostic methods cannot determine the presence of changes with certainty. This is associated with a high risk of complications during the procedure.
CT scan (CT). With the help of CT, it is possible to achieve visualization of the image of the chest organs in various projections. This allows you to evaluate the morphological structure of the heart, lungs and blood vessels.
The above diagnostic methods are the most informative, however, the presence of primary pulmonary hypertension can be determined only on the basis of a set of the obtained research results.
The grade of the disease is determined using a six-minute test. As a result, the body's tolerance to physical stress is estimated.
The presence of pulmonary hypertension involves the correction of the lifestyle of patients. These measures include:
- performing daily exercise therapy exercises to improve the tone of blood vessels, but not requiring increased respiratory activity,
- avoid excessive physical exertion
- prevention of infectious and inflammatory diseases of the respiratory system (bronchitis, pneumonia), and if they occur, timely treatment,
- prevention of anemia, which consists in taking special medications (Sorbifer),
- refusal of hormonal methods of contraception,
- smoking cessation and excessive use of alcoholic beverages.
- Vasodilator drugs (for example: Nifedipine, Cardilopin). Widely used in the treatment of PH. They reduce the tone of the vascular walls, contributing to their relaxation and reduce the overall pressure in the veins and arteries. As a result, the diameter of the vessels increases and blood flow improves.
- Prostaglandins (for example: Treprostinil). Drugs that promote the removal of vascular spasm. Receiving prostaglandins is the prevention of blood clots. Effective with all types of PH.
- Cardiac glycosides (Digoxin, Strofantin, Korglikon). When they are taken, vasospasm decreases, blood flow to the heart muscle increases, tolerance of the vascular system to external adverse factors increases. When pulmonary hypertension is used in the prevention and treatment of heart disease.
- Anticoagulants (Aspirin, Eliquis). They prevent the development of thrombosis, as well as dissolve existing blood clots.
- Diuretics (Furosemide). Diuretic drugs provide an outflow of excess fluid from the body, which reduces the volume of circulating blood. This minimizes the load on the cardiovascular system and helps reduce pressure in the large and small circulation.
- Endothelin receptor antagonists (Bozentan, Traklir). The pathological process in the vessels in patients with pulmonary arterial hypertension is caused by the growth of the inner lining of the vessels - the endothelium. Taking drugs in this group significantly slows down the development of the disease.
- Oxygen therapy. Used primarily in patients with respiratory failure and the presence of heart defects. As a result of the procedure, the blood is saturated with oxygen, which ensures normal nutrition of body tissues.
- Nitrogen oxide. Inhalation of nitric oxide has a positive effect on the vessels of the lungs. The blood flow improves, the resistance of their walls decreases. Dyspnea and pain are eliminated, and tolerance to physical exertion increases.
Rules of nutrition in pulmonary hypertension suggest a restriction in the diet of salt and animal fats. Permitted to use:
- milk products,
- low-fat meat food (beef, poultry),
- lean fish,
- nuts, dried fruits.
Avoid frequent use of coffee and strong tea.
It is important to observe the drinking regime in order to avoid edema. The volume of fluid consumed should be about 2 liters of water per day.
- Heart or lung-heart transplant. This high-tech surgery is used when standard therapy methods are exhausted. Heart and lung transplantation is a significant problem due to a shortage of donors, as well as possible problems associated with their rejection by the recipient's body. Post-transplant patients with this live an average of about ten or fifteen years.
- Thrombendarterectomy. This surgical intervention involves the mechanical removal of a blood clot in the mainstream of the pulmonary artery. Treats a minimally invasive type of surgery.
Methods of traditional medicine
Important! Methods of traditional medicine cannot be considered as a self-sufficient method of treatment, their use is possible only in combination with the main therapy in consultation with the doctor.
- Infusion of the fruits of mountain ash. A teaspoon of rowan berries should be brewed with one glass of boiling water and left to infuse for one hour. The resulting tool is filtered and consumed in half a glass 3 times a day. The duration of admission is one month. The tool helps to eliminate the severity of symptoms of the disease (elimination of shortness of breath, chest pain).
- Tincture of calendula inflorescences. 50 grams of dried inflorescences must be poured with 150 grams of alcohol and infused for a week in a dry, cold place without access to sunlight. Take the drug should be 25 drops three times a day for one month.
Methods for specific prophylaxis are absent.
General preventive measures are aimed at eliminating risk factors and improving the health of the body:
- timely treatment of infectious diseases,
- elimination of effects on the body of heavy physical exertion,
- moderate physical activity
- elimination of bad habits (alcohol, smoking),
- medication for the prevention of blood clots,
- treatment of heart defects.
If pulmonary hypertension is already diagnosed, then prevention is aimed at reducing the likelihood of possible complications. In case of primary pulmonary hypertension, it is necessary to take all the medicines prescribed by the doctor and to fully implement its recommendations. Moderate physical activity contributes to maintaining an active lifestyle.
Prediction for life
How long can you live with this disease? The prognosis depends on the neglect of pathological processes in the vessels. When diagnosing LH in advanced stages, five-year patient survival is low.
Also, the prognosis of the outcome of the disease is affected by the body's response to the treatment being performed. If the pressure in the pulmonary artery begins to decrease or remains at the same level, then with maintenance therapy the patient can live up to 10 years. With its increase to 50 mm Hg. article and above, the disease enters the stage of decompensation, when the normal functioning of the body is impossible even under conditions of adequate medical therapy.
The five-year survival rate at this stage of the disease is no more than 10%.
Pulmonary arterial hypertension is a dangerous disease with a very serious prognosis for life. Despite the fact that it is quite rare, being, in most cases, a consequence of other diseases, a complete cure is impossible. Existing methods of treatment are aimed at eliminating adverse symptoms and slowing the development of pathological processes, they can not be completely eliminated.
The development of pulmonary hypertension is triggered by the gradual narrowing of the lumen of the pulmonary vascular bed - capillaries and arterioles. Such changes are caused by a thickening of the inner layer of blood vessels - the endothelium. In a complicated course, such a process may be accompanied by inflammation and destruction of the muscular layer of the pulmonary artery.
The narrowing of the lumen of blood vessels causes a constant clotting of blood clots in it and leads to obliteration. Subsequently, the patient increases the pressure in the pulmonary artery system and develops pulmonary hypertension. This process increases the load on the right ventricle of the heart. Initially, its walls are hypertrophied, trying to compensate for the violation of hemodynamics. Subsequently, due to constant loads, their contractile ability decreases and the stage of decompensation develops, which is expressed in right ventricular failure.
Classification of pulmonary hypertension by class
Depending on the severity of symptoms and the severity of the patient's condition, four classes of pulmonary hypertension are distinguished:
- I - the physical activity of the patient does not suffer, after the usual loads he does not appear dizziness, weakness, shortness of breath and chest pain,
- II - the physical activity of the patient is slightly disturbed, at rest the state of health does not change at all, but after the usual loads, he develops dizziness, weakness, shortness of breath and chest pain,
- III - the physical activity of the patient is significantly impaired, even minor physical exertion causes dizziness, weakness, shortness of breath and chest pain,
- IV - even the minimum load causes dizziness, weakness, shortness of breath and chest pain, often the same symptoms occur at rest.
The main treachery of pulmonary hypertension is that during the compensation stage, it can be completely asymptomatic and can be detected in advanced forms. Its first signs begin to appear only when the pulmonary arterial pressure exceeds the normal values by 2 times or more.
Usually the first symptoms of pulmonary hypertension are:
- the appearance of unexplained dyspnea even with slight exertion or with complete rest,
- unexplained weight loss with the usual diet,
- hoarse voice
- persistent dry cough
- episodes of dizziness or fainting,
- noticeable pulsation of the jugular vein around the neck,
- discomfort and heaviness in the liver.
Later, the patient appears angina attacks and atrial fibrillation. In addition, blood streaks can be detected in the sputum. With increasing pulmonary edema, hemoptysis develops in a patient.
The patient complains of pain in the right hypochondrium, which is provoked by the development of venous stagnation. When probing the abdomen, the doctor determines the expansion of the boundaries of the liver and pain. Hemodynamic disturbances in the large circle of blood circulation lead to the appearance of edema in the legs (in the feet and lower legs) and fluid accumulation in the abdominal cavity (ascites).
At the terminal stage of development of pulmonary hypertension, hypertensive crises occur in the vascular bed, triggering the development of pulmonary edema. They begin with a shortness of breath, then the patient begins to have a strong cough, with bloody sputum, fear and anxiety. The skin becomes bluish, the patient develops psychomotor agitation, feces can uncontrollably be separated and light urine is liberated. In severe cases, pulmonary edema ends with the development of acute heart failure and pulmonary embolism, which are fatal.
To reduce the existing risks in pulmonary hypertension, patients are recommended:
- vaccination against influenza and pneumococcal infections, aggravating the course of the pathology,
- regular and metered exercise,
- diet for heart failure,
- prevention of pregnancy.
Relatives and relatives of patients with pulmonary hypertension should provide them with constant psychological support. Such patients often develop depressions, they feel unnecessary, often annoyed over trifles and do not want to be a burden to their surroundings. Such a depressed state adversely affects the course of pulmonary hypertension, and for its elimination, constant psychological work on the existing problem is necessary. If necessary, patients may be recommended to consult a psychotherapist.
As an additional agent for the treatment of pulmonary hypertension, the doctor may recommend the use of medicinal herbs:
- an infusion of red rowan fruit,
- fresh pumpkin juice,
- infusion of spring adonis, etc.
Patients with pulmonary hypertension should be aware that the treatment of this pathology is impossible only with the help of folk remedies. Medicinal herbs in such cases can be used only in the form of soft diuretics and fees to calm cough.
To facilitate the work of the heart, the patient may be recommended to have a balloon atrial septostomy. The essence of this intervention is the introduction of a catheter with a balloon and a blade into the left atrium, which is used to cut the septum between the atria. With the help of a balloon, the doctor expands the incision and thus seeks to facilitate the discharge of oxygenated blood from the left half of the heart to the right.
In extremely severe cases, lung and / or heart transplants may be performed. Such interventions are performed in specialized surgical centers and are often inaccessible due to the lack and high cost of donor organs. In some cases, transplantation may be absolutely contraindicated: malignant neoplasms, HIV infection, smoking, the presence of HBs antigen, hepatitis C and the use of drugs or alcohol in the last 6 months.
With secondary pulmonary hypertension, the prognosis is favorable, if the rate of development of the pathological condition remains small, the prescribed therapy is effective and leads to a decrease in pressure in the pulmonary bed. With the rapid progression of symptoms, the increase in pressure of more than 50 mm Hg. Art. and poor treatment efficacy, the prognosis worsens. In such cases, over 5 years, most of the patients die.
Idiopathic pulmonary hypertension always has extremely unfavorable prognosis. During the first year of the disease, only 68% of patients remain alive, after 3–38%, and after 5 years only 35%.
Pulmonary hypertension refers to severe and dangerous pathological conditions, which over time can become the cause of death of the patient from pulmonary embolism or ever-increasing right ventricular heart failure. Her treatment should begin as early as possible, and the patient needs constant monitoring by a pulmonologist and a cardiologist.
The first channel, the program “Live healthy!” With Elena Malysheva, in the section “About medicine” talk about pulmonary hypertension (see 34:20 min.):
Scientific report Tsareva N.A. on the topic "New in the treatment of pulmonary hypertension":
D.M.N. Konopleva L.F. talks about the treatment of pulmonary hypertension:
Pulmonary hypertension - threatening pathological condition due to persistent increase in blood pressure in the vascular bed of the pulmonary artery. The increase in pulmonary hypertension is gradual, progressive, and ultimately causes the development of right ventricular heart failure, leading to the death of the patient. The criteria for the diagnosis of pulmonary hypertension are indicators of average pressure in the pulmonary artery over 25 mm Hg. Art. at rest (at a rate of 9–16 mm Hg) and over 50 mm Hg. Art. under load. The most common pulmonary hypertension occurs in young women 30-40 years old, who suffer from this disease 4 times more often than men. There are primary pulmonary hypertension (as an independent disease) and secondary (as a complicated variant of the course of diseases of the respiratory organs and blood circulation).
Significant causes of pulmonary hypertension are not identified. Primary pulmonary hypertension is a rare disease with an unknown etiology. It is assumed that factors such as autoimmune diseases (systemic lupus erythematosus, scleroderma, rheumatoid arthritis), family history, and oral contraceptives are related to its occurrence.
In the development of secondary pulmonary hypertension, many diseases and defects of the heart, blood vessels and lungs may play a role. Most often, secondary pulmonary hypertension is a result of congestive heart failure, mitral stenosis, atrial septal defect, chronic obstructive pulmonary disease, pulmonary vein thrombosis and pulmonary artery branches, pulmonary hypoventilation, coronary artery disease, myocarditis, liver cirrhosis, etc. is higher in HIV-infected patients, drug addicts, people taking appetite suppressants. Differently, each of these conditions can cause an increase in blood pressure in the pulmonary artery.
The development of pulmonary hypertension is preceded by a gradual narrowing of the lumen of small and medium-sized vascular branches of the pulmonary artery system (capillaries, arterioles) due to thickening of the inner choroid - endothelium. In case of severe damage to the pulmonary artery, inflammatory destruction of the muscular layer of the vascular wall is possible. Damage to the walls of blood vessels leads to the development of chronic thrombosis and vascular obliteration.
These changes in the pulmonary vascular bed cause a progressive increase in intravascular pressure, i.e., pulmonary hypertension. Constantly increased blood pressure in the bed of the pulmonary artery increases the load on the right ventricle, causing hypertrophy of its walls. The progression of pulmonary hypertension leads to a decrease in the contractile ability of the right ventricle and its decompensation — right ventricular heart failure (pulmonary heart) develops.
Symptoms of pulmonary hypertension
In the stage of compensation, pulmonary hypertension may be asymptomatic, therefore the disease is often diagnosed in severe forms. The initial manifestations of pulmonary hypertension are noted with an increase in pressure in the pulmonary artery system by 2 or more times compared with the physiological norm.
With the development of pulmonary hypertension, unexplained shortness of breath, weight loss, fatigue during physical activity, palpitations, cough, hoarseness of voice appear. Relatively early in the clinic of pulmonary hypertension, dizziness and fainting may occur due to a heart rhythm disorder or the development of acute brain hypoxia. Later manifestations of pulmonary hypertension are hemoptysis, chest pain, swelling of the legs and feet, pain in the liver.
The low specificity of the symptoms of pulmonary hypertension does not allow a diagnosis based on subjective complaints. The most frequent complication of pulmonary hypertension is right ventricular heart failure, accompanied by a rhythm disorder - atrial fibrillation. In severe stages of pulmonary hypertension, pulmonary arteriole thrombosis develops.
Treatment of pulmonary hypertension
The main goals in the treatment of pulmonary hypertension are to eliminate its causes, lower blood pressure in the pulmonary artery, and prevent thrombus formation in the pulmonary vessels. The complex of treatment of patients with pulmonary hypertension includes:
- Reception of vasodilating agents relaxing the smooth muscle layer of blood vessels (prazosin, hydralazine, nifedipine). Vasodilators are effective in the early stages of the development of pulmonary hypertension before the appearance of marked changes in arterioles, their occlusions and obliterations. In this regard, the importance of early diagnosis of the disease and the establishment of the etiology of pulmonary hypertension.
- Acceptance of antiplatelet agents and indirect anticoagulants that reduce blood viscosity (acetyl-salicylic acid, dipyridamole, etc.). When expressed thickening of the blood resort to bleeding. Hemoglobin level up to 170 g / l is considered optimal for patients with pulmonary hypertension.
- Oxygen inhalation as a symptomatic therapy for severe shortness of breath and hypoxia.
- Acceptance of diuretic drugs for pulmonary hypertension, complicated by right ventricular failure.
- Heart and lung transplantation in extremely severe cases of pulmonary hypertension. The experience of such operations is still small, but indicates the effectiveness of this technique.
Prognosis and prevention
A further prognosis for already developed pulmonary hypertension depends on its root cause and the level of blood pressure in the pulmonary artery. With a good response to the therapy, the prognosis is more favorable. The higher and more stable the pressure level in the pulmonary artery system, the worse the prognosis. When expressed phenomena of decompensation and pressure in the pulmonary artery more than 50 mm Hg. a significant proportion of patients die within the next 5 years. Prognostically extremely unfavorable primary pulmonary hypertension.
Preventive measures are aimed at early detection and active treatment of pathologies leading to pulmonary hypertension.
Why does LH develop?
The reasons for the development of PH are still not completely determined. For example, in the 1960s, an increase in the number of cases was observed in Europe, associated with the excessive use of contraceptives and weight-loss drugs. Spain, 1981: complications in the form of muscle lesions that began after the popularization of rapeseed oil. Nearly 2.5% of 20,000 patients were diagnosed with arterial pulmonary hypertension. The root of evil was tryptophan (amino acid), which was in oil, which was scientifically proved much later.
Impaired function (dysfunction) of the vascular endothelium of the lungs: the cause may be a genetic predisposition, or the influence of external damaging factors. In any case, the normal balance of the exchange of nitric oxide changes, the vascular tone changes in the direction of the spasm, then inflammation, the endothelium begins to grow and the arteries lumen decreases.
Increased endothelin content (vasoconstrictor): caused either by an increase in its production in the endothelium, or a decrease in the breakdown of this substance in the lungs. It is noted in the idiopathic form of LH, congenital heart defects in children, systemic diseases.
Impaired synthesis or availability of nitric oxide (NO), reduced levels of prostacyclin, the additional excretion of potassium ions - all deviations from the norm lead to arterial spasm, the growth of the vascular muscle wall and endothelium. In any case, the end of development becomes a violation of blood flow in the pulmonary artery system.
Signs of disease
Mild pulmonary hypertension does not give any pronounced symptoms, this is the main danger. Signs of severe pulmonary hypertension are determined only in the late periods of its development, when the pulmonary arterial pressure rises, compared with the norm, two or more times. Pressure in the pulmonary artery: systolic 30 mm Hg, diastolic 15 mm Hg.
Initial symptoms of pulmonary hypertension:
- Unexplained dyspnea, even with little physical activity or in complete rest,
- Gradual weight loss, even with normal, good nutrition,
- Asthenia, constant feeling of weakness and powerlessness, depressed mood - regardless of the season, weather and time of day,
- Persistent dry cough, hoarse voice,
- Abdominal discomfort, feeling of heaviness and "bursting": the beginning of blood stagnation in the portal vein system, which conducts venous blood from the intestine to the liver,
- Dizziness, fainting - manifestations of oxygen starvation (hypoxia) of the brain,
- Heart palpitations, over time, the pulsation of the artery vein becomes noticeable on the neck.
Later manifestations of PH:
- Sputum with streaks of blood and hemoptysis: signal of increasing pulmonary edema,
- Angina attacks (chest pain, cold sweat, fear of death) is a sign of myocardial ischemia,
- Arrhythmias (cardiac arrhythmias) by type of atrial fibrillation.
Pain in the hypochondrium on the right: a large circle of blood circulation is already involved in the development of venous stagnation, the liver has increased and its shell (capsule) has stretched - so pain has appeared (the liver itself has no pain receptors, they are located only in the capsule)
Swelling of the legs, in the legs and feet. The accumulation of fluid in the abdomen (ascites): manifestation of heart failure, peripheral blood stasis, decompensation phase - a direct danger to the patient's life.
Terminal stage LH:
- Blood clots in the arterioles of the lungs lead to the death (infarction) of the active tissue, the increase in asphyxiation.
Hypertensive crises and attacks of acute pulmonary edema: more often occur at night or in the morning. They begin with a feeling of severe lack of air, then a strong cough joins, bloody sputum is released. The skin becomes bluish (cyanosis), the veins in the neck pulsate. The patient is excited and scared, loses self-control, can move erratically. In the best case, the crisis will end with a copious discharge of light urine and uncontrolled discharge of feces, at worst - a fatal outcome. The cause of death may be the overlap of the thrombus (thromboembolism) of the pulmonary artery and the subsequent acute heart failure.
The main forms of LH
- Primary, idiopathic pulmonary hypertension (from the Greek idios and pathos - "a kind of disease"): it is fixed by a separate diagnosis, in contrast to secondary LH associated with other diseases. Variants of primary LH: familial LH and hereditary predisposition of vessels for expansion and bleeding (hemorrhagic telangiectasia). The reason - genetic mutations, the frequency of 6 - 10% of all cases of PH.
- Secondary LH: manifested as a complication of the underlying disease.
Systemic connective tissue diseases - scleroderma, rheumatoid arthritis, systemic lupus erythematosus.
Congenital heart defects (with bleeding from left to right) in newborns, occurring in 1% of cases. After corrective blood flow surgery, the survival rate of this category of patients is higher than in children with other forms of PH.
Late stages of dysfunction of the liver, pulmonary and pulmonary vascular pathologies in 20% give a complication in the form of PH.
HIV infection: PH is diagnosed in 0.5% of cases, the survival rate for three years drops to 21% compared with the first year - 58%.
Intoxication: amphetamines, cocaine. The risk increases by three dozen times if these substances have been used for more than three months in a row.
Blood diseases: in some types of anemia in 20 - 40% of patients with LH are diagnosed, which increases mortality.
Chronic obstructive pulmonary disease (COPD) is caused by prolonged inhalation of particles of coal, asbestos, shale, and toxic gases. Often found as a professional disease among miners, workers in hazardous industries.
Sleep apnea syndrome: partial cessation of breathing during sleep. Dangerous, found in 15% of adults. The consequence can be LH, stroke, arrhythmias, arterial hypertension.
Chronic thrombosis: noted in 60% after interviewing patients with pulmonary hypertension.
Lesions of the heart, its left half: acquired defects, coronary disease, hypertension. About 30% is associated with pulmonary hypertension.
Diagnosing pulmonary hypertension
Diagnosis of precapillary LH (associated with COPD, pulmonary arterial hypertension, chronic thrombosis:
- Pulmonary artery pressure: ≥ 25 mm Hg at rest, more than 30 mm at voltage,
- Increased pressure of pulmonary artery wedging, blood pressure inside the left atrium, end diastolic ≥15 mm, resistance of pulmonary vessels ≥ 3 units. Wood.
Postcapillary LH (for diseases of the left half of the heart):
- Pulmonary artery pressure: mean ≥25 (mmHg)
- Initial:> 15 mm
- Difference ≥12 mm (passive PH) or> 12 mm (reactive).
ECG: right overload: enlarged ventricle, dilation and thickening of the atrium. Extrasystole (extraordinary contractions of the heart), fibrillation (chaotic contraction of muscle fibers) of both atria.
X-ray examination: increased peripheral transparency of the lung fields, lung roots are enlarged, the borders of the heart are shifted to the right, the shadow from the arc of the enlarged pulmonary artery is visible to the left along the heart contour.
photo: pulmonary hypertension on X-ray
Functional respiratory tests, qualitative and quantitative analysis of the composition of gases in the blood: The level of respiratory failure and disease severity is detected.
Echocardiography: the method is very informative - it allows you to calculate the average pressure in the pulmonary artery (SDLA), diagnose almost all defects and heart. LH is recognized already in the initial stages, with an SLA ≥ 36-50 mm.
Scintigraphy: with LH with the lumen of the pulmonary artery overlapping with a thrombus (thromboembolism). The sensitivity of the method is 90 - 100%, specific for thromboembolism by 94 - 100%.
Computed (CT) and magnetic resonance imaging (MRI): at high resolution, in combination with the use of a contrast agent (with CT), allow to assess the state of the lungs, large and small arteries, walls and cavities of the heart.
The introduction of a catheter in the cavity of the "right" heart, test the reaction of blood vessels: determining the degree of PH, blood flow problems, assessment of the effectiveness and relevance of treatment.
Treatment of pulmonary hypertension is possible only in the complex., combining general recommendations for reducing the risks of exacerbations, adequate therapy of the underlying disease, symptomatic means of influencing the general manifestations of PH, surgical methods, treatment with folk remedies and unconventional methods - only as auxiliary.
- Diuretic drugs remove the accumulated fluid, reducing the load on the heart and reducing swelling. The electrolyte composition of blood (potassium, calcium), blood pressure and kidney function are surely controlled. Overdose can cause too much water loss and pressure drop. With a decrease in the level of potassium, arrhythmias begin, muscle cramps indicate a reduction in calcium levels.
- Thrombolytics and anticoagulants dissolve the already formed blood clots and prevent the formation of new ones, ensuring the patency of the vessels. Constant monitoring of the blood coagulation system (platelets) is required.
- Oxygen (oxygen therapy), 12-15 liters per day, through a humidifier: for patients with chronic obstructive pulmonary disease (COPD) and cardiac ischemia, helps to restore blood oxygenation and stabilize the general condition. It must be remembered that too much oxygen concentration inhibits the vasomotor center (MTC): breathing slows down, blood vessels dilate, pressure drops, a person loses consciousness. For normal operation, the body needs carbon dioxide, it is after the increase in its content in the blood that the CTC "gives the command" to take a breath.
- Cardiac glycosides: active ingredients are isolated from digitalis, of which Digoxin is the best known. It improves heart function by increasing blood flow, combats arrhythmias and vascular spasm, reduces swelling and shortness of breath. In case of overdose - increased excitability of the heart muscle, arrhythmias.
- Vasodilators: the muscular wall of arteries and arterioles relaxes, their lumen increases and blood flow improves, pressure in the pulmonary artery system decreases.
- Prostaglandins (PG): a group of active substances produced in the human body. In the treatment of LH, prostacyclins are used, they relieve vascular and bronchial spasm, prevent the formation of blood clots, block the growth of the endothelium. Very promising drugs, effective for PH in the face of HIV, systemic diseases (rheumatism, scleroderma, etc.), heart defects, as well as familial and idiopathic forms of PH.
- Endothelin receptor antagonists: dilation of blood vessels, suppression of proliferation (proliferation) of the endothelium. With prolonged use, shortness of breath decreases, the person becomes more active, the pressure returns to normal. Undesirable reactions to treatment - edema, anemia, liver failure, therefore, the use of drugs is limited.
- Nitric oxide and PDE type 5 inhibitors (phosphodiesterase): they are mainly used for idiopathic LH, if standard therapy does not justify itself, but individual drugs are effective for any form of LH (Sildenafil). Action: reduction of vascular resistance and associated hypertension, relief of blood flow, resulting in increased resistance to physical exertion. Nitric oxide is inhaled daily for 5–6 hours, up to 40 ppm, a course of 2–3 weeks.
Surgical treatment of PH
Balloon atrial septostomy: performed to facilitate the discharge of oxygen-rich blood inside the heart, from left to right, due to the difference in systolic pressure. A catheter with a balloon and a blade is inserted into the left atrium. The blade cuts the septum between the atria, and the swollen balloon expands the opening.
Lung transplantation (or lung-heart complex): It is carried out according to vital indications, only in specialized medical centers. The operation was first performed in 1963, but by 2009, more than 3,000 successful lung transplants were performed annually. The main problem is the lack of donor organs. The lungs take only 15%, the heart - from 33%, and the liver and kidneys - from 88% of donors. Absolute contraindications for transplantation: chronic renal and hepatic failure, HIV - infection, malignant tumors, hepatitis C, the presence of HBs antigen, as well as smoking, use of drugs and alcohol for six months before the operation.
Treatment of folk remedies
Use only in a complex, as auxiliary means for the general improvement of state of health. No self treatment!
- Fruits of red rowan: tablespoon in a glass of boiled water, for ½ cup three times a day. The amygdalin contained in the berries reduces the sensitivity of the cells to hypoxia (lower oxygen concentration), reduces edema due to the diuretic effect, and the vitamin - mineral set has a beneficial effect on the entire body.
- Adonis (spring), grass: teaspoon per cup of boiling water, 2 hours to insist, up to 2 tablespoons on an empty stomach, 2-3 times a day. Used as a diuretic, pain reliever.
- Fresh pumpkin juice: half a glass a day. It contains a lot of potassium, useful in some types of arrhythmia.
Classification and forecast
The classification is based on the principle of functional impairment in PH, the variant is modified and associated with manifestations of heart failure (WHO, 1998):
- Class I: LH with normal physical. activity. Standard loads are well tolerated, easy for LH, 1 degree failure.
- Class II: LH plus activity decreased. The comfort is in a calm position, but dizziness, shortness of breath and chest pains begin with normal stress. Moderate pulmonary hypertension, increasing symptoms.
- Class III: LH with reduced initiative. Problems even at low loads. The high degree of blood flow disorders, the deterioration of the forecast.
- Class IV: LH with intolerance to minimal activity. Dyspnea, fatigue is felt and in complete rest. Signs of high circulatory failure - congestive manifestations in the form of ascites, hypertensive crises, pulmonary edema.
The forecast will be more favorable if:
- The rate of development of symptoms of LH is small,
- Treatment improves the patient’s condition
- The pressure in the pulmonary artery system is decreasing.
- LH symptoms develop dynamically,
- Signs of decompensation of the circulatory system (pulmonary edema, ascites) are increasing,
- Pressure level: in the pulmonary artery more than 50 mm Hg,
- With primary idiopathic PH.
The overall prognosis for pulmonary arterial hypertension is associated with the form of LH and the phase of the prevailing disease. Mortality per year, with the current methods of treatment, is 15%. Idiopathic PH: patient survival after a year is 68%, after 3 years - 48%, after 5 years - only 35%.