Pulmonary Hypertension - Symptoms And Current Treatments

2024 Author: Josephine Shorter | [email protected]. Last modified: 2024-01-17 02:10

Symptoms and treatment of pulmonary hypertension

Pulmonary hypertension is expressed as an increase in pressure within the vessels that supply blood to the respiratory system. This entails the development of heart failure, namely, failure of the right ventricle of the heart. As a result, the person dies.

Normally, the pressure in the pulmonary artery is equal to 17-23 mm. rt. Art. With pulmonary hypertension, it at rest will exceed the 25 mm mark. rt. Art. Under load, this figure rises to 30 mm. rt. Art. and more.

Disorders that occur in the body with the development of pulmonary hypertension:

• Vasoconstriction, which is characterized by vasoconstriction and spasm.
• Loss of elasticity of the vascular wall.
• Formation of small blood clots inside the vessels of the lungs.
• The proliferation of smooth muscle cells.
• Obliteration of blood vessels.
• The proliferation of connective tissue inside the vessels against the background of their destruction.

All this leads to the fact that the blood is no longer able to circulate normally in the vessels of the lungs. During its passage through the arteries, there is an increase in pressure in them. This entails an increase in pressure in the right ventricle, which provokes a violation of its functioning.

A person begins to show signs of respiratory failure, and then symptoms of heart failure join. Even at the initial stages of the development of the disease, the patient's quality of life deteriorates significantly, which is caused by the impossibility of normal breathing. As the pathology progresses, a person has to more and more restrict himself to physical activity.

Pulmonary hypertension is a serious illness that kills people. If the patient does not receive adequate therapy, then he will not live longer than 2 years. At the same time, he will need outside help for life support. Pulmonary hypertension can and should be treated, but full recovery cannot be achieved.

The detection and treatment of pulmonary hypertension is the task of the therapist, pulmonologist, cardiologist, geneticist and infectious disease specialist, that is, doctors of several specialties. If necessary, vascular and thoracic surgeons are involved in therapy.

Content:

• Pulmonary hypertension symptoms
• Pulmonary Hypertension Causes
• Pulmonary hypertension
• Diagnostics
• Disease prognosis
• Pulmonary hypertension treatment
• Modern drugs

Pulmonary hypertension symptoms

The disease begins to develop unnoticed by humans. Pathological processes at the initial stage are hidden, since the body includes compensatory mechanisms. Therefore, the patient feels well.

When the pressure in the pulmonary arteries exceeds 25 mm. rt. Art., a person begins to notice deterioration in health. However, they appear only during physical activity. As the pathology progresses, the patient will experience more and more difficulties while performing even the most familiar things.

The main symptoms of pulmonary hypertension are as follows:

• Shortness of breath, which occurs as the first manifestation of the disease. At first, it appears only during physical effort, it bothers a person while inhaling. In the future, shortness of breath will be present on an ongoing basis, even when the patient is at rest. He does not suffer from attacks of suffocation.
• Pain in the sternum. Their nature varies, pains can be aching, pressing, stabbing. A person cannot accurately inform the doctor about the time of manifestation of pain, however, notes that they become more intense during physical effort. Nitroglycerin does not eliminate pain.
• Fainting and dizziness are observed while performing any activity. Dizziness can last from 2 to 25 minutes. The same applies to episodes of loss of consciousness.
• Heart irregularities, palpitations. During the ECG, the patient is diagnosed with sinus tachycardia.
• One third of patients have a cough. It is most often dry, while there are no signs of infectious diseases in humans.
• Blood may be present in the separated sputum, but this symptom occurs in no more than 10% of patients. More often, blood in sputum is observed as a single occurrence. The reason for its appearance, experts call the presence of blood clots in the pulmonary arteries.

Pulmonary Hypertension Causes

As an independent pathology, pulmonary hypertension is diagnosed in only 6 people out of a million. In this case, its cause either cannot be established, or it is inherited. In other case histories, pulmonary hypertension is the result of some organ dysfunction.

The following pathologies can lead to pulmonary hypertension:

Classification group of pathology	Disease as its cause
Primary arterial pulmonary hypertension	The reason is not clear. Weighed down heredity. Systemic lupus erythematosus, scleroderma. Congenital heart disease. AIDS virus. Poisoning with drugs or other toxic substances. Gosher's disease. Thyroid pathology. Randu-Osler disease. Glycogenosis. The absence of a spleen in the body as a result of organ removal. Hemoglobinopathy. Pathology of the red bone marrow. The presence of blood clots in the vessels. Pulmonary capillary hemangiomatosis.
Pulmonary hypertension due to pathological changes in the left heart.	Dysfunction of the valves of the left ventricle, or the leftmost ventricle of the heart.
Pulmonary hypertension due to disturbances in the respiratory system.	Pathologies in the development of the lungs, which can be inherited, or are the result of congenital anomalies in their structure. Altitude hypoxia associated with oxygen starvation of the body when a person is at high altitudes in the mountains. Apnea. Disturbances in the work of the alveoli. COPD. Lesions of the interstitium of the lungs as a result of the proliferation of connective tissue or against the background of an inflammatory disease.
Pulmonary hypertension associated with embolism or thromboembolism.	The presence of a blood clot in the terminal and initial arteries of the lungs. Overlapping of the pulmonary alveoli with tumors, parasites, foreign bodies.
Mixed pulmonary hypertension.	Sarcoidosis Histiocytosis. Lymphangiomatosis. Lymphadenopathy, leading to compression of the pulmonary arteries. Mediastinitis, characterized by inflammation of the mediastinum.
Pulmonary hypertension due to disorders in the structure of the heart.	A defect in the septa between the atria. Patent ductus arteriosus.

Thus, the following risk factors can be identified that can lead to the development of the disease:

• Taking medications, ingestion of toxic substances. It has been established that pulmonary hypertension can occur while taking Fenfluramine, Rapeseed oil, Aminorex, Dexfenfluramine. Scientists also suggest that the pathology can be provoked by substances such as amphetamine and L-tryptophan.
• Demographic risk factors and medical factors. It has been proven that women are more likely to suffer from pathology. It is also believed that hypertension may be associated with high blood pressure and pregnancy.
• Some diseases. The relationship between PAH and HIV infection has been established. Development of pulmonary hypertension with liver pathologies is possible.

Pulmonary hypertension

Four degrees of pulmonary hypertension have been identified, which determine the severity of the course of the disease:

• The first degree is characterized by the absence of any symptoms.
• The second degree is manifested by all the symptoms of the disease, which are described above. At the same time, their intensity is extremely low, the patient does not present complaints. Pathological manifestations will bother a person only during physical activity.
• The third degree of the disease is manifested by a deterioration in well-being, even against the background of insignificant physical activity. At rest, the patient feels normal.
• The fourth degree of the disease is expressed in the fact that it is difficult for a person to perform even elementary actions. Symptoms of pulmonary hypertension persist during complete rest.

Diagnostics

If a person suspects that they are developing pulmonary hypertension, they should see a doctor.

The doctor will conduct a comprehensive diagnosis, which boils down to the following activities:

• Examination of the patient. During the initial examination, the doctor can detect cyanosis of the skin on the upper and lower extremities. The patient's nails can be thickened, in the form of "drum" sticks. If the patient has emphysema, then his chest will be expanded like a "barrel". The liver is often enlarged, which is noticeable on palpation. It is possible to identify ascites and pleurisy, in which fluid accumulates in the peritoneal cavity and in the pleura. The patient's legs are swollen, the veins in the neck are dilated.
• Listening to the heart and lungs. Above the pulmonary artery, the noises are amplified by 2 tones, characteristic single wheezing is heard. With valvular heart failure, systolic murmurs will be heard. It is possible to listen to the murmurs that characterize heart defect, if any.
• In 55% of cases, the diagnosis allows an ECG to be performed. In 87%, a dilated right ventricle with thickened walls is found. The load on the right side of the heart increases significantly, which can be determined by the corresponding signs. To the right, the electrical axis of the heart deviates in 79% of people.
• Vector electrocardiography reveals pulmonary hypertension in 63% of cases. As for the indicators, they change similarly to the ECG indicators.
• Phonocardiography, which allows you to register heart murmurs, which increases the probability of making a correct diagnosis, at least up to 76%. At the same time, it is possible to detect an increase in pressure in the pulmonary artery, changes in the structure of the right ventricle, congestion in the pulmonary circulation, heart defects.
• X-ray examination of the chest reveals a bulging of the pulmonary artery, expansion of the roots of the lungs, the right parts of the heart become larger in size. The edges of the lungs are characterized by increased transparency.
• Ultrasound of the heart or echocardiography makes it possible to detect an increase in pressure in the trunk of the pulmonary artery, to identify abnormalities in the work of the tricuspid valve and interventricular septum. Also, studies make it possible to detect heart defects, enlargement of the right heart, thickening of the walls of the right ventricle.
• The introduction of the device into the heart through large veins (catheterization of the right heart). This invasive procedure allows you to determine the pressure indicators in the pulmonary artery and in the ventricle, the level of blood oxygenation, circulatory disorders of the ventricle of the heart and lungs. In parallel, medicinal tests can be performed, which make it possible to find out the body's response to calcium antagonists. These drugs are the main ones in the treatment of pulmonary hypertension.

If the listed research methods are not enough to make the correct diagnosis, then the doctor will refer the patient for additional examinations, including:

• FVD - determination of the function of external respiration. This method allows you to exclude pathologies of the respiratory system. If the pressure in the pulmonary artery system is increased, then the doctor diagnoses a decrease in the partial pressure of oxygen and carbon monoxide.
• Ventilation-perfusion lung scintigraphy. During the study, the doctor injects radioactive particles into the blood that reach the lungs. This makes it possible to detect thrombotic masses in the pulmonary artery system.
• Multispiral computed tomography of the lungs and heart with the introduction of contrast. This method allows you to create a three-dimensional model of the respiratory system and display them on the screen. As a result, the doctor receives maximum information about the disease.
• Angiopulmonography. In the course of the study, a contrast agent is injected into the pulmonary vessels, and then a series of images is taken on X-ray machines. This test can detect blood clots in the arteries.

Disease prognosis

The prognosis of pulmonary hypertension is poor. Full recovery will not be achieved. If the patient receives treatment, then heart failure leading to death will still occur, but the patient will still be able to prolong his life.

• If the cause of pulmonary hypertension is systemic scleroderma, then the prognosis is most unfavorable. With the disease, the normal tissue of the organs degenerates into connective tissue. As a result, the person dies within the first year.
• In idiopathic pulmonary hypertension, the prognosis is slightly improved. Such patients can live, on average, three years after diagnosis.
• If a heart defect leads to pulmonary hypertension, then the patient is referred for surgery. The five-year survival rate for such patients is equal to 40-44%.
• If, against the background of pulmonary hypertension, heart failure rapidly increases with damage to the right ventricle of the heart, then death will occur within 2 years after the onset of the disease.
• If pulmonary hypertension has an uncomplicated course and amenable to drug correction, then about 67% of patients step over the 5-year line.

Pulmonary hypertension treatment

Treatment of pulmonary hypertension can be both conservative and operative. Full recovery does not occur. If the human body accepts the ongoing therapy, then this helps to improve its general well-being. A person is able to do physical work. Life expectancy can be doubled.

To ease the course of the disease, as well as increase the quality and duration of life, you must adhere to the following recommendations:

• Physical activity should be limited. This rule should be especially strictly observed immediately after a meal, as well as under the condition of being in a too cold or too hot room.
• Physical activity should be regular, but complexes need to be made only based on medical indications. This allows not to load the heart and lungs, but to maintain adequate vascular tone.
• When traveling by plane, oxygen therapy is required.
• It is necessary to prevent various diseases of the respiratory system.
• You need to stop taking hormonal drugs during menopause.
• Women with pulmonary hypertension are advised not to have children.
• You should stop taking hormonal drugs to prevent unwanted pregnancy.
• It is necessary to monitor the level of hemoglobin in the blood.

Taking medications is the main treatment for pulmonary hypertension. For therapy, several medications are used, combining them with each other.

Drugs that a person will receive throughout his life:

• Disaggregants that prevent the formation of blood clots. The main representative of this drug group is Aspirin.
• Anticoagulant drugs, which thin the blood, make it less viscous, which is also aimed at preventing blood clots from forming. Medicines in this group are Heparin and Warfarin.
• Cardiac glycosides that contribute to the normal functioning of the heart muscle. For this purpose, the patient is prescribed Digoxin.
• Calcium antagonists are drugs that are used for the main therapy of the disease. Their use is aimed at expanding the small vessels of the lungs. The main representatives of this group are: Diltiazem and Nifedipine.

Also, the courses are treated with the following drugs:

• Prostaglandin E and Treprostinil are drugs that are aimed at preventing the formation of blood clots, since they prevent the blood from becoming viscous, while simultaneously expanding the lumen of the vessels.
• Bosentan is an endothelial receptor antagonist. The drug is aimed at relieving vascular spasm from small arteries of the lungs, and also prevents their cells from carrying out pathological growth.
• Sildenafil is a phosphodiesterase inhibitor drug. This drug helps to relieve spasm from the vessels of the lungs and reduces the load on the right ventricle of the heart.

Xylorhodotherapy allows you to saturate the blood with oxygen. This is especially true in the event of unfavorable environmental conditions, or with a sharp deterioration in the patient's well-being. It is also possible to perform inhalations with nitric oxide, which are performed in a hospital room. This procedure allows you to expand blood vessels and alleviate the patient's condition.

If medical correction does not allow achieving the desired effect, the patient is referred for surgery. It is indicated when a heart defect is the cause of pulmonary hypertension.

Depending on the disease, various surgical techniques can be applied, including:

• Atrial septostomy. This method allows you to establish communication between the atria. It is recommended for those patients who suffer from right ventricular failure. Septostomy is used as a preparation for a patient before a heart or lung transplant.
• Thrombendarterectomy. This procedure aims to remove blood clots from the pulmonary arteries. The operation allows to reduce the burden on the heart, to make the symptoms of the disease less pronounced. The procedure will be performed only if the thrombotic masses have not yet begun to degenerate into connective tissues.
• Organ transplant. Lung and heart transplant is possible. The complexity of the operation leads to its rare performance.

Modern drugs

Macitentan

Macitentan is a drug belonging to the group of endothelium receptor antagonists. It is prescribed as an antihypertensive agent, that is, to lower blood pressure in patients with pulmonary hypertension.

The drug is recommended for the treatment of idiopathic pulmonary hypertension, for the treatment of PAH, which develops against the background of heart disease and against the background of connective tissue pathologies. The drug is aimed at relieving symptoms of the disease and improving the quality of life of patients.

Macitentan reduces the risk of hospitalization associated with pulmonary arterial hypertension by 51.6%. The cost of the medicine is quite high and equates to 2,800 euros.

Ventavis

Ventavis is a solution for inhalation, in which the main active ingredient is iloprost. Ventavis is a drug with antiplatelet properties. It prevents the formation of blood clots in the pulmonary arteries by inhibiting platelet aggregation.

Another effect of using Ventavis is a vasodilating effect on the arteries of the lungs. That is, the drug expands their lumen, thereby relieving the load on the heart muscle.

Ventavis is the only inhalation drug from the group of prostanoids that is available in the Russian Federation. It is inhaled 6 to 9 times a day using a nebulizer.

Ventavis therapy has proven effectiveness, which has been confirmed in scientific research. They involved 203 PAH patients who received monotherapy with the drug for 12 weeks. This reduced shortness of breath and improved NYHA functional class in 16.5% of patients. The average increase in the 6-MX distance against the background of treatment was 36.4 m.

Also, the drug can be used as a drug in the complex therapy of pulmonary arterial hypertension. The STEP study on this subject lasted 12 weeks, and participated in 67 patients with PAH. The therapy was supplemented with Bosentan. It was found that the patients' physical performance significantly increased, and other symptoms of the disease began to decline.

The Opitiz study involved 6 people who received the drug for 5 years. At the same time, the survival rate of people increased from 32% to 49% compared to patients who did not receive similar treatment.

The AIR 1 study was also conducted in which people received therapy for one and three years. After a year, the survival rates were 83%, after 2 years 78%, and after 5 years 58%.

Therefore, it can be argued that the drug not only improves the quality of life of patients with PAH, but also prolongs it. The cost of Ventavis is about 100,000 rubles per package.

Tracleer

Tracleer is a drug whose main active ingredient is bosentan. The drug is produced in the form of tablets. Its main effect is the expansion of the pulmonary arteries, due to the removal of vascular resistance from them. This allows you to reduce the load on the heart, lower blood pressure, improve the well-being of patients and increase the body's tolerance to physical activity.

This drug can help prolong life in patients with idiopathic PAH as well as those with secondary PAH. Tracleer is prescribed to patients with pulmonary hypertension on the background of heart defects. It can be used by both adults and children.

The cost of the medicine is 200,000 rubles per package.

Revazio

Revazio is a prescription drug, the main active ingredient of which is sildenafil. The leading effect of taking the drug is the removal of spasm from the pulmonary arteries, as well as the expansion of their lumen. In addition, Revazio allows you to reduce blood pressure, reduce the load on the heart muscle. The patient's body becomes tolerant to physical exertion, even with a severe course of pulmonary arterial hypertension. The drug has been proven to improve the survival rate of patients with PAH.

Revazio can be used in a complex treatment regimen for pulmonary hypertension. It is used to treat all forms of the disease. The average price per package of the drug is 50,000 rubles.

Volibris (Ambrisentan)

Volibris is a drug from the group of endothelin receptor antagonists. Its main active ingredient is Ambrisentan. Taking the drug allows you to reduce the clinical symptoms of pulmonary arterial hypertension, increase the level of exercise tolerance, and improve hemodynamic parameters. The effect of the use of the drug Volibris is stable throughout the year.

The average price for a package of a drug is 60,000 -75,000 rubles.

Adempas (Rioziguat)

Adempas preparation with the main active ingredient Riociguat. This drug belongs to the pharmacological group of antihypertensive drugs, guanylate cyclase stimulants.

Taking the drug Adempas allows you to reduce pulmonary vascular resistance, improve hemodynamics, reduce the severity of shortness of breath, and reduce the clinical manifestations of the disease. The drug used in complex therapy makes it possible to increase the one-year survival rate of patients up to 96%. The drug is prescribed for patients with PAH, accompanied by the formation of blood clots, with idiopathic, hereditary PAH, and PAH associated with connective tissue pathologies.

Aptravi (Selexipag)

The drug Aptravi is a drug from the group of selective agonists of the non-prostanoid prostacyclin receptor. It should be noted that the drug is not registered in Russia. Its main effect is the expansion of spasmodic pulmonary vessels and a decrease in blood pressure. The drug is used in a complex treatment regimen for PAH, as well as as an independent agent. The average cost of one pack of Aptravi is equal to 5200 euros.

The author of the article: Molchanov Sergey Nikolaevich | Cardiologist

Education: Diploma in "Cardiology" received at the PMGMU. I. M. Sechenov (2015). Here I completed my postgraduate studies and received a diploma "Cardiologist".