Galen vein aneurysm – causes, symptoms, diagnosis and treatment

On that day, the Icon of the Mother of God “The Sign” was brought to Yekaterinburg in the Temple on Blood. Alena Babaeva really wanted to kiss. Her pregnancy was excellent. 32 weeks already. That’s just recently, sleep has become bad at night.

It was not difficult to get to the icon: with her belly, they willingly gave way. But getting away from her is almost impossible. Tears poured down the unsentimental Alyona stream. Why? She herself could not understand. He only remembers that she left the church with the firm intention: an urgent need to do an ultrasound!

At home, her “whim” was not supported. “What a whim? You just did today! An excellent doctor looked on the recommendation. He said: everything is in order. ” But Alena stood her ground: urgent need to do it!

We enrolled in the only medical center in which the “window” remained. There was no time to choose, just to be accepted today! Late in the evening, the tired uzist hoped to finish his work day as soon as possible. But could not. A professional eye could not help but pay attention to this baby. There was clearly a mess in his head. “Most likely,” hydrocephalus, “he brought down the conclusion on Alena’s head.

In the morning, she was already standing on the threshold of the medical genetic center. Four Uzists looked at her with pitying eyes, not daring to reach a verdict. Finally, the council ruled: “Malformation of the veins of Galen.” 90% of children with this diagnosis die in utero, of the survivors, 90% die on the first day.

Arteriovenous malformation is a pathological connection between veins and arteries, in which there is no capillary network and arterial blood is directly discharged into the venous, depriving the corresponding organ of nutrition. Vienna Galena is one of the main venous collectors of the brain (collects blood from its internal structures). Galen vein arteriovenous malformation is a rare congenital disease of this vein.

But Alena did not agree. She did not want to fall into these statistics. Three weeks later, she underwent a cesarean section – and Artem was born. Alive. And he continued to survive. “Then we often fell into the smallest percentage according to statistics. That is, statistically, all this was very unlikely. “

On the third day, Artyom had his first stroke – hemorrhage. Alena sent his documents to the three most famous neurosurgical clinics in the country, including to Novosibirsk to the Academician E.N. Meshalkin National Medical Research Center.

And then it turned out that Dr. Orlov from this center should come to Yekaterinburg. Having learned about Artyom’s case, he immediately said: “We must save!” Probably, this really saved him. Because in all the official answers that came a month later, Artem was denied assistance: “inappropriate.”

After the rescue operation (this was the first operation in the Sverdlovsk region to correct the malformation of the Galen vein, which was successful), Artyom underwent another nine neurosurgical operations. Each time, giving her son for an operation, Alena realized that maybe she was kissing him for the last time.

Galen vein aneurysm can cause complications in the form of cerebral palsy, hydrocephalus, epilepsy. Artem got all the complications that can be imagined. The worst of these is West syndrome, a complex form of epilepsy. It was this syndrome that provoked 30 respiratory arrests per day. Artem lay motionless for days, his gaze seemed blurred and absent.

“He seemed to be not with us,” says Alena. “But we also managed to defeat West syndrome. The son went into long-term remission, he returned to us, his liberated brain began to perform a variety of tasks – from picking his nose to crawling. “

By the age of two, Artyom was ranked V in the GMFCS classification system for large motor functions. This meant that he would never go. Usually, Tier V children remain that way for life. But after two years, when the funds for epilepsy were selected, his body began to actively respond to rehabilitation. Today, he himself can roll over, get up on all fours, stand and walk with support. And its capabilities are evaluated as corresponding to Level III according to the GMFCS system.

“Let the walkers, but he already walks! It is unbelievable, but it is possible! And to all the women who turn to me, faced with the same diagnosis, I say: “I always repeat the prayer,“ Lord! Give me the strength to change that which I can change, the courage to accept that I cannot change, and the wisdom to distinguish one from the other. ”

Alain, along with other parents whose children were diagnosed with AVM Galena veins, creates a support base for newcomers. Together they created a website where you can get the necessary information and contact experienced mothers who have already achieved good results.

Even if no pathologies were detected during pregnancy, be careful if you notice the following symptoms in your baby: it quickly gets tired during feeding; swelling of the soft tissues of the head; an increase in head circumference, ahead of the norm; pronounced venous pattern on the head; possible: vomiting, strabismus, convulsions. Be sure to contact a neurosurgeon!

OBJECTS, METHODS AND LEVELS OF RESEARCH OF PATHOLOGICAL ANATOMY

Galen’s vein is called the cerebral vein, because it is one of the largest venous trunks that carry blood, saturated with carbon dioxide and metabolic products, from the inner parts of the brain – subcortical nuclei, optic tubercles, transparent septum, plexuses of the lateral ventricles.

The large vein of the brain belongs to the deep venous system and passes in the subarachnoid space (it is also called the cistern of the same vein in this area), which connects to the lower sagittal venous sinus with the formation of a direct sinus. The large vein of Galena flows into the direct sinus.

Galen’s vein anatomy depends on the shape of the head. Its length is about 12-14 mm, while in people with a dolichocephalic type of skull (with a long and narrow head) it can reach 2 centimeters, and for brachycephals with a short and wide head – a little more than a centimeter.

The diameter of the vessel is not associated with the shape of the skull and averages 5–7 mm, however, it has been observed that brachycephals have a relatively short, but wider trunk than dolichocephals with a long and narrower vein.

The rate of blood flow in the vein of Galen in children under one year is 4-18 cm/sec.

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Among the changes that are most often detected in the vessel under consideration are:

  • Galen vein aneurysm;
  • Arteriovenous malformation of the cerebral vein.

Galen vein aneurysm is one of the most severe and rather complex forms of vascular pathology of the brain for early diagnosis and treatment. Unfortunately, it is usually found no earlier than the third trimester of gestation, which becomes a real shock for future parents, because previous screening ultrasounds showed the norm.

At the same time, a defect detected even during pregnancy, even rather late, makes it possible for the child to decide on the tactics of treating the baby, choose a clinic and specialist, and psychologically tune into the struggle for the well-being of the child.

Galen vein aneurysm is formed in utero. Initially, the nutrition of brain structures in a growing embryo occurs by embryonic vessels, which during the first two months of gestation mature into full arteries and veins. If this process is disturbed, the embryonic vessels that give rise to aneurysms and other malformations remain undeveloped in the fetal brain.

Among the reasons that can contribute to anomalies in the formation of the vascular bed, indicate:

  1. Exposure to viruses and bacteria (herpes, rubella, respiratory infections, etc.);
  2. Taking certain medications;
  3. Influence of ionizing radiation.
  • It is important to note that the defect is laid in the first third of the gestation, but it becomes noticeable for diagnosis much later.
  • From the point of view of the anatomy of the aneurysm can be a local increase in the lumen of the vein, but much more often it looks like multiple messages between the vessels in the form of a ball consisting of vessels of the embryonic type.
  • Blood from the arteries of the brain (the system of the internal carotid and vertebro-basilar) is discharged into the abnormally formed vein, not reaching the nervous tissue of the hemispheres and subcortical structures to a sufficient extent, because of which the latter experience anemia, and the venous section is overloaded.

Embryonic vessels are devoid of a smooth muscle layer, therefore, they can easily rupture with the outflow of blood into the nervous tissue, as well as stretch with an excess volume of fluid. Stretching and increasing the diameter of the venous part of the bloodstream contributes to compression of the cerebrospinal fluid and the development of hydrocephalus.

Galen’s vein aneurysm is found in the front of the skull, behind and above the visual tubercles in the eponymous cistern. There are several forms of aneurysm:

  • Intramural when an artery flows directly into a vein.
  • Choroidal – is represented by the vascular plexus in the choroid gap.
  • Parenchymal – multiple vascular messages are located in the cerebral parenchyma.

CONTENTS AND OBJECTIVES OF THE PATHOLOGICAL ANATOMY

Pathological anatomy is an integral part of pathology (from the Greek. RaShoah – a disease), which is an extensive field of biology and medicine that studies various aspects of the disease. Pathological anatomy studies the structural (material) basis of the disease.

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This study serves both the theory of medicine and clinical practice, therefore pathological anatomy is a discipline of scientific and applied nature. The theoretical, scientific, significance of pathological anatomy is most fully revealed when studying the general laws of development of cell pathology, pathological processes and diseases, i.e., the general human pathology.

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General human pathology, primarily cell pathology and morphology of general pathological processes, is the content of the course of general pathological anatomy.

The clinical, applied, importance of pathological anatomy consists in studying the structural foundations of the whole variety of human diseases, the specifics of each disease, otherwise, in creating the anatomy of a sick person, or clinical anatomy. This section is devoted to the course of private pathological anatomy.

The study of general and particular pathological anatomy is inextricably linked, since general pathological processes in their various combinations are the content of both syndromes and human diseases.

The study of the structural foundations of syndromes and diseases is carried out in close connection with their clinical manifestations.

Clinical and anatomical direction is a distinctive feature of domestic pathological anatomy.

When studying pathological processes and diseases, pathological anatomy is interested in the causes of their occurrence (etiology), development mechanisms (pathogenesis), morphological foundations of these mechanisms (morphogenesis), various outcomes of the disease, i.e., recovery and its mechanisms (sanogenesis), disability, complications, as well as death and mechanisms of death (thanatogenesis). The task of pathological anatomy is also the development of a doctrine of diagnosis.

In recent years, pathological anatomy pays special attention to the variability of diseases (pathomorphosis) and diseases that arise in connection with the activities of a doctor (iatrogenic).

Pathomorphosis is a broad concept that reflects, on the one hand, changes in the structure of morbidity and mortality associated with changes in human living conditions, i.e.

changes in the general panorama of diseases, on the other – persistent changes in the clinical and morphological manifestations of a particular disease, nosology – nosomorphosis, which usually occurs in connection with the use of drugs (therapeutic pathomorphosis). Iatrogenic (pathology of therapy), i.e.

diseases and complications of diseases associated with medical manipulations (drug treatment, invasive diagnostic methods, surgical interventions) are very diverse and they are often based on a medical error. It should be noted the increase in iatrogenicity in recent decades.

Pathological anatomy receives material for research at autopsy, surgery, biopsy and experiment.

At autopsy of the dead – autopsy (from the Greek ai1orz1a – seeing with one’s own eyes) they find both far-reaching changes that led the patient to death, and initial changes that are more often found only with a microscopic examination.

In this case, they mainly use light-optical research, since cadaveric changes (autolysis) limit the use of more subtle methods of morphological analysis.

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At the autopsy, the correctness of the clinical diagnosis is confirmed or a diagnostic error is detected, the causes of the patient’s death, the particular course of the disease are established, the effectiveness of the use of therapeutic drugs, diagnostic manipulations is revealed, statistics on mortality and mortality are developed, etc.

Surgical material (removed organs and tissues) allows the pathologist to study the morphology of the disease at various stages of its development and use a variety of methods of morphological research.

A biopsy (from the Greek. Yoz – life and op515 – vision) is an intravital tissue capture for diagnostic purposes. The material obtained by biopsy is called a biopsy.

More than 100 years ago, as soon as the light microscope appeared, pathologists began to study the biopsy material, reinforcing the clinical diagnosis with a morphological study. At present, it is impossible to imagine a medical institution in which they would not have resorted to biopsies to clarify the diagnosis.

In modern medical institutions, a biopsy is performed for every third patient, and there is no such organ, such tissue, which would not be accessible to biopsy examination.

Not only the volume and methods of biopsy are expanding, but also the tasks that the clinic solves with its help.

Thus, the pathologist, who became known as the clinical pathologist, becomes a full participant in the diagnosis, therapeutic or surgical tactics and prognosis of the disease.

Biopsies make it possible to study the Sami initial and subtle changes in cells and tissues using an electron microscope, histochemical, histoimmunochemical, and enzyme methods, i.e., those initial changes in diseases whose clinical manifestations are still absent due to the viability of compensatory-adaptive processes.

In such cases, only the pathologist has the potential for early diagnosis. The same modern methods allow us to give a functional assessment of the structures changed during the illness, to get an idea not only about the nature and pathogenesis of the developing process, but also about the degree of compensation of impaired functions. Thus, biopsy is currently becoming one of the main objects of research in solving both practical and theoretical issues of pathological anatomy.

Kirill Orlov: “This is a deadly pathology, but we have good results”

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Kirill Yurievich Orlov, candidate of medical sciences, head of the Center for Angioneurology and Neurosurgery of the National Medical Center named after Academician E.N. Meshalkin (Novosibirsk) agreed to answer our questions.

– Kirill Yuryevich, what is the right name for the pathology we are talking about? Malformation? Aneurysm?

– Malformation of the vein of Galen and aneurysm of the vein of Galen is one and the same condition. But in our opinion, the most correct name is arteriovenous malformation of the Galen vein (AVM veins of Galen).

– Parents who are faced with this diagnosis go to the Internet and, first of all, see the statistics: mortality – 90%. Is this really so?

– Yes. If this pathology is not treated, 90% of patients die, 10% become disabled and die a bit later, until adulthood, alas, they do not survive . True, I had one patient with Galen vein malformation of forty-five years. With this disease, to my surprise, she lived for many years, suffered from headaches every day, and finally she was given the correct diagnosis.

Basically, indeed, such a vice does not allow the child to live and develop normally. Galen vein malformation is a malformation in which, from early childhood, there is a direct discharge from the artery into the Galen vein, due to this, the pressure in the venous system rises, and absorption is disturbed. Liquor with nutrients from an artery usually passes through the brain and is absorbed into the veins. And if the pressure in the veins is increased, then it is not absorbed, there is an increased pressure of this fluid. Hydrocephalus develops.

In the most severe conditions (despite the fact that the pathology is in the head), the heart grows, the smallest patients have unstable hemodynamics (blood movement in the vessels), they cannot breathe on their own and, sometimes, are on mechanical ventilation from the first days of life.

– And how to identify this malformation?

– It can be diagnosed in utero. We detected this rather rare disease in pregnant women, invited them to give birth to our Novosibirsk perinatal center, and then immediately prepared the baby for surgery in our center named after academician Meshalkin.

– What operation is needed in this case?

– Endovascular surgery. It is usually carried out in two stages. The first step is to stabilize hemodynamics. To do this, turn off the largest fistula – the tube, through which there is a direct discharge from an artery into a vein, bypassing the capillaries. When the baby grows up a bit, we invite him to the second stage of treatment and turn off the Galen vein completely.

The endovascular (intravascular) method is the main, highly effective and low-traumatic method of treating Galen AVM with low disability and mortality. The aim of endovascular treatment is to minimize or stop the flow of arterial blood into the venous system of the brain. Endovascular interventions are performed without incisions, through small punctures on the skin (1–4 mm in diameter) under X-ray control. “How does this fistula turn off?”

– We pierce the femoral artery, through it we insert a guiding catheter through the aorta, through which we then pass the microcatheter directly to the vessels of the brain, directly to this fistula. According to the microcatheter, a special glue is introduced into the fistula to close it. At this moment, it is important to close the fistula itself, without entering either the vein or the artery. Then the blood flow normalizes: the blood is not discharged along the nearest path, it goes through the capillaries and feeds the brain.

Children are very plastic, after six months or a year, they develop a normal circulatory system. Those patients who were operated on time develop well. The main thing is not to miss the moment. We have good results: 60% of our patients survived and overcame this defect.

– What age was your youngest patient who managed to save?

– That is, you do not need to wait for the baby to grow up and get stronger?

– Expectant tactics never help. Rather, the choice of tactics will be based on whether the patient will undergo surgery or not.

– And it happens that an ultrasound scan missed this defect?

– No, it is immediately visible and easy to diagnose. If he was not diagnosed in utero, rather, it is because the woman did not go for an ultrasound scan. Most likely, he will then be found in the hospital when examining the baby. We almost do not see such patients in whom Galen’s AVMs were found by chance, not in the perinatal period.

I’m not talking about adults who did not have the possibility of such a diagnosis in early childhood. Now the system works fine, it detects well. Another problem is that the doctors themselves do not know where to send such patients. In a number of neurosurgical centers there is no children’s department, somewhere there is no children’s resuscitation .

– Can you recommend pregnant women, whose children have been diagnosed with “AVM Galena’s AVM” in utero, to come to you to give birth in the hope that after the birth the baby will have an operation?

Sacral plexus and its pathology

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The most important element of the nervous system is the sacral plexus, which innervates the organs and tissues of the lower extremities.

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Pathological processes developing in this area are usually accompanied by neuralgia and negative symptoms.

In order to assess the severity of the condition and prescribe appropriate therapy, it is necessary to have an idea of ​​the anatomical features of this plexus.

The sacro-lumbar plexus combines several varieties of nerve endings. The main role in its formation is played by one of the three spinal nerve roots, which partially includes the fourth and twelfth nervous thoracic branches.

The lumbar plexus consists of the following nerves:

  • femoral-genital;
  • iliac-hypogastric;
  • obturator;
  • femoral
  • lateral;
  • ileo-inguinal.

The branches of the nerve endings are located in front of the transverse processes coming from the lumbar vertebrae. They are responsible for the nerve conduction of muscles, including the abdominal. In addition, there is a close relationship with the receptors of the genital organs, limited to the thigh and the surface of the lower leg.

The structure of the lumbosacral plexus

Nerve endings in the lumbar plexus are conditionally divided into groups.

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First

The iliac-hypogastric plexus is formed by the first lumbar and twelfth thoracic nerve endings that pass through the large lumbar muscle and intersect with the front surface of the lumbar square muscle. Further, the plexus descends, passing back to front, innervating the abdominal transverse muscle of the iliac crest, lying between the oblique abdominal muscles.

The iliac-hypogastric ending passes through the wide tendon plate of the external oblique muscle in the region of the inguinal ring and the muscle itself, after which the nerve branches into several processes. The main function of this nerve is to innervate the abdominal muscles. In addition, the endings of the first three provide innervation of the skin of the buttocks and thighs.

Iliac-inguinal plexus – is the beginning of another branch of the anterior nerve root, which also enters the lumbar plexus and is located slightly below the ileo-hypogastric.

Zones of violation of the nerve conduction of the first three plexuses (a – iliac-hypogastric, c – femoral-genital, b – iliac-inguinal, d – lateral skin thigh)

The anatomy of the nerve plexuses of the first three has small distinctive features depending on the gender of the patient. In women, they bind the central nervous system to the skin of the pubis and labia majora, while in men they pass through the inguinal canal, being separated into thinner branches innervating the surface of the thighs near the scrotum.

The second

Slightly lower than the nerve endings of the first three are 3 large nerve branches (lateral, femoral and obturator branches):

  • lateral – located in the lateral part of the inguinal ligament, it can lie superficially or inside the tailor muscle (under the connective membrane). The lateral branch is responsible for the innervation of the lateral surface of the buttocks (further from the large bony trochanter of the thigh and closer to the lateral femoral surface).
  • obturator – this branch extends next to the large lumbar muscle (downward and toward its edge), and then penetrates into the small pelvis. Further, its attachment to the circulatory system and the final exit (together with the vessels) to the thigh, between the adductors, are observed.
  • femoral – this nerve branch is closely interconnected with the adductors, the hip and the knee joint. It provides the superficial (middle) femoral part, closer to the knees. Of all the listed lumbar nerve plexuses, the femoral is the largest and originates at the border of the 5th lumbar vertebra of the same muscle groups (iliac and lumbar).

The nerve endings of the lumbar are part of the general branch system of the sacral, coccygeal and lumbar, which are interconnected and form 2 plexuses (sacral and lumbar).

Short

Short branches, in turn, consist of the following nerve endings:

  • obstructive internal and genital;
  • lower and upper buttocks;
  • piriform and femoral square muscle.

The sciatic nerve endings of the plexus are divided into the lower (together with the artery leaving the pelvis and connecting to the gluteus maximus muscle) and upper (leaving the pelvic cavity with the gluteus muscle through the supra-piriform orifice and connecting with the femoral fascia).

These branches include:

Branching of the nerve endings of the lower extremities of the lumbosacral plexus in accordance with the international classification

This branch descends vertically down to the soleus muscle of the ankle-popliteal canal, dividing all along the branches.

Some of them innervate the triceps ankle, while others pass in the long muscle flexors of the fingers, including the large one on the foot.

The more sensitive endings that are present in the lumbar and sacral plexus are combined with the knee capsule of the joint, ankle and interosseous membrane of the lower leg. The largest sensitive process is the caviar (medial cutaneous).

This branch extends slightly to the side of the fibula (neck) at the location of the popliteal fossa, from which 2 main branches already extend: deep and superficial.

Deep – provides innervation of the skin on the back of the foot (near the lateral edge) and the back of the skin surface of 3 and 2 fingers. This branch is directed downwards with the subsequent division into the intermediate and medial.

The common peroneal branch is responsible for the sensitivity of the short and long peroneal muscles.

Depending on the functional impairment of a particular nerve ending, a lesion of the lumbosacral region due to pinching (pinching) of the sciatic nerve is diagnosed. In this case, due to compression of the nerve endings, acute pain in the lower extremities appears.

This pathological condition develops one-sidedly, but there are cases of bilateral lesions, most often in men, whose activity is associated with increased physical activity.

In medical practice, such a disease is classified as sciatica (sciatic neuralgia) or lumbosacral radiculitis.

Unilateral damage to the sciatic nerve with sciatica

Pathologies can be caused by various injuries (gunshot wounds, vertebral column fractures and concomitant compression of this section by bone fragments, pelvic and abdominal tumors). In addition, the inflammatory process and impaired functionality is possible with abdominal aortic aneurysms and nerve compression by the head of the fetus during labor.

With pathological changes in the ovaries, uterus, peritoneum, pelvic fiber and the appendix, the development of secondary lumbosacral plexitis is possible. In addition, sometimes inflammation is caused by the development of infectious processes (brucellosis, flu, tuberculosis, syphilis, etc.). A characteristic feature of plexitis is unilateral damage to nerve endings.

In case of violation of the functionality of the lower trunk in the lumbar plexus, paresis of the gluteal, quadriceps femoral and twin muscles is possible. This condition provokes difficulty in the extension ability of the ankle, impaired walking, decreased or complete disappearance of the knee reflex. In addition, there is often a loss of sensitivity of the ankle joint and thigh.

Same twins with different destinies

It is hard to believe that Muscovite twins Masha and Oleg Moiseevy have traveled such a different path in their three years. Oleg is an ordinary healthy kid who developed, as in a textbook, fitting into all physical and psycho-emotional normative tables. And Masha, about whom her mother was told in the hospital: “You do not approach her, she is not a tenant!”

But by the time they were born, their life was proceeding in exactly the same way, in the same womb. Only Masha was not lucky. And at some point, a malformation of Galen’s vein happened in her head. The vessels got confused and stopped supplying the brain properly. Following the heart was injured. Usually this malformation affects boys. But for some reason, the choice fell on Masha.

When the baby was born, her heart was the size of the entire chest. At the Institute, Burdenko decided to postpone the operation for up to five months. Until three months she screamed wildly, reacting to the slightest changes in the regime and weather, and it seemed to her mother that the baby always had a headache. Oleg at that time was sleeping peacefully.

By five months, Masha had hydrocephalus, her head was heavy, she could neither lift it nor hold it, she could not turn over on her own. But after the first operation at the Burdenko Institute, and especially after the second, at eight and a half months, it was as if life had been breathed into Masha. Her vessels were no longer confused, blood supply was normal. And she began to stubbornly catch up with her brother.

Before that, it seemed that nothing interested her. And after the operation, looking at him, she gradually sat down, then she went. It does not matter that Oleg went to 11 months, and Masha – after six months. By two years, she completely caught up with him. And although this year in the kindergarten the speech therapy commission found Masha a speech lag, a month later she made a big breakthrough here too and spoke up with whole sentences.

Today, Masha has no developmental lag and she is not different in level from her twin brother.

“With the malformation of Galen’s veins, you can survive and develop normally,” says Polina, mother of twins. “Early medical intervention is important.”

If you need medical assistance or consultation, an application for remote consultation and hospitalization in specialized hospitals can be submitted here!

Tatyana Jakowenko

Editor-in-chief of the Detonic online magazine, cardiologist Yakovenko-Plahotnaya Tatyana. Author of more than 950 scientific articles, including in foreign medical journals. He has been working as a cardiologist in a clinical hospital for over 12 years. He owns modern methods of diagnosis and treatment of cardiovascular diseases and implements them in his professional activities. For example, it uses methods of resuscitation of the heart, decoding of ECG, functional tests, cyclic ergometry and knows echocardiography very well.

For 10 years, she has been an active participant in numerous medical symposia and workshops for doctors - families, therapists and cardiologists. He has many publications on a healthy lifestyle, diagnosis and treatment of heart and vascular diseases.

He regularly monitors new publications of European and American cardiology journals, writes scientific articles, prepares reports at scientific conferences and participates in European cardiology congresses.

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