What is Eisenmenger's syndrome and why does it develop? Is it dangerous for children and adults? Eisenmenger's complex is a combined heart defect.

Eisenmenger's syndrome is a severe increase in pulmonary pressure due to irreversible sclerotic changes in the vessels of the lungs. Synonym of the name: obstructive vascular pulmonary disease.

The disease was first described by the Austrian physician Viktor Eisenmenger in 1897. He reported on a patient who had been cyanotic and short of breath since childhood, who died of a heart attack and massive pulmonary hemorrhage. An autopsy showed a large hole in the interventricular septum and a displaced aorta. This was the first mention of a connection between pulmonary hypertension and.

The disease affects from 4 to 10% of carriers of congenital malformations.

Obstructive vascular pulmonary disease is an acquired syndrome that occurs secondarily in the absence or irrational treatment of congenital heart defects. The disease can have manifestations at any age when sclerosis of the pulmonary vessels occurs.

In contrast, the Eisenmenger complex is a congenital cardiac defect. The disease is represented by a complex of three anomalies: an interventricular septal defect, and its discharge from both ventricles, an increase in the right ventricle. Pathology is also accompanied by pulmonary hypertension.

Eisenmenger's syndrome always develops secondarily. For its occurrence, the presence of congenital heart disease with enrichment of the pulmonary circulation is necessary. The Eisenmenger complex develops primarily (intrauterine).

Development mechanism, causes and risk factors

Pathology is a complication of congenital heart defects with left-right blood shunt. The following causative (primary) diseases are distinguished:

- a hole in the septum between the ventricles, the most common cause of the syndrome.
- a hole in the tissue separating the atria.
- a violation in which the ductus arteriosus does not overgrow in the first days of a child's life and continues to supply arterial blood to the lungs.
An open atrioventricular canal is a complex, rare defect that combines a hole at the site of fusion of the interventricular and interatrial septum with pathology of the mitral valve.
The aortopulmonary window is an abnormal shunt between the pulmonary artery and the aorta.
Transposition of the great vessels.

With these diseases, pulmonary blood flow is enriched. In response to this, a spasm occurs in the vessels of the lungs, aimed at limiting the flow of blood. At this stage, pulmonary hypertension is reversible.

The disease can also develop in patients after surgical creation of a systemic-pulmonary shunt or anastomosis during palliative correction of congenital malformations.

Eisenmenger's syndrome develops if the heart defect goes unnoticed before symptoms of damage to the pulmonary arteries appear, or the patient does not receive adequate treatment, including surgery, to compensate for it. Most children Untreated pulmonary hypertension develops in the second year of life.

Prolonged lack of treatment of defects leads to permanent vascular spasm. Vascular rigidity develops - irreversible sclerosis of the vascular walls, which lose their ability to contract and relax. Pulmonary hypertension becomes irreversible.

Increased pulmonary vascular resistance prevents blood from reaching the lungs from the pulmonary artery. As a result, the pathological left-right shunt of blood changes to right-left.

The presence of congenital malformations in the family history also increases the risk of having a child with a similar defect and the occurrence of the syndrome.

Development and stages of the disease

In a healthy heart, the chambers and vessels are securely separated by partitions and valves that regulate the direction of blood flow. The right ventricle and atrium send venous blood to the lungs, where it is oxygenated. The left chambers take enriched blood and pump it into the aorta and further through the systemic circulation.

With defects with enrichment of the small circle, the lungs receive an excess volume of blood. Under the influence of a constant increased load, the small vessels of the lungs are damaged, the pressure in them increases. This condition is called arterial pulmonary hypertension.

Due to the increased vascular resistance, venous blood can no longer enter the lungs in full, mixes with arterial blood and is sent through the left ventricle or atrium to the aorta - a condition occurs when the direction of blood flow through the window in the septum changes.

The concentration of oxygen in the blood falls, which causes increased production of red blood cells, cyanosis, shortness of breath.

In 1958, American cardiologists Heath and Edwards proposed a description of the development of the syndrome through the stages of histological changes in the vessels of the lungs. Changes are reversible in the early stages, are characterized by stretching of the pulmonary arteries and the growth of their inner layer.

As the disease progresses, small arteries sclerotate due to the replacement of elastic connective tissues (fibrosis), and signs of atherosclerosis of large arteries appear. The expansion progresses, plexiform damage to small arteries grows. At the last stage, necrotic lesions of the arteries are observed. as a result, fibrosis, infiltration of the arterial wall with leukocytes and eosinophils.

Danger and complications

Without appropriate treatment and monitoring, Eisenmenger syndrome can develop complications, including:

Symptoms

The symptoms of Eisenmenger's syndrome and PAH are nonspecific and develop slowly. This makes it difficult to make a diagnosis in patients with previously undiagnosed heart disease.

The most common symptoms:

cyanosis, bluish or gray coloration of the skin and lips,
shortness of breath during exertion and at rest,
pain or pressure in the chest
arrhythmia or tachycardia,
syncope - fainting caused by a brief violation of blood flow in the vessels of the brain,
headache,
dizziness,
swelling, numbness of fingers and toes,
"drum sticks and watch glasses" - characteristic changes in the fingers and nails due to the growth of connective tissue.

When to see a doctor?

The symptoms described above do not necessarily indicate Eisenmenger's syndrome and pulmonary hypertension, but the occurrence of any of them is a reason to contact a general practitioner and a cardiologist, as well as the appearance.

Cyanosis, shortness of breath, edema indicate a serious malfunction of the organs and systems of the body, even if the patient has not previously been diagnosed with heart disease.

If you suspect Eisenmenger's syndrome, your doctor may need to:

Information about any heart surgery, if the defect has already been diagnosed and corrected.
Family history, that is, information about relatives with congenital heart defects, diabetes, hypertension, as well as those who have had a stroke or myocardial infarction.
A list of all medications, including vitamins and dietary supplements, that the patient is taking.

Diagnostics

If you suspect PAH and Eisenmenger's syndrome, a cardiologist should conduct a series of studies, among which may be:

- registration of the electrical activity of the heart. Can show the disturbances that caused the patient's condition.
chest x-ray- with PAH, the picture shows the expansion of the heart and pulmonary arteries.
- allows you to examine in detail changes in the structures of the heart and evaluate blood flow through the chambers and valves.
and- is prescribed to assess the characteristic changes in the composition of the blood.
CT scan- allows you to get a detailed image of the lungs, can be performed with or without contrast.
Cardiac catheterization- insertion of a catheter into the heart (usually through the femoral artery). In this study, you can measure the pressure directly in the ventricles and atria, evaluate the volume of blood circulating in the heart and lungs. It is carried out under anesthesia.
load test- takes an ECG when the patient rides an exercise bike or walks along the track.

Course and treatment in adults and children

The disease occurs in untreated patients with congenital cardiac anomalies. The appearance of the clinic depends on the severity of the defect: the more pronounced it is, the earlier spasm and rigidity of the pulmonary vessels will develop.

More frequent detection in children is due to the following reasons:

Most children with severe birth defects do not survive to adulthood.
Manifestations of congenital anomalies in children are more striking.
Specific symptoms of congenital malformations contribute to the early detection of the disease.

Differences in the clinic and course in children:

The manifestations of the underlying disease predominate.
Frequent episodes.
Pulmonary hypertension rapidly increases to 50 mm or more. rt. Art.
Cyanosis is common to all skin integuments.

In adulthood, nonspecific signs predominate:

Shortness of breath on exertion.
Cyanosis of lips, ears, fingertips.
Weakness.
Arrhythmia.
Chronic heart failure.
Headache.

In both groups of patients, an increase in the level of erythrocytes and blood clots due to chronic hypoxia are detected, however, adults are more prone to thrombosis than children. For adults, the syndrome of sudden cardiac death is also more characteristic.

Currently, there is no cure for Eisenmenger's syndrome. Patients with PAH should be seen by a cardiologist, and blood pressure and blood counts should be monitored regularly. All measures taken are aimed at maintaining the quality of life, stopping the symptoms of the disease, and preventing complications.

Treatment in children is surgical. With this disease, operations often take place in several stages and include:

Restoration of the normal anatomy of the heart.
Shunt removal.
Decreased pressure in the pulmonary trunk.

Palliative care for advanced patients (adults) aimed at improving the quality of life. The following groups of drugs are used in the treatment:

cardiac glycosides.
Diuretics.
Beta blockers and calcium channel blockers.
Cardioprotectors.

The main components of the combined drug:

Sildenafil and other phosphodiesterase type 5 inhibitors are used for their relaxing effect on the smooth muscle walls of blood vessels.
The use of endothelin receptor antagonists allows maintaining a satisfactory condition of the pulmonary vessels. The question of long-term therapy with these drugs remains open, as some research results show their negative effect on the heart.
The use of prostacyclins can reduce pressure in the pulmonary artery, improve oxygen consumption by muscles and partially reverse damage to the vessels of the lungs.
Antiarrhythmic drugs are used to equalize the heart rhythm and reduce the risks associated with arrhythmias.
Aspirin or other anticoagulants are recommended to reduce blood viscosity.

Opportunities for transplantation of the lung-heart complex are being developed. The disadvantage of this method is numerous complications and an unpredictable waiting period for the donor.

About the prevalence and life expectancy

The contingent of patients: untreated patients with congenital cardiac anomalies.

Revealing: in children - in 10-12% of cases, in adults - in 7-8% of cases.

Etiology: septal interventricular defect (60.5%), septal atrial defect (32% of cases).

Forecast: relatively favorable for early detection of the disease (in the first year of life). The discovery of the syndrome in adults often indicates that only palliative care is possible. With a long course of the disease, irreversible changes in the lungs and heart lead to thrombosis and heart failure. Under such conditions, even a full-fledged surgical treatment of a congenital defect does not lead to recovery.

The average life expectancy is 18-40 years.

Is pregnancy possible?

Pregnancy is highly undesirable in Eisenmenger's syndrome and carries a high risk of maternal and fetal death. If pregnancy persists, constant monitoring of the state of the heart and blood vessels is required, since the load on them increases as the fetus develops.

Caesarean section shows a high mortality rate for women with PAH, so vaginal delivery with epidural anesthesia is recommended.

What improves prognosis?

The prognosis for life is most favorable in children when Eisenmenger's syndrome is detected in the early stages. Surgical correction can stop vascular sclerosis and reduce pressure in the lungs. Other factors for improving the prognosis:

Low severity of heart disease.
Adequate medical preparation for surgery.
Rational surgery.
Lifelong control of the level of erythrocytes and platelets in the blood.

Eisenmenger complex precautions include:

The use of antibiotics for prevention before and after surgical interventions, including dental ones.
Vaccination against pneumococcus, influenza, and other infections that can cause high fever and increase heart workload.
Quitting smoking and being in smoking companies.
Caution when taking any medication, including dietary supplements.

Eisenmenger's syndrome is a life-threatening condition. The prognosis for this disease depends on the defect that caused it, and the ability to receive adequate treatment.

Such a pathology as Eisenmenger's disease (complex, syndrome) occurs in the fetus during the perinatal period. It becomes dangerous if a correction is not detected and made in time.
If the disease was not detected at an early age, then when its symptoms appear at an older age, it is necessary to seek the advice of a specialist as soon as possible.

So, let's figure out what is included in the concept of Eisenmenger's syndrome.

Features of the disease

The disease, caused by congenital anomalies in the region of the heart, was named after the scientist who discovered and described the disease - Eisenmenger's syndrome. Pathology refers to a type of heart disease.

The syndrome has a set of congenital abnormalities:

Displacement of the aorta from its normal position. Her deployment is called the "horseman". The abnormal location allows blood to enter the aorta from both ventricles.
The septum between the ventricles has, which makes it possible to communicate with venous and arterial blood, which is not provided for in a healthy heart. The septum may be absent altogether. This situation violates the correct ratio in the level of pressure created in different halves of the heart. In the right atrium, the pressure is increased, which has a bad effect on the blood flow of the small circle, there are difficulties for blood to get there.
The right ventricle is hypertrophied, usually it increases in volume as a result of the previous (first two) pathologies.

Deviations coincide with signs. The difference is that there is no narrowing of the aorta.

Forms and classification

The disease is divided into two periods.

Pale malformation stage

Pathological abnormalities in the anatomical structure characteristic of Eisenmenger's syndrome lead to the fact that arterial blood enters from the left ventricle into the right (venous) ventricle. This anomaly does not cause a change in the color of the skin to a bluish tone.

There is a deformation of the right ventricle, as a result of these violations, the expansion of its internal volume. Vessels in the lungs react to increased pressure in the right atrium, increasing resistance over time. Prerequisites for pulmonary hypertension are created.

The following video will tell you what pulmonary hypertension is:

Blue type defect stage

As a result of the above situation, there is a change in the direction of blood flow between the ventricles through a defect in the septum to the right-left. Dilution of arterial blood with venous blood and the ingress of oxygen-depleted blood into the systemic circulation causes oxygen starvation.

At this stage, cyanosis appears. The intensity of the blue skin tone depends on the degree of the problem. Over time, complications associated with hypoxia appear.

The blood becomes more viscous.
Compensatory mechanisms include increased erythrocyte count. Their increased decay creates the prerequisites for the emergence of new diseases:
- gout,
- cholelithiasis
- and others.

Causes

Pathology is congenital. The behavior and health of the mother during childbearing has a direct impact on his future health.

Negative factors:

impact on intrauterine life of the fetus:
- electromagnetic radiation,
- radiation,
- vibration,
- chemicals, including drugs;
- bacteria and viruses capable of causing abnormal development;
- carcinogens;
chromosomal breakdowns that cause abnormal development of organs.

Symptoms

The disease may not manifest itself for some time. Deviations cause undesirable processes, as a result of which symptoms of the disease appear over time.

loss of strength, poor health during physical activity;
under load, the skin becomes bluish in color,
pain in the left side of the chest, which can radiate to the shoulder blade, arm;
possible situations of loss of consciousness,
dyspnea,
sometimes cough with bloody sputum.

Diagnostics

There are some signs by which a specialist on examination may suggest the presence of a symptom of Eisenmenger:

cyanosis of the skin,
changing the shape of the fingertips - "drumsticks" and nails - "watch glasses",
listening to a certain type of noise.

If there are signs of the disease or if this type of defect was identified at birth and timely adjustment was not made, then it is urgent to examine the patient in order to correctly prescribe treatment.

To study the problem, methods are used:

Angiography provides comprehensive information. It is carried out if there are no contraindications.
Electrocardiography is a necessary procedure for determining rhythms that are uncharacteristic of the normal functioning of the heart. It is possible to observe the activity of the heart around the clock with this method.
Catheterization - a catheter enters the heart through a bed of blood vessels. With its help, you can conduct an examination and find out all the features of the pathology.
Echocardiography is a safe method of examination using ultrasound. Detailed information about the internal structure and deviations from the norm. Shows hemodynamic parameters.
Radiography - the procedure involves a picture of the chest. On it you can see a change in the contours of the heart, an increase in its size.

The following video will give a more detailed picture of what the Eisenmenger symptom is, and which diagnostic method to choose:

Treatment

Properly organized care for this pathology is the correction of abnormal places at an early age by surgery. If the disease has revealed itself at a later age, then the correction should be done as quickly as possible.

In adulthood, as a result of complications, an irreversible situation may occur that requires extremely radical solutions. If the patient is not ready for this, then it is possible to maintain the condition with therapeutic and medical methods in order to prolong the patient's life and improve its quality as much as possible.

Therapeutic way

The patient receives advice on how to maintain the condition in order to avoid deterioration.

Such patients are not shown:

to be in highlands,
take nonsteroidal drugs
immersion in cold water
dehydration,
overheating in the bath
suffer from acute respiratory infections,
pregnancy.

medication

If the syndrome has developed sufficiently and complications have appeared, then the patient is supported with drugs, depending on the problems that have arisen. Select the means for the patient to reduce pulmonary hypertension:

drugs that enhance nitric oxide;
endothelin antagonists,
prostacyclin antagonists.

Under the supervision of a physician, an increased number of red blood cells is tried with caution to correct by phlebotomy, while compensating the volume with saline.

Other

Early correction of pathological abnormalities in the structure of the heart involves correcting the position of the aorta and septal defect. To achieve the goal, closed (endovascular) and open surgical interventions are used. These actions can prevent the onset of a severe stage of the disease and make the patient's life full.

In the case when the disease is advanced, and complications have turned the process to an irreversible side, the situation can be saved by a heart and lung transplant operation at the same time. One of the options for surgical intervention is the correction of the septum in the heart and lung transplantation.

Sometimes, in order to maintain the patient's condition in an irreversible situation, highly qualified specialists undertake to perform some adjustment through an operation. An appointment for the installation of a pacemaker is also possible.

Disease prevention

Women need to plan pregnancy, consult with geneticists and check their health in advance.

Pregnant women should follow:

avoid contact with harmful chemicals,
if you need to take pills, consult your doctor;
not to be in places with dangerous ecology,
not be exposed to ionizing radiation,
do not use genetic products,
follow a healthy diet.

Complications

Congenital disorders must be corrected surgically in time. The disease, developing, creates more and more profound problems.

The main symptoms are added:

headache,
rachiocampsis,
frequent respiratory diseases,
pulmonary infarction,
lung bleeding,
chest deformity,
diseases associated with the formation of blood clots;

Forecast

In each case, life expectancy will depend on factors:

the severity of congenital anomalies,
whether an early adjustment has been made,
how bad the situation is.

Patients with this problem live 20 ÷ 50 years. The quality of life may be unsatisfactory, because physical activity will have to be limited. If the patient does not turn to specialists with this diagnosis for help, then his life span is limited from 20 to 30 years.

Among the numerous heart defects, there are combined types that are difficult to treat. One of them is Eisenmenger Syndrome.

With this pathology, the work of the heart muscle undergoes significant changes. The main aorta is in a mirror position, so the discharge of blood changes from left to right, develops, the right ventricle increases in size.

Eisinmenger's syndrome manifests itself mainly in the elderly, but such cases among children are also no exception.

The reasons

Eisenmenger's syndrome is a genetically determined disease. Among the causes and provoking factors of pathology, the following are distinguished:

The use by a pregnant woman of products with a high content of carcinogens, dietary supplements.
Frequent vibrations on the fetus, the effect of radioactive radiation.
We do not control the intake of drugs, in particular - diuretics ().
Severe toxicosis in the first and last trimester.
The genetic predisposition of the parents.
causing illness in a pregnant woman.

Eisenmenger's syndrome is white, in which the arteriovenous shunt of blood is not accompanied by cyanosis, and blue - with severe cyanosis of the skin.

When the normal blood flow changes, the mechanism of the hemodynamic processes is disturbed:

a veno-arterial shunt is formed;
mixing of blood occurs;
little oxygen enters the blood;
the walls of blood vessels thicken.

The main symptoms and signs of Eisenmenger's syndrome:

Enlarged jugular veins.
Prostration.
Deformation of the intervertebral discs.
Pinched intervertebral discs.
Fainting.
Protrusion of the chest.
Sharp pains in the abdominal cavity.
Speech becomes slow.
The density of the blood increases.
Frequent respiratory diseases, SARS.

At the same time, the mental and physical abilities of a person remain unchanged. Pain in the region of the heart resembles angina pectoris in intensity: it radiates to the left arm, shoulder blade, shoulder.

Patients with Eisenmenger's syndrome in its severe course can be identified by:

cyanotic color of the skin;
frequent breathing;
shortness of breath
secretions of blood mucus from the lungs.

In severe cases, it also occurs:

bacterial endocarditis;
there is pulmonary bleeding;
thromboembolism;

If patients ignore treatment, heart failure and death occur. The asymptomatic course of Eisenmenger's disease is dangerous for the development of sudden death.

Diagnostics

If a patient is suspected of Eisenmenger's syndrome, the doctor prescribes a number of examinations for diagnosis:

Electrocardiogram.
Chest x-ray.
Ultrasound of the heart.
Biochemical and.
CT of the lungs.
Cardiac catheterization.
Load test.

Treatment

Treatment of Eisenmenger's syndrome is conservative and surgical. But the disease cannot be completely cured. Patients with such a diagnosis should be regularly monitored by a cardiologist, constantly measuring blood pressure at home.

Preparations

To eliminate symptoms and the risk of complications, prescribe:

Medicines that relieve muscle tension.
Prostacyclins (to lower blood pressure).
Blood thinners.
Antiarrhythmic drugs.

Operation

In severe cases, heart and lung transplantation becomes the treatment of choice. Immunosuppressive and antiviral therapy will help improve the results of surgical treatment. With the development of life-threatening arrhythmias, it is shown.

Forecast

With the right lifestyle with a diagnosis of Eisenmenger's syndrome, the prognosis is favorable. You can live a long life if you follow certain prevention recommendations:

Patients usually lead an active lifestyle To prevent the development of hypoxemia, some restrictions are required. Experts recommend that patients avoid dehydration, frequent SARS, staying at altitude, abrupt immersion in cold water, drug use, prolonged exposure to the sun, hyperthermia, taking NSAIDs and some anesthetics.

Although primary PH is not directly related to CHD, children with this pathology are sometimes admitted to cardiac surgery clinics for differential diagnosis. Specialists repeatedly met patients with severe PH, which could not be associated with insignificant concomitant CHD. Cardiac surgeons are forced to take on advisory assistance in matters of not only diagnostics, but also therapeutic treatment of this difficult group of patients.

The first report of familial primary PH was made in 1927. Clarke et al described the clinical presentation and morphological findings on autopsy of primary PH in 5- and 8-year-old sisters. However, Dresdale et al. were the first to show familial transmission of the disease from one generation to the next. They described the case history of a family in which a woman and her son died of primary PH, respectively, at 43 and 21 years of age. In addition, her brother and sister died in early childhood at the age of 31 from right ventricular failure, probably due to primary PH. These early clinical descriptions contained many of the now well-established facts of familial primary PH, including vertical transmission, genetic prejudice, and the curious observation that in families, the clinical course of the disease is more severe in males and they die at a younger age than females.

The frequency of familial primary PH is 1-2 cases per 1 million population and 6% in the US registry of PH of various etiologies, although there is reason to believe that quite a few cases are not counted. Familial primary PH differs from the sporadic form in that it is diagnosed earlier after the onset of symptoms. However, it does not differ from sporadic either clinically or in terms of the ratio of women to men - 2:1 in adults and 1.3:1 in childhood.

Familial primary PH is transmitted vertically. So, a family is known in which 5 generations suffered from this disease. It can be passed from male to male, but a case has been reported at the Toronto Pediatric PH Clinic in which a healthy father had two daughters with primary PH from different mothers. This example of transmission excludes the X-linkage of genes and strongly suggests the presence of an autosomal dominant gene.

Histology

The histological features of familial pulmonary arteriopathy are heterogeneous and often combine thrombotic and plexiform lesions. Histologically, familial, sporadic primary PH, and Eisenmenger's complex are indistinguishable. Lee et al showed that plexiform pulmonary vascular lesions in familial primary PH contain monoclonal proliferating endothelial cells as opposed to polyclonal endothelial cell proliferation in secondary PH. The presence of monoclonal endothelial cell proliferation in primary PH indicates that somatic gene damage, similar to that in neoplastic processes, may contribute to clonal expansion of pulmonary endothelial cells. In primary PH, histological examination sometimes reveals occlusion of pulmonary venous microvessels and capillary hemangiomatosis.

Clinic

The etiology of primary pulmonary arterial hypertension is unknown. It affects predominantly young people, and the course of the disease is inexorably fatal, although isolated cases of spontaneous regression have been recorded. The diagnosis is established already at an early age, usually in advanced stages of the disease. The average life expectancy is 4 years. An important determinant of survival is right ventricular function. The life prognosis is better in patients with right atrial pressure less than 7 mm Hg. Art. A bad harbinger is low oxygen saturation of mixed venous blood. Children respond better to vasodilators than adults. The positive hemodynamic effect of treatment improves the prognosis, but not in everyone. According to lung biopsy in children, medial hypertrophy is more pronounced, which explains the tendency to vasoconstriction, and angiomatous changes and intimal fibrosis are less pronounced.

Pulmonary vascular hypertension in the absence of intracardiac shunts is poorly diagnosed in childhood, since objective symptoms are not pronounced. The most typical signs are:

fainting or semi-consciousness;

generalized convulsions;
palpitations or cyanosis during exercise;

swelling in the legs.

Load intolerance is always noted. Pain in the heart for children is atypical, unlike adults. However, myocardial ischemia can also occur in children when the pressure in the pulmonary artery exceeds the systemic pressure, as well as during exercise.

The characteristic features of PH are seen on a chest x-ray:

expansion of the heart shadow;

bulging of the second arc along the left edge of the heart;

expansion of the proximal pulmonary arteries with "chopped off" peripheral branches.

The electrocardiogram shows hypertrophy of the right atrium and ventricle with signs of overload in 70-80% of patients.

Echocardiography allows diagnosing an increase in pressure in the right ventricle, the absence of pathology of the mitral valve and pulmonary veins, as well as the absence of other probable causes of right ventricular hypertension - subvalvular, valvular and supravalvular stenosis of the pulmonary artery. The presence of narrow proximal pulmonary arteries with constant distal flow on Doppler cardiography indicates multiple peripheral pulmonary artery obstructions. Extra- and intracardiac shunts should also be excluded.

Cardiac catheterization and angiocardiography are the most important studies for establishing the correct diagnosis.

Treatment of primary PH

Until the last 10 years, conventional therapy was mainly symptomatic and limited to digoxin, diuretics, calcium channel blockers, and anticoagulants. However, the latest advances in vascular biology and molecular genetics are rapidly being introduced into practice in the form of etiopathogenetically substantiated treatment.

Calcium channel blockers

In 1992, Rich et al showed that large doses of calcium channel blockers reduced pulmonary artery pressure and resistance by more than 20% in 26% of patients with primary PH. With oral administration of nifedipine or diltiazem, patients showed a 94% survival rate for 5 years and signs of regression of right ventricular hypertrophy, improved exercise tolerance and quality of life. However, in that part of the subjects in whom the decrease in PVR was not accompanied by a decrease in pressure in the pulmonary artery, there was no decrease in symptoms during long-term therapy. It has been noted that calcium channel blockers may exacerbate right ventricular failure and should be used with caution. Calcium channel blockers are only effective in a small proportion of patients and have been superseded by newer drugs.

Vasoctive mediators and pharmacological treatment

As mentioned above, prostacyclin is an endogenous vasoactive mediator that promotes vasodilation, inhibition of platelet aggregation, and proliferation of vascular smooth muscle. Thromboxane has the opposite effect and worsens the course of pulmonary vascular disease. The ratio of prostacyclin to thromboxane is reduced in primary PH, the Eisenmenger complex, and in children with a left-to-right intracardiac shunt, and returns to normal after successful correction of the defect.

Prolonged infusion of prostocycline

Higenbottam et al. were the first to report the beneficial effects of continuous prostocycline infusion in patients with primary PH. There was an improvement in well-being, exercise tolerance and survival. Subsequently, these results were confirmed by other studies. The one-year survival rate of patients awaiting heart and lung transplantation increased by 66%. Interestingly, after 2 years of such treatment, no advantages over conventional therapy were noted, with the exception of more severe stages of the disease. In these patients, the long-term effect was not associated with the vasodilation that occurs at the beginning of a course of prolonged infusion of prostocycline. The effect is explained by mechanisms other than vasodilation, namely, inhibition of platelet aggregation and remodeling of the vascular wall.

Side effects in the form of headache, redness of the skin and abdominal pain are usually transient, lasting for 24 hours, but may reappear with an increase in dose.

Complications are mainly associated with a long standing venous catheter, pump malfunction. On average, a patient has two episodes of sepsis per year. If the infusion is interrupted, shortness of breath and loss of consciousness may occur. Over time, the need for prostacyclin and the need for dose adjustments to maintain normal cardiac output increases. However, prolonged intravenous administration of prostocycline significantly improves survival at 1, 2, and 3 years and is 88%, 76%, and 63%, respectively, which is significantly better than controls.

The leading factors determining the survival of patients are:

tolerance for physical activity;

functional class MUNA;

pressure in the right atrium;

direct vasodilating response to adenosine or inhaled NO.

After a year of treatment, cardiac output and mean pulmonary artery pressure become additional prognostic factors.

Continuous intravenous administration of prostocycline has revolutionized the chronic management of PH. However, the above disadvantages and complications are particularly burdensome in the treatment of children. Not surprisingly, many patients are reluctant to make decisions about such treatment. This stimulates the search for alternative methods of administration of prostocycline - aerosols, oral or subcutaneous analogues. Beraprost is an orally active prostocycline analogue that has been shown to be effective in both short-term and long-term treatment of PH. The efficacy of the oral analogue is comparable to that of intravenous prostocycline and is also maintained for 1 year. Adverse reactions - facial flushing, arthralgia, muscle pain, nausea or diarrhea - were noted quite often, however, severe catheter-related complications were excluded.

The inhaled aerosol form of prostocycline is comparable in efficacy to inhaled NO, however, their combination does not provide an additional answer. More favorable is the combination of inhaled iloprost with oral drugs such as bosentan or sildenafil.

Recent studies have opened up a promising alternative to the continuous intravenous administration of prostocycline in PH. The transfer of the human prostocyclin synthase gene to the liver of rats with monocrotaline-induced PH made it possible to achieve a high level of expression of the prostocyclin synthase gene in animal liver hepatocytes. As a result, the pressure in the pulmonary artery decreased from 88% to 60% relative to the systemic one, and the content of ET-1 in the lung tissue decreased by 2 times compared to the control. The survival rate of animals has increased significantly.

Inhalation NO

Inhaled NO is an instantaneous selective pulmonary vasodilator that improves intrapulmonary shunt fraction and has a short half-life. It is ideal for performing functional tests during catheterization and in neonates with persistent PH, on mechanical ventilation in intensive care units, and in children after CHD surgery. It should be noted that, despite oxidative stress, manifested by increased lipid peroxidation in patients with PH, inhaled NO does not contribute to a further increase in the formation of peroxynitrites. Nitric oxide has become the method of choice in the treatment of pulmonary hypertensive crises in pediatric cardiac surgery. However, serious technical difficulties limit its practical application for the long-term treatment of patients with chronic PH.

Sildenafil

Sildenafil is a selective inhibitor of V-phosphodiesterase, which is an enzyme that degrades cGMP and thus limits NO-mediated vasodilation. The effect of inhibition of phosphodiesterase on the vessels of the penis and its use in the treatment of erectile dysfunction is well known. It is also known that there are high concentrations of the type V enzyme in the pulmonary vessels. Preliminary reports have shown that sildenafil may have a vasodilating effect in PH, in particular, attenuate the acute rise in pulmonary artery pressure after NO inhalation is stopped, and may also be used as a drug for the treatment of chronic PH. Oral sildenafil abolishes hypoxic pulmonary vasoconstriction in humans. Sildenafil is well tolerated, available as an oral drug, and may be an alternative to prostacyclin, especially for patients whose symptoms do not justify continuous intravenous infusion. Sildenafil can act as an adjuvant in treatment with inhaled prostocycline or in combination with continuous inhaled NO. Sildenafil causes rapid and relatively selective pulmonary vasodilation, which is maintained for a sufficiently long time. The synergistic and additional action to prostocycline is due to an increase in the content of cAMP and cGMP. Interestingly, inhaled sildenafil reduces intrapulmonary shunting in animal studies and oral sildenafil reduces intrapulmonary shunting in patients with pulmonary fibrosis and secondary PH. Sildenafil is a selective pulmonary vasodilator, in contrast to other intravenous and oral drugs of the same purpose.

Blockade of ET receptors

Endothelin is a powerful vasoconstrictor that promotes the proliferation of vascular smooth muscle. There is evidence that an abnormally high level of circulating ET deepens vascular disorders in the lungs. Elevated ET, accompanied by a decrease in NO synthesis, is implicated in the pathophysiology of PH that occurs after cardiopulmonary bypass, persistent neonatal PH, and Eisenmenger's syndrome. Chronic prostocycline therapy in patients with primary PH improves pulmonary clearance of ET along with hemodynamic and clinical parameters. The action of ET is mediated through two types of receptors - ETA and ETP. ETA is present on smooth muscle cells and mediates vasoconstriction and proliferation, while the ETP receptor is found predominantly on endothelial cells. When ET binds to the ETV receptor, it causes vasorelaxation through the release of NO and prostocycline. This explains the paradox found in early work, in which ET infusion in healthy mammals caused pulmonary vasodilation even at doses that would normally result in systemic vasoconstriction. These data led to the idea of an important role of endothelial cells in maintaining pulmonary vascular homeostasis. It is possible that ETA receptors predominate in the damaged pulmonary vascular bed. While it remains unclear, pharmacological agents should act on the ETA or ETV receptors. Non-selective blockade of ET receptors may reduce the beneficial effects of ET. However, the most promising ET receptor blocker acts on both types of receptors. Intravenous administration of bosentan reduces pulmonary artery pressure and resistance in patients with primary PH, but this effect is not selective. Despite the non-selective effect of intravenous bosentan, the tablet form of the drug in two placebo-controlled studies in patients with primary and secondary PH due to scleroderma improved physical performance, hemodynamics and alleviated symptoms.

In addition to the vasodilating effect, bosentan inhibits the development of fibrosis and proliferation. Its use contributes to an increase in life expectancy. According to data published in 2005, 86% of patients treated with bosentan survived the 3-year period, compared to 48% in the comparison group. The advantage of the drug is also its oral administration, eliminating the difficulties and complications of parenteral administration.

The drug is well tolerated, has no side effects, except for a dose-dependent increase in the level of pulmonary enzymes, which decreased to normal after 2-6 weeks. after its cancellation.

Anticoagulants

Follow-up of a group of patients for 15 years showed better survival of patients treated with warfarin compared to those who did not receive it. There is histological evidence for a role for vascular thrombosis in primary PH.

Knife atrioseptostomy

It is known that patients with Eisenmenger's syndrome and an open foramen ovale live longer than patients with an intact atrial septum. Taking this observation into account, a number of cardiologists have reported that they perform knife septostomy in patients with severe forms of PH.

Animal studies and experience with Fontan fenestration procedures show that atrial communication provides decompression of congested right hearts and maintenance of cardiac output at the cost of a drop in arterial oxygen saturation, while improving systemic oxygen delivery and reducing symptoms of right ventricular failure. Knife atrial septostomy increases cardiac output and systemic oxygen transport despite a drop in arterial oxygen saturation. Survival at 1, 2, and 3 years was 80%, 73%, and 65%, respectively, which is significantly better than the predicted survival curve derived from the New York Heart Association Primary PH Registry Equation.

Knife atrioseptostomy improves the condition of patients with syncope. The procedure involves some risk. Volume loading, elevated hematocrit, and inotropic support are recommended to prevent early mortality in the perioperative period. From a technical point of view, a gradual, in several steps, balloon dilatation of the septostomy is safer.

Lung transplant

Despite advances in the understanding of PH, lung transplantation is the last resort for patients who have exhausted treatment options. The number of children who have undergone transplantation is still small. The survival rate of children during the year is 73%. Mortality after transplantation is due to four factors:

cytomegalovirus infection;

obliterating bronchiolitis;

post-transplant lymphoproliferative disease;

bronchial stenosis.

Ten-year survival in children is 30-40%.

The timing of transplantation remains ambiguous. With effective drug therapy, expectant tactics are followed. Indications for transplantation are right ventricular failure or MUNA class IV when life expectancy is less than 6 months. Lack of effect from vasodilatory therapy, suprasystemic pulmonary artery pressure, syncope, or low cardiac output are signals to the transplant team. Simple quantitative hemodynamic criteria for survival in the natural course of the disease have also been developed. If the mean right atrial pressure times the PVR index is less than 160, survival is better than after lung transplantation.

Persistent PH in newborns

Persistent pulmonary hypertension of the newborn occurs in 1 in 1500 live births and is characterized by persistent PH and cyanosis due to a right-to-left shunt through the PDA or patent foramen ovale. There is no heart defect.

The causes of PLGN are divided into 3 groups depending on the structure of the pulmonary vascular bed:

Pronounced pulmonary vasoconstriction with a normally developed pulmonary vascular bed. May occur with perinatal asphyxia, meconium aspiration, circulatory shock, streptococcal pneumonia, increased blood viscosity, hypoglycemia, and hypocalcemia. Alveolar hypoxia and acidosis, vasoactive agents - thromboxane, vasoconstrictive prostaglandins, leukotrienes, endothelin play an important role in the mechanisms of vasocontraction.

Hypertrophy of the media of the pulmonary arterioles may develop with chronic fetal hypoxia, may be a consequence of the mother taking non-steroidal anti-inflammatory drugs during pregnancy.

Underdevelopment of the pulmonary arteries, accompanied by a decrease in the cross section of the pulmonary vascular bed in congenital diaphragmatic hernia and primary pulmonary hypoplasia.

Pulmonary hypertension of a functional nature is easily reversible when the causes that caused it are eliminated: PH of the 2nd group requires intensive treatment; Group 3 PH is usually irreversible.

PLGN is accompanied by a decrease in myocardial contractility and tricuspid insufficiency due to general or subendocardial myocardial ischemia. Hypoglycemia and hypocalcemia increase mocardial hypoxia.

Clinical manifestations in the form of cyanosis, tachypnea, groaning breathing with retraction begin 6-12 hours after birth. Complications in childbirth, anamnestic data on the mother taking non-steroidal anti-inflammatory drugs in the third trimester, help to suggest PLGN.

Characterized by an increased heart beat, a loud unsplit II tone, a gallop rhythm, a soft systolic murmur of tricuspid insufficiency, and in severe cases, hypotension.

Saturation of arterial blood in samples obtained from the umbilical artery is reduced with normal saturation in the preductal arteries. Sometimes there is a difference in the color of the upper and lower half of the body. With a large discharge of blood from right to left through the open oval window, there is no difference in saturation in the upper and lower arterial pools.

The ECG is usually normal for age, sometimes there is right ventricular overload or an abnormal T wave, indicating myocardial dysfunction.

On the radiograph, cardiomegaly, enhanced pulmonary pattern, atelectasis can be detected. However, these signs may be absent.

Echocardiography showed no signs of cyanotic malformation. The only find is a large PDA with right-left or bi-directional discharge. The right ventricle is dilated, the atrial septum bulges to the left, there is an open foramen ovale. The aortic arch is normal, with no evidence of aortic coarctation or rupture of the aortic arch. The left ventricle may be enlarged, with a reduced ejection fraction.

Catheterization is not usually indicated, but if the diagnosis is unclear or the patient is refractory, catheterization and arteriography are performed to avoid misdiagnosis.

Treatment has 3 goals:

decrease in PVR and pressure in the pulmonary artery by inhalation of oxygen, the creation of respiratory alkalosis and the use of pulmonary vasodilators;

correction of myocardial dysfunction;

stabilization of the patient and treatment of concomitant pathology.

Carry out general maintenance therapy: correction of hypoglycemia, hypocalcemia, hypomagnesemia, polycythemia. Body temperature is maintained within 36.5-37.2 0 С.

To achieve arterial pO 2 100 mm Hg. Art. carry out inhalation of 100% oxygen without intubation. If there is no effect, intubation is carried out with the creation of a positive airway pressure of 2-10 cm of water. Art. with spontaneous breathing.

If these measures are ineffective, mechanical ventilation is carried out to improve oxygenation and achieve respiratory alkalosis. The following ventilation mode is used: 100% oxygen concentration, respiratory rate 40-80 per minute, inspiratory pressure 40 cm of water. Art., positive expiratory pressure 4-10 cm of water. Art., the ratio of inhalation-exhalation time 1:1. The patient is relaxed. Upon reaching normal saturation of arterial blood with oxygen within 12-24 hours, a gradual weaning from the apparatus is carried out.

Vasodilators, as a rule, are nonspecific and dilate not only pulmonary, but also systemic resistive arterioles, so they have not been used recently.

The most effective method aimed at reducing PVR is the addition of small doses of gaseous NO, a selective pulmonary vasodilator, to the respiratory mixture. This effective method, widely used in Western medical institutions, has not yet been introduced in Ukraine and is at the stage of clinical testing.

Treatment of heart failure is carried out using conventional means: dopamine at a dose of 10 mg / kg / min by intravenous administration, dobutamine p-adrenergic agent at an initial dose of 5-8 mg / kg / min by continuous intravenous administration, digoxin for chronic congestive heart failure on later stage, diuretics.

Correction of acidosis, hypocalcemia, hypoglycemia helps to improve myocardial function.

In the arsenal of leading Western clinics in some severe cases of PLGN there is such an aggressive method as extracorporeal membrane oxygenation. However, the introduction of NO inhalation has limited its scope.

Forecast

With moderate PLGN, therapeutic actions are usually effective and the prognosis is favorable. Most newborns recover without pulmonary or neurological disease. Among patients requiring prolonged ventilation, survival is worse, bronchopulmonary dysplasia and other complications develop. With underdevelopment of the pulmonary vascular bed, patients are resistant to treatment and their prognosis is poor. Many show signs of underdevelopment of the central nervous system, the frequency of hearing loss is high. These complications are directly related to the degree of alkalosis, the duration of ventilation, the use of furosemide and aminoglycosides. In 80% of patients, there are deviations in the encephalogram and in 45% - cerebral strokes.

Eisenmenger syndrome in children

In 1897, Eisenmenger described the pathological findings in a 32-year-old man with a large VSD and PH. It was not until 60 years later, in 1958, that Wood gave a definitive definition of the disease that is consistent with our understanding of this clinical syndrome today. Wood used the term "Eisenmenger's syndrome" to describe patients with systemic pulmonary artery pressure due to high PVR and a right-to-left or bidirectional shunt at the level of the great vessels, the interventricular or interatrial septum. The term "Eisenmenger complex" is used when the underlying malformation is VSD.

With the development of cardiac surgery at an early age, the incidence of Eisenmenger's syndrome decreases. It is determined by the level of culture of the population and primary health care. Eisenmenger's syndrome occurs even at the age of 2 months.

Clinic

Typical signs of the disease are cyanosis, polycythemia, right ventricular failure. In patients with complex congenital malformations - OSA, AVSD, univentricular atrioventricular junction and transposition - the symptoms of Eisenmenger's syndrome develop early and worse prognosis. In patients with trisomy 21, the disease is also severe. Usually, symptoms progress slowly and are pronounced in adolescence and in adults. All patients have reduced physical performance.

Cyanosis first appears on exertion and then becomes permanent, reflecting the magnitude of the right-left shunt. Saturation of arterial blood with oxygen is 80-85%. A constant symptom in cyanotic patients is a thickening of the terminal phalanges of the fingers in the form of drumsticks. Hypertrophic osteoarthropathy may progress with arthralgia and articular synovitis.

Arterial hypoxemia is the cause of erythrocytosis. An increase in hemoglobin increases the oxygen capacity of the blood. The increase in blood viscosity associated with polycythemia does not manifest itself until the hemoglobin level does not exceed 18-20 g / l. Symptoms of increased blood viscosity:

headache;

dizziness;

visual impairment due to occlusion of the central retinal vein.

An increase in blood viscosity is a risk factor for thrombosis and cerebral hemorrhage. Due to thrombocytopenia, prolongation of clotting time, deficiency of coagulation factors and fibrinolysis, patients are prone to bleeding during surgery and tooth extraction. In 20% of patients, pulmonary hemorrhages occur as a result of ruptures of the bronchial arteries or aneurysms of the pulmonary arteries, which develop as a result of progressive dilatation of the central pulmonary arteries.

The cause of hemoptysis can be embolism and thrombosis of dilated pulmonary arteries.

Often observed uremia is due to increased production and reduced renal clearance of uric acid. Gout develops in 13-23% of patients. Increased erythropoiesis and destruction of erythrocytes leads to bilirubinemia and an increase in bilirubin in the bile, so cholelithiasis and cholecystitis are observed in 15% of patients. In 65% of patients, kidney dysfunction with proteinuria and the development of nephrotic syndrome with an increase in serum creatinine are noted. This serves as an additional factor that reduces survival.

Cerebral complications are characteristic: a stroke at the age of about 30 years and brain abscesses at 20-25 years. Rhythm disturbances in the form of supraventricular and ventricular extrasystole, flutter and atrial fibrillation are not uncommon. Every fifth patient has fainting and presyncope conditions associated with ventricular tachycardia.

Endocarditis occurs with a frequency of about 4%. Some patients present with hoarseness and cough associated with laryngeal nerve compression, dilated pulmonary arteries. Dilated pulmonary arteries can compress the left coronary artery with anginal pain. 30% of patients die suddenly. Although the presence of a shunt prolongs the life of patients with Eisenmenger's syndrome compared with patients with primary PH, 40-50% of them die from heart failure. The latter is especially common with complex underlying defects and is due to stenosis or insufficiency of the atrioventricular or semilunar valves.

Patients with Eisenmenger's syndrome need qualified management. About 20% of deaths are due to avoidable mistakes. Non-cardiac surgery is responsible for 24% of deaths. Venesections must be performed with caution. Patients should be warned about the risk of pregnancy, staying at altitude, taking estrogen, anesthesia.

The use of vasodilators and anticoagulants should be carried out under qualified supervision, taking into account the balance between pulmonary and systemic vascular resistance and the risk of bleeding and thrombosis. Pregnancy outcomes are usually unfavorable:

spontaneous abortion or preterm birth occurs in 25%;

therapeutic abortion - in 27%;

prematurity or low weight of the child - in 26%;

maternal death - in 16%;

deterioration of the mother's condition - in 54% of patients.

Conservative treatment is ineffective. However, recently there have been reports of partial preservation of pulmonary vascular reactivity and some regression of advanced OBLS, which has renewed interest in treatment with the latest generations of vasodilators. Gorenflo et al conducted a comparative study of the effectiveness of various vasodilators in children with CHD, PH and an average Wood index of 10 U/m 2 . Pulmonary artery pressure and PVR decreased in response to oxygen inhalation in 2 of 14 patients, to NO inhalation in 4 of 14 patients, and to intravenous administration of prostacyclin additional to NO in 2 of 7 patients. Oxygen inhalation did not affect the level of vasoactive mediators. Nitric oxide at a dose of up to 80 ppm increased the cGMP level by an average of 2 times, but there was no relationship between the cGMP level and the hemodynamic response.

Rosenzweig et al used long-term prostacyclin infusion to alleviate the symptoms of Eisenmenger's syndrome and showed a 20% reduction in mean pulmonary artery pressure, an increase in cardiac index from 3.5 to 5.9 L/min/m 2 , an improvement in functional class from 3.2 to 2.0, increased exercise capacity and oxygen delivery, but arterial oxygen saturation did not increase.

Closing of the VSD after pre-narrowing of the pulmonary artery in Eisenmenger's syndrome was first described in 1971 by Azzolina and has generated a lot of debate. The regression of medial hypertrophy and intimal proliferation after pressor unloading of the pulmonary vessels were well documented experimentally and in the clinic, but it remained unclear whether far-reaching changes such as intimal fibrosis, fibrinoid necrosis, or plexiform lesions after narrowing of the pulmonary artery undergo regression. Interestingly, PVR may decrease after ductus arteriosus closure despite plexiform arteriopathy.

Nowick and co-authors proposed to close the VSD in patients with high pulmonary vascular resistance with a double patch-valve with a hole to ensure right-left shunting of blood when the pressure in the right ventricle rises above the systemic one. In 18 operated patients, vascular resistance averaged 11.4 U/m 2 , and all had cyanosis despite a predominantly left-to-right shunt. The publication did not provide data on pulmonary vascular reactivity, so it is difficult to assess the severity of obstructive vascular disease in these patients.

Lung transplant

Lung transplantation is rarely used in children with Eisenmenger syndrome. The results of these interventions in children and adults with CHD and PH are identical. Hospital mortality is 23%, 5-year survival - 57%. Lung transplantation without a heart transplant is possible in patients with ASD and PDA. In Eisenmenger complex, survival is better if the heart and lungs are transplanted at the same time, as opposed to lung-only transplantation and closure of the VSD. In adults, 1-, 5-, and 10-year survival rates are 73%, 51%, and 28%, respectively.

The obvious progress in medicine over the past few years has not led to a dramatic increase in life expectancy or the elimination of heart disease. In addition, at the turn of the 19th-20th centuries, scientists described many new diseases. The causes of some of them are still a mystery, the symptoms are blurred, and treatment is very difficult. named after an Austrian pediatrician and cardiologist, cannot be considered common. A rare pathology received a detailed description about 100 years ago. However, even today it is not necessary to talk about a clear understanding of its etiology.

general information

The Eisenmenger syndrome (sometimes called the complex) is a very dangerous pathology that spreads to the pulmonary and cardiac systems. Cardiological disease is characterized by combined and hypertension. The development of pathology leads to an increase in pressure and failures in the pulmonary circulation. As a result, a shunt is formed, which is responsible for the injection of blood from right to left, which disrupts the normal hemodynamic processes.

Also, the concept of Eisenmenger's syndrome includes any that differ in an open atrioventricular canal and arterial duct, the presence of only one ventricle. Among patients with anomalies of the interventricular septum, pathology is diagnosed in 10% of cases. In the structure of congenital defects of the main muscle of the body, the Eisenmenger complex is 3%.

Causes of the syndrome

Doctors cannot name a single reason for the development of pathology. However, there are a number of etiological factors that increase the likelihood of having children with Eisenmenger syndrome:

hereditary predisposition. Genetic disorders can be passed from parent to child, making them more likely to develop heart defects.
Environmental factors:

fetal intoxication during fetal development;
prolonged exposure to electromagnetic radiation;
chronic infectious diseases of one of the parents;
side effects from drugs and dietary supplements used by a woman during pregnancy.

The listed factors are not typical for Eisenmenger's syndrome, but theoretically they can cause the appearance of various pathologies, including this one.

How does the disease manifest itself?

Patients with Eisenmenger's syndrome usually do not complain of health problems. Therefore, early diagnosis is not always possible. The child's body first connects compensation mechanisms, but after a while the disease manifests itself. Internal resources become thinner, the cardiac system fails. What are the symptoms of Eisenmenger's syndrome?

Increased weakness, aggravated after physical exertion.
Pain in the left side of the chest.
Increasing daytime cyanosis of the skin.
Spontaneous fainting.
Coughing fits with bloody discharge.

These symptoms are not for everyone. The syndrome can develop unnoticed for a long time. If the pediatrician who observes the child recommends undergoing an additional examination at the cardiology center, they should not be neglected.

in case of illness

Russian doctors are sure that Eisenmenger's syndrome and pregnancy are incompatible. Therefore, at any time a woman is recommended to have an abortion. Their Western counterparts think differently. In their opinion, safe childbirth depends on the observance of a number of rules and recommendations.

First of all, a woman is shown hospitalization for the entire duration of pregnancy. In the hospital, doctors must constantly monitor the condition of the fetus and measure the pressure in the pulmonary capillaries. At the first sign of shortness of breath - oxygen masks. Starting from the second trimester, treatment with coagulants is prescribed. A few days before the expected date of birth, shock therapy with glycosides is recommended to maintain the cardiac system. Childbirth is possible only in a natural way.

If a woman follows all the prescriptions of doctors, the probability of a positive outcome for the child is 50-90%. The prognosis for the mother is not so favorable. That is why, when deciding whether to continue a pregnancy, a woman should think about the potential risks.

medical examination

Effective therapy for patients with Eisenmenger's syndrome cannot be imagined without a special examination. It can be passed only in specialized clinics with the appropriate equipment. If this pathology is suspected, the examination program usually includes:

chest x-ray (assessment of changes in the contours of the pulmonary artery and heart);
angiography (examination of blood vessels);
ECG (detection of hidden cardiac arrhythmias);
EchoCG (visualization of pathological changes in the walls of the ventricles);
cardiac catheterization (assessment of the course of the disease and the nature of the damage, measurement of the numerical parameters of the work of the main muscle).

Differential diagnosis is mandatory in all patients with suspected Eisenmenger's syndrome. Pentade of Fallot, arterial stenosis, cleft ductus arteriosus is a short list of pathologies to exclude.

Principles of therapy

Conservative treatment of patients is possible, but not effective enough. On the other hand, the use of surgical intervention does not always give positive results. Therefore, today doctors are increasingly resorting to a combined strategy.

Patients with a pronounced clinical picture and increased hematocrit are prescribed phlebotomy. The procedure is repeated no more than three times a year, while it is necessary to control blood iron levels. Oxygen therapy and anticoagulant treatment are used extremely rarely, rather, optionally. The main disadvantage of such procedures is a large number of side effects, the most significant of which is also patients are prescribed medications. "Epoprostenol" and "Treprostinil" improve hemodynamics, and "Traklir" reduces vascular resistance.

Surgical intervention is carried out in two versions: implantation of a pacemaker, plastic surgery of the MPD defect. With Eisenmenger's syndrome, surgery can significantly improve the prognosis of the disease. If the listed methods of treatment are ineffective, only the simultaneous and heart can help. The transplant operation is extremely complex and requires large financial costs. On the other hand, it does not eliminate the risk of complications. When there is no other solution, the material issue does not cause difficulties, a double transplant can save a person's life. Before it is carried out, it is necessary to undergo a complex medical examination.

How do patients with Eisenmenger's syndrome live?

People who have had to deal with this cardiac disease often lead active lives. However, they are forced to constantly visit a doctor and monitor health indicators. Such patients should avoid dehydration, prolonged exposure to altitude, and infectious diseases. It is important to give up addictions and medications that can cause bleeding. If the patient follows the doctor's instructions, the likelihood of being able to lead a normal life increases. Otherwise, the level of oxygen in the blood can drop to critical levels, which will lead to death.