Angina on ECG decryption example. Unstable angina (i20.0)

The syndrome or the Eisenmenger complex is one of the congenital heart defects. The defect is quite rare - the Eisenmenger syndrome in children is about 4-5% of all congenital pathologies of the heart and blood vessels. Most often develops before the child reaches puberty. Nevertheless, there are cases when the complex is developing in adolescence and progresses throughout the young age.

In many respects, the Eisenmenger syndrome is similar to a group of heart defects highlighted by Etienne-Louis Arthur Fallo - Triad, Tetrad and Pentad Fallo. It differs from them the absence of the narrowing of the pulmonary artery.

The syndrome or the Eisenmenger complex is one of the congenital heart defects.

Etiology and physiology

The most common cause of the development of the Eisenmen german syndrome is the formation of holes (shunt) between two heart cameras. Such a hole may be congenital or artificially created during the surgical operation. The shunt provokes an abnormal blood circulation not only in the heart, but also the lungs. Arterial and venous blood is mixed. Instead of entering the body, the blood returns to the lungs. Because of this, hell is rising in the lungs. The vessels become rigid and gradually thinned, which can damage the small vessels. This leads oxygen starvation in all organs and tissues, the heart has to work in a faster pace.

Other heart pathology, which can lead to the development of the complex:

• open atrioventricular channel;
• cyanotic vices of the heart (state with increased pulmonary blood flow);
• total arterial trunk.

Heredity also increases the risk of a child's birth with this pathology.

Cardiovascular diseases

The physiological progress of the Eisenmenger syndrome is represented by the spectrum of changes in the structure of the bloodstream (the totality of the cavities of the heart and blood vessels), which can be reversible or irreversible.

Cases of Eisenmengeneger's syndrome are possible even in cats and dogs. Unfortunately, the animal, in most cases, quickly dies.

Symptoms

Often the disease flows asymptomaticly and is revealed already in late time when it is seriously treatment. Therefore, you should not neglect preventive medical examinations. Regular examinations at a cardiologist can help while revealing a serious and rare disease.

Signs of the Eisenmenger complex:

• dizziness;
• pathological fatigue, weakness;
• dyspnea;
• heart palpitations;
• arrhythmia;
• feeling of compression, pain in the left side of the chest;

One of the symptoms of this ailment is arrhythmia

• frequent kola attacks;
• fainting;
• increased sweating;
• swelling of the abdominal region;
• numbness and tingling in the fingers and hands;
• blood expectoration;
• eveniness in the joints of the joints.

Unlike a group of diseases that have highlighted Fallo, cyanosis (sinusia), fingers in the form of drum sticks and nail plates in the form of watch glasses occur much later. This is due to the fact that pulmonary artery in this case does not narrow.

You can distinguish two periods of development of the disease:

• I - without cyanosis;
• II - with cyanosis.

In the first stage, the saturation of peripheral arterial blood oxygen is not impaired, or violations affect the body only slightly. This period can occur without symptoms or with a lubricated picture. In the second stage, the saturation of blood oxygen is significantly reduced, the symptoms of the disease appear obviously.

In case of incorrect treatment, infarction can happen

If the patient does not turn out to be proper therapeutic assistance, complications arise:

• embolism;
• deficiency in the organism of iron;
• chronic heart failure;
• gout;
• heart attack;
• stroke;
• inflammation of the endocarda of bacterial nature;
• brain hemorrhage;
• secondary polycythemia;
• increased blood viscosity (due to the ingress of cells of destructive vessels);
• renal failure;
• infectious processes in the brain;
• breaking blood circulation of all organs, including the dorsal and brain;
• sudden fatal outcome.

With incorrect medical care may occur renal failure

Diagnostics

Already with superficial examination, the specialist will reveal a violation of the rhythm of heart abbreviations. Additional examinations are needed to confirm the diagnosis:

• Ehoche;
• radiograph of the chest;
• cardiac catheterization.

Since patients have strong changes in blood pattern, very important for diagnostics will be indicators of general blood analysis with hematroritic number (the ratio of blood elements). As a result of the study, polycythemia can be detected, and the hematocrit rate will exceed 60%.

The most frequent differential diagnoses: arterial stenosis, Pentada Fallo, the absence of the flow of the artery.

Treatment

Sometimes patients remove a certain amount of blood from the body (phlebotomy, earlier bloodletting). To reimburse the volume of lost liquid, physiological solutions are introduced.

Patients can remove a certain amount of blood from the body - phlebotomy

The patient can prescribe oxygen, although it is not currently established whether this procedure helps prevent the deterioration in the course of the disease. In addition, sedative and vasodilators are prescribed.

Surgical treatment is very complex: during surgery, it is necessary to eliminate the interventricular septum defect and the aortic possible anomaly. Almost always the implantation of the artificial heart valve is required. Due to the complexity of operational intervention, in some cases, the procedure is extremely dangerous and can lead to the patient's death.

In severe cases, in order to save the life of the patient, you may need a heart and lung transplantation.

Forecasts

Forms with early cyanosis occur extremely unfavorable. Without surgery, sick children die without living up to mature age.

If the disease is detected to the development of cyanosis, the forecast is doubtful favorable. After the occurrence of sinushia, even when providing good medical care, patients usually do not live more than 10 years.

Patients with syndrome can live from 20 to 50 years. The course of the disease depends on the presence of other pathologies in the body, as well as age. As you know, arterial pressure indicators increase with age, which has a negative impact on the health status of patients with the Eisenmenger complex.

In general, predictions with this disease are better than with other vices with cyanosis.

Despite the fact that progress in the world of medicine is clear today, still heart pathologies are among the first, significantly reduce the quality and life expectancy of a person. In addition, doctors open and relatively new types of heart disease. One of them is the Eisenmenger Syndrome, which opened an Austrian cardiologist and pediatrician Viktor Eisenmenger. Pathology is quite rare and not fully studied. The article below we will analyze what is the Eisenmenger syndrome, what is his clinical picture and how to treat pathology in modern cardiology.

What is the Eisenmenger Syndrome?

Regarding Eisenmengeneger's disease, two terms are used - a complex and syndrome. The term "complex" means precisely the disease itself, and the term "syndrome" implies a symptom complex (combination of symptoms) in a patient.

The Eisenmenger complex itself is a congenital abnormal structure of the heart, in which the interventricular partition has extensive and high defects. In addition, the patient has the right heart chamber and some abnormal debit of aorta in the zone of both heart cameras. The code of pathology on the ICD - Q21.8 (other congenital heart abnormalities).

Important: Among patients with the Eisenmenger complex is noted in 10% of cases.

Causes of the development of pathology in the kid

The heart pathology of this species is still developing in the first 8 weeks of embryo growth. It is during this period that the heart is laid at the crumbs. Significant and proven science causes of the development of anomaly was not identified. However, doctors tend to believe that it is the course of pregnancy and a third-party impact on the human body and the fetus provoke pathology. It is assumed that the impact on the development and structure of the health of the fetus may have such factors:

• heredity (if a history of parents' parents have heart vices);
• reception of certain drugs during the pregnancy of the future mother;
• toxic and physical impact on the mother's body (alcohol, salts of heavy metals, radiation irradiation, magnetic effect, vibration, etc.);
• viral infections transferred by mom in the first trimester of pregnancy.

Symptoms of heart pathology

Eisenmenger syndrome in children and adults can proceed asymptomatic in the case of normal blood current. If hemodynamics takes right to left, the vice is manifested by such signs and symptoms:

1. Obvious cyanosis of the upper part of the body (the sinusiness of the named zone).
2. Permanent shortness of breath is even in a state of relative rest.
3. Increased fatigue and pronounced physical weakness.
4. Permanent noveling pain in the field of chest.
5. Tachycardia and feeling of heartbeat.
6. Frequent headaches.
7. Frequent pathological bleeding from the nose (and hemoplary including).
8. Visible swelling of the cervical veins.

At the same time, the presence of a complex of the Eisenmenger in a person can be determined by its appearance. Often such patients are trying to squat, thus facilitating the state of the lack of oxygen. At the same time, patients with the Eisenmen gene complex observes the deformation of the spine and explicitly protruding the chest above the heart location zone.

It is also worth noting that although in general such people do not suffer in development in development, they still have some drop in view and speech disorders. The Eisenmenome Syndrome can also be characterized by frequent pneumonia, ORVI and bronchitis.

Important: In the asymptomatic course of pathology, a sudden death may occur against the background of ventricular fibrillation. Patients with an explicit clinical picture may die from bleeding from the upper respiratory tract or from acute heart failure.

Diagnostics

A simple inspection of a newborn or adult patient and listening to the tones of the heart do not give the doctor a complete picture. To confirm the diagnosis, it is necessary to conduct a number of studies:

• Electrocardiogram (ECG). Allows the doctor to track the conductivity of electrical appeals in myocardium.
• X-ray organs in the chest. It is necessary for the margin of the parameters of the heart and its contours, as well as estimates of the state of the main vessels.
• Echocardiography (heart ultrasound). It makes it possible to see the work of the heart, vessels and valve system. Reuses impaired hemodynamics.
• Heart catheterization. Makes it possible to assess the condition of the heart in each of its departments.

Important: Often the Eisenmenger syndrome is masked under other diseases, which makes it difficult to diagnose and takes the precious time in the patient. For example, frequent bleeding can take for Willebrand disease.

Treatment of cardiac pathology

Methods of complete cure from the Eisenmenger syndrome does not exist. All medication therapy is aimed at relieving symptoms and to maintain the optimal quality of the patient's life. Therapy also includes preventive measures that are aimed at preventing possible complications such as high pulmonary hypertension, etc. Mostly, drug therapy is carried out either at the stage of preparation for the operation, or if it is impossible. A number of drugs are prescribed to the patient to maintain its condition at a more or less normal level:

• Phosphodiesterase 5 type inhibitors. Make it possible to affect all smooth muscle tissues of vessels.
• Prostacyclines. Reduce the pressure in the lung artery zone, the significantly improves the consumption of oxygen muscles. In addition, such drugs prevent the lesion of pulmonary vessels.
• Anticoagulants. Prevent blood clotting and blood tromping.
• Antiarrhythmic drugs. Prevent arrhythmia and stabilize heart rhythm.
• Endotelin receptor antagonists. Assign to maintain the work of the lung vessels. But these drugs can have a negative impact on the heart. Therefore, they are used infrequent and not long.

The operation is made at this disease or in order to formulate a pacemaker, which normalizes the heart rhythm in the patient or to eliminate. Especially good chances of life gives a second type of surgery at an early age.

Important: With the low efficiency of all listed methods, the patient shows the lungs and heart transplant. The forecast in this case is quite favorable.

Prevention

As for preventive measures, it all depends on genetics and from the mother itself. And if the genetics does not win, then the future mother can do everything on it to prevent the possible abnormal structure of the heart of the fetus. To do this, during pregnancy, it is necessary to refrain from self-admission of drugs, protect yourself from viral infections and eat right. In addition, it is advisable to avoid places with possible toxic effects on the body (enterprises of the chemical industry, radiation zones, etc.).

It is important to understand that Eisenmenger's syndrome is a pathology that does not only reduce the quality of the patient's life, but also takes it a significant part of it. Therefore, the main task of a pregnant woman is to do everything so that the baby is born healthy and strong.

The vascular disease of the lungs secondary to the left-right discharge in the heart or between arterial trunks, with an increase in pulmonary blood flow, leading to a high, irreversible pulmonary hypertension. It is a complication of simple and combined shunt vices of the heart. It is often manifested in childhood, among adults are revealed mainly in patients with interdestrous discharge and open arterial duct.

1. Subjective symptoms: a significant reduction in load tolerance, a sensation of lack of air, amplified during load, heartbeat, chest pain; with high hematocrit - signs of blood viscosity syndrome, hemoptal, bleeding from the respiratory tract; fainting in the later stages.

2. Objective symptoms: central cyanosis, symptom of drum sticks, "Heart hump", pathological pulsation on the left, palpable pulmonary component of the second tone, focus of the second tone above the pulmonary artery, III tone, in the case of hypertrophy of the right ventricle - IV tone, the disappearance of noise associated with the vice lying in the basis of the syndrome, for example, a continuous systor-diastolic noise with an open arterial protocol or systolic noise during the interventricular septum defect; Often quiet systolic noise in the left edge of the sternum is likely due to the expansion of the pulmonary artery barrel; Quite quiet protodiastolic noise with lack of pulmonary artery valve due to high vascular pulmonary resistance (Graham-style noise).

3. Natural flow: patients with simple shunt shocks live on average\u003e 40 years, with combined vices. The most frequent causes of death: heart failure, sudden heartfelt death, bleeding from the respiratory tract. Factors increasing the risk of severe complications, including death: pregnancy, use of general anesthesia, dehydration, bleeding, surgical interventions, abuse of diuretics, some oral contraceptives, anemia (most often due to unnecessary bloodstands), heart catheterization, intravenous administration medicines, pulmonary infections.

Diagnostics is based on the definition of high vascular resistance in the lungs, which does not decrease when using a vasodilator (oxygen, nitrogen rushing) in a patient with a shunt pulp. Invasive diagnostics applies if the results of the non-invasive assessment assume the possibility of a surgical correction of vice.

Additional research methods

1. Laboratory diagnostics: consequences of hypoxemia; Saturation oxygen in the blood is usually<90 %.

2. ECG: Signs of increasing the right atrium, hypertrophy and overloading of the right ventricle, incomplete blockade of PNPG (with interpresenting defect).

3. RG of the bodily cavity: The shade of the heart can be of different sizes depending on the main vice and stage of changes in the vascular bed of the lungs, the peripheral pulmonary pattern is depleted, it is possible to expand the vessels in the roots of the lungs.

4. Echocardiography: Hypertrophy of the right ventricle, other changes depending on the severity of the syndrome, for example, the insufficiency of the atrial and ventricular valves; Cleaning research can identify thrombosis of proximal segments of pulmonary arteries.

1) Annual flu vaccination against pneumococci every 5 years;

2) pay attention to the symptoms of bleeding;

3) intensive treatment of inflammatory states;

4) avoiding dehydration, excessive physical exertion, staying at large altitudes above sea level; termination of tobacco;

5) Consultation of the cardiologist with any health problems.

2. Treatment of blood viscosity syndrome:if HT\u003e 65%, after excluding the deficiency of iron and dehydration → Blooding 250-500 ml for 30-45 minutes and transfusion of the same volume of 0.9% NaCl.

3. Heat treatment: it is usually not necessary; In case of life-threatening states (gap of the aorto-pulmonary collateral, pulmonary artery or arteriole) → Cracked embolization or surgery.

The syndrome (complex) of the Eisenmenger (Code of the ICD 10) is an irreversible form of hypertension in the pulmonary artery system associated with congenital heart disease. It is believed that this state can develop at any deficent shock with significant blood discharge on the right left. The Eisenmenger Complex is found in 10% of children and adults who have not undergone operation, with a defect of the interventricular septum of any size, with a defect of the interpreservation partition - in 4-6% of patients. This serious condition has a significant impact on the life of the patients: its duration is about 40 years.

Causes and Pathophysiology Development of Eisenmenger Syndrome

The Eisenmenger syndrome may be due to both simple and complex congenital heart disease. This condition is a complication of "pale type" vices (without manifestations of cyanosis), which during progression can be transformed into "blue" (with severe cyanosis). Patients with a diagnosis of Eisenmenger syndrome reveal at least one defect in the heart:

• interventricular partition (DMCP);
• interpreservation partition (DMPP);
• open arterial duct;
• tetrad Fallo (stenosis of the output department of the right ventricle, DMWP, dextraposition of aorta, hypertrophy of the right ventricle);
• pentada Fallo (Tetrad Fallo in combination with DMPP);
• ebestein anomaly (deficiency of the tricuspid valve, an unexpressed oval window, a decrease in the cavity of the right ventricle).

In patients with similar violations of the tightness of cardiac cameras, intracardiac shunting of blood from left (systemic, with higher pressure) in the right (pulmonary) departments occurs. This leads to the oversaturation of the blood of the right atrium and the ventricle, which reflexively leads to the spa of the pulmonary arteries and increases the pressure in the pulmonary artery system. Small vessels inside the lungs are structurally changed, which further enhances intramiliary pressure and makes it difficult for subsequent treatment. Oxygen-saturated arterial blood due to defect inside the heart is mixed with venous, which leads to the development of hypoxia and cyanosis (stemming of skin).

Clinical manifestations

In many children with Eisenmenger's syndrome, at least one episode of pulmonary edema was observed due to the enhanced pulmonary blood flow, resulting from the intracardiac left-right-hand shunt of blood. Later, with an increase in the resistance of pulmonary vessels, the flow of blood in the lungs slows down, and the symptoms of hyperemia of the lungs are reduced. When the shunt from the left-right is transformed into the right-left, cyanosis and red blood cells are developing as a result of chronic hypoxia. Much less often the clinic is manifested in adulthood without specific manifestations: increasing weakness, shortness of breath, cyanosis. Most patients have the following signs:

• symptoms of hemodynamic disorders in a large circulation circle (shortness of breath during exercise, weakness, fainting);
• neurological deviations (headaches, dizziness, violations) due to red blood cell and hyperventilation;
• symptoms of stagnant heart failure;
• arrhythmias and hemopes (as the causes of sudden death).

Diagnostics

The physical examination reveals the central cyanosis, the symptom of "drum sticks" (the nail phalanxes in the hands are expanding, reminding the shape of the drum sticks), swelling of the cervical veins, the presence of a heart hump (strain of the chest in the heart of the heart), the pulsation to the left of the sternum. With auscultation, the focus of the second tone above the pulmonary artery valve, coarse systolic noise above the entire surface of the heart with a characteristic symptom of "cat purbanya" (chest trembling similar to purring).

To register sound effects, phonocardiography is used. On the radiography of the chest in direct projection, the extension of the pulmonary artery trunk is detected. Standard ECG diagnostics registers signs of overload of the right heart departments. With a catheter measurement of pressure in the pulmonary artery, its increase is detected to 60-100 mm Hg. (at a rate of up to 30 mm Hg). Echocardiography confirms the presence of one or more defects in the heart.

Treatment

A positive prognosis is possible with rapid surgical interference, which is to embed defects between interdestrial and interventricular partitions, eliminating the wrong arrangement of the aorta, closing open arterial duct. Many studies have proven the ineffectiveness of the surgical treatment of Eisenmenger's syndrome in adulthood: the earlier the operation will be carried out, the greater the likelihood of its positive outcome. But palliative (eliminating symptoms) of operations, for example, aimed at narrowing the pulmonary artery to reduce the pressure in it are possible.

Medical therapy with Eisenmenger syndrome is symptomatic, aimed at improving the activities of the heart muscle, a decrease in intra-alert pressure, a decrease in the symptoms of heart failure.

It is carried out in the preoperative period and in contraindications and the impossibility of performing cardiac surgery.

Medicines used in the therapy Eisenmenger's syndrome:

Medicine	Indications	Contraindications
Anticoagulants (warfarin)	Warning of thromboembolism in the pulmonary artery	Restrictions on systemic diseases of connective tissue
Oxygen	Hypoxia (it is recommended to use low concentrations up to 2 liters per minute)	Causes spasm of vessels, apply when monitoring
Supporting symptomatic therapy (diuretics, digoxin)	Worldwide heart failure	The toxic manifestations of digoxin are developing during its overdose
Calcium antagonists (Diltiazese, Amlodipine)		Cannot be used at a low cardiac output
Long therapy with prostaglandins (Epoprostolen, Iloprost, Treprostinyl)	Remodeling of vessels in the system of pulmonary artery, reducing the damage of vessel cells	Expensive drugs, the risk of the "Ricocht" symptom is possible
Phosphodiesterase-5 inhibitors (Sildenafil)	Pressure reduction in pulmonary artery	Reception of nitrates or nitrogen oxide donators

The Eisenmenger complex is a congenital heart disease, in which there is a combination of 3 signs: the dextroposition of the aorta (aorta seems to be riding on the right and left ventricles, i.e., it is reported with both gastrics), the defect of the interventricular partition and the right ventricular hypertrophy. Thus, it is the same Tetrad Fallo, but without stenosis of the pulmonary artery and the infundibular part of the right ventricle. It is revealed much less frequently than Tetrad Fallo, making up 5% of all cases of congenital heart defects accompanied by cyanosis. Men and women are sick about the same often. Quite often, the Eisenmenger complex is combined with other congenital heart defects, for example, with the unnecessary of the arterial duct, the defect of the interpresentation partition, the coarse of the aorta and T, d.

Plok is compatible with an uterine life. After the birth of the oval hole and the arterial duct closes normally. Venous blood flows from the right atrium into the right ventricle. From here, most of the blood is thrown into the pulmonary artery and the lungs, from where the pulmonary veins return to the left atrium. Since the aorta "sits the riding" on the defect of the interventricular partition, then some of the blood from the right ventricle is thrown into the aorta. In this case, the pressure significantly increases the pressure in the pulmonary artery. It can achieve pressure values \u200b\u200bin the aorta or even exceed it. Increased pressure in a small circulation circle is due to an increase in resistance in pulmonary arteriols (the minute volume of blood in the pulmonary circle does not increase, it is normal or, more often, reduced). It can be equal to the resistance in a large circulation circle. This is an expedient compensatory reaction that protects the small circle of blood circulation from hypervolemia, and the patient - from the eighties of the lungs. The mechanism of increase in pressure from a small circulation circle is not clear. Assumptions about the arteriole spa, about the anatomical changes in the structure of the arterioles are expressed. The latter is more likely, since it finds confirmation in histological examination.

Blood impairment with the Eisenmenger complex leads to the hypertrophy of the right ventricle. Sooner or later, cyanosis appears. This is due to venous arterial shunting. The volume of venous blood discharged to the aorta depends on several factors: the size of the interventricular septum defect, the degree of dextroposition of the aorta, the ratio of systolic pressure in the right ventricle and aorta. The volume of venous arterial shunt with increasing pulmonary hypertension increases.

From a path-physiological point of view, we can talk about two periods of development of the Eisenmenger complex: steps without cyanosis and stage with cyanosis. In the first stage, oxygen saturation of peripheral blood blood is normal or almost normal, since the shunting goes from left to right, that is, according to arterial-venous type (due to lower vascular resistance in a small circle of blood circulation compared to greater). In the second, dianetic, stage, the oxygen saturation of peripheral arterial blood is reduced, as the shunting goes to the right left, that is, according to the venous-arterial shunt (due to higher vascular resistance in the small circle of blood circulation compared to greater).

Pathological anatomy.

The position of the aorta "riding" on the ventricle is usually moderate. The defect, located in the webbed part of the interventricular partition, is of large size (usually 1.5-3 cm in diameter). The dimensions of the pulmonary artery are always normal; In most cases, the trunk and branches are sometimes significantly. There are pronounced morphological changes in pulmonary arterioles: media muscle thickening with a decrease in the lumen of the vessel. These changes are associated with a lack of reverse development in a period of extruded life, when the lungs and the remaining intrauterine type of blood circulation are started in the lungs. In the future, secondary changes are developing in the arteries (secondary fibrous intima thickening) due to increased pressure in the pulmonary artery.

Right ventricle and right atrium hypertrophied and expanded. Left ventricle is not increased. 25-20% of patients have a right-sided aortic arc.

Treatment of the Eisenmenger complex

The operation of the closure of a defect, undertaken to the development of a rack hypertension of a small circle of blood circulation and irreversible changes in the wall of the pulmonary arteries.

The forecast is doubtful to the development of the cyanosis. The form with late cyanosis is more favorable, although rarely patients live more than 10 years since the appearance of an explicit cyanosis. Death in most cases occurs at the age of 20-30 as a result of the lack of right heart. The form with early cyanosis occurs, as a rule, from the very beginning unfavorable. Patients die, usually not reaching mature age. Despite the fact that at the same time the vice live longer than with other congenital vices with cyanosis, the average life expectancy is about 25 years.

Symptoms

In infancy, the disease usually does not exhibit themselves, especially if the size of the opening is small. Often, the symptoms of the disease are unexpectedly found to achieve a man of 30 years.

The symptoms of the defect of the interpresentation partition are:

• blue skin shade, excessive pallor;
• the presence of specific noise in the heart;
• the appearance of shortness of shortness of active actions;
• swelling of legs and abdomen;
• fatigue;
• repeated lung infections;
• heart rate violation;
• stroke.

When need to contact a doctor

If your relative or you have found the following symptoms, you should go to the doctor:

• choosing, frequent shortness of breath;
• bluish skin color;
• feeling constant fatigue.

Complications

The objective possibility of developing complications in the presence of the disease depends on the size of the opening in the partition. It happens that small defects are closed without medical intervention in childhood.

• recurrent bacterial pneumonia;
• heart rate violation;
• stop the right side of the heart;
• high probability of stroke;
• reduction of a possible lifespan;
• the occurrence of pulmonary hypertension (blood pressure is significantly increasing in the lungs);
• eisenmenger syndrome (irreversible lung damage, which may occur with a large partition defect).

Causes of the disease

The appearance of a defect is associated with the fact that the partition in the embryonic period was not fully formed, which may be caused by the following reasons:

• hereditary predisposition (the presence of a defect of the intersdest partition from one of the family members);
• bad ecology of the child's parents residence (radiation, production waste);
• diseases of the pregnant mother of viral nature: windmill, rubella, herpes, syphilis, etc.;
• consumption during pregnancy drugs, which contains lithium, progesterone, tertinoin;
• diabetes of mother;
• unlimited use of alcohol during baby tooling.

Prevention of the defect of the interdestrial partition

There are no specific measures for the prevention of the disease. It develops intrauterine, so the future mother should be performed simple recommendations:

• put the vaccine from rubella long before the planning of pregnancy;
• on time to register and regularly apply visits to the gynecologist;
• do not drink alcohol and do not smoke;
• eliminate contact with harmful substances, avoid exposure to radiation;
• organize proper nutrition;
• take drugs only after consulting and under the control of the doctor;
• examine a family history (if there is a congenital heart defect in relatives to carefully observe the development of the fetus).

Diagnostics

The final diagnosis is established by a doctor based on the analysis of patient complaints, general inspection data, as well as after studying the results of designated surveys. The following methods of diagnosing the disease are possible:

• Listening to the heart with a stethoscope (determines the presence of systolic noise characteristic of the disease).
• Radiography shows the change in the shape of the heart, the state of the lungs.
• Heart ultrasound (echocardiography) is aimed at obtaining a visual idea of \u200b\u200ba defect (determining its location) and refinement of the size of the right ventricle.
• Electrocardiography (ECG) is aimed at identifying a rhythm disturbance and establishing signs of increasing the right side of the heart.
• Angiography due to the use of contrast gives you the opportunity to see the shape of the heart and establish a blood flow scheme.
• Catheterization of the cavities of the heart makes it possible to measure the pressure in vessels and cavities of the heart, contributes to the determination of the patient's treatment tactics.
• Magnetic resonance imaging (MRI) is designed to more accurately determine the nature of a congenital defect.

Treatment of the defect of the interdestrial partition

Drug treatment is carried out with the help of drugs that do not eliminate the defect, but allow us to reduce the manifestation of the disease.

In this case, treatment is used:

• preparations that make it possible to control the heart rhythm;
• drugs that reduce blood coagulation (anticoagulants), the use of which reduces the risk of stroke.

Surgical treatment makes it possible to eliminate the defect by attaching a patch on the hole in the inter-subsensudual partition.

Different operational methods are used:

• heart catheterization (summing up a catheter through a large vein to a damaged area, installation of grid patch);
• open operation on the organ (incision of the chest, overlaying patch or suturing of the opening).

Causes

To date, the exact factors affecting the development of the Eisenmenger complex are not established, however, it is believed that they are similar to the reasons that cause other varieties of the burdens of heart defects.

Possible reasons include:

• the influence of heredity;
• the pathological action of toxins of various origin (the use of some medical drugs, smoking, the influence of ecology, etc.);
• infectious and viral diseases transferred by the mother during pregnancy.

All symptoms of pathology are associated with the fact that hemodynamics suffers from the Eisenmenger complex. And due to the combination of violations, the violation is very much detected.

For example, because of the defect in the partition between the ventricles in the aorta, venous blood falls in an excessive blood, in which the oxygen content is small. Often, with a complex of Eisenmengenera, it is not just a defect of the partition between the ventricles, but its complete absence.

Promotes to get into the aort of blood with a small oxygen content and its incorrect location.

The characteristic manifestation of the complex is also an increase in pressure in the pulmonary circulation circle. This is due to the spasm of preacapillars, which is formed in response to the fact that in the pulmonary artery, the pressure turns out to be excessively high (sometimes even exceeds the level of pressure in the aorta).

Permanent hypertension in the pulmonary circulation circle leads to changes in the walls of the vessels, due to which blood saturation is complicated by oxygen.

The complex takes two main stages:

Symptoms of the Eisenmenger complex

In the early stages of development, the Eisenmenger's complex has no clinical manifestations, and that is why children with this congenital heart disease at the "White" stage are no different from the peers.

When the white stage goes into blue, the following clinical signs of pathology are developing:

• the color of the skin becomes blue;
• dyspnea appears first during exercise, and then alone;
• portability of physical exertion is dramatically reduced;
• the feeling of heartbeat appears, the ECG can be registered arrhythmia;
• signs of angina disease appear;
• the threshold of fatigue is reduced, the patient often pursues a sense of weakness.

To reduce the shortness of breath and ease your breath, patients are often forced to squat.

It is a vice, which "shifts" the heart into the right side of the chest.

Why atresia aorta is treated only with the help of surgery - the answers here.

You can also meet the following manifestations of the Eisenmenger complex:

• bouts of headaches;
• episodes of short-term loss of consciousness;
• the appearance of bleeding from the nose;
• bouts of hemopling;
• hoarseness and fast fatigue voices;
• changes of the spinal column and chest (the appearance of the "heart hump");
• changing the fingers on the hand of "drum sticks";

Patients with the Eisenmenger complex often transfer respiratory diseases such as pneumonia and bronchitis.

The survival rate of patients without timely assistance is 20-30 years, and death occurs due to heart failure.

Diagnostics

The diagnosis is beginning with the examination of the patient, noting the cyanosis of the skin, changes in the chest, visible to the pulsation of the heart.

With auscultation, it is possible to determine the presence of systolic and diastolic noise, which are explained by the anatomical changes in the structure of the heart. If the doctor is in doubt about the noise listened, then you can resort to phonocardiography.

At the palpation of the chest, the characteristic jitter is determined, which is called the symptom of the "cat purbash".

The following techniques are used from diagnostic studies:

In the process of diagnosis, it is important to distinguish the Eisenmenger complex from other congenital heart defects.

Treatment

Treatment of the Eisenmenger complex is possible only with the help of a cardiac surgery. Even with surgical intervention, the forecast is favorable only if the resistant signs of increasing pressure in a small circle of blood circulation and cyanosis have not yet developed.

In surgical intervention, the surgeon primarily eliminates the defect that struck the interventricular partition. Also be sure to adjust the position of the aorta, eliminating its anomaly.

Because there is a need to move the aorta, this is due to the need to replace the aortic valve, which in most cases is damaged during operational intervention.

Operational intervention about the Eisenmenger complex is a complex procedure that often ends with the patient's death on the operating table.

If the risks are excessively high, the doctors may decide on the operational intervention of palliative type. Its essence is in the narrowing of the pulmonary artery with the help of a special cuff. Such an operation helps to alleviate the symptoms of pathology, but does not solve the main problem, only postponing the aggravation of the disease.

To eliminate pathology, drug treatment is not used at all, as medications can no longer affect the anatomical defects, their elimination is possible only with the help of surgical intervention.

Medical treatment is useful in preparing the patient to surgical intervention. With it, they facilitate the main symptoms of the disease, partially eliminate the arrhythmias and symptoms of heart failure.

Also, medicines are applied by doctors if the surgical intervention cannot be carried out, but then therapy is rather palliative.

Forecast

The Eisenmenger complex is often accompanied by such complications as:

• endocarditis bacterial origin;
• bleeding of pulmonary origin;
• thromboembolism;
• infarction lung damage.

The forecast also depends largely on the stage of the disease. So, with a timely manifold assistance in the white stage, survival remains satisfactory.

If the complex is diagnosed and begins to be treated after the transition to the blue stage, the life expectancy of these patients rarely exceeds 10 years even during treatment.

The total average life expectancy of patients with this diagnosis is about 20-30 years.

The presence of a woman of the Eisenmenger complex is always the need to interrupt pregnancy, since the risk of mothers death in the process of the delivery is high.

It can be cured by learning and conservative therapy or surgery.

Valves of heart valves and their main species are described here.

Why an atrioventricular communication occurs - here.

If the abortion is refusing, most cases ends with either premature births or spontaneous miscarriage. Cases when women with a complex of Eisenmenger safely passed the period of gestation, very little in medical practice.

Previous article: What is atrioventricular communication

Next article: Operations with different types of heart failure

Signs and symptoms

First described by hippocratic, known as "Hippocratic Fingers". Symptoms and symptoms of Eisenmenger syndrome include:

• Cyanosis (blue tint of the skin due to lack of oxygen);
• High amount of red blood cells;
• Swollen or mace-like fingertips;
• Faint, dizziness;
• Heart failure;
• Heart rate disorders, heartbeat;
• Cough with blood;
• Iron deficiency;
• Infections (endocarditis, pneumonia);
• Kidney problems;
• Gout (rarely) due to increased resorption, urinary rehabilitation with disabilities;
• Bile problems;
• Shortness of breath during exercise (with activity);
• Shortness of breath at rest;
• Fatigue;
• Breast pain or breasts;
• Headache;
• Paresthesia (numbness, tingling fingers and legs);
• Blurred vision.

Complications:

• Thrombus (thrombosis of deep veins of limbs);
• Bleeding;
• Stroke;
• Brain abscesses;
• Gout.

The deterioration of symptoms or new complications may be early warning signs of changes in general condition. Must be appreciated by the doctor as soon as possible.

The symptoms of the Eisenmenger can resemble other diseases or heart problems. Always consult your doctor for careful diagnosis.

The Eisenmenger syndrome develops with time as a result of the effects of high blood pressure in the lungs. This high blood pressure, or pulmonary hypertension, occurs due to congenital heart defects, which force the blood to flow from the left side of the heart to the right (shunt from left to right). Congenital vices of this type include:

• Open arterial duct (PDA). The relationship between the aorta and the pulmonary artery, which allows rich oxygen (red) blood, which should enter the body, recycle through the lungs.
• Defect of the Interdest Partition (ASD). The hole in the atrial septum or partition between the two upper heart cameras, known as the right and left atrium.
• Pathology of the interventricular partition (VD). The hole between the two bottom cameras of the heart, known as the right and left ventricles.
• Defect of an atrioventricular channel (AV channel). The problem with the heart, which includes several anomalies of structures inside, ASD, VSD and incorrectly formed mitral or tricuspid valves.

Since the pressure on the left side of the heart is usually greater than in the right, the hole between the left and right side will cause the bloodstream from the left side to the right side. This shunting from left to right causes a strengthening of blood flow in the blood vessels of the lungs. Increased blood flow in the blood vessels of the lungs causes pressure increase (pulmonary hypertension).

If pulmonary hypertension continues without treatment, the pressure on the right side of the heart may increase to such an extent that it will be more than in the left. When this happens, the blood flows from the right side of the heart to the left (the shunt on the right left). It turns out that the blood with a low oxygen content is mixed with blood with its high content, usually reproached to the body with a left ventricle.

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This change (shunt right to left) causes a lack of oxygen in the blood. In addition, high pressure in the lungs gives progressive changes in pulmonary blood vessels, which lead to irreversible damage to their mucous membrane. Fibrosis may also appear (growth of scar tissue after infection, inflammation, injuries), thrombus. Changes inside the lungs are called pulmonary vascular obstructive disease or secondary pulmonary arterial hypertension (lag).

Pathogenesis

The cause of Eisenmenger's syndrome, often occurring at a later age, can be explained by changes in normal heart physiology and non-adaptive reactions arising from time. A large and more muscular left part of the heart should create a high pressure required to supply blood into an extensive blood circulation system with high resistance.

On the contrary, a smaller right side should create a much lower pressure to skip blood through low resistance and high degree of adherence. Lightweight can perform this circulation with low resistance to a large extent due to the fact that the length of the pulmonary circulation is less, and also because most of the scheme is in parallel, and not consistently.

If there is a significant anatomical defect (hole or break) between the two sides of the heart, which causes blood flowing below the normal pressure gradient from the left side to the right side. The number of shunt blood is proportional to the size of the defect.

The volume, pumping through the gap from left to right, is the percentage of the expected cardiac ejection (SV) left ventricle. The clinically low index or the percentage of stubs emitted through the shunt is harmless. High index or percentage of CB, emitted through the shunt from left to right, foreshadows the reaction of the Eisenmenger.

From left to right

Shunting from left to right leads to abnormally large blood flow and pressure aimed at the right heart circulation. Gradually leads to deducive changes, pulmonary hypertension. An increase in the right-hand volume of blood and pressure causes a cascade of pathological damage to delicate pulmonary capillaries. They are gradually replaced by a scar cloth.

Scar (dead light fabric) does not contribute to the transfer of oxygen, so the useful volume of the pulmonary system is reduced. Scar tissue also provides smaller flexibility and elasticity than normal. It causes further increase in pressure.

The weakened heart should work more intensively to continue to supply lungs, which leads to damage to a larger number of capillaries. It is because of this non-adaptive response at the beginning of Eisenmenger's syndrome, damage is considered irreversible, even if the main defect of the heart is corrected after.

Due to the increased resistance and reduction of the supplement of the vessels, the increased pressure in the pulmonary artery leads to the hyperrophy of the myocardial of the right heart (RVH).

The Eisenmenger syndrome begins when the hypertrophy of the right ventricle leads to the fact that the pressure on the right exceeds the pressure on the left. As a result, the deoxygenic blood returning from the body bypassing the lungs through the reverse shunt and moves directly into the systemic blood circulation, which leads to cyanosis, damage to the organs.

From right to left

The shunt right to left, causes a reduced saturation of arterial blood oxygen due to the mixing of saturated oxygen, returning from the lungs, with deoxygenic blood returns from the systemic circulation.

This reduced saturation is felt by the kidneys, which leads to a compensatory increase in erythropoietin production, an increase in the development of erythrocytes when trying to increase the delivery of oxygen. As the bone marrow increases the erythropoes, the number of system reticulocytes, the risk of the development of hypercase syndrome increases.

Reticulocytes are less effective when transferring oxygen, like mature red blood cells. They are less deformable, causing a violation of passage through capillary layers. This contributes to the death of pulmonary capillary layers.

A man with Eisenmenger syndrome is subject to uncontrolled bleeding due to damaged capillaries and high pressure, also spontaneous thrombones due to hypervoles and stagnation of blood.

Diagnostics

A child or teenager with a defect history of the interpreservation partition (ASD), the interventricular defect (VSD), open arterial duct (PDA) or PODAOBOD of an atrioventricular channel (AV channel), is at risk of the occurrence of Eisenmen german syndrome.

The doctor will determine the change or an increase in the intensity of noise in the heart (blowing, hoarse sound, hearing while listening to the heart, which indicates problems) during the usual physical examination. Complaints for changing the tolerance for physical activity or associated shortness of breath, chest pain, rapid heartbeat, especially in physical activity.

The doctor will hold a medical examination, hears the heart, lungs, will send to research that will help the diagnosis. For instance:

• Blood tests - to determine the increased amount of erythrocytes (red blood cell), anemia.
• Radiograph of the chest. There may be changes in the lungs due to additional blood flow, which can be seen on the X-ray.
• Electrocardiogram (ECG). Determine the abnormal rhythms (arrhythmias, disdications), the tension of the heart muscle.
• Echocardiogram (echo). The echo signal will show the direction of blood flow through the source of the shunt lesion, such as PDA. Determine how large a hole, the amount of blood passing through it.
• Heart catheterization is an invasive procedure that gives very detailed information about structures inside. During the sedation, a small thin flexible tube (catheter) is introduced into the blood vessel in the groin, headed inside the heart. In typical catheterization of pressure and oxygen measurement, the pulmonary artery, aorta is carried out in four chambers of the heart. Sometimes the contrast dye is introduced for a clearer visualization of blood flow and structures.
• Magnetic resonance tomography (MRI). The diagnostic procedure is used to clarify the amount and direction of blood shunt.

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If the defect of the heart is detected before it causes significant pulmonary hypertension, it can be corrected using surgical intervention, preventing the disease. After the pulmonary hypertension turns blood flow, the syndrome is considered irreversible.

Congitory heart-light or lung transplantation with heart restoration is the only treatment option. Transplantation is a final therapeutic option for patients with a bad forecast and quality of life. Its feasibility is a difficult decision. 5-year and 10-year survival ranges between 30 and 80%.

Preparations, methods of treatment of pulmonary hypertension are at the study stage. Air intravenous filters are recommended. They reduce the risk of random air in the vein due to increased risk of an air embolism. In case of air intake, it passes through the interventricular septum defect in arterial blood circulation - a stroke occurs.

The specific treatment of Eisenmenger syndrome is determined by the doctor on the basis of:

• Age, general health, disease history;
• Progression rate, degree of illness;
• Tolerance to certain medicines, procedures, methods;
• Forecast of the course of the disease.

The objectives of the treatment of Eisenmengene's syndrome are aimed at reducing the pressure of the pulmonary artery, an improvement in oxygenation, a decrease in the degree of cyanosis, red blood cell. Treatment methods include, but are not limited to:

Medical treatment

The treatment of Eisenmenger syndrome is based on symptoms. Types:

• • Medications. Medications are prescribed that help reduce the pulmonary hypertension. These drugs include:
• calcium channel blockers;
• prostacyclin;
• endothelinic receptor antagonists.

They work to expand blood vessels to reduce pressure. If right-sided heart failure develops, diuretics (diuretic) are prescribed to reduce blood volume.

• • Oxygen. Additional oxygen is used during sleep or leisure. Continuous use is necessary to facilitate symptoms.
  • Phlebotomy. Phlebotomy (blood removal) should not be carried out often. It is recommended when the erythrocytosis causes blood hypercycy. The volume of remote blood is replaced by intravenous (IV) physiological solution for liquefaction.

Phlebotomy is performed only when the symptoms are serious or hematocrit (percentage of erythrocytes) becomes extremely elevated.

Other treatments

The Eisenmenger Syndrome, which develops as a result of a congenital heart defect, if not eliminated, is usually not treated by restoring the source defect. Since it will increase the load on the right ventricle and will lead to a bad result. Light or heart-lung transplantation is considered when other treatment methods are no longer effective.

The syndrome is called Dr. Paul Wood in honor of Viktor Eisenmenger, who first described the state in 1897.

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Long-term perspectives

Some people with Eisenmenger syndrome, who have no other health complications, can live to the fifth or sixth decades of life. Recommendations include, but are not limited to:

• Pregnancy is not recommended for women with Eisenmenger syndrome. It is dangerous for the mother, gives complications for the fetus due to the effects of low oxygen levels.
• Any anesthesia and operation is considered to be a high risk. Must be carefully planned. Cooperation with cardiologist is recommended.
• Air travel (on an airplane), high-rise impact require special moistening (fluid consumption), additional oxygen to prevent complications.
• Strong headache, dizziness, fainting, changes in the level of consciousness should be taken seriously and evaluated urgently.
• Smoking, alcohol use is not recommended.
• Cough is eliminated with a strong suppressive drug to prevent the risk of pulmonary bleeding.
• Influenza vaccinations are recommended annually. The pneumococcal vaccine must be taken in accordance with the recommendations of the doctor.

Consult your doctor about long-term prospects for a child, a teenager, a young man with Eisenmenger syndrome.

Pathophysiology

Supravalcular, valve and linked lesions are associated with pulmonary valve stenosis. The lesions vary according to severity, from the simple valve hypertrophy to the complete obstruction of the outflow and atresia. Trileaflet pulmonal valve varies from thickened or partially fused adhesions to a non-perforated valve.

Most cases of pulled valve stenosis are congenital. Often flaps of valve development are associated with syndromes such as Nunan syndrome and leopard syndrome. The nature of the inheritance of the stenosis of the pulmonary valves is poorly studied, although these syndromes have an autosomal dominant character. Rarely, pulmonary stenosis is associated with recessively transmitted states, such as Louurens-Moon-Bidla syndrome. Mutations in the embryonic lines PTPN1 and RAF1 were associated with these vices of the valve development. Supravarvular lesion can occur under the conditions of the tetralogy of Fallo, Williams Syndrome, Alagilla's Syndrome, as well as Nunan Syndrome.

The myocardine cushion begins as an endothelial cell matrix and an outer mitochondrial layer separated by heart jelly. After the formation of the endocardial cushion, the endothelial mesenchymal transformation, which indicates endothelial cells, is differentiated and migrates in a hearty jelly. Thanks to the poorly studied process, the hearty jelly passes through the local expansion and swelling of the bolus, and cardiac valves are formed. The aortic and lung valves develop from the tract of outflow of an endocardial pillow, it is also believed that during development there is migration of the cells of the nervous crest from the shoulder ridge.

Studies show that the vascular endothelial growth factor (VEGF), the playiotropic factor is responsible for the transfer of endocardial cushion development signals. Hypoxia and glucose have a regulating effect on this factor. In children born from hyperglycemic mothers, cardiovascular disorders increase 3 times. A correlation was revealed between hyperstoxic phenomena inside the genus and valve disease. In addition, numerous signaling molecules contribute to VEGF and EMT, such as ERB-B signals in the heart jelly, transforming growth factor (TGF) / cadhelerin and BMP / TGF-beta.

The pulmonary valve develops between 6 and 9 weeks of pregnancy. Usually, the pulmonal valve is formed from 3 edema of subendocardial tissue, called semi-lounge valves. These tubercles are developing around the hole of the pulmonary tree. Edema is usually poured and changed to form 3 thin-walled lung flaps. With Nunan syndrome, excessive expansion of tissue gaskets in the sinuses violates normal mobility and valve function.

Failure to develop normally can lead to the following developmental deposits: Merge 2 sash, 3 leaflets that are thickened and partially collailed in the commission, or one cone-shaped valve.

In case of congenital rubella syndrome, the stenosis of the surface pulmonary and pulmonary arteries are often present. The acquired valve disease is rare. The most common etiologies are carcinoid syndrome, rheumatism and homotransplant dysfunction.

Stenosis can lead to sub-endocardium hypertrophy, causing significant obstruction of outflow and leading to the overload of the right ventricle and pulmonary hypertension. As this process deteriorates, an adult without symptoms gradually becomes symptoms.