Fibroelastosis

The term "fibroelastosis" in medicine refers to changes in the connective tissue of the body, covering the surface of internal organs and blood vessels, due to impaired growth of elastic fibers. In this case, there is a thickening of the walls of organs and their structures, which necessarily affects the functioning of vital systems of the body, in particular cardiovascular and respiratory systems. This in turn leads to deterioration of the patient's well-being, especially during physical exertion, affecting the quality and duration of life.

Epidemiology

In general, diseases accompanied by changes in connective tissue resulting in thickening of the membranes and septa of internal organs can be divided into 2 groups: cardiac fibroelastosis and pulmonary fibroelastosis. Heart pathology can be both congenital and acquired, with the pulmonary form of the disease we are talking about an acquired disease.

Pulmonary fibroelastosis begins to develop in middle age (closer to 55-57 years), although in half of cases the origin of the disease should be sought in childhood. It is characterized by a "light" interval, when the symptoms of the disease are absent. In this case, the disease has no sexual preference and can equally affect both women and men. This rather rare pathology is characterized by changes in the pleura tissue and parenchyma (functional cells) of the lung mainly in the upper lobe of the lung. Since the etiology and pathogenesis of the disease remain unclear, the disease is attributed to idiopathic pathologies. According to medical terminology, it is called "pleuroparenchymatous fibroelastosis". [1]

Cardiac fibroelastosis is a generalized name for the pathology of cardiac membranes characterized by their thickening and decreased functionality. Congenital forms of the pathology are characterized by diffuse (widespread) thickening of the inner lining of the heart. This is a thin connective tissue lining the cavity of the heart (its departments) and forming its valves.

In adult patients, a focal form of the disease is usually diagnosed, when the inner surface of the heart is as if covered with patches of stronger and thicker tissue (it may include not only overgrown fibers, but also thrombotic masses).

In half of the cases of cardiac fibroelastosis, not only the wall of the heart but also the valves (bicuspid mitral between atrium and ventricle, tricuspid aortic between left ventricle and aorta, pulmonic between right ventricle and pulmonary artery) become thicker. This in turn can impair valve function and cause narrowing of the arterial orifice, which is already small compared to other heart cavities.

Endocardial fibroelastosis in medical terminology is called endocardial fibroelastosis (prenatal fibroelastosis, endocardial sclerosis, fetal endocarditis, etc.). But quite often the middle muscular layer of the cardiac membrane can also be involved in the process. [2]

Anomalies of the myocardium (the muscular layer of the heart consisting of cardiomyocytes), gene mutations and severe infectious processes can cause a widespread form of fibroelastosis, when not only the endocardium but also the myocardium is involved in the process. Usually dysplastic processes in the endocardium, caused by various reasons, occur at the border of its contact with the muscle sheath, violating the contractility of this layer. In some cases, there is even an ingrowth of the inner layer into the myocardium, replacement of cardiomyocytes with fibroblasts and fibers, which affects the conduction of nerve impulses and rhythmic operation of the heart.

Compression by thickened myocardium of blood vessels in the thickness of the cardiac membrane impairs myocardial nutrition (myocardial ischemia), which in turn can lead to necrosis of cardiac muscle tissue.

Endocardial fibroelastosis with cardiac myocardial involvement is called subendocardial or endomyocardial fibroelastosis.

According to statistics, most cases of this rare disease (only 0.007% of the total number of newborns) are diagnosed with fibroelastosis of the left ventricle of the heart, although in some cases the process also extends to the right ventricle and atria, including the valves separating them.

Cardiac fibroelastosis is often accompanied by lesions of large coronary vessels, also covered with connective tissue. In adulthood, it often occurs against the background of progressive atherosclerosis of vessels.

The disease is more commonly reported in tropical African countries among populations with a low standard of living, favored by poor nutrition, frequent infections, and certain foods and plants eaten.

Endocardial thickening is also noted in the last stage of Leffler's fibroplastic endocarditis, affecting mainly middle-aged men. The pathogenesis of this disease is also associated with infectious agents that cause the development of severe eosinophilia, which is more characteristic of internal parasitic infections. In this case, the tissues of the body (primarily the heart muscle and brain) begin to experience a lack of oxygen (hypoxia). Despite the similarity of the symptoms of cardiac fibroelastosis and Leffter's fibrous endocarditis, doctors consider them to be completely different diseases.

Causes of the fibroelastosis

Fibroelastosis refers to changes in connective tissue in vital organs: heart and lungs, which is accompanied by organ dysfunction and affects the appearance and condition of the patient. The disease has been known to doctors for decades. Fibroelastosis of the inner membrane of the heart (endocardium) was described in the early 18th century, about similar changes in the lungs began to speak 2 and a half centuries later. Nevertheless, doctors have not reached a final agreement on the causes of pathologic connective tissue overgrowth.

It remains unclear what exactly causes the disorder of growth and development of connective fibers. But scientists have identified certain risk factors for such changes, considering them possible (but not definitive) causes of the disease.

Thus, in the pathogenesis of pulmonary fibroelastosis, which is considered a disease of mature people, a special role is attributed to recurrent infectious lesions of the organ, which are found in half of patients. Infections provoke inflammation of lung and pleura tissues, and prolonged inflammation predisposes to their fibrotic transformation.

Some patients have a history of fibroelastosis in their family, which suggests hereditary predisposition. In their organism nonspecific autoantibodies are found, which provoke long-term inflammatory processes of undetermined etiology.

It is believed that fibrotic changes in lung tissue may be caused by gastroesophageal reflux disease. Although this link is likely to be indirect. It is also thought that the risk of fibroelastosis is also higher in those with cardiovascular disease or pulmonary thrombosis.

Pulmonary fibroelastosis at a young and young age can remind of itself during pregnancy. Usually, the disease lurks for about 10 or more years, but it can manifest itself earlier, possibly due to the increased load on the body of the future mother and hormonal changes, but there is no exact explanation yet. Nevertheless, such a pattern of disease development was observed in 30% of the examined patients of reproductive age.

Pregnancy itself can not cause the disease, but it can accelerate the development of events, which is very sad, because the lethality of the disease is very high, and life expectancy in fibroelastosis is low.

Cardiac fibroelastosis in most cases can be attributed to pediatric diseases. Congenital pathology is detected as early as in the prenatal period in a 4-7-month-old fetus, but the diagnosis can be confirmed only after the birth of the child. In the pathogenesis of this form of the disease, several possible negative factors are considered: infectious-inflammatory diseases of the mother, transmitted to the fetus, anomalies in the development of the heart membranes, impaired blood supply of cardiac tissues, genetic mutations, oxygen deficiency.

It is believed that among infections, the greatest pathogenetic contribution to the development of cardiac fibroelastosis is made by viruses, because they are embedded in the cells of the body, destroy them, change the properties of tissues. The unformed immune system of the fetus can not provide him with protection from these pathogens, unlike the immunity of the future mother. The latter may not experience the consequences of viral infection, while in the fetus transferred intrauterine infection can provoke the appearance of various anomalies.

Some scientists believe that the decisive role in the pathogenesis of the infectious form of fibroelastosis is played by infection affecting the fetus up to 7 months of age. Thereafter, it can only cause inflammatory heart disease (myocarditis, endocarditis).

Abnormalities in the development of the heart's membranes and valves can be provoked by either an inflammatory process or inadequate autoimmune reactions, whereby cells of the immune system begin to attack the body's own cells.

Gene mutations cause improper development of connective tissue, because genes contain information about the structure and behavior of protein structures (in particular, collagen and elastin proteins).

Hypoxia and ischemia of cardiac tissues can be a consequence of abnormal heart development. In this case, we speak of secondary fibroelastosis provoked by congenital heart disease (CHD). These include such anomalies that cause obstruction (impaired patency of the heart and its vessels):

• Stenosis or narrowing of the aorta near the valve,
• Coarctation or segmental narrowing of the aorta at the junction of its arch and descending section,
• Atresia or absence of a natural orifice in the aorta,
• Underdevelopment of heart tissue (most often the left ventricle, less often the right ventricle and atria), which affects the pumping function of the heart.

It is believed that toxicosis in pregnancy may also act as a predisposing factor for fetal fibroelastosis.

In the postnatal period, the development of cardiac fibroelastosis can be promoted by infectious-inflammatory diseases of the organ membranes, hemodynamic disorders as a result of trauma, vascular thromboembolism, myocardial hemorrhage, metabolic disorders (increased fibrin formation, protein and iron metabolism disorders: amyloidosis, hemochromatosis). The same causes cause the development of the disease in adults.

Pathogenesis

Connective tissue is a special tissue of the human body that is part of almost all organs, but is not actively involved in their functions. Connective tissue is attributed to the supporting and protective function. Comprising a kind of skeleton (skeleton, stroma) and limiting the functional cells of the organ, it provides its final shape and size. Having sufficient strength, connective tissue also protects the cells of the organ from destruction and injuries, prevents the penetration of pathogens, with the help of special cells of macrophages absorbs outdated structures: dead tissue cells, foreign proteins, spent blood components, etc.

This tissue can be called auxiliary, because it does not contain cellular elements that ensure the functionality of a particular organ. Nevertheless, its role in the vital activity of the body is quite great. Being part of the shells of blood vessels, connective tissue ensures the safety and functionality of these structures, thanks to which nutrition and respiration (trophic) of the surrounding tissues of the internal environment of the body.

There are several varieties of connective tissue. The sheath covering the internal organs is called loose connective tissue. It is a semi-liquid, colorless substance containing wavy collagen fibers and straight elastin fibers, with various types of cells scattered haphazardly between them. Some of these cells (fibroblasts) are responsible for the formation of fibrous structures, others (endotheliocytes and mast cells) form a translucent matrix of connective tissue and produce special substances (heparin, histamine), others (macrophages) provide phagocytosis, etc.

The second type of fibrous tissue is dense connective tissue that does not contain a large number of individual cells, which in turn is divided into white and yellow tissue. White tissue consists of densely packed collagen fibers (ligaments, tendons, periosteum), and yellow tissue consists of chaotically interwoven elastin fibers with fibroblasts (ligaments, blood vessel sheaths, lungs).

Connective tissues also include: blood, fat, bone and cartilage tissue, but we are not interested in them yet, because, speaking of fibroelastosis, imply changes in fibrous structures. And elastic and elastic fibers contain only loose and dense connective tissues.

Synthesis of fibroblasts and formation of connective tissue fibers from them is regulated at the level of the brain. This ensures the constancy of its characteristics (strength, elasticity, thickness). If due to some pathological reasons the synthesis and development of auxiliary tissue is disturbed (the number of fibroblasts increases, their "behavior" changes), there is an overgrowth of strong collagen fibers or a change in the growth of elastic fibers (they remain short, wriggle), which leads to changes in the properties of the shell of organs and some internal structures covered with connective tissue. They become thicker than necessary, denser, stronger and inelastic, resembling fibrous tissue in ligaments and tendons, which requires great effort to stretch.

Such tissue does not stretch well, limiting the movements of the organ (automatic rhythmic movements of the heart and blood vessels, changes in the size of the lungs during inhalation and exhalation), hence disorders of the blood supply and respiratory organs, entailing oxygen deficiency.

The fact is that the blood supply of the body is carried out thanks to the heart, which works as a pump, and two circulatory circles. The small circulatory circle is responsible for blood supply and gas exchange in the lungs, from where oxygen with the blood flow is delivered to the heart, and from there to the large circulatory circle and spread throughout the body, providing respiration of organs and tissues.

The elastic sheath, limiting the contraction of the heart muscle, reduces the functionality of the heart, which is not as active in pumping blood, and with it oxygen. In case of pulmonary fibroelastosis their ventilation (osigenation) is disturbed, it is clear that less oxygen begins to enter the blood, which even with normal heart function, will contribute to oxygen starvation (hypoxia) of tissues and organs. [3]

Symptoms of the fibroelastosis

Cardiac and pulmonary fibroelastosis are two types of disease characterized by impaired synthesis of fibers within connective tissue. They have different localizations, but both are potentially life-threatening because they are associated with progressive or severe heart and respiratory failure.

Pulmonary fibroelastosis is a rare type of interstitial diseases of this important organ of the respiratory system. These include chronic pathologies of lung parenchyma with lesions of alveolar walls (inflammation, disruption of their structure and structure), inner lining of pulmonary capillaries, etc. Fibroelastosis is often considered as a special rare form of progressive pneumonia with a tendency to fibrotic changes in lung and pleural tissues.

It is almost impossible to detect the disease at the very beginning, because for about 10 years it may not remind of itself in any way. This period is called the light interval. The beginning of pathological changes, which do not yet affect the volume of the lungs and gas exchange, can be detected accidentally, conducting a detailed examination of the lungs in connection with another disease of the respiratory system or trauma.

The disease is characterized by a slow progression of symptoms, so the first manifestations of the disease may be significantly delayed in time from its onset. The symptoms worsen gradually.

Coughing and increasing shortness of breath are the first signs of the disease to look out for. These symptoms are often a consequence of a respiratory illness and may therefore be associated with a cold and its consequences for a long time. Dyspnea is often perceived as a cardiac disorder or age-related changes. However, the disease is diagnosed in people approaching old age.

Mistakes can be made by both patients and doctors examining them, which leads to late detection of a dangerous disease. It is worth paying attention to the cough, which in fibroelastosis is unproductive, but is not stimulated by mucolytics and expectorants, but is controlled by cough suppressants. A prolonged cough of this nature is a characteristic symptom of pulmonary fibroelastosis.

Dyspnea is caused by progressive respiratory failure due to thickening of the alveolar walls and pleura, reduction in the volume and number of alveolar cavities in the lung (the organ parenchyma is seen on X-ray in the form of honeycomb). The symptom intensifies under the influence of physical exertion, first significant, and then even small. As the disease progresses, it worsens, which becomes the cause of disability and death of the patient.

Progression of fibroelastosis is accompanied by deterioration of the general condition: hypoxia leads to weakness and dizziness, body weight decreases (anorexia develops), nail phalanges change like drumsticks, the skin becomes pale, has a painful appearance.

Half of patients have nonspecific symptoms in the form of difficulty breathing and chest pain, characteristic of pneumothorax (accumulation of gases in the pleural cavity). This anomaly can also occur as a consequence of trauma, primary and secondary lung diseases, improper treatment, so it is not possible to make a diagnosis based on it.

For fibroelastosis of the heart as well as for the pathology of growth of connective tissue of the lungs, characterized by: pale skin, weight loss, weakness, which often has an attack-like character, dyspnea. There may also be a persistent subfebrile temperature without signs of cold or infection.

Many patients experience a change in the size of the liver. It becomes enlarged without symptoms of dysfunction. Swelling of the legs, face, arms, and sacral region is also possible.

The characteristic manifestation of the disease is considered to be increasing circulatory insufficiency associated with cardiac dysfunction. In this case, tachycardia (increase in the number of heartbeats often combined with arrhythmias), shortness of breath (including in the absence of physical exertion), cyanosis of tissues (blue coloring caused by the accumulation of carboxyhemoglobin in the blood, i.e. A compound of hemoglobin with carbon dioxide due to impaired blood flow and, consequently, gas exchange) are diagnosed.

At the same time, symptoms can appear both immediately after the birth of a child with this pathology, and for some time. In older children and adults, signs of severe heart failure usually appear against the background of respiratory tract infection, which acts as a trigger. [4]

Fibroelastosis in children

If pulmonary fibroelastosis is a disease of adults, often beginning in childhood, but for a long time does not remind about itself, this pathology of the endocardium of the heart often appears before the birth of the baby and affects his life from the first moments of birth. This rare but severe pathology causes the development of difficult to correct heart failure in infants, many of whom die within 2 years. [5]

Endocardial fibroelastosis in newborns in most cases is the result of pathological processes occurring in the baby's body while still in utero. Infections received from the mother, genetic mutations, abnormalities in the development of the cardiovascular system, hereditary metabolic diseases - all this, according to scientists, can lead to changes in the connective tissue in the heart membranes. Especially if a 4-7-month-old fetus is exposed to two or more factors at the same time.

For example, a combination of anomalies of the heart and coronary vessels (stenosis, atresia, coarctation of the aorta, abnormal development of myocardial cells, endocardial weakness, etc.), contributing to tissue ischemia, combined with an inflammatory process due to infection practically leave the child no chance for more or less life. If the defects in the development of the organ can still somehow be promptly corrected, the progressive fibroelastosis can only be slowed down, but not cured.

Fetal cardiac fibroelastosis is usually detected already during pregnancy during the second to third trimester ultrasonography. Ultrasound and echocardiography at 20 to 38 weeks showed hyperechogenicity, which indicates thickening and thickening of the endocardium (more often diffuse, less often focal), changes in the size and shape of the heart (the organ is enlarged in size and takes the shape of a ball or bullet, internal structures are gradually flattened). [6]

In 30-35% of cases fibroelastosis was detected before 26 weeks of gestation, in 65-70% - in the subsequent period. In more than 80% of newborns, fibroelastosis is combined with obstructive heart defects, i.e. It is secondary, despite its early detection. Left ventricular hyperplasia was detected in half of the sick children, which explains the high prevalence of fibroelastosis of this heart structure. Pathologies of the aorta and its valve, detected in one third of children with endocardial overgrowth, also lead to enlargement (dilatation) of the left ventricular chamber and impairment of its functionality.

When cardiac fibroelastosis is instrumentally confirmed, doctors recommend termination of pregnancy. Almost all born children whose mothers refused medical abortion have confirmed signs of the disease. Symptoms of heart failure, characteristic of fibroelastosis, appear within a year (rarely in the 2-3 year of life). In children with the combined form of the disease, signs of heart failure are detected from the first days of life.

Congenital forms of primary and combined fibroelastosis in children most often have a rapid course with the development of severe heart failure. Ill health is indicated by low activity, lethargy of the child, refusal of the breast due to rapid fatigue, poor appetite, increased sweating. All this leads to the fact that the child does not gain weight well. The baby's skin is painfully pale, some with a bluish tint, most often in the area of the nasolabial triangle.

There are signs of poor immunity, so such children often and quickly catch respiratory infections, complicating the situation. Sometimes in the first days and months of life the child is not diagnosed with circulatory disorders, but frequent infections and lung diseases become a trigger for congestive heart failure.

Additional health examinations of newborns and infants with suspected or previously diagnosed fibroelastosis showed low blood pressure (hypotension), increased heart size (cardiomegaly), muffled tones on cardiac examination, sometimes a systolic murmur characteristic of mitral valve insufficiency, tachycardia, and dyspnea. Lung audition shows the presence of wheezing, indicating congestion.

Endocardial damage to the left ventricle often results in weakening of the muscle layer of the heart (myocardium). The normal heart rhythm consists of two rhythmically alternating tones. In fibroelastosis, a third (and sometimes a fourth) tone may appear. This pathological rhythm is well audible and resembles a three-stroke horse gallop, therefore it is called a gallop rhythm.

Another symptom of fibroelastosis in young children can be considered the appearance of a heart hump. The fact is that the child's ribs in the early postnatal period remain neo-osteal and are represented by cartilaginous tissue. Increase in the size of the heart leads to the fact that it begins to press on the "soft" ribs, as a result of which they bend and take a constant bent forward shape (heart hump). In adults with fibroelastosis, the heart hump does not form due to the strength and stiffness of the rib bone, even if all heart structures are enlarged.

By itself, the formation of a heart hump indicates only a congenital heart defect without specifying its nature. But in any case, it is associated with an increase in the size of the heart and its ventricles.

Edema syndrome in fibroelastosis in children is rarely diagnosed, but many babies have an enlarged liver, which begins to protrude an average of 3 cm from under the edge of the rib arch.

If fibroelastosis is acquired (e.g., as a consequence of inflammatory diseases of the heart lining), the clinical picture is most often slowly progressive. For some time, there may be no symptoms at all, then there are mild signs of cardiac dysfunction in the form of shortness of breath during exercise, increased heart rate, rapid fatigue and low physical endurance. A little later, the liver begins to enlarge, edema and dizzy spells appear.

All symptoms of acquired fibroelastosis are nonspecific, which makes it difficult to diagnose the disease, reminiscent of cardiomyopathies or liver and kidney diseases. The disease is most often diagnosed at the stage of severe heart failure, which negatively affects the results of treatment.

Complications and consequences

It should be said that fibroelastosis of the heart and lungs are severe pathologies, the course of which depends on different circumstances. Congenital heart defects, which can be surgically corrected at an early age, complicate the situation considerably, but there is still a rather high risk of death (about 10%).

It is believed that the earlier the disease develops, the more severe its consequences will be. This is confirmed by the fact that congenital fibroelastosis in most cases has a lightning or acute course with rapid progression of heart failure. The development of acute CH in a child under 6 months of age is considered a poor prognostic sign.

In this case, treatment does not guarantee full recovery of cardiac function, but only inhibits the progression of symptoms of heart failure. On the other hand, the absence of such supportive treatment leads to death during the first two years of life of the baby.

If heart failure is detected in the first few days or months of a baby's life, it is likely that the baby will not live for a week. The response to treatment varies among children. In the absence of therapeutic effect, there is virtually no hope. But with the help provided, the life expectancy of a sick child is short (from several months to several years).

Surgical intervention and correction of congenital heart defects that caused fibroelastosis usually improves the patient's condition. With successful surgical treatment of hyperplasia of the left ventricle of the heart and fulfillment of the requirements of the doctor, the disease can acquire a benign course: heart failure will have a chronic course without signs of progression. Although there is little hope for such an outcome.

As for the acquired form of cardiac fibroelastosis, it quickly acquires a chronic course and gradually progresses. Drug treatment can slow down the process, but not stop it.

Lung fibroelastosis regardless of the time of appearance of changes in the parenchyma and membranes of the organ after the light period begins to progress rapidly and actually kills a person in a couple of years, provoking severe respiratory failure. The sad thing is that effective methods of treatment of the disease have not yet been developed. [7]

Diagnostics of the fibroelastosis

Endomyocardial fibroelastosis, the symptoms of which are mostly detected at an early age, is a congenital disease. If we exclude those rare cases when the disease began to develop in older childhood and adulthood as a complication of trauma and somatic diseases, the pathology can be detected in the prenatal period, i.e., before birth.

Doctors believe that pathological changes in the endocardial tissues, changes in the shape of the fetal heart and some features of its work, characteristic of fibroelastosis, can be detected as early as 14 weeks of pregnancy. But this is still quite a small period, and it can not be excluded that the disease can manifest itself somewhat later, closer to the third trimester of pregnancy, and sometimes even a couple of months before delivery. For this reason, it is recommended that clinical ultrasound screening of the fetal heart be performed at intervals of a few weeks when monitoring pregnant women.

On what signs can doctors suspect the disease during the next ultrasound? Much depends on the form of the disease. Most often, fibroelastosis is diagnosed in the area of the left ventricle, but not always this structure turns out to be enlarged. Dilated form of the disease with an increase in the left ventricle of the heart is easily determined during ultrasound examination by the spherical shape of the heart, the apex of which is represented by the left ventricle, a general increase in the size of the organ, bulging of the interventricular septum towards the right ventricle. But the main sign of fibroelastosis is the thickening of the endocardium, as well as cardiac septa with a characteristic increase in the echogenicity of these structures, which is determined through a specific ultrasound study.

The study is performed using special ultrasound equipment with cardiology programs. Fetal echocardiography is not harmful to the mother and unborn child, but it allows to detect not only anatomical changes in the heart, but also to determine the state of the coronary vessels, the presence of blood clots in them, changes in the thickness of the cardiac membranes.

Fetal echocardiography is prescribed not only in the presence of abnormalities during the interpretation of ultrasound results, but also in the case of a mother's infection (especially viral), intake of potent drugs, hereditary predisposition, the presence of metabolic disorders, as well as congenital cardiac pathologies in older children.

Fetal echocardiography can be used to detect other congenital forms of fibroelastosis. For example, right ventricular fibroelastosis, a widespread process with simultaneous involvement of the left ventricle and adjacent structures: right ventricle, heart valves, atria, combined forms of fibroelastosis, endomyocardial fibroelastosis with thickening of the ventricular inner membrane and involvement of part of the myocardium (usually combined with wall thrombosis).

Endocardial fibroelastosis detected prenatally has a very poor prognosis, so doctors recommend termination of pregnancy in this case. The possibility of an erroneous diagnosis is excluded through a repeat ultrasound of the fetal heart, which is carried out 4 weeks after the first examination that revealed the pathology. It is clear that the final decision on the termination or preservation of pregnancy is left to the parents, but they should be aware of the life to which they are condemning the child.

Endocardial fibroelastosis is not always detected during pregnancy, especially if we take into account the fact that not all future mothers become registered at the women's consultation and undergo preventive ultrasound diagnostics. The disease of the child in her womb practically does not affect the condition of the pregnant woman, so the birth of a sick baby often becomes an unpleasant surprise.

In some cases, both parents and doctors learn about the baby's illness several months after the baby is born. In this case, laboratory blood tests may show nothing, except for an increase in sodium concentration (hypernatremia). But their results will be useful in differential diagnosis to exclude inflammatory diseases.

There is hope for instrumental diagnostics. A standard cardiac examination (ECG) is not particularly revealing in cases of fibroelastosis. It helps to identify disorders of the heart and electrical conduction of the heart muscle, but does not specify the causes of such disorders of functioning. So the change in ECG voltages (in younger age it is usually underestimated, in older - on the contrary, excessively high) indicates cardiomyopathy, which may be associated not only with heart pathologies, but also with metabolic disorders. Tachycardia is a symptom of cardiologic diseases. And when both ventricles of the heart are affected, the cardiogram may appear normal at all. [8]

Computed tomography (CT) is an excellent non-invasive tool to detect cardiovascular calcification and rule out pericarditis. [9]

Magnetic resonance imaging (MRI) may be useful in detecting fibroelastosis because biopsy is invasive. A hypointense rim in a myocardial perfusion sequence and a hyperintense rim in a delayed-enhancement sequence indicate fibroelastosis. [10]

But this does not mean that the study should be abandoned, because it helps to determine the nature of the heart work and the degree of developing heart failure.

When symptoms of heart failure appear and the patient is referred to a doctor, the patient is also prescribed: chest X-ray, computer or magnetic resonance imaging of the heart, echocardiography (echocardiography). In doubtful cases, it is necessary to resort to biopsy of heart tissue with subsequent histologic examination. The diagnosis is very serious, so it requires the same approach to diagnosis, although treatment differs little from symptomatic therapy of CHD and heart failure.

But even such a meticulous examination will not be useful if its results are not used in the differential diagnosis. ECG results can be used to differentiate acute fibroelastosis from idiopathic myocarditis, exudative pericarditis, aortic stenosis. In this case, laboratory studies will not show signs of inflammation (leukocytosis, elevated COE, etc.), and temperature measurements will not show hyperthermia.

To distinguish endocardial fibroelastosis from isolated mitral valve insufficiency and mitral heart disease helps to analyze heart tones and murmurs, changes in atrial size, and history.

Analysis of anamnestic data is useful in differentiating between fibroelastosis and cardiac and aortic stenosis. In aortic stenosis, the preservation of sinus rhythm and the absence of thromboembolism are also worth noting. Heart rhythm disturbance and thrombus deposition are not observed in exudative pericarditis, but the disease is manifested by fever and fever.

Differentiation of endocardial fibroelastosis and congestive cardiomyopathy causes the greatest difficulty. In this case, although fibroelastosis in most cases is not accompanied by marked cardiac conduction disturbances, it has a less favorable prognosis of treatment.

With combined pathologies, it is necessary to pay attention to any abnormalities detected during a CT scan or ultrasound of the heart, because congenital malformations significantly complicate the course of fibroelastosis. If combined endocardial fibroelastosis is detected in the intrauterine period, it is inappropriate to preserve pregnancy. It is much more humane to terminate it.

Diagnosis of pulmonary fibroelastosis

Diagnosing pulmonary fibroelastosis also requires from the doctor certain knowledge and skills. The fact is that the symptoms of the disease are quite heterogeneous. On the one hand, they indicate congestive lung disease (unproductive cough, shortness of breath), and on the other hand may be a manifestation of cardiac pathology. Therefore, the diagnosis of the disease cannot be reduced only to the statement of symptoms and auscultation.

Blood tests of the patient help to exclude inflammatory lung diseases, but do not provide information about quantitative and qualitative changes in tissues. The presence of signs of eosinophilia help to differentiate the disease from similar manifestations of pulmonary fibrosis, but do not reject or confirm the fact of fibroelastosis.

More indicative are considered instrumental studies: radiography of the lungs and tomographic study of the respiratory organs, as well as functional tests consisting in the determination of respiratory volumes, vital capacity of the lungs, pressure in the organ.

In pulmonary fibroelastosis, it is worth paying attention to the decrease in external respiratory function measured during spirometry. Reduction of active alveolar cavities has a noticeable effect on the vital capacity of the lungs (VC), and thickening of the walls of internal structures - on the diffusion capacity of the organ (DCL), which provides ventilation and gas exchange functions (in simple words, to absorb carbon dioxide from the blood and give oxygen).

Characteristic features of pleuroparenchymal fibroelastosis are a combination of restricted air entry into the lungs (obstruction) and impaired lung expansion on inspiration (restriction), deterioration of external respiratory function, moderate pulmonary hypertension (increased pressure in the lungs), diagnosed in half of patients.

Biopsy of lung tissue shows characteristic changes in the internal structure of the organ. These include: fibrosis of pleura and parenchyma combined with elastosis of alveolar walls, accumulation of lymphocytes in the area of compacted partitions of alveoli, transformation of fibroblasts into uncharacteristic for them muscle tissue, the presence of edematous fluid.

The tomogram shows lung damage in the upper parts of the lungs in the form of foci of pleural thickening and structural changes in the parenchyma. Overgrown connective tissue of lungs by color and properties resembles muscular, but the volume of lungs is reduced. Quite large air-containing cavities (cysts) are found in the parenchyma. Irreversible focal (or diffuse) expansion of bronchi and bronchioles (traction bronchiectasis), low standing of the diaphragm dome are characteristic.

Radiologic studies in many patients reveal areas of "frosted glass" and "honeycomb lung", indicating uneven lung ventilation due to the presence of foci of tissue thickening. About half of patients have enlarged lymph nodes and liver.

Fibroelastosis of the lung should be differentiated from fibrosis caused by parasitic infection and associated eosinophilia, endocardial fibroelastosis, lung diseases with impaired ventilation and the picture of a "honeycomb lung", autoimmune disease histiocytosis X (one form of this pathology with lung damage is called Hend-Schuller-Krischen disease), manifestations of sarcoidosis and pulmonary tuberculosis.

Treatment of the fibroelastosis

Fibroelastosis, whatever its localization, is considered a dangerous and virtually incurable disease. Pathological changes in the pleura and lung parenchyma cannot be restored by medication. And even the use of hormonal anti-inflammatory drugs (corticosteroids) in combination with bronchodilators does not give the desired result. Bronchodilators help to slightly relieve the patient's condition, relieving the obstructive syndrome, but they do not affect the processes occurring in the lungs, so they can be used only as supportive therapy.

Surgical treatment for pulmonary fibroelastosis is also ineffective. The only surgery that could change the situation is transplantation of a donor organ. But lung transplantation, alas, still has the same unfavorable prognosis. [11]

According to foreign scientists, fibroelastosis can be considered as one of the frequent complications of lung or bone marrow stem cell transplantation. In both cases, there are changes in the fibers of connective tissue of the lungs, affecting the function of external respiration.

The disease without treatment (and there is no effective treatment to date) progresses, and within 1.5-2 years about 40% of patients die of respiratory failure. The life expectancy of those who remain is also severely limited (up to 10-20 years), as well as the ability to work. The person becomes disabled.

Cardiac fibroelastosis is also considered a medically incurable disease, especially if it is a congenital pathology. Usually children do not live to the age of 2 years. They can only be saved by heart transplantation, which in itself is a difficult operation with a high degree of risk and unpredictable consequences, especially at such a young age.

Surgically in some babies it is possible to correct congenital heart anomalies, so that they do not aggravate the condition of the sick child. With arterial stenosis, it is practiced to install a vessel dilator - a shunt (aortocoronary bypass). When the left ventricle of the heart is dilated, its shape is promptly restored. But even such an operation does not guarantee that the child can do without transplantation. About 20-25% of babies survive, but they suffer from heart failure all their lives, i.e. They are not considered healthy.

If the disease is acquired, it is worth fighting for the life of the child with the help of medication. But it should be understood that the earlier the disease appears, the more difficult it will be to fight it.

Medication treatment is aimed at combating and preventing exacerbations of heart failure. Patients are prescribed such cardiac drugs:

• Angiotensin-converting enzyme (ACE) inhibitors that affect blood pressure and keep it normal (captopril, enalapril, benazepril, etc.),
• Beta-adrenoblockers used for treatment of heart rhythm disorders, arterial hypertension, prevention of myocardial infarction (anapriline, bisoprolol, metoprolol),
• Cardiac glycosides, which with prolonged use not only support heart function (increase potassium content in cardiomyocytes and improve myocardial conduction), but also can slightly reduce the degree of endocardial thickening (digoxin, pitoxin, strophanthin),
• Potassium-saving diuretics (spironolactone, verospiron, dekriz), preventing tissue edema,
• Antithrombotic therapy with anticoagulants (cardiomagnil, magnicor), preventing the formation of blood clots and impaired blood circulation in the coronary vessels.

In congenital endocardial fibroelastosis, ongoing supportive treatment does not promote recovery but reduces the risk of death from heart failure or thromboembolism by 70-75%. [12]

Medications

As we can see, the treatment of endocardial fibroelastosis does not differ much from that of heart failure. In both cases, cardiologists take into account the severity of cardiopathy. The prescription of drugs is strictly individualized, taking into account the patient's age, comorbidities, form and degree of heart failure.

There are 5 groups of drugs used in the treatment of acquired endocardial fibroelastosis. Let's consider one drug from each group.

"Enalapril" - a drug from the group of ACE inhibitors, available in the form of tablets of different dosage. The drug increases coronary blood flow, dilates arteries, reduces blood pressure without affecting cerebral circulation, slows and reduces the expansion of the left ventricle of the heart. The drug improves blood supply to the myocardium, reducing the effects of ischemia, slightly reduces blood coagulation, preventing the formation of blood clots, has a slight diuretic effect.

In heart failure, the medicine is prescribed for a period of more than six months or on a permanent basis. The drug is started with the minimum dose (2.5 mg), gradually increasing it by 2.5-5 mg every 3-4 days. The permanent dose will be the one that is well tolerated by the patient and maintains blood pressure within normal limits.

The maximum daily dose is 40 mg. It can be taken once or divided into 2 doses.

In the event that the BP is set below normal, the dose of the drug is gradually reduced. Abruptly discontinue treatment with "Enalapril" is not allowed. It is recommended to take a maintenance dose of 5 mg per day.

The drug is intended for treatment of adult patients, but it can be prescribed to a child (safety is not officially established, but in case of fibroelastosis, the life of a small patient is at stake, so the risk ratio is taken into account). ACE inhibitor is not prescribed to patients with intolerance to the drug components, in porphyria, pregnancy and during breastfeeding. If the patient has previously had Quincke's edema on the background of taking any drugs of this group, "Enalapril" is prohibited.

Caution should be observed when prescribing the drug to patients with concomitant pathologies: severe kidney and liver disease, hyperkalemia, hyperaldosteronism, aortic or mitral valve stenosis, systemic pathologies of connective tissue, cardiac ischemia, cerebral disease, diabetes mellitus.

During treatment with the drug do not take conventional diuretics to avoid dehydration and strong hypotensive effect. Concomitant administration with potassium-saving diuretics requires dose adjustment, as there is a high risk of hyperkalemia, which in turn provokes heart rhythm disorders, convulsive seizures, decreased muscle tone, increased weakness, etc.

The drug "Enalapril" is usually tolerated quite well, but some patients may develop side effects. The most common are considered to be: severe decrease in blood pressure up to collapse, headaches and dizziness, sleep disturbances, increased fatigue, reversible impairment of balance, hearing and vision, tinnitus, shortness of breath, coughing without sputum production, changes in blood and urine, usually indicating incorrect liver and kidney function. Possible: hair loss, decreased sex drive, symptoms of "hot flashes" (feeling of heat and heart palpitations, hyperemia of the skin of the face, etc.).

"Bisoprolol" - beta-adrenoblocker with selective action, which has a hypotensive and anti-ischemic effect, helps to combat manifestations of tachycardia and arrhythmia. Budget means in the form of tablets, preventing the progression of heart failure in endocardial fibroelastosis. [13]

Like many other drugs prescribed for CHD and CHF, "Bisoprolol" is prescribed for a long time. It is desirable to take it in the morning hours before or during meals.

As for the recommended dosages, they are selected individually depending on the BP values and those drugs that are prescribed in parallel with this drug. On average, the single (aka daily) dose is 5-10 mg, but in case of a slight increase in pressure can be reduced to 2.5 mg. The maximum dose that can be administered to a patient with normally functioning kidneys is 20 mg, but only in case of stable high BP.

Increase of the indicated dosages is possible only with the permission of a doctor. But in severe liver and kidney diseases 10 mg is considered the maximum permissible dose.

In complex treatment of heart failure against the background of left ventricular dysfunction, which most often occurs in fibroelastosis, the effective dose is selected by gradually increasing the dosage by 1.25 mg. At the same time, start with the lowest possible dose (1.25 mg). Dosage increase is carried out at intervals of 1 week.

When the dose reaches 5 mg, the interval is increased to 28 days. After 4 weeks, the dose is increased by 2.5 mg. Adhering to such an interval and norm, it is reached 10 mg, which the patient will have to take for a long time or permanently.

If this dosage is poorly tolerated, it is gradually reduced to a comfortable dosage. Withdrawal from beta-blocker treatment should also not be abrupt.

The drug should not be administered in case of hypersensitivity to the active and excipients of the drug, acute and decompensated heart failure, cardiogenic shock, 2-3 degree antrioventricular block, bradycardia, stable low blood pressure and some other cardiac pathologies, severe bronchial asthma, bronchoobstruction, severe peripheral circulation disorders, metabolic acidosis.

Caution should be observed when prescribing complex treatment. Thus, it is not recommended to combine "Bisoprolol" with some antiarrhythmic agents (quinidine, lidocaine, phenytoin, etc.), calcium antagonists and central hypotensive drugs.

Unpleasant symptoms and disorders that are possible during treatment with "Bisoprolol": increased fatigue, headaches, hot flashes, sleep disorders, pressure drop and dizziness when getting out of bed, hearing impairment, GI symptoms, liver and kidney disorders, decreased potency, muscle weakness and cramps. Sometimes patients complain of peripheral circulation disorders, which manifests as a decrease in temperature or numbness of the extremities, especially fingers and toes.

In the presence of concomitant diseases of the bronchopulmonary system, kidneys, liver, diabetes mellitus, the risk of adverse effects is higher, indicating an exacerbation of the disease.

"Digoxin" is a popular budget cardiac glycoside based on the foxglove plant, which is dispensed strictly on the prescription of a doctor (in tablets) and should be used under his supervision. Injection treatment is carried out in hospital conditions in case of exacerbation of CHD and CHF, tablets are prescribed on a permanent basis in minimally effective doses, since the drug has a toxic and drug-like effect.

The therapeutic effect is to change the strength and amplitude of myocardial contractions (gives the heart energy, supports it in conditions of ischemia). The drug also has vasodilatory (reduces congestion) and some diuretic action, which helps to relieve edema and reduce the strength of respiratory failure, manifested as dyspnea.

The danger of "Digoxin" and other cardiac glycosides is that in overdose they can provoke heart rhythm disorders caused by increased excitability of the myocardium.

In the exacerbation of CHD, the drug is administered as injections, selecting an individual dosage taking into account the severity of the condition and age of the patient. When the condition stabilizes, the drug is switched to tablets.

Usually, the standard single dose of the drug is 0.25 mg. The frequency of administration can vary from 1 to 5 times a day with equal intervals. In the acute stage of CHF, the daily dose can reach 1.25 mg, when the condition stabilizes on a permanent basis, it is necessary to take a maintenance dose of 0.25 (less often 0.5) mg per day.

When prescribing the drug for children, the weight of the patient is taken into account. The effective and safe dose is calculated as 0.05-0.08 mg per kg of body weight. But the drug is not administered continuously, but for 1-7 days.

The dosage of cardiac glycoside should be prescribed by a doctor, taking into account the condition and age of the patient. In this case, it is very dangerous to adjust doses on your own or take 2 drugs with such action at the same time.

"Digoxin" is not prescribed in unstable angina pectoris, marked heart rhythm disturbances, AV block of the heart 2-3 degree, cardiac tamponade, Adams-Stokes-Morganian syndrome, isolated bicuspid valve stenosis and aortic stenosis, congenital heart anomaly called Wolff-Parkinson-White syndrome, hypertrophic obstructive cardiomyopathy, endo, peri- and myocarditis, thoracic aortic aneurysm, hypercalcemia, hypokalemia and some other pathologies. The list of contraindications is quite large and includes syndromes with multiple manifestations, so the decision on the possibility of using this drug can only be made by a specialist.

Digoxin also has side effects. These include heart rhythm disorders (as a consequence of incorrectly selected dose and overdose), deterioration of appetite, nausea (often with vomiting), stool disorders, severe weakness and high fatigue, headaches, the appearance of "flies" in front of the eyes, decreased platelet count and blood clotting disorders, allergic reactions. Most often, the appearance of these and other symptoms is associated with taking large doses of the drug, less often with prolonged therapy.

"Spironolactone" refers to mineralcorticoid antagonists. It has a diuretic effect, promoting the excretion of sodium, chlorine and water, but retaining potassium, necessary for the normal functioning of the heart, since its conductive function is based mainly on this element. Helps to relieve edema. Used as an aid in congestive heart failure.

The drug is administered depending on the phase of the disease. In acute exacerbation, the drug may be administered as injections and in tablets in a dosage of 50-100 mg per day. When the condition is stabilized, a maintenance dose of 25-50 mg is prescribed for a long time. If the balance of potassium and sodium is disturbed in the direction of reduction of the former, the dose can be increased until the normal concentration of trace elements is established.

In pediatrics, the calculation of effective dosage is based on the ratio of 1-3 mg of spironolactone for each kilogram of body weight of the patient.

As we can see, here, too, the selection of the recommended dose is individualized, as well as in the prescription of many other drugs used in cardiology.

Contraindications to the use of diuretics may be: excess potassium or low sodium level in the body, pathology associated with the absence of urination (anuria), severe kidney disease with impaired kidney function. The drug is not prescribed to pregnant women and nursing mothers, as well as those who have intolerance to the components of the drug.

Caution in the use of the drug should be observed in patients with AV heart block (possible exacerbation), excess calcium (hypercalcemia), metabolic acidosis, diabetes mellitus, menstrual disorders, liver disease.

Taking the drug may cause headaches, drowsiness, disorders of balance and coordination of movements (ataxia), enlargement of mammary glands in men (gynecomastia) and impaired potency, changes in menstruation patterns, coarsening of voice and excessive hair loss in women (hirsutism), epigastric pain and GI disorders, intestinal colic, kidney and mineral balance disorders. Skin and allergic reactions are possible.

Usually adverse symptoms are observed when the required dosage is exceeded. Swelling may occur in case of insufficient dosage.

"Magnicor" - a drug that prevents the formation of blood clots, based on acetylsalicylic acid and magnesium hydroxide. One of the effective means of antithrombotic therapy prescribed for heart failure. It has analgesic, anti-inflammatory, antiaggregant effect, affects respiratory function. Magnesium hydroxide reduces the negative effect of acetylsalicylic acid on the mucosa of the GI tract.

In endomyocardial fibroelastosis, the drug is prescribed for prophylactic purposes, so the minimum effective dose is 75 mg, which corresponds to 1 tablet. In cardiac ischemia due to thrombosis and subsequent narrowing of the coronary lumen, the initial dose is 2 tablets, and the maintenance dose corresponds to the prophylactic dose.

Exceeding recommended dosages significantly increases the risk of bleeding that is difficult to stop.

Dosages are indicated for adult patients due to the fact that the drug contains acetylsalicylic acid, the administration of which under the age of 15 years may have severe consequences.

The drug is not administered to patients of pediatric and young adolescent age, in case of intolerance to acetylsalicylic acid and other components of the drug, "aspirin" asthma (in anamnesis), acute course of erosive gastritis, peptic ulcer, hemorrhagic diathesis, severe liver and kidney diseases, in case of severe decompensated heart failure.

In pregnancy, "Magnicor" is prescribed only if absolutely necessary and only in the 1-2 trimester, taking into account the possible negative effect on the fetus and the course of pregnancy. In the 3rd trimester of pregnancy, such treatment is undesirable, because it contributes to a decrease in the contractility of the uterus (prolonged labor) and can cause severe bleeding. The fetus may suffer from pulmonary hypertension and kidney dysfunction.

Side effects of the drug include symptoms on the side of the GI tract (dyspepsia, epigastric and abdominal pain, some risk of gastric bleeding with the development of iron deficiency anemia). Nasal bleeding, bleeding of gums and urinary system organs are possible against the background of drug administration,

In case of overdose, dizziness, fainting, ringing in the ears are possible. Allergic reactions are not uncommon, especially against the background of hypersensitivity to salicylates. But anaphylaxis and respiratory failure are rare adverse reactions.

Selection of drugs as part of complex therapy and recommended dosages should be strictly individualized. Particular caution should be exercised in the treatment of pregnant women, nursing mothers, children and elderly patients.

Folk treatment and homeopathy

Cardiac fibroelastosis is a serious and severe disease with a characteristic progressive course and practically no chance of recovery. It is clear that effective treatment of such a disease with folk remedies is impossible. Folk medicine recipes, which are mainly reduced to the treatment of herbs, can be used only as auxiliary means and only with the permission of the doctor, so as not to complicate the already poor prognosis.

As for homeopathic remedies, their use is not prohibited, and can be part of the complex treatment of heart failure. However, in this case it is not so much about treatment as about the prevention of progression of CHF.

The drugs should be prescribed by an experienced homeopath, and questions about the possibility of their inclusion in the complex therapy are in the competence of the attending physician.

What homeopathic remedies help to delay the progression of heart failure in fibroelastosis? In acute heart failure, homeopaths turn to the following remedies: Arsenicum album, Antimonium tartaricum, Carbo vegetabilis, Acidum oxalicum. Despite the similarity of indications in choosing an effective drug doctors rely on the external manifestations of ischemia in the form of cyanosis (its degree and prevalence) and the nature of the pain syndrome.

In CCN, maintenance therapy may include: Lahesis and Nayu, Lycopus (in the initial stages of cardiac enlargement), Laurocerazus (for dyspnea at rest), Latrodectus mactans (for valve pathologies), hawthorn preparations (especially useful in endomyocardial lesions).

In case of palpitations, for symptomatic treatment may be prescribed: Spigelia, Glonoinum (for tachycardia), Aurum metallicum (for hypertension).

Grindelia, Spongia, and Lahegis can be used to reduce the severity of shortness of breath. To control cardiac pain may be prescribed: Cactus, Cereus, Naja, Cuprum, to relieve anxiety on this background - Aconitum. In the development of cardiac asthma are indicated: Digitalis, Laurocerazus, Lycopus.

Prevention

Prevention of acquired fibroelastosis of the heart and lungs consists in the prevention and timely treatment of infectious and inflammatory diseases, especially when it comes to affecting vital organs. Effective treatment of the underlying disease helps prevent dangerous consequences, which is exactly what fibroelastosis is. This is an excellent reason to take good care of your health and the health of the next generations, the so-called work for a healthy future and longevity.

Forecast

Changes in connective tissue in cardiac and pulmonary fibroelastosis are considered irreversible. Although some drugs with long-term therapy can slightly reduce endocardial thickness, they do not guarantee a cure. Although the condition is not always fatal, the prognosis is still relatively unfavorable. The 4-year survival rate is 77%. [14]

The worst prognosis, as we have already mentioned, is in congenital cardiac fibroelastosis, where manifestations of heart failure are already visible in the first weeks and months of the child's life. Only heart transplantation can save the baby, which in itself is a risky operation in such an early period, and it should be done before the age of 2 years. Such children usually do not live any longer.

Other surgeries can only avoid early death of the child (and not always), but cannot completely cure the child of heart failure. Death occurs with decompensation and respiratory failure.

The prognosis of pulmonary fibroelastosis depends on the course of the disease. In the lightning development of symptoms, the chances are extremely low. If the disease progresses gradually, the patient may live about 10-20 years, until the onset of respiratory failure due to changes in the alveoli of the lungs.

Many intractable pathologies can be avoided by following preventive measures. In the case of cardiac fibroelastosis, it is primarily the prevention of those factors that can affect the development of the heart and circulatory system of the fetus (with the exception of hereditary predisposition and mutations, before which the doctors are powerless). If they could not be avoided, early diagnosis helps to detect pathology at a stage when it is possible to terminate the pregnancy, which in this situation is considered humane.