^

Health

A
A
A

Fibroelastosis

 
, medical expert
Last reviewed: 29.06.2022
 
Fact-checked
х

All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.

We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.

If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.

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 a violation of the growth of elastic fibers. At the same time, there is a thickening of the walls of organs and their structures, which necessarily affects the functioning of the vital systems of the body, in particular the cardiovascular and respiratory systems. This, in turn, entails a deterioration in the patient's well-being, especially during physical exertion, affecting the quality and duration of life.

Epidemiology

In general, diseases accompanied by changes in the connective tissue, as a result of which there is a thickening of the membranes and partitions of the internal organs, can be divided into 2 groups: fibroelastosis of the heart and fibroelastosis of the lung. Heart pathology can be both congenital and acquired, with a pulmonary form of the disease, we are talking about an acquired disease.

Fibroelastosis of the lungs begins to develop in middle age (closer to 55-57 years), although in half of the cases the source of the disease must be sought in childhood. A "light" period is characteristic, when the symptoms of the disease are absent. At the same time, the disease does not have sexual preferences and can equally affect both women and men. This rather rare pathology is characterized by changes in the tissues of the pleura and parenchyma (functional cells) of the lung, mainly in the upper lobe of the lung. Since the etiology and pathogenesis of the disease remain unexplained, the disease is referred to as idiopathic pathologies. According to medical terminology, it is called "pleuroparenchymal fibroelastosis". [1]

Fibroelastosis of the heart is a generalized name for the pathology of the heart membranes, characterized by their thickening and reduced functionality. For congenital forms of pathology, a diffuse (common) thickening of the inner lining of the heart is characteristic. 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 seems to be 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 fibroelastosis of the heart, not only the wall of the organ becomes thicker, but also the valves (bicuspid mitral between the atrium and ventricle of the same name, tricuspid aortic between the left ventricle and aorta, pulmonary between the right ventricle and pulmonary artery). This, in turn, can disrupt the functioning of the valves and cause narrowing of the arterial orifice, which is already small compared to other cavities of the heart.

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 heart membrane can also be involved in the process. [2]

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

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

Fibroelastosis of the endocardium with damage to the myocardium of the heart is called subendocardial or endomyocardial fibroelastosis.

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

Fibroelastosis of the heart is often accompanied by damage to large coronary vessels, also covered with connective tissue. In adulthood, it often occurs against the background of progressive vascular atherosclerosis.

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

Thickening of the endocardium is also noted in the last stage of Loeffler's fibroplastic endocarditis, which mainly affects 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 symptoms of cardiac fibroelastosis and Leffter's fibrous endocarditis, doctors consider them to be completely different diseases.

Causes of the fibroelastosis

Fibroelastosis means changes in the connective tissue in vital organs: the heart and lungs, which is accompanied by a malfunction of the organs and affects the appearance and condition of the patient. The disease has been known to doctors for decades. Fibroelastosis of the inner lining of the heart (endocardium) was described at the beginning of the 18th century; similar changes in the lungs began to be discussed 2 and a half centuries later. However, doctors have not reached a final agreement on the causes of the pathological proliferation of connective tissue.

So it remains unclear what exactly causes a violation of the growth and development of connective fibers. But scientists identify certain risk factors for such changes, considering them to be possible (but not final) causes of the disease.

So in the pathogenesis of pulmonary fibroelastosis, which is considered a disease of mature people, a special role is assigned to recurrent infectious lesions of the organ, which are found in half of the patients. Infections provoke inflammation of the tissues of the lung and pleura, and long-term inflammation predisposes to their fibrous transformation.

In some patients, cases of fibroelastosis were found in the family, which suggests a hereditary predisposition. Nonspecific autoantibodies were found in their body, which provoke long-term inflammatory processes of uncertain etiology.

It is believed that fibrotic changes in lung tissue can be caused by gastroesophageal reflux disease. Although this relationship is likely to be indirect. It is also believed that the risk of fibroelastosis is higher in those who suffer from cardiovascular disease or pulmonary thrombosis.

Fibroelastosis of the lungs at a young and young age can remind you of yourself during pregnancy. Usually, the disease lurks for about 10 years or more, but it can manifest itself earlier, perhaps due to the increased load on the body of the expectant mother and hormonal changes, but there is still no exact explanation. However, a similar picture of the development of the disease was observed in 30% of the examined patients of reproductive age.

Pregnancy itself cannot cause the disease, but it can accelerate the development of events, which is very sad, because the mortality rate of the disease is very high, and life expectancy with fibroelastosis is short.

Fibroelastosis of the heart in most cases can be attributed to childhood diseases. Congenital pathology is detected even 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 and inflammatory diseases of the mother transmitted to the fetus, anomalies in the development of the membranes of the heart, impaired blood supply to the heart 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, and change the properties of tissues. The immature immune system of the fetus cannot provide him with protection against these pathogens, in contrast to the immunity of the expectant mother. The latter may not experience the consequences of a viral infection, while in the fetus, a transferred intrauterine infection can provoke the appearance of various anomalies.

Some scientists believe that an infection that affects the fetus up to 7 months of age plays a decisive role in the pathogenesis of the infectious form of fibroelastosis. In the future, it can only cause inflammatory diseases of the heart (myocarditis, endocarditis).

Anomalies in the development of the membranes and valves of the heart can be triggered by both the inflammatory process and inadequate autoimmune reactions, as a result of which the cells of the immune system begin to infect the body's own cells.

Gene mutations cause abnormal 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 the heart tissue may be the result of abnormal development of the heart. In this case, they speak of secondary fibloelastosis, provoked by congenital heart defects (CHD). These include such anomalies that cause obstruction phenomena (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 opening in the aorta,
  • underdevelopment of heart tissues (most often the left ventricle, less often the right and atria), which affects the pumping function of the heart.

It is believed that toxicosis during pregnancy can also act as a predisposing factor for fibroelastosis in the fetus.

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

Pathogenesis

Connective tissue is a special tissue of the human body, which is part of almost all organs, but does not take an active part in the performance of their functions. Connective tissue is credited with a supporting and protective function. Composing a kind of skeleton (skeleton, stroma) and limiting the functional cells of the organ, it provides its final shape and size. Having sufficient strength, the connective tissue also protects the cells of the body from destruction and injury, prevents the penetration of pathogens, absorbs obsolete structures with the help of special macrophage cells: dead tissue cells, foreign proteins, waste blood components, etc.

This tissue can be called auxiliary, because it does not contain cellular elements that ensure the functionality of a particular organ. However, its role in the life of the body is quite large. Being part of the membranes of blood vessels, the connective tissue ensures the safety and functionality of these structures, due to which nutrition and respiration (trophism) of the surrounding tissues of the internal environment of the body are carried out.

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

The second type of fibrous tissue is a dense connective tissue that does not contain a large number of individual cells, which in turn is divided into white and yellow. The white tissue consists of densely packed collagen fibers (ligaments, tendons, periosteum), and the yellow tissue consists of randomly intertwined elastin fibers interspersed with fibroblasts (part of the ligaments, membranes of blood vessels, lungs).

Connective tissues also include: blood, adipose, bone and cartilage tissues, but they are not of interest to us yet, because, speaking of fibroelastosis, they imply changes in fibrous structures. And elastic and elastic fibers contain only loose and dense connective tissues.

The synthesis of fibroblasts and the 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 the auxiliary tissue is disrupted (the number of fibroblasts increases, their “behavior” changes), strong collagen fibers grow or change in the growth of elastic ones (they remain short, writhe), which leads to a change in the properties of the organ membrane and some internal structures covered with connective tissue. They become thicker than necessary, become denser, stronger and inelastic, resembling fibrous tissue in the composition of 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 the disruption of the blood supply and respiration organs, resulting in 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 circles of blood circulation. The pulmonary circulation is responsible for blood supply and gas exchange in the lungs, from where oxygen is delivered to the heart with the blood flow, and from there to the systemic circulation and is distributed throughout the body, providing respiration of organs and tissues.

The elastic shell, limiting the contraction of the heart muscle, reduces the functionality of the heart, which does not pump blood as actively, and with it oxygen. With fibroelastosis of the lungs, their ventilation (oxygenation) is disturbed, it is clear that a smaller amount of 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

Fibroelastosis of the heart and lungs are two types of disease characterized by a violation of the synthesis of fibers in the connective tissue. They have different localizations, but both are potentially life-threatening because they are associated with progressive or severe heart and respiratory failure.

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

It is almost impossible to identify 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 period. The onset of pathological changes that do not yet affect the volume of the lungs and gas exchange can be detected by chance by conducting a detailed examination of the lungs in connection with another disease of the respiratory system or injury.

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

The first signs of the disease, which you should pay attention to, can be considered coughing and increasing shortness of breath. Such symptoms are often the result of a respiratory illness, so they can be associated with a cold and its consequences for a long time. Shortness of breath is often perceived as a cardiac disorder or age-related changes. Still, the disease is diagnosed in people approaching old age.

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

Shortness of breath is caused by progressive respiratory failure due to thickening of the alveolar walls and pleura, a decrease in the volume and number of alveolar cavities in the lung (the parenchyma of the organ is visible on an x-ray in the form of honeycombs). The symptom is aggravated under the influence of physical exertion, at first significant, and then even small. As the disease progresses, it worsens, which causes disability and death of the patient. 

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

Half of the patients develop non-specific symptoms in the form of shortness of breath and chest pain, characteristic of pneumothorax (accumulation of gases in the pleural cavity). This anomaly can also occur as a result of injuries, primary and secondary lung diseases, and improper treatment, so it cannot be diagnosed.

For fibroelastosis of the heart, as well as for the pathology of the growth of the connective tissue of the lungs, are characterized by: pallor of the skin, weight loss, weakness, which is often paroxysmal in nature, shortness of breath. There may also be persistent low-grade fever without signs of a cold or infection.

Many patients have a change in the size of the liver. It increases without symptoms of dysfunction. It is also possible the appearance of edema of the legs, face, hands, sacral region.

A characteristic manifestation of the disease is considered to be an increasing circulatory failure associated with a violation of the heart. At the same time, tachycardia is diagnosed (an increase in the number of heartbeats is often combined with arrhythmia), shortness of breath (including in the absence of physical exertion), tissue cyanosis (cyanotic color caused by the accumulation of carboxyhemoglobin in the blood, i.e. The combination of hemoglobin with carbon dioxide due to disturbed blood flow, and, accordingly, gas exchange).

In this case, 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 on the background of a respiratory tract infection, which acts as a trigger. [4]

Fibroelastosis in children

If pulmonary fibroelastosis is a disease of adults, often originating in childhood, but not reminiscent of itself for a long time, then such a pathology of the endocardium of the heart often appears even before the birth of the baby and affects his life from the first moments of birth. This rare but severe pathology is the cause of difficult-to-correct heart failure in infants, many of whom die within 2 years. [5]

Fibroelastosis of the endocardium in newborns in most cases is the result of pathological processes that occur in the baby's body even in prenatal age. Infections received from the mother, genetic mutations, anomalies 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 membranes of the heart. Especially if a 4-7-month-old fetus is affected simultaneously by two or more factors.

For example, a combination of anomalies in the development of the heart and coronary vessels (stenosis, atresia, coarctation of the aorta, pathological development of myocardial cells, weakness of the endocardium, etc.), which contributes to tissue ischemia, combined with an inflammatory process due to infection, practically does not leave the child a chance for more less life. If defects in the development of an organ can somehow be quickly corrected, then progressive fibroelastosis can only be slowed down, but not cured.

Usually, fibroelastosis of the heart in the fetus is detected already during pregnancy during ultrasound diagnostics in the second or third trimester. Ultrasound and echocardiography at a period of 20 to 38 weeks showed hyperechogenicity, which indicates thickening and compaction of the endocardium (often diffuse, less often focal), a change in the size and shape of the heart (the organ is enlarged and takes the form of a ball or bullet, internal structures are gradually smoothed out ). [6]

In 30-35% of cases, fibroelastosis was detected before 26 weeks of pregnancy, in 65-70% - in the subsequent period. In more than 80% of newborns, fibroelastosis is combined with obstructive heart disease, i.e. Is secondary, despite the early timing of its detection. In half of the sick children, left ventricular hyperplasia was found, which explains the high prevalence of fibroelastosis of this particular heart structure. Pathologies of the aorta and its valve, determined in a third of children with endocardial overgrowth, also lead to an increase (dilatation) of the left ventricular chamber and disruption of its functionality.

With instrumentally confirmed cardiac fibroelastosis, doctors recommend terminating the pregnancy. In almost all children born, whose mothers refused medical abortion, the signs of the disease were confirmed. Symptoms of heart failure, characteristic of fibroelastosis, appear within a year (rarely at 2-3 years of age). In children with a 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. Illness is indicated by low activity, lethargy of the child, refusal of the breast due to fatigue, poor appetite, excessive sweating. All this leads to the fact that the child is not gaining weight well. The skin of the baby is painfully pale, in some with a bluish tint, most often in the area of the nasolabial triangle.

There are signs of poor immunity, so these children often and quickly pick up respiratory infections that complicate the situation. Sometimes in the first days and months of life, a child does not have circulatory disorders, but frequent infections and pulmonary diseases become a trigger for congestive heart failure.

Additional studies of the health of newborns and young children with suspected or previously diagnosed fibroelastosis showed low blood pressure (hypotension), an increase in the size of the heart (cardiomegaly), muffled tones when listening to the heart, sometimes there was a systolic murmur characteristic of mitral valve insufficiency, tachycardia, shortness of breath Auscultation of the lungs shows the presence of wheezing, indicating congestion.

The defeat of the endocardium of the left ventricle often leads to a weakening of the muscular layer of the heart (myocardium). The normal heart rhythm consists of two rhythmically alternating tones. With fibroelastosis, a third (and sometimes fourth) tone may appear. Such a pathological rhythm is well audible and in its sound resembles a three-beat horse gait (gallop), therefore it is called the gallop rhythm.

Another symptom of fibroelastosis in young children can be considered the appearance of a heart hump. The fact is that the ribs of the child in the early postnatal period remain non-ossified and are represented by cartilaginous tissue. An increase in the size of the heart leads to the fact that it begins to put pressure on the "soft" ribs, as a result of which they bend and take on a permanent forward curved shape (heart hump). With fibroelastosis in adults, the heart hump does not form due to the strength and rigidity of the bone tissue of the ribs, even if all the structures of the heart are enlarged.

In itself, the formation of a heart hump indicates only a congenital heart disease without specifying its nature. But in any case, this is due to an increase in the size of the heart and its ventricles.

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

If fibroelastosis has an acquired character (for example, it is the result of inflammatory diseases of the membranes of the heart), the clinical picture most often has a slowly progressive character. 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 physical exertion, an increase in heart rate, fatigue and low physical endurance. A little later, the liver begins to increase, swelling and paroxysmal dizziness appear.

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

Complications and consequences

It must be said that fibroelastosis of the heart and lungs is a serious pathology, the course of which depends on various circumstances. Congenital heart defects significantly complicate the situation, which can be eliminated surgically at an early age, but there remains a rather high risk of death (approximately 10%).

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

At the same time, treatment does not guarantee a complete restoration of cardiac function, but only slows down the increase in symptoms of heart failure. On the other hand, the absence of such supportive treatment leads to death during the first two years of a baby's life.

If heart failure is detected in the first days and months of a baby's life, most likely the child will not live even a week. Children respond differently to treatment. In the absence of a therapeutic effect, there is virtually no hope. But with the assistance provided, the life expectancy of a sick child is small (from several months to several years).

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

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.

Fibroelastosis of the lungs, regardless of the time of appearance of changes in the parenchyma and membranes of the organ after a 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 treating the disease have not yet been developed. [7]

Diagnostics of the fibroelastosis

Endomyocardial fibroelastosis, the symptoms of which in most cases are 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 injuries and somatic diseases, it is possible to identify the pathology even in the prenatal period, i.e. Before the birth of the child.

Doctors believe that pathological changes in the tissues of the endocardium, a change in the shape of the fetal heart and some features of its work, characteristic of fibroelastosis, can be determined already at the 14th week of pregnancy. But this is still a rather short period, and it cannot be ruled out that the disease may appear a little later, closer to the third trimester of pregnancy, and sometimes even a couple of months before childbirth. That is why, when observing pregnant women, it is recommended to conduct clinical ultrasound screenings of the fetal heart with an interval of several weeks.

By 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 region of the left ventricle, but this structure is not always enlarged. The dilated form of the disease with an increase in the left ventricle of the heart is easily determined during an ultrasound examination by a spherical shape of the heart, the apex of which is represented by the left ventricle, a general increase in the size of the organ, and bulging of the interventricular septum towards the right ventricle. But the main sign of fibroelastosis is the thickening of the endocardium, as well as the cardiac septa with a characteristic increase in the echogenicity of these structures, which is determined by means of a specific ultrasound examination.

The study is carried out using special ultrasound equipment with cardiological programs. Echocardiography of the fetus does not harm the mother and the unborn child, but allows you to identify not only anatomical changes in the heart, but also determine the condition of the coronary vessels, the presence of blood clots in them, and changes in the thickness of the heart membranes.

Fetal echocardiography is prescribed not only in the presence of deviations during the decoding of the ultrasound results, but also in the case of an infection (especially viral) transferred by the mother, the use of potent drugs, a hereditary predisposition, the presence of metabolic disorders, as well as congenital cardiac pathologies in older children.

With the help of fetal echocardiography, other congenital forms of fibroelastosis can also be detected. For example, fibroelastosis of the right ventricle, a common process with simultaneous damage to the left ventricle and nearby structures: the right ventricle, heart valves, atria, combined forms of fibroelastosis, endomyocardial fibroelastosis with thickening of the inner lining of the ventricles and involvement of part of the myocardium in the pathological process (usually combined with wall thrombosis).

Endocardial fibroelastosis detected in prenatal age has a very poor prognosis, so doctors recommend terminating the pregnancy in this case. The possibility of an erroneous diagnosis is excluded by repeated ultrasound of the fetal heart, which is performed 4 weeks after the first examination that revealed the pathology. It is clear that the final decision to terminate or maintain the pregnancy remains with the parents, but they must be aware of the kind of life they doom the child to.

Endocardial fibroelastosis is not always detected during pregnancy, especially considering the fact that not all expectant mothers are registered with the antenatal clinic and undergo preventive ultrasound diagnostics. The illness of a child in her womb practically does not affect the condition of a 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 a few months after the birth of the child. In this case, laboratory blood tests may not show anything, except for an increase in the concentration of sodium (hypernatremia). But their results will be useful in differential diagnosis to exclude inflammatory diseases.

Hope remains on instrumental diagnostics. A standard study of the work of the heart (ECG) in the case of fibroelastosis is not particularly revealing. It helps to identify disorders of the heart and electrical conduction of the heart muscle, but does not specify the causes of such dysfunctions. So a change in the ECG voltage (at a younger age it is usually underestimated, at an older age it is, on the contrary, excessively high) indicates cardiomyopathy, which can be associated not only with heart pathologies, but also with metabolic disorders. Tachycardia is a symptom of cardiac disease. And with the defeat of both ventricles of the heart, the cardiogram may generally seem normal. [8]

Computed tomography (CT) is an excellent non-invasive tool for detecting cardiovascular calcification and ruling out pericarditis.[9]

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

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

If symptoms of heart failure appear and the patient is referred to a doctor about this, the patient is also prescribed: chest x-ray, computed or magnetic resonance imaging of the heart, echocardiography (EchoCG). In doubtful cases, it is necessary to resort to a biopsy of the heart tissues with their subsequent histological examination. The diagnosis is very serious, therefore, requires the same approach to diagnosis, although the treatment is not much different from the symptomatic treatment of coronary artery disease and heart failure.

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

To distinguish endocardial fibroelastosis from isolated mitral valve insufficiency and mitral heart disease helps the analysis of tones and murmurs in the heart, a change in the size of the atria, and the study of anamnesis.

Analysis of the history is useful in distinguishing between fibroelastosis and cardiac and aortic stenosis. When narrowing the aorta, it is also worth paying attention not to the preservation of sinus rhythm and the absence of thromboembolism. Heart rhythm disturbances and thrombus settling are not observed with exudative pericarditis, but the disease manifests itself with fever and fever.

The greatest difficulty is the differentiation of endocardial fibroblastosis and congestive cardiomyopathy. At the same time, fibroelastosis, although in most cases it is not accompanied by severe cardiac conduction disorders, has a less favorable treatment prognosis.

With combined pathologies, attention should be paid to any abnormalities detected during a tomogram or ultrasound of the heart, because congenital malformations greatly complicate the course of fibroelastosis. If combined endocardial fibroelastosis is detected in the prenatal period, it is not advisable to maintain pregnancy. It's much more humane to interrupt it.

Diagnosis of pulmonary fibroelastosis

Making a diagnosis of pulmonary fibroelastosis also requires certain knowledge and skills from the doctor. The fact is that the symptoms of the disease are quite heterogeneous. On the one hand, they indicate congestive lung diseases (unproductive cough, shortness of breath), and on the other hand, they may also 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 diseases of the lungs, but do not provide information about quantitative and qualitative changes in the tissues. The presence of signs of eosinophilia helps to differentiate the disease from similar manifestations of pulmonary fibrosis, but does not reject or confirm the fact of fibroelastosis.

Instrumental studies are considered more indicative: X-ray of the lungs and tomographic examination of the respiratory organs, as well as functional analyzes, consisting in determining respiratory volumes, vital capacity, and pressure in the organ.

With pulmonary fibroelastosis, attention should be paid to a decrease in the function of external respiration, measured during spirometry. A decrease in active alveolar cavities significantly affects the vital capacity of the lungs (VC), and the thickening of the walls of internal structures affects the diffusion capacity of the organ (DSL), which provides ventilation and gas exchange functions (in simple words, absorb carbon dioxide from the blood and release oxygen).

The characteristic signs of pleuroparenchymal fibroelastosis are a combination of limited air intake into the lungs (obstruction) and a violation of the expansion of the lung on inspiration (restriction), deterioration in the function of external respiration, moderate pulmonary hypertension (increased pressure in the lungs), diagnosed in half of patients.

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

On the tomogram, lung damage in the upper sections is noticeable in the form of foci of pleural compaction and structural changes in the parenchyma. The overgrown connective tissue of the lungs resembles muscle tissue in color and properties, but the volume of the lungs decreases. Quite large air-containing cavities (cysts) are found in the parenchyma. Characterized by irreversible focal (or diffuse) expansion of the bronchi and bronchioles (traction bronchiectasis), low standing of the dome of the diaphragm.

In radiological studies, many patients reveal areas of "frosted glass" and "honeycomb lung", indicating uneven ventilation of the lungs due to the presence of foci of tissue compaction. Approximately half of patients have an increase in lymph nodes and liver.

Fibroelastosis of the lung must be differentiated from fibrosis caused by a parasitic infection and associated eosinophilia, endocardial fibroelastosis, lung diseases with impaired ventilation and a “honeycombed lung” pattern, autoimmune disease histicytosis X (one of the forms of this pathology with lung damage is called Hand-Schüller disease - Christian), manifestations of sarcoidosis and pulmonary tuberculosis.

Treatment of the fibroelastosis

Fibroelastosis, whatever localization it may be, is considered a dangerous and practically incurable disease. Pathological changes in the pleura and lung parenchyma cannot be restored medicinally. And even the use of hormonal anti-inflammatory drugs (corticosteroids) in combination with bronchodilators does not give the desired result. Bronchodilator drugs help to slightly alleviate the patient's condition, removing obstructive syndrome, but they do not affect the processes occurring in the lungs, therefore they can only be used as maintenance therapy.

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

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

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

Fibroelastosis of the heart is also considered a medically incurable disease, especially when it comes to congenital pathology. Children usually do not live beyond 2 years of age. Only a heart transplant can save them, 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 a sick child. With stenosis, arts practice the installation of a vasodilator - a shunt (coronary bypass grafting). With the expansion of the left ventricle of the heart, its shape is promptly restored. But even such an operation does not guarantee that the child will be able to do without transplantation. About 20-25% of babies survive, while they suffer from heart failure all their lives, i.e. Are not considered healthy.

If the disease is acquired, it is worth fighting for the life of the child with the help of medications. But you need to understand that the earlier the disease manifests itself, the more difficult it will be to fight it.

Drug 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-blockers used to treat heart rhythm disorders, arterial hypertension, prevention of myocardial infarction (anaprilin, bisoprolol, metoprolol),
  • cardiac glycosides, which, with prolonged use, not only support the work of the heart (increase the potassium content in cardiomyocytes and improve myocardial conductivity), but also can somewhat reduce the degree of thickening of the endocardium (digoxin, gitoxin, strophanthin),
  • potassium-sparing diuretics (spironolactone, veroshpiron, dekriz), preventing tissue edema,
  • antithrombotic therapy with anticoagulants (cardiomagnyl, magnicor), which prevents the formation of blood clots and circulatory disorders in the coronary vessels.

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

Medications

As you can see, the treatment of endocardial fibroelastosis practically does not differ from that in heart failure. In both cases, cardiologists take into account the severity of cardiopathy. The prescription of drugs is purely individual, taking into account the age of the patient, concomitant diseases, the form and degree of heart failure.

In the treatment of acquired endocardial fibroelastosis, drugs of 5 groups are used. Consider one drug from each group.

Enalapril is a drug from the group of ACE inhibitors, produced in the form of tablets of various dosages. The drug enhances coronary blood flow, dilates arteries, lowers blood pressure without affecting cerebral circulation, slows down and reduces the expansion of the left ventricle of the heart. The drug improves myocardial blood supply, reducing the effects of ischemia, somewhat reduces blood clotting, preventing the formation of blood clots, and has a slight diuretic effect.

In heart failure, a medicine is prescribed for a period of more than six months or on an ongoing basis. Start taking the medicine with a minimum dose (2.5 mg), gradually increasing it by 2.5-5 mg every 3-4 days. A constant dose will be 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 blood pressure is set below normal, the dose of the drug is gradually reduced. It is impossible to abruptly stop treatment with Enalapril. A maintenance dose of 5 mg per day is recommended.

The drug is intended for the treatment of adult patients, but it can also be prescribed to a child (safety has not been officially established, but in the case of fibroelastosis, we are talking about the life of a small patient, so the risk ratio is taken into account). Do not prescribe an ACE inhibitor to patients with intolerance to the components of the drug, with porphyria, pregnancy and during breastfeeding. If earlier the patient had Quincke's edema while taking any drugs of this group, Enalapril is prohibited.

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

When treating with the drug, do not take conventional diuretics in order to avoid dehydration and a strong hypotensive effect. Simultaneous administration with potassium-sparing diuretics requires dose adjustment, since there is a high risk of hyperkalemia, which in turn provokes cardiac arrhythmias, convulsive seizures, decreased muscle tone, increased weakness, etc.

Enalapril is usually well tolerated, but some patients may develop side effects. The most common are: a strong decrease in pressure up to collapse, headaches and dizziness, sleep disturbances, increased fatigue, a reversible disturbance of balance, hearing and vision, the appearance of tinnitus, shortness of breath, coughing without sputum, changes in the composition of blood and urine, usually indicating malfunction of the liver and kidneys. Possible: hair loss, decreased sexual desire, symptoms of "hot flashes" (sensation of heat and palpitations, flushing of the skin of the face, etc.).

"Bisoprolol" is a beta-blocker with selective action, which has a hypotensive and anti-ischemic effect, helps to fight the manifestations of tachycardia and arrhythmia. A budget remedy in the form of tablets that prevents the progression of heart failure in endocardial fibroelastosis. [13]

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

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

Increasing the indicated dosages is possible only with the permission of the doctor. But in severe diseases of the liver and kidneys, 10 mg is considered the maximum allowable dose.

In the complex treatment of heart failure against the background of left ventricular dysfunction, which most often occurs with fibroelastosis, an effective dose is selected, gradually increasing the dosage by 1.25 mg. In this case, they start with the lowest possible dose (1.25 mg). The increase in dosage 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, they reach 10 mg, which the patient will have to take for a long time or constantly.

If such a dosage is poorly tolerated, it is gradually reduced to a comfortable level. Refusal of treatment with a beta-blocker should also not be abrupt.

The drug should not be prescribed in case of hypersensitivity to the active and excipients of the drug, acute and decompensated heart failure, cardiogenic shock, atrioventricular blockade of 2-3 degrees, bradycardia, stable low blood pressure and some other heart pathologies, severe bronchial asthma, bronchial obstruction, severe peripheral circulatory disorders, metabolic acidosis.

Caution must be observed when prescribing complex treatment. So the combination of "Bisoprolol" with some antiarrhythmic drugs (quinidine, lidocaine, phenytoin, etc.), calcium antagonists and central antihypertensive drugs is not recommended.

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

In the presence of concomitant diseases of the broncho-pulmonary system, kidneys, liver, in diabetes mellitus, the risk of undesirable effects is higher, which indicates an exacerbation of the disease.

Digoxin is a popular budget digitalis-based cardiac glycoside, which is released strictly on prescription (in tablets) and should be used under his supervision. Injection treatment is carried out in a hospital with an exacerbation of coronary artery disease and chronic heart failure, tablets are prescribed on an ongoing basis in minimally effective doses, since the drug has a toxic and narcotic-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). Also, the drug is characterized by a vasodilator (reduces congestion) and some diuretic action, which helps relieve swelling and reduce the strength of respiratory failure, which manifests itself in the form of shortness of breath.

The danger of Digoxin and other cardiac glycosides is that, in case of an overdose, they can provoke heart rhythm disturbances caused by increased myocardial excitability.

In case of exacerbation of CHF, the drug is prescribed in the form of injections, selecting an individual dosage, taking into account the severity of the condition and the age of the patient. When the condition stabilizes, they switch to tablets.

Usually the standard single dose of the drug is 0.25 mg. The multiplicity of reception 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 an ongoing basis, a maintenance dose of 0.25 (less often 0.5) mg per day should be taken.

When prescribing the drug to children, the weight of the patient is taken into account. An effective and safe dose is calculated as 0.05-0.08 mg per kg of body weight. But the drug is not prescribed constantly, 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. At the same time, it is very dangerous to adjust the doses on your own or take 2 drugs with this effect at the same time.

"Digoxin" is not prescribed for unstable angina, severe cardiac arrhythmias, AV heart block of 2-3 degrees, cardiac tamponade, Adams-Stokes-Morgani syndrome, isolated bicuspid valve stenosis and aortic stenosis, a congenital heart anomaly called Wolf syndrome Parkinson-White, 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 that have multiple manifestations, so only a specialist can decide on the possibility of using this drug.

 Digoxin also has side effects. These include heart rhythm disturbances (as a result of an incorrectly selected dose and overdose), loss of appetite, nausea (often with vomiting), stool disorders, severe weakness and fatigue, headaches, the appearance of “flies” before the eyes, a decrease in platelet levels 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 long-term therapy.

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

With CHF, the drug is prescribed depending on the phase of the disease. In case of exacerbation, the drug can be prescribed both in the form of injections and in tablets at a dosage of 50-100 mg per day. When the condition stabilizes, 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 decreasing the first, the dose can be increased until the normal concentration of trace elements is established.

In pediatrics, the effective dosage is calculated based on the ratio of 1-3 mg of spironolactone per kilogram of the patient's body weight.

As you can see, here, too, the selection of the recommended dose is individual, as in the appointment of many other drugs used in cardiology.

Contraindications to the use of a diuretic can be: an excess of potassium or a low level of sodium in the body, a pathology associated with a lack of urination (anuria), severe kidney disease with a violation of their performance. The drug is not prescribed to pregnant women and nursing mothers, as well as to 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 irregularities, and liver diseases.

Taking the drug can cause headaches, drowsiness, imbalance and coordination of movements (ataxia), enlargement of the mammary glands in men (gynecomastia) and impaired potency, changes in the nature of menstruation, coarsening of the voice and excessive hairiness in women (hirsutism), epigastric pain and disorders work of the gastrointestinal tract, intestinal colic, disorders of the kidneys and the balance of minerals. Skin and allergic reactions are possible.

Usually, side effects are observed when the required norm is exceeded. With insufficient dosage, edema may appear.

"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, antiplatelet effect, affects the function of breathing. Magnesium hydroxide reduces the negative effect of acetylsalicylic acid on the gastrointestinal mucosa.

With fibroelastosis of the endomyocardium, the drug is prescribed for prophylactic purposes, therefore, the minimum effective dose is adhered to - 75 mg, which corresponds to 1 tablet. With ischemia of the heart due to thrombosis and subsequent narrowing of the lumen of the coronary vessels, the initial dose is 2 tablets, and the maintenance dose corresponds to the prophylactic dose.

Exceeding the 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 use of which under the age of 15 years can have serious consequences.

The drug is not prescribed to patients of childhood and early adolescence, with intolerance to acetylsalicylic acid and other components of the drug, "aspirin" asthma (in history), acute erosive gastritis, peptic ulcer, hemorrhagic diathesis, severe liver and kidney diseases, in case of severe decompensated heart failure.

During pregnancy, "Magnicor" is prescribed only in case of emergency and only in the 1-2 trimester, given the possible negative effect on the fetus and the course of pregnancy. In the 3rd trimester of pregnancy, such treatment is undesirable, since it helps to reduce uterine contractility (prolonged labor) and can cause severe bleeding. The fetus may have: pulmonary hypertension and impaired renal function.

Side effects of the drug include symptoms from the gastrointestinal tract (dyspepsia, epigastric and abdominal pain, some risk of gastric bleeding with the development of iron deficiency anemia). While taking the medicine, nosebleeds, bleeding of the gums and organs of the urinary system are possible,

In case of an 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.

The selection of drugs as part of complex therapy and recommended dosages should be strictly individual. Particular care should be taken in the treatment of pregnant women, nursing mothers, children and elderly patients.

Alternative medicine and homeopathy

Fibroelastosis of the heart is a serious and severe disease with a characteristic progressive course and almost no chance of recovery. It is clear that an effective treatment of such a disease by alternative means is impossible. Alternative medicine prescriptions, which are mostly herbal treatments, can only be used as an aid and only with the permission of a doctor, so as not to complicate an already poor prognosis.

As for homeopathic remedies, their use is not forbidden, but may be part of the complex treatment of heart failure. True, in this case it is not so much about treatment as about preventing the progression of CHF.

An experienced homeopath should prescribe drugs, and the possibility of including them in complex therapy is within the competence of the attending physician.

What homeopathic remedies help delay the progression of heart failure in fibroelastosis? In acute heart failure, homeopaths turn to the help of drugs: Arsenicum albumum, Antimonium tartaricum, Carbo vegetabilis, Acidum oxalicum. Despite the similarity of indications, when 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.

With CHF, maintenance therapy can include: Lachesis and Nayu, Lycopus (in the initial stages of heart expansion), Laurocerasus (with shortness of breath at rest), Latrodectus mactans (with valvular pathologies), hawthorn preparations (especially useful for endomyocardial lesions).

With a strong heartbeat for symptomatic treatment, Spigelia, Glonoinum (with tachycardia), Aurum metallicum (with hypertension) can be prescribed.

To reduce the severity of shortness of breath help: Grindelia, Spongia and Lahegis. For the relief of heart pain, the following can be prescribed: Cactus, Cereus, Naya, Cuprum, to relieve anxiety against this background - Aconitum. With the development of cardiac asthma are shown: Digitalis, Laurocerasus, 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 damage to vital organs. Effective treatment of the underlying disease helps prevent dangerous consequences, to which fibroelastosis belongs. This is a great opportunity to take good care of your health and the health of future generations, the so-called work for a healthy future and longevity.

Forecast

Changes in the connective tissue in fibroelastosis of the heart and lungs are considered irreversible. Although some drugs with long-term therapy are able to somewhat reduce the thickness of the endocardium, they do not guarantee a cure. Although this condition is not always fatal, the prognosis is still relatively poor. The 4-year survival rate is 77%. [14]

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

Other operations can only avoid the early death of a child (and even then not always), but they cannot completely cure him of heart failure. Death occurs with decompensation and the development of respiratory failure.

The prognosis of pulmonary fibroelastosis depends on the characteristics of the course of the disease. With lightning-fast development of symptoms, the chances are extremely small. If the disease progresses gradually, the patient can live for 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 if preventive measures are followed. In the case of fibroelastosis of the heart, this is, first of all, 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 doctors are powerless). If they could not be avoided, early diagnosis helps to identify the pathology at the stage when abortion is possible, which in this situation is considered humane.

Translation Disclaimer: The original language of this article is Russian. For the convenience of users of the iLive portal who do not speak Russian, this article has been translated into the current language, but has not yet been verified by a native speaker who has the necessary qualifications for this. In this regard, we warn you that the translation of this article may be incorrect, may contain lexical, syntactic and grammatical errors.

You are reporting a typo in the following text:
Simply click the "Send typo report" button to complete the report. You can also include a comment.