Moller, Lucas, Adams, Anderson, Jorgens, Edwards (1964); Wenger (1964); Hastreiter (1968); Schryer, Karnauchow (1974).
Endocardial fibroelastosis consists of diffuse thickening of the endocardium of one or more cardiac chambers, formed by collagen or elastic tissue.
This lesion can be isolated or combined with other congenital heart defects, such as valvular stenosis, etc.
It is difficult to determine whether the endocardial thickening is “secondary” as a result of changes in hemodynamics caused by a stenotic lesion, or “primary”, transferred to the valves. Therefore, the terms “isolated” and “complicated” fibroelastosis will be most appropriate.
Synonyms: endocardial sclerosis, endomyocardial fibroelastosis.
The first description belongs to Lancusi (1740); T. Weinberg, A. J. Himmelfarb (1943).
Classification of endocardial fibroelastosis

1. Fibroelastosis of the left ventricular endocardium (common).

A. Dilated type (often).

1. Isolated.
2. Complicated:

a) with accompanying anatomical lesions:

1. involving mitral valve(insufficiency or stenosis);
2. involving the aorta (stenosis);

1. with coarctation of the aorta;
2. with open ductus arteriosus;
3. with hypoplasia of the left ventricle;
4. with anomalous origin of the left coronary artery.

B. Contractile type (rare).

1. Isolated.
2. Complicated:

a) with involvement of the mitral valve;
b) with “involvement of the aortic valve;
c) with other obstructive anomalies on the left
side of the heart.

1. Fibroelastosis of the endocardium of the right ventricle (rare).

1. Isolated.
2. Complicated:

a) involving the valve pulmonary artery(stenosis or atresia);
b) involving the tricuspid valve (stenosis or insufficiency).
Frequency and gender distribution
Pathological data: 5.4% (author’s pathological material per 1000 cases birth defects hearts; 3.1% - isolated fibroelastosis; 2.3% complicated fibroelastosis).
The incidence of cases according to the literature ranges from 4% to 17%.
There may be some predominance among females.
Etiology and pathogenesis
The etiology and pathogenesis are unclear. The confusion on this issue is reflected in the numerous hypotheses that currently exist, including inflammatory processes in the endocardium and myocardium, Coxsackie B virus, mumps, hypoxia, mechanical obstruction to blood flow, myocardial hyperplasia, endocardial hyperplasia, elastic hyperplasia, collagen disease, autoimmunity, hereditary disorders, congenital disorders exchange, obstructions to lymph flow, maternal toxins and many other assumptions.
Some researchers consider endocardial damage primary disease, while others view endocardial thickening as a secondary response to ever-increasing intraventricular stress and stretch and thus consider myocardial disease to be a primary disorder.

Pathological anatomy (Fig. 54)
Endocardial fibroelastosis most often affects the left ventricle and often also the left atrium and sometimes the right chambers of the heart.
In our own observations“in 54 patients the left ventricle was involved in 41 patients with to varying degrees participation of the left atrium, the right ventricle only in 6 and both ventricles in 7 patients.
The endocardium is diffusely covered with a grayish-white layer of tissue consisting of collagen and elastic fibers with a predominance of elastic tissue. The thickness of the endocardium can reach several millimeters. The opalescent milky white appearance of the endocardium has been described in the literature as a "sugar coating". In older patients, endocardial thickening is associated with a greater degree of myocardial fibrosis. Thrombi in the wall (15%) and endocardial calcification were sometimes observed.
Endocardial fibroelastosis, according to the size of the left ventricle, is divided into two types: dilated, which occurs most often, in which the left ventricle is dilated and hypertrophied, and (contractile, in which the size of the ventricle is normal or reduced, but not hypoplastic, although its walls may be hypertrophied.

Histologically, the endocardium consists of many dense layers of elastic tissue, usually located in parallel and separated by varying amounts of collagen. Penetration of its IB adjacent myocardium varies in severity and, apparently, follows the myocardial sinusoids and the course vascular channels. The degree of myocardial fibrosis varies. It is impossible to differentiate isolated (primary) andocardial fibroelastosis from complicated (secondary) one based on the histological appearance of the endocardial layer or its thickness.
Isolated fibroelastosis of the left ventricular endocardium. Dilated type
Isolated involvement of the left ventricle accounts for about one third of all cases of endocardial fibrosis.
The heart is usually significantly enlarged (2-4 times larger normal weight), its shape is spherical and the apex of the heart is completely formed by the left ventricle. The wall of the left ventricle is thickened, its cavity is spherically expanded and interventricular septum protrudes into the right ventricle. The thickness of the right ventricle is usually moderately increased, its cavity in some cases is flattened and slit-like, but the right ventricle and right atrium may undergo terminal expansion.
Involvement of the left atrium was observed in more than % of cases, of the right ventricle - in approximately XU cases. The right atrium is affected in only one in 10 cases.
The papillary muscles are partially involved in the process of fibroelastosis. They are small and occur higher on the stomach wall than normal. The chordae tendineae are shortened and thickened.
Isolated fibroelastosis of the left ventricular endocardium. Contractile type
This anomaly is very rare. There are reports of only a few cases in the literature.
The left ventricle is significantly smaller than the right ventricle and may be normal size or even less than normal. This disproportion in size is sometimes such that the left ventricle resembles an appendage of the right. The right ventricle is noticeably dilated and hypertrophied.
It is possible that at least a few cases of the contractile type are a transitional form between the anatomical complexes with hypoplastic left ventricle and the more common variant of endocardial fibroelastosis of the dilated type.
Combination with congenital deficiency mitral valve: the valve is diffusely and unevenly thickened and noticeably
deformed The edges of the valves are relatively ordinary; chordae and papillary muscles are also involved in the process. Left ventricular dilatation worsens mitral valve insufficiency.
Combination with congenital mitral valve stenosis: the valve is thickened and deformed, significant fusion of the commissures leads to a diaphragmatic or funnel-shaped valve.
Combination with congenital supravalvular mitral stenosis: there is a supravalvular ring consisting of a rim of fibrous tissue extending into the cavity of the left atrium directly above the mitral valve (see p. 141).
Combination with the “parachute” mitral valve: the mitral valve leaflets and commissures are normal, but the chordae tendineae converge and attach to the papillary muscle, making up with it large complex. The combination of the thickness of the chordae and their convergent attachment to the papillary muscle makes the valve immobile.
It is believed that, at least in some cases, the abnormal position of the papillary muscles is acquired rather than a true developmental anomaly, and is pathogenetically associated with endocardial fibroelastosis.
Combination with bicuspid aortic valve (see p. 169).
Combination with congenital aortic valve stenosis: the aortic valve often has gross malformations and is undifferentiated; extensive fusion of commissures often makes individual valves indistinguishable.
The question has repeatedly arisen whether changes in the aortic valve are primary, and the process of endocardial fibroelastosis is secondary due to obstruction of outflow from the left ventricle, or whether changes in the aortic valve represent an extension of the process of fibroelastosis in the endocardial wall.
Combination with congenital subvalvular aortic stenosis: fibrous type of subvalvular aortic stenosis can be considered as a localized form of fibroelastosis of the left ventricular endocardium. The so-called endocardial pockets of the left ventricle may be pathogenetically related to endocardial fibroelastosis.
Combination with coarctation of the aorta: given the distance from the site of endocardial fibroelastosis of the left ventricle to the site of coarctation of the aorta, some authors believe that it is unlikely that both lesions can be considered as a common pathogenetic process, and explain endocardial fibroelastosis as a secondary change in hemodynamics.
Combination with patent ductus arteriosus: there is a statistically significant difference between these two lesions
connection. Open ductus arteriosus is the defect that usually accompanies almost any type of congenital obstructive left-sided heart disease.
Combination with left ventricular hypoplasia: fibroelastosis
left ventricle (occurs only in case of open mit
ral valve, but not mitral atresia.
Combination with an anomalous origin of the left coronary artery from the pulmonary trunk: endocardial fibroelastosis can be considered secondary reaction to impaired blood supply to the myocardium.
.Combination with congenital complete heart block: this condition, although relatively rare, occurs quite often
as a clinicopathological complex.
Careful histological examination reveals in almost every case degenerative changes in the atrioventricular node.
Combination with Wolff-Parkinson-White syndrome: this combination occurs so often that it can be called a syndrome. There is no anatomically characteristic picture.
Isolated fibroelastosis of the right ventricular endocardium
Involvement of exclusively the right ventricle in the process is very rare. The cavity of the right ventricle tends to expand rather than contract.
Complicated fibroelastosis of the right ventricular endocardium
It is usually associated with pulmonary valve stenosis or atresia, often with tricuspid valve stenosis and sometimes with tricuspid valve regurgitation. The size of the right ventricle ranges from extremely small to larger than normal. The latter is observed only in combination with tricuspid valve insufficiency.
Fibroelastosis of the left ventricular endocardium in adolescents and adults
Such cases, apparently, cannot be considered examples of the survival of patients after endocardial and algal fibroelastosis in childhood; rather, they are a nonspecific reaction to other pathological conditions myocardium. Endocardial thickening is usually patchy and is associated with a significant degree of myocardial fibrosis.
Endocardial sclerosis in adults is probably caused by two main mechanisms: 1) reactive endocardial

DIAL hyperplasia ib response to increased intraventricular tension or ventricular dilatation; 2) reparative fibrosis associated with changes in the myocardium.
Associated cardiac and extracardiac anomalies
Typical combinations with other cardiac anomalies are given above. In addition, endocardial fibroelastosis has been observed in association with congenital left ventricular aneurysm, idiopathic right atrial enlargement, infantile coronary calcification, situs inversus, and cardiac dextroversion. Extracardiac anomalies are rare, and no significant combinations with them have been observed.
Hemodynamics
Endocardial fibroelastosis affects the contractility and distensibility of the left ventricular myocardium. But "in most cases there is no decrease in diastolic filling or decrease in stroke volume, because in the dilated type, for any given increase in volume, much less inward wall excursion will be required in the dilated ventricle than in a normal, non-dilated ventricle. Since the left ventricle is markedly dilated, normal stroke volume is achieved with less excursion of the ventricular wall than normal. A combined defect of the mitral and (or) aortic valve is. the usual reason clinical symptoms. With contractile type pulmonary hypertension becomes pronounced.
Life expectancy and causes of death
Symptoms of congestive heart failure begin to appear between birth and 10 months of postnatal life.
The vast majority of children die in the 2nd year of life and about 50% - ib age younger than 6 months.
Symptoms may begin less dramatically and may last up to to a certain extent chronic, or they may occur suddenly and cause unexpected death.
ENDOMYOCARDIAL FIBROELASTOSIS (DAVIS DISEASE)
Reports of this disease came not only from the African continent, but also from other parts of the world.

The main symptom is massive fibrous thickening of the endocardium of the apex of the ventricles with thrombosis of the wall above it in 50% of cases. This fibrosis involves the inner myocardium and extends to the mitral and tricuspid valves, binding the papillary muscles and chordae tendineae so that these valves begin to open. reverse side, which leads to regurgitation.
Histologically, the surface of the endocardial zone consists of collagen tissue; the middle layer is occupied by fibrous tissue; the deepest layer consists of granular tissue with chronically inflamed cells and often with varying amounts eosinophils. From this layer, fibrous septa extend into the myocardium, which may experience degenerative changes.
The etiology of the disease is unknown; hypersensitivity reactions are discussed.

It was first described in the early 18th century by G. M. Lancisi. Kreysig (F. L. Kreysig, 1816) called the disease fetal endocarditis. In the domestic literature, the first publication about subendocardial fibroelastosis (1957) belongs to A. M. Wichert (see full body of knowledge). There are isolated subendocardial fibroelastosis and combined with congenital heart defects (see the full body of knowledge: Congenital heart defects) - combined subendocardial fibroelastosis Depending on the size of the left ventricular cavity, Edwards (J. E. Edwards, 1953) distinguished dilated and contractile types subendocardial fibroelastosis The latter in present, time is attributed to congenital heart disease - hypoplastic left ventricular syndrome.

The etiology and pathogenesis are not completely clear. Most researchers believe that the cause of isolated subendocardial fibroelastosis is viruses. The disease appears in the IV-VII months of intrauterine development and belongs to early fetopathy (see full body of knowledge: Antenatal pathology). During this period, following alterative changes, proliferation of elastic and collagen fibers occurs. According to the observations of T. E. Ivanovskaya, A. V. Tsinzerling (1976), when the heart is damaged in the prenatal period after the 7th month, the usual inflammatory reaction, and fibroelastosis does not develop. The presence of foci of sclerosis and signs of inflammation in other organs in patients with subendocardial fibroelastosis indicates a generalized intrauterine infection, one of the manifestations of which is heart damage.

With combined fibroelastosis subendocardial etiological factor simultaneously with the virus, there may be hemodynamic disturbances and hypoxia (see full body of knowledge). In cases of combination of subendocardial fibroelastosis with coarctation of the aorta (see full body of knowledge) and aortic stenosis (see full body of knowledge: Aorta), hemodynamic factors (pressure overload of the left ventricular myocardium), coronary blood flow deficiency associated with myocardial hypertrophy are important. When subendocardial fibroelastosis is combined with left ventricular hypoplasia syndrome, a decrease in coronary blood flow in the myocardium of the left ventricle is also important due to the retrograde flow of blood into the coronary arteries from the aorta. With an abnormal origin of the left coronary artery from the pulmonary trunk (see the full body of knowledge: Bland-White-Garland syndrome), hypoxia is determined by a deficiency of intercoronary anastomoses, the presence of steal syndrome (steal syndrome), which occurs as a result of the discharge of part of the blood from the right coronary artery through the left into the pulmonary trunk , bypassing intercoronary anastomoses. Thus, subendocardial fibroelastosis is the result of a nonspecific reaction of the body in response to exposure to infectious and non-infectious factors, the total effect of which is reduced to hypoxia of the endocardium and the adjacent myocardium. There are other theories of the occurrence of subendocardial fibroelastosis. Thus, Black-Schaffer (B. Black-Schaffer, 1957) believes that diffuse thickening of the endocardium can develop in the case of primary myocardial damage, Johnson (F. Johnson, 1952) regards subendocardial fibroelastosis in congenital heart defects as a result of insufficient oxygenation of the endocardium and myocardium, Noren (G. Noren, 1970) and his colleagues point to the role of myocarditis in the occurrence of subendocardial fibroelastosis. Essentially, in all of these theories, the pathogenetic basis for the formation of subendocardial fibroelastosis is also hypoxia and associated damage to the endocardium and myocardium. Cases of subendocardial fibroelastosis, identified by Westwood (M. Westwood, 1975) and colleagues in siblings (see full body of knowledge: Proband), indicate the possibility of a hereditary predisposition to the disease.

Pathological anatomy. Pathological changes in subendocardial fibroelastosis are quite characteristic.

In isolated subendocardial fibroelastosis, expansion of the left ventricular cavity is observed. The valve apparatus, as a rule, is not changed, but deformation of the mitral and, less commonly, aortic valves is possible. Macroscopically, the heart with subendocardial fibroelastosis is enlarged in size, its weight (mass) increases by 2-4 times. The heart is cut with a crunch; the section reveals diffuse thickening (up to 2-4 millimeters) of the parietal endocardium - typical sign Subendocardial fibroelastosis Usually the endocardium of the left ventricle is involved in the process; at the same time, changes in the endocardium of the atria and right ventricle can be observed. The surface of the thickened endocardium is smooth, whitish or yellowish-gray, pearlescent. Fleshy trabeculae and papillary muscles are immured in the thickened endocardium, the mouths of the smallest veins of the heart (thebesian) are not differentiated. In case of changes in the valve apparatus, diffuse or focal thickening of the cusps of the mitral and aortic valves is detected, in the form of nodules or ridges along the closure line. Such changes are characteristic of both orifice stenosis and valve insufficiency. The chordae tendineae are thickened and sometimes shortened. In the cavities of the heart (especially the left ventricle), parietal thrombi are often found, which can become a source of thromboembolism (see full body of knowledge) of blood vessels great circle blood circulation The myocardium with subendocardial fibroelastosis is flabby, thickened, hypertrophied, similar to boiled meat, dull in section, with whitish cords, papillary muscles and fleshy trabeculae are flattened.

Microscopic picture Subendocardial fibroelastosis is characterized by peculiar changes in both the endocardium and myocardium. The endocardium is thickened 10-15 times and is represented mainly by parallel-running bundles of intertwined elastic and collagen fibers, which is clearly visible when staining with fuchselin (see the full body of knowledge: Weigert staining methods) and picrofuchsin (see the full body of knowledge: Van Gieson method ). The superficial areas of the thickened endocardium are covered with one layer of endothelial cells, under which there is a network of delicate argyrophilic and elastic fibers; not found in the basophilically stained ground substance large number cellular elements, mainly fibrocytes. Under surface layer there is a large number of coarse thick elastic fibers, forming multiple parallel plates with wavy contours. Deeper, thicker and more mature elastic fibers are located in large numbers. Along the elastic fibers, collagen fibers of varying thickness are revealed. Blood vessels capillary type are lined with ordinary endothelium, are few in number and are located in the thickened endocardium under different angles or in the form of chains. Sometimes you can see the fibers of the conduction system of the heart, as if immured in the overgrown connective tissue of the endocardium. Less common are individual atrophic cardiomyocytes, in the sarcoplasm of which drops of lipids and lumps of lime can be found. Small strip-like foci of dystrophic calcification are found in the thickened endocardium and along the capillaries. Inflammatory infiltrates in the endocardium are not detected in subendocardial fibroelastosis; only sometimes small accumulations of lymphoid and macrophage cellular elements can be found in its thickness. The boundary between the altered endocardium and the underlying layers of the myocardium can be clear; more often, however, there is an ingrowth of connective tissue strands from the endocardium into the myocardium, which in the form of wedges penetrate between cardiomyocytes. The connective tissue that grows in the endocardium, rich in elastic and collagen fibers, usually extends to the smallest veins of the heart, the walls of which are significantly thickened, and the lumen in the area of ​​the orifices is obliterated. Directly under the thickened endocardium there is a large number of dilated blood-filled vessels with thin walls and layers connective tissue between them. The formation of these vessels may be associated with the closure of the mouths of the smallest veins of the heart. There is a decrease in the number of coronary arteries (per unit of heart weight), especially pronounced in the subendocardial region. IN small branches Coronary arteries of the heart show mild thickening of the walls due to the proliferation of elastic and collagen fibers in the intima, moderate hypertrophy of the middle layer and perivascular sclerosis with elastosis phenomena.

Myocardial changes in subendocardial fibroelastosis can be expressed in varying degrees. Cardiomyocytes of the subendocardial layer, as a rule, are hypertrophied and have signs of vacuolar and fatty degeneration (see full body of knowledge: Dystrophy of cells and tissues). In areas where elastic and collagen fibers grow, they are atrophic. Fleshy trabeculae are represented by small round islands of atrophic cardiomyocytes located among powerful concentric overlays of elastic and collagen fibers. In the subendocardial layer of the myocardium, necrotic cardiomyocytes are often found, forming small and sometimes significant foci that undergo organization - focal cardiosclerosis (see full body of knowledge). In the thickness of the myocardium scar changes minor and not always found. Foci of cardiosclerosis may contain a large number of elastic fibers; areas of dystrophic calcification of scar tissue can be found in them. In the intramural and subepicardial layers of the myocardium, hypertrophy and focal fatty degeneration cardiomyocytes, coarsening of the stroma, moderate proliferation of connective tissue between cardiomyocytes and around the vessels. Inflammatory infiltrates in the myocardium are usually absent. The epicardium is without features, sometimes you can detect areas of sclerosis, as well as adhesions between the layers of the pericardium.

In internal organs with subendocardial fibroelastosis, a picture of venous stagnation is detected, sometimes - foci of sclerosis and inflammation.

With combined fibroelastosis subendocardial macro and microscopic changes in the endocardium of the left ventricle correspond to the pathological picture of isolated fibroelastosis subendocardial However, in the myocardium, mainly in the basin of the left coronary artery, subendocardial and transmural myocardial infarctions (see full body of knowledge) of varying duration, focal and diffuse cardiosclerosis with the formation of chronic cardiac aneurysms (see full body of knowledge).

Clinical picture. The disease can occur lightning fast, acute and chronic. Symptoms of isolated subendocardial fibroelastosis appear, as a rule, in the first 6-12 months, less often in the 2-3rd year of life and consist of cardiac and extracardiac signs. Children experience pallor, lethargy, sweating, slight cyanosis, fatigue during feeding, and poor weight gain. From the outside cardiovascular system observed arterial hypotension(see full body of knowledge: Arterial hypotension), early developing cardiac hump, cardiomegaly, dull heart sounds, gallop rhythm (see full body of knowledge: Gallop rhythm), absence of murmur or systolic murmur associated with relative mitral valve insufficiency. Symptoms of refractory left ventricular heart failure predominate (see full body of knowledge) in the form of shortness of breath, tachycardia, congestive wheezing in the lungs. In most children, the liver protrudes 2-4 centimeters from under the edge of the costal arch. Swelling is rare. In the chronic course of Fibroelastosis, subendocardial cardiomegaly, dull sounds, and refractory heart failure remain stable. In cases of a relatively benign course, the size of the heart decreases, but dullness of tones and changes in the ECG and radiograph remain, indicating cardiosclerosis.

When subendocardial fibroelastosis is combined with congenital heart defects, there is a discrepancy between the clinical manifestations of the defect and significantly increased heart size, dull sounds, and intracardiac blockades.

Complications of subendocardial fibroelastosis include Morgagni-Adams-Stokes syndrome (see full body of knowledge: Morgagni-Adams-Stokes syndrome) against the background of complete transverse heart block, thromboembolic syndrome (see full body of knowledge: Thromboembolism) with the development of hemiparesis.

The diagnosis is made based on medical history ( viral infections or exacerbation of chronic infectious diseases of the mother, as well as toxicosis of pregnant women in the first half of pregnancy), clinical manifestations disease and instrumental examination results.

At X-ray examination a normal or slightly enhanced pulmonary pattern, cardiomegaly, spherical or ovoid shape of the heart is revealed (Figure). Pulsation is reduced. Cardiothoracic index - the ratio of the transverse size of the heart to the transverse size chest at the level of the diaphragm, expressed as a percentage, is 70-75% in most patients (normally 50% in children). On the ECG (see full body of knowledge: Electrocardiography) - normal position electrical axis heart, high wave voltage, rigid rapid rhythm, signs of myocardial hypertrophy of the left atrium and ventricle, deep negative or smoothed T waves in leads V 4-6, descent of the ST segment below the isoline. Rhythm and conduction disturbances are not typical for subendocardial fibroelastosis, however, extra asystole may occur (see full body of knowledge), paroxysmal tachycardia(see full body of knowledge), intraventricular and atrioventricular blockade (see full body of knowledge: Heart block). A phonocardiographic study (see full body of knowledge: Phonocardiography) reveals a significant decrease in the amplitude of the first tone and a ribbon-shaped systolic murmur in the region of the apex of the heart.

Using echocardiography (see full body of knowledge), dilatation of the left ventricular cavity is detected. The diameter of the cavity in systole and diastole changes little, the thickness of the walls is increased or approaches normal.

During catheterization of the heart cavity (see full body of knowledge: Cardiac catheterization) and angiocardiography (see full body of knowledge) sharp dilatation and spherical shape of the left ventricle, signs of mitral valve insufficiency, impaired myocardial contractility, increased end-diastolic pressure in the left ventricle, pressure in the pulmonary capillaries, pulmonary artery.

In cases of unclear diagnosis, endomyocardial biopsy of the left ventricle is sometimes used.

Differential diagnosis is carried out with an anomalous origin of the left coronary artery from the pulmonary trunk, which is characterized by a systolic-diastolic murmur in the second intercostal space on the left, attacks of sudden anxiety accompanied by a cry, focal changes on the ECG in the area of ​​the anterolateral wall of the left ventricle (see full body of knowledge: Myocardial infarction); with glycogenosis type II (see full body of knowledge: Glycogenosis), in which generalized muscle weakness, macroglossia, hepatomegaly, shortening of the PR interval on the ECG. In children early age Subendocardial fibroelastosis is differentiated from acquired myocarditis (see full body of knowledge), which is evidenced by the positive dynamics of the process during treatment; in adults - with secondary fibrotic changes myocardium against the background of previous heart diseases (myocardial infarction, endocarditis and others).

Treatment. Specific treatment Subendocardial fibroelastosis no. The complex of therapeutic measures includes restriction motor activity and prescribing an appropriate diet (restriction of salt and fluid) during the period of severe heart failure. When a viral bacterial infection occurs, young children are given a course of antibacterial therapy. They prescribe prednisolone, cardiac glycosides, diuretics - furosemide (Lasix), veroshpiron; drugs that improve metabolic processes in the myocardium (panangin, potassium orotate, cocarboxylase, riboxin, vitamins B 6, B 15, B 5); for tachycardia - P-blockers, for example, anaprilin (obzidan).

The prognosis is usually unfavorable.

Prevention. Pregnant women should avoid contact with people with acute respiratory diseases(wearing a mask, instilling interferon into the nose, UV irradiation of the apartment); reception recommended ascorbic acid, B vitamins.

Are you categorically unhappy with the prospect of disappearing from this world forever? You don't want to finish your life path in the form of a disgusting rotting organic mass devoured by grave worms swarming in it? Do you want to return to your youth and live another life? Start all over again? Correct the mistakes made? Make unfulfilled dreams come true? Follow the link:

Myocardial fibroelastosis- rare disease, which is detected mainly in children of the first year of life. In most cases it is congenital pathology hearts.

Etiology and pathogenesis are not clear. There are different points of view regarding the causes of the occurrence: congenital malformation of the aortic cone, congenital cardiosclerosis due to intrauterine endomyocarditis or hypoxia. Some authors consider fibroelastosis as special form collagenosis.

Pathomorphology. On section, the heart is sharply enlarged, myocardial hypertrophy, thickening of the endocardium, as well as the heart valves are noted due to the proliferation and sclerosis of collagen and elastic fibers.

Clinic. In children in the first months of life, fulminant or acute form, may be observed in older age chronic course diseases. At acute course there is a progressive increase in the phenomena cardiovascular failure. Shortness of breath and pallor are observed skin with a cyanotic tint, cough, displacement of the borders of the heart, dullness of heart sounds, irregular heartbeat. Sometimes heart murmurs are heard.

Diagnosis is established on the basis of the clinical picture and X-ray examination data, which reveals a sharply enlarged spherical heart. Angiocardiography reveals wall thickening, a significant increase in the size of the left ventricle and its slow emptying.

Differential diagnosis. The clinical picture is similar to idiopathic myocarditis and congenital heart defects.
The prognosis is often unfavorable. 90% of children do not live to be two years old.

TREATMENT

Usually you have to use various heart medications, corticosteroids and vitamins for a long time. Most a common complication congenital heart defects is bacterial endocarditis.

Children with heart defects, accompanied by an increase in blood supply to the lungs, are prone to frequent infections respiratory tract. Against the background of congenital heart disease, a rheumatic process can develop.

A serious complication is brain damage developing against the background of severe brain hypoxia. Thrombosis of cerebral vessels develops more often in children with cyanotic defects and increased viscosity blood. A factor contributing to the development of cerebral vascular thrombosis may be dehydration.

Patients with cyanotic defects may develop brain abscesses. In addition, in patients with reduced blood supply to the pulmonary circulation, tuberculosis may develop.

Endocardial fibroelastosis is a rare developmental defect - the proliferation of connective tissue rich in elastic fibers. Frequency 1 in 5500 newborns. In 93% of cases, the heart is sharply enlarged in size. Fibroelastosis, in most cases, occurs sporadically, but there are also recessively inherited forms. 75% of children die in the first year of life. Repeated genetic risk ranges from 3.8% to 25% (depending on the form).

At the same time, it should be emphasized that along with the rigidly programmed “work” of genes, the entire genetic apparatus environment during ontogeny, it has a diverse effect on gene activity (link).

The cause of most congenital heart defects is unknown. It is possible that many cases are due to exposure to unknown teratogens in the first trimester of pregnancy during cardiac development. Teratogen is an external agent - it can be anything to which a woman is susceptible - elevated temperature, we are taking medications, even banal citramon or aspirin – M.b. Your friend took some drugs during her pregnancy—and teratogens act not only on the gene (i.e., activating or suppressing), but also on the cells of the embryo or fetus. Those. such a pathology may indeed not manifest itself if, for example, the provoking agent is identified and excluded external factor(i.e. leave only heredity). Those. You need to study in detail with a good doctor the entire ontogenesis of pregnancy, how it proceeded, eliminate possible irritating factors (extra medications, stress, etc.), restore strength after such a difficult physically difficult period, and try again. You can, for example, try to carry and give birth in another city... I’m already fantasizing about this... But here it’s really difficult to advise anything, especially not to a specialist in congenital diseases (my medical education is rather weak, I can only explain the nature)

Good luck to your friend. Let him not despair and, first of all, give his body a good rest.

--------------------
in the EMOTIONS competition Sofochka is number 73
follow the VOTE link - number of your favorite photo
***
Win a laptop from Balitka