Non-infectious fetopathies. Endocardial fibroelastosis, diabetic fetopathy. Subendocardial fibroelastosis in newborns, symptoms and treatment

Endocardial fibroelastosis is a diffuse thickening of the endocardium of one or more cardiac chambers, formed by collagen or elastic tissue. The lesion can be isolated or combined with other congenital anomalies of the heart, causing congenital heart rhythm disorders. The first description dates back to the 18th century (Lancusi, 1740). According to the literature, the frequency ranges from 4 to 17% (Bankle G., 1980) [I].

The etiology of the pathology is unclear. Currently, according to many hypotheses, among the most likely causes are inflammatory processes under the influence of the Coxsackie mumps virus. TO possible reasons also include collagen diseases, autoimmunity, inborn metabolic disorders, difficulty in lymphatic drainage (Schryer M.J., Karnauchow P.N., 1974). Heredity is also a subject of discussion, since there are cases of hereditary transmission of the disease according to an autosomal recessive type or X-linked type of inheritance (Chen S. et al. 1971, Westwood M. et al, 1975).

Endocardial fibroelastosis most often affects the left side of the heart. According to the size of the left ventricle, it is divided into two types: dilated, the most common, in which the left ventricle is enlarged and hypertrophied, and contractile, in which the size of the left ventricle is normal or even reduced. The degree of myocardial fibrosis varies (Hastreiter A.F., 1968).

Extracardiac anomalies are rarely combined with endocardial fibroelastosis.

Materials and methods

In the department functional diagnostics Science Center In obstetrics and perinatology of the Russian Academy of Medical Sciences, more than 2,000 pregnant women undergo screening tests annually. Endocardial fibroelastosis was detected in 3 cases in isolated form (dilated type - 2 patients and contractile type - 1 case) and in 7 cases in combination with congenital heart defects. Relative to the total number of diagnosed congenital anomalies of cardio-vascular system pathology was 2.7%.

Most often, fibroelastosis was observed in pathologies of the left heart: stenosis and coarctation of the aorta, hypoplastic left ventricular syndrome, congenital mitral stenosis and parachute-shaped mitral valve. In addition, there was a combination of stenosis pulmonary artery and tricuspid atresia with endocardial fibroelastosis.

The studies were carried out on high-resolution ultrasonic devices using 3.5 and 5.0 MHz transducers. The following modifications of the method were used: B-scanning, M-method, flow, .

This message is dedicated to describing the possibilities ultrasound scanning in the diagnosis of an isolated form of fibroelastosis.

results

A screening study in 3 observations of young primigravidas with a minimum gestational age of 24 weeks revealed identical changes in the fetal heart. There was a significant increase in the size of the heart due to the left ventricle with its spherical configuration. The apex of the heart is completely formed by the left ventricle. The myocardium of the left ventricle is thickened and the cavity is spherically dilated, while the interventricular septum protrudes into the cavity of the right ventricle (Fig. 1). The papillary muscles are also thickened. As an ultrasound sign that is not noted in any other cardiac anomaly, attention was drawn to the sign of a “luminous” endocardium - dense and shiny, the degree of reflection from which is observed even with a slight decrease in the gain of the ultrasound beam.

The contractility of the left ventricular myocardium is significantly changed: the amplitudes of contraction and diastolic relaxation are presented almost in the form of a straight line (Fig. 2). The degree of leaflet opening (3 mm) and blood flow through the mitral valve are significantly reduced (Fig. 3). Unlike the mitral valve, blood flow through the tricuspid valve is increased. Tricuspid regurgitation is also detected (Fig. 4).

It should be noted that in none of the observations of an isolated form of endocardial fibroelastosis, heart failure was observed in the fetus.

Based on the data obtained, indicating pronounced disturbances in cardiac function, a sharp disturbance in the contractility of the left ventricular myocardium, and significant ultrasonic density of the myocardium, round shape an enlarged left ventricle and its deflection into the cavity of the right ventricle, the “luminous” nature of the myocardium and a decrease in blood flow through the mitral orifice, it was suggested that there was an isolated form of endocardial fibroelastosis.

Pregnancy in all patients was terminated. Autopsy data confirmed the presumptive diagnosis of antenatal ultrasound examination.

Discussion

In the literature there are descriptions of isolated cases of a presumptive diagnosis of endocardial fibroelastosis. The diagnosis was made definitively after the birth of the child in the presence of signs of congestive heart failure (Allan L. et al., 1981). The authors who described the ultrasound signs of fibroelastosis paid attention to the contractile abilities of the myocardium (Bovicelli L. et al., 1984), and took into account the shortening index anterior-posterior size ventricles according to the formula proposed for adults (Feigenbaum H., 1976).

The use of transvaginal echography allowed Rustico M. et al. (1995) to identify pathology at 14 weeks of gestation, confirming the diagnosis at 16 weeks, as well as during a sectional study after termination of pregnancy. The authors pay attention to increased echogenicity endocardium, decreased myocardial contractility. The authors' observations also included obstruction of the left ventricular outflow tract and narrowing of the ascending aorta, through which blood flow was not recorded.

Of course, antenatal diagnosis of fibroelastosis is based largely on impaired myocardial contractility and endocardial density. According to our data, the most characteristic is the density of the endocardium, reaching several millimeters, the echopositivity of the endocardial structure and the “luminous” endocardium (see Fig. 1). Depending on the shape, the cavity can be expanded and present classic version pathologies - motionless walls, shiny dense endocardium and protrusion interventricular septum towards the right ventricle, i.e. spherical shape of the left ventricle.

The absence of heart failure with such pronounced disorders of the fetal heart can be explained by significant dilatation of the left ventricle, since with any increase in blood volume, less internal excursion of the wall of the dilated ventricle is required, and normal stroke volume is achieved by a lower contractile force compared to the norm, especially since The right ventricle is subject to the main load antenatally.

The presented observations are an illustration of antenatal ultrasound diagnostics isolated dilated form of fibroelastosis of the left ventricular endocardium.

In the presence of endocardial fibroelastosis, termination of pregnancy is indicated, since symptoms of circulatory failure occur in the first months after birth and most children die before two years of age.

Thus, the observational results confirm the importance of ultrasound diagnostics in identifying such rare congenital heart anomalies in the fetus as endocardial fibroelastosis, which makes it possible to determine obstetric management tactics for pregnant women and a priori help reduce neonatal mortality.

Literature

1. G. Buncle. Congenital defects of the heart and large vessels. - M.: Medicine, 1980.
2. Schryer M.J.P., Karnauchow P.N. Endocardial fibroelastosis. - Am. Heart J. - 1974. - v.88. - p.557.
3. Chen S., Thompson M.W., Rose V. Endocardial fibroelastosis: Family studies with special reference to counseling. - J.Pediatr., 1971.-79.-385.
4. Westwood M., Hariss R., Burn J. et al. Heredity in primary endocardial fibroelastosis. - Br. Heart J. 1975. - 37. - 1077.
5. Hastreiter A.F. Endocardial Fibroelastosis. In: Heart Disease in Infants, Children and Adolescents. Moss-Adams, Williams and Vilkins Co., Baltimore, 1968.
6. Allan L., Litlle D., Campbell S., Whitenead M. Fetal ascites associated with Congenital Heart Disease: Case Report. Brit J. of Obstetrics and Gynecology 1981. - v.88. -p.453-455.
7. Bovicelli L., Picchio F.M., Piu G. et al. Prenatal diagnosis of endocardial fibroelastosis. - Pren. Diagn. - 1984. - 4.-67.
8. Feigenbaum H. Echocardiography. Philadelphia, 1976.
9. Rustico M.A., Benettoni A., Bussani R., Maieron A. et al. Early fetal endocardial fibroelastosis and critical aortic stenosis: A case report // Ultrasound Obstetr. Gynecol. - 1995. - v.5. - p. 202-205.

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 valve stenosis, etc.
It is difficult to determine whether endocardial thickening is “secondary” as a result of changes in hemodynamics caused by stenotic lesions, or “primary”, transferred to the valves. Therefore, the terms “isolated” and “complicated” fibroelastosis would 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. with involvement of the 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 an 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 right ventricular endocardium (rare).

1. Isolated.
2. Complicated:

a) involving the pulmonary valve (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 of congenital heart defects; 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 by numerous currently existing hypotheses, including inflammatory processes in the endocardium and myocardium, Coxsackie B virus, mumps, hypoxia, mechanical obstruction of blood flow, myocardial hyperplasia, endocardial hyperplasia, elastic hyperplasia, collagen disease, autoimmunity, hereditary disorders, congenital metabolic disorders, 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 of the vascular canals. 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 the 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. Right atrium is affected in only one out of 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 according to at least Several cases of the contractile type are a transitional form between the anatomical complexes with hypoplasia of the left ventricle and the more common variant of endocardial fibroelastosis of the dilated type.
Combination with congenital mitral valve insufficiency: 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, forming a large complex with it. 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 abnormal position papillary muscles is more likely acquired 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 difficulty in 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: the 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 a 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 was observed in combination with congenital left ventricular aneurysm, idiopathic right atrium enlargement, infantile calcification coronary arteries, situs inversus and dextroversion of the heart. 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 be somewhat 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 variable numbers of 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.

Definition

Endocardial fibroelastosis has recently been classified as a cardiomyopathy. The disease may be congenital primary or secondary, associated with obstruction upper section left ventricle.

Causes

The primary form of endocardial fibroelastosis occurs due to various etiological factors acting on the fetus, so some authors classify it as so-called fetal endocarditis. The first trimester of pregnancy is vulnerable. If the fetal endocardium experiences damage in more than late dates, a relatively benign form of the disease occurs, which can exist relatively longer without fatal outcome.

Morphological changes in the endocardium determine the rigidity of the left ventricular cavity and the rigidity of its volume throughout the entire period of the child’s postnatal life. Inner shell the heart thickens, its elastic fibers are replaced by collagen tissue.

Along with this, contractile changes occur in the myocardium itself, leading to its hypertrophy. Coronary blood supply is disrupted, and signs of subendocardial ischemia develop. Both the contractile function of the heart and the diastolic filling of the left ventricular cavity are affected. A condition occurs in which, as a result of replacement of the internal elastic layer connective tissue the left ventricle is not able to accommodate the required physiological volume of blood for age, and the myocardium, through the mechanical resistance of the altered endocardium, is not able to displace it into the aorta. The consequence of these processes is the invariance of shock release into the systemic circulation throughout the child’s life. The minute volume is compensatory supported by the heart rate. Exhaustion of chronotropic regulation of hemodynamics leads to severe disorders heart rate with attacks of supraventricular tachycardia and an increase in total heart failure.

Symptoms

The disease has a severe progressive course and ends in death from increasing circulatory failure in newborns and children early age. Primary signs diseases can manifest as lethargy, inability to suck, shortness of breath, perioral and diffuse cyanosis, symptoms of left ventricular failure. Hemodynamic disorders rapidly progress, acquiring signs of refractoriness to cardiotropic therapy.

The disease has a severe course, unfavorable prognosis and ends in death within the first months. Clinically, changes in the heart are very diverse. Percussion, the boundaries of cardiac dullness may be unchanged or slightly shifted to the left. Auscultation against the background of age-related or paroxysmal tachycardia, the sonority of tones can be sufficient, and sometimes louder. In some cases, embryocardia occurs. An important sign is the absence of 279 murmurs above the precordial area and outside it, which to a certain extent may complicate the diagnosis of primary cardiac damage.

Diagnostics

Diagnostic assistance in these cases is provided by ECG registration. The most characteristic changes are considered to be nomotopic heart rhythm disturbances, most often in the form of its rigid acceleration, increased electrical activity schulochny complex, intraventricular block and a pronounced left-type ECG. In addition, signs of left ventricular hypertrophy, disturbances of subendocardial blood flow, repolarization processes, and ischemia are determined.

It is advisable to carry out an X-ray examination in two projections, taking into account the physiological characteristics of this period of the child’s life, as well as the dominant lesions of the left side of the heart. In frontal photographs, in typical cases, the heart has the shape of a ball. X-ray examination in the left lateral projection with barium contrast of the esophagus makes it possible to determine the degree of hypertrophy of the left chambers of the heart and reduction of the retrocardial space.

Echocardiographic imaging reveals a decrease in the cavity of the left ventricle against the background of endocardial compaction. Possible enlargement of the left atrium. The myocardium of the left ventricle is hypertrophied, and the mobility of the interventricular septum is reduced. Contractile and relaxation parameters of the heart are reduced.

Prevention

Features of the treatment of children with endomyocardial disease due to fibroelastosis is insensitivity to inotropic drugs, i.e. cardiac glycosides and non-glycoside drugs, their positive inotropic effect can cause a deterioration in the child’s condition with the onset of instant death through cardiac tamponade.

Basic treatment of fibroelastosis includes lifelong corticosteroids, diuretics, calcium and aldosterone antagonists. Surgical intervention, aimed at endocardectomy, was not found today wide application.

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Elena: 03/21/2017
Hello. Please explain the electrocardiogram of my son, who is now 17 years old. Pediatric cardiologist in our town there is none, and an adult cardiologist will only accept him at the age of 18. My son is applying to a military school. How serious are these deviations from the norm? A year ago, my son had an ECG done several times: both at the military registration and enlistment office and at the children's hospital. None of the doctors spoke about the abnormalities, and accordingly, no treatment was prescribed, although I asked them about it, because I knew that my son dreamed of military service. Even at the military registration and enlistment office, he was given health group A1 based on this ECG and ultrasound of the heart, which did not reveal any abnormalities. Please tell me whether the deviations are serious, how to treat it and whether it can be cured, can he play sports without restrictions (he is a track and field athlete) and will this become an obstacle to entering a military school? My son is very worried that his dream is impossible. Thank you. ECG readings from a year ago: RR 0.76 PQ 0.12 QRS 0.10 QT 0.36 Heart rate 78 Sinus rhythm. EOS was not rejected. Incomplete blockade right leg His bundle. Increased electrical left ventricular myocardial activity. Disruption of myocardial repolarization processes in the inferior wall of the left ventricle. ECG readings in 2017: RR 0.78 PQ 0.12 QRS 0.12 QT 0.38 Heart rate 77 Sinus rhythm. EOS is closer to vertical. Incomplete blockade of the right bundle branch. Increased electrical left ventricular myocardial activity. Moderate changes in the inferior wall of the left ventricle.

Endocardial fibroelastosis is more often detected in young children, less often in adults. Individual cases have been described when it was first recognized in elderly people (P. D. White, 1960). Apparently, the point of view of those authors who distinguish between primary and secondary forms is correct. In the latter case, fibroelastosis develops in patients with congenital heart defects that occur with increased intraventricular pressure (aortic stenosis, coarctation or hypoplasia of the aorta, pulmonary stenosis). We consider the opinion of the authors who explain these cases to be a combination of two birth defects less correct.

With endocardial fibroelastosis, due to significant thickening of the endocardium, emptying of the cavities of the heart, primarily the left ventricle and atrium, becomes difficult. As a result, myocardial hypertrophy occurs, and then heart failure. Dilatation of the heart chambers may be absent. In some cases, due to valve involvement, slight mitral regurgitation develops, which is most often associated with relative mitral regurgitation. With fibroelastosis, shortness of breath, tachycardia, and cyanosis develop early. When examining the heart, an increase in its size is detected, especially in the left ventricle and atrium. Heart murmurs may be absent or a systolic murmur may be heard, characteristic of mitral insufficiency noise. The ECG shows signs of left ventricular hypertrophy. Later, various other changes appear, including conduction disturbances, decreased QRS voltage, and ectopic arrhythmias. However, sometimes paroxysms of atrial fibrillation occur long before the onset of heart failure. Serious complication, which is more common in late stages diseases are thromboembolism in both the small and big circle blood circulation Usually they proceed favorably, without serious consequences.

Differential diagnosis is very difficult, especially when identifying this disease in adults. In those more in rare cases When the size of the heart is slightly enlarged, the more common constrictive pericarditis is sometimes mistakenly recognized. With large heart sizes, chronic myocarditis is most often suspected, which is usually indicated by a more protracted course and absence of signs of active inflammatory process. However, it should be borne in mind that these patients (including children) are very prone to secondary pulmonary infection, which, periodically exacerbating, can cause the appearance of these signs. Angiography reveals limited excursion of the left ventricular wall and delayed emptying of its cavity, although cardiac index most often remains normal.

As an example, we can cite the medical history of a patient we observed, whose diagnosis was verified at autopsy.

Patient M, 40 years old, was in the clinic from October 31 to December 22, 1972.

In 1966, pain in the heart region began to bother me for the first time. Since the summer of 1971, the condition worsened: pain in the heart area became more frequent, shortness of breath appeared and began to increase during physical activity. In March 1972, an attack developed sharp pain in the epigastric region with irradiation to the left half of the chest and left hypochondrium. In May after the postponed bilateral pneumonia She began to notice shortness of breath at rest, and peripheral edema appeared. In July, during hospitalization in a niche clinic, an increase in LD to 150/110 mm Hg was noted. Art. During auscultation of the heart, a systolic murmur was detected at all points with a maximum at the left edge of the sternum. Proteinuria up to 0.15 g/l (0.15%0) was detected. The clinic discussed diagnoses of subaortic stenosis or birth defect hearts. The patient was treated with cardiac glycosides and diuretics. She was discharged for outpatient treatment in relatively satisfactory condition.

Upon re-admission, she complained of periodic stabbing and squeezing pain in the heart area, severe shortness of breath at rest, and swelling lower limbs, sweating, weakness, weight loss.

General condition of the patient moderate severity. Position in bed with the head end elevated. Cyanosis of the lips, severe acrocyanosis. The shape of the fingers resembles drumsticks. Severe swelling of the lower extremities. Shortness of breath at rest up to 30 breaths per minute. Percussion over lungs pulmonary boxy sound. On auscultation breathing is hard, no wheezing. The area of ​​the heart and large vessels is not changed. There is pronounced pulsation carotid arteries. Borders of the heart: right - along the right edge of the sternum, upper - along the third intercostal space, left 2 cm outward from the left midclavicular line. Auscultation: sounds are somewhat muffled, rough systolic murmur at the apex and at the V point. Blood pressure 130/90 mm Hg, pulse 102 per minute, rhythmic. There is no free fluid in the abdomen. The liver is slightly enlarged. The spleen is not palpable. On chest x-ray, the pulmonary pattern in the middle and lower zones is enhanced due to pronounced venous stagnation. The roots of the lungs are expanded. A small amount of fluid is detected in the left external sinus. The heart is significantly expanded in diameter, mainly to the left. The waist of the heart is smoothed. In the first oblique projection, the contrasted esophagus is deviated posteriorly along an arc of large radius. In the second oblique projection there is a significant increase in the left ventricle. The right chambers of the heart are slightly enlarged.

There is a deviation on the ECG electrical axis hearts to the left. Horizontal position. Sinus tachycardia. Pronounced changes in the myocardium of the hypertrophied left ventricle. In dynamics, polytopic extrasystole, periodically bigeminy, and trigeminy were recorded. On the ECG from 19/XII temporary blockade left bundle branch.

Common blood test and data biochemical research without deviations from the norm.

Urinalysis: protein 0.33 g/l (0.33 ‰), otherwise unremarkable. The diagnosis remained unclear. The diagnosis of rheumatism, combined rheumatic heart disease, congenital heart disease, and late fibroelastosis was discussed.

In the clinic in the background bed rest treatment was carried out with cardiac glycosides, diuretics, potassium preparations, cocarboxylase, acetylsalicylic acid. The patient's condition improved somewhat, swelling disappeared, shortness of breath and acrocyanosis decreased, although they remained transient disturbances rhythm. On December 22, at 1:30 p.m., the patient died suddenly.

Pathological diagnosis: endocardial fibroelastosis, diffuse small-focal cardiosclerosis, severe myocardial hypertrophy (heart weight 520 g, wall thickness of the left ventricle 1.8 cm, right 0.4 cm), chronic venous congestion internal organs, nutmeg liver fibrosis.

Thus, the patient developed cardiac damage in adulthood with left ventricular hypertrophy, subsequent dilatation of the cavities, the appearance of widespread systolic murmur and progressive heart failure. At the same time, there were no signs of the inflammatory process, and the nature of the changes in the heart made it possible to more likely deny both acquired and congenital defects. The hypertension was apparently incidental, and the proteinuria appeared to be associated with congestion. The patient had endocardial fibroelastosis with concomitant myocardial damage very likely, which was confirmed at autopsy. Noteworthy was the late manifestation of the disease, accompanied by a fairly pronounced systolic murmur, which, obviously, was of muscular, but not valvular origin.

Endomyocardial fibroelastosis(FE) is a disease characterized by the presence of inner surface ventricles and heart valves of diffusely thickened endocardium. The incidence of FE is 1:70,000 newborns. The disease has been described under various names, including fetal endocarditis, endocardial fibrosis, prenatal fibroelastosis, elastic tissue hyperplasia, and endocardial sclerosis.

Etiology of endomyocardial fibroelastosis. There are primary and secondary FE:
with primary fibroelastosis of the endomyocardium, it is impossible to identify obvious etiological factors;
with secondary fibroelastosis of the endomyocardium, the child exhibits signs of severe congenital heart disease of the left side of the heart of the obstructive type (stenosis or atresia of the aorta, coarctation of the aorta, LV hypoplasia).

Until now time genesis of the disease remains unclear. Possible etiological factors include inflammatory or infectious process, impaired development of the endocardium or insufficiency of its blood supply. Endomyocardial fibroelastosis can also be induced by genetic and hypoxic factors. In some cases, the appearance of PE may be a consequence of hemorrhage into the myocardium, which predetermines subsequent dilatation of the left ventricular cavity. Changes in the endocardium can also be caused by primary myocardial damage, in which, due to expansion of the heart and stretching of the endocardium, proliferation of fibroelastic fibers begins.

During pathological examination the heart is often significantly enlarged in size. The LV cavity in secondary forms of FE is reduced, while in primary FE the LV cavity is sharply expanded. A thickening of the fibroelastic fibers of the endocardium of a milky white color is detected, which is especially pronounced in the left parts of the heart. The process can spread to the valves, especially often to the aortic and mitral valves.

Thickening is determined microscopically endocardial fibroelastic fibers accompanying trabecular sinusoids. This process leads to degenerative changes in the subendocardial region of the ventricular myocardium with subsequent necrosis of muscle fibers in this area. Involvement in pathological process valves is characterized by myxomatous proliferation with an increase in collagen elements.

Clinical manifestations vary.
In the first variant of the course of FE, the clinical picture of the disease can be determined already from the first hours of the child’s life. A gallop rhythm is heard in combination with a systolic murmur at the apex of the heart. Then signs of acute heart failure increase in combination with central nervous system depression. Heart rhythm disturbances are often diagnosed. These patients are similar in appearance to children with sepsis and pneumonia.
In the second variant of the course of FE, the disease begins before the age of six months and is characterized by the development of severe heart failure in an apparently healthy child, which is often provoked by infection respiratory tract. With primary FE, the child is often in critical condition. He develops shortness of breath, cough and anorexia. Cyanosis is rarely detected and appears in terminal stage diseases. The pressure in the jugular venous system increases, the liver increases significantly in size, and swelling of the extremities, in the sacrum or face, occurs. Moist rales of various sizes are heard in the lungs, caused by stagnation of blood in the vessels of the pulmonary circulation. The percussion dimensions of the heart increase. Murmurs of mitral valve insufficiency are often heard.

Diagnosis of endomyocardial fibroelastosis.

Ultrasonography allows diagnosing endomyocardial fibroelastosis in fetuses at the age of 20 weeks of gestation. Repeated ultrasound examinations fetuses show a gradual evolution of the left ventricular cavity - from its dilation to the formation of a small cavity with a very thick wall. Such changes are confirmed at autopsy in the absence of other cardiac development abnormalities in the fetus.

On the electrocardiogram in newborns children with endomyocardial fibroelastosis signs of hemodynamic overload and LV hypertrophy, T wave inversion in I, II and left precordial leads are recorded.

Echocardiography shows dilatation LV cavity and moderate thickening of its wall with dense thickened endocardium. The exile faction has been shortened.
On a chest x-ray, an increase in the size of the heart is observed until the formation of a “spherical” heart.

Treatment of endomyocardial fibroelastosis aimed at relieving heart failure and preventing concomitant infections. Therapy is symptomatic and depends on the degree of hemodynamic disturbances. Maintenance therapy with cardiac glycosides and diuretics is used. The use of cardiac glycosides is long-term, for several months, sometimes more than a year.

Prognosis of endomyocardial fibroelastosis depends on the time of occurrence of the typical clinical picture. The earlier the symptoms of fibroelastosis appear, the more serious the prognosis. If signs of heart failure are recorded in the first days of life, the prognosis is unfavorable. When PE is combined with valvular heart defects or congenital pathology cardiovascular system, children die during the first months of life.