Possibilities of fetal ultrasound cardiology in the diagnosis of endocardial fibroelastosis. Endocardial fibroelastosis

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

With endocardial fibroelastosis, due to a significant thickening of the endocardium, it is difficult to empty the heart cavities, primarily the left ventricle and atrium. As a result, myocardial hypertrophy occurs, and then heart failure. Dilatation of the chambers of the heart may be absent. In some cases, due to the involvement of the valves in the process, a small mitral regurgitation develops, which is most often associated with relative mitral insufficiency. With fibroelastosis, shortness of breath, tachycardia, and cyanosis develop early. Examination of the heart reveals an increase in its size, especially in the left ventricle and atrium. Murmurs in the heart may be absent or a systolic murmur characteristic of mitral insufficiency may be heard. The ECG shows signs of left ventricular hypertrophy. Later, various other changes appear, including conduction disturbances, a decrease in the voltage of the QRS complex, ectopic arrhythmias. However, sometimes paroxysms of atrial fibrillation occur long before the onset of heart failure. A serious complication, which is more common in the later stages of the disease, is thromboembolism in both the pulmonary and systemic circulation. Usually they proceed favorably, without serious consequences.

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

As an example, we can cite the case history of a patient we observed, in which the diagnosis was verified at autopsy.

Patient M, 40 years old, was in the clinic from 31/X to 22/XII 1972.

In 1966, for the first time, pains in the region of the heart began to disturb. Since the summer of 1971, his condition worsened: pain in the region of the heart became more frequent, shortness of breath appeared and began to increase during physical exertion. In March 1972, an attack of sharp pains developed in the epigastric region with irradiation to the left half of the chest and left hypochondrium. In May, after suffering from bilateral pneumonia, she began to notice shortness of breath at rest, peripheral edema appeared. In July, during admission to a niche clinic, an increase in LD to 150/110 mm Hg was noted. Art. Auscultation of the heart revealed systolic murmur at all points with a maximum at the left edge of the sternum. Proteinuria up to 0.15 g/l (0.15% 0) was found. The clinic discussed the diagnosis of subaortic stenosis or congenital heart disease. The patient was treated with cardiac glycosides, diuretics. In a relatively satisfactory condition, she was discharged for outpatient treatment.

On re-admission, she complained of recurrent pain in the region of the heart of a stabbing and squeezing nature, severe shortness of breath at rest, swelling of the lower extremities, sweating, weakness, and weight loss.

The general condition of the patient is moderate. Position in bed with a raised head end. Cyanosis of the lips, pronounced acrocyanosis. The shape of the fingers resembles drumsticks. Severe edema of the lower extremities. Shortness of breath at rest up to 30 breaths per minute. Percussion above the lungs pulmonary sound with a box shade. On auscultation, breathing is hard, no wheezing. The area of ​​the heart and large vessels is not changed. There is marked pulsation of the 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. Auscultatory: the tones are somewhat muffled, rough systolic murmur at the apex and at the V point. BP 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 radiography, the pulmonary pattern in the middle and lower zones is enhanced due to pronounced venous congestion. The roots of the lungs are expanded. On the left in the external sinus is determined by a small amount of fluid. Heart is considerably expanded in the diameter, mainly to the left. The waist of the heart is flattened. In the first oblique view, the contrasted esophagus deviates posteriorly along a large radius arc. In the second oblique projection there is a significant increase in the left ventricle. The right parts of the heart are slightly enlarged.

On the ECG, the deviation of the electrical axis of the heart to the left. horizontal position. Sinus tachycardia. Pronounced changes in the myocardium of the hypertrophied left ventricle. In dynamics, polytopic extrasystole, periodically bigeminia, trigeminia were registered. On the ECG from 19/XII transient blockade of the left leg of the bundle of His.

Communal blood test, as well as data from biochemical studies without deviations from the norm.

Urinalysis: protein 0.33 g/l (0.33 ‰), otherwise without features. The diagnosis remained unclear. The diagnosis of rheumatism, concomitant rheumatic heart disease, congenital heart disease, late fibroelastosis was discussed.

In the clinic, against the background of bed rest, treatment was carried out with cardiac glycosides, diuretics, potassium preparations, cocarboxylase, acetylsalicylic acid. The patient's condition improved somewhat, edema disappeared, shortness of breath, acrocyanosis decreased, although transient rhythm disturbances remained. On December 22, at 13:30, the patient died suddenly.

Pathological anatomical 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 plethora of internal organs, nutmeg liver fibrosis.

Thus, the patient in adulthood developed heart damage 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 quickly deny both acquired and congenital malformations. Hypertension appears to have been incidental, and proteinuria appears to be associated with congestion. In the patient, endocardial fibroelastosis with concomitant myocardial damage seemed very likely, which was confirmed at autopsy. Attention was drawn to the late manifestation of the disease, accompanied by a fairly pronounced systolic murmur, which, obviously, had a muscular, but not valvular, origin.

Definition

Endocardial fibroelastosis has recently been referred to as a cardiomyopathy. The disease can be congenital primary or secondary associated with obstruction of the upper left ventricle.

The reasons

The primary form of endocardial fibroelastosis occurs due to various etiological factors affecting the fetus, so some authors refer it to the so-called fetal endocarditis. Vulnerable is the first trimester of pregnancy. If the fetal endocardium experiences lesions at a later date, a relatively benign form of the disease occurs, which can exist relatively longer without a fatal outcome.

Morphological changes in the endocardium determine the rigidity of the cavity of the left ventricle and the rigidity of its volume during the entire period of the child's postnatal life. The inner shell of the heart thickens, its elastic fibers are replaced by collagen tissue.

Along with this, there are contractile changes in the myocardium itself, leading to its hypertrophy. Coronary blood supply is disturbed, signs of subendocardial ischemia develop. Both the contractile function of the heart and the diastolic filling of the cavity of the left ventricle suffer. A condition arises in which, as a result of the replacement of the inner elastic layer with connective tissue, the left ventricle is not able to accommodate the volume of blood required for age, and the myocardium, through the mechanical resistance of the altered endocardium, is not able to force it into the aorta. The consequence of these processes is the invariability throughout the life of the child of the shock release into the systemic circulation. Minute volume is compensatory supported by heart rate. The exhaustion of the chronotropic regulation of hemodynamics leads to the occurrence of severe cardiac arrhythmias 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 young children. Primary signs of the disease can be manifested by lethargy, inability to suck, shortness of breath, perioral and diffuse cyanosis, symptoms of left ventricular failure. Hemodynamic disorders progress rapidly, acquiring signs of refractoriness to cardiotropic therapy.

The disease has a severe course, an unfavorable prognosis and ends in death during the first months. Clinically, changes in the heart are very diverse. Percussion borders of cardiac dullness may be unchanged or slightly shifted to the left. Auscultatory 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 feature is the absence of murmurs above and outside the precordial region, which to a certain extent may complicate the diagnosis of primary heart damage.

Diagnostics

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

X-ray examination should be carried out 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 heart. On face shots, in typical cases, the heart has the shape of a ball. X-ray examination in the left lateral projection with barium contrasting of the esophagus makes it possible to determine the degree of hypertrophy of the left heart and a decrease in 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. The contractile and relaxation parameters of the heart are reduced.

Prevention

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

The basic treatment of fibroelastosis includes lifelong corticosteroids, the use of diuretics, calcium antagonists and aldosterone. Surgical intervention aimed at endocardectomy has not found wide application today.

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Specialization: Cardiologist

Elena: 03/21/2017
Hello. Please explain the electrocardiogram of my son, who is now 17 years old. There is no pediatric cardiologist in our town, and an adult cardiologist will see him only at the age of 18. The son submits documents to the military school. How serious are these deviations from the norm? A year ago, my son did an ECG several times: both at the military registration and enlistment office and at the children's hospital. None of the doctors spoke about deviations, therefore, no treatment was prescribed, although I asked them about it, because I knew that my son dreams of military service. Even in the military registration and enlistment office, he was assigned the A1 health group based on this ECG and ultrasound of the heart, which did not reveal any abnormalities. Tell me, please, are the deviations serious, how is it treated and can it be cured, can he play sports without restrictions (he is an athlete-athlete) and will this become an obstacle to entering a military school? The son is very worried that his dream is not feasible. Thank you. ECG readings from a year ago: RR 0.76 PQ 0.12 QRS 0.10 QT 0.36 HR 78 Sinus rhythm. EOS is not rejected. Incomplete blockade of the right leg of the bundle of His. Increased electrical activity of the myocardium of the left ventricle. Violation of the processes of myocardial repolarization in the lower wall of the left ventricle. 2017 ECG readings: RR 0.78 PQ 0.12 QRS 0.12 QT 0.38 HR 77 Sinus rhythm. EOS is closer to vertical. Incomplete blockade of the right leg of the bundle of His. Increased electrical activity of the myocardium of the left ventricle. Moderate changes in the lower wall of the left ventricle.

Subendocardial fibroelastosis is a rare heart disease in children of the first years of life, accompanied by damage to the endo- and myocardium. In acute cases, urgent therapeutic measures are necessary. The causes of the disease are not yet known, but presumably they are congenital defects in the development of the endocardium, oxygen starvation of the fetus, intrauterine diseases of the fetus, including the myocardium, hereditary and family factors, intrauterine inflammatory lesions of the endocardium with viral infections (rubella, chicken pox).

Morphologically, the disease is characterized by progressive thickening of the endocardium due to the growth of collagen fibers. Connective tissue elements gradually compress the myocardium adjacent to the endocardium, causing dystrophic changes in it. With the growth of connective tissue, the wall of the ventricles, especially the left one, sharply thickens, while the myocardium becomes thinner, its contractile and other functions progressively worsen.

Symptoms. There are fulminant, acute and chronic forms. With a fulminant form, which is observed in the first weeks of a child's life, death occurs after a few hours or days from the initial signs with increasing symptoms of cardiovascular insufficiency. In the acute form, the first symptoms of the disease occur somewhat later, more often in the second half of the first year of life. The chronic form of the disease occurs in older children and lasts for months and years.

The disease begins with an increase in the pallor of the skin (sometimes pronounced), with a slight cyanosis of the face and cold sweat. In the future, shortness of breath occurs, especially when sucking the breast, and breathing is sometimes noisy, with coughing fits. At the same time, weakness, lethargy and refusal to eat are noted. Cardiovascular insufficiency increases more and more, edema appears, cyanosis of the skin intensifies, the liver enlarges, and due to the expansion of the boundaries of the heart, especially to the left, there is a bulging of the anterior wall of the left side of the chest. Heart sounds are muffled. At the beginning of the disease, there is no heart murmur. However, in advanced cases, with a sharp increase in the left ~ ventricle, systolic and even diastolic murmurs and heart rhythm disturbance (such as extrasystole) are heard. The same changes are observed when fibroelastosis is combined with congenital heart defects.

An X-ray examination of the chest reveals a spherical shape of the heart, its increase is more to the left. When combined with congenital heart disease, its configuration changes depending on the type of defect.

An electrocardiographic study establishes signs of left ventricular hypertrophy.

Treatment mainly symptomatic and aimed at combating circulatory failure. For this purpose, digitalis preparations are used, and in young children it is preferable to use digitoxin in doses of saturation, then maintenance doses. The duration of treatment is at least 5-6 months. At the same time, vitamin B15 is prescribed (25-50 mg per day for 20-30 days), cocarboxylase (10-50 mg per day subcutaneously, intramuscularly, intravenously 1-2 times a day), ATP (0.1-0 3 ml of 1% solution once a day), 8-10 injections of one of these drugs.

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Fibroelastosis is a rare heart disease. It is characterized by excessive growth of elastic fibers of the connective tissue, a violation of their growth. The disease is typical for young children or newborn babies.

Modern research methods make it possible to detect pathologies of organs and the heart during fetal development. Sometimes the birth of a child with a similar pathology ends in a short life, children may die immediately after birth, suddenly, or after a few months. Depends on the degree of pathology and timely medical measures taken. This pathology of the heart is called endocardial fibroelastosis.

The pathology of the growth of fibrous tissue is expressed in the anatomically incorrect state of the heart muscle, which leads to an abnormal expansion of the heart, especially the left ventricle, designed to pump blood to all organs and tissues of the body, except for the lungs (blood enters the lungs through the right ventricle). This state of expansion of the heart muscle is otherwise called hypertrophy. It leads to congestive heart failure.

Causes and predisposing factors

The exact cause of the development of fibroelastosis is unknown, there are several hypotheses that explain the occurrence of the disease.

The main reasons are:

1. The result of "random" mutations in chromosomes.
2. The result of the negative impact of viral infections (mumps, Coxsackie B virus).
3. The use of pregnant drugs, alcohol.
4. subendocardial ischemia.
5. Changes in the outflow of lymph from the heart.
6. Systemic carnitine deficiency.
7. Blood relationship between parents.

Additional predisposing factors, according to scientists, may be the results of metabolic changes (for example, Barth's syndrome). Sometimes myocarditis is noted as a precursor of fibroelastosis.

Symptoms, signs and classification of endocardial fibroelastosis

Intrauterine lesions of the heart muscle in the later stages appear already in the first weeks after the appearance of the newborn. In this case, the following are involved in the pathological process:

• conducting system of the heart;
• membranes of the heart muscle;
• coronary vessels and aorta.

Also, signs such as cardiosclerosis and hypertrophy (proliferation of connective tissue), symptoms of thickening of the mucous membrane of the chambers are determined. Hypoplasia, that is, a decrease in the size of the left ventricle, is much less common.

Common symptoms of fibroelastosis include:

• frequent shortness of breath;
• shortness of breath with a dry cough;
• general weakness of the body;
• fast fatiguability;
• pale integuments of the face;
• the addition of neurological disorders, such as irritability, moodiness and nervousness for no reason;
• poor sleep, restless sleep.

Quite dangerous symptoms for a baby and a teenage child are the occurrence of arrhythmias and palpitations.

Common symptoms of the disease in babies include the following signs:

• poor appetite, frequent refusal of food;
• regular abdominal pain;
• heavy sweating, especially at night;
• low hemoglobin in the blood;
• pallor of the skin;
• puffiness;
• systolic murmur (when combined with a congenital defect);
• the occurrence of a rash.

Such and other conditions can occur both in the first days after birth, and in the first hours immediately after birth.

Constant refusal of food leads to low hemoglobin, and as a result, the development of anemia. There is a delay in the development of growth, weight gain, mental and mental development. These children are far behind their peers. Poor sleep and pain negatively affect the nervous system, causing the child to become capricious, lethargic, sleepy. Pain in the abdomen may indicate the development of coronary insufficiency, the progression of complications.

An electrocardiogram usually shows:

• atrioventricular block;
• blockade of branches of the bundle of His;
• changes in heart rate;
• muffled tones;
• systolic murmur;
• congestion in the blood circulation (in the small circle);
• overload in the work and condition of the atria, the left ventricle.

In some children, blue streaks of capillary vessels stand out well through pale skin, the liver is enlarged, the veins in the neck are very tense.

The condition is divided into acute (clinical manifestations can be observed up to 2 months), subacute (up to six months) and chronic (more than six months).

Severe acute and subacute course of the disease often lead to an early death.

There are also primary and secondary forms of the disease.

1. The primary form is a clinical syndrome in infancy characterized by enlargement and enlargement of the heart muscle with congestive insufficiency and signs of systolic dysfunction.
2. The secondary form is a complex of congenital abnormalities, heart defects, which create an overload for the left ventricle, cause its increased stress. An example of such a condition would be aortic valve atresia.

X-ray images show an increase in the heart, which occurs mainly due to an increase in the cavity of the left ventricle. Sometimes such a heart looks like a big ball, while the walls of the heart can be enlarged in different directions. The pulsation of the left ventricle is marked sluggish and insufficient.

There is also atelectasis of the lower lobe of the lung.

Diagnostics

It includes auscultation and instrumental studies:

• electrocardiogram (ECG);
• phonogram (FCG);
• echocardiogram (Echo-KG);
• complete blood count (leukocytosis is observed);
• chest x-ray.

Echocardiography shows thickening of the free wall and interventricular septum. Arrhythmia and conduction changes during the electrocardiogram in this disease are observed in every third or fourth patient. In 50% of patients, atrial hypertrophy, axis deviation to the right is observed.

If necessary, additional studies are assigned, for example, computed tomography, MRI, and others.

Forecast

Quite disappointing, since most of the children born with this diagnosis die. Mortality due to chronic endocardial fibroelastosis is more than 40%. Basically, death occurs on the basis of refractory heart failure.

Treatment

Prescribed almost the same as in heart failure. The main direction in treatment is the elimination of congestive insufficiency and preventive measures of concomitant bacterial and / or viral infections.

Assigned: cardiac glycosides, ACE inhibitors, hormonal and antibacterial drugs, anti-enzyme treatment, drugs to increase and strengthen the body's immune system, complexes of vitamins and minerals. Diet and outdoor exercise are also important.

Galina Vladimirovna

SYNONYMS

The disease is known by various names, including "fetal endocarditis", "endocardial fibrosis", "subendocardial fibroelastosis", "prenatal fibroelastosis", "elastic tissue hyperplasia" and

"endocardial sclerosis".

DEFINITION

Endomyocardial fibroelastosis is a congenital disease of unknown etiology that begins in the prenatal period in fetuses, but in the vast majority of cases manifests itself in newborns or infants and is characterized by significant thickening and fibrosis of the endocardium of one or more chambers of the heart, cardiomegaly, and early congestive heart failure.

CLASSIFICATION

There are primary and secondary endomyocardial fibroelastosis. In the primary, there are no predisposing factors in the form of valvular defects or other congenital structural anomalies of the heart.

ETIOLOGY

The etiology of primary fibroelastosis has not been established. Possible predisposing factors include an inflammatory or infectious process transferred in utero, more often in the early fetal period, developmental disorders, as well as hypoxia and ischemia of the endocardium. Changes in the endocardium may be due to initial myocardial damage, in which, due to the expansion of the heart and stretching of the endocardium, the proliferation of fibroelastic fibers begins. Cases have been reported in siblings. Genetic forms are described.

In secondary fibroelastosis, severe congenital malformations are diagnosed more often in the left sections of the heart of an obstructive type (stenosis or atresia of the aorta, critical coarctation or interruption of the aortic arch, other forms of hypoplasia of the left sections of the heart).

PATHOGENESIS

Given that fibroelastosis is predominantly observed in fetuses, newborns and infants, N.A. Belokon (1987) considered it as an outcome of carditis suffered in the early fetal period, referring to "early congenital carditis" in her working classification. This approach is not considered universally recognized, it is not accepted in foreign practice and, according to modern ideas, is ambiguous.

Many authors believe that fibroelastosis is not an independent disease, but a nonspecific reaction of the endocardium to any myocardial stress, which can be hemodynamic overload due to CHD and vessels and their combinations, viral or bacterial agents, genetic aberrations, hypoxia, ischemia, cardiomyopathy, carnitine deficiency, etc. The reasons why myocardial stress in the early fetal period causes proliferation of endocardial smooth muscle cells with their transformation into fibroblasts with subsequent synthesis of collagen and elastin are not well understood, but may be associated with local humoral effects, pathological intercellular interaction, which established in a number of experimental studies.

Morphologically, fibroelastosis is a diffuse thickening of the endocardium of one or more cardiac chambers, formed by collagen or elastic tissue with a characteristic milky white color. The changes are especially pronounced in the left parts of the heart, and so much so that the inner surface of the chamber loses its trabecular structure. Sometimes the process goes to the valves, especially often to the aortic and mitral. Microscopically, the thickening of the fibroelastic fibers of the endocardium accompanying the trabecular sinusoids is determined, which can lead to degenerative changes in the subendocardium or muscle necrosis with vacuolization of the muscle fibers. Involvement of valves in the process is characterized by their myxomatous proliferation with an increase in the number of collagen elements.

CLINICAL PICTURE

The clinical manifestations of the disease vary, but cardiomegaly and early decompensation of blood circulation appearing already in newborns and infants (up to 6 months) are typical. Signs of severe congestive insufficiency may develop in a previously clinically healthy child. Manifestation and progression of the disease are possible against the background of recurrent respiratory, bronchopulmonary infections.

Symptoms of the disease can be constantly progressive. Less often, periodic deterioration of the condition with periods of remission is observed.

DIAGNOSTICS

Physical examination

They note anxiety, shortness of breath, food refusal, late cyanosis, enlargement of the liver and spleen, body weight due to fluid retention. In cases of the periodic nature of the development of congestive heart failure, the condition of a newborn or infant may suddenly become critical. Shortness of breath, cough, hepatomegaly, anorexia sharply increase or suddenly appear. With an increase in pressure in the system of peripheral veins, edema of the extremities, the area of ​​​​the sacrum or face is possible. Different-sized moist rales heard in the lungs are caused by both stagnation in the vessels of the pulmonary circulation and recurrent pulmonary infections, which requires monitoring and prescribing glycosides and diuretics against the background of antibiotic therapy.

During auscultation of the heart, the appearance of a gallop rhythm, sometimes a soft systolic murmur of mitral insufficiency at point I, is noted.

Instrumental Research

♦ ECG reveals signs of left ventricular hypertrophy and overload in the left chest leads (Y4-Y6), 8T segment depression, isoelectricity, or T wave inversion.

Arrhythmia is often noted, especially supraventricular tachycardia, dysfunction of the conduction system (various forms of partial or complete AV blockade, blockade of the right leg and the anterior branch of the left leg of the His bundle).

♦ X-ray reveals cardiomegaly, sometimes signs of intercurrent pulmonary infections or pulmonary edema.

♦ Echo-CG with primary fibroelastosis left ventricular cavity of the heart is expanded with a decrease in its systolic and diastolic functions, and with secondary fibroelastosis, a decrease in the left ventricular cavity is possible.

Differential Diagnosis

Differential diagnosis of fibroelastosis is necessary with other so-called idiopathic cardiac hypertrophies in newborns, manifested by congenital cardiomegaly. Relatively rare diseases should be ruled out.

♦ Glycogenous cardiomegaly (Pompe disease or type II glycogenosis) - a disease with a poor prognosis (no more than a few months) and muscle hypotension.

♦ Congenital myopathies.

♦ Aphonic or poorly manifested CHD on auscultation, such as infaltile type of abnormal origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome), with insufficiently developed anastomoses between the left and right coronary arteries, full form of the common open atrioventricular canal, large defect interatrial septum (ASD), common atrium, partial or total anomalous drainage of the pulmonary veins, isolated pronounced coarctation of the aorta.

Medical treatment

Treatment is aimed at stopping congestive insufficiency (glycosides, diuretics, ACE inhibitors, hormones, parenteral and oral forms of metabolic drugs) and preventing concomitant infections (antibacterial drugs, immunotherapy, antienzymatic therapy).

The prognosis is unfavorable. In most cases, children die. With secondary fibroelastosis, combined with valvular defects or CHD, without surgical treatment, children die within the first months of life. The risk of cardiac surgery itself in the presence of endomyocardial fibroelastosis is significantly higher, and the postoperative course is much more difficult.

The introduction in recent years of more effective means of combating heart failure in newborns and infants, including more effective diuretics and ACE inhibitors, has somewhat improved the immediate prognosis. In surviving children, the clinical diagnosis remains unconfirmed, since without pathological anatomical examination it is impossible to be sure that the initial heart damage is due to endocardial fibroelastosis, and not to another myocardial disease.

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