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

Endocardial fibroelastosis is more common in children early age, 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 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 pulmonary and systemic 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. At large sizes hearts are most often suspected chronic myocarditis, which is usually evidenced by a more protracted course and the 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 the cardiac index 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 V epigastric region with irradiation in left half chest And left hypochondrium. In May, after suffering from 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. Diagnoses of subaortic stenosis or congenital heart disease were discussed in the clinic. 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.

The general condition of the patient is moderate. 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. Heart area and large vessels 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. In a stomach free liquid No. 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 the 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. ECG from 19/12 shows transient blockade of the 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 in mature age heart damage developed 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 related to stagnation. The patient had a very probable endocardial fibroelastosis with collateral damage myocardium, 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.

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 lesions at a later date, 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. The inner lining of 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. Suffering like contractile function heart and diastolic filling of the left ventricular cavity. A condition arises in which, as a result of the replacement of the internal elastic layer with 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 throughout the child’s life of shock emission in big circle blood circulation The minute volume is compensatory supported by the heart rate. The exhaustion of chronotropic regulation of hemodynamics leads to the emergence severe violations 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 young children. 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 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 or paroxysmal tachycardia the sonority of the 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 can complicate diagnosis primary lesion hearts.

Diagnostics

Diagnostic assistance in these cases is provided by ECG registration. The most characteristic changes are considered to be nomotopic disturbances of the heart rhythm, most often in the form of its rigid acceleration, increased electrical activity of the shunt 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 physiological characteristics this period of the child’s life, as well as 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 mobility interventricular septum 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. The son submits documents to 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 deviations; accordingly, no treatment was prescribed, although I asked them about it, because I knew that my son was dreaming 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.

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. The causes of the disease are not yet known, but presumably they are birth defects endocardial development, oxygen starvation fruit, intrauterine diseases fetus, including myocardium, hereditary-familial factors, intrauterine inflammatory lesions endocardium with viral infections (rubella, chickenpox).

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

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

The disease begins with increasing pallor skin(sometimes pronounced), with slight cyanosis of the face and cold sweat. Subsequently, shortness of breath occurs, especially when sucking the breast, and the breathing is sometimes noisy, with coughing attacks. At the same time, weakness, lethargy and refusal to eat are noted. Cardiovascular failure it increases more and more, swelling appears, cyanosis of the skin intensifies, the liver enlarges, and due to the expansion of the borders 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 onset of the disease, there is no heart murmur. However, in advanced cases, with a sharp increase in the left ventricle, systolic and even diastolic murmur and heart rhythm disturbances (such as extrasystole). The same changes are observed in cases where fibroelastosis is combined with congenital heart defects.

At x-ray examination the chest reveals a spherical shape of the heart, its enlargement is greater to the left. When combined with a congenital heart defect, its configuration changes depending on the type of defect.

An electrocardiographic study reveals 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 saturation doses, 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 1 time per 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 connective tissue and disruption of their growth. The disease is typical for small children or newborn babies.

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

Pathology of proliferation fibrous tissue is expressed in an anatomically abnormal state of the heart muscle, which leads to abnormal expansion of the heart, especially the left ventricle, which is designed to pump blood to all organs and tissues of the body except 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 to explain the occurrence of the disease.

The main reasons are considered:

1. The result of “random” mutations in chromosomes.
2. Result negative impact viral infections(mumps, Coxsackie B virus).
3. Consumption by pregnant women narcotic drugs, alcohol.
4. Subendocardial ischemia.
5. Changes in the outflow of lymph from the heart.
6. Systemic carnitine deficiency.
7. Consanguinity between parents.

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

Symptoms, signs and classification of endocardial fibroelastosis

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

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

Signs such as cardiosclerosis and hypertrophy (overgrowth of connective tissue), symptoms of thickening of the mucous membrane of the chambers are also determined. Hypoplasia, that is, a decrease in the size of the left ventricle, is observed much less frequently.

TO general symptoms fibroelastosis include:

• frequent shortness of breath;
• difficult shallow breathing with the appearance of a dry cough;
• general weakness of the body;
• fast fatiguability;
• pale facial coverings;
• accession neurological disorders, for example, irritability, moodiness and nervousness for no reason;
• difficulty falling asleep, restless sleep.

Enough dangerous symptoms for baby and child adolescence is the occurrence of arrhythmia and rapid heartbeat.

Common symptoms of the disease in children include the following:

• poor appetite, frequent refusal to eat;
• regular abdominal pain;
• heavy sweating, especially at night;
• decreased hemoglobin in the blood;
• pale skin;
• swelling;
• systolic murmur (when combined with a congenital defect);
• the appearance of a rash.

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

Constant refusal to eat leads to low hemoglobin, and as a result, the development of anemia. There is a delay in growth development, weight gain, mental and mental development. Such children lag significantly behind their peers. Bad dream and pain negatively affects nervous system, why child becomes moody, lethargic, sleepy. Pain in the abdomen may indicate the development coronary insufficiency, progression of complications.

The electrocardiogram usually shows:

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

In some children, blue veins of capillary vessels are clearly visible through pale skin, the liver is enlarged, and the veins in the neck are very tense.

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

Heavy spicy and subacute course diseases often lead to rapid death.

There are also primary and secondary forms of the disease.

1. Primary form – clinical syndrome in infancy, characterized by expansion and enlargement of the heart muscle with congestive failure, signs of systolic dysfunction.
2. The secondary form is a complex of congenital abnormalities and heart defects that create an overload for the left ventricle and cause it increased voltage. An example of such a condition would be aortic valve atresia.

On X-ray images There is an enlargement of the heart, which occurs mainly due to an increase in the cavity of the left ventricle. Sometimes such a heart looks like a large ball; the walls of the heart can be enlarged by different sides. The pulsation of the left ventricle is sluggish and insufficient.

Atelectasis of the lower lobe of the lung is also observed.

Diagnostics

It includes auscultation and instrumental studies:

• electrocardiogram (ECG);
• phonogram (FKG);
• echocardiogram (Echo-CG);
• general blood test (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 and axis deviation to the right are observed.

If necessary, appointed additional research, For example CT scan, MRI and others.

Forecast

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

Treatment

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

Prescribed: cardiac glycosides, ACE inhibitors, hormonal and antibacterial drugs, anti-enzyme treatment, drugs to increase and strengthen immune system body, complexes of vitamins and minerals. Also important has a diet and walks in the fresh air.

Galina Vladimirovna

SYNONYMS

The disease is known as different names, including “fetal endocarditis”, “endocardial fibrosis”, “subendocardial fibroelastosis”, “prenatal fibroelastosis”, “elastic tissue hyperplasia” and

"endocardial sclerosis".

DEFINITION

Endomyocardial fibroelastosis - congenital disease unknown etiology, starting in prenatal period in fetuses, but in the vast majority of cases manifesting in newborns or infants and 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 primary cases, there are no predisposing factors in the form of valve defects or other congenital structural abnormalities of the heart.

ETIOLOGY

The etiology of primary fibroelastosis has not been established. Possible predisposing factors include inflammatory or infectious process, developmental disorders, as well as endocardial hypoxia and ischemia. Changes in the endocardium may be caused by initial damage to the myocardium, in which, due to expansion of the heart and stretching of the endocardium, proliferation of fibroelastic fibers begins. Cases have been reported in siblings. Genetic forms have been described.

With secondary fibroelastosis, severe congenital defects are diagnosed, most often of the left heart of the obstructive type (stenosis or atresia of the aorta, critical coarctation or interruption of the aortic arch, other forms of hypoplasia of the left heart).

PATHOGENESIS

Considering that fibroelastosis is mainly observed in fetuses, newborns and infants, N.A. Belokon (1987) considered it as an outcome of carditis suffered in the early fetal period, classifying it in her working classification as “early congenital carditis" This approach is not considered generally accepted; it is not accepted in foreign practice and by modern ideas ambiguous.

Many authors believe that fibroelastosis is not independent disease, but a nonspecific reaction of the endocardium to any myocardial stress, which can be hemodynamic overload due to congenital heart disease and blood vessels and their combinations, viral or bacterial agents, genetic aberrations, hypoxia, ischemia, cardiomyopathy, carnitine deficiency, etc. 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, have not been sufficiently studied, but may be associated with local humoral effects, pathological intercellular interaction, which has been 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, so much so that inner surface the chamber loses its trabecular structure. Sometimes the process spreads to the valves, especially often to the aortic and mitral valves. Microscopically, thickening of the endocardial fibroelastic fibers accompanying the trabecular sinusoids is determined, which can lead to degenerative changes in the subendocardium or muscle necrosis with vacuolization of 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

Clinical manifestations of the disease vary, but cardiomegaly and early onset in newborns and children are typical. infancy(up to 6 months) circulatory decompensation. Signs of severe congestive failure may develop in previously clinically healthy child. Manifestation and progression of the disease are possible against the background of recurrent respiratory and bronchopulmonary infections.

Symptoms of the disease can be constantly progressive. Periodic deterioration of the condition with periods of remission is less common.

DIAGNOSTICS

Physical examination

Anxiety, shortness of breath, refusal to eat, late cyanosis, enlarged liver and spleen, and body weight due to fluid retention are noted. In cases of periodic development of congestive heart failure, the condition of the newborn or infant may suddenly become critical. Shortness of breath, cough, hepatomegaly, anorexia sharply increase or appear suddenly. With increased pressure in the peripheral venous system, swelling of the limbs, sacrum or face is possible. Various-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 the prescription of glycosides and diuretics against the background of antibacterial therapy.

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

Instrumental studies

♦ ECG reveals signs of hypertrophy and overload of the left ventricle in the left precordial leads (U4-U6), depression of the 8T segment, isoelectricity or inversion of the T wave.

Arrhythmia, especially supraventricular tachycardia, dysfunction of the conduction system ( various shapes partial or complete AV blockade, blockade of the right bundle branch and anterior branch of the left bundle branch).

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

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

Differential diagnosis

Differential diagnosis of fibroelastosis is necessary with other, so-called idiopathic cardiac hypertrophies in newborns, manifested by congenital cardiomegaly. Should be excluded regarding rare diseases.

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

♦ Congenital myopathies.

♦ Aphonic or weakly manifested by auscultation of congenital heart disease, such as the infantile type of abnormal origin of the left coronary artery from pulmonary artery(Bland-White-Garland syndrome), with insufficiently developed anastomoses between the left and right coronary arteries, full form 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.

Drug treatment

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

The prognosis is unfavorable. In most cases, children die. With secondary fibroelastosis, combined with valve defects or congenital heart disease, 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 significantly more severe.

Introduction to last years into practice more effective means combating heart failure in newborns and infants, including more effective diuretics and ACE inhibitors, slightly improved the short-term prognosis. In surviving children clinical diagnosis remains unconfirmed, since without a pathological examination it is impossible to verify that the initial heart damage is caused by endocardial fibroelastosis and not by another myocardial disease.

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