FIBROELASTOSIS SUBENDOCARDIAL

From Big Medical Encyclopedia

FIBROELASTOSIS of

SUBENDOKAR-DIALNYY (Latin fibra fiber + Greek elast f ikos] elastic + - osis; lat. sub - the prefix meaning stay under..., - f-Greek endo-the prefix meaning from within, inside, + kardia heart; synonym: a fibroelastosis of an endocardium, an endocarditis inborn, an endocarditis a fetalis, endocardial sclerosis, an endomyocardial fibroelastosis) — an inborn disease, ha rasterizuyushcheesya growth in an endocardium and subendocardial departments of a myocardium of fibrous and elastic fabric.

Lanchizi is for the first time described at the beginning of 18 century (G. M of Lancisi). Kreyzig (F. L. Kreysig, 1816) called a disease of «a fetalis endocarditis». In domestic literature the first publication about F. the page (1957) belongs to A. M. Vikhert (see t. 10, additional materials). Distinguish isolated F. page and combined with inborn heart diseases (see Heart diseases inborn) — combined F. page. Depending on the sizes of a cavity of a left ventricle Edwards (J. E. Edwards, 1953) allocated dilatirovanny and kontraktil-ny types F. page. The last in a crust, time is carried to inborn heart disease — a syndrome of a hypoplasia of a left ventricle.

The etiology and a pathogeny are up to the end not clear. The reason isolated F. villages most of researchers consider viruses. The disease develops for the IV—VII month of pre-natal development and belongs to an early fetopathy (see. Antenatal pathology). During this period after altera-tivny changes there is a proliferation of elastic and collagenic fibers. On T.E. Ivanovskaya's observations, A. V. Tsinzer-linga (1976), at damage of heart in the pre-natal period after the VII month is marked out usual inflammatory reaction, and the fibroelastosis does not develop. Existence of the centers of a sclerosis and signs of an inflammation in other bodies at patients with F. the page testifies to a generalized pre-natal infection, the cut is one of manifestations damage of heart.

At combined F. pages an etiological factor along with a virus can be hemodynamic disturbances and a hypoxia (see). In cases of a combination F. page with coarctation of an aorta (see) and a stenosis of an aorta (see the Aorta) hemodynamic factors (an overload pressure of a myocardium of a left ventricle), the deficit of a coronary blood-groove connected with a hypertrophy of a myocardium matter. At a combination F. the page with a syndrome of a hypoplasia of a left ventricle also matters decrease in a coronary blood-groove in a myocardium of a left ventricle in connection with retrograde intake of blood in coronary arteries from an aorta. At abnormal otkhozhde-niya of the left coronary artery from a pulmonary trunk (see Bland — Whyte — Garland a syndrome) the hypoxia is defined by deficit of a mezhkoronar-ny anastomosis, existence of a steal-syndrome (a syndrome of the burglarizing) arising owing to dropping a part of blood from the right coronary artery through left in a pulmonary trunk, passing an intercoronary anastomosis. Thus, T. the page is result of nonspecific reaction of an organism in response to influence during the early fetalis period of infectious and noninfectious factors, the cooperative effect to-rykh comes down to a hypoxia of an endocardium and departments of a myocardium, adjacent to it. There are also other theories of emergence F. page. So, Black - Schaffer (V. of Black-Schaffer, 1957) considers that the diffusion thickening of an endocardium can develop in case of primary damage of a myocardium, Johnson (F. Johnson, 1952) regards F. page at inborn heart diseases as result of insufficient oxygenation of an endocardium and a myocardium, Noren (G. Noren, 1970) with sotr. point to a role of myocarditis in emergence F. page. In essence in all specified theories a pathogenetic basis of education F. the page also is a hypoxia and the related damage of an endocardium and a myocardium. Cases F. the pages revealed by Uestvu-dom (M. of Westwood, 1975) and sotr. at sibs (see the Proband), point to a possibility of genetic predisposition to a disease.

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Pathological anatomy. Pathoanatomical changes at F. pages are rather characteristic. At isolated F. the page is observed expansion of a cavity of a left ventricle. The valve device is, as a rule, not changed, however deformation of mitral is possible, is more rare aortal than valves. Heart at F is macroscopic. the page is increased in sizes, the weight (weight) it increases by 2 — 4 times. Heart is cut with a crunch, on a cut the diffusion thickening (to 2 — 4 mm) a pristenochny endocardium — a typical prize sign F is found. page. Usually the endocardium of a left ventricle is involved in process, changes of an endocardium of auricles and a right ventricle at the same time can be noted. Surface of a reinforced endocardium smooth, whitish or yellowish-gray color, «nacreous». Fleshy trabeculas and papillary muscles «are immured» in a reinforced endocardium, mouths of the smallest veins of heart (tebeziyevy) are not differentiated. In case of change of the valve device find diffusion or focal, in the form of small knots or rollers in the area of a smykaniye, a thickening of shutters mitral and gates aortal valves. Such changes are characteristic both of a stenosis of openings, and of insufficiency of valves. Tendinous chords are thickened, sometimes shortened. In cardial cavities (especially left ventricle) pristenochny blood clots often are found, to-rye can become a source of a thromboembolism (see) vessels of a big circle of blood circulation. A myocardium at F. page

flabby, reinforced, hypertrophied, it is similar to boiled meat, on a section dim, with whitish tyazha, papillary muscles and mya-sr1sty trabeculas are flattened.

Microscopic picture F. the page is characterized by peculiar changes both an endocardium, and a myocardium. The endocardium is thickened by 10 — 15 times and presented generally in parallel by the going bunches of the elastic and collagenic fibers intertwining among themselves that clearly is visible at use of colourings Fuchs Ling (see Veygert methods of coloring) and pikrofuksiny (see Van-Gizona a method). Superficial sites of a reinforced endocardium are covered with one layer of endotheliocytes, under to-rymi the network of gentle argyrophil and elastic fibers is located; in bazofilno the painted main substance a small amount of cellular elements, preferential fibrocytes meets. Under a surface layer a large amount of the coarse thick elastic fibers forming the going plates multiple in parallel with wavy contours is located. More deeply thicker and mature elastic fibers are located in a large number. On the course of elastic fibers collagenic fibers come to light various thickness. Blood vessels of capillary type are covered by a usual endothelium, are not numerous and are located in a reinforced endocardium under different corners or in the form of chains. Sometimes it is possible to see the fibers of the carrying-out system of heart as if immured in the expanded connecting fabric of an endocardium. Less often separate atrofichny cardiomyocytes meet, in a sarcoplasm to-rykh drops of lipids and a glybka of lime can be found. Small poloskovidny ochazhka of dystrophic calcification meet in a reinforced endocardium and on the course of capillaries. Inflammatory infiltrates in an endocardium at F. pages are not found, only sometimes in its thickness small accumulations of lymphoid and macrophagic cellular elements can meet. The border between the changed endocardium and the subject layers of a myocardium can be accurate, more often, however, growing from an endocardium into a myocardium connective tissue tyazhy is observed, to-rye in the form of wedges get between cardiomyocytes. The connecting fabric rich with elastic and collagenic fibers which expanded in an endocardium, usually spreads to the smallest veins of heart, walls to-rykh are considerably thickened, and the gleam in the field of mouths is obliterated. Directly under a reinforced endocardium a large number of the vessels expanded, filled with blood with thin walls and layers of connecting fabric between them is located. Formation of these vessels can be connected with closing of mouths of the smallest veins of heart. The reduction of quantity of coronary arteries (on a weight unit of heart) which is especially expressed in subendokardi-alny area is noted. In small branches of coronary arteries of heart the unsharp thickening of walls due to growth of elastic and collagenic fibers in an intima, a moderate hypertrophy of the interlayer and a perivascular sclerosis with the phenomena of an elastosis is found.

Changes of a myocardium at F. pages can be expressed in various degree. Cardiomyocytes of a suben-dokardialny layer are, as a rule, hypertrophied and have symptoms of vacuolar and fatty dystrophy (see Dystrophy of cells and fabrics). In sites of growth of elastic and collagenic fibers they are atrofichna. Fleshy trabeculas are presented by the small roundish islands of atrofichny cardiomyocytes located among powerful concentric imposings of elastic and collagenic fibers. In a subendocardial layer of a myocardium the nek-rotizirovanny cardiomyocytes forming small, and sometimes the considerable centers which are exposed to the organization — a focal cardiosclerosis quite often are found (see). In the thickness of a myocardium minor cicatricial changes also meet not always. The centers of a cardiosclerosis may contain a large amount of elastic fibers, in them it is possible to find sites of dystrophic calcification of cicatricial fabric. In intramural and subenikardialny layers of a myocardium the hypertrophy and focal fatty dystrophy cord noil, and that and, coarsening of a stroma, moderately expressed growth of connecting fabric between cardiomyocytes and around vessels is noted. Inflammatory infiltrates in a myocardium usually are absent. An epicardium without features, sometimes it is possible to find sites of a sclerosis, and also commissure between leaves of a pericardium.

In internals at F. the page is found a picture of venous stagnation, sometimes — the centers of a sclerosis and an inflammation.

At combined F. pages macro - and microscopic changes of an endocardium of a left ventricle correspond to a pathoanatomical picture isolated F. page. However in a myocardium, it is preferential in the pool of the left coronary artery, subendocardial and transmural myocardial infarctions are found (see) different prescription, a focal and diffusion cardiosclerosis with formation hron. aneurisms of heart (I eat. To

the Clinical picture. The disease can proceed immediately, sharply and chronically. Symptoms isolated F. pages appear, as a rule, in the first 6 — 12 months, is 2 more rare — the 3rd year of life and consist of cardial and noncardiac signs. At children pallor, slackness, perspiration, small cyanosis, fatigue is noted during feeding, a bad increase in weight. From cardiovascular system are observed arterial hypotension (see Hypotension arterial), the cordial hump early developing, carat - a diomega Leah, deaf cardiac sounds, a cantering rhythm (I eat. Gallop a rhythm), lack of noise or the systolic noise connected with relative insufficiency of the mitral valve. Symptoms of refractory left ventricular heart failure prevail (see) in the form of an asthma, tachycardia, congestive rattles in lungs. At most of children the liver acts on 2 — 4 cm from under edge of a costal arch. Hypostases happen seldom. At hron. current F. pages a cardiomegaly, deaf tones, refractory heart failure remain stable. In cases of rather high-quality current the sizes of heart decrease, however dullness of tones, changes on the ECG and the roentgenogram, testimonial of a cardiosclerosis remain.

At a combination F. the page with inborn heart diseases is noted discrepancy between a wedge, manifestations of defect and considerably oversizes of heart, deaf tones, endocardiac blockade.

To complications F. the page should carry a syndrome of Morganyi — Adams — Stokes (see M of an organya — Adams — Stokes a syndrome) against the background of a full cross heart block, a thromboembolic syndrome (see the Thromboembolism) with development of a hemiparesis.

The diagnosis is made on the basis of data of the anamnesis (viral infections or an aggravation hron. infectious diseases of mother, and also toxicoses of pregnant women in the first half of pregnancy), a wedge. displays of a disease and results of a tool research.

At rentgenol. a research the pulmonary drawing, a cardiomegaly, a spherical or ovoidny shape of heart (fig.) comes to light normal or a little strengthened but to a venous bed. The pulsation is lowered. The cardiothoracic index — the attitude of cross sectional dimension of heart towards cross sectional dimension of a thorax at the level of a diaphragm expressed as a percentage — makes


Fig. The roentgenogram body in grudne of a cell (a direct projection) of the patient with a sub-endocardial fibroelastosis: the pulmonary drawing is not changed; heart is increased, an ovoidny form.

at the majority of sick 70 — 75% (50% are normal at children). On an ECG (see Elektrokardiografiya) — the normal provision of an electrical axis of heart, a high voltage of teeth, a rigid frequent rhythm, signs of a hypertrophy of a myocardium of the left auricle and a ventricle, deep negative or smoothed teeth of T in assignments of V 4_6, omission of a segment S T is lower than the isoline. Disturbances of a rhythm and conductivity are not characteristic for F. the page, however is possible emergence of premature ventricular contraction (see), a Bouveret's disease (see), intra ventricular and atrioventricular blockade (see the Heart block). At a phonocardiographic research (see Fonokardiografiya) considerable decrease in amplitude of the I tone and systolic noise of a tape-like form in the field of a top of heart comes to light.

292 FIBROELASTOSIS SUBENDOCARDIAL


by means of an echocardiography (see) find dilatation of a cavity of a left ventricle. Diameter of a cavity in a systole and a diastole changes a little, thickness of walls is increased or approaches normal.

At catheterization of a cardial cavity (see Catheterization of heart) and angiocardiography (see) sharp dilatation and spherical shape of a left ventricle, signs of insufficiency of the mitral valve, disturbance of sokratitelny ability of a myocardium, increase in konechnodiastolichesky pressure in a left ventricle, pressure in pulmonary capillaries, a pulmonary artery come to light.

In cases of not clear diagnosis sometimes apply an endomyocardial biopsy of a left ventricle.

D and f f e r e ii of c and alny d and - to the agnostic carry out with an abnormal otkhozhdeniye of the left coronary artery from a pulmonary trunk, for to-rogo attacks of the sudden concern which is followed by shout, focal changes on an ECG in the field of a perednebokovy wall of a left ventricle (see the Myocardial infarction)', with a glycogenosis P types are characteristic sistolodiastolichesky noise in the second between-reberye at the left (see Glycogenoses), at Krom generalized muscular weakness, a macroglossia, a hepatomegalia, shortening of an interval of PR on an ECG is observed. At children of early age F. pages differentiate with the acquired myocarditis (see), about Krom positive dynamics of process against the background of treatment testifies; at adults — with secondary fibrous changes of a myocardium against the background of the postponed heart diseases (a myocardial infarction, an endocarditis, etc.).

Treatment. Specific treatment F. there is no page. The complex of therapeutic actions includes restriction of a physical activity and purpose of the corresponding diet (restriction of salt and liquid) in the period of the expressed heart failure. At accession of a virus and bacterial infection to children of early age during 2 — 3 weeks conduct a course of antibacterial therapy. Appoint irednizolon, cardiac glycosides, diuretics — furosemide (lasixum), ve-roshpiron; the drugs improving exchange processes in a myocardium (Pananginum, orotat potassium, cocarboxylase, inosine, rat anti-acrodynia factors, B15, B5); at tachycardia — | 3-adrenoblo-katory, napr, aiaprilin (Obsidanum).

Forecast, as a rule, adverse.

Prevention. Pregnant women should avoid contact with sick acute respiratory diseases (wearing a mask, an instillation in a nose of interferon, UF-radiation of the apartment); reception ascorbic to - you, vitamins of group B is recommended.

Bibliography: Vikhert A. M. Vrozh

denny fibroelastosis of an endocardium, Arkh. patol., t. 19, No. 6, page 57, 1957; To N about l-l of e X. Primary thickening of an endocardium at early children's age, in the same place, t. 25, No. 8, page 60, 1963; Crimean L. D. Pathological anatomy of inborn heart diseases and complications after their surgical treatment, page 209, M., 1963; E Is opaque. E. and Postnov Yu. V. Fibroelastoz of an endocardium at adults, Arkh. patol., t. 27, No. 12, page 68, 1965; Pathological anatomy of diseases of a fruit and the child, under the editorship of T. E. Ivanovo and B. S. Gusman, t. 1, page 202, M., 1981;

Petrosyan Yu. S., etc. An endomyocardial biopsy in diagnosis of some forms of cardiomyopathies at children, Cardiology, t. 15, No. 11, page 17, 1975; P a shouting about sh and K. K N. A cardiorrhesis at a fibroelastosis of an endocardium, Arkh. patol., t. 25, No. 11, page 81, 1963; Chernova M. P., etc. About the frequency of identification of a syndrome of a hypoplasia of a left ventricle at newborn children and its relation to primary fibroelastosis of an endocardium at children, Pediatrics, No. 6, page 43, 1976; E d-wards J. E. Congenital malformations of the heart and great vessels, in book: Pathology of the heart, ed. by S. E. Gould, p. 266, Springfield, 1953; Factor S. M. Endocardial fibroelastosis, myocardial and vascular alterations associated with viral-like nuclear particles,

Amer. Heart J., v. 96, p. 791, 1978;


Keith J. D., Rowe R. D. a. V lad P. Heart disease in the infancy and childhood, p. 941, N. Y., 1978; Mol-Je r J. H. a. Neal W. A. Heart disease in infancy, p. 276, N. Y., 1981;

R o s s i E. >Die Fibroelastosis endocardiea { F. E.) und ihre Differentialdiagnose, Pa-diat. Padol., Bd li, S. 23, 1976; Schr y-e r M. J. a. Karnauchow P. N. Endocardial fibroelastosis, Etiologic and pathogenetic considerations in children, Amer. Heart J., v. 88, p. 557, 1974;

S with o t t i T h. M. Heart, Endocardial fibroelastosis, in book: Pathology, ed.

by W. A. D. Anderson a. J. M.Kissane, v. 1, p. 834, St Louis, 1977; Westwood M. o. Heredity in primary endocardial fibroelastosis, Brit. Heart J., v. 37, p. 1077, 1975.

Ii. A. Belokon; G. A. Chekareva (stalemate. An.).

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