Endocardium fibroelastosis

Description:

The Endocardium Fibroelastosis (EF) means the diffusion thickening of an endocardium caused by proliferation of the fibrous and elastic fabric leading to decrease in plasticity and deterioration in diastolic function of heart. FE belongs to rare VPS and in most cases leads to death in the perinatal period in connection with reduced sokratitelny ability of a myocardium. Defeat can be isolated or be combined with other anomalies.

Endocardium fibroelastosis reasons:

The etiology of a fibroelastosis of an endocardium is finally not clear. Among hypotheses of probable causes now most often call inflammatory processes. Refer also autoimmune processes, collagenic diseases, inborn disbolism to the possible reasons. The factor of hereditary transfer is also a subject of discussion as observations of inheritance of FE on autosomal recessively type or the H-linked mode of inheritance are described.

Types of a fibroelastosis of an endocardium:

Distinguish the following forms of a fibroelastosis of an endocardium.
A. Fibroelastosis of an endocardium of a left ventricle. Process of defeat reaches 90%. According to the sizes of a left ventricle of FE it is subdivided into two types: 1) dilatirovanny, the most frequent at which the left ventricle is considerably increased in sizes, a form its spherical and the top of heart is completely created by a left ventricle which wall is thickened and at the same time the interventricular partition eminates in a cavity of a right ventricle; 2) kontraktilny at which the sizes of a left ventricle normal or are even reduced. The right ventricle is hypertrophied and his cavity is expanded. B. Fibroelastoz of an endocardium of a right ventricle. B. An endomyocardial fibroelastosis of an endocardium (Davies's disease) - massive fibrous thickenings of an endocardium of tops of ventricles with thrombosis of a wall and involvement in process of an internal part of a myocardium.

Prenatal diagnosis:

The prenatal ultrasonic picture of a fibroelastosis of an endocardium depends on its form. At the FE dilatatsionny form the sizes of a left ventricle are considerably expanded, its wall is thickened, and the interventricular partition eminates in a cavity of a right ventricle. Heart at the same time is increased in sizes and gets the form close to spherical. The cavity of a left ventricle at the FE kontraktilny form has the normal sizes or reduction of the sizes of a left ventricle is more often observed. It should be noted that quite often the FE kontraktilny form is prenatalno treated as a hypoplastic syndrome of the left departments of heart. Distinctive feature of FE is the expressed increase in echogenicity of an endocardium of a left ventricle. According to data of A. Carceller and соавт., transition of a dilatatsionny form to the FE kontraktilny form is possible. Many authors point to development of congestive heart failure and edema of a fruit at an endocardial fibroelastosis both in prenatal, and in post-natal the periods. Prenatal ultrasonic diagnosis of a fibroelastosis of an endocardium is possible since the beginning of the II trimester of pregnancy. However it should be noted that FE not in all cases can be shown to 20 weeks of pregnancy. The clinical observation described by L. Hornberger соавт in this respect is indicative. At the patient of 38 years the first screening ultrasonography was conducted in 17 weeks of pregnancy during which changes of a four-chamber cut of heart of a fruit are noted, the sizes of ventricles were identical. At additional studying of the main arteries also changes were not revealed, diameter of the ascending aorta made 2-2,5 mm. Repeated ultrasonography was conducted in 25 weeks of pregnancy at which expansion of a cavity of a left ventricle is revealed, its sokratitelny ability was sharply reduced, and the endocardium looked hyper echoic. During the dopplerekhokardiografichesky research of a fruit the expressed decrease in a flow of blood from the left auricle in a left ventricle, and also moderate mitral regurgitation is noted. Diameter of the ascending aorta at the level of the aortal valve did not exceed 2,5 mm and in it only the retrograde blood stream was registered. On the basis of the obtained data the diagnosis of a critical stenosis of an aorta and FE was exposed. In a type of the adverse forecast parents made the decision to interrupt pregnancy. In researches of domestic authors the term of prenatal detection of FE varied from 23 to 34 weeks, having averaged 26,4 weeks. Along with characteristic changes of A. V. Sidorov and соавт. noted atypical prenatal manifestations of FE. In all 3 cases presented by them in a cavity of a left ventricle multiple hyper echoic inclusions were visualized that gives the grounds to consider them as one of options of manifestation of pathological process in the valve device at FE. Important additional value at FE has TsDK which allows to estimate a condition of an endocardiac blood-groove. In all observations published by domestic researchers adverse perinatal outcomes are noted.