From Big Medical Encyclopedia

EBSHTYOYNA ANOMALIYA (W. E-stein, it is mute. the therapist, 1836 — 1912) — the inborn heart disease which is characterized by the shift of shutters of the right atrioventricular valve owing to their abnormal attachment in a right ventricle. At the same time one or two shutters of the valve are attached not to a fibrous ring, and below it, and their tendinous chords — not to papillary muscles, and to walls of a right ventricle. The cavity of the last is divided by the plane of a valve opening into a so-called atrializovan-ny part and actually a ventricle (see fig. 1, to St. Heart diseases are inborn, t. 20, Art. 267). Diameter of the right fibrous ring is increased. Quite often at patients the open oval window remains or there is a defect of an interatrial partition.

AA. and. it is for the first time described by Ebstein in 1866. Meets seldom: according to domestic and foreign researchers, AA. and. makes from 0,1 to 1% of inborn heart diseases. It is observed equally often at persons men's and female.

As a result of disturbance of switching function of the three-leaved valve at E. and. a part of a venous blood comes back in the right auricle owing to what the auricle hypertrophies and considerably increases in volume. As a result of it sometimes contains in the right half of heart till 1500 .it blood.

From the level of the displaced shutters of the right atrioventricular (three-leaved) valve to an opening of a pulmonary trunk the true right ventricle is located. This reduced part of a right ventricle is the department which is carrying out movement of blood in a small circle of blood circulation. If the three-leaved valve is displaced no more than on 1 — 2 cm from a fibrous ring or from the right atrioventricular opening, an atria-lizovanny part of a right ventricle can be absent. Sometimes shutters of the right atrioventricular valve are displaced to output department of a right ventricle. In these cases an atrializovanny part happens very considerable, and a right ventricle respectively very small. The increased right auricle together with an atrializo-bathing part of a right ventricle become anatomically a uniform cavity of the right auricle. Actually right auricle is reduced according to the general auricular systole while an atrializovanny part of a right ventricle is reduced along with a systole of a ventricle that leads to difficulty of emptying of the right auricle. Owing to a combination of the small sizes of a right ventricle and insufficiency of the three-leaved valve there is an outflow of a part of blood in the right auricle and even more the systolic volume of a right ventricle decreases. As a result the pulmonary blood stream decreases.

The hypertrophy and dilatation of the right auricle lead to the progressing build-up of pressure in it. When pressure in the right auricle becomes more, than in left, blood gets to the left auricle through a nezarashchenny oval window and there is a venoznoarterialny shunt.

In a myocardium of the right auricle the hypertrophy and dystrophy of muscle fibers, in a right ventricle — the disturbance of very tectonics of a myocardium which is especially expressed in an atrializovanny part are noted. The thickening of an endocardium in the right departments of heart, expansion of cardiac veins and tebeziyevy vessels is a consequence of hemodynamic disturbances. P ri electronic microscopic examination (see the Submicroscopy) in cardiomyocytes the mitochondrions which are sharply increased in sizes (see) forming huge conglomerates are defined. Similar change of mitochondrions is considered kompensatorno - adaptive.

Nek-ry researchers distinguish

3 degrees of shift of shutters of the three-leaved valve and atrialization of a right ventricle: the first (13,2% of cases) — the shift of shutters to 2 cm, an atrialization is not expressed; the second (66% of cases) — the shift of shutters is from 2 to 4 cm, an atrialization moderate; the third (20,8% of cases) — the shift of St. 4 cm, an atrialization of extreme degree, a wall of a ventricle is thinned, his aneurism is quite often formed. AA. and. it can be combined with other heart diseases (defect of an interventricular partition, a stenosis of a pulmonary artery, defect of the mitral valve, coarctation of an aorta, etc.), and also with Wolff's syndrome — Parkinson — Whyte (see Voljff — Parkinson — Whyte a syndrome, Heart diseases inborn) that considerably makes heavier a condition of patients and complicates methods of surgical correction. Therefore allocate simple (with defect of an interatrial partition or without it) and difficult (in combination with other heart diseases) forms E. and.

Wedge, current E. and. depends on size and extent of deformation of the three-leaved valve and the pathophysiological changes of a right ventricle and auricle connected with it, and also on effects of the venous and arterial shunt. More often the disease is shown at children's age and proceeds hard, but symptoms of a disease can sometimes be absent a long time. In such cases patients lead a usual life and can even transfer exercise stresses.

On weight of hemodynamic frustration it is possible to allocate three stages a wedge, courses of a disease: I \an asymptomatic stage (meets quite seldom); II \a stage expressed a wedge, manifestations of defect (Pas — without attacks of disturbance of a cordial rhythm, PB — with frequent attacks of disturbance of a cordial rhythm); III \stage of a resistant decompensation.

Patients with E. and. attacks of heartbeat complain of an asthma (see), (see). Cyanosis is observed (see). Arterial pressure is usually lowered. Pulse, as a rule, small, weak filling. At percussion (see) note increase in the sizes of heart though the zone of a vascular bundle is usually not expanded. At auscultation of heart (see) define three - or the four-membered cantering rhythm (see Gallop a rhythm), to-ry appears as a result of bifurcation of I and II cardiac sounds or at emergence of additional III and IV tones. Also soft hissing systolic noise (see Noise cordial) which is a little reminding a pericardial rub is listened (see the Pericardis). Often there is a splitting of the II tone over a pulmonary trunk.

On the electrocardiogram (see Elektrokardiografiya) reveal total or incomplete block of the right leg of a ventriculonector with the deformed ventricular complex in the right chest assignments, a point of a tooth P in the I—II standard and right chest leads. At a phonocardiography (see) note a cantering rhythm.

At rentgenol. a research in a direct projection the shadow of heart is expanded in both parties and reminds in a form a picture at a vypotny pericardis (see). The vascular bundle is presented in the form of a narrow shadow. The right contour of a cordial shadow is formed by a dilatirovanny right auricle and an atrializovanny part of a right ventricle, the left contour — a right ventricle.

One of distinctive rentgenol. signs E. and. — significant increase in the right auricle. In the left (second) slanting projection the third arch on a front contour of heart (see fig. 10 to St. Heart, t. 23, Art. 162) is badly expressed and at sharp increase in the right auricle is straightened so that forms a right angle with a dome of a diaphragm. In the right (first) slanting projection in connection with significant increase in the right auricle and turn of heart to the left the cone of a pulmonary trunk does not manage to be tracked. In this situation only the front contour of heart is defined generally that distinguishes E. and. from other heart diseases. Note also depletion of the pulmonary vascular drawing and a hypoplasia of a pulmonary trunk and its branches.

At a rentgenokimografiya (see) teeth of ventricular type of big amplitude are determined by the right contour of heart. Teeth of a pulsation of a left ventricle in apical area, on the contrary, have small amplitude and are a little deformed. Amplitude of a pulsation of an aorta small. The zone of a pulsation of the right auricle is expanded.

At angiocardiography (see) the main sign E. and. division of a cavity of a right ventricle into two parts separated one from another as if by the crossing point corresponding to the abnormal location of the three-leaved valve (a symptom of «notch») is. Note a long delay of radiopaque substance in an upper vena cava and the right auricle. On an angiokardiogramma the distal department of a right ventricle is located on the left contour of heart and directed from left to right. Thus, in an arterial phase three cardial cavities — the right auricle, an atrializovanny part of a right ventricle and actually right ventricle are contrasted. The sizes of a pulmonary trunk meet standard, branches of a pulmonary artery narrow and are almost not contrasted. With defect of an interatrial partition radiopaque substance comes to the left auricle from right. The left departments of heart are contrasted indistinctly, an aorta of a gipoplastichn.

Catheterization of heart (see) at E. and. allows to establish reduction of a blood-groove in a pulmonary trunk and its branches, build-up of pressure in the right auricle and low pressure in a right ventricle, a pressure gradient between the right auricle and a ventricle, the shift of shutters of the three-leaved valve, existence of the message between an auricle * to a hole. However hl resort to this diagnostic method only in rare instances. obr. for an exception of the accompanying anomalies. since patients with E. and. very badly postpone this research in connection with the attacks of a Bouveret's disease developing at them (see). In doubtful cases for detection of defect in the course of catheterization of heart carry out record of intra atrial potentials by means of an electrocardiography.

AA. and. it is necessary to differentiate with a vypotny pericardis, Abramov's myocarditis — Fidlera (see Myocarditis:), the insufficiencies of the right venous mouth and the stenosis of the three-leaved valve isolated by a stenosis of a pulmonary trunk, inborn insufficiency of the three-leaved valve and an idiopatychesky cardiomegaly acquired (rheumatic). Nearly E. and. differentiate with those heart diseases, to-rye are followed by reduction of a pulmonary blood-groove (see Heart diseases inborn, the Heart diseases acquired).

Conservative treatment is ineffective. As relative contraindications to operation serve age of the patient less than 4 — 5 years and existence of irreversible organic changes in internals. At the palliative operative measures directed to increase in a pulmonary blood-groove by imposing of an interarterial anastomosis, operations of closing of the interatrial message and an atrioventricular ilikation of positive effect did not observe.

Radical operational treatment E. and. became possible only using prosthetics the valve, ov hearts (see). For the first time reported about radical operation in 1963 Mr. Barnard and Shrir (Page N. Barnard, U. Schrire). Radical operation includes recovery of switching function of the three-leaved valve that is reached by means of its prosthetics, elimination and a trial of an isobathing part of a right ventricle by means of an ilikation of this part to a fibrous ring, reduction of a cavity of the right auricle by excision of a part of its wall, elimination of the interatrial message, intervention on the carrying-out system of heart.

In the USSR the first successful radical operation on an occasion E. and. it was made in 1964 in All-Union scientific research institute of clinical and experimental surgery M3 of the USSR (nowadays All-Union scientific center of a hirugiya of the USSR Academy of Medical Sciences). Now the accurate technique of operation is developed. After introduction of the patient to an anesthesia allocate a femoral artery. By a sternotomy (see the Mediastinotomy) or Torahs and to weary and (see) open a chest cavity. Insert a cannula into a femoral artery and a venous catheter through an ear of the right auricle. All other manipulations carry out in the conditions of artificial circulation (see). Open a cavity of the right auricle. The section is carried out from the mouth of the lower vena cava parallel to a coronal furrow and finished with a section of an ear of the right auricle. After cultivation of edges of a wound of an auricle good access to an interatrial partition and the three-leaved valve opens. Defect between auricles is taken in. Shutters of the three-leaved valve do not exsect. Separate 11-shaped seams hem to a fibrous ring ksenoklapan (see Prosthetics of valves of heart).

Zone of conduction paths of heart (area of a bunch of the Yew) located kpered and medially from the mouth of a coronal sine (see. The carrying-out sistelga of heart), is most dangerous during the imposing of P-shaped seams. Irreversible or passing blockade of the carrying-out system of heart is a specific complication of operation for Ebstein's anomaly in the conditions of artificial circulation. For the prevention of an injury of conduction paths of heart operation carry out at the kept heart rhythm under constant electrocardiographic control (see M onitorny observation). If at stitching there is a cross block, the seam is removed at once. For prevention of an atrioventricular block (see the Heart block) the prosthesis of the valve is fixed with use of a basic patch from an autoperikard in the field of the carrying-out system of heart. The Atri a lizovanny part of a right ventricle is liquidated by its dublikatura or plication. P-shaped stitches are put between a true fibrous ring and the displaced shutters of the three-leaved valve. During the tightening of seams there is plication of an atrializovanny part of a right ventricle and the hemodynamics is normalized. However plication is admissible only when length of an atrializovanny part does not exceed 5 — 6 cm, differently perhaps sharp reduction of a cavity of a true right ventricle leading to death of the patient. A part of a dilatirovan-ny auricle is resected, danger of his thrombosis as a result decreases. The wound of the right auricle is taken in a two-row continuous suture. Delete venous catheters of the cardiopulmonary bypass. Put rare stitches on a pericardium. And in the right pleural cavity enter drainages into a cavity of a pericardium, a front mediastinum (see Drainage). The wound of a chest wall is layer-by-layer taken in.

Long-term results of operational treatment E. and. satisfactory. Average life expectancy of not operated patients, according to most of researchers, makes 20 years; usually fibrillation of ventricles is the reason of their sudden death (see Fibrillation of ventricles of heart). Bibliography: Zubarev P. IT. Ebstein's anomaly, M., 1975; Savelyev V. S. Sounding and angiocardiography at inborn heart diseases, M., 1961;

Caralps J. M and. lake of Ebstein's anomaly, Surgical treatment with tricuspid replacement without right ventricular plication, Ann. thorac. Surg., v. 31, p. 277, 1981; E b s t e i n W. Ueber einen sehr seltenen Fall von Insufficienz des Valvula tricuspi-dalis bedingt durch eine angeborene hoch-gradige Missbildung derselben, Arch. Anat. Physiol. (Lpz.), Bd 33, S. 238, 1866.

P. P. Zubarev.