Ebstein's anomaly - Causes, Symptoms and Treatment .MF .

August 12, 2017 17:52 | Congenital Anomalies

anomaly Ebstein - congenital heart disease, which is characterized by displacement of the insertion of one or two valves right atrioventricular (tricuspid or tricuspid) valve into the cavity of the right ventricle towards the apex of the heart, resulting in a reduction in the right ventricular cavity, andrespectively, an increase of the cavity of the right atrium.This congenital heart disease was first described by German physician Ebstein in 1866.

In this congenital anomaly, one or two doors tricuspid located not at the right level: they are not attached to the fibrous ring between the right atrium and right ventricle, and slightly below it;tendon chord of the leaflets are attached not to the papillary muscles, and to the walls of the right ventricle.Always with Ebstein's anomaly in the right ventricular cavity is shifted back flap tricuspid valve, often enough - and even septal flap.By the atrioventricular fibrous ring remains attached only a single front flap tricuspid.This pathology is also accompa

nied by an expansion of the fibrous ring, which leads to insufficient tricuspid valve.

Thus, right ventricular cavity is divided by a plane offset below on the blood flow of the valve hole into two parts: the actual ventricle (below the plane), and the so-called atrializovannuyu or supravalvular part (above the plane), which is combined with the cavity of the right atrium.

Schematic illustration of a healthy heart (left) and heart with Ebstein's anomaly (right)

In 80-85% of cases with Ebstein's anomaly has an abnormal communication between the left and right atria, which are usually caused by stretching the edges of the ovalholes (it is normal shortly after the birth of a child should be closed and overgrown) or, more rarely, the existence of secondary atrial septal defect (ASD).

Ebstein anomaly is rare: its rate is 0.5-1% of all congenital heart defects.Observed with equal frequency in males and females.Ebstein's anomaly in 5% of cases associated with other congenital heart disease: ventricular septal defect (VSD), pulmonary artery stenosis, patent ductus arteriosus, stenosis or mitral valve insufficiency, aortic atresia.

Causes of Ebstein's anomaly

Causes of Ebstein's anomaly to date remain insufficiently clarified, but noted an important role of genetic factors and environmental factors.In some cases, a link between intake of a pregnant woman or lithium preparations prenesёnnymi during pregnancy some infectious diseases and birth of a child with Ebstein's anomaly.

mechanism of hemodynamics (blood flow) disorders

right atrium with atrializovannoy part of the right ventricle becomes anatomically single cavity of the right atrium.However, it is actually the right atrium is reduced during systole (during muscle contraction) of the atria and right ventricle of the atrializovannaya continued to decline in ventricular systole.This leads to significant difficulty emptying of blood from the right atrium into the right ventricle.Due to the combination of small size itself of the right ventricle and the resulting abnormal structure of the valves insufficiency tricuspid valve of the blood flowing back into the right atrium (which normally should not be), thereby even further reduced systolic volume of the right ventricle, and hence the volume of blood,coming out of it in a small (pulmonary) circulation.It develops gradually increasing muscle mass and size of the right atrium, leading to a progressive increase in blood pressure in it.At the moment when the pathologically elevated pressure in the right atrium is greater than in the left atrium appears abnormal discharge of blood from the right atrium to the left through the oval window nezaraschёnnoe - thus develops arterial-venous shunt.

When enlarged right atrium loses the ability to continue to expand, it creates difficulty in entering the blood from the vena cava, bringing blood to the heart, into the cavity of the right atrium.As a result of developing venous stasis in the systemic circulation.

With a small offset tricuspid valve hemodynamic disturbances are minimal, and the most severe violations of developing a combination of significant bias, and the pathological changes in the valves tricuspid valve with absence of interatrial communication or the small size of the message.In such a situation it is determined by a significant expansion of the right atrium, forming, together with part of the right ventricle atrializovannoy huge cavity, which can hold up to 2.5 liters of blood.

symptoms of Ebstein's anomaly

clinical course of Ebstein's anomaly depends on the severity of displacement and deformation of the tricuspid valve, the related pathological changes in the right ventricle and the right atrium;In addition, the severity of clinical manifestations affect the presence and severity formed arterio-venous shunt and its consequences.

favorable variant of Ebstein's anomaly is long enough to be asymptomatic and detected only during the x-ray of the chest or electrocardiography.When expressed clinical manifestations of the congenital heart disease diagnosed in the hospital or in the first weeks and months of life of a sick child.

physical development of these children his age.They may complain of heart pain, shortness of breath, fatigue with exertion, heart attacks, which in some cases can be protracted and accompanied by a loss of consciousness.

On examination of children with Ebstein's anomaly is defined by cyanosis (blueness of the skin), which occurs in 75-85% of children.In most patients, cyanosis is noted at birth, rarely appears in the age of 3-12 years.Intensity cyanosis varies in accordance with an increase in arterial-venous reset also happens to change its color to magenta cyanotic.If no atrial message, cyanosis does not develop.It is often swelling of the neck veins, rarely (usually in the final stages) - their so-called systolic pulsation (filling veins in synchronization with the heartbeat).In addition, in patients with cyanosis develops a characteristic deformation of the terminal phalanges of the fingers and toes - "drum your fingers" (flask-shaped clubbing) and "hour glass" (a characteristic deformation of the nail plate in the form of a watch glass).In those cases, if the congenital defect combined with pulmonary artery stenosis, characterized by an even greater decrease in the oxygen content in the arterial blood, which clinically manifested by the fact that these patients sit down on his haunches.

In addition, approximately 50% of patients during the inspection is determined by the so-called "heart hump" on the left of the sternum - his appearance is caused by abnormally huge size of the right atrium.Apical impulse palpable reductions in heart to the left and below the usual place - in the fifth or sixth intercostal space at the anterior axillary line.

Auscultation (listening to) the heart doctor determines deafness and weakened heart sounds, as well as the so-called rhythm "canter" (three- or chetyrёhchlenny rhythm).Most patients auscultated soft tone systolic (when the heart contraction) noise from the left of the sternum due to tricuspid insufficiency.When stenosis (narrowing) tricuspid in the same place can be auscultated mild diastolic (the phase of the heart muscle relaxation) noise.

for Ebstein anomaly characterized by the development of heart failure, right heart blood stagnation phenomena in the systemic circulation, which sometimes appears with the first months of a child's life, but it can develop very late in some cases.Her clinical symptoms are shortness of breath, heart palpitations, liver enlargement, throbbing neck veins;it has a negative prognostic value.

Diagnostics Ebstein anomaly

1. Electrocardiography (ECG): In most cases, the electrocardiogram recorded electrocardiographic signs of hypertrophy (increase in muscle mass) of the right atrium, the heart's electrical axis deviation to the right, as well as cardiac arrhythmias: atrial fibrillation and flutter, ventricular and supraventricular arrhythmias, paroxysmal tachycardia.In those cases where a patent foramen ovale, and the associated large abnormal discharge of blood from the right atrium to the left atrium, in addition to signs of myocardial hypertrophy are determined right atrium and electrocardiographic signs of increased left atrium.

2. phonocardiography (recording vibrations and sound signals produced when the activity of the heart and blood vessels): Determined weakening tone I on the left edge of the sternum in the third or fourth intercostal space, can be detected IV tone.In the second intercostal space at the left edge of the sternum is determined tone weakened II.In the fourth or fifth intercostal space on the left is recorded low amplitude systolic murmur due to tricuspid insufficiency.In those cases where a tricuspid stenosis, phonocardiogram for this is reflected in the form of short protodiastolic (contraction of the heart immediately after the beginning of its relaxation) noise.

3. Echocardiography (ultrasound of the heart).Echocardiography revealed the main features of Ebstein's anomaly: anamalnoe location tricuspid valve (closer to the apex of the right ventricle), volumetric expansion of the right atrium and right ventricle of the atrializovannoy, deformation tricuspid leaflets, the lag time slam tricuspid occasionally slam butterfly valve.Doppler echocardiography reveals signs of tricuspid insufficiency and abnormal discharge of blood through the oval window.

4. chest organ cells (heart and lungs).There depletion of lung pattern, which is accompanied by an increase in the transparency of lung fields.Heart, as a rule, increased in size has the form of a sphere or "inverted bowl".Determined signs of increased right atrium and right ventricle of the upper parts of what is best revealed in the oblique projections.Vascular narrow beam of heart, the left side of the heart is not enlarged.When X-rays has been a sharp reduction in the amplitude of the pulsation of the heart and pulmonary arteries circuit.

5. catheterization of heart cavities is carried out to measure the pressure in the right atrium, right ventricle and pulmonary artery, as well as determining the blood oxygen saturation level.In Ebstein's anomaly is detected high blood pressure in the right atrium and the pulmonary artery - reduced.The right ventricle are determined by two different blood pressure zones, which are separated by a diseased tricuspid valve located.In those cases where a patent foramen ovale, left atrial determined reduced compared with the normal blood oxygen saturation that is caused by abnormal discharge of blood from the right to the left atrium.

6. Angiocardiography - the introduction of contrast medium into the heart cavity through a special catheter.In Ebstein's anomaly is defined by intense opacification of the enlarged right atrium and right ventricle of the atrializovannoy.The distal (end) of the right ventricle is weaker and contrasted with the slowdown, the decrease is determined by its diameter.

Treatment of Ebstein's anomaly

Ebstein anomaly Conservative therapy is aimed at eliminating cardiac arrhythmias and decrease the severity of heart failure.As an anti-arrhythmic drug prescribed beta-blockers, calcium channel blockers, cardiac glycosides.In heart failure, appointed diuretics, cardiac glycosides, angiotensin converting enzyme inhibitors (ACE inhibitors).

Surgical treatment is best done in adolescence: optimal for surgery is the age of 15 years and older.With no or small amounts of atrial septal in children aged 2-3 months may develop a critical state, since there is no discharge of the right atrium through the blood discharge from the right atrium to the left;such children may die from progressive heart failure.To avoid such an outcome, to urgently carry out an artificial extension of ventricular septal defect cylinder (the so-called procedure Rashkinda).

Radical surgery of Ebstein's anomaly is performed open-heart surgery with cardiopulmonary bypass.It is the elimination of "extra" portion of the right atrium by suturing, plastics tricuspid valve, which is lifted and strengthened through the joints in the normal position close to as well as the elimination of sources of cardiac arrhythmias and atrial septal defect closure.If the plastic correction of tricuspid valve can not be made to be replaced by artificial prosthesis.

prognosis of Ebstein's anomaly

When significant shift tricuspid and significant deformation of its flaps manifestations of this congenital defect observed in early childhood, when developing severe heart failure from which the progression of sick children may die.The cause of death in older patients not operated on for blemish is a slowly progressive heart failure and arrhythmias, which can also lead to sudden death.Early infant mortality in patients with Ebstein's anomaly is relatively rare, most of the patients die in 20 or, especially, to 30 years.Only very few described cases in the medical literature of patients with Ebstein's anomaly lived to 60 years.Deterioration usually occurs in young adulthood, which manifests the appearance and the growth of cardiac insufficiency.Besides heavy heart failure and serious cardiac arrhythmias other causes of death of these patients are thromboembolism (acute blood stasis occurring thrombus), brain abscesses, infective endocarditis (damage inner lining of the heart and heart valves, caused by an infection).

Surgeon Kletkin ME