Pathology is rare( as well as SBH, and sinus aneurysm of Valsava).Early detection of the disease will help to avoid unpleasant surprises and be treated on time. Knowing the signs of the violation, parents will be able to notice the dangerous symptoms of their children and make a diagnosis, so as not to miss the moment of timely assistance.
Today we will tell you about how the abnormality of Ebstein in adults and children is taking place, is being treated and diagnosed.
Features of the disease
A healthy heart has four chambers, and the blood flow is provided in one direction. This is ensured by the functioning of the valve system. Any changes are fraught with serious malfunctions of the heart.
Nature provides that between the right atrium and the ventricle is a valve. It has a tricuspid structure. It is an intermediary between the structural parts, arranged so that it allows venous blood to pass into the ventricular compartment from the atrium, closing the retreat.
In the case of Ebstein's anomaly, a dangerous congenital pathology in the structure of the heart, a rare form of defect, is observed.
The three-leaf valve occupies an incorrect position.It is located below the supposed site of the atrium and ventricle. Therefore, the atrium lengthens, and its area, which anomalously increases it, due to the understated position of the valve, is called the atrialized part. The ventricle is reduced. Since the right half of the heart is responsible for receiving the venous current and directing it to the lungs, where the blood receives oxygen, the pathology of the right half, associated with an incorrect valve dislocation, introduces disturbances into this function.
Blood can again enter the atrium. The overflow of this part of the heart creates tension in it. The rescue circle is a defect that often accompanies such pathologies - the possibility of flowing through the septum of some part of the blood to the left half of the heart.
The following video will tell you more about the features of the Ebstein anomaly:
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The opinion of doctors. .. & gt; & gt;
Forms and classification of
The violation of the tricuspid valve due to its displacement from the correct arrangement can be of varying degrees of complexity.
- Severe anomaly A significant increase in the right atrium, causing dangerous preconditions for a number of functional disorders.
- Small disturbance Minor displacements can introduce small disruptions in the heart. In this case, the work of the right ventricle as a whole has some deviations from the norm, the function is preserved.
About what are the causes of the anomaly of Ebstein, read further.
Comparative diagram of healthy heart and susceptible abnormality of Ebstein
Causes of occurrence of
The specialists do not have a clear idea why the fetus has such a pathology. The risk factors are:
- If a woman is infected during the gestation and as a result has been ill, there have been cases when the child was found to have an abnormality of Ebstein. A number of infectious diseases are a danger.
- To disruption can lead to a propensity for this disease at the level of genes.
- An unfavorable ecological situation can cause different mutations.
- It is noted that preparations with lithium content can cause this pathology. There were cases when they were taken during pregnancy and this fact coincided with the detection of an abnormality in the fetus.
Next we will tell you about the symptoms of Ebstein's anomaly.
Symptoms of
If the valve displacement relative to the correct location is small, the pathology may not be noticed for a long time. In such cases, the most pronounced symptom is bluish lips and skin. With a serious deviation of the valve from the natural position, the disease is usually found in the hospital.
Symptoms of anomaly
- The patient suffers from fatigue.
- Cyanosis of the skin - this effect is due to the fact that in the arterial blood in the left half, a discharge of venous blood through the hole in the wall occurs. If a part of the blood does not spread to the adjacent half, then the cyanosis will not be observed in the skin. In complex cases, it can be dangerous to overload the right atrium.
- Shortness of breath also occurs due to the mixing of blood in the "arterial heart".A lack of oxygen is obtained in the arterial blood.
- Patients may experience heart attacks.
- In connection with, a greatly enlarged right atrium, an eruption may appear to the left of the sternum, which is called the heart hump.
- There is often a thickening on the tips of the fingers and toes. This symptom usually happens in patients with cyanotic skin.
- Cervical veins have a swollen appearance.
- A doctor may suspect a valve failure while listening to a patient.
- Possible enlargement of the liver.
- Lower extremities flow.
Diagnostics
Let's see how the diagnosis of the Ebstein anomaly occurs.
- The specialist can determine the disturbances of rhythms during listening and notice the signs of an abnormality of Ebstein.
- X-ray examination can detect an enlarged atrium.
- ECG with Ebstein abnormality will show a graphic representation of the rhythms, where you can track violations. Conductivity between sections is also observed.
- Echocardiography using signal recording can detect structural disorders in the heart.
- Electrophysiological study - studies the nuances of the abnormal pathway in the event of cardiac arrhythmias.
- Cardiac catheterization is performed by inserting a catheter into the right atrium. It makes it possible to make intracavitary ECG, to investigate blood for oxygen saturation. This procedure for diagnosis of Ebstein's abnormality is infrequent. Enough other methods of examination.
- Angiocardiography - contrast material helps to see the sizes of the right atrial cavity and other details.
Next, we'll talk about the correction( treatment) of the anomaly of Ebstein.
The following video will show you what the Ebstein anomaly looks like from the inside:
Treatment
Pathology requires surgery. Without surgery, you can get by if the anomaly has a slight deviation from the norm.
Then conduct therapy, which basically involves drug treatment.
Therapeutic method
In case of violations related to the displacement of the valve, state corrective agents are prescribed:
- in the determination of heart failure - ACE inhibitors, diuretics, glycosides;
- for rhythm disturbance - antiorhythmics.
Next, we will talk about the surgical intervention and the peculiarities of anesthesia during surgery with an abnormality of Ebstein.
Operation
There may be two types of intervention:
- installation of a prosthetic valve,
- reconstruction of an existing valve by plastic surgery.
In the first case, an artificial valve is made of natural materials or a titanium alloy. The mechanical valve is more durable, but with its introduction the patient will have to take blood thinning agents for life.
In the second case, suture the excess part of the atrium, restore the correct location of the valve. Such an operation is called valve-saving, but not in every case there is an opportunity to do it. This depends on the degree of pathological deviation of the valve.
An anesthesiologist during surgery should consider the possible instability of pressure. After the operation, you should continue to observe the pressure for a while.
Prevention of the disease
Due to the fact that the disease is congenital, the preventive measures also apply to the period of pregnancy:
- in time to register and carry out all the advice of a specialist,
- not to smoke,
- if you need to take medication - do this under strict supervisiondoctor;
- if there are diseases that can destroy tissues - it will be treated before pregnancy;
- avoid contact with harmful chemicals,
- do not take funds with lithium content,
- if the expectant mother has a heart disease, you should carefully monitor the fetus.
Complications of
The disease is life-threatening due to the progression:
- of heart failure,
- of possible stroke,
- heart rhythm disturbances,
- if surgery is performed and the valve does not create a precedent for serious anomalies - if advised by experts, complications are not expected.
Forecast
In the case where the location of the valve is very different from the normal location, centenarians almost never occur. There are several cases where patients reached the age of sixty.
In addition to valve failure, life expectancy can decrease:
- blood clots( thromboembolism),
- cardiac rhythm disturbance,
- infectious inflammation of internal cardiac membranes.
If the patient has undergone the necessary treatment and an operation is performed, then the forecast is optimistic. The main thing is to safely transfer surgical intervention, there are cases of death. After this procedure, a person can lead a normal lifestyle, following the advice of a doctor.
Whether it is necessary to experience, if the doctor puts the woman at pregnancy the diagnosis "anomaly of Ebstein".Read about this in conclusion.
Ebstein's anomaly diagram in humans
Special case: Ebstein's anomaly in the fetus
If the diagnosis is made during pregnancy and the situation is a threat to the life of the fetus, then surgery is possible. The wall of the uterus spreads and the surgeon can operate the baby.
If the pathology is not very dangerous, surgical intervention is postponed until adolescence.
Even more useful information contains a video from the popular TV show, presented below: