One of the rare heart defects is Ebstein's anomaly. This is a congenital disease in which the tricuspid valve of the heart is moved to the right ventricle, although it should be on the border between the ventricle and the atrium. It is located well downstream of the bloodstream.
Anatomical features
Ebstein's anomaly leads to the fact that the cavity of the right ventricle becomes smaller, and the right atrium - more than normal. The valve stems are also different. They may be incorrectly developed or displaced. The development of the entire chordal and muscular apparatus, which sets the tricuspid apparatus in motion, is disrupted.
Due to the small size of the right ventricle, less blood is ejected from it. And the increased size of the right atrium is due to the fact that part of the venous blood enters it.
Many patients with Ebstein's anomaly also have an atrial septal defect and a patent foramen ovale. This concomitant vice for many is life-saving. Through the defect of the septum from the overflowing right atrium there is a discharge of blood. True, whilevenous blood in the left atrium mixes with arterial blood. This causes oxygen starvation of tissues and organs.
Causes of disease development
Less than 1% of people with congenital heart defects are diagnosed with Ebstein anomaly. The reasons for its development could not be precisely determined. According to assumptions, it appears due to genetic anomalies. Also, according to one version, the use of drugs containing lithium s alts during pregnancy can lead to such a development of the heart muscle.
This congenital disease is accompanied in 50% of cases by an atrial septal defect. In other cases, there is a violation of the rhythm. Doctors have not yet been able to figure out why certain combinations of heart defects occur.
Possible variants of the course of the disease
Ebstein anomaly is a critical congenital heart disease. It occurs in both boys and girls. The course of the disease and prognosis will depend on the degree of deformation of the tricuspid valve. Also important are the pathophysiological changes that took place in the right atrium and ventricle.
In some cases, the defect significantly disrupts the blood circulation of the fetus. Heart failure occurs, dropsy develops, and in 27% of cases, intrauterine death of the child occurs. With a deep pathology after birth, babies die within the first month of life. According to statistics, this is about 25% of all children who have Ebstein's anomaly. The diagnosis during pregnancy can be established as early as the 20thweek.
About 68% of newborns with this pathology live up to six months, and up to 5 years - 64% of children. This group includes babies in whom the function of the tricuspid valve and the right ventricle is satisfactory. They die in most cases from progressive heart failure and arrhythmias.
Classification of disease types
Specialists identify several stages of the disease, known as Ebstein's anomaly. Symptoms will depend on how large the damage to the heart is. The most rare is the asymptomatic stage. Such patients may not even be aware of the pathology and lead a normal life. They tolerate physical activity well.
At the second stage, the clinical manifestations are pronounced. This anomaly manifests itself even in childhood and proceeds quite hard. Stages are distinguished separately:
- II a - it is characterized by the absence of cardiac arrhythmias;
- II b – seizures are common.
The third stage is called the period of persistent decompensation. It is diagnosed when the body is unable to compensate for the activity of the heart with any auxiliary mechanisms.
Clinical picture of the disease
At birth, babies with Ebstein anomaly are blue. Cyanosis after 2-3 months of life decreases due to the fact that the resistance of the vessels of the lungs decreases. But in children with a minor defect of the heart septum, the risk of death from complications of cyanosis and progressiveheart failure.
Children with Ebstein's anomaly often have the following symptoms:
- shortness of breath even at rest;
- swelling that is visible on the lower extremities;
- increased fatigue, especially noticeable during physical exertion;
- heart rhythm disturbances;
- cyanosis of the skin and lips.
Blueness appears in children earlier than the symptoms of heart failure. Older children may complain of intermittent palpitations.
Diagnosis of disease
In some cases, the disease is determined during pregnancy or immediately after birth in the hospital. There are several tell-tale signs that a doctor may suspect that a child has Ebstein's anomaly. Diagnosis includes auscultation, chest x-ray, echocardiography, ECG. All these methods in combination allow you to accurately establish the diagnosis.
During auscultation, the doctor can hear a characteristic rhythm, it can be three or four times. It drowns out the high-frequency quiet systolic murmur of tricuspid insufficiency. In addition, the splitting of the second tone is pronounced, it does not change during breathing. A mid-diastolic raspy soft murmur should be heard at the apex of the left sternum.
The x-ray shows an enlarged cardiac shadow, this is due to a significant increase in the right atrium. The vascular pattern of the lungs is usually pale. The heart often takes the form of a ball.
Changes are observed andon the ECG. The cardiogram shows signs of right atrial hypertrophy, the PQ interval is prolonged, and there is a complete or partial blockade of the right bundle branch block.
On echocardiography, you can see a wide opening of the tricuspid valve. The septum is shifted towards the top.
Tactics of action in case of congenital anomaly
After establishing the diagnosis and determining the degree of damage, treatment is selected. Of course, such patients need surgical intervention. The only exception is that small group of patients who have asymptomatic Ebstein anomaly.
Treatment is based on other associated problems. If the patient has survived infancy normally, surgery may be delayed until severe symptoms of heart failure appear.
All babies with this problem should be seen by a pediatric cardiologist who specializes in congenital heart defects. Also, his condition should be monitored by cardiac surgeons.
If patients have a pronounced decrease in pulmonary blood flow and signs of heart failure are observed, then infusions of ionotropic drugs, group E prostaglandins are prescribed. Correction of metabolic acidosis is also indicated. This treatment increases cardiac output and reduces compression of the left ventricle by the enlarged right side.
In cases where tachycardia is observed, it is necessary to carry out therapy with special antiarrhythmic drugs.
Indicationsfor surgical intervention
In the neonatal period, they try not to have surgery. But it is necessary in cases where right ventricular dysplasia is pronounced and is accompanied by impaired antegrade blood flow to the pulmonary bed. At the same time, there is a sharp expansion of the right sections and simultaneous compression of the left ventricle.
Relative contraindications include age up to 4-5 years. But if necessary, they also perform surgery on newborns. Also, surgery is not performed for those who have organic irreversible changes in their internal organs.
Patients diagnosed with Ebstein's anomaly can live up to an average of 20 years without surgery. Their death is usually sudden. It occurs due to ventricular fibrillation of the heart.
Operating
Only with the help of surgery can the situation be completely corrected. At the same time, doctors recommend, if possible, to do it at an older age due to the too small size of the heart in infancy.
The operation is carried out on an open heart, to implement the possibility of its implementation, the process of cardiopulmonary bypass is organized. The excess portion of the right atrium is sutured, and the tricuspid valve is raised with sutures to a position that is close to normal. In cases where this is not possible, it is removed and replaced with a prosthesis. This allows you to forget that the patient had an Ebstein anomaly. In adult patients, prosthetics can be performed, butchildren under 15 years of age undergo valve plastic surgery.
Mortality during operations does not exceed 2-5%. The probability of death depends both on the experience of the cardiac surgeon and on the severity of the defect. In almost 90% of cases, patients can return to their normal lifestyle within a year.
Special attention should be paid to such patients by anesthesiologists. After all, a special approach is required to patients who have Ebstein's anomaly. Features of anesthesia should be known to the doctor. He must take into account that the pressure in such patients may be unstable. Therefore, the anesthesiologist monitors his patients after surgery.
Types of prostheses
In most cases, it is possible to decide exactly how the operation will be performed only when the patient is already on the operating table. If it is possible to perform valve plastic surgery, then this option is preferred. But there are situations when a prosthesis is needed for patients who have Ebstein's anomaly. This is the name of an artificial valve, which looks like a ring covered with a synthetic cushion. Inside it is a mechanism that can open and close the so-called gateway. It can be mechanical or biological. The first option is made from a titanium alloy, while the second can be made from a pig valve or a human heart shirt fabric.
When installing a mechanical valve, the patient needs to constantly drink blood-thinning medications, but if the regime is followed, it can function longer. The biological device isless durable.
