Multiple atrial septal defects in a newborn. What is an atrial septal defect and why is it dangerous?

Defect interatrial septum(ASD) is the second most common congenital heart defect

With this defect, there is a hole in the septum that separates the right and left atria into two separate chambers. The fetus, as we said above, not only has this hole (open oval window), but is also necessary for normal blood circulation. Immediately after birth it closes in the vast majority of people. In some cases, however, it remains open without people being aware of it. The discharge through it is so insignificant that a person not only does not feel that “there is something wrong with the heart,” but he can also calmly live to see old age. (It is interesting that thanks to the capabilities of ultrasound, this defect in the interatrial septum is clearly visible, and in last years articles have appeared that show that among such adults and healthy people, who cannot be classified as patients with congenital heart disease, the number of people suffering from migraines—severe headaches—is significantly higher. These data, however, have yet to be proven).

Unlike non-union of open oval window, true atrial septal defects can be very large sizes. They are located in different departments the septum itself, and then they talk about a “central defect” or “a defect without an upper or lower edge,” “primary” or “secondary.” (We mention this because the choice of treatment may depend on the type and location of the hole).

If there is a hole in the septum, a shunt occurs with blood discharge from left to right. With an ASD, blood from the left atrium partially flows into the right atrium with each contraction. Accordingly, the right chambers of the heart and lungs become overfilled, because they have to pass through themselves a larger, extra volume of blood, and one more time that has already passed through the lungs. Therefore, the pulmonary vessels are filled with blood. Hence the tendency to pneumonia. The pressure in the atria, however, is low, and the right atrium is the most “distensible” chamber of the heart. Therefore, while increasing in size, it copes with the load for the time being (usually up to 12-15 years, and sometimes more) quite easily. High pulmonary hypertension, which causes irreversible changes in the pulmonary vessels, never occurs in patients with ASD.

Newborns and infants, and children too early age The vast majority grow and develop absolutely normally. Parents may notice their tendency to frequent colds, sometimes ending in pneumonia, which should be alarming. Often these children, in 2/3 of cases girls, grow up pale, thin and somewhat different from their healthy peers. They try to avoid physical activity as much as possible, which in the family can be explained by their natural laziness and reluctance to tire themselves out.

Heart complaints can and, as a rule, appear in adolescence, and often after 20 years. Usually these are complaints about “interruptions” heart rate which a person feels. Over time, they become more frequent, and sometimes lead to the fact that the patient becomes incapable of normal, ordinary physical activity. This does not always happen: G.E. Falkowski once had to operate on a patient aged 60, a professional driver, with a huge atrial septal defect, but this is an exception to the rule.

To avoid such a “natural” course of the defect, it is recommended to close the hole surgically. Unlike VSD, interatrial defect It will never overgrow on its own. Surgery for ASD is performed under conditions of artificial circulation, on open heart, and consists of suturing the hole or covering it with a patch. This patch is cut out from the heart sac - the pericardium - the sac surrounding the heart. The size of the patch depends on the size of the hole. It must be said that ASD closure was the first open heart surgery, and it was performed more than half a century ago.

Sometimes an atrial septal defect can be combined with an irregular, anomalous entry of one or two pulmonary veins into the right atrium instead of the left. Clinically, this does not manifest itself in any way, and is a finding when examining a child with a large defect. This does not complicate the operation: the patch is simply larger and is made in the form of a tunnel in the cavity of the right atrium, directing blood oxidized in the lungs to the left parts of the heart.

Today, except surgery in some cases it is possible to safely close the defect using X-ray surgery technology. Instead of suturing the defect or sewing in a patch, it is closed with a special umbrella-shaped device - an occluder, which is passed along the catheter in a folded form and opened, passing through the defect.

This is done in the X-ray surgery room, and we described everything related to such a procedure above when we touched on probing and angiography. Closing a defect with such a “non-surgical” method is not always possible and requires certain conditions: anatomical location of the hole, sufficient age of the child, etc. Of course, if they are present, this method is less traumatic than open-heart surgery. The patient is discharged after 2-3 days. However, it is not always feasible: for example, in the presence of abnormal vein drainage.

Today, both methods are widely used, and the results are excellent. In any case, the intervention is elective and not urgent. But you need to do it in early childhood, although it is possible earlier if the frequency of colds and, especially, pneumonia becomes frightening and threatens bronchial asthma, and the size of the heart increases. In general, the sooner the operation is performed, the faster baby and you will forget about it, but this does not mean that you should be in a particular hurry with this vice.

It is a pathological anastomosis between the right and left atria. It accounts for about 20% of all congenital heart defects. The defect can be located in various parts of the septum, have different shape and sizes. Conventionally, primary and secondary (high) atrial septal defects are distinguished. The primary defect (Fig. 5) occurs due to underdevelopment of the primordial septum during embryogenesis. Its distinctive feature is its localization - at the level of the fibrous ring of the atrioventricular valves. The lower wall of such a defect is the fibrous rings of the mitral and tricuspid valves. The defect is sometimes combined with splitting of the mitral or trcuspid valves and is a component of the so-called patent atrioventricular canal. A secondary defect is formed due to disturbances in the embryogenesis of the secondary septum. The lower edge of such a defect is the interatrial septum (Fig. 5).

Hemodynamic essence of the defect consists in the discharge of arterial blood from the left atrium to the right and mixing with venous blood, which causes hypervolemia of the pulmonary circulation, and subsequently the development of pulmonary hypertension. Pulmonary hypertension with atrial septal defect is very malignant in nature, since irreversible changes in the lungs develop quickly and early, leading to severe decompensation of the right heart. A common complication Such a defect is septic endocarditis.

Clinical picture and diagnosis. Usually complaints are associated with circulatory decompensation. Its severity depends on the severity of overload of the right sections and the development of pulmonary hypertension. Patients complain about fatigue, shortness of breath, easy susceptibility colds, especially in the early childhood. On auscultation, an atrial septal defect is manifested by a gentle systolic murmur with its epicenter over the pulmonary artery. The murmur is caused by relative stenosis of the base of the pulmonary trunk, through which excess blood flows. The second sound above the pulmonary artery is intensified and often split. The ECG shows signs of overload of the right heart with hypertrophy of the right ventricle and atrium. Quite often, incomplete or complete blockade of the right bundle branch is detected.

At x-ray examination the enlargement of the heart is determined due to the right atrium, ventricle and trunk of the pulmonary artery. There is also increased pulsation of the roots of the lungs and an increase in the general vascular pattern of the lung tissue.

Using an echocardiographic study, it is possible to visualize the atrial septal defect, clarify its nature (primary or secondary), and assess the direction of discharge through the defect (Fig. 6, 7).

Probing of the heart reveals increased pressure in the right atrium, right ventricle and pulmonary trunk. The probe can pass from the right atrium to the left. Contrast agent, introduced into the left atrium, enters the right atrium through the atrial septal defect and then into the pulmonary circulation. Based on X-ray data, it is possible to calculate the volume of blood discharge, determine the location and size of the defect. Life expectancy with an atrial septal defect is on average about 25 years.

Treatment only operational. Open heart surgery is mainly performed under conditions of artificial circulation or hypothermia (general or craniocerebral). Secondary medium-sized defects can be sutured (Fig. 8). Large secondary and all primary defects are usually closed using plastic materials (auto- or xenopericardium, synthetic fabrics) (Fig. 9). In the last decade, a technique for transcatheter closure of atrial septal defect using “button devices” has been developed. The essence of the method is to deliver and install two button-shaped disk structures into the defect area under X-ray control using special probe instruments. Moreover, one of the structures (the so-called counter-occluder) is installed from the right atrium, and the other (occluder) is installed from the left atrium. The structures are connected to each other in the area of the defect using a special nylon loop and block the communication between the atria.

Good results from the operation can only be obtained if it is performed in early childhood. Performing the operation in more late dates does not allow achieving comprehensive rehabilitation of patients due to the formation of secondary morphological changes in the lungs, myocardium and liver. Moreover, the development of pulmonary hypertension with right-to-left shunting is a contraindication to surgical treatment.

Atrial septal defects account for 30% of all cases of congenital heart defects in adults. The magnitude and direction of shunt are determined by the size of the defect and the relative compliance of the ventricles. In most adults, the right ventricle is more compliant than the left; as a result, discharge occurs from the left atrium to the right. A small shunt leads to a moderate volume overload of the right heart, and pulmonary artery pressure remains normal. The severity of pulmonary hypertension may be insignificant even with a large discharge. Only in rare cases does severe pulmonary hypertension, leading to right ventricular failure (liver enlargement, ascites) and right-to-left shunting (cyanosis, symptom drumsticks, paradoxical emboli). Unlike ventricular septal defects, in which significant shunt results in volume overload in both ventricles, with atrial septal defects the shunt is smaller and affects only the right side of the heart.

1. Medication. In case of uncomplicated atrial septal defects such as ostium secundum, prophylaxis of infective endocarditis is usually not carried out. On the contrary, with defects of the interatrial septum such as ostium primum and large defects type sinus venosus, antibiotics are prescribed before and for 6 months after uncomplicated surgical correction. For right ventricular failure, diuretics are prescribed.

2. Surgical. Surgical closure (single suture or patch) is indicated for QP/QS ≤ 1.5:1, even in the absence of symptoms. This prevents pulmonary hypertension and right ventricular failure and reduces the risk of infective endocarditis. Surgical closure is ineffective in severe pulmonary hypertension when the ratio of pulmonary vascular resistance to peripheral vascular resistance is 0.9. Complications: for uncomplicated defects of the ostium secundum type, perioperative mortality is less than 1%, it is slightly higher for defects of the ostium primum type, which also require prosthetics or plastic surgery mitral valve. Postoperative complications include dysfunction sinus node(after correction of sinus venosus type defects) and complete AV block (after correction of ostium primum type defects). If there is mitral regurgitation before surgery, symptoms may worsen after correction of the atrial septal defect, since shunt to the right atrium is blocked, and mitral regurgitation remains. Atrial fibrillation, which arose before the operation, as a rule, persists after it.

Atrial septal defect (ASD)– any hole in the interatrial septum, except for the patent foramen ovale (PFO).

According to the classification, ASDs are divided into 3 main groups:
- primary atrial septal defect
- secondary atrial septal defect
- sinus venosus defect

Primary defect– a defect of the membranous septum and is included in a group of defects called atrioventricular communication (AVC).
Venous sinus defect– atrial septal defect at the site of introduction of the vena cava, almost always accompanied by partial anomalous drainage of the right pulmonary veins.
Secondary atrial septal defect– defect of the primordial septum, depending on the location it can be: central, anterior, posterior, lower, upper. Two or more defects are called multiple atrial septal defects.

EchoCG (B-mode and B-mode with color flow) of secondary ASD.

Why is it necessary to operate on an ASD?

ASD leads to overload of the pulmonary circulation due to the additional volume of blood flowing from the left atrium to the right. Next, additional blood volume enters the right ventricle and then into the pulmonary artery, pulmonary veins and again into the left atrium. Thus, a constantly circulating ballast volume of blood is formed, overload of the right parts of the heart, pulmonary hypervolemia develops, ultimately leading to heart failure (HF) and pulmonary hypertension.

When should ASD surgery be performed?

The presence of pulmonary hypervolemia (Qp/Qs more than 1.5:1.0) will already be an indication for surgery. Such children often suffer from colds and may lag behind in physical development from peers. Like almost all congenital heart defects, it is advisable to operate this defect during the first year of the child’s life. In the presence of a clinical picture of progressive heart failure and pulmonary hypertension, indications for surgery may be urgent, regardless of age.
At older ages, the risk of ASD closure increases due to the development of high pulmonary hypertension and heart failure.

Clinical manifestations of ASD defect.

In children with small ASDs long time There may be no clinical signs of heart failure; congenital heart disease itself is often detected by chance during a routine examination or during examination for another disease. With large ASDs, heart failure develops quite quickly and already by 12 months. Pulmonary hypertension may develop.
Children with ASD are more susceptible to frequent colds; during examination, it is important to pay attention to the presence of shortness of breath and retardation in physical development. On auscultation, an accent of 2 tones is heard over the pulmonary artery.
According to radiography, there is an increase in the pulmonary pattern, an increase in the size of the heart ( SLK).
According to the ECG, there is a deviation of the EOS to the right, hypertrophy of the right heart.
EchoCG allows you to assess the anatomy of the defect, identify enlargement of the right heart, and assess the degree of pulmonary hypervolemia ( Qp/Qs), pulmonary hypertension (PHP), as well as identify concomitant heart defects.

ASD or LLC.

LLC - fetal communication, necessary for normal blood supply to the fetus. After birth, the LLC closes, but for 20% of people it remains open throughout life. PFO does not lead to circulatory impairment and requires closure only for certain indications (paradoxical thromboembolism).
EchoCG signs of LLC - a small defect in the central part of the MPP (usually 4-6 mm), the LLC valve is often visualized; Qp/Qs does not exceed 1.5:1.0, pulmonary hypertension is not recorded. Neither according to ECG data, nor according to X-ray data chest no changes are detected.
The presence of LLC is not congenital heart disease and does not require monitoring by a cardiologist at the Federal Center for Cardiovascular Surgery.

Methods for closing an ASD.

Today, both open surgery and minimally invasive interventions using occluding devices are possible. Indications for a particular type of intervention are determined individually by a cardiologist at an appointment at the clinic of the Federal Center for Cardiovascular Surgery. For minimally invasive procedures, it is necessary to have sufficient atrial septal margins to secure the occlusion device.

In patients with ASD and unclear edges of the interatrial septum, as well as in the presence of concomitant pathology that needs correction, open surgery is performed under artificial circulation. The ASD is closed with a patch from the autopericardium or xenopericardium.

The age of patients with ASD treated in our center starts from 6 months. During the 2011 work of the Federal Center for Cardiovascular Surgery, 103 children were treated with ASD, of which 18 were children with ASD in combination with abnormal drainage of the pulmonary veins and as part of a VKA. In 26 of them, minimally invasive interventions were used (endovascular closure of the ASD with an occluding device). The duration of hospital stay after open interventions is 5-7 days, after endovascular interventions 3-4 days.

Among all heart defects, atrial septal defect is most often determined. This anomaly is often combined with damage to the interventricular septum. Up to 80% of this defect heals in the first years of a child’s life, so ASD takes the leading place in the frequency of distribution among congenital heart defects.

An atrial septal defect (ASD) is a disruption of the structure of the wall between the two upper chambers of the heart (atria), most often presented as a hole. The condition is often discovered at birth, so the anomaly is considered congenital. It can manifest itself both in childhood (in newborns) and in adults. In every individual case The most appropriate treatment tactics or monitoring of the patient’s condition is considered.

The disease may not require surgical treatment, but is often recommended surgery, since otherwise the risk of developing complications that can lead to death increases.

Before surgery, patients must undergo comprehensive examination. The location of the defect is carefully studied, other violations and their severity are determined. Equally important after surgery is rehabilitation period. At correct execution of all actions, the prognostic conclusion is most often favorable.

Video ASD or atrial septal defect: causes, symptoms, diagnosis and treatment of ASD

What is an ASD?

Among congenital heart defects, the most common type is atrial septal defect. Close attention to the disease began to be paid in 1900, when Bedford created detailed description diseases. Additions were made by Pappa and Parkinson in 1941, and then physical, electrocardiographic, and radiological diagnostics.

ASD is often associated with other congenital lesions - patent ductus arteriosus, anomaly of the interventricular septum, pulmonary valve stenosis, transposition of the trunks of the great arteries. It is often combined with defects of the ventricular septum, ductus arteriosus, and rare venous anomalies during emptying of the pulmonary veins. Thus, atrial septal defect can accompany almost any congenital lesion, but it often appears in an isolated form.

Some statistics:

The interatrial septum is completely absent in 3% of cases.
Ostium secundum type defect: the most common type of ASD and accounts for 75% of all cases of pathology, this is approximately 7% of all congenital heart defects and 30-40% of such anomalies in patients over 40 years of age.
Ostium primum defect: the second most common type of defect and accounts for 15-20% of all cases.
Sinus venosus defect: the least common of the three variants of ASD and is observed in 5-10% of all developmental anomalies.
The ratio of the incidence of ASD between women and men is approximately 2:1.
By age 40, 90% of untreated patients have symptoms of shortness of breath, fatigue, palpitations, prolonged arrhythmias, or even signs of heart failure.

Pathogenesis

IN in good condition the heart is divided into four hollow chambers: two on the right and two on the left. To pump blood throughout the body, the heart uses the left and right sides to perform various tasks. The right half moves blood towards the lungs through the pulmonary arteries. In the alveoli, the blood is saturated with oxygen, after which it returns to the left half of the heart through the pulmonary veins. The left atrium and ventricle pump blood through the aorta, directing it to the rest of the organ and tissues.

When an atrial septal defect is present, oxygenated blood flows from the left upper chamber of the heart (left atrium) to the right upper chamber of the heart (right atrium). There it mixes with oxygen-free blood and is pumped back into the lungs, although it was already saturated with oxygen before.

In a large atrial septal defect, this extra volume of blood can fill the lungs and overload the right side of the heart. Therefore, in the absence of disease, the right side of the heart will eventually hypertrophy and weaken. If this process continues further, arterial pressure in the lungs increases markedly, which immediately leads to pulmonary hypertension.

Causes

Basically, all predisposing risk factors and causes of ASD are associated with intrauterine development fetus In some circumstances, there is a particularly high chance of having a baby with a congenital heart defect.

Rubella ( infection) . Infection with the rubella virus during the first few months of pregnancy may increase the risk of fetal heart defects, including ASD.
Drug addiction, smoking or alcoholism, and exposure to certain substances. Using certain medications, tobacco, alcohol, or drugs such as cocaine during pregnancy can harm the developing fetus.
Diabetes mellitus or systemic lupus erythematosus. If such diseases are noted (especially in a pregnant woman), then the chances of having a child with a heart defect increase.
Obesity. Excess body weight, such as obesity, may play a role in increasing the risk of having a child with an atrial septal defect.
Phenylketonuria (PKU). If a woman has this condition, she may be more likely to have a child with a serious defect.

Clinic

Many children born with atrial septal defects have no detectable characteristic features. In adults, symptoms may begin around age 30 or older.

The symptom complex for atrial septal defect may include:

shortness of breath, especially during exercise;
fatigue;
fast or irregular heartbeat;
stroke.

When should you see a doctor?

You should not delay visiting your doctor if a child or adult has any of the following signs:

intermittent breathing;
swelling of the legs, arms or abdomen;
fatigue, especially after exercise;
rapid heartbeat or extrasystoles.

Most often this indicates the presence of heart failure or other complications arising from congenital disease hearts.

Complications

A minor atrial septal defect most often does not lead to serious violations hemodynamics. Small ASDs generally close on their own during infancy.

Large MPP defects often cause serious violations:

Right-sided heart failure
Heart rhythm disorders (arrhythmias)
Increased risk of stroke
Premature wear of the heart muscle

Less common serious complications:

Pulmonary hypertension. If a large atrial septal defect is left untreated, increased blood flow to the lungs increases blood pressure in the pulmonary arteries, resulting in the development of a complication such as pulmonary hypertension.
Eisenmenger syndrome. Long-term pulmonary hypertension can cause permanent lung damage. This complication usually develops over many years and occurs in people with large atrial septal anomalies.

Timely treatment can prevent or help manage many of these complications.

ASD and pregnancy

Most women with an atrial septal defect can tolerate pregnancy without any problems. But if there is a large defect or concomitant diseases type of heart failure, arrhythmia or pulmonary hypertension, the risk of complications during pregnancy increases significantly.

Doctors strongly advise women with Eisenmenger syndrome not to conceive a child because it could put both of their lives at risk.

The risk of congenital heart disease is higher in children whose parents have congenital heart defects, either the father or mother. Anyone with a congenital heart defect, repaired or not, who is considering starting a family should discuss it carefully in advance with their doctor. Some medications may need to be removed from the prescription or adjusted before pregnancy occurs because they may cause serious problems in a developing fetus.

Video Live great! Atrial septal defect

Diagnostics

If ASD big size, the doctor, during auscultation of the patient’s heart, can hear pathological heart murmur. With small defects it may be faintly audible. Because many people with uncorrected ASDs do not have severe symptoms, pathology can be detected in adolescence or already into an adult.

The most common diagnostic test used to confirm an ASD is an echocardiogram (ECG) or ultrasonography hearts.

Other tests that the doctor may prescribe for the patient are the following:

Chest X-ray
Electrocardiogram (ECG)
Cardiac magnetic resonance imaging (MRI)
Transesophageal echocardiography
Cardiac catheterization

Treatment

If a small ASD is identified, closure is usually not recommended. However, if the patient exhibits symptoms such as fatigue, difficulty breathing, pre-stroke condition, atrial fibrillation, or if the right ventricle increases in size, then the possibility of reconstructive plastic surgery is considered.

If the patient has severe pulmonary hypertension due to Eisenmenger syndrome, plastic reconstruction of the defect is not recommended.

In 1953, Dr. John H. Gibbon successfully closed the MPP defect using open heart surgery and a cardiopulmonary device. Since then, ASDs have been repaired using different materials And surgical methods. Until the early 1990s, all ASDs were closed by open surgery on the heart. Today, the method of choice is to close the hole through cardiac catheterization. If the size of the defect is too large or the patient has any other types of congenital defects, open surgery is recommended.

At early diagnosis and restoration of ASD, the result is usually excellent. In such cases, a good long-term outcome is expected, especially if the defect was diagnosed early and closed in adulthood or the patient is determined to have normal pressure in the pulmonary arteries. However, there is often a risk of developing atrial arrhythmias due to scars in the closure area. In a small number of cases it may be necessary reoperation. Other risks that may occur later include the possibility of developing heart failure or hypertension.

Depending on whether the patient had ASD specific treatment or not, the following recommendations are given:

All adults who have an unrepaired ASD should be followed regularly throughout their lives by a congenital heart specialist.
Patients whose defect was closed in childhood or adulthood require periodic heart checks; which should be taken at least once a year in a profiled medical center. This is necessary for correct diagnosis and assessment of the effectiveness of previous treatment.
Further need for follow-up care will be determined by the treating cardiologist specializing in congenital defects hearts.

Forecast

Adults with small ASDs that do not affect heart function usually do not require treatment. In such cases, it is still important to see a doctor at least once a year to make sure nothing has changed. On the other hand, if the hole is too large and blood flows from the left side of the heart into right side, then the prognosis worsens. This is because excess blood enters the lungs, causing the heart and lungs to work harder and less efficiently. When this happens, there is a high risk of developing other diseases of the heart, lungs and circulatory system.

The prognosis for ASD worsens if the following complications are identified:

The right side of the heart is enlarged, which can cause heart failure
Irregular and rapid heartbeats such as fibrillation, especially in the atria
Stroke
Damage to the pulmonary arteries
Eisenmenger syndrome
Damage to the tricuspid and mitral valves

Prevention

Most often, the development of an atrial septal defect cannot be prevented. If you are planning a pregnancy, then you should first consult your doctor. This visit should include:

Testing for the presence of immunity to rubella. If susceptibility to the virus is determined, then vaccination is carried out.
Analysis general condition health and identification of medications taken. Certain health problems will need to be closely monitored during pregnancy if necessary. Your doctor may also recommend adjusting your dose or eliminating certain medications that may harm your pregnancy.
Heredity analysis. If you have a family history of heart defects or other genetic disorders, you should consider seeking genetic counseling to help determine your risks for a future pregnancy.

Video Ventricular septal defect