In cardiological practice, cases of pulmonary stenosis in children are very often recorded. This is a common pathology, which is characterized by a narrowing of the duct of the pulmonary artery, which leads to disruption of the normal blood flow and overload of the right heart. In most cases, this ailment is congenital and is sometimes diagnosed in combination with other defects of the cardiovascular system.
Of course, many newly made parents are looking for any available information. Why does pulmonary stenosis develop in children? What are the first symptoms of the disease? What forms can pathology take? What can a cardiologist offer in terms of treatment? Is drug therapy possible? The answers to these questions are worth exploring.
General description of the pathology
Before considering the causes, symptoms and effective methods of therapy, it is worth examining general information about stenosis. The pulmonary arteries are paired, fairly large branches of the pulmonary trunk, which departs from the right ventricle.
The main function of this vessel is the transport of venous (oxygen-poor) blood from the right atrium to the capillary system of the lungs, where gas exchange occurs and the blood is saturated with oxygen. In fact, here begins a small circle of blood circulation. At the exit of the pulmonary trunk from the right atrium, there is a tricuspid valve, which regulates the flow of blood.
Pulmonary artery stenosis is spoken of if there is a narrowing of the vessel itself or a malfunction of the valve (for example, if the valve leaves are fused together, the blood cannot circulate normally). Violation of blood flow adversely affects the condition of the whole organism. First of all, the heart suffers - the right parts of the myocardium are often hypertrophied in order to compensate for problems with the release of blood and to cope with high pressure in the right ventricle.
According to statistics, this pathology in most cases is congenital. Timely diagnosis and correctly conducted therapy can avoid possible complications.
Causes of Stenosis
Pulmonary stenosis in children can develop under the influence of various factors. Unfortunately, the exact mechanisms of the development of the disease have not yet been fully studied. Nevertheless, a list of prerequisites is worth a look.
- An important role is played by genetic heredity. The risk of developing stenosis is increased if the child's parents or close relatives have been diagnosed with congenital heart defects.
- The presence of metabolic and systemic pathologies in the mother.
- Infectious diseases (especially those of viral origin) carried by a woman in early pregnancy are potentially dangerous.
- The development of stenosis can lead to the use of drugs with teratogenic properties.
- The risk of this pathology increases with multiple pregnancy.
- Various chromosomal diseases are dangerous.
- Risk factors include impaired fetoplacental blood flow.
- The likelihood of developing such congenital malformations in a child increases if the mother is over 38 years old and this is her first pregnancy.
Often the development of stenosis is associated with exposure to several unfavorable factors.
Varieties of stenosis
Depending on the place of narrowing of the vessel, several types of pathology are distinguished.
- Nadklapanny stenosis of the pulmonary artery in children is accompanied by a slight narrowing of the pulmonary artery, the formation of an incomplete or complete membrane in its lumen. In addition, the formation of multiple peripheral narrowing of the pulmonary artery is possible.
- Pulmonary valve stenosis in children is a very common pathology. In this case, the valve flaps are partially fused. The valve itself takes on a dome shape with a small hole in the middle. Of course, such a structure makes normal blood circulation impossible.
- Subvalvular stenosis of the pulmonary artery in children is often associated with various defects of the septum between the ventricles of the heart. The pathology is characterized by a funnel-shaped outgoing part of the right ventricle or an incorrect arrangement of the muscle bundle, which disrupts the release of blood into the pulmonary circulation.
Stages of the development of pathology
It is customary to distinguish four stages of the development of this disease.
- The first stage, or moderate stenosis of the pulmonary artery, in a child is rarely accompanied by any noticeable external symptoms. There is a slight overload of the right ventricle, which can be noticed during the ECG. The kid, as a rule, feels quite normal.
- The second stage is a pronounced form of the disease, which is already accompanied by noticeable disorders.
- The third stage, or severe stenosis, is characterized by a severe deterioration in the condition of a small patient. Pulmonary valve pressure rises. You may notice signs of a serious circulatory disorder.
- The decompensation stage is accompanied by a severe violation of the blood flow. Right ventricular failure develops, dystrophic processes in the myocardial tissues are triggered.
Pulmonary stenosis in children: symptoms
Of course, the symptoms of the disease directly depend on the stage of its development. In the initial stages, symptoms may be completely absent. But as the pathology progresses, the first sign appears - shortness of breath. First, breathing problems appear during physical activity, and then at rest.
The child quickly gets tired, suffers from weakness and dizziness. A small patient becomes drowsy. Sometimes you can notice a strong swelling and a pronounced pulsation of the veins on the neck. Older children sometimes complain of a burning sensation and chest pain. The child often loses consciousness. The doctor may hear a heart murmur during auscultation. More serious circulatory disorders are accompanied by pallor - the skin often acquires a bluish tint (the color is most pronounced in the region of the nasolabial triangle).
Pulmonary stenosis in children: how dangerous is the pathology? Possible complications
This is a very dangerous pathology, which in no case can be ignored. A child with stenosis is more prone to the development of various inflammatory diseases of the respiratory tract.
Violation of the blood flow leads to a gradual myocardial dystrophy, which can result in a heart attack, stroke, severe insufficiency of the right ventricle. The most dangerous complication of isolated pulmonary stenosis in children is septic endocarditis. That is why a child with such a diagnosis should always be under vigilant supervision.
Diagnostic measures
As a rule, such a disease is diagnosed immediately after the birth of the baby or in the first months of life. During examination in the second intercostal space (on the left side of the sternum) systolic murmur is heard .
An obligatory part of the diagnosis is an electrocardiogram. If we are talking about the initial stage of development of the ailment, then some changes may be absent. However, in most cases, during the procedure, signs of right ventricular hypertrophy can be detected. Occasionally, the appearance of supraventricular arrhythmias is observed.
Echocardiography is also informative. During the procedure, the doctor may notice expansion of the pulmonary artery and dilatation of the right ventricle of the heart. Sometimes Dopplerography is additionally performed - this procedure allows you to determine the pressure difference between the right gastric and pulmonary trunk.
Diagnosis necessarily includes chest x-ray. In the picture you can see the expansion of the trunk of the pulmonary artery. A very characteristic symptom is the impoverishment of the pulmonary pattern.
Is medication possible?
The treatment regimen is selected individually, since much here depends on the stage of development of the pathology. If the narrowing is small and blood flow is not impaired, then special treatment may not be required - sometimes a mild degree of stenosis goes away on its own with age.
However, surgery is the only way to correct the narrowing. Drug therapy is carried out only as a preparation for surgery. In particular, antibiotics are prescribed for small patients to protect against bacterial infection and prevent septic endocarditis.
When is surgery necessary?
Surgical treatment of pulmonary artery stenosis in children can be performed in different ways - in this case, the location of the narrowed area is of great importance.
The most effective and efficient procedure is endovascular balloon valvuloplasty. A special balloon is introduced through the venous wall into the affected area of the vessels. Using a catheter, air is pumped into the balloon, as a result of which the device inflates and expands the walls of the vessel, thereby eliminating the narrowing.
Open valvuloplasty is much less common. In this case, the patient needs to be connected to a heart-lung machine. The doctor needs direct access to the heart and blood vessels in order to carefully examine the affected area and conduct excision of the fused commissures.
Sometimes a small patient needs a systemic pulmonary bypass. If supravalvular stenosis occurs, then reconstruction is performed using a patch from the patient’s own tissues. In some cases, the installation of a prosthesis is required.
In most cases, surgery is performed when the child reaches 5-10 years of age. Nevertheless, in the presence of severe stenosis, surgery can be prescribed in infancy. In most cases, the procedure ends with success. Nevertheless, even after successful correction of stenosis, the child (and then the adult patient) should regularly undergo examinations by a cardiologist and carefully monitor the state of health.
Prevention: how to avoid danger?
Unfortunately, specific prophylaxis does not exist, since the development of an ailment is associated with factors that can not always be affected. It is very important for the expectant mother to monitor the course of pregnancy, maintain the normal functioning of the body, eat properly, avoid contact with infectious patients and not take any medications without the permission of a doctor. Of course, you need to regularly undergo scheduled examinations and ultrasound - this makes it possible to diagnose certain pathologies in the fetus even before its birth.
Predictions for small patients
Valvular stenosis of the pulmonary artery in a child (as well as any other form of narrowing) is a pathology that in no case should be ignored. Fortunately, timely diagnosis allows doctors to detect the disease in time and take appropriate measures.
Minimal narrowing of the pulmonary artery practically does not affect the quality of life or the development of the baby. Nevertheless, children with such a diagnosis should be registered with a cardiologist and periodically undergo comprehensive examinations. If we are talking about valve stenosis of the pulmonary artery in a child, then there is a possibility that in adulthood the patient will need surgery to replace the valve.