Isolated pulmonary stenosis in children

To detect pulmonary stenosis, imaging research methods are shown:

  • Ultrasound of the heart (echocardiography);
  • radiography;
  • ECG;
  • dopplerometry;
  • sounding.

Depending on the type of disease, the doctor prescribes treatment, but in any form and degree it will be surgical. However, medications can be prescribed as part of complex supportive care. Most often, with a congenital form, surgery is performed at the age of 5-10 years. In the first or second degree, when the symptoms are mild, there is no need for an operation at an earlier date. Only in severe cases, surgery is indicated in the first days after the birth of the baby.

Surgery should be performed immediately if an indicator such as the pressure gradient between the ventricle and artery exceeds 50 mm. Hg. Art.

Pulmonary stenosis in children is one of the common diseases associated with heart and vascular defects. The main measure of prevention of its occurrence is the normal course of pregnancy and minimization of harmful effects at this time.

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.

The only effective way to treat ALS is surgery – to remove the narrowing. The operation is indicated for patients with moderate and critical ALS. Minimal narrowing of the vessel does not require surgical intervention and in some cases it can go spontaneously.

The type of operation is selected depending on the severity of the patient’s condition. In severe cases, the operation is performed in the first months of life. The average degree of stenosis undergoes surgical correction after the patient reaches the age of 5-10 years.

With isolated valve ALS, different variations of valvuloplasty have proven themselves well.

Stenoz javljaetsja prichinoj pereboev v transportirovke krovi v legkie - Isolated pulmonary stenosis in children

In most cases, endovascular balloon valvuloplasty is performed. The essence of the method is the introduction of an inflatable balloon into the stenotic area, into which air is pumped through the catheter. The expanding balloon mechanically expands the narrowed portion.

Less commonly used is open valvuloplasty, in which the surgeon dissects the fused commissures. Typically, an open surgery is performed when balloon valvuloplasty is ineffective. Sometimes patients with a critical stage of pathology undergo balloon atrioseptostomy. Some forms of ALS require surgical correction in the form of systemic pulmonary bypass grafting.

With supravalvular stenosis in the narrowing area, reconstruction is performed using a patch from the patient’s own tissue or by installing a xenopericardial prosthesis. Tactics for the treatment of subvalvular stenosis involves the removal of a narrowed section of the artery.

If the patient has a large shunt from left to right, for example, an open ductus arteriosus or interventricular septal defect, and suffers from respiratory distress, diuresis is effective in reducing cyanosis secondary to pulmonary edema.

Oxygen should be prescribed to any patient with cyanosis in respiratory failure. The use of oxygen can reduce pulmonary artery pressure in patients with reactive pulmonary vascular system, thereby increasing pulmonary blood flow.

Treatment depends on the degree of stenosis. Thus, clinical assessment as well as imaging will be an immediate guide. Cardiology or cardiothoracic consultation is necessary if visual examinations show obstruction of the right outflow.

It is necessary to pay attention to the following:

Severe stenosis, peak gradient 60 mmHg. Art. and more, requires emergency valve dilatation with balloon valvotomy.

Patients with mild stenosis of lung valves usually do not need treatment.

Patients with severe or symptomatic infundibular or supravalvular lung stenosis require immediate intervention. Valvulotomy is the standard of care for these patients.

Often cyanotic children with respiratory distress and hypotension/shock are screened in the same way as in patients with sepsis.

In the second Natural-historical study of congenital heart defects, 592 patients with pulmonary valvular stenosis were examined for 10 688 person-years; only one patient had an episode of bacterial endocarditis.

Prevention of endocarditis within 6 months after restoration of congenital heart disease is recommended. In addition, prevention is required for life for people who have valve prostheses.

Intervention with balloon angioplasty or valve repair is indicated for patients with peak valve gradients greater than 50 mm Hg. Art. or patients with angina pectoris, fainting, shortness of breath during physical exertion or with a presyncope. Correction options include open heart surgery, balloon angioplasty, percutaneous stenting, percutaneous valve replacement, or percutaneous placement of the tubing.

Patients with severe or symptomatic infundibular or supravalvular lung stenosis require surgery.

Critical pulmonary valvular stenosis can occur with almost pulmonary atresia (cyanotic lesions) with a small and often inadequate right ventricle. These patients survive due to open ductus arteriosus. Atresia of the pulmonary valve or critical stenosis of the pulmonary valve with an inadequate right ventricle requires a shunt after the duct has been patented pharmacologically with prostaglandin E1.

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Final recovery may not be possible if the right ventricle is hypoplastic, requiring a single ventricular palliation, such as the Fontaine procedure, or variations, such as a direct appendage of the right atrium to the anastomosis of the main pulmonary artery. Often the trunk and branch pulmonary arteries need to be enlarged.

Pulmonary stenosis is a heart disease of congenital or acquired etiology, as a result of which the patient has a narrowing of the lumen of the pulmonary trunk, which is a factor in the development of circulatory disorders.

To understand the danger of a pathology, you need to have an idea of ​​its causes, the clinical picture, diagnostic methods, as well as the treatment and prognosis of the disease.

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Types and causes

Based on the level of circulatory disorders, doctors divide the defect into four types:

  1. Subvalvular type of stenosis, 5% of the total. With it, funnel-shaped changes in the outgoing structures of the right ventricle are observed due to abnormal proliferation of muscle and fibrous fibers.
  2. Nadklapanny stenosis is observed in 5-10% of patients. This is an underdevelopment or damage to the walls of the artery.
  3. Valve form of stenosis. It develops in 85% of cases. This is a condition in which valve flaps thicken, deform, and interfere with blood flow.
  4. Combined form. It is characterized by a combination of two or more types of stenosis with the development of severe clinical symptoms.

For practicality, a classification is used, which is based on an indicator of the pressure gradient between the artery and the cavity of the right ventricle, as well as systolic blood pressure in it:

  1. The first stage of stenosis is a moderate narrowing. In this case, the indicators of the pressure gradient and the level of systolic pressure are in the range of 20-30 mm RT. Art. and 60 mmHg. Art. respectively.
  2. The second stage of the pathology is severe stenosis of the lumen of the artery. The gradient level is 30-80 mm Hg. Art., and systolic blood pressure – 60-100 mm RT. Art.
  3. The third stage of the disease is severe stenosis. The pressure gradient level is more than 80 mm Hg. Art., and an indicator of systolic pressure of more than 100 mm RT. Art.
  4. At the fourth stage of the disease, decompensation processes develop. In this case, a decrease in the gradient and level of systolic pressure is observed as a result of the development of contractile type of insufficiency.

Congenital malformation

Among the etiological factors of CHD in newborns, doctors distinguish:

  • adverse conditions of life and work of a woman during pregnancy, affecting the health of the fetus;
  • the presence of a genetic predisposition to various defects in the structures of the cardiovascular system in the fetus;
  • future mother consuming alcoholic beverages, psychoactive and narcotic substances, antibacterial drugs;
  • diseases of viral etiology in a pregnant woman, among which rubella and infectious mononucleosis are considered the most dangerous;
  • exposure to x-ray and other types of ionizing radiation during the period of gestation.

Acquired defect

The main factors in the development of the acquired form of the disease are:

  • atherosclerotic lesions of the walls of the coronary arteries;
  • rheumatic heart valve disease;
  • the presence of calcification changes in the zone of valves of the pulmonary trunk and aorta;
  • hypertrophic cardiomyopathy;
  • compression of the artery with an increase in lymph nodes, the presence of malignant neoplasms and aneurysms;
  • foci of inflammation in the vascular walls that are caused by pathogens of tuberculosis infection and syphilis.

Symptoms

Stenosis of the mouth of the pulmonary artery can manifest itself in a diverse clinical picture, which depends on the stage of the disease.

With insignificant degrees of narrowing, signs of pathology are not noted.

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Children

The clinical picture of the disease in childhood is characterized by:

  • pallor of the skin or acrocyanosis – blue cheeks, lips, fingertips, shortness of breath, frequent pneumonia, a tendency to colds;
  • child lag in physical development;
  • fatigue;
  • pathological murmurs when listening to the heart;
  • fainting.

In adults

The most common signs of narrowing of the lumen in older people are:

  • frequent dizziness;
  • difficulty breathing;
  • general weakness;
  • hemodynamic impairment;
  • the presence of systolic tremor during auscultation;
  • compressive pain in the left side of the chest;
  • swelling of the lower extremities;
  • ascites;
  • anasarka.

Diagnostics

What can you do?

With a slight severity of narrowing of the pulmonary artery, special therapeutic measures are not carried out. Treatment is symptomatic. If the disease progresses, the degree of stenosis increases, the child is shown surgery. The most favorable age is considered to be from 5 to 10 years.

What does the doctor do?

Depending on the stage and type of stenosis, a treatment technique is selected. With valve stenosis, an operation is performed by the method of open or balloon valvuloplasty.

The supravalvular type of stenosis is treated by changing the area of ​​narrowing using a prosthesis or a patch. As a rule, the forecast is favorable.

The rehabilitation period for children is about three months. Two years after the operation, physical activity is allowed. Without treatment, stenosis of a significant form of development can lead to hypertrophy and heart failure from the right ventricle. This can be fatal.

If stenosis was detected in the fetus during ultrasound and Doppler ultrasound, do not despair. The operation can be performed after the birth of the baby, and he will be able to live a full life. Identified stenosis in the fetus will allow you to prepare in advance for the operation and take emergency measures in a timely manner.

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.

Whatever the form of stenosis, the disease can cause serious problems that affect the duration and quality of life. Timely diagnosis and surgery will help to avoid these problems.

What is the danger of pulmonary stenosis? This pathology is the cause of the development of myocardial dystrophy. Without treatment, narrowing of the lumen of the pulmonary artery can lead to consequences such as stroke, myocardial infarction, and right ventricular failure. Without surgery, a patient with stenosis dies in five years.

If your child has pulmonary stenosis, do not panic. Timely and adequate treatment will allow your baby to develop harmoniously along with peers and live a full life for many years.

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.

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.

Svetlana Borszavich

General practitioner, cardiologist, with active work in therapy, gastroenterology, cardiology, rheumatology, immunology with allergology.
Fluent in general clinical methods for the diagnosis and treatment of heart disease, as well as electrocardiography, echocardiography, monitoring of cholera on an ECG and daily monitoring of blood pressure.
The treatment complex developed by the author significantly helps with cerebrovascular injuries and metabolic disorders in the brain and vascular diseases: hypertension and complications caused by diabetes.
The author is a member of the European Society of Therapists, a regular participant in scientific conferences and congresses in the field of cardiology and general medicine. She has repeatedly participated in a research program at a private university in Japan in the field of reconstructive medicine.

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