Hypoplasia of the left and right heart includes a complex of manifestations of underdevelopment of the left or right calving of the heart. They can include stenosis of the mitral or aortic valve, arthrosis, as well as aortic ascending hypoplasia. The combination of these disorders also refers to this type of lesion of the cardiac system. The syndrome occurs in 5-7% of cases of detection of congenital heart diseases.
Features of the disease course
This pathology can begin to manifest itself during the closing of the aortic valve. Manifestations of the disease may differ to some extent in the newborn child and in the fetus. Consider these differences in more detail.
Scheme of hypoplasia of the left heart
In the fetus
The presence of congenital heart disease in the form of underdevelopment of the left or right heart can be detected even in the fetal stage. This makes it possible to significantly reduce the number of possible complications after the birth of a child with this developmental anomaly, as well as provide the necessary care for a newborn with such an anomaly of the heart.
This underdevelopment of the right or left ventricle of the heart is revealed during Echocardiography of a pregnant woman.
In newborns
It is at this time that the newborn can manifest symptoms of this pathological condition. These include the following symptoms:
- faint heartbeat and uneven heart rate;
- tachypnea;
- sheer pallor of the skin, her cyanosis;
- hypothermia.
You can find out how hypoplasia of the left heart looks like from the following video:
For prevention and treatment of cardiovascular diseases, our readers advise the drug "NORMALIFE".This is a natural remedy that affects the cause of the disease, completely preventing the risk of getting a heart attack or stroke. NORMALIFE has no contraindications and begins to act within a few hours after its use. The effectiveness and safety of the drug has been repeatedly proven by clinical studies and long-term therapeutic experience.
The opinion of doctors. .. & gt; & gt;
Types and forms of
Today, there is a classification of the syndrome of left ventricular maldevelopment in two morphological types of this pathology:
- includes the pathology of the underdevelopment of the left heart ventricle in combination with atrial aortic atresia, and mitral valve atresia is also observed. As a result, the size of the left ventricle is greatly altered and it is a slotted hole, its volume is less than 1 ml. This type of hypoplasia syndrome is the most severe;
- , the second species is a less severe condition, manifested in left ventricular hypoplasia, which develops against the background of cardiac aortic stenosis and the ventricular volume is much larger - about 1.5-4.5 ml. The second version of the syndrome of hypoplasia is considered the most common.
Both types of left ventricular and right ventricular hypoplasia syndrome discussed above are accompanied by an open oval window, as well as a significant extension of the right heart. Right ventricular myocardium can be observed.
What are the causes and progression of hypoplasia of the left heart in the fetus and the newborn?
Causes of
To date, the true causes of the onset of the examined heart disease have not been fully revealed.
However, the most common reasons for manifesting such a pathology include the following:
- genetic predisposition - this includes types such as autosomal recessive, polygenic and autosomal dominant types of inheritance;
- there is also a theory of the multifactorial reason for the appearance of a defect.
Symptoms of
Manifestations of the hypoplasia syndrome of the right or left heart are most often detected within the first 24 hours after the birth of the child. The manifestations of this pathology are in many respects similar to the symptoms of respiratory distress syndrome or cardiogenic shock:
- respiratory weakness;
- marked pallor of the skin;
- heart failure;
- in the lungs are observed wheezing;
- in newborns has a grayish skin tone;
- the limbs of the patient become cold;
- cyanosis of the skin at birth is negligible, however, since the time on the skin appear nodules;
- hyperthermia can be observed in the lower part of the trunk.
After birth, the baby's liver gradually grows in size, wheezing in the lungs is heard more clearly, peripheral edema occurs. When the arterial duct is suddenly closed, the child quickly dies.
Diagnosis of
Detection of cardiac vascular defects can be made already with intrauterine development. This facilitates the course of the disease after birth and allows you to prepare for the necessary treatment activities.
- To diagnose left and right heart hypoplasia syndrome, a number of studies are being carried out, the results of which are confirmed by two-dimensional echocardiography. Carrying out a cardiac catheterization is appointed when clarifying the features of the anatomical structure of the heart before performing a surgical procedure.
- Chest radiography is also performed, which allows to detect changes in the pulmonary pattern and the degree of cardiomegaly.
- In the presence of hypoplasia of the right ventricle, a differentiated study is necessary, which will reveal the heart lesions and more accurately establish the diagnosis.
Surgical and medicamentous treatment of left heart hypoplasia syndrome
This pathology of the cardiac system as a therapeutic treatment can be applied to newborns and directed such treatment to preventing the closure of the arterial duct, as well as to attempt to open it. For these purposes, infusion of prostaglandin E is used. Inotropic drugs and various kinds of diuretics are introduced.
The most effective method of treatment will be surgical intervention, which is carried out in stages. The scheme of the operation is as follows:
- The first stage involves carrying out the Norwood operation, which is carried out during the first two weeks of life. It is necessary to reduce the degree of stress on the arteries of the heart and improves blood supply to the aorta;
- in the second stage carried out the operation Gemi-Fontena, which takes place three months after the birth of the child;
- , the third stage involves correction of the heart defect and is performed about a year after the birth of the child - Fontaine's operation.
On how doctors perform surgery for hypoplasia of the left heart in the fetus, the following video story will tell:
Prevention of
All people who have this pathology should carry out endocarditis prophylaxis - it is performed before any surgical intervention and dental visit.
It is also necessary to conduct a permanent examination with cardiologists, this avoids relapse.
Complications of
A significant percentage of deaths is the main complication of this cardiac pathology. And the more at an earlier stage the pathology is revealed, the more possibilities for its successful treatment.
Forecast
Survival of patients is an indicator of the success of treatment. Since heart transplantation is the most effective method of treatment, and donor search is very difficult, many patients do not wait their turn and die. The first month of life is characterized by the death of 90% of newborns with this pathology, but after the first stage of correction, the survival rate increases to 75%.
Five-year survival after surgical correction is about 70% of the total number of children with left and right heart hypoplasia syndrome.