Modern methods of the study of restrictive cardiomyopathy
The diagnosis of restrictive cardiomyopathy is rather rare, and this pathology is characterized by significant malfunctions in the work of one or both of the ventricles of the heart. At the same time, the amount of blood filling the ventricles decreases in the diastole, decreases without changing the size of the wall, which significantly complicates the course of the disease.
- Causes of pathology
- Signs of pathology
- Symptomatology of the disease
- Features of the course of cardiomyopathy in children
- Diagnosis of pathology
- Treatment of pathology
Causes of pathology
Cardiomyopathies are rare but serious diseases that develop as a result of the action of hereditary factors or as acquired pathologies. However, regardless of the cause, significant changes in the myocardium become manifestations.
Possible causes of cardiomyopathy:
- Non-infiltrative changes: scleroderma, diabetic CML, idiopathic RCMP, elastoma, hypertrophic or familial CMS;
- Changes in the infiltrative nature: amyloidosis, Gaucher's disease, sarcoidosis, fatty infiltration and Fabry's disease;
- Pathologies of accumulative character: glycogenosis and hemochromatosis;
- Diseases endomiokardialnogo nature: fibrosis, endocarditis, carcinoid, hypereosinophilic syndrome, restrictive heart syndrome, Leffler, metastatic lesion.
Less often cardiomyopathy can occur under the influence of radiation, as a result of toxic poisoning or after taking certain medications.
Signs of pathology
The most often restrictive form of pathology is manifested in the violation of the function of filling the left The ventricle, which occurs as a result of a pathological increase in the myocardium or fibrous formations in heart. With such violations in the cardiovascular system, the pressure on the right and left ventricle area increases significantly, which causes the heart to be heavily loaded during operation.
If you do not start treatment at an early stage of pathology, then soon, cardiomyopathy will go into pulmonary hypertension, and the symptoms of the disease will change significantly, which in turn will affect the complexity of diagnosis of the disease. In the process of progression, a restrictive cardiac syndrome leads to the formation of chronic heart failure.
Important! With conventional heart failure, the volume of the left ventricle in patients is significantly increased, while cardiomyopathy there is a decrease in the volume of one or both of the ventricles, which becomes a characteristic feature pathology..
Symptomatology of the disease
Although a restrictive heart syndrome is a rare occurrence, it can often be observed in patients with chronic or congestive heart failure, which occurs without sudden disruption of the normal functioning of the left ventricle. As for the symptomatology, cardiomyopathy can provoke the following characteristic sensations in the patient:
- Sudden onset of shortness of breath (occurs with physical activity of any type).
- Painful attacks (for this pathology, from the pain sensations the area of the chest, the right hypochondrium and abdomen suffer. With physical exertion, the pain tangibly passes into the heart, and if allowed to spread further, the patient may die of a heart attack).
- Visually noticeable increase in the abdominal cavity and cervical veins.
- The appearance of unnatural noises in the heart and myocardium (clearly audible with a phonendoscope).
In addition to visual signs, this disease is characterized by significant internal abnormalities, such as pulmonary edema, liver enlargement, ascites.
As you can see, cardiomyopathy is similar in its symptoms to other cardiovascular pathologies, and therefore, it will not be possible to diagnose it solely by visual signs. That the doctor could establish the exact diagnosis and appoint effective treatment, the patient should pass a number of laboratory researches which will confirm characteristic changes of a myocardium.
Features of the course of cardiomyopathy in children
Restrictive cardiomyopathy in children occurs at least as often as in adults who have formed people, but it is more difficult to diagnose pathology at this age, which increases the risk of death. By its features, the restrictive heart syndrome in newborns is divided into two main categories:
- Intrinsic disease. A pathology of this nature causes a significant delay in intrauterine development in the child, adaptation (characteristic of newborns), chronic fatigue, significant deviations in physical development.
- Disease directly affecting the myocardium and heart. With this type of pathology, the child will have serious violations of respiratory function (accompanied by shortness of breath or suffocation), swelling of the upper and lower limbs (arise as a result of the fact that the heart does not provide enough blood in them), hypertrophic changes in the heart and liver, pressing pains in breasts.
It is assumed that in children the pathology is genetically determined, but the exact mechanisms are still incomprehensible. Hypereosinophilic syndrome is one of the precisely identified factors. The disease is divided into primary (idiopathic) and secondary, which develops as a result of systemic infiltrative diseases (sarcoidosis, amyloidosis). Individual cases are associated with parasitic, bacterial or viral infections suffered in childhood..
Important! Diagnosis and treatment of this disease in children is significantly different from the methods applied to adults. The nature of the disease can also differ, because the myocardium of the child is not yet formed, as a result of which cardiomyopathy may well provoke a fatal outcome within a few days..
Diagnosis of pathology
Cardiomyopathy, like most cardiovascular pathologies, is diagnosed simultaneously by several medical studies:
- ECG. If the patient has a restrictive heart syndrome, the ECG apparatus will be able to detect this disease by the characteristic formation of a blockage of the left (less often right) Leg of the bundle. Another cardiomyopathy on the ECG is expressed by the reduced voltage of the QRS complex with a simultaneous change in the ST segment and the T wave, which have a nonspecific character. If you allow the disease to develop, then in the later stages of the ECG will detect arrhythmia and a significant overload of the left ventricle (a region of the heart that has been working under pressure for a long time).
- Echocardiography with dopplerography. Cardiomyopathy in this study is manifested by a characteristic decrease in the cavity of the ventricle, affected by pathology. The auricles are enlarged, regurgitation in the MC and TC, signs of pulmonary hypertension are noted. The walls of the heart are usually not changed.
- Radiography. In the photographs of the X-ray apparatus, a restrictive cardiac syndrome manifests itself in venous pulmonary congestion with normal dimensions and contours of the heart.
- Myocardial biopsy. As mentioned earlier, cardiomyopathy often becomes a complication of some early cardiac pathologies in the patient's body. Therefore, by biopsy, the doctor determines the disease, as a result of which the patient developed a restrictive heart syndrome to understand the cause of the pathology. Well, this, in turn, increases the chances of prescribing the right therapy.
Important! Cardiomyopathy by differential diagnosis is distinguished from constrictive pericarditis..
Treatment of pathology
Restrictive heart syndrome, unlike most other diseases affecting the cardiovascular system, does not have a specific method of therapy. Treatment performs only one task - to stabilize the patient's condition and avoid the formation of complications. As a rule, if a patient is confirmed with cardiomyopathy, his treatment is under the supervision of a cardiologist, in a hospital.
If the patient has a higher risk of death, he is immediately transferred to the intensive care unit for further urgent medical intervention. Usually, a restrictive heart syndrome in the acute stage manifests itself in the form of the following pathologies:
- pulmonary edema;
- ventricular extrasystole;
At the initial stages of the disease, the doctor can allow the patient to undergo a course of therapy at home. However, physical activity should be avoided, as any increase in the load can lead to serious complications.
When treating pathology at home, the patient is recommended to follow a special diet that limits the amount of salt and fat consumed per day. If the patient has addictions (smoking and alcohol), then it is better to refuse them, otherwise the chances for successful rehabilitation are significantly reduced.
Treatment includes the treatment of a disease that caused cardiomyopathy, if any, symptomatic therapy of CHF and heart rhythm disturbances. The only cardinal method of getting rid of restrictive changes in the myocardium is a heart transplant.
With a properly selected course of therapy, the condition stabilizes in a few weeks, so it is strongly discouraged to delay the treatment with cardiomyopathy. Otherwise, the patient will wait for serious complications, which, most likely, will end with a fatal outcome... .