Restrictive cardiac cardiomyopathy( RSCM) develops for various reasons, some of them up to now can not be established with precision. The disease is characterized by the fact that on the myocardium areas are formed that resist diastolic filling due to inextensible walls. Both ventricles are affected by this pathology, but the left most often suffers, to which the greatest burden is distributed.
Features of
Disease Restrictive cardiomyopathy refers to the least common types of cardiomyopathies, which is why it remains the most poorly understood. Most RCFs are affected by young people, as well as children. Doctors say that in its manifestations, it is similar to hypertrophic cardiomyopathy.
This disease is usually attributed to pathologies of mixed genesis, that is, when there are factors favorable for the development of RCMP in the form of hereditary predilection and concomitant disease. Most often, its signs are most noticeable during physical exertion, as the altered heart resists normal filling with blood.
On the classification of diseases with the diagnosis of "cardiomyopathy" read on.
Difference of different types of cardiomyopathy( scheme)
Classification and forms of
Allocate primary RCMP, which is classified as idiopathic( i.e., unknown), and secondary. The latter can appear due to the development in the body of a variety of diseases.
RCMF also occurs in the form of endomyocardial disease, divided into two separate subspecies:
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The opinion of doctors. .. & gt; & gt;
- With eosinophilia. These are special cells in the blood and when the disease develops, their number rises. Against this background, the walls of the heart become denser. Its damage occurs as a result of death of eosinophils.
- Endomyovascular fibrosis. The inner walls of the muscle also become denser, but there is no change in the composition of the blood. Pathology is inherent in newborn children and causes a rapid death.
The obliterating form( deposits under the heart cavities and inside them), as well as diffuse infiltration, are distinguished by the form of sediment spread. In the latter case, the deposits are distributed evenly.
Causes of the onset of
The cause of the appearance of the primary RCMP is not revealed, but the secondary one arises as a result of such pathologies as:
- scleroderma,
- hemochromatosis,
- glycogenoses,
- sarcoidosis,
- carcinoid,
- amyloidosis,
- tumor development,
- radiation damage to the heart muscle,
- taking medicines.
Infectious causes of endomiocardial fibrosis include the presence of parasites in the body. Next we will talk about the primary and secondary signs of restrictive cardiomyopathy.
Symptoms of restrictive cardiomyopathy in humans
The severity of the symptom depends on how severe the heart is in the patient. Early signs of development of RCMP are as follows:
- Increased fatigue;
- Arrhythmic heartbeat;
- Shortness of breath and shallow breathing when there is dissatisfaction with inspiration;
- Swelling of the feet;
- Confusion or loss of consciousness.
Diagnostics
Diagnostic methods are very extensive. First, they collect an anamnesis. For the physical condition of the patient is characterized by the presence of pulmonary rales, swollen veins, his pulse is accelerated, heart tones are poorly expressed. More accurate diagnosis includes the following measures:
- ECG.Although the study is not able to identify specific signs, it indicates a change in the thickness of the walls and reveals irregularities in the beat rhythm.
- Radiography helps evaluate the change in heart size, determine whether the atrium is enlarged and whether there is stasis in the vessels.
- Echocardiography. It is used to determine the thickness and volume of the muscle cavities, the detection of thrombi and the violation of blood circulation in the heart.
- Angiography. The method in which a special colorant is injected into the cavity of the vessels. Contrast allows you to determine their exact size and reveal a tendency to thromboembolism.
- MRI and SCT allow you to get the most accurate image of organs.
- Catheterization in which special catheter tubes are inserted into the vascular cavity in order to measure blood pressure in the heart and determine the presence of a reverse blood flow.
- Biopsy is performed with catheterization, but this method of research is rarely selected, provided that other methods of investigation do not help to identify the cause of development of RCMP.
Treatment of
Treatment of secondary restrictive cardiomyopathy is performed only to eliminate symptoms and prevent complications. There is no specific therapy so far.
Therapeutic method
Bleeding is a somewhat outdated method, but it is used together with chelates to remove excess iron.
Not the least role in this is playing a special set of exercises and playing sports in general, as well as maintaining a diet. Restricting will not only use the products indicated by the doctor, but also water, as well as salt.
Drug method
Drugs in each case are selected by the doctor, as the list of drugs used is different and will depend on the causes that led to the development of the disease. Therapy of the underlying disease is one of the main goals. So, with hemochromatosis it is shown to take chelates - medicines that bind iron for excretion from the body. This helps prevent its accumulation in the heart, which prevents the extensibility of its walls.
If the cause of the disease is sarcoidosis, glucocorticosteroids are prescribed, and calcium preparations are taken for better myocardial dilatation.
To prevent complications that are likely to occur with restrictive cardiomyopathy, the following medicines are used:
- Beta-blockers. With the development of heart failure, they are used in combination with ACE inhibitors.
- Diuretics. Allow to get rid of salt and liquid stagnation.
- Angiotensin receptor antagonists are prescribed as a substitute for ACE inhibitors.
- Cardiac glycosides help improve muscle contractility.
- Instead of diuretics, antagonists of aldosterone receptors may be prescribed. Together with a weak diuretic effect, they have the ability to retain potassium in the body.
In the prevention of thromboembolism, a special role is played by medications from the category of direct and indirect anticoagulants. They help to resolve blood clots, and also prevent their formation on the walls of blood vessels. If you neglect such therapy, sudden death can occur at any time.
Operative intervention
Operative intervention is also used for the treatment of restrictive cardiomyopathy. In particular, excision of a portion of a thickened myocardium can be prescribed, which restores the heart to relaxation and thereby restores diastolic functions.
In severe cases, when the valves of the heart do not cope with their work and do not retain blood flow, replace them with dentures. If the electrical impulse is seriously disturbed, then the pacemaker is already installed, which will act as the pacemaker.
Cardiac transplantation is the most cardinal method of treatment. However, it is proved that transplantation is not always effective, because under the influence of the same factors, the development of repeated restrictive cardiomyopathy is possible.
The following video will tell you about surgical intervention with restrictive cardiomyopathy:
Prevention of
disease For restrictive cardiomyopathy, not only specific treatment methods are available, but also prevention. Even under the condition of maintaining an HLS, the risk of its occurrence remains high, if it is transmitted hereditarily.
Prevention of secondary cardiomyopathy is possible only if the full prevention of all diseases that can provoke it. Since not the last role is played by the fact of timely detection of pathology, it is important to undergo annual planned prophylactic medical examination every year. Only in this way it will be possible to prevent the aggravation of the disease, to cure it in a timely manner and to avoid a negative prognosis.
Complications of
The most common complications that accompany restrictive cardiomyopathy are:
- Delay in electrical pulse conductivity in the heart muscle;
- Circulatory disturbance due to chronic insufficiency;
- Formation of thrombi in the cavity of the heart vessels;
- Appearance of rhythm disturbances.
Forecast
Even after 5 years after the detection of the disease, up to 50% of patients die. The cause of this outcome is either the progression of cardiomyopathy, or the appearance of complications that lead to a fatal outcome. However, according to statistics, about 50% of patients( provided all the rules and recommendations for the therapy of the disease are observed) are able to live more than 10 years.