Amyloidosis of the heart is a pathological condition of the metabolic organ of the circulatory system. In the heart tissues, especially the myocardium, the deposition of substances that are not characteristic of the myocardium develops. In its chemical nature, amyloid is a protein associated with polysaccharides.
Amyloid accumulation leads to abnormalities in the heart rate and the ability of the heart chambers to contract, pressure in the heart chambers, the pathologies of the valve structure, the progress of inadequate cardiac function. And today we will consider the features of treatment of amyloidosis of the heart, its pathogenesis and the mechanism of development.
Features and pathogenesis of the disease
The diagnosis of "amyloidosis of the heart" is established on the basis of laboratory hematological analyzes, aspiration biopsy, electrocardiographic, scintigraphic, X-ray and other instrumental methods. Amyloidosis of the heart is treated in 2 directions: polychemotherapeutic, providing the possibility of slowing the accumulation of amyloids and medicamentous, weakening the inadequate work of the heart.
The basis of amyloid pathology is a violation of the protein metabolism system, in which the fibrillar type of polypeptides accumulates in the striated tissue and connective tissue of the heart, the pericardial bag, the walls of the coronary arteries and the aorta. Amyloid proteins, in addition to the heart, can be deposited in the organs of the digestive, hematopoietic, respiratory and excretory systems.
Deep old age, familial and idiopathic forms of the disease leave no chance for the lack of the ability to accumulate amyloid polypeptides in the cardiac muscle. The tissues of the pericardium sac and the inner layer of the heart are also susceptible to accumulation of pathogenic substances, although to a lesser extent than the myocardium. Amyloid substances are found to a greater extent in the extracellular space between cardiac myocytes, which causes the development of stenosis and spasms of the intramural arteries and small arterioles.
The degree of stretching of the heart tissues and chambers decreases with an increase in the concentration of amyloid polypeptides due to the greater density of tissues. The volume of ejection of blood from the heart also suffers because of the inability of the chambers to change the amount of incoming blood. With the old type of amyloid deposition, the degeneration of the muscle fibers of the heart and the accumulation of pathogenic metabolites in the walls of the aorta and coronary arteries are noted. The dangerous consequences of amyloidosis are expressed by a decrease in the amount of ejected blood from the chambers, violation of the processes of contraction and relaxation of the cardiac myocytes of the heart, which forms chronic heart failure.
With the accumulation of amyloids in the sinus-atrial, atrioventricular nodes, Guissé beams and Purkinje fibers, problems arise with the conductivity of electrical impulses of varying severity( depending on the site of their accumulation).The deposition of amyloid polypeptides in valvular structures is the cause of the progression of valvular heart failure. It's time to talk about the stages and types of amyloidosis.
Classification and forms of amyloidosis of the heart
Progress of amyloidosis of the heart is classified by the World Health Organizations with a passage of 4 stages.
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;
- Non-invasive diagnostic methods do not detect signs of disease. Even carrying out a biopsy does not guarantee a reliable result.
- Symptoms of amyloidosis are absent, but instrumental examination methods without penetration into the heart cavity reveal the detected disorders. The reliability of the results of the biopsy is high.
- It is used as a compensating stage, in which it is possible to stop the phenomenon of myocardial cell degeneration. Symptoms are fairly vivid.
- Cardiomyopathy is developed to a decompensatory character, when it is possible to support cardiac activity, but the process of amyloidosis becomes irreversible.
Forms of impairment of protein metabolism with the deposition of amyloids have a shortened abbreviation in accordance with the Latin name of the pathological process.
Next, you will learn about the causes of amyloidosis of the heart.
Causes of the appearance of
Patterns of amyloidosis of the heart can not be detected for any population category. The family form of amyloidosis is inherited by one of the chromosomes of the genetic apparatus, which has a mutant dominant gene. Most often, the hereditary form of the disease is noted among residents of the Mediterranean basin states.
The most common, observed and observed secondary form of amyloidosis of the heart, which occurs in response to the chronic form of insufficient kidney function, chronic disorders of the respiratory, musculoskeletal and some venereal diseases.
Symptoms of
Symptoms of progressive amyloidosis are similar to other heart diseases( ischemic disease, cardiomyopathy by hypertrophic type).For this reason, the diagnosis of the disease, focused exclusively on the symptomatic picture, is impossible.
In the early stages of the disease either does not make itself felt by any signs, or does not show any specific symptoms, leaving for diagnosis many variants of the diagnosis. The patient can quickly feel fatigue, complains of sudden dizziness, excessive irritability, susceptibility to swelling and weight loss.
Usually, before the onset of amyloidosis symptoms, a number of factors serve as triggers for the disease:
- acute viral infection,
- psycho-emotional overstrain,
- frequent or prolonged stress.
The onset of amyloidosis is judged by persistent hypotension, heart rate failures, "chest toad" pains. Further dyspnea develops on increasing, swelling of the tissues increases, which indicates chronic heart failure. Attempts to eliminate amyloidosis with glycosides, which help restore normal heart activity in other cardiological diseases, do not lead to an improvement in the patient's condition. The progress of the disease is rapid.
Penetration of amyloids into the conduction system of the heart, especially in its initial sections, causes a decrease in heart rate and is often the cause of cardiac arrest( clinical death).Amyloidosis, covering many functional systems, is characterized by damage to the organs of these functional systems( kidneys, skin, brain, etc.).Next, we will tell you about echocardiography and other methods of diagnosing amyloidosis of the heart.
Diagnosis
The absence of specific symptoms of amyloidosis reduces the frequency of diagnostic tests. In most cases, amyloidosis is detected with post mortem autopsy of a sick person.
During the examination of a patient during life, an experienced cardiologist will pay attention to a number of problems.
- Firstly, progressive amyloidosis detects noise and deafness of heart sounds while listening to a phonendoscope.
- Secondly, an electrocardiographic study shows the scale deviations from normal conduction and failure of cardiac excitation.
- Third, the ultrasound of the heart shows thickenings on all surfaces of the chambers, partitions. Echogram also decreases the kinetic activity of cardiomyocytes. Successful for the doctor-diagnostician is the detection of hypoechoic areas in the heart cavities, reflected on the image in the form of granules. These are amyloid congestions.
In amyloidosis, the radiograph shows the presence of exudate in the pleural cavity and a larger shadow trace from the heart. The most reliable result among the diagnostic methods for determining amyloidosis is magnetic resonance imaging and the method of scintigraphy. Determine the morphology of amyloidosis is often possible biopsy of organs that have undergone abnormal accumulation of amyloid peptides.
Laboratory methods reveal a rise in the concentration of globulin and a decrease in the number of albumins and other disorders in pathology. When establishing the diagnosis of "amyloidosis" through a comprehensive examination, Fabry's disease, hypothyroidism with pathological changes in the heart, aortic stenosis and other pathologies are excluded.
Treatment
Therapeutic
The measures are aimed at reducing the concentration of proteins from which amyloids are formed. In addition to the main direction, a set of measures is also applied to eliminate puffiness, arrhythmia, correction of systolic pressure and other non-specific symptoms.
Medication
- If secondary amyloidosis of the heart takes place, medicines are used to reduce the pathogenic effect in other systems with colchicine and corticosteroids.
- In primary amyloidosis it is customary to use anti-tumor drugs, the scheme of which is developed individually. For example, melphalan and prednisolone or thalidomide and chemotherapeutic drugs.
Operative intervention
Radical methods of treatment suggest 4 types of transplantation:
- of stem cells.
- Pacemaker.
- In general, the heart.
- Liver.
Traditional medicine
- From the methods of traditional medicine, phytotherapy using herbs of St. John's wort, camomile chamomile, birch buds, oat seed infusion, etc. has proved to be a positive one.
- Juniper, blueberry, blackberry rowan is used from fruits of plants.
Following are recommendations for the prevention of amyloidosis of the heart.
Prevention
It is recommended that antibiotics be treated in a timely manner in the secondary type of the disease. It is not possible to avoid recurrences with amyloidosis, even taking into account preventive measures.
The hereditary form of the disease involves a timely study of the fetal karyotype.
Complications of
The most unfavorable complication in a patient with amyloidosis of the heart is death. Dangerous consequences for the health and life of a sick person are ischemic stroke, myocardial infarction, infectious processes in different functional systems( erysipelas, glomerulonephritis, parotitis, pneumonia, etc.).
The most common outcome of amyloidosis of the heart is described below.
Forecast
Life expectancy is affected by 3 factors:
- rare occurrence and insufficient instrumentation for diagnosis;
- no specific symptoms;
- steady progress of the disease.
From the moment of detection of the disease( heart failure) to a lethal outcome, usually no more than 2 years.
You can understand what amyloidosis of the heart is from the following video: