Symptoms and treatment of restrictive cardiomyopathy

The definition of “cardiomyopathy” is collective for a group of idiopathic (unknown origin) myocardial diseases, which are based on dystrophic and sclerotic processes in cardiac cells – cardiomyocytes. With cardiomyopathies, the function of the ventricles of the heart always suffers.

Myocardial lesions in coronary heart disease, hypertension, vasculitis, symptomatic arterial hypertension, diffuse connective tissue diseases, myocarditis, myocardial dystrophy and other pathological conditions (toxic, medicinal, alcoholic effects) are secondary and are considered as specific secondary cardiomyopathies caused by the underlying disease.

3How the heart loses elasticity

This is how restrictive cardiomyopathy can be described in one phrase. This disease is characterized by a decrease in the extensibility of the left or right ventricles at the time of filling them with blood (diastole). The muscular wall (myocardium) loses its former elasticity, becomes rigid and unyielding. Their sizes do not increase, but, on the contrary, become somewhat smaller.

Under such conditions, the ventricles cannot absorb the right amount of blood. With the progression of the disease, heart failure develops. This disease has been little studied. However, the available data suggest the lowest prevalence of this disease relative to other types of cardiomyopathies. Mostly sick children and young people.

Reducing the ability of the heart muscles to stretch

It would seem that what is common between these diseases and restrictive cardiomyopathy? However, it is these diseases and conditions, and often unknown reasons, that are responsible for the development of heart failure. “Saturating” metabolic products and other substances, or due to fibrosis, myocardium and endocardium lose their ability to stretch. The pathological links of restrictive cardiomyopathy are still poorly understood. Losing its ability to fill, the heart becomes “hard.”

In this case, however, the systolic function of the heart does not suffer. And the processes of hypertrophy and dilatation are slightly expressed. So, in the atrial systole phase, blood from the indicated chambers of the heart begins to flow into the ventricles. Despite all the zeal of the atria and ventricles, the latter are filled with blood as much as their “rigid” wall allows.

The right or left ventricles do not receive the entire portion of the blood. And everything that was received is expelled into large efferent vessels. The remaining blood in the atria causes a load in their work. Atria are overloaded with blood volume. With the progression of the disease, heart failure is inevitable. With the involvement of the left ventricle, left ventricular failure develops, with the involvement of the right ventricular failure. If both ventricles are affected, total heart failure develops.

Causes

The etiology of primary cardiomyopathies has not yet been fully studied. Among the likely causes of the development of cardiomyopathies are:

  • viral infections caused by the viruses of Coxsackie, herpes simplex, influenza, etc .;
  • hereditary predisposition (genetically inherited defect, causing abnormal formation and functioning of muscle fibers in hypertrophic cardiomyopathy);
  • myocarditis;
  • damage to cardiomyocytes with toxins and allergens;
  • violation of endocrine regulation (fatal effect on the cardiomyocytes of growth hormone and catecholamines);
  • impaired immune regulation.

2 Reasons

Gaucher disease is characterized by damage to the spleen

The reasons leading to the development of RCMP are many. In 1997, a classification of the disease was proposed in accordance with the reasons underlying its development. The authors of the classification proposed combining all cardiomyopathies into two large groups.

Group 1 – myocardial restrictive cardiomyopathies. The term myocardial means that only the muscle layer of the ventricles of the myocardium is affected.

Infiltrative. The myocardium can be affected due to infiltration (impregnation) of it with various substances. This can be amyloid protein in amyloidosis, granulomas in sarcoidosis, fatty deposits (Gaucher cells) in Gaucher disease, mucopolysaccharides in Hurler’s disease, etc.

Diseases of accumulation. In addition to myocardial infiltration, it can affect accumulation diseases. These include hemochromatosis (deposition of iron in the myocardium and other internal organs), deposition of glycogen, and the accumulation of sphingolipids.

Non-infiltrative. Myocardium can lose its ability to stretch and adequately fill due to diseases such as diabetes mellitus, scleroderma, or the cause of the disease has not been established (idiopathic restrictive cardiomyopathy).

Group 2 – endomyocardial restrictive cardiomyopathies. The pathological process affects not only the myocardium, but also the inner shell of the heart endocardium. This group includes heart damage by radiation, tumor metastases, endomyocardial fibrosis processes, toxic damage due to the use of anthracyclines, serotonin, ergotamine, etc.

Dilated (congestive) cardiomyopathy

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Symptoms

Dilated cardiomyopathy (DCMP) is characterized by a significant expansion of all cardiac cavities, symptoms of hypertrophy and a decrease in myocardial contractility. Signs of dilated cardiomyopathy manifest themselves at a young age – at 30-35 years. In the etiology of DCMP, infectious and toxic effects, metabolic, hormonal, and autoimmune disorders presumably play a role; in 10-20% of cases, cardiomyopathy is familial.

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The severity of hemodynamic disorders in dilated cardiomyopathy due to the degree of reduction of contractility and pumping function of the myocardium. This causes an increase in pressure, first in the left and then in the right cavities of the heart. Clinically, dilated cardiomyopathy is manifested by signs of left ventricular failure (shortness of breath, cyanosis, attacks of cardiac asthma and pulmonary edema), right ventricular failure (acrocyanosis, pain and enlargement of the liver, ascites, edema, swelling of the veins of the neck), heart pains, non-cupping, nitroglyceris.

Diagnostics

Objectively observed deformation of the chest (cardiac hump); cardiomegaly with the expansion of the borders to the left, right and up; deafness of cardiac tones at the apex, systolic murmur (with relative insufficiency of the mitral or tricuspid valve), gallop rhythm is heard. With dilated cardiomyopathy, hypotension and severe forms of arrhythmias (paroxysmal tachycardia, extrasystole, atrial fibrillation, blockade) are detected.

An electrocardiographic study records hypertrophy of the predominantly left ventricle, cardiac conduction and rhythm disturbances. Echocardiography shows diffuse myocardial damage, a sharp dilatation of the heart cavities and its prevalence over hypertrophy, intactness of the heart valves, diastolic dysfunction of the left ventricle. X-ray with dilated cardiomyopathy, the expansion of the borders of the heart is determined.

4 Symptoms and signs of the disease

Signs of right ventricular failure

When does the disease begin to cause inconvenience to patients? At that moment when compensatory mechanisms are not enough, and their failure occurs, the heart begins to “get sick”. Depending on which ventricle is suffering, the symptoms will be appropriate. Right ventricular failure is more common due to the frequent involvement of this particular heart chamber. Patients complain of pain in the right hypochondrium, swelling of the legs and feet.

If the left ventricle suffers, shortness of breath and weakness, which become more pronounced during physical exertion, are disturbing. Due to insufficiency of peripheral circulation, the tips of the fingers and nose, earlobes, lips take a bluish tint. The lower limbs are pasty or swollen, the cervical veins are swollen. In the study of the pulse revealed its increased frequency and arrhythmia, weak filling.

During auscultation of the heart region, extrasystoles (extraordinary contractions), irregular and dull tones can be heard, systolic murmur can also be detected. Restrictive cardiomyopathy is also characterized by the phenomenon of “cat purring in the heart.” In the lungs, in the presence of congestion, wheezing can be heard against a background of weakened vesicular breathing. The abdomen with right ventricular failure is increased due to stagnation in the liver and accumulation of fluid in the abdominal cavity.

Hypertrophic Cardiomyopathy

Characterization

Hypertrophic cardiomyopathy (HCMP) is characterized by a limited or diffuse thickening (hypertrophy) of the myocardium and a decrease in the chambers of the ventricles (mainly the left). HCMP is an inherited pathology with an autosomal dominant type of inheritance; it often develops in men of different ages.

With the hypertrophic form of cardiomyopathy, symmetric or asymmetric hypertrophy of the muscle layer of the ventricle is observed. Asymmetric hypertrophy is characterized by a predominant thickening of the interventricular septum, symmetric HCMP – a uniform thickening of the walls of the ventricle.

Based on the presence of ventricular obstruction, there are 2 forms of hypertrophic cardiomyopathy – obstructive and non-obstructive. With obstructive cardiomyopathy (subaortic stenosis), the outflow of blood from the cavity of the left ventricle is disturbed, with non-obstructive HCM, stenosis of the outflow paths is absent.

Symptoms

Specific manifestations of hypertrophic cardiomyopathy are symptoms of aortic stenosis: cardialgia, dizziness, weakness, fainting, palpitations, shortness of breath, pallor. At a later date, the phenomena of congestive heart failure join.

Diagnostics

Heart enlargement is percussion (more to the left), auscultatory – muffled heart sounds, systolic murmurs in the III-IV intercostal space and in the apex, arrhythmia. The shift of the heart beat down and to the left, a small and slow heart rate on the periphery, is determined. Changes in the ECG with hypertrophic cardiomyopathy are expressed in myocardial hypertrophy of the mainly left parts of the heart, T wave inversion, registration of the pathological Q wave.

Of the non-invasive diagnostic methods for HCM, the most informative is echocardiography, which reveals a decrease in the size of the heart cavities, thickening and poor mobility of the interventricular septum (with obstructive cardiomyopathy), a decrease in myocardial contractile activity, and abnormal systolic mitral valve leaflet prolapse.

Diagnostics

When diagnosing cardiomyopathies, the clinical picture of the disease and the data of additional instrumental methods are taken into account. The ECG usually shows signs of myocardial hypertrophy, various forms of rhythm and conduction disturbances, changes in the ST segment of the ventricular complex. When radiography of the lungs, dilation, myocardial hypertrophy, and congestion in the lungs can be detected.

Especially informative in cardiomyopathies is the data of echocardiography, which determine myocardial dysfunction and hypertrophy, its severity and the leading pathophysiological mechanism (diastolic or systolic insufficiency). According to indications, an invasive examination is possible – ventriculography. Modern methods of visualization of all parts of the heart are MRI of the heart and MSCT. Probing of cardiac cavities allows the collection of cardiobiopaths from cardiac cavities for morphological studies.

CT scan of the chest. Marked expansion of the heart (mainly left parts) in a patient with cardiomyopathy.

Complaints and examination of the patient are not specific and only indicate the presence of heart failure in the patient. Each method of instrumental diagnostics separately cannot give a clear picture of restrictive cardiomyopathy. However, an integrated approach allows you to get an idea of ​​the existing disease in the patient.

Electrocardiography There are no specific signs of the disease on the electrocardiogram. ECG signs may indicate an increase in the left atrium. Later signs of right ventricular hypertrophy join. On the ECG, various heart rhythm and conduction disturbances can also be recorded. Extrasystoles, atrial fibrillation, paroxysmal tachycardia, atrioventricular blockade, blockade of the bundle of His bundle can be detected.

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Echocardiographic examination (ultrasound of the heart). This diagnostic method is leading in the diagnosis of restrictive cardiomyopathy. The absence of expansion of the ventricular cavities with an increase in the left atrium, and subsequently an increase in the right atrium, may indicate a violation of the diastolic function of the ventricles.

Chest x-ray (OGC). On the x-ray of the OGK, you can see the increased sizes of the atria against the background of the normal sizes of the ventricles. Signs of stagnation in the lungs may also be visible.

Cardiac catheterization. The method allows you to establish an increase in ventricular end-diastolic pressure, to evaluate the pulse pressure and ejection fraction.

When making a diagnosis, as well as for the purpose of differential diagnosis, computed tomography, magnetic resonance imaging, endomyocardial biopsy can be used.

Diagnostic procedures for detecting restrictive cardiomyopathy are quite diverse. Used laboratory study of biological fluids, instrumental examination, minimally invasive methods. An approximate list of the main methods of diagnosis:

  • X-ray of the heart and lungs.

The method is simple and affordable, but it can not be used often because of the potential danger of x-rays to human health. The examination helps to see how the size and contours of the heart are changed, gives an idea of ​​congestion in the lungs. With the help of the image, the enlarged left atrial part is visualized, you can notice superdense areas of the lung tissue due to the accumulation of eosinophilols. They are able to migrate throughout the organ.

Changes specific to restrictive cardiomyopathy are not displayed on the ECG. Large atria can be detected, while the ventricles remain the same. It is possible to detect heart block, a failure of the rhythm of contractions is recorded. If necessary, daily ECG monitoring is used (Holter study).

It is carried out to determine the cause of chest pain, identify heart failure. This method is based on the use of x-rays. But he has an important advantage over a conventional x-ray. As a result of the study, the doctor has the opportunity to examine the heart from all sides thanks to the obtained three-dimensional image.

Rays are fed in small doses, they scan a diseased organ in different directions. Using ultra-sensitive sensors, the scanning results are recorded in the form of a series of layered images. After processing them using special programs, a three-dimensional computer model of the object under study is built. Spiral tomography is the most common; there are also multispiral, emission, and cone-beam types of CT. If necessary, the patient is injected with a contrast agent for greater image clarity, which accumulates in the diseased organ.

This type of examination is similar to the previous one. The difference is that MRI uses electromagnetic waves instead of X-rays. There are also certain differences in the indications and contraindications of both types of diagnostics.

Another name is ultrasound (ultrasound). Thanks to this method, the doctor assesses the size of the heart chambers, the level of compaction of the organ wall, reveals malformations. The presence of restrictive cardiomyopathy attracts attention with an enlarged layer of the endocardium. At the same time, the myocardial volume is not changed, the parameters of the left ventricle remain within normal limits, and systolic function is not impaired.

When forming valvular defects, one can see the limited movement of the valves. A thrombus is detected in the upper part of the myocardium. An obvious symptom is the low rate of filling blood of the ventricular cavities with diastole, since the endocardium becomes rehydrated. To study hemodynamic disturbances in the heart chambers and vessels, you can use the Doppler echocardiography.

  • Endomyocardial biopsy.

This procedure refers to the puncture of the heart tissue (a myocardial particle along with the endocardium). Material for research is taken using a catheter. A biopsy is recommended in extreme cases, if there is no other way to obtain accurate information. It allows you to identify connective tissue fibers in the heart muscle (late stage of development of cardiomyopathy), and also determines an elevated level of eosinophilic cells (in the early stage of the disease).

Fields of application: fluoroscopy, radiography, CT. Using a contrast element introduced into the vessels, an accurate picture of the state of the vascular system is obtained, and hemodynamic processes are also analyzed. In the case of restrictive cardiomyopathy, arterial thromboembolism is often diagnosed. Angiography can detect a similar pathology.

  • Laboratory examination of urine and blood.

A blood test if a pathology is detected will show a high level of eosinophils and an increased ESR. The secondary form of restrictive cardiomyopathy will manifest itself in changes in the composition of blood and urine that occur against the background of the underlying disease. If there is a tumor, hemoglobin will be reduced, protein in the urine will indicate amyloidosis.

A biochemical study of blood cells will detect cholesterol, sugar, creatinine, uric acid in order to establish a disease corresponding to pathological changes.

Restrictive Cardiomyopathy

Characterization

Restrictive cardiomyopathy (RKMP) is a rare myocardial lesion, usually occurring with endocardial interest (fibrosis), inadequate diastolic relaxation of the ventricles and impaired cardiac hemodynamics with preserved myocardial contractility and the absence of its pronounced hypertrophy.

In the development of RCMP, a significant role is given to pronounced eosinophilia, which has a toxic effect on cardiomyocytes. With restrictive cardiomyopathy, a thickening of the endocardium and infiltrative, necrotic, fibrotic changes in the myocardium occur. The development of RCMP goes through 3 stages:

  • Stage I – necrotic – is characterized by severe eosinophilic myocardial infiltration and the development of coronaritis and myocarditis;
  • Stage II – thrombotic – manifests itself as endocardial hypertrophy, parietal fibrinous overlays in the cavities of the heart, vascular myocardial thrombosis;
  • Stage III – fibrotic – is characterized by widespread intramural myocardial fibrosis and nonspecific obliterating coronary artery endarteritis.
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Symptoms

Restrictive cardiomyopathy can occur in two types: obliterating (with fibrosis and obliteration of the ventricular cavity) and diffuse (without obliteration). With restrictive cardiomyopathy, there are phenomena of severe, rapidly progressive congestive circulatory failure: severe shortness of breath, weakness with little physical effort, increasing edema, ascites, hepatomegaly, neck vein swelling.

Diagnostics

The size of the heart is usually not increased, with auscultation the rhythm of the gallop is heard. Atrial fibrillation, ventricular arrhythmias are recorded on the ECG, a decrease in the ST segment with inversion of the T wave can be determined. The phenomena of venous congestion in the lungs, slightly enlarged or unchanged heart sizes, are noted. The echoscopic picture reflects insufficiency of the tricuspid and mitral valves, a decrease in the size of the obliterated cavity of the ventricle, and a violation of the pumping and diastolic function of the heart. Eosinophilia is noted in the blood.

Forecast

The outcome of the course of restrictive cardiomyopathy is affected by the diagnosis. The more accurately and earlier the diagnosis was made, the greater the chance of survival appears in a sick person. The complexity of the fight against the disease is due to the lack of specific preventive recommendations to prevent the development of pathology.

In general, the prognosis of such violations cannot be called comforting. The quality of life of patients with ventricular diastole dysfunction is significantly deteriorating, many people die from the complications that have arisen: heart failure, thromboembolism, arrhythmia. After detecting the primary manifestations of the disease, death occurs in 50 cases out of 100 after a two-year period.

In the case of a secondary form of restrictive cardiomyopathy, the prognosis largely depends on how much the underlying disease is treatable.

For women who have been given a similar diagnosis, you need to protect yourself from a possible pregnancy. The expectant mother may not survive the upcoming birth process.

Restrictive cardiomyopathy in clinical practice is quite rare, poorly treated, often leading to death. Since the true causes of violations in most cases cannot be established, the only way to protect yourself from danger is to regularly undergo a routine examination. It is especially important to monitor the state of the heart for people who have relatives with the same diagnosis.

Diuretics (diuretics), cardiac glycosides, vasodilator drugs, blood clot inhibitors

Unfortunately, there is currently no specific treatment for restrictive cardiomyopathy. If the reason for its development is clear, then, as a rule, the underlying disease is treated. Therapy of restrictive cardiomyopathy is symptomatic and is aimed at reducing overload in the large and small circles of blood circulation, reducing the load on the left ventricle, improving the pumping function of the heart. Diuretics (diuretics), cardiac glycosides are used; vasodilator drugs; drugs that prevent the formation of blood clots.

The prognosis of the disease is usually poor. Surgical treatment (heart transplant) can increase longevity. However, it was found that after some time the same pathological changes develop in the transplanted heart.

In relation to the prognosis, the course of cardiomyopathies is extremely unfavorable: heart failure is steadily progressing, the likelihood of arrhythmic, thromboembolic complications and sudden death is high. After diagnosis of dilated cardiomyopathy, the 5-year survival rate is 30%. With systematic treatment, stabilization of the state for an indefinite period is possible. There are cases exceeding the 10-year survival rate of patients after heart transplant surgery.

Surgical treatment of subaortic stenosis with hypertrophic cardiomyopathy, although it gives an undoubted positive result, is associated with a high risk of death of the patient during or shortly after surgery (every 6th operated person dies). Women with cardiomyopathies should abstain from pregnancy because of the high probability of maternal mortality. Measures for the specific prevention of cardiomyopathies have not been developed.

Arrhythmogenic right ventricular cardiomyopathy

Characterization

The development of arrhythmogenic cardiomyopathy of the right ventricle (ACP) characterizes the progressive replacement of cardiomyocytes of the right ventricle with fibrous or adipose tissue, accompanied by various disorders of the ventricular rhythm, including ventricular fibrillation. The disease is rare and poorly studied, heredity, apoptosis, viral and chemical agents are called as possible etiological factors.

Symptoms

Arrhythmogenic cardiomyopathy can develop already in adolescence or youth and is manifested by palpitations, paroxysmal tachycardia, dizziness, or fainting. In the future, the development of life-threatening types of arrhythmias is dangerous: ventricular extrasystole or tachycardia, episodes of ventricular fibrillation, atrial tachyarrhythmias, atrial fibrillation or flutter.

Diagnostics

With arrhythmogenic cardiomyopathy, the morphometric parameters of the heart are not changed. With echocardiography, a moderate expansion of the right ventricle, dyskinesia and local protrusion of the apex or lower wall of the heart are visualized. The MRI method reveals structural changes in the myocardium: local thinning of the myocardial wall, aneurysm.

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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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