Cardiomyopathy – primary damage of a cardiac muscle which is not connected with inflammatory, tumoral, ischemic genesis as which typical manifestations serve the kardiomegaliya, the progressing heart failure and arrhythmias. Distinguish dilatatsionny, hypertrophic, restrictive and aritmogenny a cardiomyopathy. Within diagnostics of a cardiomyopathy the ECG, EhoKG, a X-ray analysis of a thorax, heart MPT and MCK is carried out. At cardiomyopathies the sparing mode, medicamentous therapy (diuretics, warm glycosides, antiarhythmic medicines, anticoagulants and antiagregant) is appointed; according to indications cardiac intervention is carried out.
Definition of "cardiomyopathy" is collective for group idiopathic (an unknown origin) myocardium diseases which development is the cornerstone dystrophic and sclerous processes in warm cages – kardiomiotsita. At cardiomyopathies function of ventricles of heart always suffers. Damages of a myocardium at IBS, a hypertension, vaskulita, symptomatic arterial hypertension, diffusion diseases of connecting fabric, myocardites, a miokardiodistrofiya and other pathological states (toxic, medicinal, alcoholic influences) are secondary and are considered as specific secondary a cardiomyopathy, caused by the main disease.
Classification of cardiomyopathies
On anatomic and functional changes of a myocardium distinguish several types of cardiomyopathies:
- dilatatsionny (or stagnant);
- hypertrophic: asymmetric and symmetric; obstructive and not obstructive;
- restrictive: obliterating and diffusion;
- aritmogenny right ventricular cardiomyopathy.
Reasons of cardiomyopathies
The etiology of primary cardiomyopathies is up to the end not studied today. Among the probable causes causing development of cardiomyopathies call:
- the viral infections caused by Koksaki's viruses, simple herpes, flu, etc.;
- hereditary predisposition (genetically inherited defect causing the wrong formation and functioning of muscle fibers at a hypertrophic cardiomyopathy);
- the postponed myocardites;
- defeat of kardiomiotsit toxins and allergens;
- violations of endocrine regulation (pernicious impact on kardiomiotsita of somatotropny hormone and catecholamines);
- violations of immune regulation.
Dilatatsionny (stagnant) cardiomyopathy
The Dilatatsionny cardiomyopathy (DKMP) is characterized by essential expansion of all cavities of heart, the phenomena of a hypertrophy and decrease in sokratitelny ability of a myocardium. Signs of a dilatatsionny cardiomyopathy prove already at young age – in 30-35 years. In DKMP etiology presumably infectious and toxic influences, exchange, hormonal, autoimmune violations play a role, in 10-20% of cases the cardiomyopathy has family character.
Expressiveness of haemo dynamic frustration at a dilatatsionny cardiomyopathy is caused by extent of decrease in a sokratimost and pump function of a myocardium. It causes increase in pressure at first in left, and then in the right cavities of heart. Clinically dilatatsionny cardiomyopathy is shown by signs of left ventricular insufficiency (short wind, cyanosis, attacks of cardiac asthma and hypostasis of a lung), right ventricular insufficiency (akrotsianozy, pains and increase in a liver, ascites, hypostases, swelling of veins of a neck), cardiac pains, not stopped nitroglycerine, heartbeat.
Deformation of a thorax (warm hump) is objectively noted; a kardiomegaliya with expansion of borders to the left, to the right and up; dullness of warm tones on a top, systolic noise (is listened at relative insufficiency of the mitralny or trikuspidalny valve), a gallop rhythm. At a dilatatsionny cardiomyopathy hypotonia and severe forms of arrhythmias (paroksizmalny tachycardia, premature ventricular contraction, vibrating arrhythmia, blockade) come to light.
At an electrocardiographic research the hypertrophy of mainly left ventricle, violation of warm conductivity and a rhythm are fixed. shows diffusion damage of a myocardium, sharp dilatation of cavities of heart and its prevalence over a hypertrophy, an intaktnost of heart valves, diastolic dysfunction of the left ventricle. Radiological at a dilatatsionny cardiomyopathy expansion of borders of heart is defined.
The hypertrophic cardiomyopathy (GKMP) is characterized by a limited or diffusion thickening (hypertrophy) of a myocardium and reduction of cameras of ventricles (mainly left). GKMP is hereditary pathology with autosomno-prepotent type of inheritance, develops at men of different age more often.
At a hypertrophic form of a cardiomyopathy the symmetric or asymmetric hypertrophy of a muscular layer of a ventricle is observed. The asymmetric hypertrophy is characterized by the prevailing thickening of an interventricular partition, symmetric GKMP - a uniform thickening of walls of a ventricle.
On the basis of existence of obstruction of ventricles distinguish 2 forms of a hypertrophic cardiomyopathy – obstructive and not obstructive. At an obstructive cardiomyopathy (a subaortal stenosis) outflow of blood from a cavity of the left ventricle is broken, at not obstructive GKMP the stenozirovaniye of ways of outflow is absent. As specific manifestations of a hypertrophic cardiomyopathy serve symptoms of an aortal stenosis: kardialgiya, dizziness attacks, weakness, faints, heartbeat, short wind, pallor. In later terms the phenomena of stagnant heart failure join.
Perkussionno is defined increase in heart (more to the left), auskultativno – deaf tones of heart, systolic noise in the III-IV mezhreberye and in the field of a top, arrhythmia. Shift of a warm push down and to the left, the small and slowed-down pulse is defined on peripheries. Changes on the ECG at a hypertrophic cardiomyopathy are expressed in a hypertrophy of a myocardium of mainly left departments of heart, inversion of a tooth of T, registration of a pathological tooth of Q.
From noninvasive diagnostic techniques at GKMP the echocardiography which reveals reduction of the sizes of cavities of heart, a thickening and bad mobility of an interventricular partition (at an obstructive cardiomyopathy), decrease in sokratitelny activity of a myocardium, an abnormal systolic prolapse of a shutter of the mitralny valve is most informative.
Restrictive cardiomyopathy (RKMP) – seldom found damage of a myocardium proceeding usually with interest of an endokard (fibrozirovaniye), inadequate diastolic relaxation of ventricles and violation of warm haemo dynamics at the kept sokratitelny ability of a myocardium and lack of its expressed hypertrophy.
In development of RKMP the big part is assigned to the expressed eozinofiliya having toxic effect on kardiomiotsita. At a restrictive cardiomyopathy there is a thickening of an endokard and infiltrative, necrotic, fibrous changes in a myocardium. Development of RKMP takes place 3 stages:
- The I stage — necrotic – is characterized by the expressed eozinofilny infiltration of a myocardium and development of a koronarit and myocarditis;
- The II stage — trombotichesky - is shown by a hypertrophy of an endokard, pristenochny fibrinozny imposings in heart cavities, vascular thrombosis of a myocardium;
- The III stage — fibrotichesky – is characterized by widespread intramuralny fibrosis of a myocardium and nonspecific obliterating endarteriity coronal arteries.
The restrictive cardiomyopathy can proceed on two types: obliterating (with a fibrozirovaniye and an obliteration of a cavity of a ventricle) and diffusion (without obliteration). At a restrictive cardiomyopathy the phenomena of the heavy, quickly progressing stagnant insufficiency of blood circulation are noted: the expressed short wind, weakness at insignificant physical efforts, the accruing hypostases, ascites, a gepatomegaliya, swelling of veins of a neck.
In sizes heart is usually not increased, at an auskultation the gallop rhythm is listened. On the ECG blinking of auricles, ventricular arrhythmias are registered, decrease in a ST segment can decide on inversion of a tooth of T. Rentgenologicheski the phenomena of venous stagnation in lungs which are a little increased or not changed heart sizes are noted. The Ekhoskopichesky picture reflects insufficiency of trikuspidalny and mitralny valves, reduction of the sizes of the obliterated ventricle cavity, violation of pump and diastolic function of heart. In blood the eozinofiliya is noted.
Aritmogenny right ventricular cardiomyopathy
Development of the aritmogenny cardiomyopathy of the right ventricle (ACRV) characterizes the progressing replacement of kardiomiotsit of the right ventricle with fibrous or fatty tissue which is followed by various violations of a ventricular rhythm, including fibrillation of ventricles. The disease rare and poorly studied, as possible etiologichesky factors is called by heredity, apoptosis, virus and chemical agents.
The Aritmogenny cardiomyopathy can develop already in the teenage or youthful period and is shown by heartbeat, paroksizmalny tachycardia, dizziness or faints. Further development of zhizneugrozhayushchy types of arrhythmias is dangerous: ventricular premature ventricular contraction or tachycardia, episodes of fibrillation of ventricles, predserdny takhiaritmiya, blinking or trembling of auricles.
At an aritmogenny cardiomyopathy morphometric parameters of heart are not changed. At an echocardiography moderate expansion of the right ventricle, dyskinesia and local protrusion of a top or lower wall of heart is visualized. By MRT method structural changes of a myocardium come to light: local thinning of a wall of a myocardium, aneurism.
Complications of cardiomyopathies
At all types of cardiomyopathies heart failure progresses, development of arterial and pulmonary tromboemboliya, violations of conductivity of heart, heavy arrhythmias (vibrating arrhythmia, ventricular premature ventricular contraction, paroksizmalny tachycardia), a syndrome of sudden warm death is possible.
Diagnostics of cardiomyopathies
At diagnostics of cardiomyopathies consider a clinical picture of a disease and data of additional tool methods. On the ECG myocardium hypertrophy signs, various forms of violations of a rhythm and conductivity, change of a ST segment of a ventricular complex are usually fixed. At a X-ray analysis of lungs it is possible to reveal dilatation, a myocardium hypertrophy, developments of stagnation in lungs.
Are especially informative at cardiomyopathies the data of EhoKG defining dysfunction and a hypertrophy of a myocardium, its expressiveness and the leading pathophysiological mechanism (diastolic or systolic insufficiency). According to indications carrying out invasive inspection – a ventrikulografiya is possible. Modern methods of visualization of all departments of heart are MRT of heart and MSKT. Sounding of cavities of heart allows to take kardiobioptat from heart cavities for a morphological research.
Treatment of cardiomyopathies
Specific therapy of cardiomyopathies is absent therefore all medical actions aim at prevention of complications incompatible with life. Treatment of cardiomyopathies in a stable phase out-patient, with the assistance of the cardiologist; periodic planned hospitalization in office of cardiology is shown to patients with heavy heart failure, emergency – in cases of development of not stopped paroxysms of tachycardia, ventricular premature ventricular contraction, vibrating arrhythmia, tromboemboliya, hypostasis of lungs.
Patients with cardiomyopathies need decrease in physical activity, observance of a diet with limited consumption of animal fats and salts, an exception of harmful surrounding factors and habits. These actions significantly reduce load of a cardiac muscle and slow down progressing of heart failure.
At cardiomyopathies purpose of diuretics for reduction of pulmonary and system venous stagnation is expedient. At violations of a sokratimost and pump function of a myocardium warm glycosides are applied. For correction of a warm rhythm purpose of antiarhythmic medicines is shown. Use of anticoagulants and antiagregant in treatment of patients with cardiomyopathies allows to prevent tromboembolichesky complications.
In exclusively hard cases carry out surgical treatment of cardiomyopathies: a septalny miotomiya (a resection of the hypertrophied site of an interventricular partition) with prosthetics of the mitralny valve or transplantation of heart.
The forecast at cardiomyopathies
Concerning the forecast the current of cardiomyopathies is extremely adverse: heart failure steadily progresses, the probability of arhythmic, tromboembolichesky complications and sudden death is high. After diagnostics of a dilatatsionny cardiomyopathy 5-year survival makes 30%. At systematic treatment stabilization of a state for an indefinite term is possible. The cases exceeding 10-year survival of patients after carrying out operations of transplantation of heart are observed.
Surgical treatment of a subaortal stenosis at a hypertrophic cardiomyopathy though yields undoubted positive result, but is accompanied by high risk of death of the patient in time or soon after operation (every 6th operated dies). To the women sick with cardiomyopathies, it is necessary to refrain from pregnancy in view of high probability of maternal mortality. Measures for specific prevention of cardiomyopathies are not developed.