Amiloidoz of heart – system , leading to adjournment of an amiloid – specific proteinaceous a complex in heart tissues. Amiloidoz of heart is followed by a myocardium hypertrophy, violation of its sokratitelny ability, development of chronic insufficiency of blood circulation, arrhythmias, hypotensions, valvate defects. The clinical diagnosis of an amiloidoz of heart is established by means of data of an electrocardiography, echocardiography, X-ray analysis, stsintigrafiya, a laboratory research of proteins of serum of blood, a biopsy of fabrics. Treatment at an amiloidoza of heart is directed to braking of an amiloidogenez (polychemotherapy) and delay of progressing of heart failure (diuretics, warm glycosides, vitamins).
Amiloidoz of heart
Amiloidoz of heart (an amyloid kardiopatiya) – a form of system violation of proteinaceous exchange with primary adjournment of protein of b-fibrillyarnoy of structure (amiloid) in a myocardium, an endokarda, a pericardium, an aorta and walls of coronary vessels. Damage of heart can be observed at the following types of an amiloidoz: AL - an amiloidoza of easy chains, SSA - a senile system amiloidoz, FAP - a family amiloidoz, AA - a secondary amiloidoz. At a generalized amiloidoz heart is the dominating target organ, however in certain cases can meet isolated auricles. Along with amiloidozy hearts, most often occur among other clinical forms of a disease kidneys, lungs, a spleen, intestines and other bodies.
Reasons of an amiloidoz of heart
It is not possible to speak about trigger factors of an amiloidogenez in most cases. At a family amiloidoz the autosomno-prepotent type of transfer of a disease takes place. Hereditary hearts meets at representatives of the Mediterranean ethnic group more often.
It is more studied secondary system which development can be accompanied by the long course of rheumatoid arthritis, an ankiloziruyushchy spondilit, psoriatichesky arthritis, bronkhoektatichesky disease, bronchitis, tuberculosis, a limfogranulematoz, a disease Krone, syphilis, actinomycosis. At patients with a chronic renal failure development of the amiloidoz associated with a hemodialysis is possible.
Pathogenesis of an amiloidoz of heart
At the family, idiopathic and far come senile form of an amiloidoz damage of heart occurs at 80-100% of patients. Most often adjournment of an amiloid happens in a muscular cover of heart, is more rare – in a pericardium and . In a myocardium collects between miofibrilla, leading to a sdavleniye of intramuralny arteries and .
At a heart amiloidoza the myocardium is condensed, becomes low-extensible (a "rubber" myocardium); the volume of cavities of heart usually changes slightly. Senile hearts proceeds as diffusion defeat of muscle fibers that is followed by their atrophy; adjournment of an amiloid quite often comes to light in coronary arteries and an aorta. Violations of systolic and diastolic function, reduction of warm emission and symptoms of heart failure are a consequence of structural changes of a myocardium.
In a case adjournment of an amiloid in the field of valves the clinic of valvate heart disease develops; at a congestion of fibrillyarny protein in Gis's bunch, sinusovy and atrioventricular knots to the forefront there are symptoms of blockade of the carrying-out system of heart.
In the classification developed by WHO 4 stages of an amiloidoz of heart taking into account extent of involvement of body in pathological process are allocated:
- І – symptoms and signs of an amiloidoz of heart at noninvasive inspection or a biopsy do not come to light;
- ІІ – damage of heart is confirmed by noninvasive testing (the ECG, EhoKG) or biopsies, however a current of an amiloidoz asymptomatic;
- ІІІ – a stage of the compensated simptomny amiloidoz of heart;
- ІV - a stage of a dekompensirovanny cardiomyopathy.
Symptoms of an amiloidoz of heart
Amiloidoz of heart quite often masks under clinic of coronary heart disease, a hypertrophic cardiomyopathy that complicates timely recognition of an amyloid kardiopatiya. In initial stages development of an amiloidoz of heart happens gradually, malosimptomno. Weight reduction, fatigue, irritability, dizziness, puffiness can be noted.
Sharp deterioration in health is usually preceded by the postponed interkurentny disease, including a respiratory infection, a stress, an emotional overstrain. In the developed stage of an amiloidoz of heart the lowered arterial pressure with the phenomena of orthostatic hypotension pays attention; the pains in heart having stenokardichesky character; sinkopalny states, arrhythmias. Short wind, a gepatomegaliya, hypostases are signs of the developing chronic heart failure. Distinctive feature of heart failure at an amiloidoza of heart is its bystry progressing and resistance to therapy by warm glycosides.
Quite often the pericardiac exudate and ascites is found in patients with amiloidozy hearts. Owing to amyloid infiltration the syndrome of weakness of sinusovy knot develops that is followed by bradycardia and can lead to sudden warm death.
In case hearts is one of manifestations of a system amiloidoz, at patients the renal failure, a dysphagy, diarrhea, a makroglossiya, skin gemorragiya, periorbitalny purple, defeat of nervous system can be observed. For a generalized amiloidoz typically consecutive accession all of the new and new symptoms creating a motley clinical picture with polyorgan manifestations.
Diagnostics of an amiloidoz of heart
Lack of patognomonichny symptoms complicates lifetime diagnostics of an amiloidoz of heart. Often reliable data for an amyloid kardiopatiya come to light only on autopsy.
At a heart auskultation at patients with amiloidozy dullness of warm tones, systolic noise of a regurgitation on predserdno-ventricular valves are listened. On the ECG decrease in a voltage of QRS complexes, different violations of excitability and conductivity of a myocardium is registered.
It is necessary to carry a thickening of interventricular and mezhpredserdny partitions, walls of ventricles, heart valves, dilatation of auricles, a myocardium gipokineziya to EhoKG-priznakam of an amiloidoz of heart. Functional changes are characterized by sharp decrease in fraction of emission of the left ventricle, violation of diastolic filling, a valvate regurgitation. Sometimes in a myocardium it is possible to reveal ekhopozitivny inclusions in a look small the granules representing deposits of an amiloid.
On the roentgenogram of bodies of a thorax increase in a shadow of heart, ekssudativny pleurisy can be defined. More highly as sensitive and specific methods of lifetime diagnostics of an amiloidoz of heart serve the stsintigrafiya of a myocardium and MRT of heart.
Morphological confirmation of an amiloidoz of heart can be received by a biopsy of a myocardium or other struck bodies (language, a gum, hypodermic and fatty cellulose, lymph nodes, a kidney, a rectum etc.).
In laboratory tests of urine and blood at an amiloidoza of heart the proteinuria, a gipoproteinemiya, a giperglobulinemiya and a gipoalbuminemiya is found.
Differential diagnostics of an amiloidoz of heart is carried out with a hereditary dystonic lipidoz (Fabri's disease), a miksedematozny cardiomyopathy at a hypothyroidism, a gipertofichesky cardiomyopathy, an idiopathic restrictive cardiomyopathy, pulmonary heart, hemochromatosis, an aorta stenosis, sarkoidozy, glikogenoza.
Treatment of an amiloidoz of heart
Treatment of patients with amiloidozy hearts presents considerable difficulties. For the purpose of suppression of an amiloidogenez various schemes of polychemotherapy, immunosuppressive medicines are used. In most cases treatment of an amiloidoz of heart comes down to delay of progressing of heart failure. Symptomatic medicines – diuretics, warm glycosides, vitamins, anticoagulants are appointed.
At SSSU implantation of the artificial driver of a rhythm can be used. Now in cardiology begin to apply transplantation of the struck bodies to treatment of an amiloidoz, including heart. After establishment of the diagnosis patients with amiloidozy of heart have to be under systematic observation of the cardiologist, hematologist, neurologist, nephrologist, etc. experts.
The forecast at a heart amiloidoza
Timely lifetime diagnostics of an amiloidoz of heart and its effective treatment are a complex problem in cardiological practice. It is connected with rather rare prevalence of pathology, not expressiveness of specific symptoms in clinic of a disease, lack in Russia of the specialized centers dealing with problems of an amiloidoz.
Forecast of an amiloidoz of heart adverse: heart failure is inclined to steady progressing. Death of patients occurs on average in 1,5-2,5 after emergence of signs of damage of heart from heart failure or from vnekardialny complications.