Heart amyloidosis

Description:

Heart amyloidosis (amyloid cardiopathy) – a form of system disturbance of protein metabolism with preferential adjournment of protein of b-fibrillar structure (amyloid) in a myocardium, an endocardium, a pericardium, an aorta and walls of coronary vessels. Damage of heart can be observed at the following types of an amyloidosis: AL - an amyloidosis of light chains, SSA - a senile system amyloidosis, FAP - a family amyloidosis, AA - a secondary amyloidosis. At a generalized amyloidosis heart is the dominating target organ, however the isolated amyloidosis of auricles in certain cases can meet. Along with a heart amyloidosis, an amyloidosis of kidneys, lungs, spleens, intestines and other bodies most often occur among other clinical forms of a disease.

Heart amyloidosis reasons:

It is not possible to speak about trigger factors of an amiloidogenez in most cases. At a family amyloidosis the autosomal and dominant type of transfer of a disease takes place. The hereditary amyloidosis of heart meets at representatives of the Mediterranean ethnic group more often.
The secondary system amyloidosis which development can be accompanied by a long current of a pseudorheumatism, an ankylosing spondylitis, psoriasis arthritis, a bronchoectatic disease, bronchitis, tuberculosis, a lymphogranulomatosis, a disease Krone, syphilis, an actinomycosis is more studied. At patients with a chronic renal failure development of the amyloidosis associated with a hemodialysis is possible.

Pathogeny:

At the family, idiopathic and far come senile form of an amyloidosis damage of heart occurs at 80-100% of patients. Most often adjournment of amyloid happens in a muscular coat of heart, is more rare – in a pericardium and an endocardium. In a myocardium amyloid collects between myofibrils, leading to a prelum of intramural arteries and arterioles.
At a heart amyloidosis the myocardium is condensed, becomes low-extensible (a "rubber" myocardium); the volume of cardial cavities usually changes slightly. The senile amyloidosis of heart proceeds as diffusion defeat of muscle fibers that is followed by their atrophy; amyloid adjournment quite often comes to light in coronary arteries and an aorta. Disturbances of systolic and diastolic function, reduction of cordial emission and symptoms of heart failure are a consequence of structural changes of a myocardium.
In a case adjournment of amyloid in the field of valves the clinic of valve heart disease develops; at accumulation of fibrous protein in a ventriculonector, sinus and atrioventricular nodes 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 amyloidosis of heart taking into account extent of involvement of body in pathological process are allocated:
І – symptoms and signs of an amyloidosis of heart at noninvasive inspection or a biopsy do not come to light;
ІІ – damage of heart is confirmed by noninvasive testing (an ECG, EhoKG) or biopsies, however a current of an amyloidosis asymptomatic;
ІІІ – a stage of the compensated simptomny amyloidosis of heart;
ІV - a stage of a dekompensirovanny cardiomyopathy.

Heart amyloidosis symptoms:

The heart amyloidosis quite often masks under clinic of coronary heart disease, a hypertrophic cardiomyopathy that complicates timely recognition of an amyloid cardiopathy. In initial stages development of an amyloidosis of heart happens gradually, malosimptomno. Weight reduction, fatigue, irritability, dizziness, puffiness can be noted.
Sharp deterioration in health is usually preceded by the postponed intercurrent disease, including a respiratory infection, a stress, an emotional overstrain. In the developed stage of an amyloidosis of heart the lowered arterial pressure with the phenomena of orthostatic hypotension pays attention; the pains in heart having stenokardichesky character; syncopal states, arrhythmias. An asthma, a hepatomegalia, hypostases are signs of the developing chronic heart failure. Distinctive feature of heart failure at an amyloidosis of heart is its bystry progressing and resistance to therapy by cardiac glycosides.
Quite often the pericardiac exudate and ascites is found in patients with an amyloidosis of heart. Owing to amyloid infiltration the sick sinus syndrome develops that is followed by bradycardia and can lead to a sudden cardiac death.
If the amyloidosis of heart is one of manifestations of a system amyloidosis, at patients the renal failure, a dysphagy, diarrhea, a macroglossia, skin hemorrhages, a periorbital purpura, defeat of a nervous system can be observed. For a generalized amyloidosis typically consecutive accession all of the new and new symptoms creating a motley clinical picture with multiorgan manifestations.

Diagnosis:

Lack of pathognomonic symptoms complicates intravital diagnosis of an amyloidosis of heart. Often reliable data for an amyloid cardiopathy come to light only on autopsy.
At heart auscultation at patients with an amyloidosis dullness of cordial tones, systolic noise of regurgitation on atrioventricular valves are listened. On an ECG decrease in a voltage of QRS complexes, different disturbances of excitability and conductivity of a myocardium is registered.
It is necessary to carry a thickening of interventricular and interatrial partitions, walls of ventricles, heart valves, dilatation of auricles, a myocardium hypokinesia to EhoKG-priznakam of an amyloidosis of heart. Functional changes are characterized by sharp decrease in fraction of emission of a left ventricle, disturbance of diastolic filling, valve regurgitation. Sometimes in a myocardium it is possible to reveal ekhopozitivny inclusions in a look small the granules representing amyloid deposits.
On the roentgenogram of bodies of a thorax increase in a shadow of heart, exudative pleurisy can be defined. More highly as sensitive and specific methods of intravital diagnosis of an amyloidosis of heart serve the stsintigrafiya of a myocardium and MRT of heart.
Morphological confirmation of an amyloidosis of heart can be received by a biopsy of a myocardium or other struck bodies (language, a gingiva, a hypodermic fatty tissue, lymph nodes, a kidney, a rectum etc.).
In laboratory tests of urine and blood at an amyloidosis of heart the proteinuria, a hypoproteinemia, a hyperglobulinemia and a hypoalbuminemia is found.
Differential diagnosis of an amyloidosis of heart is carried out with a hereditary dystonic lipidosis (Fabri's disease), a myxedematous cardiomyopathy at a hypothyroidism, a gipertofichesky cardiomyopathy, an idiopathic restrictive cardiomyopathy, a pulmonary heart, hemochromatosis, an aorta stenosis, a sarcoidosis, glycogenoses.

Treatment of an amyloidosis of heart:

Treatment of patients with an amyloidosis of heart presents considerable difficulties. For the purpose of suppression of an amiloidogenez various schemes of polychemotherapy, immunodepressive drugs are used.
In most cases treatment of an amyloidosis of heart comes down to delay of progressing of heart failure. Symptomatic medicines – diuretics, cardiac glycosides, vitamins, anticoagulants are appointed.
At SSSU implantation of an artificial pacemaker can be used. Now in cardiology begin to apply transplantation of the struck bodies to treatment of an amyloidosis, including heart.
After establishment of the diagnosis patients with an amyloidosis of heart have to be under systematic observation of the cardiologist, hematologist, neurologist, nephrologist, etc. specialists.

Forecast:

Timely intravital diagnosis of an amyloidosis 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 amyloidosis.
Forecast of an amyloidosis 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 extra cardial complications.