Idiopathic myocarditis of Abramov-Fidlera – the heavy nonspecific inflammation of a myocardium proceeding with development of a kardiomegaliya, heart failure, the expressed violations of a rhythm and conductivity, a tromboembolichesky syndrome. Clinical displays of myocarditis of Abramov-Fidlera include short wind, fatigue, cyanosis, tachycardia, interruptions in work of heart, temperature increase of a body. Diagnosis of myocarditis of Abramov-Fidlera is carried out taking into account laboratory data, a X-ray analysis of a thorax, an echocardiography, an electrocardiography, coronary angiography, a stsintigrafiya, MRT, a myocardium biopsy. Treatment of myocarditis of Abramov-Fidlera – mainly, symptomatic (nitrates, diuretics, APF inhibitors, beta-blockers, anticoagulants); according to indications – transplantation of heart.
Idiopathic myocarditis of Abramov-Fidlera
Idiopathic myocarditis of Abramov-Fidlera (myocarditis malignant, isolated, allergic, interweft) - the myocarditis with not clear etiology differing in profound diffusion inflammatory, dystrophic and degenerate changes of a cardiac muscle.
In cardiology Abramov-Fidlera's myocarditis is regarded as extremely severe form of not rheumatic myocarditis with a high lethality. With Abramov-Fidlera's myocarditis young and rather healthy people usually get sick. Average age of patients with idiopathic myocarditis makes 42 years; a percentage ratio of persons of both sexes among the diseased approximately identical. The disease is for the first time described by the Russian doctor S. S. Abramov in 1887; it is allocated in a separate nosological form in 1897 by the German clinical physician K. Fidler.
Reasons of idiopathic myocarditis of Abramov-Fidlera
The name of myocarditis of Abramov-Fidlera – "idiopathic" - indicates not clear etiology of a disease. A number of researchers (A. I. Abrikosov, Ya. L. Rapoport, etc.) proved the allergic nature and the autoallergichesky mechanism of development of myocarditis of Abramov-Fidlera. Quite often its beginning is preceded by a serumal disease, eczema, a medicinal allergy. In recent years the role of other possible trigger factors starting mechanisms of idiopathic myocarditis, in particular, of a viral infection and autoimmune reactions is discussed.
The hypothesis of virus genesis of myocarditis of Abramov-Fidlera is confirmed by statistical data: at the patients who had sharp viral myocarditis, chronic idiopathic myocarditis arises in 4-9% of cases against 0,005% in the general population. There is a point of view that Abramov-Fidlera's myocarditis represents extreme option of infectious and allergic myocarditis.
Approximately at 20% of patients development of myocarditis of Abramov-Fidlera is noted against the background of autoimmune diseases: Takayasu's diseases, Hashimoto's tireoidit, disease Krone. For the immunopathological mechanism of an inflammation identification of antibodies to a myocardium and cellular cytotoxicity testifies.
Typical morphological features of myocarditis of Abramov-Fidlera are: the isolated damage of heart, a combination of extensive dystrophic, infiltrative and inflammatory changes of a myocardium to a widespread cardiosclerosis, an intracardial tromboobrazovaniye and an embolism of arteries of a big circle of blood circulation.
Macroscopically at Abramov-Fidlera's myocarditis flabbiness of walls and stretching of cavities of heart, existence of pristenochny blood clots is found; on a section - diversity of coloring of a myocardium. At microscopic studying the hypertrophy of muscle fibers pays attention (more - sosochkovy muscles and subendokardialny layers of a myocardium); extensive fields of a mioliz with replacement of muscular tissue connecting; existence of signs of a koronarit - inflammatory infiltrates on the course of small branches of coronary vessels.
Classification of idiopathic myocarditis of Abramov-Fidlera
On the basis of histologic signs allocate four options (type) of idiopathic myocarditis: dystrophic, inflammatory and infiltrative, mixed and vascular. At dystrophic (destructive) type of myocarditis of Abramov-Fidlera processes of gidropichesky dystrophy of muscle fibers with their subsequent total death and miolizy on an affected area prevail.
The inflammatory and infiltrative option of idiopathic myocarditis is characterized by hypostasis of interweft fabric and its infiltration by various cellular elements. In a sharp stage in exudate in a large number polimorfnoyaderny neytrofilny granulocytes or eozinofilny leukocytes are found. At a long current as a part of infiltrate plasmatic or multinuclear huge cages prevail.
The mixed type of myocarditis of Abramov-Fidlera represents a combination of dystrophic and inflammatory and infiltrative options. The vascular type of myocarditis of Abramov-Fidlera proceeds with primary defeat of small branches of coronary arteries.
On a current Abramov-Fidlera's myocarditis can be sharp (2-8 weeks), subsharp (from 3 to 18 months) and recidivous chronic (lasts for years). Less often the latent form of idiopathic myocarditis proceeding without accurate symptomatology meets. On clinical signs distinguish the asistolichesky, arhythmic, tromboembolichesky, pseudo-coronary and mixed forms of myocarditis of Abramov-Fidlera.
Symptoms of idiopathic myocarditis of Abramov-Fidlera
Abramov-Fidlera's myocarditis differs in heavy, quite often malignant current. At a sharp form symptoms of the right ventricular or progressing total heart failure quickly accrue. There are short wind, , cyanosis, tachycardia, arterial hypotonia, hypostases, ascites, a gepatomegaliya and a splenomegaliya. Development of cardiac asthma and hypostasis of lungs is possible. Quite often there are kardialny pains reminding stenocardia. In a sharp stage temperature increase of a body to 38 — 39 °C is noted.
Sometimes the tromboembolichesky syndrome happens the leader in clinic; in this case the disease can demonstrate with TELA, a thrombembolia of cerebral, kidney, splenic vessels. The lightning course of myocarditis of Abramov-Fidlera always comes to an end with a lethal outcome.
At a chronic form of myocarditis of Abramov-Fidlera course of a disease recidivous; after each aggravation myocardium fibrosis, a kardiomegaliya and irreversible violation of blood circulation steadily progresses. Cases of a latent course of myocarditis of Abramov-Fidlera leading to sudden death are known.
Diagnosis of idiopathic myocarditis of Abramov-Fidlera
At fizikalny inspection pulse is noted frequent, arrhythmic, weak filling; perkutorny expansion of borders of heart; auskultativny phenomena (a gallop rhythm, systolic noise in a heart top projection), dullness of warm tones and so forth. In lungs melkopuzyrchaty damp rattles, a krepitation are listened. Peripheral hypostases, increase and morbidity of a liver, ascites are signs of total heart failure.
Biochemical blood test at Abramov-Fidlera's myocarditis finds increase in S-jet protein, content of fibrin, a seromukoid, gaptoglobin, a-and γ-globulins, activities of a kreatinkinaza and a troponin of T. For an exception of system diseases rheumatologic screening is carried out (ASL-O, a rheumatoid factor, antibodies to ds DNA and anti-nuclear antibodies).
The X-ray analysis of bodies of a thorax reveals significant increase in the sizes of heart, signs of venous stagnation in lungs. The echocardiography allows to exclude other reasons of heart failure ( hearts, congenital heart diseases and so forth); to find a pericardiac exudate, dilatation of cavities of heart, a myocardium hypertrophy, intracavitary blood clots; to estimate mobility and thickness of walls.
ECGs changes at Abramov-Fidlera's myocarditis are not specific: sinusovy tachycardia, incomplete blockade of legs of a bunch of Gis, atrioventricular blockade, ventricular arrhythmia, fibrillation of ventricles can be found, vibrating arrhythmia and premature ventricular contraction is more rare; in case of myocardium tissue damage - the psevdoinfarktny picture demanding performing differential diagnostics with IBS and a heart attack of a miokaryod.
Data of coronary angiography at Abramov-Fidlera's myocarditis confirm the coronary ischemia caused by cardiac dysfunction. For detection of an inflammation of a cardiac muscle highly specific and sensitive methods are the antimiozinovy stsintigrafiya gadoliniumno-improved by heart MRT.
As the standard procedure of diagnosis of myocardites of various etiology serves the myocardium biopsy, however this method is interfaced with high frequency both false positive, and false-negative results.
Treatment of idiopathic myocarditis of Abramov-Fidlera
Etiotropny therapy of myocarditis of Abramov-Fidlera is not developed. Symptomatic treatment of heart failure, arrhythmias, prevention of tromboembolichesky complications is carried out. The bed rest, a dietotherapy, reception of medicines are for this purpose appointed (nitrates, warm glycosides, diuretics, APF inhibitors, beta-blockers, antagonists of aldosteronovy receptors, anticoagulants).
Anti-inflammatory therapy includes purpose of NPVS (indometacin, diclofenac); at severe forms of myocarditis of Abramov-Fidyolera with high activity of immune reyoaktion glucocorticoids are applied; at an allergic background - the desensibilizing means. Medicines of metabolic action are in addition used (cocarboxylase, inosine, potassium , vitamins). At gigantokletochny myocarditis transplantation of heart is shown.
Forecast of idiopathic myocarditis of Abramov-Fidlera
The course of myocarditis of Abramov-Fidlera is in most cases adverse, mortality is extremely high. Sharp forms of a disease can come to an end letalno within several days or weeks, subsharp - within several months. Death comes from an acute heart failure, fibrillation of ventricles, is more rare - tromboembolichesky complications.
Chronic and latent options of a course of idiopathic myocarditis lead to progressing of a kardiomegaliya and irreversible violation of blood circulation. Specific prevention of myocarditis of Abramov-Fidlera is not developed.