Restrictive cardiomyopathy

Description:

The restrictive cardiomyopathy represents a rare disease of a myocardium with frequent involvement in process of an endocardium which is characterized by disturbance of filling of one or both ventricles with reduction of their volume at not changed thickness of walls. It can be idiopathic or caused by infiltrative or general diseases. Carry a fibroplastic parietal endocarditis to an idiopathic restrictive kardiomiyoopatiya as a disease of a myocardium of an unknown etiology (or an endomyocardial disease) Lefflera and endomyocardial fibrosis of Davies. The fibroplastic endocarditis of Leffler is combined with the permanent increase in maintenance of eosinophils in peripheral blood which is not connected with any known reason or a general disease. Endomyocardial fibrosis proceeds without eosinophilia. In view of similarity of morphological changes in a myocardium, pathophysiological mechanisms of disturbance of a cardiohemodynamics and clinic now most of authors consider both diseases as options of the same pathology of a myocardium and an endocardium, in both cases, obviously, caused by toxic influence of eosinophils. For their designation also terms an endomyocardial disease with an eosinophilia and an endomiokardalny disease without eosinophilia were widely adopted.

Epidemiology. The fibroplastic endocarditis of Leffler meets generally in the countries a temperate climate and is very rare disease. Endomyocardial fibrosis affects almost only inhabitants of tropics, is more rare than subtropics, and is most widespread in Uganda, Nigeria, the southern India, Sri Lanka where about 10 — 15% of cases of death of congestive heart failure fall to its share. Though in literature residents of Western Europe and the USA have many messages on cases of this disease, most of such patients a long time lived in the tropical countries.

Reasons of a restrictive cardiomyopathy:

The reason of an endomyocardial disease is not finalized. The assumption of an etiological role of parasitic and other infections was not confirmed. There are data on communication of prevalence of endomyocardial fibrosis with contents in the soil of monazites and accumulation in a myocardium and an endocardium of sick certain representatives of this family of chemical elements, in particular the thorium and cerium capable to stimulate collagen synthesis with heart fibroblasts. The leading role in a pathogeny is assigned to the activated eosinophils which easily are exposed to degranulation under the influence of the particles covered with immunoglobulins G and the SZ component of system of a complement. The cationic proteins which are released at the same time cause damage mio-and an endocardium and stimulate proliferation of connecting fabric. Perhaps, the endomyocardial disease, like an idiopathic dilatatsionny cardiomyopathy, represents the general final stage of the damage of a myocardium caused by several various etiological factors.

Pathological anatomy. Macroscopically the sizes of heart are increased slightly, the hypertrophy is practically absent. The left ventricle is more often involved in pathological process, but there can be also an isolated defeat right or both ventricles. The sharp thickening of an endocardium is very characteristic, is preferential in the field of ways of inflow of blood and a top, with trombotichesky imposings that can lead to reduction of a cavity of a ventricle, sometimes considerable (the outdate term from here — a regional iterative cardiomyopathy). Fibrosis of atrioventricular valves, papillary muscles and the endocardium covering them leads to development of mitral insufficiency and insufficiency of the three-leaved valve.

At a histologic research of heart of the patient with a fibroplastic endocarditis allocate three stages of defeat. For the first, necrotic, the expressed eosinophilic infiltration of a myocardium with development of myocarditis and a coronaritisis is characteristic. Within about 10 months eosinophils gradually disappear from the centers of an inflammation and there comes the trombotichesky stage. It is shown by an endocardium thickening owing to fibrinoid changes and trombotichesky imposings and thrombosis of small coronal arteries. On average in 24 months the fibrosis stage with a characteristic considerable thickening of connective tissue elements of an endocardium and widespread intersticial fibrosis of a myocardium which, despite the accompanying nonspecific obliterating endarteritis of intramural coronal arteries, has preferential nekoronarogenny origin develops.

Pathogeny:

Disturbances of a cardiohemodynamics are characterized by development of a so-called syndrome of restriction, sharp increase in elastic rigidity of a ventricle is its cornerstone owing to what after the short period of the bystry filling provided with substantial increase of pressure, further intake of blood in a ventricle practically stops so its volume during the second half of a diastole significantly does not increase. It is followed by characteristic change of a curve of diastolic pressure in ventricles which gets a form of a square root (fig. 11) with considerable pressure decrease at the beginning of a diastole and the subsequent sharp rise with transition to the plateau. At the same time the expressed increase in level of intra ventricular KDD, pressure in system and pulmonary veins and systolic pressure in a pulmonary artery is defined. Contractility of a myocardium and systolic function of ventricles are usually kept what not changed sizes KDO (less ON cm3/sq.m) and FV (more than 50%) testify to.

Symptoms of a restrictive cardiomyopathy:

The main clinical manifestations are caused by the restriction syndrome which is characterized by venous stagnation of blood on the ways of inflow to the left and right ventricles, usually with dominance of stagnation of blood in a big circle of blood circulation. As a rule, patients are disturbed by the expressed asthma and the weakness at the slightest exercise stress connected with aggravation of disturbance of diastolic filling at tachycardia. Quickly the peripheral hypostases, a hepatomegalia and ascites often steady against the carried-out therapy accrue. At an objective research swelling of cervical veins, increase in TsVD which often increases on a breath (Kussmaul's sign) are noted. Paradoxical pulse can be defined. Discrepancy between weight of displays of heart failure and lack of a cardiomegaly attracts attention that else before performance of tool inspection allows to assume existence of diastolic dysfunction of ventricles. At auscultation of heart are noted a protodiastolic, presystolic or summatsionny cantering rhythm and in large part cases — also systolic noise of mitral insufficiency or insufficiency of the three-leaved valve. The disease often is complicated by atrial fibrillation and ectopic ventricular arrhythmias, and also thromboembolisms in arteries of a small and big circle of blood circulation which sources usually are blood clots in ventricles.

The fibroplastic endocarditis of Leffler often begins subacutely with system manifestations — fevers, weight loss, the skin rash, a pulmonitis, touch polyneuropathy which are followed by an eosinophilia. These manifestations are absent at endomyocardial fibrosis of which gradual development and progressing of congestive heart failure are characteristic.

Diagnosis:

At a thorax X-ray analysis the sizes of heart are not changed or a little increased. The cardiomegaly can be observed only at development of a hydrocardia. Also increase in one or both auricles and venous stagnation in lungs are noted.

Changes on an ECG meet often, but are deprived of specificity. Signs of a hypertrophy of auricles and ventricles, blockade left or right (more rare) ventriculonector legs, nonspecific disturbances of repolarization, various disturbances of a cordial rhythm can be registered.

At a dopplerekhokardiografiya dilatation and a hypertrophy of ventricles are absent, their sokratitelny ability is not changed, At a number of patients the cavity of ventricles is reduced at the expense of an obliteration of an apical segment where blood clots often are defined. Often liquid in a pericardium cavity comes to light, and at a Doppler research — the moderate regurgitation of blood from auricles in ventricles which is followed by dilatation of auricles, sometimes — moderate fibrosis of valves. Find signs of a valvulitis and vegetation on atrioventricular valves in a number of patients with a fibroplastic endocarditis of Leffler (F. Cetta and соавт., 1995, etc.) - are Very characteristic, but are not specific inherent to restrictive type of diastolic dysfunction increase in the maximum speed of early diastolic filling (E) which considerably prevails over that in the period of an auricular systole (Е/А> 1,5), and shortening of the period of delay of speed of early filling.

Other noninvasive methods of visualization of heart — such as computer and magnetic resonance tomography — use generally for an exception of chronic cardial compression at which the pericardium thickening not characteristic of a restrictive cardiomyopathy is noted.

At catheterization of heart are noted characteristic of a hemodynamic syndrome of restriction increase To Yes, in ventricles and change of a form of a curve of diastolic pressure in them { see fig. 11). At the same time owing to inhomogeneity of damage of a myocardium and an endocardium of the left and right ventricles of size of their KDD are usually not identical and differ more than on 5 mm of mercury. Disturbance of diastolic filling of ventricles at not changed indicators of systolic function is defined also at a X-ray contrast ventrikulografiya. At the same time at most of patients this or that degree of regurgitation of blood from auricles in ventricles is noted.

At an endomyocardial biopsy in an early inflammatory stage of a fibroplastic endocarditis of Leffler it is possible to find characteristic eosinophilic infiltrates, and in a late stage of a disease and at endomyocardial fibrosis — nonspecific changes in a type of more or less widespread interstadialny fibrosis and dystrophy of cardiomyocytes. At the same time the main role of a biopsy consists in an exception of chronic cardial compression and a restrictive cardiomyopathy owing to systemic and infiltrative lesions of a myocardium.

Increase in maintenance of eosinophils in peripheral blood is a distinguishing laboratory character of a fibroplastic endocarditis of Leffler (more 1,5-109/l) that often is followed by anemia and nonspecific inflammatory shifts.

Treatment of a restrictive cardiomyopathy:

In the presence of active inflammatory process in a myocardium and small vessels what the eosinophilia and system displays of a disease indirectly testify to, as means of pathogenetic therapy use glucocorticosteroids in combination with cytostatic immunosuppressants, mainly hydroxurea. After obtaining clinical effect pass to long reception of maintenance doses. Early enough the begun such therapy is capable to improve survival several. Widely use a symptomatic treatment of diastolic heart failure which in general is a little effective. It includes restriction of liquid and salt and very careful, in order to avoid deterioration in diastolic filling of ventricles and decrease in MOS, use of diuretics and peripheral vazodilatator — nitrates and APF inhibitors. Cardiac glycosides are effective only at a ciliary arrhythmia. For prevention and treatment of a thromboembolism use anticoagulants of indirect action.

At the expressed thickening of an endocardium and existence of signs of an obliteration of a cavity of a ventricle at a part of patients it is possible to execute an endokardektomiya which sometimes gives essential relief. At the expressed mitral insufficiency and insufficiency of the three-leaved valve resort to prosthetics or plastics of these valves that, however, is accompanied by a high lethality.

The forecast is adverse. A half of patients die within 2 years after emergence of the first symptoms of damage of heart. Prevention of a disease is not developed.