Internal illnesses. Restrictive cardiomyopathy (lecture notes)

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Internal illnesses. Restrictive cardiomyopathy (lecture notes)

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LECTURE № 18. Restrictive cardiomyopathy

Restrictive cardiomyopathy is a decrease in myocardial extensibility with limited filling of the left ventricle, a decrease in stroke volume and the development of heart failure. The group with these symptoms includes diseases with obliteration of the cavities of the ventricles, phenomena of atrioventricular regurgitation, parietal thrombosis. The disease is extremely rare, often considered as a complication of endomyocardial (eosinophilic) disease (Leffler's fibroplastic myocarditis) and endocardial fibrosis, fibroelastosis.

Pathological anatomy. The endocardium is primarily affected (except for endomyocardial fibrosis), and the myocardium is involved in later periods of the disease. Connective tissue develops, obliteration of the cavities of the ventricles and the development of parietal thrombosis occur, the proliferation of endothelial cells leads to occlusion of small vessels of the myocardium.

Clinic. Clinical manifestations are characterized by the appearance of complaints of shortness of breath, especially with physical exertion, chest pain, weight loss. In the initial periods, the boundaries of the heart are not changed, in the future there is an increase in the left or right ventricles (where fibrosis predominates), in the later periods there is an increase in toto. At the beginning, the tones are rhythmic, the third tone is heard, above the apex there is a systolic murmur. Symptoms of heart failure increase gradually.

Additional diagnostic study. An ECG study determines changes characteristic of diffuse myocardial damage. X-ray examination reveals dilatation of the heart cavities, venous congestion in the lungs. Echocardiography reveals early rapid filling of the ventricles.

A study of hemodynamic parameters is carried out, which establishes an increase in filling pressure in both ventricles, an increase in end-diastolic pressure in the left ventricle, and pressure in the pulmonary artery.

When ventriculography is determined by increased contraction of the ventricles, smooth contours of the walls, a possible filling defect in the apex during obliteration.

In life, the diagnosis can only be established by endomyocardial biopsy.

Complications. Complications include circulatory failure, thromboembolism, rhythm disturbances (less often than with hypertrophic cardiomyopathy).

Differential diagnostics. Should be performed with amyloidosis, hemochromatosis, sarcoidosis, scleroderma, Abramov-Fiedler idiopathic myocarditis (diagnosis is difficult even with autopsy).

Treatment. In the early stages of the disease in the presence of eosinophilia, corticosteroids are used. Diuretics, vasodilators, anticoagulants (for the prevention of thromboembolism), calcium antagonists are used: they affect the diastolic function of the left ventricle.

Surgical treatment is possible at the stage of fibrosis to remove the altered endocardium, prosthetics of the mitral and tricuspid valves.

Flow. The course of the disease is slowly progressive.

Forecast. With damage to the left ventricle - relatively favorable, with involvement in the process of the valvular apparatus - doubtful.

Author: Myshkina A.A.

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