[Left ventricular noncompaction: A clinical and morphological study].

The data of clinical, macro- and micrometric, histological, and immunohistochemical studies of the heart were analyzed in patients with left ventricular noncompaction (LVNC). Materials from 7 patients: 5 hearts of recipients after heart transplantation, one heart of a dead patient, and one endomyocardial biopsy specimen were investigated. The investigations showed that this disease was accompanied by a preponderance of a noncompact layer with its ratio to a compact layer (2.4:6.6) in the left ventricle and by myocardial hypertrophy and fibrosis in all cases, by endocardial fibroelastosis and discomplexation of muscle fibers by more than 15% of the specimen area in 6 of the 7 cases, by right ventricular hypertrabeculation and myocarditis in 5 cases, and by lipomatosis and impaired connexin 43 expression in 4 cases. Only one of the four patients was found to have MYH 7 gene mutation. The results of MRI of the extracted heart coincided with morphological findings in 100% of cases. The comparative study demonstrated that this disease had simultaneously morphological features of both LVNC and restrictive, hypertrophic, dilated cardiomyopathy. The findings may suggest that the LVNC phenotype may be formed under the influence of various modifying factors (hemodynamic and inflammatory ones).

[Autoimmunity and Prognosis of Patients With Morphologically Documented Myocarditis].

AIM: to assess clinical and prognostic value of circulating cardiospecific autoantibodies (AAB) and CD4+ T-regulatory cells in patients with myocarditis. MATERIAL AND METHODS: We included into this study 47 patients with lymphocytic myocarditis verified by analysis of histological and immunohistochemical data. Comparison group consisted of 30 practically healthy persons. Content of marker cardiotropic AAT were measured with the help of standardized immune enzyme test-systems. Number of circulating CD4+CD25+ and CD4+CD25+FoxP3+ T-lymphocytes were evaluated by flow cytometry. RESULTS: Among factors determining prognosis of patients with lymphocytic myocarditis factors of key significance were the presence of clinically overt heart failure at the disease debut, and degree of reduction of left ventricular ejection fraction. Distinctive feature of active myocarditis was elevation of titer of AABs to sarcomeric, cytoskeleton, and cytoplasmic proteins of cardiomyocytes, as well as elevated level of AABs to various epitopes of adenine nucleotide translocator. Elevated level of AAB to 1-adrenoreceptors was an independent predictor of unfavorable outcome in patients with lymphocytic myocarditis. Increased population of circulating CD4+CD25+ T-regulatory cells was as sociated with elevated concentration of of natriuretic peptide. CONCLUSION: Abnormalities in the system of autoimmunity play key role not only in pathogenesis but also in prognosis of inflammatory myocardial diseases. Changes of profile of circulating cardiospecific AABs and T-regulatory cells can bear a protective function.