RESUMO
Non-compacted left ventricle in adults is a rare occurrence, though it is diagnosed even more rarely. As a rule in patients with non-compacted left ventricle (LVNC) other pathologic condition is diagnosed, notably hypertrophic or dilated cardiomyopathy. The majority of LVNC cases are diagnosed in early infancy but currently there are asymptomatic cases detected by means of echocardiographic examination. Real prevalence of LVNC is unknown. According to many authors LVNC occurs in 9.2-9.5% of children with diagnosed cardiomyopathies. The majority of such children do not survive till adulthood because of progressive severe heart failure, fatal arrhythmias and thromboembolisms. This value ranges from 0.014 to 0.05% in adult population. The article presents a clinical case illustrating the stages in establishing the diagnosis of non-compacted left ventricle in a young patient with myocardial infarction and congestive heart failure. Common characteristics of non-compacted left ventricle and connective tissue dysplasia syndrome in the patient suggested etiopathogenetic relationship between these two pathologic states. The basic common characteristic feature of both non-compacted left ventricle and connective tissue dysplasia syndrome proved to be multiple abnormal chords of the left ventricle. The patient was supposed to have some coronary circulation abnormality inherited together with non-compacted left ventricle and connective tissue dysplasia syndrome. Adverse prognosis and high mortality in non-compacted left ventricle require its early recognition and differentiated approach to treatment depending on the severity of the disease and using all modern methods of treatment both conservative and surgical.