[Isolated left ventricular noncompaction--first description in a Hungarian patient].

The case of a young female patient is presented in whom the typical diagnostic features of isolated ventricular noncompaction were recognized by two-dimensional echocardiography after 11 year follow-up. This rare cardiomyopathy is characterized by prominent trabeculations of the apex and the distal lateral wall of the left ventricle, systolic dysfunction, restrictive dysfunction, functional mitral and tricuspid insufficiency. Literature review reveals the underlying abnormality of the myocardial embryogenesis with a delayed presentation in adulthood and variable clinical manifestations ranging from long asymptomatic periods to malignant courses including sudden death, transplantation and decreased survival.