Left ventricular noncompaction (LVNC) is a unique inherited cardiomyopathy, characterized by an increased risk of adverse cardiovascular events such as heart failure, arrhythmia or sudden cardiac death. Although in comparison to dilated cardiomyopathy, the number of clinical studies concerning LVNC is still small, it is quickly increasing, which reflects a huge effort of the cardiovascular society to develop data to improve understanding of this cardiomyopathy. However, the predictors of adverse outcomes in LVNC are not well established. The aim of this review is to systematize the available data obtained from the medical literature in order to establish a proper prognosis, so that affected patients can receive the most appropriate treatment. The review considers issues connected with various areas of risk in LVNC, referring to its incidence and prevalence, comorbidity, genetics, morphology, symptoms, thromboembolic events, incidence of arrhythmia, sudden cardiac death, and mortality. Beginning with a genetic approach to the disease, passing through diagnostic tools, and finishing with issues relating to invasive methods of treatment, the article points out the most important and valuable clues for predicting a poor prognosis in LVNC. The review confirms that LVNC is not a disease, but a type of cardiac abnormality laden with a variety of prognostic factors of poor outcomes in terms of life-threatening ventricular arrhythmia and progression of heart failure. Thus, establishing a proper prognosis for individual patients is crucial for implementing the most appropriate treatment, and it should be based on the outcomes of a variety of clinical tests.
Keywords: cardiovascular risk factors; left ventricular noncompaction; sudden cardiac death.