Cardiomyopathies: Classification, diagnosis and treatment modalities

Abstract

Cardiomyopathies (CM) are a heterogeneous group of diseases of various etiologies that lead to morphological and functional damage to the heart. Throughout history, there have been numerous attempts to classify them, and the latest division of the European Society of Cardiology (2008) has classified all CM into four basic phenotypes: dilated CM, hypertrophic CM, restrictive CM and arrhythmogenic CM of right ventricle. Other CM are classified as unclassified. According to the form of inheritance, they are divided into familial (genetic) and non-familial forms of the disease. The importance of CM is reflected not only in their high incidence in the general population but also in the very difficult clinical presentation at the time of their diagnosis. Complaints in most patients occur gradually and quietly, and the first manifestation of the disease can be some of the fatal complications (malignant arrhythmia or systemic embolization). Distinguishing CM from hypertensive, ischemic and valvular heart disease is a difficult differential diagnostic problem that needs to be solved due to different modalities of treatment of these conditions. CM is one of the most common causes of heart failure and terminal heart failure is the leading indication for heart transplantation. Therefore, the treatment of CM aims to reduce the symptoms of heart failure (following the latest guidelines for the treatment of heart failure) and improve heart function. The frequency of malignant heart rhythm disorders and sudden cardiac death is much more common in the population of patients with CM. In addition to standard drug therapy, immunosuppressive therapy, surgical procedures, (installation of pacemakers, implantable cardioverter defibrillators, septal myectomy, installation of temporary mechanical circulatory support devices (EKMO, Impella, LVAD) are increasingly used in the treatment of these patients. Given that a large number of CM are hereditary diseases and that their treatment is often complicated, preventive examinations of the population are very important in order to timely diagnose these diseases as well as genetic screening of close relatives suffering from CM.