Position statement of Polish Cardiac Society experts on cardiomyopathy

Abstract

Cardiomyopathies (CMs) are a very broad group of diseases, including genetically determined and acquired, and their classification is based on phenotypic characteristics. There is always a need to search for the etiology (often also to try to identify the genetic cause), which may determine the appropriate choice of clinical management. The geographical distribution of genetic variants varies as does the prevalence across populations, ethnic groups, regions, and countries. The most reliable data on the distribution of individual genetic variants come from developed countries. The phenotypic classification includes 5 main types of CM, i.e., dilated CM, hypertrophic, restrictive, arrhythmogenic right ventricular CM, and non-dilated left ventricular (LV) CM. Individual CMs are characterized by a variety of causes and different phenotypic pictures, which affect their presentation, diagnosis, and response to treatment. Within each type of CM, there are both familial and sporadic (acquired) forms. The complex presentation of CM, as well as the limited availability of screening and diagnostic tests, causes CMs to be diagnosed late, often at an advanced stage of the disease. Therapeutic management of CM is strictly determined by its type and clinical picture. Diagnostics include the assessment of symptoms, the results of imaging and genetic tests, as well as morphological, functional, and often histological assessment. This allows for personalized and dedicated clinical management. To optimize thediagnosis, treatment, and care of patients with CMs, an individualized, expert, systemic, coordinated, and often multidisciplinary structure of care is necessary. Hence, it is important to create multidisciplinary teams for CM management. We present examples of existing systemic solutions for the care of CM patients in Europe (France and Spain). Not all of these options are available to patients in our country. This article presents issues related to CM and may be the basis for developing a diagnostic and therapeutic model allowing for earlier detection of CM in Polish patients and their effective treatment.

Keywords: Polish Cardiac Society; cardiomyopathy; position of experts.