Classification of cardiomyopathies. One goal,many proposals

Especial Miocardiopatías

Abstract

The importance of myocardial pathology as a cause of illness and death from cardiac origin is undeniable. Currently, almost half of the patients who die suddenly in childhood and adolescence or receive a heart transplant are affected by cardiomyopathy.
However, myocardial diseases have historically constituted a perplexing group, both in relation to their origin and in their nosological systematization.
Initially identified with inflammatory pathology, and then conceived as disorders of unknown cause or as a manifestation of multiple systemic diseases, cardiomyopathies were later categorized in certain patterns of morphological and functional presentation, and were object in the last 30 years of intense research in the field of basic sciences, which allowed to recognize the genetic origin of many of these entities.
Already in this century, that new information sustained classification initiatives by the American Heart Association (AHA) and the European Society of Cardiology (ESC), which despite being a valuable improvement over previous attempts, exhibit areas of uncertainty and substantive differences that are subject of debate. A more recent proposal, the MOGE (S) classification, stresses on the growing information provided by molecular genetics and on the implementation of a phenotype-genotype descriptive nosology that enables maximum accuracy in nomenclature and clinical diagnosis.
This chapter reviews the evolution of the concepts that sustained the successive classifications published, and analyzes the discrepancies between the proposals of the AHA and the ESC, concluding on the need for a joint approach to the problem in order to generate a coherent and universally shared taxonomic arrangement and nomenclature.