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Fifty years ago, Donald Teare,1 a well-known forensic pathologist in London, reported 8 cases of “asymmetrical hypertrophy or benign tumor” of the heart. Each had disproportionate hypertrophy of the interventricular septum, a coarse myocardial texture, and a bizarre arrangement of muscle fibers, separated by excessive connective tissue and clefts. At the time, numerous names for this entity were proposed, but after a relatively short period, the term hypertrophic cardiomyopathy (HCM) was adopted and has remained the accepted nomenclature to this day. In this issue of Circulation: Cardiovascular Genetics , Maron et al2 propose a change to this convention by advocating a much more restrictive use of the term to describe myocardial hypertrophy caused by mutations in genes that encode sarcomere proteins. In our view, the rationale for this proposal is flawed and, following as it does on the tail of 2 recent updates of the cardiomyopathy classification, will result in increased confusion.Response by Maron et al see p 87 In a remarkably prescient article published in 1957, Brigden3 observed that when describing heart muscle disease “adjectives such as isolated, idiopathic, nonspecific, specific, interstitial, diffuse, and circumscribed abound in the literature; others, such as acute, subacute, chronic pernicious, and malignant, relate to the clinical picture; while still others, such as eosinophilic, allergic, idiosyncratic, and granulomatous hint at etiology, as does familial cardiomegal.” His view at the time was that the term cardiomyopathy should be used to denote “isolated noncoronary myocardial disease.” In 1961, Goodwin4,5 observed that “no definition of the cardiomyopathies is entirely satisfactory,” but suggested that it would be appropriate to consider cardiomyopathies as disorders of heart muscle “of unknown or obscure etiology, often with endocardial, and sometimes with pericardial involvement, but not atheroscerotic in origin.” In subsequent publications, the concept of primary myocardial disorders, …

How should hypertrophic cardiomyopathy be classified?