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Controversies in Cardiovascular Genetics

How should hypertrophic cardiomyopathy be classified?

What’s in a Name? Dilemmas in Nomenclature Characterizing Hypertrophic Cardiomyopathy and Left Ventricular Hypertrophy

Barry J. Maron, MD; Christine E. Seidman, MD; Michael J. Ackerman, MD, PhD; Jeffrey A. Towbin, MD; Martin S. Maron, MD; Steve R. Ommen, MD; Rick A. Nishimura, MD and Bernard J. Gersh, ChB, MB, DPhil

From the Hypertrophic Cardiomyopathy Center (B.J.M.), Minneapolis Heart Institute Foundation, Minneapolis, Minn; Department of Genetics (C.E.S.), Harvard Medical School, Boston, Mass; Departments of Medicine and Pediatrics/Divisions of Cardiovascular Diseases and Pediatric Cardiology (M.J.A., S.R.O., R.A.N., B.J.G.), Mayo Clinic, Rochester, Minn; Department of Pediatrics and Cardiology (J.A.T.), Baylor College of Medicine, Houston, Tex; and Hypertrophic Cardiomyopathy Center (M.S.M.), Tufts Medical Center, Boston, Mass.

Correspondence to Barry J. Maron, MD, Director, Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, 920 E 28th Street, Suite 620, Minneapolis, MN 55407. E-mail hcm.maron@mhif.org

An extract of the first 250 words of the full text is provided, because this article has no abstract.

	Introduction

 
Almost 50 years of study have underscored the considerable heterogeneity in clinical presentation, natural history, morphology, and the genetic substrates that characterize inherited hypertrophic cardiomyopathy.^1–5 Similarly, the nomenclature applied to this complex cardiac disease has persistently created a measure of confusion for both patients and physicians with regard to the definition and perceptions of the disease. This ambiguity has had, in turn, substantial impact on accurate diagnosis, full understanding of disease expression, and ultimately, decisions regarding management. At this juncture, it would seem appropriate and timely to conceptually revisit and clarify this important issue of names, consistent with the evolving knowledge of the cardiomyopathies.⁶

Response by Elliott and McKenna see p 81

	Historical Context

 
The historical confusion over the names used to describe the entity of hypertrophic cardiomyopathy has arisen over the years in 2 fundamental areas. The first of these concerns the nomenclature used in prior efforts to characterize the disease. For example, at last count there are more than 80 individual names that have been used over the last 5 decades (most by early investigators) to describe the disease entity, which is the subject of this commentary (ie, hypertrophic cardiomyopathy)⁷ (Figure 1). It is likely that the confusing array of designations used to describe hypertrophic cardiomyopathy has also undoubtedly contributed to its relatively low visibility among the general public despite a prevalence (ie, 1:500), which greatly exceeds that of many other better known but less common cardiac or noncardiac diseases^8,9 (Figure 2).

View larger version (69K):   Figure 1. The multitude . . . [Full Text of this Article]

 

Related Article

How should hypertrophic cardiomyopathy be classified?: What’s in a Name? Dilemmas in Nomenclature Characterizing Hypertrophic Cardiomyopathy and Left Ventricular Hypertrophy

Barry J. Maron, Christine E. Seidman, Michael J. Ackerman, Jeffrey A. Towbin, Martin S. Maron, Steve R. Ommen, Rick A. Nishimura, and Bernard J. Gersh
Circ Cardiovasc Genet 2009 2: 81-86. [Extract] [Full Text] [PDF]

This article has been cited by other articles:

				P. Elliott and W. J. McKenna How should hypertrophic cardiomyopathy be classified?: Molecular Diagnosis for Hypertrophic Cardiomyopathy: Not Ready for Prime Time Circ Cardiovasc Genet, February 1, 2009; 2(1): 87 - 89. [Full Text] [PDF]