- HCM: the disease
- What is Hypertrophic Cardiomyopathy (HCM)?
- How common is Hypertrophic Cardiomyopathy?
- History and other names
- How does Hypertrophic Cardiomyopathy affect the heart?
- When Does Hypertrophic Cardiomyopathy Develop?
- What is "obstruction"in HCM?
- Atrial fibrillation in HCM
- How is Hypertrophic Cardiomyopathy Diagnosed?
- Heart Failure in HCM
- What Symptoms Does Hypertrophic Cardiomyopathy Cause?
- How to screen for HCM
- Treatment Options
- HCM: Genetics
- Is a Cure Available?
- After a Loss
- HCM Spectrum Disorders
History and other names
It is confusing, remarkably, this disease (hypertrophic cardiomyopathy) has been given 75 separate names by individual investigators over the last 50 years. Literally, no other disease can make that claim!
Why has this occurred? The principal reason for the proliferation of names has undoubtedly been the heterogeneity and diversity in which the disease is expressed. Few individual investigators have seen large numbers of patients with hypertrophic cardiomyopathy, and therefore individuals have come to regard the overall disease based on their own (sometimes limited) experience. Many of the names are somewhat misleading since they emphasize obstruction to left ventricular outflow which is a highly visible feature of the disease but is probably present in no more than about 25% of all patients. These names include IHSS (Idiopathic Hypertrophic Subaortic Stenosis) which was the first popular term used in the United States; “stenosis” means obstruction. The same can be said for HOCM (Hypertrophic Obstructive Cardiomyopathy) which is still used in the United Kingdom…largely out of habit and convenience.
Other names that may still appear in some medical records: ASH (Asymmetric Septal Hypertrophy), Brock's Disease, SAS (Subaortic Hypertrophic Stenosis), Familial Hypertrophic Cardiomyopathy - for a complete list see "A guide to HCM" available with membership.
Nevertheless, virtually all HCM experts and other cardiovascular specialists now regard as the best single name for this broad disease spectrum — Hypertrophic Cardiomyopathy or HCM. This term emphasizes the hypertrophy which is the diagnostic marker in most patients and the fact that this disease is a form of cardiomyopathy — or heart muscle disorder… without mentioning obstruction. Therefore, it is preferable to describe the disease as either “HCM with obstruction” or “HCM without obstruction.”
“Cardiomyopathy” itself is a very general term referring to any condition (and there are many) importantly affecting the heart muscle itself while “hypertrophic cardiomyopathy” refers to a specific and genetic condition which usually shows a familial pattern. The most characteristic feature of HCM is a hypertrophied left ventricle (asymmetric thickening of the wall usually most prominently involving the ventricular septum) without abnormal enlargement of the ventricular cavities.
History - The first patient in the USA diagnosed with HCM was Mr. Claude Brady - a salesman from Virginia. In 1959 Dr. Eugene Braunwald the diagnosis was made after a review Mr. Bradys siblings. Interestingly Mr. Brady lived a relatively normal life until 1988 when he underwent a heart transplant, today, Mr. Brady is still living with his transplanted heart.
Maron, B.J. and Salberg, L. Hypertrophic Cardiomyopathy: For patients, their families and interested physicians. Blackwell Futura: 1 st edition 2001,81 pages; 2ndedition 2006, 113 pages; 3rd 204
Gersh, B.J., Maron, B.J., Bonow, R.O., Dearani, J.A., Fifer, M.A., Link, M.S., et al. (2011). 2011 ACCF/AHA guidelines for the diagnosis and treatment of hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation/American Heart Asociation Task Force on practice guidelines. Journal of the American College of Cardiology and Circulation, 58, e212-260.