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which is also known as '''nonobstructive hypertrophic cardiomyopathy''' and '''Japanese variant hypertrophic cardiomyopathy''' or the '''Yamaguchi variant''' (since the first cases described were all in individuals of Japanese descent).
which is also known as '''nonobstructive hypertrophic cardiomyopathy''' and '''Japanese variant hypertrophic cardiomyopathy''' or the '''Yamaguchi variant''' (since the first cases described were all in individuals of Japanese descent).


'''Hypertrophic cardiomyopathy''' is a disease of the [[myocardium]] (the [[muscle]] of the [[heart]]) in which a portion of the myocardium is [[left ventricular hypertrophy|hypertrophied]] (thickened) without any obvious cause.<ref name="Richardson, McKenna et al. 1996">{{cite journal |author=Richardson P, McKenna W, Bristow M, ''et al.'' |title=Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies |journal=Circulation |volume=93 |issue=5 |pages=841–2 |year=1996 |month=Mar |pmid=8598070 |doi= |url=http://circ.ahajournals.org/cgi/content/full/93/5/841}}</ref><ref name="Maron 2002">{{cite journal |author=Maron BJ |title=Hypertrophic cardiomyopathy: a systematic review |journal=[[Journal of the American Medical Association|JAMA]] |volume=287 |issue=10 |pages=1308–20 |year=2002 |month=Mar |pmid=11886323 |doi= 10.1001/jama.287.10.1308|url=http://jama.ama-assn.org/cgi/pmidlookup?view=long&pmid=11886323}}</ref><ref name="Sherrid Chaudhry et al. 2003">{{cite journal |author=Sherrid MV, Chaudhry FA, Swistel DG |title=Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and the continuing evolution of surgery for obstruction |journal=Ann Thorac Surg. |volume=75 |issue=2 |pages=620–32 |year=2003 |month=Feb |pmid=12607696 |doi= 10.1016/S0003-4975(02)04546-0|url=}}</ref><ref name="Wigle, Sasson et al. 1985">{{cite journal |author=Wigle ED, Sasson Z, Henderson MA, ''et al.'' |title=Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review |journal=Prog Cardiovasc Dis. |volume=28 |issue=1 |pages=1–83 |year=1985 |pmid=3160067 |doi= 10.1016/0033-0620(85)90024-6|url=http://linkinghub.elsevier.com/retrieve/pii/0033-0620(85)90024-6}}</ref><ref name="Wigle, Rakowski et al. 1995">{{cite journal |author=Wigle ED, Rakowski H, Kimball BP, Williams WG |title=Hypertrophic cardiomyopathy. Clinical spectrum and treatment |journal=[[Circulation (journal)|Circulation]] |volume=92 |issue=7 |pages=1680–92 |year=1995 |month=Oct |pmid=7671349 |doi= |url=http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=7671349}}</ref><ref name="Maron, McKenna et al. 2003">{{cite journal |author=Maron BJ, McKenna WJ, Danielson GK, ''et al.'' |title=[[American College of Cardiology]]/[[European Society of Cardiology]] clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines |journal=[[Journal of the American College of Cardiology|J Am Coll Cardiol]] |volume=42 |issue=9 |pages=1687–713 |year=2003 |month=Nov |pmid=14607462 |doi= 10.1016/S0735-1097(03)00941-0|url=http://linkinghub.elsevier.com/retrieve/pii/S0735109703009410}}
==Pathophysiology==
</ref> It is perhaps most well known as a leading cause of [[sudden cardiac death]] in young athletes.<ref name="Maron, Thompson et al. 1996">{{cite journal |author=Maron BJ, Thompson PD, Puffer JC, ''et al.'' |title=Cardiovascular preparticipation screening of competitive athletes. A statement for health professionals from the Sudden Death Committee (clinical cardiology) and Congenital Cardiac Defects Committee (cardiovascular disease in the young), American Heart Association |journal=Circulation |volume=94 |issue=4 |pages=850–6 |year=1996 |month=Aug |pmid=8772711 |doi= |url=http://circ.ahajournals.org/cgi/content/full/94/4/850}}</ref>
In hypertrophic cardiomyopathy (HCM), the [[sarcomeres]] (contractile elements) in the heart replicate causing heart muscle cells to increase in size, which results in the thickening of the heart muscle. In addition, the normal alignment of muscle cells is disrupted, a phenomenon known as ''[[myocardial disarray]]''. [[Myosin]] heavy chain mutations are associated with development of familial hypertrophic cardiomyopathy. HCM also causes disruptions of the electrical functions of the heart.
The occurrence of hypertrophic cardiomyopathy is a significant cause of sudden unexpected cardiac death in any age group and as a cause of disabling cardiac symptoms. Younger people are likely to have a more severe form of hypertrophic cardiomyopathy
 
===Genetics===
HCM is most commonly due to a mutation in one of 9 sarcomeric [[gene]]s that results in a mutated protein in the sarcomere, the primary component of the [[myocyte]] (the muscle cell of the heart).
 
===Epidemiology and Demographcs===
While most literature so far focuses on European, American, and Japanese populations, HCM appears in all racial groups. The prevalence of HCM is about 0.2% to 0.5% of the general population.
 
==Screening==
HCM is frequently asymptomatic until sudden cardiac death, and for this reason some suggest routinely screening certain populations for this disease.<ref name="slate"/>


==References==
==References==

Revision as of 18:06, 7 August 2011

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Editors-In-Chief: C. Michael Gibson, M.S., M.D. [1], Martin S. Maron, M.D., and Barry J. Maron, M.D.

Overview

Hypertrophic cardiomyopathy, or HCM, is a disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is hypertrophied (thickened) without any alternate cause such as hypertension, amyloid or aortic stenosis.[1][2][3][4][5][6] Although HCM has gained notoriety as a leading cause of sudden cardiac death in young athletes, [7] it should be noted that HCM is a cause of sudden cardiac death in any age group and may be associated with cardiac morbidity and disabling cardiac symptoms as well.

Variants

A non-obstructive variant of HCM is known as apical hypertrophic cardiomyopathy [8], which is also known as nonobstructive hypertrophic cardiomyopathy and Japanese variant hypertrophic cardiomyopathy or the Yamaguchi variant (since the first cases described were all in individuals of Japanese descent).

Pathophysiology

In hypertrophic cardiomyopathy (HCM), the sarcomeres (contractile elements) in the heart replicate causing heart muscle cells to increase in size, which results in the thickening of the heart muscle. In addition, the normal alignment of muscle cells is disrupted, a phenomenon known as myocardial disarray. Myosin heavy chain mutations are associated with development of familial hypertrophic cardiomyopathy. HCM also causes disruptions of the electrical functions of the heart.

Genetics

HCM is most commonly due to a mutation in one of 9 sarcomeric genes that results in a mutated protein in the sarcomere, the primary component of the myocyte (the muscle cell of the heart).

Epidemiology and Demographcs

While most literature so far focuses on European, American, and Japanese populations, HCM appears in all racial groups. The prevalence of HCM is about 0.2% to 0.5% of the general population.

Screening

HCM is frequently asymptomatic until sudden cardiac death, and for this reason some suggest routinely screening certain populations for this disease.[9]

References

  1. ↑ Richardson P, McKenna W, Bristow M, Maisch B, Mautner B, O'Connell J, Olsen E, Thiene G, Goodwin J, Gyarfas I, Martin I, Nordet P. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation. 1996 Mar 1; 93(5):841–2. (Medline abstract; Full text)
  2. ↑ Maron B. Hypertrophic cardiomyopathy: a systematic review. JAMA 2002. 287:1308–20
  3. ↑ Sherrid M, Chaudhry FA, Swistel DG. Obstructive hypertrophic cardiomyopathy. Echocardiography, pathophysiology, and the continuing evolution of surgery for obstruction. Annals of Thoracic Surgery 2003; 75:620–32
  4. ↑ Wigle D, Sasson Z, Henderson MA, Ruddy TD, Fulop J, Rakowski H, Williams WG. Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review. Progress in Cardiovascular Diseases 1985; 28:1–83
  5. ↑ Wigle ED, Rakowski H, Kimball BP, Williams WG. Hypertrophic cardiomyopathy — clinical spectrum and treatment. Circulation 1995; 92:1680–92
  6. ↑ Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH III, Spirito P, Ten Cate FJ, Wigle ED. American College of Cardiology / European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. J Am Coll Cardiol. 2003; 42:1687–713
  7. ↑ Maron BJ, Thompson PD, Puffer JC, McGrew CA, Strong WB, Douglas PS, Clark LT, Mitten MJ, Crawford MH, Atkins DL, Driscoll DJ, Epstein AE. Cardiovascular preparticipation screening of competitive athletes. A statement for health professionals from the Sudden Death Committee (clinical cardiology) and Congenital Cardiac Defects Committee (cardiovascular disease in the young), American Heart Association. Circulation. 1996 Aug 15; 94(4):850-6. (Medline abstract; Full text)
  8. ↑ Rivera-Diaz J, Moosvi AR. Apical hypertrophic cardiomyopathy. South Med J. 1996 Jul; 89(7):711-3. (Medline abstract; Full text)
  9. ↑ Invalid <ref> tag; no text was provided for refs named slate

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