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{{CMG}}; '''Associate Editors-In-Chief:''' {{CZ}}
{{CMG}}; '''Associate Editors-In-Chief:''' {{CZ}}


'''''Synonyms and keywords:''''' NCC, spongiform cardiomyopathy
'''''Synonyms and keywords:''''' left ventricular noncompaction, LVNC, NCC, spongiform cardiomyopathy, (Isolated) noncompaction of the ventricular myocardium, (I)NVM, non-compaction of the left ventricular myocardium, left ventricular hypertrabeculation, spongy myocardium.


==[[Noncompaction cardiomyopathy overview|Overview]]==
==[[Noncompaction cardiomyopathy overview|Overview]]==


==[[Noncompaction cardiomyopathy pathophysiology|Pathophysiology]]==
==[[Noncompaction cardiomyopathy historical perspective|Historical Perspective]]==
==[[Noncompaction cardiomyopathy classification|Classification System and Nomenclature]]==


==[[Noncompaction cardiomyopathy epidemiology and demographics|Epidemiology]]==
==Pathophysiology==


==[[Noncompaction_cardiomyopathy_natural_history|Natural History, Complications & Prognosis]]==
[[Noncompaction cardiomyopathy embryology|Embryology]] | [[Noncompaction cardiomyopathy histology|Histology]] | [[Noncompaction cardiomyopathy genetics|Genetics]] | [[Noncompaction cardiomyopathy left ventricular dysfunction|Left ventricular dysfunction]] | [[Noncompaction cardiomyopathy thromboembolism|Thromboembolism]] | [[Noncompaction cardiomyopathy neuromuscular disorders|Neuromuscular disorders]]


==Diagnosis==
==[[Noncompaction cardiomyopathy epidemiology and demographics|Epidemiology and Demographics]]==
===[[Noncompaction cardiomyopathy history and symptoms|Symptoms]]===


===Echocardiography===
==[[Noncompaction cardiomyopathy screening|Screening]]==


===Misdiagnosis===
==[[Noncompaction_cardiomyopathy_natural_history|Natural History, Complications & Prognosis]]==
 
In a recent study <ref name= Espinola-Zavaleta >{{Cite journal |  last =Espinola-Zavaleta| first =Nilda. | last2 =Soto | first2 =Elena. |  last3 =Castellanos| first3 =Luis Munoz | last4 =Játiva-Chávez| first4  =Silvio | last5 =Keirns | first5 =Candace. | title = Non-compacted  Cardiomyopathy: Clinical-Echocardiographic Study | journal = Cardiovasc  Ultrasound | publisher = Medscape | volume = 4 | issue =1 | pages =|  year =[[2006]] | url= http://www.medscape.com/viewarticle/551882_1 | format = webpage | id =}}</ref>  of 53 patients with non-compaction cardiomyopathy, the first 42 were  misdiagnosed with another form of heart disease.  Improved awareness and  improved imaging modalities allowed the correct diagnosis to be made in  the last 11 cases. The most common misdiagnoses included:
 
* [[Dilated cardiomyopathy]]: 30 Cases
* [[Congenital heart disease]]: 6 Cases
* [[Ischemic heart disease]]: 2 Cases
* Disease of the heart valves: 2 Cases
* Dilated phase hypertensive cardiomyopathy: 1 Case
* [[Restrictive cardiomyopathy]]: 1 Case
 
The high number of misdiagnosis can be attributed due to non-compaction [[cardiomyopathy]]  only being first reported in 1990, and so diagnosis is often overlooked  or delayed. Advances in medical imaging equipment (Echo and MRI) has  made it easier to diagnosis NCC.


==[[Noncompaction_cardiomyopathy_differential diagnosis|Differentiating Noncompaction Cardiomyopathy from other Diseases]]==


 
==Diagnosis==
==Prognosis==
[[Noncompaction cardiomyopathy history and symptoms|History & Symptoms]] | [[Noncompaction cardiomyopathy physical examination|Physical Examination]] | [[Noncompaction cardiomyopathy laboratory studies|Laboratory Studies]] | [[Noncompaction cardiomyopathy genetic testing|Genetic Testing]] |
 
[[Noncompaction cardiomyopathy electrocardiogram|Electrocardiogram]] | [[Noncompaction cardiomyopathy chest x ray|Chest X Ray]] | [[Noncompaction cardiomyopathy echocardiography or ultrasound|Echocardiography]] | [[Noncompaction cardiomyopathy MRI|MRI]] | [[Noncompaction cardiomyopathy CT|CT]] | [[Noncompaction cardiomyopathy other diagnostic studies|Biopsy]]
Due to non-compaction cardiomyopathy being a relatively new disease in terms of understanding it's impact on the human life expectancy is not very well understood. In a 2005 study <ref name= Oechslin>{{Cite journal | last =Oechslin| first =Erwin | last2 =Jenni | first2 =Rolf | title = Non-compaction of the Left Ventricular Myocardium - From Clinical Observation to the Discovery of a New Disease | journal =  | publisher =  | volume =  | issue =| pages =  | year = 2005| url= http://www.touchcardiology.com/compaction-left-ventricular-myocardium-a352-3.html| format = webpage | id =}}</ref> which documented the long term follow up of 34 patients with NCC, 35% had died at the age of 42 +/- 40 months with a further 12% having to under go a heart transplant due to heart failure. However, this study was based upon symptomatic patients referred to a tertiary care center, and so were suffering from more severe forms of NCC than might be found typically in the population. As NCC is a genetic disease, as a precaution immediate family members are being tested which is turning up more supposedly healthy people with NCC who are not suffering from it. The long term prognosis for these people is currently unknown.


==Management==
==Management==
The management of NCC is similar to that of other cardiomyopathies and includes the use of [[angiotensin-converting enzyme|ACE inhibitors]], [[Beta blocker|beta blockers]] and [[aspirin]]. Implantation of an [[automatic internal cardiac defibrillator]] [[AICD]] may be appropriate in patients who are deemed to be at increased risk of [[sudden cardiac death]].  A small study from the Netherlands identified young females with [[premature ventricular contractions]] ([[PVCs]]) at risk of [[sudden death]]<ref>[http://spo.escardio.org/eslides/view.aspx?eevtid=33&fp=4147]</ref>.
[[Noncompaction cardiomyopathy medical therapy|Medical Therapy]] | [[Noncompaction cardiomyopathy surgery|Surgery]]
 
In severe NCC cases, in which severe [[heart failure]] has developed, a [[heart transplant]] may be necessary.
 
==Source==
*{{cite web | title=Left Ventriuclar noncompaction| work=Orphanet| url=http://www.orpha.net/data/patho/GB/uk-LVNC.pdf}}


==See also==
==See also==
[[Cardiomyopathy]], [[Barth syndrome]], [[Emery-Dreifuss muscular dystrophy]], [[myotubular myopathy]], genes responsible for NCC are located also in the area that cause these conditions.


[[Barth syndrome]], [[Emery-Dreifuss muscular dystrophy]], [[myotubular myopathy]], genes responsible for NCC are located also in the area that cause these conditions.
{{Circulatory system pathology}}


==References==
{{Reflist|2}}
==Additional Resources==
*{{cite web | title=Left Ventricular Non-compaction | work=Baylor College of Medicine| url=http://www.bcm.edu/pediatrics/index.cfm?realm=99992426&This_Template=LVNC}}
*{{cite web | title=Contemporary Definitions and Classification of the Cardiomyopathies| work=American Heart Association Scientific Statement | url=http://circ.ahajournals.org/cgi/content/full/113/14/1807}}
{{Circulatory system pathology}}
{{SIB}}
[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Cardiomyopathy]]
[[Category:Cardiomyopathy]]

Latest revision as of 16:04, 7 August 2011

Noncompaction cardiomyopathy

Noncompaction Cardiomyopathy Microchapters

Home

Patient Info

Overview

Historical Perspective

Classification & Nomenclature

Pathophysiology

Genetics
Embryology
Histology
Left Ventricular Dysfunction
Arrhythmias
Thromboembolism
Neuromuscular Disorders

Epidemiology & Demographics

Screening

Natural History, Complications & Prognosis

Differentiating Noncompaction Cardiomyopathy from other Diseases

Diagnosis

History & Symptoms

Physical Examination

Laboratory Studies

Genetic Testing

Electrocardiogram

Holter Monitoring

Chest X Ray

MRI

CT

Echocardiography

Biopsy

Treatment

Medical Therapy

Surgery

Noncompaction cardiomyopathy On the Web

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US National Guidelines Clearinghouse

NICE Guidance

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Synonyms and keywords: left ventricular noncompaction, LVNC, NCC, spongiform cardiomyopathy, (Isolated) noncompaction of the ventricular myocardium, (I)NVM, non-compaction of the left ventricular myocardium, left ventricular hypertrabeculation, spongy myocardium.

Overview

Historical Perspective

Classification System and Nomenclature

Pathophysiology

Embryology | Histology | Genetics | Left ventricular dysfunction | Thromboembolism | Neuromuscular disorders

Epidemiology and Demographics

Screening

Natural History, Complications & Prognosis

Differentiating Noncompaction Cardiomyopathy from other Diseases

Diagnosis

History & Symptoms | Physical Examination | Laboratory Studies | Genetic Testing | Electrocardiogram | Chest X Ray | Echocardiography | MRI | CT | Biopsy

Management

Medical Therapy | Surgery

See also

Cardiomyopathy, Barth syndrome, Emery-Dreifuss muscular dystrophy, myotubular myopathy, genes responsible for NCC are located also in the area that cause these conditions.


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