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| {{CMG}}; '''Associate Editors-In-Chief:''' {{CZ}} | | {{CMG}}; '''Associate Editors-In-Chief:''' {{CZ}} |
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| '''''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. |
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| ==[[Noncompaction cardiomyopathy overview|Overview]]== | | ==[[Noncompaction cardiomyopathy overview|Overview]]== |
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| ==[[Noncompaction cardiomyopathy pathophysiology|Pathophysiology]]== | | ==[[Noncompaction cardiomyopathy historical perspective|Historical Perspective]]== |
| | ==[[Noncompaction cardiomyopathy classification|Classification System and Nomenclature]]== |
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| ==Epidemiology== | | ==Pathophysiology== |
| The incidence is estimated to be 0.12 cases per 100,000 people.
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| ==Diagnosis==
| | [[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]] |
| ===Symptoms===
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| Subjects symptoms from non-compaction cardiomyopathy range widely. It is possible to be diagnosed with the condition, yet not to suffer from any of the symptoms associated with heart disease <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>. Likewise it possible to suffer from severe [[heart failure]] <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 =| url= http://www.touchcardiology.com/compaction-left-ventricular-myocardium-a352-3.html| format = webpage | id =}}</ref>, which even though the condition is present from birth, may only manifest itself later in life.<ref name= Espinola-Zavaleta>. Differences in symptoms between adults and children are also prevalent with adults more likely to suffer from heart failure and children from depression of systolic function. <ref name= Espinola-Zavaleta>
| | ==[[Noncompaction cardiomyopathy epidemiology and demographics|Epidemiology and Demographics]]== |
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| Common symptoms though associated with a reduced pumping performance of the heart are<ref>{{cite web |url= http:// http://www.cardiomyopathy.org/index.php?id=274
| | ==[[Noncompaction cardiomyopathy screening|Screening]]== |
| |title= LV Non-compaction
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| |author= The Cardiomyopathy Association
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| |date=2007-July-23
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| | format = website
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| }}</ref>
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| :
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| * Shortness of breath and [[dyspnea on exertion]] ([[DOE]])
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| * [[Fatigue]]
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| * [[Pedal edema]] or swelling of the ankles
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| * Limited physical capacity and exercise intolerance
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| ===Echocardiography=== | | ==[[Noncompaction_cardiomyopathy_natural_history|Natural History, Complications & Prognosis]]== |
| The ratio of compacted to non-compacted myoacardium may very at various developmental stages and ages. Non-compaction cardiomyopathy is characterized anatomically by deep trabeculations in the ventricular wall, which define recesses communicating with the main ventricular chamber. Major clinical correlates include systolic and diastolic dysfunction, associated at times with systemic embolic events. <ref>Weiford BC, Subbarao VD, Mulhern KM, Noncompaction of the ventricular myocardium. Circulation 109 (24): 2965-71 2004</ref> On echocardiography the left ventricular wall is thick with a two layered appearance. The epicardial layer is compacted and thin and the endocardial layer is non-compacted and thick. The ratio of the non-compacted endocardial layer to the epicardial layer is > 2.
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| | ==[[Noncompaction_cardiomyopathy_differential diagnosis|Differentiating Noncompaction Cardiomyopathy from other Diseases]]== |
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| | | ==Diagnosis== |
| ===Misdiagnosis===
| | [[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]] |
| 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:
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| * [[Dilated cardiomyopathy]]: 30 Cases
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| * [[Congenital heart disease]]: 6 Cases
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| * [[Ischemic heart disease]]: 2 Cases
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| * Disease of the heart valves: 2 Cases
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| * Dilated phase hypertensive cardiomyopathy: 1 Case
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| * [[Restrictive cardiomyopathy]]: 1 Case
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| 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.
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| ==Complications of NCC==
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| The presence of NCC can also lead to other complications around the heart and in other organs. These are not necessarily common complications and no paper has yet to document the incidence of these complicationcs.
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| *Heart Related
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| ** Abnormalities of the origin of the [[left coronary artery]]
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| ** [[Pulmonary atresia]]
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| ** [[Stenosis]]
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| ** Right or Left ventricle obstruction
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| ** [[Hypoplastic left heart syndrome|Hypoplastic left ventricle]]
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| ** [[Mitral regurgitation]]
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| ** [[Tachyarrhythmias]] and [[sudden cardiac death]]
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| ** Thrombus in the [[left ventricle]]
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| *Neuromuscular (Pertaining to both nerves and muscles)
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| ** [[Becker's muscular dystrophy]]
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| ** [[Mitochondrial myopathy]]
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| ** [[Myopathy|Polyneuropathy and metabolic myopathy]]
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| *Genetic related
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| ** [[Emery-Dreifuss muscular dystrophy]]
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| ** [[Muscular dystrophy|Myotubular cardiomyopathy]]
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| ** [[Barth syndrome]]
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| ==Prognosis==
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| 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.
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| ==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]] |
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| In severe NCC cases, in which severe [[heart failure]] has developed, a [[heart transplant]] may be necessary.
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| ==Source==
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| *{{cite web | title=Left Ventriuclar noncompaction| work=Orphanet| url=http://www.orpha.net/data/patho/GB/uk-LVNC.pdf}}
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| ==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. |
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| [[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}} |
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| ==References==
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| {{Reflist|2}}
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| ==Additional Resources==
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| *{{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}}
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| *{{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}}
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| {{Circulatory system pathology}}
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| {{SIB}}
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| [[Category:Cardiology]] | | [[Category:Cardiology]] |
| [[Category:Cardiomyopathy]] | | [[Category:Cardiomyopathy]] |