Noncompaction cardiomyopathy
You don't need to be Editor-In-Chief to add or edit content to WikiDoc. You can begin to add to or edit text on this WikiDoc page by clicking on the edit button at the top of this page. Next enter or edit the information that you would like to appear here. Once you are done editing, scroll down and click the Save page button at the bottom of the page.
| Noncompaction cardiomyopathy Classification and external resources |
| Cardiology Network |
 Discuss Noncompaction cardiomyopathy further in the WikiDoc Cardiology Network |
| Adult Congenital |
|---|
| Biomarkers |
| Cardiac Rehabilitation |
| Congestive Heart Failure |
| CT Angiography |
| Echocardiography |
| Electrophysiology |
| Cardiology General |
| Genetics |
| Health Economics |
| Hypertension |
| Interventional Cardiology |
| MRI |
| Nuclear Cardiology |
| Peripheral Arterial Disease |
| Prevention |
| Public Policy |
| Pulmonary Embolism |
| Stable Angina |
| Valvular Heart Disease |
| Vascular Medicine |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [5] Phone:617-632-7753
Associate Editors-In-Chief: Cafer Zorkun, M.D., Ph.D. [6]; Keri Shafer, M.D. [7]
Please Join in Editing This Page and Apply to be an Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [8] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.
Overview
Non-compaction cardiomyopathy (NCC),sometimes known as "spongiform cardiomyopathy" is a rare congenital cardiomyopathy that affects both children and adults. It refers to a type of cardiomyopathy where the myocardial development is hindered while the baby is in the womb during the embryogenesis stage [1] [2]. This hindrance begins to occur around the 8 week mark in the womb. The myocardium at this period of development is often refered to looking like a sponge as it is still a meshwork of interwoven myocardial fibers. This is beneficial at this stage as there is an increased surface area which allows perfusion of the myocardium from the left ventricular cavity. However as the embryo grows the myocardium should begin to be compact, in the case of NCC though the heart fails to fully compact leaving the myocardium to still have a spongy form.
Symptoms can range however are often a result of a poor pumping performance by the heart. The disease can be associated with other problems with the heart and th ebody or by itself
Noncompaction of ventricular myocardium is recently included in the 2006 classification of cardiomyopathies as a genetic cardiomyopathy [3].
Description
Non-compaction Cardiomyopathy can affect both ventricle walls of the heart separately or together however it generally only affects the left ventricle wall and therefore is sometimes known as left ventricular noncompaction. Due to its recent diagnosis and it being an unclassified Cardiomyopathy according to the WHO, it is not fully understood how common the condition is. Some reports suggest that it is in the order of 0.12 cases per 100,000. The low number of reported cases though is due to the lack of any large population studies into the disease and have been based primarily upon patients suffering from advanced heart failure. A similar situation occurred with Hypertrophic cardiomyopathy which was initially considered very rare however is not thought to occur in one in every 500 people in the population.
Again due to the recent nature of the condition diagnosis is not definitive and there are on going discussions as to various points such as the ratio of compacted to non-compacted at various age stages. However it is universally understood that non-compaction Cardiomyopathy will by characterized anatomically be 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. [4]
Symptoms
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 [1]. Likewise it possible to suffer from serve heart failure [2], which even though the condition is present from birth, may only manifest itself later in life.[1]
- Breathlessness
- Fatigue
- Swelling of the ankles
- Limited physical capacity and exercise intolerance
Two conditions though that are more prevalent in Noncompaction Cardiomyopathy are the possibilities of Tachyarrhythmia which can lead to Sudden cardiac death and clotting of the blood in the heart.
Other Complications
The presence of NCC can also lead to other complications around the heart and else where in the body. These are not nesesarily common complications and no paper has yet to comment on how frequentently these complicationcs occur with NCC as well.
- Heart Related
- Abnormalities of the origin of the left coronary artery
- Pulmonary atresia
- Stenosis
- Right or Left ventricle obstruction
- Hypoplastic left ventricle
- Mitral regurgitation
- Neuromuscular (Pertaining to both nerves and muscles)
- Genetic related
Misdiagnosis
In a recent study [1] carried out on 53 patients with the condition in New York, 42 had been diagnosed with another form of heart disease and only in the most recent 11 cases that ventricular noncompaction was diagnosed and this took several echocardiograms to confirm.
The most common miss diagnosis were
- 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 has made it easier to diagnosis the problem in particle the wider use of MRI's
Prognosis
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 [2] 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
One paper [5] has listed the various types of management of care that have been used for various types of NCC. These are similar to management programs for other types of cardiomyopathies which include the use of ACE inhibitors, beta blockers and aspirin therapy to relieve the pressure on the heart, surgical options such as the installation of pacemaker is also an option for those thought to be at a high risk of [Cardiac arrhythmia|arrhythmia] problems.
In severe cases, where NCC has led to heart failure, a heart transplant may be necessary.
Images
 Two-dimensional apical four chamber and parasternal short axis images at the level of the ventricles show dilatation of both ventricles, multiple trabeculae and intertrabecular recesses in inferior, lateral, anterior walls, middle and apical portions of the septum and apex of the left ventricle. [1] |
 Transthoracic two-dimensional study with color and continuous wave Doppler shows left ventricular noncompaction associated with patent ductus arteriosus (PDA). [2] |
 Transthoracic two-dimensional echocardiogram in apical four chamber and parasternal short axis at the level of both ventricles demonstrate dilatation, deep trabeculae and intertrabecular recesses in the inferior, lateral, anterior walls, middle and apical portions of the septum and apex of the left ventricle. [3] |
 Two-dimensional parasternal and color Doppler images at the level of both ventricles that show the noncompacted:compacted wall ratio and how the color enters the intertrabecular recesses [4] |
Source
- Left Ventriuclar noncompaction. Orphanet.
See also
Barth syndrome, Emery-Dreifuss muscular dystrophy, myotubular myopathy, genes responsible for NCC are located also in the area that cause these conditions.
Links
References
- ↑ 1.0 1.1 1.2 1.3 Espinola-Zavaleta, Nilda. (2006). "Non-compacted Cardiomyopathy: Clinical-Echocardiographic Study" (webpage). Cardiovasc Ultrasound 4 (1). Medscape.
- ↑ 2.0 2.1 2.2 Oechslin, Erwin (2005). "Non-compaction of the Left Ventricular Myocardium - From Clinical Observation to the Discovery of a New Disease" (webpage).
- ↑ Maron, Barry. (2006). "Contemporary Definitions and Classification of the Cardiomyopathies" (webpage). American Heart Association Journals 113 (14). American Heart Association t. 113:1807-1816.
- ↑ Weiford BC, Subbarao VD, Mulhern KM, Noncompaction of the ventricular myocardium. Circulation 109 (24): 2965-71 2004
- ↑ Lorenzo Botto, MD (2004-Sept). Left Ventricular Non-compacted (PDF).
Additional Resources
- Non-compaction of Myocardium Cardiomyopathy. Yale University.
- Cardiomyopathy Caused by Isolated Noncompaction of the Left Ventricle in Adults. Medscape Cardiology.
- Non-compacted Cardiomyopathy: Clinical-Echocardiographic Study. Medscape Cardiology.
- Left Ventricular Non-compaction. Baylor College of Medicine.
- Contemporary Definitions and Classification of the Cardiomyopathies. American Heart Association Scientific Statement.
- The Failing Heart. Nature.
- Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician. Sao Paulo Medical Journal.
- Non-compaction of the Left Ventricular Myocardium - From Clinical Observation to the Discovery of a New Disease. Touch Cardiology.
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
