Noncompaction cardiomyopathy
| Noncompaction cardiomyopathy |
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Noncompaction Cardiomyopathy Microchapters |
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Pathophysiology |
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Differentiating Noncompaction Cardiomyopathy from other Diseases |
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Noncompaction cardiomyopathy On the Web |
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Risk calculators and risk factors for Noncompaction cardiomyopathy |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editors-In-Chief: Cafer Zorkun, M.D., Ph.D. [3]
Synonyms and keywords: NCC, spongiform cardiomyopathy
Overview
Pathophysiology
Epidemiology
Natural History, Complications & Prognosis
Diagnosis
Symptoms
Echocardiography
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 [1] 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
The management of NCC is similar to that of other cardiomyopathies and includes the use of ACE inhibitors, 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[2].
In severe NCC cases, in which severe heart failure has developed, a heart transplant may be necessary.
Source
- "Left Ventriuclar noncompaction" (PDF). 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.
References
- ↑ Oechslin, Erwin; Jenni, Rolf (2005). "Non-compaction of the Left Ventricular Myocardium - From Clinical Observation to the Discovery of a New Disease" (webpage).
- ↑ [1]
Additional Resources
- "Left Ventricular Non-compaction". Baylor College of Medicine.
- "Contemporary Definitions and Classification of the Cardiomyopathies". American Heart Association Scientific Statement.