Noncompaction cardiomyopathy

Noncompaction cardiomyopathy
Other namesSpongiform cardiomyopathy
Noncompaction cardiomyopathy is inherited in an autosomal dominant manner
SpecialtyCardiology Edit this on Wikidata

Noncompaction cardiomyopathy (NCC) is a rare congenital disease of heart muscle that affects both children and adults.[1] It results from abnormal prenatal development of heart muscle.[2][3]

During development, the majority of the heart muscle is a sponge-like meshwork of interwoven myocardial fibers. As normal development progresses, these trabeculated structures undergo significant compaction that transforms them from spongy to solid. This process is particularly apparent in the ventricles, and particularly so in the left ventricle. Noncompaction cardiomyopathy results when there is failure of this process of compaction. Because the consequence of non-compaction is particularly evident in the left ventricle, the condition is also called left ventricular noncompaction. Other hypotheses and models have been proposed, none of which is as widely accepted as the noncompaction model.

Symptoms range greatly in severity. Most are a result of a poor pumping performance by the heart. The disease can be associated with other problems with the heart and the body.

  1. ^ Pignatelli RH, McMahon CJ, Dreyer WJ, Denfield SW, Price J, Belmont JW, et al. (November 2003). "Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy". Circulation. 108 (21): 2672–8. doi:10.1161/01.CIR.0000100664.10777.B8. PMID 14623814.
  2. ^ Espinola-Zavaleta N, Soto ME, Castellanos LM, Játiva-Chávez S, Keirns C (September 2006). "Non-compacted cardiomyopathy: clinical-echocardiographic study". Cardiovascular Ultrasound. 4: 35. doi:10.1186/1476-7120-4-35. PMC 1592122. PMID 17002802.
  3. ^ Jenni R, Oechslin E (2005). "Non-compaction of the Left Ventricular Myocardium – From Clinical Observation to the Discovery of a New Disease". European Cardiology Review. 1 (1): 23. doi:10.15420/ECR.2005.23. ISSN 1758-3756. S2CID 221234437.