Noncompaction cardiomyopathy overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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 during the early stages of fetal development (the embryogenesis stage). [1] [2]. It results from the failure of myocardial development during embryogenesis.[1][2] The phenotypic expression is variable. The can be symptoms of systolic dysfunction and heart failure, while in other patients there can be minimal symptoms and hypercontractility. The condition can occur as either an isolated disease or as part of constellation of other findings of congenital heart disease (frequently pulmonic stenosis, atrial septal defect, ventricular septal defect, or hypoplastic left ventricle. On echocardiography there is ventricular hypertrophy with deep recesses which tend to be located apically.

References

  1. 1.0 1.1 Espinola-Zavaleta, Nilda.; Soto, Elena.; Castellanos, Luis Munoz; Játiva-Chávez, Silvio; Keirns, Candace. (2006). "Non-compacted Cardiomyopathy: Clinical-Echocardiographic Study" (webpage). Cardiovasc Ultrasound. Medscape. 4 (1).
  2. 2.0 2.1 Oechslin, Erwin; Jenni, Rolf (2005). "Non-compaction of the Left Ventricular Myocardium - From Clinical Observation to the Discovery of a New Disease" (webpage).

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