BC44

Noncompaction cardiomyopathy

Cardiomiopatia não compactada

Category

Definition

Noncompaction cardiomyopathy is a morphologic abnormality of the myocardium predominantly affecting the apex of the ventricle characterised by hypertrabeculation and deep inter-trabecular recesses, usually accompanied by an abnormally thin subepicardial layer of compacted myocardium, that is generally but not always associated with ventricular dysfunction. Additional information. Noncompaction cardiomyopathy classically involves the left ventricle but can also involve only the right ventricle or both. It can occur as an isolated finding or in association with a dilated, hypertrophic, or mixed cardiomyopathic phenotype. It has been described in association with complex congenital heart disease, coronary artery anomalies and as an isolated finding, with and without musculoskeletal and other system abnormalities.

Index Terms

Noncompaction cardiomyopathyLeft ventricular noncompactionFamilial-genetic noncompaction cardiomyopathySarcomeric noncompaction cardiomyopathyX-linked noncompaction cardiomyopathyNoncompaction cardiomyopathy due to Barth syndromeNoncompaction cardiomyopathy due to laminopathyNoncompaction cardiomyopathy due to Z-line protein/Cypher mutationNoncompaction cardiomyopathy due to Z-line protein/Cypher mutation (Zaspopathy)Left ventricular noncompaction cardiomyopathyRight ventricular noncompaction cardiomyopathyBiventricular noncompaction cardiomyopathyNoncompaction cardiomyopathy associated with congenital heart disease