Isolated ventricular non-compaction (IVNC) is a cardiomyopathy caused by an arrest in cardiac embryogenesis with failure of normal compaction of the ventricular endomyocardium.


Inheritance: Some cases are familial with X-linked recessive inheritance. Other cases are sporadic.


Implicated genetic locus: Xq28


The onset of clinical findings may be anytime from in utero to adulthood depending on severity.


Clinical features include:

(1) systolic and/or diastolic dysfunction with heart failure

(2) cardiac arrhythmias

(3) embolic events arising from an affected ventricle


The heart shows characteristic features on echocardiography.


The pathologic changes are seen primarily in the left ventricle with or without concomitant involvement of the right ventricle.


Pathologic features:

(1) deep ventricular trabeculations

(2) prominent endocardial fibroelastosis

(3) compact but relatively thin epicardium


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