left ventricular non-compaction 1

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left ventricular non-compaction 12016-11-26T15:16:08+00:00

left ventricular non-compaction 1

CARDIOLOGY

Definition A congenital heart malformation (OMIM:604169) caused by arrested myocardial development and characterised by a hypertrophic left ventricle with deep trabeculations and poor systolic function, variably accompanied by left ventricular dilation and other congenital heart anomalies–e.g., ventricular septal defects, pulmonary artery stenosis, atrial septal defects and right ventricular defects.

Molecular pathology Defects of DTNA, which encodes a protein of the dystrophin protein complex, cause left ventricular non-compaction 1. 

Synonyms Left ventricular non-compaction 1 with or without congenital heart defects

References http://www.uniprot.org/uniprot/Q9Y4J8 

http://www.omim.org/entry/604169 

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