Molecular mechanism of ventricular trabeculation/compaction and the pathogenesis of the left ventricular noncompaction cardiomyopathy (LVNC)

W Zhang, H Chen, X Qu, CP Chang… - American Journal of …, 2013 - Wiley Online Library
W Zhang, H Chen, X Qu, CP Chang, W Shou
American Journal of Medical Genetics Part C: Seminars in Medical …, 2013Wiley Online Library
Ventricular trabeculation and compaction are two of the many essential steps for generating
a functionally competent ventricular wall. A significant reduction in trabeculation is usually
associated with ventricular compact zone deficiencies (hypoplastic wall), which commonly
leads to embryonic heart failure and early embryonic lethality. In contrast, hypertrabeculation
and lack of ventricular wall compaction (noncompaction) are closely related defects in
cardiac embryogenesis associated with left ventricular noncompaction (LVNC), a genetically …
Abstract
Ventricular trabeculation and compaction are two of the many essential steps for generating a functionally competent ventricular wall. A significant reduction in trabeculation is usually associated with ventricular compact zone deficiencies (hypoplastic wall), which commonly leads to embryonic heart failure and early embryonic lethality. In contrast, hypertrabeculation and lack of ventricular wall compaction (noncompaction) are closely related defects in cardiac embryogenesis associated with left ventricular noncompaction (LVNC), a genetically heterogenous disorder. Here we review recent findings through summarizing several genetically engineered mouse models that have defects in cardiac trabeculation and compaction. © 2013 Wiley Periodicals, Inc.
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