Analysis of ventricular hypertrabeculation and noncompaction using genetically engineered mouse models

H Chen, W Zhang, D Li, TM Cordes, R Mark Payne… - Pediatric …, 2009 - Springer
H Chen, W Zhang, D Li, TM Cordes, R Mark Payne, W Shou
Pediatric cardiology, 2009Springer
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
lead 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, 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 lead 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, a genetically heterogeneous disorder. Here we summarize our recent findings through the analyses of several genetically engineered mouse models that have defects in cardiac trabeculation and compaction. Our data indicate that cellular growth and differentiation signaling pathways are keys in these ventricular morphogenetic events.
Springer