Phenotype Image Detail

Image

Caption

A: Superior-inferior ventricles with congested superior ventricle and atrial appendages with aorta (Ao) and pulmonary artery (PA) side by side. B: 3D model shows morphologic left ventricle (mLV) is inferior and right-sided, while the morphologic right ventricle (mRV) is superior and left-sided. C: 3D reconstruction in a apical orientation looking anteriorly at 2 papillary muscles in the smaller, inferior mLV as indicated by the 2 arrowheads. There is also a small, muscular ventricular septal defect (VSD) present near the apex. D: Anterior view shows a larger, superior mRV with the septomarginalis (M) between the superior ventricular outflow tract and the body of the right ventricle. The aortic and pulmonic valves (AV and PV) both arise from the mRV, giving rise to a double outlet right ventricle (DORV) with a VSD. The arrowhead indicates the AV canal defect. E: Anterior view of 3D Episcopic flourescence image capture (EFIC) reconstruction illustrates side-by-side, semilunar valves of similar height associated with DORV. The bicuspid aortic valve (AV) is on the left, and the tricuspid pulmonic valve (PV) is on the right. An AV canal defect (AVC) is situated at the crux of the heart. F: Frontal 2D section showing the same AVC as in E entering both the mRV and mLV, with a VSD denoted by the arrow. LA, left atrium. Scale bars: 500um.

Copyright

This image is from Tan SY, J Clin Invest 2007 Dec;117(12):3742-52 and is displayed with the permission of the American Society for Clinical Investigation who owns the Copyright. J:130755

Associated Alleles

Symbol	Name
Dnah5^hlb612	dynein, axonemal, heavy chain 5; heart, lung and blood 612

Associated Genotypes

Allelic Composition	Genetic Background
Dnah5^hlb612/Dnah5^hlb612	involves: C57BL/6J