Heterotaxy and complex structural heart defects in a mutant mouse model of primary ciliary dyskinesia
J. Clin. Invest. Serena Y. Tan, et al. 117:3742 doi:10.1172/JCI33284 [
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Figure 3Histological analysis of cardiac anomalies. (
A and
B) Outflow tract defects. (
A) Posterior view of heart exhibiting dextrocardia with TOF. The aorta (Ao) overrides the VSD and is continuous with both the left-sided morphologic right ventricle (mRV) and the right-sided morphologic left ventricle (mLV). The pulmonary artery (PA) is narrower than the Ao. The mitral valve (MV) is continuous with the aortic valve and opens into the mLV. (
B) Posterior view of heart with levocardia and DORV. Both the PA and Ao open into the right-sided mRV. (
C and
D) Atrial isomerism and common AV canal. (
C) Frontal view showing left atrial isomerism with both SVCs entering the common atrium (CA) laterally. Rather than the right SVC entering the roof of the right atrium and the left SVC entering the right atrium via the coronary sinus, the SVCs return symmetrically to the sides of the CA, which serves as both the right atrium and the coronary sinus. Two hepatic veins (HVs) also return directly via midline to the common atrium inferiorly. (
D) Resectioned 2D transverse slice from 3D reconstruction, showing a common AV canal (AVC) with a single AV leaflet. Note the symmetry of the 2 SVCs. Scale bars: 200 μm.
