High Incidence of Cardiac Malformations in Connexin40-Deficient Mice

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High Incidence of Cardiac Malformations in Connexin40-Deficient Mice by Hong Gu, Frank C. Smith, Steven M. Taffet, and Mario Delmar Circulation Research Volume 93(3):201-206 August 8, 2003 Copyright © American Heart Association, Inc. All rights reserved.

Figure 1. Bifid left atrial appendage in a Cx40+/− mouse. Figure 1. Bifid left atrial appendage in a Cx40+/− mouse. A, Heart of a Cx40+/− fetus at 18.5 ED. RA indicates right atrial appendage. Left atrial appendage (LA) is abnormal with two separate “pouches” (arrows). B, Hematoxylin-eosin-stained coronal section reveals two distinct lobes of the left atrial appendage. Bar=1 mm. Hong Gu et al. Circ Res. 2003;93:201-206 Copyright © American Heart Association, Inc. All rights reserved.

Figure 2. Aortic arch branch vessel abnormality in a Cx40+/− mouse. Figure 2. Aortic arch branch vessel abnormality in a Cx40+/− mouse. A, Normal heart of a wild-type 6-hour-old mouse. B, Heart of affected Cx40+/− mouse. Take-off of two branch vessels from the aorta (arrows) is more proximal than normal and the left vessel courses leftward and laterally. Bar=1 mm. Hong Gu et al. Circ Res. 2003;93:201-206 Copyright © American Heart Association, Inc. All rights reserved.

Figure 3. Double-outlet right ventricle in a Cx40−/− mouse. Figure 3. Double-outlet right ventricle in a Cx40−/− mouse. A through C, Hematoxylin-eosin-stained serial coronal sections of the heart of a 6-hour-old Cx40−/− mouse. D through F, Similar sections from a Cx40+/+ littermate with a normal heart. A, There is significant muscular obstruction (open arrow) proximal to the pulmonary valve (PV). B, Section posterior to section A. *Ventricular septal defect (VSD). C, More posterior section showing VSD (*) with aortic override (Ao) and mild fibrous discontinuity between the anterior mitral valve leaflet (open arrow) and the aortic valve. D, Pulmonary valve (PV) and right ventricle (RV). E, Aorta (Ao), left ventricle (LV). F, Aortic valve leaflets (Ao) in continuity with mitral valve leaflet (open arrow). Bar=1 mm. Hong Gu et al. Circ Res. 2003;93:201-206 Copyright © American Heart Association, Inc. All rights reserved.

Figure 4. Unbalanced partial endocardial cushion defect in Cx40−/− mouse. Figure 4. Unbalanced partial endocardial cushion defect in Cx40−/− mouse. A through C, Serial coronal hematoxylin-eosin-stained sections from a 6-hour-old Cx40−/− mouse. B, Common atrioventricular valve (arrow). C, Large atrial septal defect (double-headed arrow). Bar=1 mm. Hong Gu et al. Circ Res. 2003;93:201-206 Copyright © American Heart Association, Inc. All rights reserved.

Figure 5. Tetralogy of Fallot with pulmonary atresia in a 6-hour-old Cx40−/− mouse. Figure 5. Tetralogy of Fallot with pulmonary atresia in a 6-hour-old Cx40−/− mouse. A, Heart and great vessels. Severe hypoplasia of the main pulmonary artery (PA). Interventricular cleft (open arrow). B, Pulmonary artery (PA) and aorta (Ao). C, Aorta (Ao) overrides a ventricular septal defect (*). Bar=1 mm. Hong Gu et al. Circ Res. 2003;93:201-206 Copyright © American Heart Association, Inc. All rights reserved.