Fig. 3. External aspect of the hearts of Bmpr2^E2/E2 mutants. (A,B) Frontal views of wild-type (A) and Bmpr2^E2/E2 mutant (B) hearts at E16 showing a normally septated ascending arch of the aorta (Ao) and pulmonary trunk (PT) distally, but not proximally to the heart. The black arrowhead in A indicates the semilunar valves of the PT. The pink arrowhead in B indicates a dimple at the apex of the ventricle, a common sign of ventricular septal defect. (C-G) Ink-injected E13.5 hearts. Left lateral views of whole-mount India ink-injected wild-type (C) and Bmpr2^E2/E2 (D) hearts. The arrowhead in C indicates the semilunar valve level. Semilunar valves are absent in mutants. The white asterisk in D indicates the non-septated conotruncus of the mutants. DAo, descending aorta. (E-G) Interruption of the aortic arch (purple arrow) of varying severity in two mutants (F,G) is visible between the left common carotid artery (LCC) and the left subclavian artery (LSA) in the ink-injected outflow tract (upper panel) and derived cartoons (lower panels). The ductus arteriosus (Du) is enlarged. Pulmonary arteries (PAs) stem normally off of the pulmonary trunk in the mutants. RCC: right common carotid artery. (H,I) Cartoon of a WT (H) and Bmpr2^E2/E2 mutant (I) heart showing the association of persistent truncus arteriosus (PTA), ventricular septal defect (VSD) and interrupted aortic arch type B (IAA) that constitute the type A4 persistent truncus arteriosus (Jacobs, 2000). Ao, aorta; DAo, descending aorta; Du, ductus arteriosus; IVS, interventricular septum; LA and RA, left and right atria, respectively; LCC and RCC, left and right common carotids, respectively; LPA and RPA, left and right pulmonary arteries, respectively; LSA, left subclavian artery; PT, pulmonary trunk.

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