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Interrupted Aortic Arch: Spectrum of MRI Findings

¹ Department of Radiology, University of Michigan Health System, University Hospital, 1500 E Medical Center Dr., Ann Arbor, MI 48109.
² Section of Pediatric Radiology, University of Michigan Health System, C. S. Mott Children's Hospital, Ann Arbor, MI.

View larger version (33K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —Drawings show three types of interrupted aortic arch. Arrow = patent ductus arteriosus. Type A interruption occurs just distal to left subclavian artery. Patent ductus arteriosus provides blood flow to descending thoracic aorta.

View larger version (34K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —Drawings show three types of interrupted aortic arch. Arrow = patent ductus arteriosus. Type B interruption occurs between left common carotid and left subclavian arteries. Patent ductus arteriosus provides blood flow to left subclavian and descending thoracic arteries.

View larger version (34K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C —Drawings show three types of interrupted aortic arch. Arrow = patent ductus arteriosus. Type C interruption occurs between innominate and left common carotid arteries. Patent ductus arteriosus provides blood flow to left common carotid, left subclavian, and descending thoracic arteries.

View larger version (142K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A —1-week-old female neonate with type A interrupted aortic arch, ventricular septal defect, and patent ductus arteriosus. Coronal gadolinium-enhanced MR angiography image shows small-caliber ascending aorta (AA) arising from left ventricle. Right common carotid artery (RCCA) and main pulmonary artery (MPA) can also be seen. Incidental note is also made of venous contamination (V).

View larger version (133K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B —1-week-old female neonate with type A interrupted aortic arch, ventricular septal defect, and patent ductus arteriosus. Left common carotid artery (LCCA) arises from aortic arch, and origin of right pulmonary artery (RPA) is visualized at same level.

View larger version (137K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2C —1-week-old female neonate with type A interrupted aortic arch, ventricular septal defect, and patent ductus arteriosus. Aortic arch terminates as left subclavian artery (LSCLA). There is apparent interruption (INT) of aortic arch between left subclavian artery and descending thoracic aorta. Both right and left vertebral arteries (RVA and LVA, respectively) are also seen.

View larger version (142K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2D —1-week-old female neonate with type A interrupted aortic arch, ventricular septal defect, and patent ductus arteriosus. Large patent ductus arteriosus (PDA) arises from left pulmonary artery (LPA).

View larger version (144K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2E —1-week-old female neonate with type A interrupted aortic arch, ventricular septal defect, and patent ductus arteriosus. Patent ductus arteriosus (PDA) provides blood flow to right descending thoracic aorta (DA).

View larger version (137K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2F —1-week-old female neonate with type A interrupted aortic arch, ventricular septal defect, and patent ductus arteriosus. Maximum-intensity-projection image also shows site of aortic arch interruption (INT).

View larger version (137K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3A —1-week-old male neonate with type B interrupted aortic arch (IAA) and ventricular septal defect. Coronal double inversion recovery fast spin-echo black blood MR image reveals normal ascending aorta (AA) arising from left ventricle. Main pulmonary artery (MPA) and ventricular septal defect (VSD) are also visualized.

View larger version (138K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3B —1-week-old male neonate with type B interrupted aortic arch (IAA) and ventricular septal defect. MR image shows right and left common carotid arteries (RCCA and LCCA, respectively) arise from proximal aortic arch.

View larger version (130K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3C —1-week-old male neonate with type B interrupted aortic arch (IAA) and ventricular septal defect. Slightly more posterior within thorax, patent ductus arteriosus (PDA) arises from left pulmonary artery (LPA). Right pulmonary artery (RPA) and left atrium (LA) are also seen at this level.

View larger version (155K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3D —1-week-old male neonate with type B interrupted aortic arch (IAA) and ventricular septal defect. Sagittal black blood MR image shows vascular arch that is almost completely visualized in single sagittal plane. This structure is formed from main pulmonary artery (MPA) and patent ductus arteriosus (PDA) and appears flattened compared with normal aortic arch, confirming presence of IAA. Patent ductus arteriosus provides blood flow to descending thoracic aorta (DA). Left pulmonary artery (LPA) is also seen.

View larger version (151K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4A —3-day-old male neonate with type B interrupted aortic arch, large aortopulmonary window, and pulmonary sling. Coronal gradient-recalled echo "white blood" MR image shows abnormal communication between ascending aorta (AA) and main pulmonary artery (MPA), so-called aortopulmonary window (APW).

View larger version (146K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4B —3-day-old male neonate with type B interrupted aortic arch, large aortopulmonary window, and pulmonary sling. MR image shows innominate artery (IA) and left common carotid artery (LCCA) arising from proximal aortic arch in V configuration.

View larger version (146K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4C —3-day-old male neonate with type B interrupted aortic arch, large aortopulmonary window, and pulmonary sling. MR image shows that left pulmonary artery (LPA) arises from right pulmonary artery (RPA) at level of left atrium (LA), confirming presence of pulmonary sling. Patent ductus arteriosus (PDA) provides blood flow to descending thoracic aorta (DA).

View larger version (142K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4D —3-day-old male neonate with type B interrupted aortic arch, large aortopulmonary window, and pulmonary sling. Sagittal gradient recalled-echo white blood MR image shows complete vascular arch in single sagittal plane is formed from pulmonary artery and patent ductus arteriosus (PDA). DA = descending thoracic aorta.

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5A —4-day-old female neonate with DiGeorge syndrome, type 2 truncus arteriosus, surgically confirmed interrupted aortic arch, postductal origins of left carotid and left subclavian arteries, and aberrant retroesophageal right innominate arteries. Sagittal oblique subvolume maximum-intensity-projection (MIP) gadolinium-enhanced 3D MR angiography image reveals that ascending aorta and main pulmonary artery arise from single outflow tract, consistent with truncus arteriosus (TA). Patent ductus arteriosus (PDA) directly communicates with descending thoracic aorta (DA). Both right and left common carotid arteries (RCCA and LCCA, respectively) arise from postductal aorta. This interrupted aortic arch branching pattern does not fit criteria for any of the three previously described types. Normal aortic arch is not seen.

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5B —4-day-old female neonate with DiGeorge syndrome, type 2 truncus arteriosus, surgically confirmed interrupted aortic arch, postductal origins of left carotid and left subclavian arteries, and aberrant retroesophageal right innominate arteries. Subvolume MIP image in sagittal obliquity slightly different from A confirms that right and left pulmonary arteries (RPA and LPA, respectively) arise separately from common trunk, consistent with type 2 truncus arteriosus (TA). Origin of patent ductus arteriosus (PDA) is also seen. DA = descending thoracic aorta.

View larger version (109K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5C —4-day-old female neonate with DiGeorge syndrome, type 2 truncus arteriosus, surgically confirmed interrupted aortic arch, postductal origins of left carotid and left subclavian arteries, and aberrant retroesophageal right innominate arteries. Axial subvolume MIP image confirms postductal aberrant retroesophageal innominate artery (IA) gives rise to right common carotid and right subclavian arteries. PDA = patent ductus arteriosus, TA = truncus arteriosus.

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6A —3-day-old female neonate with interrupted aortic arch, aberrant left subclavian artery from left patent ductus arteriosus, aberrant right subclavian artery from descending thoracic aorta, and right descending thoracic aorta. Gadolinium-enhanced 3D MR angiography images show small-caliber ascending aorta (AA in A) arising from left ventricle. Right and left common carotid arteries (RCCA and LCCA, respectively) form V configuration.

View larger version (120K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6B —3-day-old female neonate with interrupted aortic arch, aberrant left subclavian artery from left patent ductus arteriosus, aberrant right subclavian artery from descending thoracic aorta, and right descending thoracic aorta. Gadolinium-enhanced 3D MR angiography images show small-caliber ascending aorta (AA in A) arising from left ventricle. Right and left common carotid arteries (RCCA and LCCA, respectively) form V configuration.

View larger version (129K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6C —3-day-old female neonate with interrupted aortic arch, aberrant left subclavian artery from left patent ductus arteriosus, aberrant right subclavian artery from descending thoracic aorta, and right descending thoracic aorta. Coronal oblique subvolume maximum-intensity-projection (MIP) and volume-rendered images confirm presence of interrupted aortic arch. Right patent ductus arteriosus (RPDA) supplies blood flow to descending thoracic aorta (DA). Left subclavian artery (LSCLA) arises from small left patent ductus arteriosus (LPDA), and right subclavian artery (RSCLA) arises from postductal descending thoracic aorta. This interrupted aortic arch branching pattern does not fit criteria for any of the three previously described types. MPA = main pulmonary artery; in C, RPA = right pulmonary artery; in D, AA = ascending aorta, RCCA = right common carotid artery, LCCA = left common carotid artery.

View larger version (73K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6D —3-day-old female neonate with interrupted aortic arch, aberrant left subclavian artery from left patent ductus arteriosus, aberrant right subclavian artery from descending thoracic aorta, and right descending thoracic aorta. Coronal oblique subvolume maximum-intensity-projection (MIP) and volume-rendered images confirm presence of interrupted aortic arch. Right patent ductus arteriosus (RPDA) supplies blood flow to descending thoracic aorta (DA). Left subclavian artery (LSCLA) arises from small left patent ductus arteriosus (LPDA), and right subclavian artery (RSCLA) arises from postductal descending thoracic aorta. This interrupted aortic arch branching pattern does not fit criteria for any of the three previously described types. MPA = main pulmonary artery; in C, RPA = right pulmonary artery; in D, AA = ascending aorta, RCCA = right common carotid artery, LCCA = left common carotid artery.

View larger version (104K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7A —2-day-old female neonate with Down syndrome, right aortic arch with left descending thoracic aorta and hypoplastic retroesophageal segment, ventricular septal defect, and bilateral superior venae cavae. Coronal maximum-intensity-projection gadolinium-enhanced 3D MR angiography image shows apparent interruption (INT) of aortic arch between left common carotid artery (LCCA) and left subclavian artery (LSCLA). Right pulmonary artery (RPA) is seen. PDA = patent ductus arteriosus, DA = descending thoracic aorta.

View larger version (77K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7B —2-day-old female neonate with Down syndrome, right aortic arch with left descending thoracic aorta and hypoplastic retroesophageal segment, ventricular septal defect, and bilateral superior venae cavae. Volume-rendered image confirms presence of hypoplastic retroesophageal aortic segment (REA) on closer inspection. Patent ductus arteriosus (PDA) also supplies blood flow to descending thoracic aorta (DA). MPA = main pulmonary artery.

View larger version (129K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8 —21-year-old man with history of type A interrupted aortic arch (IAA) being evaluated following surgical repair. Sagittal maximum-intensity-projection (MIP) gadolinium-enhanced 3D MR angiography image shows vertically oriented ascending aorta (A) giving rise to origins of all great vessels (arrow). Synthetic patch is seen bridging area of aortic arch interruption and supplies blood flow to descending aorta (DA). Main pulmonary artery (PA) is also seen.

View larger version (79K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9A —14-year-old boy with history of type A interrupted aortic arch (IAA) being evaluated for status after repair of IAA. Synthetic graft material was used to bridge interrupted portion of aortic arch. Axial double inversion recovery fast spin-echo black blood MR image reveals area of proximal anastomotic narrowing (arrow).

View larger version (136K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9B —14-year-old boy with history of type A interrupted aortic arch (IAA) being evaluated for status after repair of IAA. Synthetic graft material was used to bridge interrupted portion of aortic arch. Sagittal oblique 2D balanced steady-state free precession image shows that ascending aorta (A) is not dilated proximally. Finding seen in A (arrow) is confirmed.

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