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1、1,Vascular Anomalies of Aorta, Pulmonary and Systemic vessels,2,Overview of Arch Anomalies,1.Aberrant Right subclavian artery. 2.Innominate artery compression syndrome . 3.Right Arch Mirror Image . 4.Right Arch with Aberrant left subclavian . 5.Double Aortic Arch . 6.Double Arch with Atretic Segment
2、 .,3,Embryology,Double Arch: In the embryo a double arch with two brachiocephalic vessels on each side is present. If double aortic arch persists, it forms a vascular ring around trachea and esophagus.,Double Arch with Atretic Segment: Posterior part of the left arch becomes atretic. This remnant pe
3、rsists as a fibrous cord tethering the anterior left arch to the descending aorta.,4,Normal Left Arch: The posterior part of the right arch involutes. The two brachiocephalic vessels on the right form the right innominate artery. Right Arch with mirror branching: Mirror image of normal left arch. Po
4、sterior part of the left arch involutes. The two brachiocephalic vessels on the left form the left innominate artery.,5,Left Arch with aberrant right subclavian artery: Right arch between the right subclavian and right common carotid artery involutes. First branch is the right common carotid, follow
5、ed by the left carotid and the left subclavian artery. The last branch is the right aberrant subclavian artery. Right Arch with aberrant left subclavian artery: Mirror image of the left arch with aberrant right subclavian First branch is left common carotid, followed by right carotid and right subcl
6、avian artery. The last branch is the left aberrant subclavian artery.,6,Aortic Arch Anomalies,Right Arch Mirror Image This is the mirror-image variety of the left arch. a 2 year old girl with wheezing and coughing. On the axial image there is a right arch. On the VR there is mirror image branching o
7、f the brachiocephalic arteries, no aberrant subclavian artery, so this is a right arch mirror image.,7,This anomaly is asymptomatic, because there is no obstructing ring. Almost all of these patients however come to our attention because they have associated congenital heart disease in 98% of cases.
8、 This patient had a mirror image aortic arch and a VSD.,8,above an adult who was operated in his childhood for a Tetralogy of Fallot (pulmonary stenosis, right ventricular hypertrophy, VSD, overriding aorta). At surgery the VSD was patched and the pulmonary outflow tract was enlarged. Notice that th
9、ere is also a right arch.,9,Right Arch with Aberrant left subclavian,The Right Aortic Arch with an aberrant left subclavian is an obstructing arch anomaly.,10,Below a patient with a right arch with an aberrant left subclavian (indicated by the yellow arrow). The yellow arrow indicates the azygos vei
10、n.? The green arrow indicates the left superior intercostal vein, a normal variant, that we will discuss later.,11,Posterior oblique view: Right Arch with Aberrant left subclavian (yellow arrow),In a mirror type right arch, the left subclavian is the first brach and forms the left innominate togethe
11、r with the left common carotid.,12,Below a symptomatic child. On the axial image there is a right arch with the left subclavian artery that comes off on the posterior side and runs behind the trachea and the esophagus. The compression of the trachea is demonstrated on VR,13,Double Aortic Arch On the
12、 left a chest film of a 6-month old boy with stridor and cough. The trachea is deviated to the left, otherwise the chest film is normal. So there is some mass effect on the right side.,On the left the reconstructions demonstrating a double aortic arch. There are branches coming off the right arch an
13、d branches coming off the left arch.,14,The right arch is typically larger and higher than the left. There is a complete ring that encircles the esophagus and the trachea and usually there is stridor or dysphagia. Two brachiocephalic arteries arise on each side separately (four vessel sign) and ther
14、e is no brachiocephalic artery.,15,Above a chest film of a young adult with a cough. There is a right paratracheal mass. The differential diagnosis is tumor, adenopathy or vessel (right arch, dilated azygos vein, dilated aberrant right subclavian artery).,16,The findings are: 1.four vessel sign 2.do
15、uble arch 3.right arch higher and larger 4.esophagus and trachea are completely encircled,17,The narrowing of the trachea is seen on the axial images, but better appreciated on the MPR and VR,18,Above preoperative and postoperative MDCT studies of a 2-month-old female infant with double aortic arch
16、presenting with stridor and repeated apnea. The smaller left arch is partially resected.,19,Double Arch with Atretic Segment Occasionally the double arch can have an atretic segment. You should not confuse it for a right arch. The left arch is just very small and there is still a four vessel sign.,20,Above a dominant right arch and a small left arch. The atretic segment is marked by the arrow. Notice the four vessel sign.,21,On a posterior view the interruption is nicely demons
