主动脉血管变异肺动静脉课件.ppt
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1、Vascular Anomalies of Aorta,Pulmonary and Systemic vessels1Overview of Arch Anomalies1.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.2Embryology Do
2、uble 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 persists as a fibrous cord
3、 tethering the anterior left arch to the descending aorta.3 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.Posterior part of the left arch
4、involutes.The two brachiocephalic vessels on the left form the left innominate artery.4Left 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,followed by the left carotid and the left
5、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 subclavian artery.The last branch is the left
6、 aberrant subclavian artery.5Aortic Arch AnomaliesRight Arch Mirror ImageThis 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 of the brachiocephalic arteries,no aberrant subcl
7、avian artery,so this is a right arch mirror image.6This 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.This patient had a mirror image aortic arch and a VSD.7
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 there is also a right arch.8Right Arch with Aberrant left subclavi
9、an The Right Aortic Arch with an aberrant left subclavian is an obstructing arch anomaly.9Below a patient with a right arch with an aberrant left subclavian(indicated by the yellow arrow).The yellow arrow indicates the azygos vein.?The green arrow indicates the left superior intercostal vein,a norma
10、l variant,that we will discuss later.10Posterior 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 together with the left common carotid.11Below a symptomatic child.On the axial image th
11、ere 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 VR12Double Aortic ArchOn the left a chest film of a 6-month old boy with stridor and cough.The trachea is deviated
12、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 and branches coming off the left arch.13The right arch is typically larger and higher than the
13、 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 there is no brachiocephalic artery.14Above a chest film of a young adult with a cough.There is a right
14、 paratracheal mass.The differential diagnosis is tumor,adenopathy or vessel(right arch,dilated azygos vein,dilated aberrant right subclavian artery).15The findings are:1.four vessel sign 2.double arch3.right arch higher and larger4.esophagus and trachea are completely encircled 16The narrowing of th
15、e trachea is seen on the axial images,but better appreciated on the MPR and VR17Above preoperative and postoperative MDCT studies of a 2-month-old female infant with double aortic arch presenting with stridor and repeated apnea.The smaller left arch is partially resected.18Double Arch with Atretic S
16、egment 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.19Above a dominant right arch and a small left arch.The atretic segment is marked by the arrow.Notice the four vessel sign
17、.20On a posterior view the interruption is nicely demonstrated.Remember that there is still a ring,so there is still obstruction.21Another case Above.Do not call this a right arch.It still is a double arch and there is a atretic fibrotic segment on the posterior side of the left arch,that completes
18、the ring.Notice the four vessel sign.22Same patient.Always look at the airways.On the reconstruction the impression on the trachea is better appreciated.23Left Arch Aberrant Right SCA Also known as arteria lusoria.Most common arch anomaly.Not a true ring Usually asymptomatic.24Aberrant Right SCA,no
19、compression of the tracheaAbove a young patient,who has a CT for another reason.Notice that there is a left arch,but the right subclavian artery is the last brachiocephalic artery to branch off the arch.25Only rarely these patients become dysphagic,when the origin of the right subclavian artery beco
20、mes dilated.On a barium study of the esophagus you will see a posterior impression with an oblique course directed towards the right shoulder.Below a 78 year old woman with dysphagia.There is consolidation in the right upper lobe,maybe due to aspiration.There is a dilated vessel that compresses the
21、esophagus and it originates from the left-sided aorta,i.e.an aberrant right subclavian artery.26Dysphagia in patient with dilated aberrant right subclavian artery.27 the same patient with dilated aberrant right subclavian artery.Coronal reconstruction.28 Below another patient with an aberrant right
22、subclavian.When you follow the artery from inferior to superior,it starts on the left side of the arch and travels obliquely behind the esophagus to go to the right.2930a 5 year old girl with noisy breathing and occasional cyanosis.The findings are:1.anterior compression of the trachea 2.brachioceph
23、alic(innominate)artery is located more to the left and compresses the trachea Innominate artery compression syndrome 31 The diagnosis is the innominate artery compression syndrome.In infants the innominate artery arises more to the left than in adults,so its got to go in front of the trachea.It may
24、compress the trachea,leading to stridor,cough and dyspnea.This compression decreases with age.32The compression in the innominate artery compression syndrome is located on the right anterior side and at the level of the thoracic inlet.33On the left another case with mild compression on the trachea.3
25、4 Narrowing at level of distal arch/descending aorta.Chest film:figure 3 sign,inferior rib notching.Intervention when gradient 20 mm Hg.Associated with bicuspid aortic valve(75%),cerebral aneurysms(5-10%)and Turner syndrome(20%have coarctation)Aortic Coarctation35The findings are:Large thymus which
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