主动脉血管变异肺动静脉英.ppt
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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.,3,Embryo
2、logy,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 persists as a fibr
3、ous 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.Posterior part of the l
4、eft 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,followed by the left carotid an
5、d 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 subclavian artery.The last branch
6、is the left aberrant subclavian artery.,6,Aortic Arch Anomalies,Right 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
7、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.This patient had a mirror image aortic
8、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 there is also a right arch.,9,Right Arch with Ab
9、errant 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 vein.?The green arrow indicates the left superior i
10、ntercostal 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 together with the left common carotid.,12,Below a symptomatic
11、 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 ArchOn the left a chest film of a 6-month old boy with stridor and
12、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 and branches coming off the left arch.,14,The right arch is typ
13、ically 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 there is no brachiocephalic artery.,15,Above a chest film of a young
14、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.double arch3.right arch higher and larger4.esophagus and trachea are compl
15、etely 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 presenting with stridor and repeated apnea.The smaller left arch is partial
16、ly 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 mar
17、ked by the arrow.Notice the four vessel sign.,21,On a posterior view the interruption is nicely demonstrated.Remember that there is still a ring,so there is still obstruction.,22,Another case Above.Do not call this a right arch.It still is a double arch and there is a atretic fibrotic segment on the
18、 posterior side of the left arch,that completes the ring.Notice the four vessel sign.,23,Same patient.Always look at the airways.On the reconstruction the impression on the trachea is better appreciated.,24,Left Arch Aberrant Right SCA,Also known as arteria lusoria.Most common arch anomaly.Not a tru
19、e ringUsually asymptomatic.,25,Aberrant Right SCA,no compression of the trachea,Above 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.,26,Only rarely these patients become dysph
20、agic,when the origin of the right subclavian artery becomes 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 a
21、spiration.There is a dilated vessel that compresses the esophagus and it originates from the left-sided aorta,i.e.an aberrant right subclavian artery.,27,Dysphagia in patient with dilated aberrant right subclavian artery.,28,the same patient with dilated aberrant right subclavian artery.Coronal reco
22、nstruction.,29,Below another patient with an aberrant right 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.,30,31,a 5 year old girl with noisy breathing and occasional cyanosis.The f
23、indings are:1.anterior compression of the trachea 2.brachiocephalic(innominate)artery is located more to the left and compresses the trachea,Innominate artery compression syndrome,32,The diagnosis is the innominate artery compression syndrome.In infants the innominate artery arises more to the left
24、than in adults,so its got to go in front of the trachea.It may compress the trachea,leading to stridor,cough and dyspnea.This compression decreases with age.,33,The compression in the innominate artery compression syndrome is located on the right anterior side and at the level of the thoracic inlet.
25、,34,On the left another case with mild compression on the trachea.,35,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
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