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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
26、coarctation),Aortic Coarctation,36,The findings are:Large thymus which is normal for a 2 month old.Striking discrepancy between diameter of ascending and descending aorta.The diagnosis is coarctation,which is nicely demonstrated on the posterior view of the reconstruction.,a 2 month old boy with hea
27、rt failure.,37,There are two types of coarctation.The type we usually see is the post-ductal type,which is distal to the left subclavian artery.The uncommon pre-ductal type is seen in neonates.They present with severe heart failure,mostly within the first week of life,usually on the first day.The oc
28、clusion is in front of the left subclavian.,38,Intercostal collaterals in aortic coarctation,The intercostal collaterals typically occur between the 3rd and the 8th rib.,39,The findings are:Big internal mammarian arteries on the axial image due to a high grade stenosis as a result of a coarctation.P
29、robably could not make the diagnosis based on the axial images alone.Post-ductal coartation only seen on sagittal reconstruction.Intercostal collaterals.,40,Above two neonates with the pre-ductal type of coarctation.The stenosis is in front of the left subclavia and there is arch hypoplasia.Collater
30、als do not occur,probably because they dont have time to develop.,Pre-ductal type of coarctation,41,Coarctation is treated with angioplasty,stent placement or patch aortoplasty.The image on the far left is the result after angioplasty.Next to it a patient who was treated with a stent.Notice that the
31、 stent is obstructing the orfice of the left subclavian artery.,42,BelowOn the far left a patient who was treated with a stent.But the stent ruptured causing restenosis.Next to it two patients with pseudo-aneurysm.One after angioplasty and another who developed a pseudo-aneurysm after stent placemen
32、t.They have to be repaired because they will rupture.Pseudo-aneurysms are seen in10%after angioplasty.30%after patch aortoplasty.,43,Pseudo-aneurysm in coarctation treated with stent-placement,44,Pulmonary Arterial anomalies,They most common anomalies of the pulmonary arteries are listed in the tabl
33、e on the left.,45,Pulmonary agenesis Also called congenital interruption of the pulmonary artery.Unilateral absence of the pulmonary artery.Small lung and hilum.Compensatory hyperinflation of contralateral lung with herniation.Below a young adult,who had cyanotic spells as a child.She is now in good
34、 health and comes in for another reason.On the chest film the differential is atelectasis,pneumonia or maybe a tumor.,46,Pulmonary agenesis on the right side,The CT shows,that he right lung is not developed and the space around the atresic pulmonary artery is filled with fibrofatty tissue with colla
35、terals.So this is pulmonary agenesis,47,Pulmonary agenesis on the left side,Another case of absent pulmonary artery with absence of lung development.On the CT the left lung is absent.These patients may be totally asymptomatic.,48,Pulmonary SlingBelow a 4 month old girl with abnormal echo,benign hear
36、t murmur and no respiratory or feeding difficulties.The sagittal reconstruction shows an anomalous vessel on the posterior side of the trachea.There is a little mass effect on the trachea.,49,In pulmonary sling,the left PA originates from the right PA and courses between the esophagus and the trache
37、a,where it compresses the right main bronchus.Pulmonary sling is seen more frequent in children as it is more symptomatic than in adults,because the chest is smaller,but you can also encounter it in adults.,50,Below a child with wheezing and dyspnea.The left PA comes off the right PA and runs betwee
38、n the esophagus and the trachea.Some of these patients also have long segment stenosis in the trachea because of cartilagenous rings.,Pulmonary Sling with long segment stenosis of the trachea.(Courtesy J.Schoef),51,Patent Ductus ArteriosusBelow an adolescent with a murmur.On axial image and reconstr
39、uction the patent ductus arteriosus is seen.,52,The ductus arteriosus is the communication between the pulmonary artery and the proximal descending aorta.It shunts blood in utero from the right ventricle to the aorta to bypass the non-functioning lungs.On the first day of life there is a functional
40、closure and an anatomic closure with fibrosis in the first two weeks.If it does not close these patients come to attention either with a murmur or later with pulmonary hypertension.,53,On the left a young adult with a murmur.The cardiologists are not interested in the flow direction,but just want to
41、 confirm the diagnosis.Notice the connection between the pulmonary artery and the descending aorta.,54,When the duct closes it may also calcify.This a normal ariant.,55,Pulmonary venous anomalies,Partial Anomalous Venous ReturnThe most common features of PAVR are listed in the table.,56,The anomalou
42、s veins drain into the following structures:RUL:SVC association with sinus venosus-type ASD.RLL:IVC(usually),sometimes Portal or Hepatic vein.Can be isolated finding or combined with pulmonary hypoplasia(Scimitar syndrome).LUL:Brachiocephalic vein(isolated finding).LLL:rare(if you find a case publis
43、h it).,57,Right upper lobe anomalous venous returnBelow a 2 month old,who is asymptomatic but has a murmur on physical examination.There is a connection between the SVC and a pulmonary vein,so this is an anomalous venous return.,58,All these partially anomalous pulmonary venous returns are left to r
44、ight shunts,but when small,they are clinically insignificant.When there is a significant shunt,they may cause(late)pulmonary hypertension as seen in the case on the left.The chest film in this adult shows large pulmonary arteries and a large right atrium and ventricle as a result of pulmonary hypert
45、ension.,59,60,Right upper lobe anomalous return(2)Below a patient with a murmur.There is an anomalous return of the right upper lobe to the SVC.At a slightly inferior level there is also an ASD.Contrast is seen going almost immediately into the left atrium.This type of ASD is called the sinus venosu
46、s-type ASD.,61,62,A similar case.Notice the anomalous return of the right upper lobe vein into the VCS and the additional ASD at a lower level.,63,The vein drains into the IVC.The anomalous vein gently curves to the right cardiophrenic angle and is shaped like a Turkish sword(Scimitar),Right lower l
47、obe anomalous return,64,Right lower lobe anomalous venous return into the azygos vein.,On the left another right lower lobe anomalous return.The vein drains into the azygos vein.Upper lobe veins may also drain into the azygos vein.,65,A 10 year old girl suspected of having pneumonia.Study the images
48、 carefully,because there are three findings and then continue reading.The findings are:Small right lung due to hypoplasia Anomalous venous returnRight aortic archThis patient has a scimitar syndrome and also a right arch.So the lesson is,that when you see one anomaly,look for another one.,66,67,Scim
49、itar syndromeThe features in scimitar syndrome are listed in the table,68,Scimitar syndrome with a hypoplastic right lung.,Another patient with a scimitar syndrome.There is a hypoplastic right lung with mediastinal shift and there is anomalous venous return.Notice that on the coronal MIP you can nic
50、ely see the difference in vascularization of the lungs with hypovascularity on the right.,69,Notice how the left upper lobe vein runs from the hilum cranially into the brachiocephalic vein.,Left upper lobe anomalous venous return into brachiocephalic vein.,70,The differential diagnosis of a left upp
