1、Vascular Anomalies of Aorta,Pulmonary and Systemic vessels主动脉血管变异肺动静脉英1Overview 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.主动
2、脉血管变异肺动静脉英2Embryology 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.主动脉血管变异肺动静脉英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 a
4、rch.Posterior part of the left arch involutes.The two brachiocephalic vessels on the left form the left innominate artery.主动脉血管变异肺动静脉英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 ca
5、rotid,followed 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 rig
6、ht subclavian artery.The last branch is the left aberrant subclavian artery.主动脉血管变异肺动静脉英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
7、 branching of the brachiocephalic arteries,no aberrant subclavian artery,so this is a right arch mirror image.主动脉血管变异肺动静脉英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 diseas
8、e in 98%of cases.This patient had a mirror image aortic arch and a VSD.主动脉血管变异肺动静脉英7above 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 enl
9、arged.Notice that there is also a right arch.主动脉血管变异肺动静脉英8Right Arch with Aberrant left subclavian The Right Aortic Arch with an aberrant left subclavian is an obstructing arch anomaly.主动脉血管变异肺动静脉英9Below a patient with a right arch with an aberrant left subclavian(indicated by the yellow arrow).The
10、yellow arrow indicates the azygos vein.?The green arrow indicates the left superior intercostal vein,a normal variant,that we will discuss later.主动脉血管变异肺动静脉英10Posterior oblique view:Right Arch with Aberrant left subclavian(yellow arrow)In a mirror type right arch,the left subclavian is the first bra
11、ch and forms the left innominate together with the left common carotid.主动脉血管变异肺动静脉英11Below 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
12、 demonstrated on VR主动脉血管变异肺动静脉英12Double Aortic ArchOn the 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
13、arch.There are branches coming off the right arch and branches coming off the left arch.主动脉血管变异肺动静脉英13The 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 arteri
14、es arise on each side separately(four vessel sign)and there is no brachiocephalic artery.主动脉血管变异肺动静脉英14Above 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 su
15、bclavian artery).主动脉血管变异肺动静脉英15The findings are:1.four vessel sign 2.double arch3.right arch higher and larger4.esophagus and trachea are completely encircled 主动脉血管变异肺动静脉英16The narrowing of the trachea is seen on the axial images,but better appreciated on the MPR and VR主动脉血管变异肺动静脉英17Above preoperati
16、ve 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.主动脉血管变异肺动静脉英18Double Arch with Atretic Segment Occasionally the double arch can have an atretic segment.You should not confu
17、se it for a right arch.The left arch is just very small and there is still a four vessel sign.主动脉血管变异肺动静脉英19Above a dominant right arch and a small left arch.The atretic segment is marked by the arrow.Notice the four vessel sign.主动脉血管变异肺动静脉英20On a posterior view the interruption is nicely demonstrat
18、ed.Remember that there is still a ring,so there is still obstruction.主动脉血管变异肺动静脉英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 the ring.Notice the four vessel sign.主动脉血管变异肺动静脉
19、英22Same patient.Always look at the airways.On the reconstruction the impression on the trachea is better appreciated.主动脉血管变异肺动静脉英23Left Arch Aberrant Right SCA Also known as arteria lusoria.Most common arch anomaly.Not a true ring Usually asymptomatic.主动脉血管变异肺动静脉英24Aberrant Right SCA,no compression
20、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.主动脉血管变异肺动静脉英25Only rarely these patients become dysphagic,when the origin of the right subclavian artery beco
21、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
22、esophagus and it originates from the left-sided aorta,i.e.an aberrant right subclavian artery.主动脉血管变异肺动静脉英26Dysphagia in patient with dilated aberrant right subclavian artery.主动脉血管变异肺动静脉英27 the same patient with dilated aberrant right subclavian artery.Coronal reconstruction.主动脉血管变异肺动静脉英28 Below ano
23、ther 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.主动脉血管变异肺动静脉英29主动脉血管变异肺动静脉英30a 5 year old girl with noisy breathing and occasional cyanosis.The find
24、ings 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 主动脉血管变异肺动静脉英31 The diagnosis is the innominate artery compression syndrome.In infants the innominate artery arises more to
25、the left 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.主动脉血管变异肺动静脉英32The compression in the innominate artery compression syndrome is located on the right anterior side and at the level of
26、the thoracic inlet.主动脉血管变异肺动静脉英33On the left another case with mild compression on the trachea.主动脉血管变异肺动静脉英34 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 aneu
27、rysms(5-10%)and Turner syndrome(20%have coarctation)Aortic Coarctation主动脉血管变异肺动静脉英35The 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
28、of the reconstruction.a 2 month old boy with heart failure.主动脉血管变异肺动静脉英36There 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 wit
29、hin the first week of life,usually on the first day.The occlusion is in front of the left subclavian.主动脉血管变异肺动静脉英37Intercostal collaterals in aortic coarctationThe intercostal collaterals typically occur between the 3rd and the 8th rib.主动脉血管变异肺动静脉英38The findings are:Big internal mammarian arteries o
30、n the axial image due to a high grade stenosis as a result of a coarctation.Probably could not make the diagnosis based on the axial images alone.Post-ductal coartation only seen on sagittal reconstruction.Intercostal collaterals.主动脉血管变异肺动静脉英39Above two neonates with the pre-ductal type of coarctati
31、on.The stenosis is in front of the left subclavia and there is arch hypoplasia.Collaterals do not occur,probably because they dont have time to develop.Pre-ductal type of coarctation主动脉血管变异肺动静脉英40Coarctation is treated with angioplasty,stent placement or patch aortoplasty.The image on the far left i
32、s the result after angioplasty.Next to it a patient who was treated with a stent.Notice that the stent is obstructing the orfice of the left subclavian artery.主动脉血管变异肺动静脉英41 Below On the far left a patient who was treated with a stent.But the stent ruptured causing restenosis.Next to it two patients
33、 with pseudo-aneurysm.One after angioplasty and another who developed a pseudo-aneurysm after stent placement.They have to be repaired because they will rupture.Pseudo-aneurysms are seen in 10%after angioplasty.30%after patch aortoplasty.主动脉血管变异肺动静脉英42Pseudo-aneurysm in coarctation treated with sten
34、t-placement 主动脉血管变异肺动静脉英43Pulmonary Arterial anomalies They most common anomalies of the pulmonary arteries are listed in the table on the left.主动脉血管变异肺动静脉英44Pulmonary agenesis Also called congenital interruption of the pulmonary artery.Unilateral absence of the pulmonary artery.Small lung and hilum
35、.Compensatory hyperinflation of contralateral lung with herniation.Below a young adult,who had cyanotic spells as a child.She is now in good health and comes in for another reason.On the chest film the differential is atelectasis,pneumonia or maybe a tumor.主动脉血管变异肺动静脉英45Pulmonary agenesis on the rig
36、ht sideThe CT shows,that he right lung is not developed and the space around the atresic pulmonary artery is filled with fibrofatty tissue with collaterals.So this is pulmonary agenesis主动脉血管变异肺动静脉英46Pulmonary agenesis on the left sideAnother case of absent pulmonary artery with absence of lung devel
37、opment.On the CT the left lung is absent.These patients may be totally asymptomatic.主动脉血管变异肺动静脉英47Pulmonary SlingBelow a 4 month old girl with abnormal echo,benign heart murmur and no respiratory or feeding difficulties.The sagittal reconstruction shows an anomalous vessel on the posterior side of t
38、he trachea.There is a little mass effect on the trachea.主动脉血管变异肺动静脉英48In pulmonary sling,the left PA originates from the right PA and courses between the esophagus and the trachea,where it compresses the right main bronchus.Pulmonary sling is seen more frequent in children as it is more symptomatic
39、than in adults,because the chest is smaller,but you can also encounter it in adults.主动脉血管变异肺动静脉英49Below a child with wheezing and dyspnea.The left PA comes off the right PA and runs between the esophagus and the trachea.Some of these patients also have long segment stenosis in the trachea because of
40、 cartilagenous rings.Pulmonary Sling with long segment stenosis of the trachea.(Courtesy J.Schoef)主动脉血管变异肺动静脉英50Patent Ductus ArteriosusBelow an adolescent with a murmur.On axial image and reconstruction the patent ductus arteriosus is seen.主动脉血管变异肺动静脉英51The ductus arteriosus is the communication be
41、tween 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 closure and an anatomic closure with fibrosis in the first two weeks.If it does not close the
42、se patients come to attention either with a murmur or later with pulmonary hypertension.主动脉血管变异肺动静脉英52On the left a young adult with a murmur.The cardiologists are not interested in the flow direction,but just want to confirm the diagnosis.Notice the connection between the pulmonary artery and the d
43、escending aorta.主动脉血管变异肺动静脉英53When the duct closes it may also calcify.This a normal ariant.主动脉血管变异肺动静脉英54Pulmonary venous anomalies Partial Anomalous Venous Return The most common features of PAVR are listed in the table.主动脉血管变异肺动静脉英55 The anomalous veins drain into the following structures:RUL:SVC
44、 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 publish it).主动脉血管变异肺动静脉英56Right upper lobe anomalous veno
45、us 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.主动脉血管变异肺动静脉英57All these partially anomalous pulmonary venous returns are left to right shunts,but when small,they
46、 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 hypertension.主动脉血管变异肺动静脉英58主动脉血管变异肺动静
47、脉英59Right 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 venosus-type ASD
48、.主动脉血管变异肺动静脉英60主动脉血管变异肺动静脉英61A similar case.Notice the anomalous return of the right upper lobe vein into the VCS and the additional ASD at a lower level.主动脉血管变异肺动静脉英62The vein drains into the IVC.The anomalous vein gently curves to the right cardiophrenic angle and is shaped like a Turkish sword(Sc
49、imitar)Right lower lobe anomalous return主动脉血管变异肺动静脉英63Right 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.主动脉血管变异肺动静脉英64A 10 year old girl suspect
50、ed of having pneumonia.Study the images 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 a
