主动脉血管变异肺动静脉英课件

1、Vascular Anomalies of Aorta, Pulmonary and Systemic vessels主动脉血管变异肺动静脉英1Vascular Anomalies of Aorta, POverview 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

2、.Double Arch with Atretic Segment .主动脉血管变异肺动静脉英2Overview of Arch Anomalies1.AbEmbryologyDouble 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 Se

3、gment: Posterior part of the left arch becomes atretic. This remnant persists 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3EmbryologyDouble Arch: DoublNormal Left Arch: The posterior part of the right arch involutes. The two brachiocephalic vessels on the r

4、ight form the right innominate artery. Right Arch with mirror branching: Mirror image of normal left arch. 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4Normal Left Arch: 主动脉血管变异肺动静脉Left Arch with aberrant right su

5、bclavian 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 subclavian artery. The last branch is the right aberrant subclavian artery.Right Arch with aberrant left subclavian

6、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 aberrant subclavian artery.主动脉血管变异肺动静脉英5Left Arch with aberrant rightAortic Arch AnomaliesRight Arch Mirror Ima

7、geThis 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 subclavian artery, so this is a right arch mirror image. 主动脉血管变异肺动静脉英6Aort

8、ic Arch AnomaliesRight ArcThis 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. 主动脉血管变异肺动静脉英7This anoma

9、ly is asymptomatic, 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.主动脉血管变异肺动静脉英8ab

10、ove an adult who was operatRight Arch with Aberrant left subclavianThe 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9Right Arch with Aberrant left Below a patient with a right arch with an aberrant left subclavian (indicated by the yellow arrow).The y

11、ellow 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10Below a patient with a right aPosterior oblique view: Right Arch with Aberrant left subclavian (yellow arrow)In a mirror type right arch, th

12、e left subclavian is the first brach and forms the left innominate together with the left common carotid.主动脉血管变异肺动静脉英11Posterior oblique view: Right 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

13、 the trachea and the esophagus.The compression of the trachea is demonstrated on VR主动脉血管变异肺动静脉英12Below a symptomatic child.On Double 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 som

14、e 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. 主动脉血管变异肺动静脉英13Double Aortic ArchOn the left The right arch is typically larger and higher than the left.There is a c

15、omplete 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. 主动脉血管变异肺动静脉英14The right arch is typically laAbove a chest film of a young adult w

16、ith 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). 主动脉血管变异肺动静脉英15Above a chest film of a young The findings are: 1.four vessel sign 2.double arch3.right arch higher and

17、larger4.esophagus and trachea are completely encircled 主动脉血管变异肺动静脉英16The findings are: 主动脉血管变异肺动静脉英The 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17The narrowing of the trachea iAbove preoperative and postoperative MDCT studies of a 2-mo

18、nth-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18Above preoperative and postopeDouble Arch with Atretic Segment Occasionally the double arch can have an atretic segment. You should not confuse it for a

19、 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19Double Arch with Atretic SegmeAbove 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20Above a dominant right arch anOn a

20、posterior view the interruption is nicely demonstrated. Remember that there is still a ring, so there is still obstruction. 主动脉血管变异肺动静脉英21On a posterior view the interrAnother 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

21、 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英22Another case Above.主动脉血管变异肺动静脉Same 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23Same patient. Always look at tLeft Arch Aberran

22、t Right SCAAlso known as arteria lusoria. Most common arch anomaly. Not a true ringUsually asymptomatic. 主动脉血管变异肺动静脉英24Left Arch Aberrant Right SCAAAberrant Right SCA, no compression of the tracheaAbove a young patient, who has a CT for another reason. Notice that there is a left arch, but the right

23、 subclavian artery is the last brachiocephalic artery to branch off the arch. 主动脉血管变异肺动静脉英25Aberrant Right SCA, no compresOnly rarely these patients become dysphagic , when the origin of the right subclavian artery becomes dilated. On a barium study of the esophagus you will see a posterior impressi

24、on 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 esophagus and it originates from the left-sided aorta, i.e. an aberrant righ

25、t subclavian artery. 主动脉血管变异肺动静脉英26Only rarely these patients becDysphagia in patient with dilated aberrant right subclavian artery.主动脉血管变异肺动静脉英27Dysphagia in patient with dila the same patient with dilated aberrant right subclavian artery. Coronal reconstruction. 主动脉血管变异肺动静脉英28 the same patient wit

26、h dilatedBelow 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. 主动脉血管变异肺动静脉英29Below another patient with an 主动脉血管变异肺动静脉英30主动脉血管变异肺动静脉英30a 5 yea

27、r old girl with noisy breathing and occasional cyanosis.The findings 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31a 5 year old girl with noisy bThe diagnosis

28、 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 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 diagnosis is t

29、he innominatThe compression in the innominate artery compression syndrome is located on the right anterior side and at the level of the thoracic inlet.主动脉血管变异肺动静脉英33The compression in the innominOn the left another case with mild compression on the trachea.主动脉血管变异肺动静脉英34On the left another case with

30、 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 Coarctation主动脉血管变异肺动静脉英35Narrowing at l

31、evel of distal 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 heart failure. 主动脉血管变异肺

32、动静脉英36The findings are:a 2 month oldThere 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

33、 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37There are two types of coarctaIntercostal 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38Intercostal collaterals in aorThe findings are:Big in

34、ternal mammarian arteries on 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39The findings are:主动脉血管变异肺动

35、静脉英3Above two neonates with the pre-ductal type of coarctation.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40Above two neonates with the prCoarctation

36、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 stent is obstructing the orfice of the left subclavian artery. 主动脉血管变异肺动静脉英41Coarctation is treated with anBel

37、owOn 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 placement.They have to be repaired because they will rupture.Pseudo-aneurysms

38、 are seen in10% after angioplasty.30% after patch aortoplasty.主动脉血管变异肺动静脉英42Below主动脉血管变异肺动静脉英42Pseudo-aneurysm in coarctation treated with stent-placement 主动脉血管变异肺动静脉英43Pseudo-aneurysm in coarctationPulmonary Arterial anomaliesThey most common anomalies of the pulmonary arteries are listed in the ta

39、ble on the left.主动脉血管变异肺动静脉英44Pulmonary Arterial anomaliesThPulmonary 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 cy

40、anotic 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 主动脉血管变异肺动静脉Pulmonary agenesis on the right sideThe CT shows, that he right lung is not developed and the s

41、pace 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 righPulmonary agenesis on the left sideAnother case of absent pulmonary artery with absence of lung development.On the CT the left lung is a

42、bsent.These patients may be totally asymptomatic. 主动脉血管变异肺动静脉英47Pulmonary agenesis on the leftPulmonary 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 the

43、trachea.There is a little mass effect on the trachea. 主动脉血管变异肺动静脉英48Pulmonary Sling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 childr

44、en as it is more symptomatic 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49In pulmonary sling ,the left PBelow 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 pati

45、ents also have long segment stenosis in the trachea because of 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50Below a child with wheezing aPatent Ductus ArteriosusBelow an adolescent with a murmur.On axial image and reconstruction th

46、e patent ductus arteriosus is seen. 主动脉血管变异肺动静脉英51Patent Ductus Arteriosus主动脉血管变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 da

47、y of life there is a functional 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. 主动脉血管变异肺动静脉英52The ductus arteriosus is the cOn the left a young adult with a murmur.Th

48、e cardiologists are not interested in the flow direction, but just want to confirm the diagnosis.Notice the connection between the pulmonary artery and the descending aorta. 主动脉血管变异肺动静脉英53On the left a young adult withWhen the duct closes it may also calcify.This a normal ariant.主动脉血管变异肺动静脉英54When t

49、he duct closes it may alPulmonary venous anomaliesPartial Anomalous Venous ReturnThe most common features of PAVR are listed in the table .主动脉血管变异肺动静脉英55Pulmonary venous anomaliesPart The anomalous veins drain into the following structures:RUL: SVC association with sinus venosus-type ASD.RLL: IVC (u

50、sually), 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56 The anomalous veins drain inRight upper lobe anomalous venous returnBe

51、low 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57Right upper lobe anomalous venAll these partially anomalous pulmonary venous returns are left to right shun

52、ts, 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 hypertensi

53、on. 主动脉血管变异肺动静脉英58All these partially anomalous 主动脉血管变异肺动静脉英59主动脉血管变异肺动静脉英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 i

54、nto the left atrium.This type of ASD is called the sinus venosus-type ASD.主动脉血管变异肺动静脉英60Right upper lobe anomalous ret主动脉血管变异肺动静脉英61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62A similar case

55、.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 (Scimitar) Right lower lobe anomalous return主动脉血管变异肺动静脉英63The vein drains into the IVC. Right lower lobe anomalous venous return into the azygos vein.On th

56、e 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64Right lower lobe anomalous venA 10 year old girl suspected of having pneumonia.Study the images carefully, because there are three findings and th

57、en 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. 主动脉血管变异肺动静脉英65A 10 year old girl suspected o主动脉血管变异肺动静脉英6

58、6主动脉血管变异肺动静脉英66Scimitar syndromeThe features in scimitar syndrome are listed in the table 主动脉血管变异肺动静脉英67Scimitar syndrome主动脉血管变异肺动静脉英6Scimitar syndrome with a hypoplastic right lung.Another patient with a scimitar syndrome. There is a hypoplastic right lung with mediastinal shift and there is anomal

59、ous venous return. Notice that on the coronal MIP you can nicely see the difference in vascularization of the lungs with hypovascularity on the right. 主动脉血管变异肺动静脉英68Scimitar syndrome with a hypopNotice how the left upper lobe vein runs from the hilum cranially into the brachiocephalic vein. Left upp

60、er lobe anomalous venous return into brachiocephalic vein.主动脉血管变异肺动静脉英69Notice how the left upper lobeThe differential diagnosis of a left upper lobe anomalous venous return into brachiocephalic veins is a left Superior Vena Cava (SVC).A left SVC however drains into the coronary sinus. 主动脉血管变异肺动静脉英7