主动脉血管变异肺动静脉英.ppt

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

2、 .,3,Embryology,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.,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. Po

4、sterior part of the left 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, follow

5、ed 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 right subcl

6、avian artery. The last branch is the left aberrant subclavian artery.,6,Aortic Arch Anomalies,Right Arch Mirror Image This 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 o

7、f the brachiocephalic arteries, no 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.

8、 This patient had a mirror image aortic 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 th

9、ere is also a right arch.,9,Right Arch with Aberrant 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 vei

10、n.? The green arrow indicates the left superior intercostal 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 togethe

11、r with the left common carotid.,12,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 the trachea and the esophagus.The compression of the trachea is demonstrated on VR,13,Double Aortic Arch On the l

12、eft 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 arch. There are branches coming off the right arch and

13、branches coming off the left arch.,14,The 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 arteries arise on each side separately (four vessel sign) and there

14、is no brachiocephalic artery.,15,Above 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 subclavian artery).,16,The findings are: 1.four vessel sign 2.doub

15、le arch 3.right arch higher and larger 4.esophagus and trachea are completely 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 pr

16、esenting with stridor and repeated apnea. The smaller left arch is partially 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,

17、Above a dominant right arch and a small left arch. The atretic segment is marked 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 ri

18、ght 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.,23,Same patient. Always look at the airways. On the reconstruction the impression on the trachea is better appreciated.,24,Left Ar

19、ch Aberrant Right SCA,Also known as arteria lusoria. Most common arch anomaly. Not a true ring Usually 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 l

20、ast brachiocephalic artery to branch off the arch.,26,Only 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 impression with an oblique course directed towards the right shoulder. Below

21、 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 right subclavian artery.,27,Dysphagia in patient with dilated aberrant r

22、ight subclavian artery.,28,the same patient with dilated aberrant right subclavian artery. Coronal reconstruction.,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

23、 esophagus to go to the right.,30,31,a 5 year 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,32,The diag

24、nosis 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 .,33,The compression in the

25、innominate artery compression syndrome is located on the right anterior side and at the level of the thoracic inlet.,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. Interventi

26、on when gradient 20 mm Hg. Associated with bicuspid aortic valve (75%), cerebral aneurysms (5-10%) and Turner syndrome (20% have 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 aort

27、a. The diagnosis is coarctation, which is nicely demonstrated on the posterior view of the reconstruction.,a 2 month old boy with heart 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-duc

28、tal type is seen in neonates. They present with severe heart failure, mostly within the first week of life, usually on the first day. The occlusion is in front of the left subclavian.,38,Intercostal collaterals in aortic coarctation,The intercostal collaterals typically occur between the 3rd and the

29、 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. Probably could not make the diagnosis based on the axial images alone. Post-ductal coartation only seen on sagittal reconstruction. Intercostal collaterals.,40

30、,Above 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,41,Coarctation is treated with angioplasty, stent placem

31、ent 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.,42,Below On the far left a patient who was treated with a stent.But the stent rupture

32、d 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 are seen in 10% after angioplasty. 30% after patch aortoplasty.,43,Pseudo-

33、aneurysm in coarctation treated with stent-placement,44,Pulmonary Arterial anomalies,They most common anomalies of the pulmonary arteries are listed in the table on the left.,45,Pulmonary agenesis Also called congenital interruption of the pulmonary artery. Unilateral absence of the pulmonary artery

34、. 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 health and comes in for another reason. On the chest film the differential is atelectasis, pneumonia or maybe a tumor.,46,Pulmonary a

35、genesis 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 collaterals.So this is pulmonary agenesis,47,Pulmonary agenesis on the left side,Another case of absent pulmonary artery with absence of

36、 lung development. On the CT the left lung is absent. These patients may be totally asymptomatic.,48,Pulmonary Sling Below 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 sid

37、e 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 trachea, where it compresses the right main bronchus. Pulmonary sling is seen more frequent in children as it is more symptomatic

38、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 between the esophagus and the trachea. Some of these patients also have long segment stenosis in the trachea because of carti

39、lagenous rings.,Pulmonary Sling with long segment stenosis of the trachea. (Courtesy J. Schoef),51,Patent Ductus Arteriosus Below an adolescent with a murmur. On axial image and reconstruction the patent ductus arteriosus is seen.,52,The ductus arteriosus is the communication between the pulmonary a

40、rtery 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 these patients come t

41、o 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 confirm the diagnosis. Notice the connection between the pulmonary artery and the descending aorta.,54,When

42、the duct closes it may also calcify.This a normal ariant.,55,Pulmonary venous anomalies,Partial Anomalous Venous Return The most common features of PAVR are listed in the table .,56,The anomalous veins drain into the following structures: RUL: SVC association with sinus venosus-type ASD. RLL: IVC (u

43、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).,57,Right upper lobe anomalous venous return Below a 2 month old, who is asymptomatic

44、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 right shunts, but when small, they are clinically insignificant. When there is a signific

45、ant 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.,59,60,Right upper lobe anomalous return (2) Below a patient with a murmur

46、. 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.,61,62,A similar case. Notice the anomalous return of the right

47、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 lobe anomalous return,64,Right lower lobe anomalous venous return into th

48、e 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 carefully, because there are three findings and then continue readin

49、g. The findings are: Small right lung due to hypoplasia Anomalous venous return Right aortic arch This 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,Scimitar syndrome The features in scimitar syndrome are listed in

50、 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 nicely see the difference in vascularization of the lungs wit

51、h 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 upper lobe anomalous venous return into brachiocephalic veins

52、 is a left Superior Vena Cava (SVC). A left SVC however drains into the coronary sinus.,71,Systemic veins,Left Superior Vena Cava Represents persistent left common cardinal vein Passes anterior to left main bronchus and drains into dilated coronary sinus 0.5% of general population and 5% of patients

53、 with congenital heart disease Small Right SVC in 90% of cases,72,There is a vascular structure, that runs inferiorly below the level of the left hilum and enters into a dilated coronary sinus. The diagnosis is left or double superior vena cava.,73,Left Superior Intercostal Vein. This is an anastomo

54、sis between the accessory hemiazygos vein and the left brachiocephalic vein. It courses along the lateral margin of the aortic arch (aortic nipple). It is a normal variant and if you look for this structure you will frequently notice it. Catheters or pacemaker leads may course along left side of med

55、iastinum. A patient with a left superior intercostal vein. Notice the aortic nipple sign.,74,75,On the left another example of a left superior intercostal vein.It courses along the lateral margin of the aortic arch from the the accessory hemiazygos vein to the left brachiocephalic vein.,76,Summary o

56、f left paramediastinal structures 1.Left VCS: from subclavian vein to coronary sinus 2.Anomalous LUL pulmonary vein: from left pulmonary hilum to brachiocephalic vein. 3.Left superior intercostal vein: from accessory hemiazygos vein to left brachiocephalic vein.,77,Azygos Continuation of IVC Abcense

57、 of hepatic segment of IVC with azygos continuation. IVC interrupted above level of renal veins. Association with congenital heart disease and polysplenia,78,Ideally a 64 slice scanner is used, but even a 4-slice scanner will suffice for studying vascular anomalies. The technique for these anomalies

58、 in the chest is the same as we use for pulmonary embolus detection. Thin collimation is used in combination with a fast table speed in order to get the highest resolution with the lowest radiation exposure. Usually a pitch of 1.5 is used. In children we preferably do not use thin collimation, becau

59、se of the higher radiation exposure, but these anomalies can be very small (voorbeeld dia 18), so thin collimation is necessary.,Technique and Protocol,79,80,mAs and kVp In a child with a weight of less than 10Kg 40mAs will work in the chest. In children with a weight more than 45 Kg adult protocols are used with 100 mAs or more. In small children under 50 kg you can decrease the kVp to 80 and that works very well in the chest. Remember in the chest there is inherent contrast from the lungs and by dropping the kVp you enhance this contrast.,81,82,On the left a 3-year old. Non-brea