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1、Cardiovascular MRI in Congenital Heart DiseaseAn Imaging AtlasShankar Sridharan Gemma PriceOliver Tann Marina Hughes Vivek MuthuranguAndrew M. TaylorCardiovascular MRIin Congenital HeartDiseaseAn Imaging Atlas13From:The Centre for Cardiovascular MRUCL Institute of Child Health & Great Ormond Street
2、Hospital for ChildrenCardiovascular UnitGreat Ormond StreetLondon WC1N 3JHUKDr. Shankar SridharanLocum Consultant Paediatric CardiologistGemma PriceMedical IllustratorDr. Oliver TannConsultant In Cardiovascular ImagingDr. Marina HughesConsultant Paediatric CardiologistClinical Lead for CMRDr. Vivek
3、MuthuranguBHF Intermediate Research FellowProfessor Andrew M. TaylorProfessor of Cardiovascular ImagingDirector Centre for Cardiovascular MRISBN 978-3-540-69836-4eISBN 978-3-540-69837-1DOI 10.1007/978-3-540-69837-1Springer Heidelberg Dordrecht London New YorkLibrary of Congress Control Number: 20099
4、38029 Springer-Verlag Berlin Heidelberg 2010This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned,speci cally the rights of translation, reprinting, reuse of illustrations, recitations, broadcasting, reproduction onmicro lm or in any other
5、 way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, andpermission for use must always be obtained from Springer-Verlag. Violations are liable for prosecution
6、 under theGerman Copyright Law.The use of general descriptive names, trademarks, etc. in this publication does not imply, even in the absence of aspeci c statement, that such names are exempt from the relevant protective laws and regulations and therefore freefor general use.Product liability: The p
7、ublishers cannot guarantee the accuracy of any information about dosage and applicationcontained in this book. In every individual case the user must check such information by consulting the relevantliterature.Cover design: eStudio Calamar Figueres BerlinPrinted on acid-free paper9876543210VPrefaceT
8、he last 10 years has seen explosive expansion of the number of centres performing cardiovas-cular magnetic resonance (CMR) imaging. The majority of this expansion has been in the eldof adult ischaemic imaging, but congenital heart disease remains one of the main indicationsfor CMR. Importantly, the
9、greatly improved survival of patients with congenital heart diseasegives us a burgeoning adult population living with the sequelae of the disease (grown-up con-genital heart disease GUCH).Without previous experience or formal training, the interpretation of CMR images ofpatients with congenital hear
10、t disease can be difcult. The main aim of this book is to create aportable resource that offers efcient access to high-quality MR (and where appropriate, CT)images of the common congenital and structural heart abnormalities. We hope that by provid-ing key images for each condition and a clear interp
11、retation of the MR appearances, we willimprove the readers understanding of the conditions, facilitate their interpretation of imagesand optimise the planning of the imaging protocols during their own practice of congenitalCMR.As with any publication from a single institution, the contents of this b
12、ook represent ourown practice. We have not written a denitive or exhaustive description of the conditions.However, we hope that we have produced a factual, simple and eye-pleasing guide for fellowstraining in CMR, radiographers and technicians performing CMR scans, physician users ofCMR, and perhaps
13、 those few in adult ischaemic practice, who may need the occasional aidememoir for incidental ndings!We hope that you will nd this book useful in your everyday practice and learning.Shankar SridharanGemma PriceOliver TannMarina HughesVivek MuthuranguLondon, UKAndrew TaylorVIIContents12345678Technica
14、l Considerations. . . . . . . . . . . . . . . . . . .MR Imaging Under GA. . . . . . . . . . . . . . . . . . . .Imaging Protocol . . . . . . . . . . . . . . . . . . . . . . . . .Normal Anatomy-Axial. . . . . . . . . . . . . . . . . . . .Normal Anatomy-Coronal. . . . . . . . . . . . . . . . . .Normal
15、Anatomy-Sagittal . . . . . . . . . . . . . . . . . .Image Planes-Ventricles . . . . . . . . . . . . . . . . . . .Imaging Planes-Left Ventricular Outow Tract . . . . . . . . . . . . . .1236810121432 Tetralogy of Fallot: Repaired. . . . . . . . . . . . . . . . 7833 Pulmonary Stenosis . . . . . . . . .
16、 . . . . . . . . . . . . . . 8434 Percutaneous Pulmonary ValveImplantation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8635 Pulmonary Atresia and VSD . . . . . . . . . . . . . . . . 9036 Transposition of the Great Arteries:Arterial Switch Operation . . . . . . . . . . . . . . . . . . 92
17、37 Transposition of the Great Arteries:Senning and Mustard Repair . . . . . . . . . . . . . . . . 969 Imaging Planes-38 TGA with VSD and PS . . . . . . . . . . . . . . . . . . . . 100Right Ventricular Outow Tract . . . . . . . . . . . . .10a Imaging Planes-Branch PAs . . . . . . . . . . . . . . . .1
18、0b Imaging Planes-Thoracic Aorta. . . . . . . . . . . . . .11a Imaging Planes-Tricuspid Value . . . . . . . . . . . . .11b Imaging Planes-Mitral Value . . . . . . . . . . . . . . . .12 Imaging Planes-Coronary Arteries . . . . . . . . . . .13 Atrial Septal Defect . . . . . . . . . . . . . . . . . . .
19、 . . . .14 Sinus Venosus Defect . . . . . . . . . . . . . . . . . . . . .15 Atrioventricular Septal Defect. . . . . . . . . . . . . . .16 Ventricular Septal Defect. . . . . . . . . . . . . . . . . . .17 Aortic Valve Stenosis. . . . . . . . . . . . . . . . . . . . . .18 Aortic Valve Incompetence . .
20、. . . . . . . . . . . . . . .19 Coarctation of the Aorta . . . . . . . . . . . . . . . . . . .20 Repaired Coarctation of the Aorta:Complications . . . . . . . . . . . . . . . . . . . . . . . . . . .21 Interrupted Aortic Arch . . . . . . . . . . . . . . . . . . . .22 Aortic Vascular Rings . . . . . .
21、 . . . . . . . . . . . . . . .23 Left Pulmonary Artery Sling . . . . . . . . . . . . . . . .24 Marfan Syndrome . . . . . . . . . . . . . . . . . . . . . . . .25 Williams Syndrome . . . . . . . . . . . . . . . . . . . . . . .26 Mitral Stenosis. . . . . . . . . . . . . . . . . . . . . . . . . . .27 Mi
22、tral Regurgitation . . . . . . . . . . . . . . . . . . . . . .28 Hypertrophic Cardiomyopathy . . . . . . . . . . . . . .29 Dilated Cardiomypathy . . . . . . . . . . . . . . . . . . . .30 Noncompaction Cardiomyopathy . . . . . . . . . . . .31 Tetralogy of Fallot . . . . . . . . . . . . . . . . . . .
23、. . . . .1618192021222426283236404246485054565862646670727439 Congenitally Corrected Transpositionof the Great Arteries . . . . . . . . . . . . . . . . . . . . . . 10440 Common Arterial Trunk . . . . . . . . . . . . . . . . . . . 10841 Double Outlet Right Ventricle . . . . . . . . . . . . . . . 1124
24、2 Double Inlet Left Ventricle . . . . . . . . . . . . . . . . . 11643 Hypoplastic Left Heart Syndrome:Norwood Stage 1 . . . . . . . . . . . . . . . . . . . . . . . . . 11844 Bi-directional Cavo-pulmonary(Glenn) shunt . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12245 Fontan-Type Circulati
25、on(Tricuspid Atresia). . . . . . . . . . . . . . . . . . . . . . . . 12646 Total Cavo-pulmonary Connection . . . . . . . . . . . 13047 Anomalous Coronary Arteries. . . . . . . . . . . . . . . 13448 Anomalous Left Coronary Arteryfrom Pulmonary Artery . . . . . . . . . . . . . . . . . . . . 13849 Kawa
26、saki Disease . . . . . . . . . . . . . . . . . . . . . . . . 14050 Total Anomalous PulmonaryVenous Drainage . . . . . . . . . . . . . . . . . . . . . . . . . 14451 Partial Anomalous PulmonaryVenous Drainage . . . . . . . . . . . . . . . . . . . . . . . . . 14652 Ebsteins Anomaly . . . . . . . . . .
27、. . . . . . . . . . . . . 15053 Right Isomerism . . . . . . . . . . . . . . . . . . . . . . . . . 15454 Left Isomerism. . . . . . . . . . . . . . . . . . . . . . . . . . . 15855 List of Abbreviations . . . . . . . . . . . . . . . . . . . . . . 16256 Further reading . . . . . . . . . . . . . . . . .
28、. . . . . . . . . 16411Technical ConsiderationsPaediatric Challenges The usual technical difculties faced when perform-ing a cardiac MR examination are further ampliedwhen imaging small children Optimal image quality may be compromisedbecause of The smaller size of structures Faster heart rates The
29、reduced time for image acquisition (inabilityor difculty with breath-holding) The imaging protocol should be prioritised to obtainthe most crucial diagnostic information, in case thepatients cooperation is limited1. Spatial ResolutionSmaller eld-of-views (FOVs) and the use of thinnerslices are requi
30、red to image small anatomical structures.This leads to increased image resolution, but acorresponding reduction in signal:noise (S/N) ratio.This can be compensated by Increasing the number of acquisitions (disadvan-tage: increase in scan time) Removing parallel imaging features (disadvan-tage: incre
31、ase in scan time) Using a coarser matrix, to increase diagnostic imagequality, albeit at the cost of reduced resolution2. Appropriate Coil SelectionAppropriate coil selection is important to maximiseS/N ratio. A dedicated extremity (knee coil) should be used inneonates or very small children. A tran
32、smit/receive coil can reduce noise and increaseS/N ratio. If the child is too large for this, then a body matrixand spine coil combination achieves good results.3. Faster Heart RatesFaster heart rates in small children result in a short R-Rperiod. For sequences where repetition times (TR) arelonger
33、than the R-R period, gating, using the sec-ond or third R wave as the trigger facilitates moretime for the appropriate recovery of longitudinalmagnetisation. For cine-imaging, reducing the number of phaseencode steps in each frame will decrease the acqui-sition period for each frame, improving tempo
34、ralresolution and image sharpness. However, thisincreases scan times.4. Strategies to Reduce Motion Artefact Play therapy or pre-examination visits to the scan-ner can help a child overcome any anxiety andimprove in-magnet stillness. Installing a DVD/Video system is a worthwhileinvestment to promote
35、 prolonged distraction andcooperation. For children who have difculty breath-holding,images can be acquired during free breathing.Additionally: Use manual shimming techniques, as they areessential to minimise ow artefacts, particularlyon balanced SSFP sequences Increase the number of acquisitions (N
36、EX) from1 to 3 Use respiratory compensation methods toacquire data, e.g. use of navigator echoes, phasere-ordering algorithms. Acquire data using real time imaging sequences(where imaging systems allow).5. Contrast Administration in ChildrenFor angiography, we use 0.20.4 mL/kg of Dotarem,(Guerbet, P
37、aris) which corresponds to 0.10.2 mmol/kg. All Gadolinium contrast agents need to be givenin accordance with Institutional and National guide-lines to avoid nephrogenic sytemic brosis (NSF).For further information on this, see the UK RoyalCollege of Radiologists document on this subject:h t t p : /
38、/ w w w. r c r. a c . u k / d o c s / r a d i o l o g y / p d f /BFCR0714_Gadolinium_NSF_guidanceNov07.pdf6. Consider Alternative Imaging StrategiesCT is potentially useful if MR assessment is limited orhampered by technical restraints.2Cardiovascular MRI in Congenital Heart Disease2MR Imaging Under
39、 GAIndications for General Anaesthesia (GA)for Paediatric MRPractice varies throughout the world. However, mostcentres in the UK will perform cardiovascular MRunder general anaesthetic (GA) for children under theage of 7 years.General Safety Issues Specic to PaediatricCardiac Imaging Patient metal c
40、hecked and the safety questionnaireperformed with parents before the child isanaesthetised. Senior cardiac anaesthetist continuously present inevery case. Full monitoring: pulse oximetry, end-tidal gas anal-ysis, ECG and non-invasive BP. Wrap the patient in gamgee or blankets to keep himor her warm.
41、 Ten metre circle breathing system needed, to linkthe patient to anaesthetist in MR control room. Breath-holding in passive expiration, controlled byTechnical Factors Specic to MR in Infantsand Small Children Prolonged, multiple breath holds are required. Thiscan cause hypoxia. Adequate pause for ve
42、ntilationcontrol between breath holds is required. A reliable ECG is vital for gating during imageacquisition. Monitor patient temperature closely. The low ambi-ent temperature in MR scanning room produces ahypothermia risk, particularly for small infants.breaking the circuit in the control room. Th
43、e large dead space prohibits low owanaesthesia. Reversal of anaesthesia and extubation in CMRinduction room. Ensure that the team is aware of thecardiac arrest procedure.Importantly, the child MUST be withdrawn from theMR room for resuscitation. Metallic objects such asresuscitation trolley MUST NOT
44、 be brought into thescanning room.Environmental and Physical ConstraintsPerforming general anaesthesia (GA) in a magneticresonance (MR) environment is challenging for manyreasons During the scan, there is limited access to the childPowerinjectorAnaestheticmachinePhysiologicalmonitoringand ventilatio
45、n equipment. Care is required for staff and patient safety withregard to ferromagnetic equipment. There is a potential for RF interference with moni-toring equipment.Fig. 2.1. Photography showing one of our dedicatedpaediatric cardiac MR labs. Inset, control room withmonitoring equipment and long an
46、aesthetic tubing toenable the anaesthetist to sit in the controlroom duringMR scanning3Imaging Protocol3Imaging Protocol3Table 3.1 Suggested imaging protocols for given conditionsVent cinesCE-MRAFlowConditionsAxial BBNotesSooutVLA4-ChAVSA stackPAAoSVC3D SSFPRVOT CinesLVOT cinesPAAoLGEShuntsASD, SVD,
47、 AVSD,VSDPerfusionConsider AVSD ascomplex CHD. CEMRA for VSDValvarAS, AI, MR, MSAortaCoarctation, rings andslings, MarfanECG-gated CT indicatedfor coarctation stentassessmentRVOT/PAPS, ToF, PA, TGA,truncusECG-gated CT indicatedfor stent assessmentCardiomyopathyHCM, DCM, noncompactionCoronary arterie
48、sAnomalous,ALCAPA, KawasakiUse thin slice 3D SSFPECG-gated coronary CTindicated for identifyingstenosesComplex CHDDORV, DILV,CCTGA, HLHS, BCPC,Fontan,TCPC, EbsteinDelayed CE MRAessential for BCPC(Glenn), Fontan andTCPC circulations. LGEand stress perfusion maybe useful in some4Cardiovascular MRI in
49、Congenital Heart DiseaseTable 3.2 Imaging protocol (standard sequences and views in the order of workow)Sequence Planning1 PurposeIso-centering of the heart in thescannerPlanning subsequent cineimaging planesProvides a map of thoracicanatomy2 Purpose3 Images in all 3 orthogonal planesCoverage from l
50、iver to neckInclude aortic arch & proximal branchesInclude systemic & pulmonary veinsFrom axial stackPlace perpendicular plane through long axis ofventricle, from mid-atrioventricular (AV) valve toventricular apexFrom axial stackPlace perpendicular plane parallel to, & on apicalside of AVCheck that
51、orientation is parallel to the verticalaxis of the AV valves on RVLA & LVLA viewsFrom AV valves viewPlace perpendicular plane across both AV valveoricesFrom LVLA cine check that this plane passesthrough mid-mitral valve and LV apexFrom RVLA check that the plane passes throughmid-tricuspid valve and
52、RV apexFrom end-diastolic frame of 4-chamber cinePlace perpendicular plane at hingepoints of bothAV valves, with special care to include the entirebasal ventricular blood poolFrom VLA views, check that the rst slice isperpendicular to AV valve hingepointsContiguous slices are then placed to cover th
53、e entireventricular mass to the apexProvides the images requiredfor segmentation of ventricularvolumesAssessment of the ventricularseptum, ventricular myocardialmorphology & wall motionabnormalities, outow tractsSubjective assessment of atrialsize, biventricular size &function, ventricular wallmotio
54、n, AV valve regurgitationPlanning short axis (SA) stackPlanning the 4-chamber andLV outow tract (LVOT)imagesPlanning the true 4-chamberimageAssessment of anterior & inferiormyocardium, AV valves,ventricular sizesScoutSingle shot bSSFP imagesAxial stackRespiratory-navigated,ECG-gated, “black-blood”im
55、ages (HASTE or TSE).Contiguous axial slicesVentricularlong-axis(RVLA/LVLA)Breath-held, ECG-gated, bSSFPcine imagesAV valvesBreath-held, ECG-gated, bSSFPcine imageSubjective evaluation of AVvalve function4-Chamber viewBreath-held, ECG-gated, bSSFPcine imageSA stackBreath-held, ECG-gated, bSSFPcine im
56、age3Imaging Protocol5Table 3.2 (continued)SequencePlanningIsotropic voxels (1.11.6 mm). Planned on axialHASTE stack, for coronal-orientated raw data.Include antero-posterior chest wall and lungs.Image acquisition triggered with bolus-trackingto ensure maximum signal in structure ofinterest. Two acqu
57、isitions routinely acquired,with no interval in young children, or a 15 sinterval in older childrenAngiographic views of largeand small thoracic vessels.Images less subject to artifactcaused by low velocity orturbulent ow. The second passacquisition allows assessmentof systemic and pulmonaryvenous a
58、natomyProvides high-resolutionimages of intracardiac anatomy,including coronary arteries.Allows multiplanarreformattingPlanned on axial HASTE stack for sagittalorientation of raw data. Isotropic voxels (1.11.6mm). Respiratory navigator placed mid-rightdome of diaphragm, avoiding cardiac region ofint
59、erest1 Purpose2 PurposeMR angiographyBreath-held, not ECG-gatedGadolinium injection 0.20.4mL/kg Infants: injection rate 2mL/s with 5 mL ush. Olderchildren: injection rate 3 mL/s,10 mL ushSubjective determination ofpreferential blood ow. Can beexpanded to perform time-resolved angiography or4-dimensi
60、onal angiography3D bSSFPFree breathing, respiratorynavigated, ecg-gated. Dataacquisition optimised to occur indiastole. Signal improvedfollowing gadolinium injection& in tachycardic pts bytriggering acquisition everysecond beat. Acquisition time815 minFrom the AV valves cine. Place a perpendicularpl
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