法乐氏四联症的CT诊断课件.ppt

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1、法乐氏四联症的CT诊断TheDiagnosticCTImagingoftetralogyofFallot昆明市延安医院放射科TheDiagnosticCTImagingofTOF定义（Definition）胚胎发生（Embryogeny）病理生理（Pathophysiology）临床表现（SymptomsandSigns）影像诊断(Diagnosis)定义：包含肺动脉狭窄、室间隔缺损、主动脉骑跨和右心室肥厚四种病理改变为特征的先天性心脏病。其中肺动脉狭窄、室间隔缺损法乐氏四联症的主要畸形基础。是发绀型先天性心脏病中最多的一种。男

2、：女约1:3。1888年由法国内科医生EtienneFallot完整描述改变，1945年行第一例外科手术定义（Definition）TetralogyofFallotreferstoatypeofcongenitalheartdefect.Consistsof4features:VentricularseptaldefectNarrowingofthepulmonaryoutflowtractOver-ridingoftheaorta.RightventricularhypertrophyTheDiagnosticCTImagin

3、gofTOF胚胎发生（Embryogeny）胚胎第5-6周心球部近端吸收消退发生障碍漏斗部间隔向前移位前上移位向右移位右室漏斗部及肺动脉狭窄室间隔缺损主动脉骑跨TheDiagnosticCTImagingofTOF病理生理（Pathophysiology）肺动脉狭窄室间隔缺损主动脉骑跨程度越重右向左分流严重肺血减少、动脉血氧饱和度下降右心室肥厚紫绀、呼吸困难杵状指（趾）TheDiagnosticCTImagingofTOFTheDiagnosticCTImagingofTOFTheDiagnosticCTImagingofTOFT

4、heDiagnosticCTImagingofTOF临床表现（clinicalfeature）体征（signs）胸骨左缘3-4肋间可闻及粗糙收缩期杂音，伴震颤，肺动脉瓣区第二心音减弱症状（Symptoms）发绀（cyanosis）、活动后心慌(palpitation)、气促(dyspnea)、杵状指（趾）(clubbed-finger)，喜蹲踞(squat)TheDiagnosticCTImagingofTOF诊断（diagnosis）胸片（Chestx-ray）超声心动图（ECG）DSA螺旋CT（MSCT）TheDiagnost

5、icCTImagingofTOF鉴别诊断MSCT表现直接征象(directsign)肺动脉狭窄漏斗部狭窄（局限型、弥漫型）肺动脉瓣狭窄（瓣膜增厚）肺动脉主干及其左、右肺动脉发育情况TheDiagnosticCTImagingofTOFTheDiagnosticCTImagingofTOFRVRALALVTheDiagnosticCTImagingofTOFRightVentricularoutflowtractstenosisTheDiagnosticCTImagingofTOFTheDiagnosticCTImagingofTO

6、FTheDiagnosticCTImagingofTOFPulmonaryValveStenosisTheDiagnosticCTImagingofTOFVentricularSeptalDefectTheDiagnosticCTImagingofTOFOverridingAortaTheDiagnosticCTImagingofTOFTheDiagnosticCTImagingofTOFRightVentricularHypertrophyTheDiagnosticCTImagingofTOFTheDiagnosticCTIma

7、gingofTOF间接征象右心房扩大、上、下腔静脉扩张左心室、左心房内径正常或偏小肺动脉瓣闭锁，对比剂不显影侧枝循环建立合并畸形：ASDPDAPFOPLSVC冠脉起源异常间接征象（indirectsign）右心房扩大、上、下腔静脉扩张TheDiagnosticCTImagingofTOF肺动脉瓣闭锁(pulmonaryvalveatresia)TheDiagnosticCTImagingofTOF侧枝循环建立(aorticopulmonarycollateralvessels)TheDiagnosticCTImagingofTOF

8、TheDiagnosticCTImagingofTOFTheDiagnosticCTImagingofTOFPersistentLeftSuperiorVenaCavarightaorticarchTheDiagnosticCTImagingofT