法乐氏四联症的CT诊断.ppt

1、法乐氏四联症的CT诊断 The Diagnostic CT Imaging of tetralogy of Fallot,昆明市延安医院放射科,The Diagnostic CT Imaging of TOF,定义（Definition） 胚胎发生（Embryogeny） 病理生理（Pathophysiology ） 临床表现（Symptoms and Signs） 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：女约1:

2、3。,1888年由法国内科医生Etienne Fallot完整描述改变，1945年行第一例外科手术,定义（Definition） Tetralogy of Fallot refers to a type of congenital heart defect . Consists of 4 features: Ventricular septal defect Narrowing of the pulmonary outflow tract Over-riding of the aorta. Right ventricular hypertrophy,The Diagnostic CT Imag

3、ing of TOF,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,The Diagnostic CT Imaging of TOF,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）,The Diagnostic CT Imaging of TOF,The Diagnostic CT Imaging of TOF,The Diagnostic C

4、T Imaging of TOF,The Diagnostic CT Imaging of TOF,临床表现（clinical feature）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 ),The Diagnostic CT Imaging of TOF,诊断（diagnosis） 胸片（Chest x-ray ） 超声心动图（ECG） DSA 螺旋CT（MSCT

5、）,The Diagnostic CT Imaging of TOF,鉴别诊断,MSCT表现直接征象(direct sign)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,The Diagnostic CT Imaging of TOF,The Diagnostic CT Imaging of TOF,RV,RA,LA,LV,The Diagnostic CT Imaging of TOF,Right Ventricular outflow tract stenosis,The Diagnostic CT Imaging of TOF,

6、The Diagnostic CT Imaging of TOF,The Diagnostic CT Imaging of TOF,Pulmonary Valve Stenosis,The Diagnostic CT Imaging of TOF,Ventricular Septal Defect,The Diagnostic CT Imaging of TOF,Overriding Aorta,The Diagnostic CT Imaging of TOF,The Diagnostic CT Imaging of TOF,Right Ventricular Hypertrophy,The

7、Diagnostic CT Imaging of TOF,The Diagnostic CT Imaging of TOF,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：ASD PDA PFO PLSVC 冠脉起源异常,间接征象（indirect sign）右心房扩大、上、下腔静脉扩张,The Diagnostic CT Imaging of TOF,肺动脉瓣闭锁(pulmonary valve atresia),The Diagnostic CT Imaging of TOF,侧枝循环建立(aorticopulmon

8、ary collateral vessels ),The Diagnostic CT Imaging of TOF,The Diagnostic CT Imaging of TOF,The Diagnostic CT Imaging of TOF,Persistent Left Superior Vena Cava,right aortic arch,The Diagnostic CT Imaging of TOF,ASD,F/14Y 自幼出现活动后青紫，喜蹲踞；胸骨左缘3-4肋间可闻及粗糙收缩期杂音,The Diagnostic CT Imaging of TOF,The Diagnostic CT Imaging of TOF,手术： TOF+PLSVC,The Diagnostic CT Imaging of TOF,Summary： 1、Tetralogy of Fallot is a group of four birth defects in the structure of the heart. 2、 Common sign of tetralogy of Fallot is a heart murmur，the important symptom