法乐氏四联症的CT诊断.ppt

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昆明市延安医院放射科The Diagnostic CT Imaging of TOFThe 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)n n定义：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1:3。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n n 定义定义（Definition）Tetralogy of Fallot refers to a type of congenital heart Tetralogy of Fallot refers to a type of congenital heart defect.defect.Consists of 4 features:Consists of 4 features:Ventricular septal defect Ventricular septal defect Narrowing of the pulmonary outflow tract Narrowing of the pulmonary outflow tract Over-riding of the aorta.Over-riding of the aorta.Right ventricular hypertrophy Right ventricular hypertrophyThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFn n胚胎发生（胚胎发生（Embryogeny）胚胚胎胎第第5-6周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 TOFThe Diagnostic CT Imaging of TOFn n病理生理（病理生理（Pathophysiology）肺动脉狭窄肺动脉狭窄室间隔缺损室间隔缺损主动脉骑跨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 TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFn n临床表现（临床表现（clinical feature）体征（体征（signssigns）胸骨左缘胸骨左缘3-43-4肋间可闻及粗糙收缩期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杂音，伴震颤，肺动脉瓣区第二心音减弱 症状（症状（Symptoms Symptoms）发绀（发绀（cyanosis）、活动后心慌、活动后心慌(palpitation)、气促气促(dyspnea)(dyspnea)、杵状指（趾）、杵状指（趾）(clubbed-finger)(clubbed-finger)，喜，喜蹲踞蹲踞(squat)The Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFn n诊断（诊断（diagnosis）胸片（Chest x-ray）超声心动图（ECG）DSA 螺旋CT（MSCT）The Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOF鉴别诊断鉴别诊断n nMSCT表现表现直接征象直接征象(direct sign)(direct sign)肺动脉狭窄肺动脉狭窄 漏斗部狭窄（局限型、弥漫型）漏斗部狭窄（局限型、弥漫型）肺动脉瓣狭窄（瓣膜增厚）肺动脉瓣狭窄（瓣膜增厚）肺动脉主干及其左、右肺动脉发育情况肺动脉主干及其左、右肺动脉发育情况 The Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFRVRALALVThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFRight Ventricular outflow Right Ventricular outflow tract stenosistract stenosisThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFPulmonary Valve StenosisPulmonary Valve StenosisThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFVentricular Septal DefectVentricular Septal DefectThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFOverriding AortaOverriding AortaThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFRight Ventricular HypertrophyRight Ventricular HypertrophyThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFn n间接征象 右心房扩大、上、下腔静脉扩张右心房扩大、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，对比剂不显影肺动脉瓣闭锁，对比剂不显影 侧枝循环建立侧枝循环建立 合并畸形：合并畸形：ASD PDA PFO PLSVCASD PDA PFO PLSVC 冠脉起源异常冠脉起源异常n n间接征象（间接征象（indirect signindirect sign）右心房扩大、上、下腔静脉扩张右心房扩大、上、下腔静脉扩张The Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFn n肺动脉瓣闭锁(pulmonary valve atresia)The Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOF 侧枝循环建立(aorticopulmonary collateral vessels)The Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFPersistent Left Superior Vena Cava right aortic arch The Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFASDF/14Y 自幼出现活动后青紫，喜蹲踞；胸骨左缘胸骨左缘3-43-4肋间可闻及粗糙收缩期杂音肋间可闻及粗糙收缩期杂音The Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOF手术：TOF+PLSVCThe Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOFSummary：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 is cyanosis.3、In diagnosis,MSCT can provide enough information,including associated anomalies.The Diagnostic CT Imaging of TOFThe Diagnostic CT Imaging of TOF.谢谢！