TOF基础知识及进展.ppt

法乐氏四联症基础知识及进展 INTRODUCTION Tetralogy of Fallot represents of 10% of all congenital cardiac defects. Named after Etienne-Louise Fallot. In 1888, Fallot described the four characteristics of TOF. History 1944年，Blalock-Taussig姑息性手术， 1954年，Lillehei异体交叉循环TOF矫治， 1955年，CPB下TOF根治， 1957年，补片加宽RVOT, 1959年，跨瓣环补片， 1983年，婴幼儿手术成功。 外科解剖 PULMONARY STENOSIS/RVOTO VSD RV HYPERTROPHY OVER RIDING AORTA 外科解剖 Van Praagh“单联症”学说， 75%肺动脉瓣环发育不良， 2/3肺动脉瓣为二叶畸形， 肺动脉发育状况差异大， LPA（3%）或RPA缺如， RVOTO分型 单纯漏斗部：26% 漏斗部+PvS：26% 漏斗部+PvS+PaS：16% 弥漫性管状狭窄 单纯肺动脉瓣膜及瓣环狭窄：5% CAUSES Most often sporadiac /by chance Chromosomal abnormality eg 22q11 deletion, trisomy 18,21 Di George Syndrome和腭心面综合征，与染色体Del22有关，又称CATCH22综合征 CHARGE综合征 VACTERL综合征 Maternal alcohol/Foetal alcohol syndrome Maternal medication eg anti-epileptic drugs Maternal PKU-phenylketonuria EMBYOLOGY During alignment of the muscular septum, the infundibular septum is displaced anteriorly causing a VSD, displacement of aortic valve and RVOTO. HAEMODYNAMICS Blood from RV to PA is obstructed. If severe obstruction, RV pressure LV. Blood shunts R to L. Desaturated blood to aorta. High RV pressure causes RV hypertrophy. CLINICAL PRESENTATION Cyanosis from birth dependent upon amount of RVOTO. Mild stenosis = cyanosis with exertion. Severe stenosis = cyanosis at rest. Murmur( systolic ejection) audible = flow through RVOTO. Old child = small for age, clubbing. Hyper cyanotic spells. Brain abscess INVESTIGATIONS Chest X-ray ECG Blood Tests Cardiac Catheterisation Echocardiogram CT MRI Chest X-Ray Boot shaped heart. Small PA and RVH. Normal heart size due to no CCF. Pulmonary vascular markings. 25% Right sided aortic arch. May be normal in acyanotic TOF. ECG RVH-Right heart leads Ｖ1,Ｖ2,Ｖ3 Height of R wave＞depth of S wave. RAH-peaked P waves. BLOOD TESTS Arterial Blood Gas-SaO2 variable. Cyanosis polycythemia - O2 carrying capacity. Polycythemia, Blood viscosity. Risk cerebral vascular accidents. CATHETERISATION May precipitate hypercyanotic spell. Clari