儿科学：Congenital Heart Disease先天性心脏病.ppt

Interventional Treatment Those patients with left to right shunt (VSD, PDA, ASD, et) whose shunts have become partially or totally right to left as a result of the development of pulmonary vascular disease 所有左向右分流的先天性心脏病（ASD、VSD、PDA 等）由于器质性肺血管病变导致梗阻性肺动脉高压，右心系统压力等于或超过左心系统，出现双向或右向左分流 Eisenmenger syndrome 艾森曼格综合征 Tetralogy of Fallot  （TOF） Pathophysiology Obstruction to right ventricular outflow tract (infundibular stenosis) -- the most important malformation (sometimes with pulmonary valve and artery stenosis) Ventricular septal defect large and nonrestrictive VSD just below the aortic valve Override of the aorta (dextroposition of the aorta in 25% patients) Right ventricular hypertrophy Pathophysiology The degree of right ventricular outflow obstruction determines the timing of onset of symptoms, the severity of cyanosis, and the degree of right ventricular hypertrophy The degree of right ventricular outflow obstruction and override of the aorta can become more and more severe with growth   Obstruction to right ventricular outflow tract  Spasm of Stenotic Infundibulum   Hypoxic Spells  RVP↑↑  RVP≥LVP Pulmonary Blood Flow↓↓ Collateral Artery From DAO  RVH Override of the aorta   RV to LV Shunt Gas Exchange in Lung ↓↓ AO Blood from LV and RV