CHDs儿童先天性心脏病各论PPT课件

Ventricular Septal Defect（VSD）,Ventricular Septal Defect,The disorder of embryological development of interventricular septum Most common form of CHD in children Accounting for 25,Position of VSD： Membranous(60-70%): the commonest locationtSubpulmonic(3-6%): risk of aortic valve prolapseMuscular(20-30%) : occur anywhere in the muscular part of septum,Anatomic Types,Size of VSD,Small: 10 mm,Hemodynamic Characteristics,Small VSD asymptomatic Pan-systolic murmur of grade heard at left sternal border in the 3rd4th intercostal spaces, radiating over precordium ( 34 LSB SM o),Clinical Manifestations,MediumLarge VSD (symptoms) Pulmonary plethora-Recurrent chest infection Systemic blood flow -Failure to thrive (slow weight gain ） Poor cardiac function: Cyanosis when right-to-left shunt occurs, mostly due to severe pulmonary hypertension,Clinical Manifestations,MediumLarge VSD Poor cardiac function: at infancy：difficulty with feeding, sweating, tachypnea, and hepatomegaly; in older children：dyspnea on excursion, easy fatigability, palpitation, exercise intolerance,Clinical Manifestations,MediumLarge VSD (signs) 24 LSB SM o DM at apex due to large blood flow across normal mitral valve (relative mitral stenosis) P2 increased with split Cyanosis with clubbing in late stage,Clinical Manefestation,Electrocardiogram,Small VSD: ECG usually normal Mediumlarge LV hypertrophy when pulmonary vascular resistance is normal Both LV & RV hypertrophy when pulmonary hypertension occurs due to increased vascular resistance & increased flow RV hypertrophy in Eisenmengers syndrome,Chest X-ray,Small VSD: may be normal Mediumlarge VSD: Increased vascular markings in lungs Heart/chest ratio: 0.55 Enlargement of LV and/or RV Dilated main pulmonary artery segment Smaller aorta in size,Echocardiogram,Display position and size of the defect Display shunting Measure pressure gradient Display size of chambers and vessels: Enlarged LA, LV and/or RV,Echocardiogram,2DE & CDE displays VSD,Prognosis & Complications,Asymptomatic 3050 close spontaneously by 2 years of age Congestive heart failure Pulmonary hypertension Infundibulum stenosis (漏斗部狭窄) Prolapse of aortic valve (主动脉瓣脱垂) Infective endocarditis (感染性心内膜炎),Medical Management,Physical activities properly Prevention and cure of infection timely Follow-up regularly Anti congestive measures: digitalis(洋地黄) diuretics(利尿剂) vasodilators (扩管药物)Transcatheter closure (经导管封堵术）,Indications for Surgical Repair,Congestive heart failure with failure to thrive or recurrent pneumonia Progressive pulmonary hypertension Evidence of infundibulum stenosis Evidence of prolase of aortic valve Supracristal VSD History of infective endocarditis,Atrial Septal Defect（ASD）,Atrial Septal Defect,The disorder of embryological development of interatrial septum Accounting for 10 of CHD,fossa ovalis ASD（75%）,Sinus venosus ASDs （5%）,Ostium primum ASD(15%),Anatomic Types,Coronary sinus ASD（2%）,Hemodynamic Characteristics,Symptoms are similar to VSDs such as poor growth and development, recurrent pneumonia, poor cardiac function but occur less frequently in infants Some patients even remain asymptomatic through life,Clinical Manifestations,Signs： 2,3LSB SM o The murmur is caused by increased flow across pulmonic valves (i.e. relative pulmonary stenosis) 4LSB DM can often be heard (relative tricuspid stenosis) P2 increased with fixed split (固定分裂）,Clinical Manifestations,Electrocardiogram,Axis right deviation, V1, V3R have incomplete right bundle branch block diagram,导联以S为主，导联以R为主,电轴右偏。RaVR0.5mv, RS 1, V1呈RSr, QRS0.08，示不完全右束支转导阻滞。 RV1 SV5 2.5mv，提示右室大。,Chest X-ray,Increased vascular markings in lungs Heart/chest ratio: 0.55 Enlargement of RA, RV Dilated main pulmonary artery segment Smaller aorta in size,Echocardiogram,Display position and size Display shunting Display paradoxic motion (矛盾运动) of ventricular septum Display size of chambers and vessels: Enlarged RA and RV Dilated MPA Smaller AO,Echocardiogram,ostium primum ASD,ostium secundum ASD,Prognosis & Complications,Asymptomatic (often in childhood) Heart failure (occur in middle adulthood) Atrial tachyarrhythmias (adulthood) Pulmonary hypertension (uncommon) Infective endocarditis (rarely occur） Spontaneous closure most frequently if ASD 4mmfrequently if ASD 8 mm mostly closed before age 2 years,Medical Management,No need of physical restriction for most patients Prevention and cure of infection timely Follow-up regularly Anti congestive measures: digitalis diuretics vasodilators Transcatheter closure (经导管封堵术）,Patent Ductus Arteriosus（PDA）,Accounting for 15 of CHD Incidence may be as high as 2060% in preterm infants weighing 0.55 Enlargement of LA, LVDilated main pulmonary artery segment prominent aorta in size,Echocardiogram,ductal shunting,Prognosis & Complications,Asymptomatic Congestive heart failure Pulmonary hypertension Infective endocarditis spontaneous closure of ductal shunt 90% close functionally by 4 days after birth 80% close anatomically in 3 month, and 95% in 1 year of age,Medical Management,Physical activities properly Prevention and cure of infection timely Follow-up regularly Anti congestive measures: digitalis diuretics vasodilators Transcatheter closure (经导管封堵术）,Tetralogy of Fallot（TOF）,Tetralogy of Fallot,Accounting for 10 of CHD,Obstruction to RV outflow：infundibular and/or valvular level with hypoplasia of PA Large VSD Aorta that overrides the VSD Hypertrophy of RV,Anatomic features,Hemodynamic Characteristics,Symptoms：Owing to anoxia Cyanosis (mostly seen from 4 months of age and progressive) Retarded growth and development, easy fatigability and dyspnea on excursion Squatting when walking Hypoxemic spell （缺氧发作）: sudden onset of dyspnea; deepening of cyanosis; irritability or syncope; convulsion；absence of cardiac murmur（a hallmark of severe situation）,Clinical Manifestations,Signs： Cyanosis Clubbing of fingers and toes 3LSB SM 0 , radiating widely P2 decreased S2 usually predominantly aortic and single,Clinical Manifestation,Clubbing杵状指,Laboratory Findings,Reponses to anoxia: Hemoglobin increased Hematocrit elevated RBC count increased blood mucosity increased,Electrocardiogram,Right axis deviation & RV hypertrophy in almost all cases RA expand in some cases,12-year-old boy with TOF,Chest X-ray,Decrease