先天性心脏病个论英文稿课件

Common problems(CHD) v Atrial septal defect.ASD v Ventricular septal defectVSD v Patent ductus arteriosus.PDA v Tetralogy of FallotTOF Common problems-1 Atrial septal defect ASD ASD-history Leornardo da Vinci described the patent foramen ovale in the early 1500s “I have found a perforating channel from left auricle to right auricle” In 1875, Karl von Rokitansky provided a superb account of pathological anatomy of the atrial septal defect together with its embryological basis He even distinguished between primum and secundum defects ASD-summary Accounts for 20% of CHD Two to three times more common in women Unoperated survival beyond age 40-50y is no more than 50% with a subsequent increase in mortality at 6% per year after age 50y ASD- classification v Ostium secundum defect Most common (60-70%) Mid portion of IAS Isolated defect associated with MVP v Ostium Primum defect 15-20% of ASDs Inferolateral portion of IAS Frequently associated with cleft in anterior MV leaflet and MR ASD- classification v Sinus Venosus defect 5-10% of ASDs Superior and posterior in relation to fossa ovalis Almost always associated with PAPVD into RA or IVC/SVC v Coronary sinus defect 50 mmHg), and CV symptoms VSD classification 1: membranes 2:outflow 3:inflow 4:muscular IVC, SVC RA RV dialated PA dialated Pulmonary congestion LA overload LV overload Aortic volume decreased Systemic volume decreased VSD Pulmonary blood overload Hemodynamic changes in VSD Hemodynamic changes in VSD Hemodynamic changes in VSD 1. 1. L R shunt Pulmonary circulation L R shunt Pulmonary circulation Systemic circulation Systemic circulation 2. Cardiac volume LA, LV, RV2. Cardiac volume LA, LV, RV 3. Pulmonary Hypertension 3. Pulmonary Hypertension EisenmengerEisenmenger syndrome syndrome VSDVSD Why the left atrium can enlarge with VSD? LVRV LARA VSD clinical presentations Depends on size, PVR, associated lesionsDepends on size, PVR, associated lesions vvSmall-moderate VSDSmall-moderate VSD Grade II-VI harsh Grade II-VI harsh pansystolicpansystolic murmur murmur at lower left at lower left sternalsternal border border Normal P Normal P 2 2 (pulmonic second sound) (pulmonic second sound) vvLarge shunt VSD without PH (pulmonary Hypertension)Large shunt VSD without PH (pulmonary Hypertension) grade III-VI PSM ,P grade III-VI PSM ,P 2 2 is normal, is normal, Congestive heart failure may present Congestive heart failure may present vvIn present of PHIn present of PH P P 2 2 is loud, shot systolic ejection murmur left is loud, shot systolic ejection murmur left sternalsternal border border may be cyanotic if reversal of shunt may be cyanotic if reversal of shunt VSD Laboratory findings In moderate- large VSDIn moderate- large VSD CXR: cardiac enlargement and increased PA flowCXR: cardiac enlargement and increased PA flow ECG: may variableECG: may variable In large defectIn large defect CXR:marked CXR:marked cardiomegaly,andcardiomegaly,and increased pulmonary increased pulmonary VascularityVascularity ECG: RVH, LVH, or bothECG: RVH, LVH, or both Echocardiography: defining position and sizeEchocardiography: defining position and size Doppler echo: estimate the pulmonary systolic pressureDoppler echo: estimate the pulmonary systolic pressure VSDComplications BronchopneumoniaBronchopneumonia Congestive heart failureCongestive heart failure Pulmonary edemaPulmonary edema SubacuteSubacute bacterial endocarditis bacterial endocarditis EisenmengerEisenmenger syndrome syndrome VSDTreatment Small defect may rarely need medical management other Small defect may rarely need medical management other than prophylaxis for SBE than prophylaxis for SBE Significant spontaneous closure : 25%Significant spontaneous closure : 25% Anti congestive medications for CHF Interventional therapy : transvenous closure with an occluding device is now available in some medical centers Surgical management: refractory CHF, failure to thrive,Surgical management: refractory CHF, failure to thrive, repeated pneumonia , reversible PH repeated pneumonia , reversible PH VSD treatment by surgery patch Direct to sow up 返回返回 Orbit wayOrbit way：FVFVIVC RARV VSD IVC RARV VSD LV AAO DAO FA LV AAO DAO FA Common problems-3 Patent Ductus Arteriosus PDA PDA-summary 15% of CHD risks and history that of the shunt magnitude small, restrictive-asymptomatic, risk of endocarditis moderate to large-heart failure, arterial “steal” Hemodynamic changes in PDA RA RV PA congestion PA congestion LV dialated Ao Systemic volume decreased PA dialated Descending ao dialatd Ascending ao dialatd PH Diastolic BP decreased PDA LA dialated Hemodynamic changes in PDA 1. 1. L R shunt Pulmonary circulation L R shunt Pulmonary circulation Systemic circulation Systemic circulation 2. Cardiac volume LA, LV RV2. Cardiac volume LA, LV RV 3. Pulmonary Hypertension 3. Pulmonary Hypertension differential cyanosis differential cyanosis PDAPDA Patent Patent DuctusDuctus ArteriosusArteriosus RA LA RV LV PAAo PDA clinical presentations A symptomatic when the A symptomatic when the ductusductus is mall, when defect is is mall, when defect is large, CHF may developlarge, CHF may develop A grade I-VI continuous (machinery) murmur audible at A grade I-VI continuous (machinery) murmur audible at under left under left sternalsternal border border，ULSB or left ULSB or left infraclaviculainfraclavicula An apical diastolic rumble is audible (large shunt)An apical diastolic rumble is audible (large shunt) Bounding peripheral pulses with wide pulse pressure Bounding peripheral pulses with wide pulse pressure Puls pressure enlargeCapillary pulsation sighn Arterial pistol shot sound Water hammer pulse Perivascular sighn PDA Laboratory findings EKG findings are similar to those of VSDEKG findings are similar to those of VSD CXR: normal in small PDA, CXR: normal in small PDA, CardiomegalyCardiomegaly in in large PDAlarge PDA Echocardiography can directly determine the Echocardiography can directly determine the hemodynamic significancehemodynamic significance Left atrium and ventricular hypertrophy 肺多血，肺动脉段 PDAEchocardiography PDAComplications BronchopneumoniaBronchopneumonia Congestive heart failureCongestive heart failure Pulmonary edemaPulmonary edema SubacuteSubacute bacterial endocarditis bacterial endocarditis EisenmengerEisenmenger syndrome syndrome Large shunt PDA: similar to VSD PDATreatment IndomethacinIndomethacin is effective in infant within one is effective in infant within one week of ageweek of age Prophylaxis should be used when SBE Prophylaxis should be used when SBE arisedarised Interventional therapy : Catheter closure of Catheter closure of PDA with various devices PDA with various devices Surgical ligation and division without Surgical ligation and division without cardiopulmonary bypass is indicated for all cardiopulmonary bypass is indicated for all PDAsPDAs, regardless of size, regardless of size 蘑菇伞堵塞术（图示） PDA 术后造影无分流 Common problems-4 Tetralogy of Fallot TOF TOF-summary R-to-L shunts: CYANOSIS in 1year after birth (or diagnosis if Tetralogy of Fallot), prevalence 10% of CHD Basic abnormality is malalignment of the outflow (infundibular) portion of the ventricular septum Pathoanatomy: VSD aortic over ride pulmonary outflow tract stenosis right ventricular hypertrophy TOF- Pathology Infundibular deviated to anterior, superior and left 1. 50 RVOTO 2. 20%50 PS 3. Superior pulmonary valve stenosis or peripheral pulmonary stenosis or absent left pulmonary artery 4. Asssociated heart malformation 40inner heart malformation 2030out heart malformation 20% right sided aortic arch Hemodynamic changes in TOF 1. 1. R R L L shunt shunt 2. RV volume , LV relatively small2. RV volume , LV relatively small 3. PA flow 3. PA flow hypoximiahypoximia VSDVSD VSDVSD LV AO overridingoverriding cyanosis RV PA blood flow decreasedPA blood flow decreased TOF clinical presentations Cyanosis Dyspnea Squatting position Anoxia spells Severe degree of the presentations is related to the degree of right ventricular outflow obstruction. Cyanosis Squatting 蹲踞 Clubbing Clubbing Thrombosis Thrombosis TOF clinical presentations vpresentation typically in infancy -murmur-present from initial assessment -cyanosis if outflow obstruction is severe -range of presentation a reflection of the variability of outflow obstruction -most critical obstructive lesion-pulmonary atresia -may be associated with hypoplasia of the pulmonary arteries TOF clinical presentations v Hypoxic spell: Occur at 2m9m -A paroxysm of hyperpnea (rapid and deep respiration) -increasing cyanosis -decreased intensity of the heart murmur TOF Laboratory findings ECG show RA dilated and RV hypertrophyECG show RA dilated and RV hypertrophy CXR: CXR: Normal heart size, decreased Normal heart size, decreased PVMsPVMs, boot-, boot- shape heart with a concave MPA segment,right shape heart with a concave MPA segment,right aortic arch is presentaortic arch is present Echocardiography can directly determine the Echocardiography can directly determine the hemodynamic significancehemodynamic significance Cardiac catheterization BootBo