先心病 (73p).ppt

1、Congenital Heart Disease (CHD),Shenzhou Hospital Paediatric Department He Lihui,Overview,Congenital heart defects are abnormalities in the hearts structure that are present at birth. Approximately 8 out of every 1,000 newborns have congenital heart defects, ranging from mild to severe.,Etiology,1.Ge

2、netic factor (internalfactor): Geneticand chromosomal aberrations,2. Environmental factor (external factor): High altitude,4. Inherited factor,3. Other related factors: Viral infections of pregnancy , Mothers who are diabetic, alcoholics or drug addictive Drugs and metabolic factors,Prevention,The h

3、ealth protection of pregnant woman should be enhanced. High risk factors, such as drugs, radiation, viral infection, et.should be avoided. Suit dosage Folic Acid should be filled up in early pregnancy stage.,Evaluating a child with a heart murmur,Does the child have heart disease? Is it congenital h

4、eart disease? If it is congenital heart disease, what is the lesion? What is the severity of the lesion?,Assessment of a child for the presence of heart disease,Major Systolic murmur garde III or more specially with a thrill Diastolic murmur Cyanosis Cingestive heart failure,Minor Systolic murmur le

5、ss than grade III in intensity Abnormal S2 Abnormal ECG Abnormal X-ray Abnormal BP,Manifestation of shunts,Atrial Septal Defect （ASD）,Definition: ASD is an abnormal communication between the two atria. Classification: Ostium secundum type Ostium primum type Endocardial cushion type,ASD,Accounts abou

6、t 5%10% of all CHD cases. The incidence is estimated to be 1 per 1500 live births. Is the most common CHD in adult. Male : Female 1: 2,PV,LA,PA,LV,PV,SVC,RA,AO,IVC,RV,Hemodynamics Figure of ASD,ASD murmur,Pathophysiology of ASD,LR shunt determined by ： Size of the ASD lesion Pressure difference betw

7、een two atria RV diastolic accommodation,Hemodymamics of ASD,Obstructive PAH,Eisenmangers syndrome,Pre Obstructive PA Hypertension,SVC IVC Pulmonary Vein Shunting Hypertrophy RA LA Hypertrophy RV LV （Volume of blood） ( Ejection of Blood ） Pul. Artery（Dilated） Aorta（Blood） Pul. Circulation ( Congesti

8、ve） Sys. Circulation（Insufficiency） Frequent Chest Infection Failure To Thrive Congestive CHF,Post Obstructive PA Hypertension,SVC IVC Pul. Vein Shunting Hypertrophy RA LA Hypertrophy RV LV（Mixed Blood） Pul. Artery（Dilated） Hyperkinetic PA Hypertension Sys. Circulation (Mixed Blood） Obstructive PA H

9、ypertension Cyanosis （Eisenmangers Syndrome）,Symptoms Generally asymptomatic Pulmonary plethoric: frequent chest infections Systemic Circulation Insufficiency：Failure to thrive、poor weight gain、feeding difficulty 、fatigue、 shortness of breathe、sweating Cyanosis：Severe cyanosis in large lesions，softe

10、r heart murmur and accentuated P2. 扩张的动脉压迫喉返神经：声音嘶哑,Clinical findings of ASD,Sign of ASD,sound: S1 accentuated - loud S2 widely split and fixed( Volume in RV -Prolonged ejection phase-Pul.Valve closes late） P2 accentuated Murmurs Shunt Murmur: Absent Flow Murmurs: (a) A grade -/ ejection systolic mu

11、rmur is heard best at LSB2-3 which widely transmitted all over the chest. No thrill. (relative Pul. Valve stenosis) (b) Delayed Diastolic Murmur at LLSB ( relative Tricuspid sterno）,Complication of ASD,Bronchopneumonia Congestive heart failure Infective endocarditis,ECG of ASD,Right Ventricle Hypert

12、rophy（RVH) Right Axis Deviation Incompleted right bundle branch block (IRBBB)（I0）,X-ray findings,Plethoric Lung fields RA and RV enlargement Prominent PA segment Normal or small aortic shadow,USG findings,RA , RV enlargement RV overloaded Parallel shunt between atria in Doppler,Catheterization 1、SaO

13、2 in RAin Vena Cava 2、Pressure of RV and PA is mormal or mildly 3、Catheter passing through the lesion can enter RV from RA.,Prognosis and treatment of ASD,Prognosis: Lesions diameter8mm rarely close without any intervention. Large shunt（Qp/Qs1.5）needs operation. Invasive cardiac catheterization：Ampl

14、azer、cardia seal ect. device to seal the lesion,Ventricular Septal Defect （VSD）,VSD accounts for 50% of all congenital heart diseases in our country. It is the most common CHD in pediatrics.,Hemodynamics Figure of VSD,PV,SVC,RA,PA,IVC,RV,PV,LA,AO,LV,VSD murmur,Classification of VSD,Acooding to the s

15、ize of lesions：,VSD Pathology,Shunts from L to R related with ： Size of VSD lesion Pressure difference between LV and RV Resistance of pulmonay and systemic circulation Appearance of Eisenmengers syndrome,Hemodynamics of VSD,Pre Obstructive Pul. Arterial Hypertension(Left side hypertrophy）,SVC, IVC

16、Pul. Vein RA LA Hypertrophy Shunt Hypertrophy RV LV Hypertrophy （Volume of blood） （ Ejection） Pul. Artery（Dilated） Aorta（Volume of blood） Pul. Circulation（Congestive） Systemic Circulation （Insufficiency） Frequent Chest Infection Failure To Thrive Congestive Heart Failure,Post Obstructive Pul. Arteri

17、al Hypertension(Right side hypertrophy）,SVC, IVC Pul. Vein RA LA Shunt Hypertrophy RV LV Pul. Artery （Dilated） Hyperkinetic Pul. Arterial Hypertension Systemic Circulatio （Mixed Blood） Obstructive Pul. Arterial Hypertension Cyanosis （Eisenmangers Syndrom）,Eisenmengers syndrome,Occurs in late stage o

18、f L to R shunted heart diseases. Pathogenesis Irreversible pulmonay artrial hypertension occurs Pressure of RV is greater than that of the LV causing R to L shunts or bi-direction shunts Manifestation：Persistant Cyanosis Contraindicator of operation If the lesion is corrected，RL shunt stops, congest

19、ive heart failure occurs.,Eisenmengers syndrome：,is defined as the process in which a left-to-right shunt caused by a atrial septal defects, ventricular septal defects, patent ductus arteriosus, and more complex types of acyanotic heart disease causes increased flow through the pulmonary vasculature

20、, causing pulmonary hypertension, which in turn, causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt.,Manifestation of VSD,Symptoms Asymptomatic in mild cases Pulmonary plethoric: frequent chest infections Systemic Circulation Insufficiency：

21、Failure to thrive、poor weight gain、feeding difficulty 、fatigue、shortness of breathe、sweating Cyanosis：Severe cyanosis in large lesions，softer heart murmur and accentuated P2. Hoarseness: Dilated pulumonary artery presses recurrent laryngeal nerver,Auscultation Sounds: S1: Marsked by murmur S2: Maske

22、d by murmur S3: With small LR shunts P2: Accentuated when Pulmonary Artery Hypertension presence Murmurs: Shunt Murmur: A or grade pansystolic murmur may be in the third or fourth interspace of left sternal border with a wild transmission and thrill on palpation. (Etiology：blood shunting through the

23、 lesion） Flow Murmurs: (a) Pulmonary: Ejection systolic murmur( Drowned ) (b) Mitral delayed diastolic murmur (Etiology：Large volume of blood passes through a normal mitral valve）,symptoms,Fast breathing Sweating Pallor Very fast heartbeats Decreased feeding Poor weight gain Fatigue in older childre

24、n.,Male，3yrs. VSD, PA hypertension, physical growing delayed.,Complications,L to R shuntBlood in Pul. Circulation, plethoric lung fields，fliud in lung tissue frequent Chest Infection Large LR Shunt，Right side overloaded Congestive heart failure, hydropneumonia. Heart abnormalitychanged blood stream

25、impact endocardium pathogen breeding easilyInfective endocarditis,VSD ECG findings,Enlargement of LV or both ventricles, may be enlargement of LA,X-ray findings,LV and RV enlargement with Left ventricular type Pulmonary vasculature is increased. Prominent PA segment Normal or smaller aorta,ECHO,2D e

26、chocardiagram show defect on ventricular septum. Paralelle shunt between ventricles in Doppler. Estimates difference in pressure between ventricles, PA pressure, and Qp ,PV,SVC,RA,PA,IVC,RV,PDA,PV,LA,AO,LV,Hemodynamics Figure of PDA,PDA murmur,Pathophysiology of PDA,L to R shunt related to： Size of

27、the ductus Pressure gradient between Aorta and PA Differential cyanosis: when a R to L shunt has appeared because of Pul. Arterial Hypertension. Sonce the R to L shunt through the PDA flows down the descending aorta , cyanosis is present in toes but not in fingers. It is characteristic of PDA with P

28、ul. Arterial hypertension and R to L shunt.,Blood in RV Systemic circulation Diostolic pressure Pul. Artery Aorta insufficiency Wild pulse pressure Volume of blood Pul. Arterial Eisenmangers in Pul. circulation Hypertension Syndrome （differential cyanosis) （RV hypertrophy） Enlargement of RA and RV,P

29、DA,Hemodynamics of PDA,Clinical findings of PDA, Symptoms: Similar with those of VSD Signs: Peripheral vascular sigh 水冲脉、指甲床毛细血管搏动 Differential cyanosis and clubbing,Auscultation,Sounds S1Accentuated, loud M1 S2Splitting Murmurs Shunt murmur: Rough and loud machinary countinuous murmur at LSB2（ star

30、ts insystole, afterthe first sound, and reaches a peak at the second sound, then diminished in intensity and is audible during only a part of the diastole. ） Flow murmur: Mitral delayed diastolic murmur at apex.(a large volume of blood in LR across the mitral and delay the closure of the mitral),Com

31、plication of PDA,Bronchopneumonia Infective endocarditis Congestive heart failure (First six weeks of life),X-ray finding in PDA,Cardiac enlargement with a LV silhouette May be LA enlargement Porminent ascending aorta and aorta knuckle Plethoric lung field Prominent PA segment,USG of PDA,2D echocard

32、iogram can idendtify the PDA which can be confirmed by Doppler.,Treatment of PDA,Medical intervention In first two weeks, Indomethacin, a prostaglandin synthetase inhibitor, can be given orally to close the ductus. It is effective for 90% preterm PDA cases. Surgical intervention Patients can be sent

33、 up for operation as soon as the diagnosis is made. Cardiac catheterization,Common features of L to R shunted CHD,Generally no cyanosis. Appear cyanosis only at crying ,having pnemonia and cardiac failure when the pressure of RH exceeds that of LH. Pulmonary vasculature becomes plethoric causing fre

34、qent chest infection。 Systemic circulation insufficiency delays growing。 Rough systolic murmur at precordium, best heard at left sternal border.,Tetralogy of Fallot （TOF）,The most common cyanotic CHD in living infants. Account for about 10% all CHD cases. R to L shunted CHD,PV,SVC,RA,PA,IVC,RV,PV,LA

35、,AO,LV,Hemodynamics Figure of TOF,TOF murmur,The four malformations of TOF,Obstruction to right ventricular outflow Ventricular septal defect Overriding or dextroposed aorta Right ventricular hypertrophy,RV（hypertrophy） LV Pulmonic stenosis Aorta （blood flow , dilated) Pulmonary circulation Deoxygen

36、ated blood (clear lung field) enters systematic circulation （cyanotic、growth delay、 Low saO2 （clubbing） squatting、anoxic spell）,bypass,bypass,Hemodynamics of TOF,TOF临床表现,Symptoms 1、Cyanosis：central，worsen when exercise or fast breathing. 2、Squatting：seen older children Squatting: Lower extremities a

37、rteries pressed ,resistence of systemic circulation，R to L shunt Flexion of lower extremities，blood in venous system reture，heart load 3、Clubbing fingers(toes) 4、Anoxic spell 5、Delayed growing( Failure to thrive) 6、Dyspea,anoxic spell,Occurs predominantly after waking up or following exertion(feedin

38、g,crying,), anemia and infection. Pathogenesis：PA infundibulum inconstant convulsion and obstruction cause brain anoxia. Manifestation: Paroxysmal dyspnea、fainting、convulsion even death. Treatment：Knee-chest position Humidified oxygen Propranolol 0.1mg/kg IV Correct acidosis:5%Sodium bicarbonate 1.55.0ml/kg IV orphine 0.10.2mg/kg Subcutaneous injection Prevention：Propranolol 13mg/kg/d Po.,Clubbing of fingers,Clubbing of fingers in a patient with Eisenmengers syndrome; first described by Hippocrates, clubbing is also,Clubbing of fingers