1、龐玉生龐玉生Chapter 13 循環系統疾病Circulation system disease 教學目的 初步熟悉、了解先天性心臟病 掌握先天性心臟病的診斷和治療 教學重點 詳細介紹先天性心臟病的臨床表現和診斷方法 教學難點 先天性心臟病血液動力學改變與臨床表現的關系Contents小兒心血管系統解剖生理特點及檢查方法小兒先天性心臟病概述房間隔缺損室間隔缺損動脈導管未閉法洛四聯癥第一節 小兒心血管系統解剖生理 特點及檢查方法一 心臟的胚胎發育Cardiac development during fetal period The primitive heart tube forms by 3

2、 W Cardiac looping by 2224 days Bend ventrally and toward the right Cardiac septation第一隔第一隔原發孔原發孔心內膜墊心內膜墊靜脈竇瓣靜脈竇瓣膜膜第二隔第二隔繼發孔繼發孔第一隔第一隔室間孔室間孔第二隔第二隔繼發孔繼發孔第一隔第一隔卵園孔卵園孔第一隔第一隔室間孔室間孔第二隔第二隔靜脈竇瓣靜脈竇瓣膜膜胚胎房室分隔及房室間隔發育胚胎房室分隔及房室間隔發育室間隔形成室間隔形成 心室間隔組成三個來源心室間隔組成三個來源 肌隔肌隔 心內膜墊向下生長與肌隔相合心內膜墊向下生長與肌隔相合 動脈總干及心球分化成主動脈動脈總干及心

3、球分化成主動脈 與肺動脈時的中隔向下延伸部分與肺動脈時的中隔向下延伸部分室間隔膜部Aortic arch development Right and left truncoconal ridges The aortic and pulmonary outflow tracts are fully separated The coronary sinus The semilunar valves主肺動脈由總干呈螺旋形分隔主肺動脈由總干呈螺旋形分隔AOPAPAAOPAPAAOAOPAPAPAPA大動脈位置變移系列示意圖大動脈位置變移系列示意圖TOFTGADORVTaussig-Bing 房室管、心

4、房、房室管、心房、 及心室的分隔及心室的分隔室間隔膜部的形成室間隔膜部的形成及室間隔的封閉及室間隔的封閉u第第2周原始心臟形成周原始心臟形成u第第4周心臟起循環作用周心臟起循環作用u第第8周四腔心臟形成周四腔心臟形成正常血液循環途徑二 胎兒新生兒循環轉換The placenta: gas exchange, metabolic exchange The lung: constrictedThree cardiovascular structure: Ductus venosus Foramen ovale Ductus arteriosus The Fetal Circulation胎兒血循環

5、特點營養和氣體代謝通過臍血管和胎盤與母體進行彌漫式交換絕大部分為混合血供應心臟、腦、肝及上肢血氧含量遠較下半身高肺處于壓縮狀態無氣體交換卵圓孔、動脈導管和靜脈導管開放右心室優勢The Fetal Circulation胎兒血液循環途徑胎兒血液循環途徑通過動脈導管通過動脈導管通過卵圓孔通過卵圓孔血氧含量較高血氧含量較高血氧含量較低血氧含量較低通過靜脈導管通過靜脈導管下腔靜脈下腔靜脈右心房右心房右心室右心室左心房左心房左心室左心室升主動脈升主動脈心腦及上肢心腦及上肢肺動脈肺動脈降主動脈降主動脈下半身下半身上半身靜脈血上半身靜脈血上腔靜脈上腔靜脈臍靜脈動脈血臍靜脈動脈血門靜脈靜脈血門靜脈靜脈血母體母

6、體下半身靜脈血下半身靜脈血肺循環肺循環50%1/3Circulation Changes after birth The umbilical cord clamped breathing commences Pulmonary circulation Providing gas exchange The foramen ovale and ductus arteriosus: functionally closed胎兒與出生后血液循環比較A胎兒期B出生后由母體循環完成氣體交換由母體循環完成氣體交換由肺循環完成氣體交換由肺循環完成氣體交換多為混合血，心、腦、上多為混合血，心、腦、上半身血氧含量高

7、于下半身半身血氧含量高于下半身靜脈血和動脈血分開靜脈血和動脈血分開卵圓孔、動脈導管、靜脈卵圓孔、動脈導管、靜脈導管開放導管開放卵圓孔、動脈導管、靜卵圓孔、動脈導管、靜脈導管閉合脈導管閉合肺動脈壓與主動脈相似，肺動脈壓與主動脈相似，肺循環阻力高肺循環阻力高肺動脈壓下降，肺循環肺動脈壓下降，肺循環阻力低阻力低右心室高負荷右心室高負荷左心室高負荷左心室高負荷ABCommon symtoms Congestive heart failure Feeding difficulties / Exercise intolerance Respiratory distress Poor growth Crac

8、ked, cough Squatting Syncope Cyanosis三 兒童心血管疾病體格檢查方法Physical examination Commonly manifestations Evaluation for BW and BH Respiratory distress Cyanosis Clubbing digits Hepatomegaly Distended neck veins Sign of liver -jugular vein Peripheral edemaCardiovascular ExaminationInspection: Prominence of th

9、e precordium Cardiomegaly Palpation: heave, thrill Percussion: size and position of the heartAuscultation: HR, rhythm (1) Normal heart sounds: S1, S2, S3, S4 (2) Abnormal heart sounds: P2 fixed splitting (3) Murmurs 小兒聽診常用聽診區二尖瓣聽診區肺動脈瓣聽診區主動脈瓣聽診區三尖瓣聽診區 (1) Location and radiation (2) Timing: Systolic,

10、 diastolic and continuous (3) Quality: Harsh, musical, or rough; high, medium or low in pitch (4) Intensity: grade , , , , , (5) Variation with positionMurmur 周圍血管征周圍血管征四肢脈搏及血壓 主動脈縮窄： 股動脈搏動減弱或消失 下肢血壓低于上肢 動脈導管未閉或主動脈關閉不全： 脈壓增寬 毛細血管搏動 股動脈槍擊音四 心臟的特殊檢查 (1) Chest x-ray (2) Electrocardiogram (3) Echocardio

11、gram (4) Cardiac catheterization (5) Angiocardiography (6) Radionuclide angiocardiography (7) Magnetic resonance imaging（8）Computed tomographyChest Radiographl character of the pulmonary vasculaturel Position of the cardiac apexl cardiac size (cardiothoracic ratio)l cardiac configuration, heart shap

12、el Position of the abdominal visceraNormal cardiothoracic imaging landmarks, PA view Anterior radiographic view of cardiac anatomy X-ray你的你的X線診線診斷分別是什斷分別是什么？么？ The sequence of ECG: rate, rhythm, P wave, PR interval, QRS complex ( axis, amplitude, and duration), QT interval, ST segment, and T wave. A

13、ssess atrioventricular hypertrophy Diagnosis of dysrhythmiasElectrocardiogram, ECGNormal ECG A powerful noninvasive method: M-mode, two and three dimensional, color and Doppler Evaluate cardiac structure Estimate intracardiac pressure and gradient across stenotic valves and vessels Quantitate cardia

14、c contractile function Determine the direction of flow across a defect Examine the integrity of the coronary arteries Evaluate endocarditis, pericardiac fluid, cardiac tumors, or chamber thrombi.EchocardiographyM-mode ECHOLong-axis view for LV2-D ECHOFour-chamber view at apex2-D ECHOShort-axis view

15、at the great artery2-D color ECHO (four-chamber view)2-D color Doppler ECHO 3-D ReconstructionSVCIVCRVCardiac Catheterization Estimate Cardiac output according to Oxygen content and saturation, pulmonary and systemic blood flow. Determine the pressures in all chambers and vessels Calculate the pulmo

16、nary and systemic vascular resistance Interventional catheterization (therapeutic procedures) 20%20%1214%1214%30/30/1212121214%14%1214%1214%4 84 80 50 5100/10100/1030/530/5100/60100/60Angiocardiography 選擇性造影 Selective angiocardiography 數字減影造影(DSA) 診斷復雜先心病的主要手段 常用成角造影 長軸斜位：左前斜6075向頭成角2030 肝鎖位：左前斜4045

17、向頭成角40 坐觀位：正位向頭成角40 心血管造影心血管造影心血管造影Radionuclide angiocardiography 99m锝 iv 釋放射線 初次循環心臟造影、平衡心臟血池造影 檢測左向右分流 評估心功能Magnetic resonance imaging Noninvasive tool Follow-up Evaluate the heart and the great arteries Provide the imaging of vascular structures of the thorax Paul C. Lauterbur, United States, and

18、 Sir Peter Mansfield, Britain, wined the 2003 Nobel Prize for discoveries in magnetic resonance imagingSEAOPAAOLVLARVCine MRICine-MRIMRA3-D MRIMRI is in valuable in the long term follow-up of coarctation of the aorta after angioplasty. (surgical procedure) Computed tomography Follow-up Evaluate the

19、heart and the great arteries Provide the imaging of vascular structures of the thorax第二節 小兒先天性心臟病概述概念：CHD是胎兒期心臟及大血管發育異常而致的心血管畸形，是小兒最常見的心臟病發病率:68，上海6.87我國每年出生15萬CHD診治研究取得很大進展，預后大為改觀 病 因 內因：與遺傳有關，染色體異常或基因突變。 外因：（1 1）早期宮內感染：風疹、流感、腮腺炎、柯薩奇病毒等。（2 2）孕母接觸大量放射線服用藥物史（抗癌藥、抗癲癇藥) )（3 3）代謝性疾病: :糖尿病、高鈣血癥（4 4）宮內慢性缺

20、氧（5 5）妊娠早期酗酒、吸食毒品環境因素遺傳因素Categories of congenital heart diseaseThe left-to-right shunt type (ASD VSD PDA) Acyanotic early Pulmonary blood increased Systemic blood decreased Pulmonary hypertension Eisenmenger syndrome: cyanotic lately due to right-to-left shuntsThe right-to-left shunt type Tetralogy

21、of Fallot ( TOF), transposition of the great arteries (TGA) Cyanotic early Cyanotic lesions with increased pulmonary blood flow Cyanotic lesions with decreased pulmonary blood flow The systemic circulation is hypoxemia No-shunt lesions Pulmonary stenosis (PS), Coarctation of the aorta (Coa) Acyanoti

22、c Hypertrophy resulting in increased pressure loadVan Praagh 順序分段診斷方法 心房位置判斷 心室位置判斷 大血管位置判斷 房室連接診斷 心室大動脈連接診斷 心臟位置“三層樓結構”“四層樓結構”Diagnostic process for CHD Two groups based on PE acyanotic and cyanotic lesions Further be subdivided based on X-ray increased, normal or decreased pulmonary vascular marki

23、ngs Determine whether right, left, or biventricular hypertrophy Confirm the diagnosis by ECHO or Catheterization, or both 第三節 幾種常見的先天性心臟病Common congenital heart diseasesAtrial Septal defect （ASD)ASD General considerations Definition: an opening in the atrial septum permitting the shunting of blood b

24、etween the atria Incidence: 10% in all CHD Three major types: (1) The ostium secundum type (2) The sinus venosus type (3) The ostium primum type ASD PathophysiologyThe degree of L-R shunt(1) The size of the defect (2) The relative compliances of RV and LV(3) the relative vascular resistances in the

25、pulmonary and systemic circulationASD Pathophysiology In neonate and early infant limiting the L R shunt Small ASD Large ASD Qp:Qs 2 4:1 PAH：R L shunt可在臨床上表現出來可在臨床上表現出來ASD分流分流右心室擴大右心室擴大肺動脈充血肺動脈充血肺循環充血肺循環充血肺動脈高壓肺動脈高壓肺靜脈動脈血肺靜脈動脈血左心房左心房左心室左心室主動脈搏血減少主動脈搏血減少體循環缺血體循環缺血上下腔靜脈上下腔靜脈右心房擴大右心房擴大ASD血液循環途徑血液循環途徑LV

26、RVLARAWhy the LA is not enlarged ？ Small ASD： Most often asymptomatic discovered during PE Large ASD（large shunt）： (1)Pulmonry blood ：repeated URI or pneumonia (2)Systemic blood ：poor growth, pale, lack of power, hidrosis, tachypnea after exercise (3)Pulmonary hypertension, Eisenmengger syndrome（cya

27、nosis） ASD Clinical manifestationASD Cardiac sign Inspection: left precordial bulge and increased cardiac activity Palpation: systolic lift, seldom with thrill Percussion: cardiomegaly Auscultation: loud S1 2,3 LSB systolic ejection murmur, grade fixed splitting of the 2nd heart sound Lower LSB dias

28、tolic murmur Early systolic click , P2 accentuated (PAH)P2P2亢進伴固定分裂，喀喇音亢進伴固定分裂，喀喇音ASD -ASD -/ SM/ SMASD ECG Volume overload of the RV: QRS axis: normal or right axis deviation with a clockwise loop in the frontal plane RV hypertrophy A minor right ventricular conduction delay P-R delay In the right precordial leads, a rsR patternASD ECGI AVBASD X-rayYThe pulmonary