動(dòng)脈導(dǎo)管未閉

1、先天性心臟病 Congenital Heart Disease (CHD,陳小榮,包頭醫(yī)學(xué)院第二附屬醫(yī)院兒科,第十三章,概述 Overview,CHD is defined as an abnormality in circulatory structure or function that is present at birth, even if it is discovered much later,CHD is present in 0.687% in China,先天性心臟病是胎兒時(shí)期心臟血管發(fā)育異常而導(dǎo)致的先天性畸形,出生后1年內(nèi)先天性心血管發(fā)育畸形出現(xiàn)率占活產(chǎn)嬰兒的6.87,By

2、the progression of all kinds of inspection techniques and interventional therapy, the prognosis of CHD has taken on a new look,概述 Overview,由于各種心血管檢查技術(shù)（特別是彩色多普勒超聲心動(dòng)圖）的應(yīng)用，深低溫麻醉和體外循環(huán)下心內(nèi)直視手術(shù)的發(fā)展，先心病介入性治療進(jìn)展，臨床上對(duì)先心病的診斷和治療發(fā)生了很大變化 ，預(yù)后大為改觀,目的要求 Objective and Request,掌握室間隔缺損、房間隔缺損、動(dòng)脈導(dǎo)管未閉、法洛四聯(lián)癥的血流動(dòng)力學(xué)、臨床表現(xiàn)及常見并發(fā)癥

3、 To master the hemodynamics and clinical situation and the diagnosis of common complications in VSD, ASD, PDA and TOF,熟悉先天性心臟病的病因、分類、預(yù)防及治療原則 Familiar with the etiology and classification of CHD,病因 Etiology,遺傳因素(內(nèi)在因素) 與基因突變、染色體畸變有關(guān) Genetic factor (internal factor) CHD result from gene mutation or chr

4、omosome aberration,環(huán)境因素(外界因素) 與宮內(nèi)病毒感染有關(guān) Environmental factor (external factor) CHD is mainly correlation to the intrauterine infection,預(yù)防 Prevention,加強(qiáng)孕婦保健 The health protection of pregnant woman should be enhanced,避免接觸藥物、輻射等高危因素High risk factors should be avoided,妊娠早期適量補(bǔ)充葉酸 Suit dosage Folic Acid s

5、hould be filled up in early pregnancy stage,分類Classification,左向右分流型(潛在青紫型) left-to-right shunts (noncyanosis,右向左分流型(青紫型) right-to-left shunts (cyanosis,無分流型(無青紫型) non shunts (noncyanosis,PS,VSD,TOF,臨床常見的先心病 Common CHD in Clinic,室間隔缺損 Ventricular septal defect (VSD,房間隔缺損 Atrial septal defect (ASD,動(dòng)脈導(dǎo)

6、管未閉 Patent ductus arteriosus (PDA,法洛四聯(lián)癥 Tetralogy of Fallot (TOF,室間隔缺損 Ventricular Septal Defect （VSD,室間隔缺損 Ventricular Septal Defect （VSD,室間隔缺損 Ventricular Septal Defect （VSD,由胚胎期室間隔(流入道,小梁部和流出 道)發(fā)育不全所致,是最常見的先天性心臟病,約占我國(guó)先心病的50%.最多見為膜周部缺損,病理生理,分流量多少取決于缺損面積.心室間壓差及肺小動(dòng)脈,大致可分為三種類型: 小型室缺:缺損直徑小于5mm或缺損面積0.5

7、cm/m體表面積.血流動(dòng)力學(xué)變化不大 中型室缺:缺損直徑515mm或缺損面積0.51.0cm/m體表面積分流量較多,但 因肺血管床有很豐富的后備容受量,肺動(dòng)脈收縮壓和肺血管阻力可在較長(zhǎng)時(shí)期不增高,缺損,病理生理,大型室間隔缺損:缺損直徑大于15mm或缺損面積1.0cm/m體表面積.隨著肺血管病變進(jìn)行系性發(fā)展則漸變?yōu)椴豢赡娴淖枇π苑蝿?dòng)脈高壓. 當(dāng)右室收縮壓超過左室收縮壓,左向右分流逆轉(zhuǎn)為雙向分流或右向左分流,出現(xiàn)紫紺,即艾森曼格(Eiseenmenger)綜合癥,室間隔缺損 血流動(dòng)力學(xué)示意圖 Hemodynamics Figure of VSD,PV,SVC,RA,PA,IVC,RV,PV,LA

8、,AO,LV,肺靜脈,上腔靜脈,右心房,肺動(dòng)脈,下腔靜脈,右心室,肺靜脈,左心房,主動(dòng)脈,左心室,VSD murmur,室間隔缺損血流動(dòng)力學(xué)變化 Hemodynamics of VSD,小室缺 可無血流動(dòng)力學(xué)變化Small VSD no hemodynamics changes,大室缺 大量左向右分流 Large VSD Large left-to-right shunts,動(dòng)力型肺動(dòng)脈高壓 Dynamic PH,梗阻型肺動(dòng)脈高壓 Obstructed PH,艾森門格綜合征 Eisenmenger Syndrome,肺動(dòng)脈高壓Pulmonary Hypertension (PH,VSD臨床表現(xiàn)

9、Clinical findings of VSD,癥狀 Symptoms,反復(fù)呼吸道感染，生長(zhǎng)發(fā)育落后、乏力、氣短、聲嘶 frequent respiratory infections, grow slowly, very poor weight, dyspnea, exercise intolerance, fatigue, congestive heart failure,體征 Signs,LSB3-4可聞3-4/6級(jí)粗糙全收縮期吹風(fēng)樣雜音，向周圍廣泛傳導(dǎo)，伴震顫 A grade -/, medium-to-high pitched , harsh pansystolic murmur a

10、t the left sternal border (LSB) in the 3rd and 4th intercostal spaces,肺動(dòng)脈瓣第二音亢進(jìn) The pulmonary component of S2 is accentuation in intensity,合并主動(dòng)脈瓣關(guān)閉不全時(shí)可聞舒張期雜音 A diastolic murmur indicate VSD combine with aortic regurgitation,室間隔缺損并發(fā)癥Complication of VSD,支氣管肺炎 Bronchopneumonia 充血性心力衰竭 Congestive heart

11、failure 肺水腫 Pulmonary edema 感染性心內(nèi)膜炎 Infective endocarditis,房間隔缺損 Atrial Septal Defect （ASD,房間隔缺損,房間隔缺損(atrial septal defect)是小兒時(shí)期常見的先天性心臟病,該病的發(fā)病率約為活產(chǎn)嬰兒的1/1500,占先天性心臟病發(fā)病總數(shù)的5%-10%.女性較多見,男女性別比例為1:2,病理解剖,原發(fā)孔型房間隔缺損 部分型心內(nèi)膜墊缺損 繼發(fā)孔型房間隔缺損 中央型 靜脈竇型房間隔缺損 上腔型和下腔型 冠狀靜脈竇型 又稱冠狀靜脈竇型缺損 無頂冠狀竇,PV,LA,PA,LV,PV,SVC,RA,AO

12、,IVC,RV,肺靜脈,肺靜脈,上腔靜脈,右心房,主動(dòng)脈,下腔靜脈,右心室,左心房,肺動(dòng)脈,左心室,房間隔缺損 血流動(dòng)力學(xué)示意圖 Hemodynamics Figure of ASD,ASD murmur,上、下腔靜脈血 肺靜脈 右心房（擴(kuò)大） 左心房 右心室 （增大） 左心室（血量 減少） 肺血流量明顯增加（肺充血） 肺小動(dòng)脈痙攣、增厚 體循環(huán)供血不足 右向左分流 （消瘦、乏力、心悸、氣短等） 艾森門格綜合征 (少數(shù)病人晚期,ASD,房間隔缺損 血流動(dòng)力學(xué)變化 Hemodynamics of ASD,癥狀 Symptoms,同室缺相似 Symptoms of ASD are similar

13、with that of VSD,體征 Signs,LSB2-3可聞1-3/6級(jí)柔和收縮期噴射音，無震顫 A grade -/ ejection SM is heard best at the LSB in the 2nd intercostal space，no thrill,P2固定分裂 S2 at the pulmonary area is widely split and often fixed,肺動(dòng)脈瓣第二音亢進(jìn) The pulmonary component of S2 is accentuation in intensity,三尖瓣區(qū)可聞舒張期雜音 A mid-diastolic

14、 murmur can often be heard in tricuspid area,ASD臨床表現(xiàn)Clinical findings of ASD,房間隔缺損并發(fā)癥Complication of ASD,支氣管肺炎 Bronchopneumonia 充血性心力衰竭 Congestive heart failure 感染性心內(nèi)膜炎 Infective endocarditis,輔助檢查,X線表現(xiàn) 透視下可見肺動(dòng)脈總干及分支隨心臟搏動(dòng)而一明一暗的”肺門舞蹈”征,心影略呈梨形.原發(fā)孔型房缺伴二尖瓣裂缺者,左心房及左心室增大,治療,小于3mm的房間隔缺損多在3個(gè)月內(nèi)自然閉合,大于8mm的房缺一般

15、不會(huì)自然閉合. 反復(fù)呼吸道感染,發(fā)生心力衰竭或合并肺動(dòng)脈高壓者應(yīng)盡早手術(shù).房間隔缺損也可通過介入性 心導(dǎo)管術(shù),應(yīng)用雙面蘑菇傘關(guān)閉缺損,動(dòng)脈導(dǎo)管未閉 Patent ductus arteriosus (PDA,動(dòng)脈導(dǎo)管未閉,為小兒先天性心臟病常見類型之一,占先天性心臟病發(fā)病總數(shù)的15%.胎兒期動(dòng)脈導(dǎo)管被動(dòng)開放是血液循環(huán)的重要通道,出生后,大約15小時(shí)既發(fā)生功能性關(guān)閉,80%在生后3個(gè)月解剖性關(guān)閉.到一年,在解剖學(xué)上應(yīng)完全關(guān)閉.若持續(xù)開放,并產(chǎn)生病理,生理改變,即稱動(dòng)脈導(dǎo)管未閉,病理分型,管型:導(dǎo)管長(zhǎng)度多在1cm左右,直徑粗細(xì)不等 漏斗型:長(zhǎng)度與管型相似,但其近主動(dòng)脈端粗大, 向肺動(dòng)脈端逐漸變窄;

16、 窗型:肺動(dòng)脈與主動(dòng)脈緊貼,兩者之間為一孔道, 直徑往往較大,PV,SVC,RA,PA,IVC,RV,PDA,PV,LA,AO,LV,肺靜脈,肺靜脈,上腔靜脈,下腔靜脈,右心房,主動(dòng)脈,肺動(dòng)脈,右心室,左心房,左心室,動(dòng)脈導(dǎo)管未閉 血流動(dòng)力學(xué)示意圖 Hemodynamics Figure of PDA,動(dòng)脈導(dǎo)管,PDA murmur,右心室血流 體循環(huán) 舒張壓 肺動(dòng)脈 主動(dòng)脈 供血減少 脈壓增寬 肺血流量 肺動(dòng)脈高壓 艾森門格綜合征 左房、左室擴(kuò)大 （差異性紫紺） （右心室肥大,PDA,動(dòng)脈導(dǎo)管未閉 血流動(dòng)力學(xué)變化 Hemodynamics of PDA,癥狀 Symptoms,與VSD及AS

17、D相同 Symptoms of PDA are similar with that of VSD and ASD,體征 Signs,LSB2聞及粗糙、響亮的連續(xù)性機(jī)器樣雜音，占據(jù)整個(gè)收縮期及舒張期 A characteristic thrill and continuous and rough “machinery” murmur with a late systolic accentuation,心尖區(qū)可聞及舒張中期隆隆樣雜音 A diastolic flow murmur is often heard at the apex,周圍血管征 Peripheral vascular sigh,下

18、半身青紫(差異性紫紺)和杵狀趾 Differential cyanosis and clubbing,PDA臨床表現(xiàn)Clinical findings of PDA,差異性紫紺,當(dāng)肺動(dòng)脈壓力超過主動(dòng)脈壓時(shí),左向右分流明顯減少或停止,產(chǎn)生肺動(dòng)脈血流逆向分流入主動(dòng)脈,患兒呈現(xiàn)差異性紫紺(differential cyanosis),下半身青紫,左上肢有輕度青紫,右上肢正常,PDA堵閉前心音圖,S1,S2,PDA堵閉后心音圖,S1,S2,動(dòng)脈導(dǎo)管未閉并發(fā)癥Complication of PDA,支氣管肺炎 Bronchopneumonia 感染性心內(nèi)膜炎 Infective endocarditis

19、 充血性心力衰竭 Congestive heart failure 感染性動(dòng)脈炎 少見的并發(fā)癥有肺動(dòng)脈和動(dòng)脈導(dǎo)管瘤樣擴(kuò)張,動(dòng)脈導(dǎo)管鈣化及血栓形成,治療,早產(chǎn)兒動(dòng)脈導(dǎo)管未閉的處理視分流大小,呼吸窘迫綜合癥情況而定.生后一周內(nèi)使用消炎痛治療,仍有10%的病人需手術(shù)治療.采用介入療法選擇彈簧圈,蘑菇傘等關(guān)閉動(dòng)脈導(dǎo)管. 但在有些病例中,如完全性大血管轉(zhuǎn)位,肺動(dòng)脈閉鎖,三尖瓣閉鎖,嚴(yán)重的肺動(dòng)脈狹窄中,動(dòng)脈導(dǎo)管為依賴性者,對(duì)維持患嬰生命致關(guān)重要,此時(shí)應(yīng)該應(yīng)用前列腺素E以維持動(dòng)脈導(dǎo)管開放,左向右分流先心病共同臨床特點(diǎn)Common features of left-to-right shunting CHD,

20、一般情況下無青紫 In general there are noncyanosis 心前區(qū)有粗糙的收縮期雜音 A rough systolic murmur is heard in precordium 肺循環(huán)血量多，易患肺炎 Pulmonary flow increase and easy to suffer from bronchopneumonia 體循環(huán)血量少，影響生長(zhǎng)發(fā)育 Systemic flow decrease and the patient grow slowly,法洛四聯(lián)癥 Tetralogy of Fallot （TOF,法洛四聯(lián)癥,法洛四聯(lián)癥(tetralogy of

21、Fallot, TOF)是嬰兒期后最常見的青紫型先天性心臟病,約占所有先天性心臟病的10%.1888年法國(guó)醫(yī)生Etienne Fallot詳細(xì)描述了該病的病理改變及臨床表現(xiàn),故而得名,法洛四聯(lián)癥四種解剖畸形 The four malformations of TOF,右室流出道梗阻 室間隔缺損 Ventricular septal defect 主動(dòng)脈騎跨 Overriding of the aorta 右心室肥厚 Right ventricular hypertrophy 以上四種畸形中僅室間隔缺損 及右心室流出道狹窄是必須存在,室間隔缺損必須足夠大使左右心室的壓力相等;右心室流出道狹窄是決

22、定患兒的病理生理,病情嚴(yán)重程度及預(yù)后的主要原因.而且,狹窄可隨時(shí)間推移逐漸加重,PV,SVC,RA,PA,IVC,RV,PV,LA,AO,LV,肺靜脈,肺靜脈,上腔靜脈,右心房,肺動(dòng)脈,下腔靜脈,右心室,左心房,主動(dòng)脈,左心室,法洛四聯(lián)癥 血流動(dòng)力學(xué)示意圖 Hemodynamics Figure of TOF,TOF murmur,法洛四聯(lián)癥四種解剖畸形 The four malformations of TOF,肺動(dòng)脈狹窄Obstruction to right ventricular outflow 室間隔缺損 Ventricular septal defect 主動(dòng)脈騎跨 Overrid

23、ing of the aorta 右心室肥厚 Right ventricular hypertrophy,右 心室（肥厚） 左心室 肺動(dòng)脈狹窄 主動(dòng)脈 （血流量 、擴(kuò)張） 肺血流量減少（肺野清晰） 混合血進(jìn)入循環(huán) （青紫、發(fā)育落后、乏力 血氧合不足 （杵狀指趾等） 蹲踞、陣發(fā)性昏厥,分流,分流,法洛四聯(lián)癥 血流動(dòng)力學(xué)變化 Hemodynamics of TOF,青紫：本病最突出的癥狀 Cyanosis is the main symptoms,氣促和缺氧發(fā)作 Dyspnea and hypoxemic spells,蹲踞癥狀 Squatting posture,心臟體征 LSB2-4聞及 2-

24、4/6級(jí)收縮期噴射性雜音 There is a grade -/, rough, ejection-type SM that is maximal at the LSB in the 2nd to 3rd intercostal space and that radiates well to the back,杵狀指趾 Clubbing of the terminal digits,TOF臨床表現(xiàn)Clinical findings of TOF,杵狀指 Clubbing of fingers,杵狀指趾 Clubbing of fingers and toes,腦血栓 Cerebral embo

25、lism 腦膿腫Cerebral abscess 感染性心內(nèi)膜炎Infective endocarditis,法洛四聯(lián)癥并發(fā)癥Complication of TOF,X Ray of TOF,X線檢查 右室大、心尖上翹呈靴形，肺動(dòng)脈段凹陷，肺野清晰,治療,一般護(hù)理 平時(shí)應(yīng)經(jīng)常飲水,預(yù)防感染,及時(shí)補(bǔ)液,防治脫水和并發(fā)癥. 缺氧發(fā)作的治療 發(fā)作輕者使其取胸膝位即可緩解,重者應(yīng)立即吸氧,給予新福林 每次0.05mg/kg靜注,或心得安每次0.1mg/kg.必要時(shí)也可皮下注射嗎啡每次0.1-0.2mg/kg,糾正酸中毒,給予5%碳酸氫鈉1.55.0ml/kg靜注,經(jīng)常有缺氧發(fā)作者,可口服心得安1-3mg/(kg.d