本文選題：肺動脈狹窄 + 胎兒��； 參考：《第三軍醫(yī)大學(xué)》2017年碩士論文

【摘要】：背景胎兒先天性心臟病是胎兒系統(tǒng)篩查最常見的畸形,也屬于重大出生缺陷之一,在活產(chǎn)兒中約占4~13‰,大約有20%新生兒和50%的嬰兒因患先天性心臟病死亡,患有該疾病的胎兒宮內(nèi)死胎的發(fā)生率較正常胎兒提高4到5倍,這給患兒家庭帶來嚴重的精神和經(jīng)濟負擔。因此,胎兒先天性心臟病準確的產(chǎn)前診斷在圍產(chǎn)期、新生兒管理中具有重要的臨床意義,起著早期預(yù)警的作用。肺動脈狹窄是常見的先天性心臟病之一,占所有先天性心臟病的10%~20%,它可以單獨發(fā)生、成為單純性肺動脈狹窄,也可以伴發(fā)其他心內(nèi)外畸形或染色體異常。由于胎兒期心血管循環(huán)系統(tǒng)具有特殊的血流動力學(xué)特點,肺動脈狹窄在胎兒期的圖像特征和血流動力學(xué)改變不典型,容易漏診,但是一部分胎兒的肺動脈狹窄會隨著孕周的增加,狹窄的程度呈持續(xù)性進展,由胎兒期的輕微病變演變?yōu)樾律鷥褐囟确蝿用}狹窄,可致右室肥厚、右室腔狹小,甚至肺動脈閉鎖,出現(xiàn)嚴重的癥狀和體征。因此,如何通過超聲心動圖盡早發(fā)現(xiàn)胎兒肺動脈狹窄及其它相關(guān)疾病,準確評估胎兒肺動脈狹窄的預(yù)后和臨床轉(zhuǎn)歸,為針對性地干預(yù)治療提供依據(jù),一直是產(chǎn)前超聲診斷領(lǐng)域研究的難點和熱點問題。研究目的1.應(yīng)用超聲心動圖觀察胎兒肺動脈的結(jié)構(gòu),采用兩種定量測量指標(包括肺動脈(瓣)/主動脈(瓣)[PA(PV)/AO(AV)]比值和肺動脈的Z評分)診斷胎兒肺動脈狹窄,探討并系統(tǒng)分析胎兒肺動脈狹窄的產(chǎn)前超聲心動圖表現(xiàn)特點及其并發(fā)心內(nèi)外畸形情況、與染色體異常的相關(guān)性。2.探討胎兒單純性肺動脈狹窄的超聲圖像特征,隨訪整個孕期的發(fā)展變化及其出生后的預(yù)后和臨床轉(zhuǎn)歸。研究方法1.回顧性分析2014年1月~2016年12月在第三軍醫(yī)大學(xué)第一附屬醫(yī)院超聲科進行產(chǎn)前超聲系統(tǒng)畸形篩查的30382例胎兒資料,產(chǎn)前超聲心動圖診斷胎兒肺動脈狹窄共127例。首先進行胎兒生物測量來確定超聲孕周,采用胎兒心臟序列切面系統(tǒng)掃查方法常規(guī)留取各個胎兒心臟切面圖像,同時采用3D/4D時間-空間關(guān)聯(lián)成像技術(shù)(STIC)留取實時動態(tài)圖。對胎兒心臟解剖結(jié)構(gòu)和血流動力學(xué)改變進行系統(tǒng)檢測,測量各房室大小,肺動脈(瓣)、主動脈(瓣)內(nèi)徑及其比值,計算肺動脈的Z評分,將肺動脈的Z評分與肺動脈(瓣)/主動脈(瓣)的比值進行比較,分析這兩種指標在診斷胎兒肺動脈狹窄的差異,并對合并心內(nèi)、心外其他畸形及染色體異常的相關(guān)性進行分析。2.上述產(chǎn)前超聲診斷的127例肺動脈狹窄的胎兒中,選取23例單純性肺動脈狹窄病例的資料,探討和分析胎兒單純性肺動脈狹窄的聲像圖變化,并與產(chǎn)后超聲心動圖進行對比,觀察產(chǎn)前產(chǎn)后血流動力學(xué)變化規(guī)律。結(jié)果1.30382例胎兒中,產(chǎn)前超聲診斷胎兒肺動脈狹窄127例,經(jīng)尸檢或產(chǎn)后超聲心動圖證實肺動脈狹窄共105例,失訪13例,6例產(chǎn)后超聲心動圖證實肺動脈內(nèi)徑及流速正常,3例尸檢證實主動脈增寬,肺動脈內(nèi)徑正常。PA(PV)/AO(AV)比值在輕度肺動脈狹窄中的陽性檢出率高于肺動脈的Z評分,但肺動脈Z評分的診斷符合率明顯高于PA(PV)/AO(AV)比值。105例胎兒中肺動脈瓣狹窄32例(30.48%),肺動脈主干狹窄73例(69.52%)。在合并畸形方面,合并心內(nèi)畸形85例(80.95%),以室間隔缺損、法洛氏四聯(lián)癥和右室雙出口最多見,同時發(fā)現(xiàn)肺動脈干狹窄的病例中均合并心內(nèi)外畸形;合并心外畸形37例(35.24%),以單臍動脈、泌尿系統(tǒng)畸形和神經(jīng)系統(tǒng)畸形最常見;未合并其他畸形20例(19.05%),均為單純性肺動脈瓣狹窄。105例肺動脈狹窄病例中,7例染色體異常(6.67%),其中3例為肺動脈瓣狹窄,4例為肺動脈干狹窄。2.產(chǎn)前超聲診斷23例單純性肺動脈狹窄,其中20例為單純性肺動脈瓣狹窄(86.96%);3例為單純性肺動脈干狹窄(13.04%)。23例中有16例正常分娩和7例肺動脈瓣狹窄選擇引產(chǎn)。7例引產(chǎn)中5例尸檢證實為肺動脈瓣重度狹窄和1例染色體異常。16例正常分娩的新生兒產(chǎn)后超聲心動圖證實13例有不同程度的肺動脈瓣狹窄:9例輕度狹窄,1例中度狹窄,3例重度狹窄。其中,輕中度肺動脈瓣狹窄預(yù)后較好,5例輕度狹窄后期隨訪肺動脈跨瓣壓變化不大;2例出生時輕度狹窄的患兒隨著年齡的增加,肺動脈跨瓣壓差降低、甚至恢復(fù)正常;另外2例出生時為輕度狹窄,后期隨訪肺動脈跨瓣壓差增加至中度狹窄。重度肺動脈瓣狹窄者預(yù)后差,可致嚴重心功能不全。3例產(chǎn)前診斷為單純性肺動脈干狹窄均為輕度,出生后超聲心動圖證實肺動脈內(nèi)徑及流速正常。結(jié)論1.兩種定量方法應(yīng)進行綜合評估,才能提高胎兒時期肺動脈狹窄及其程度的診斷準確性。肺動脈狹窄容易合并心內(nèi)大血管畸形及心外畸形,尤其是肺動脈干狹窄;肺動脈狹窄與染色體異常也有一定的相關(guān)性,合并有心內(nèi)外畸形的胎兒染色體異常率高于無合并畸形的胎兒,肺動脈瓣狹窄伴染色體異常率高于肺動脈干狹窄。因此,當產(chǎn)前超聲診斷出肺動脈狹窄尤其合并有心內(nèi)外畸形時,應(yīng)常規(guī)進行產(chǎn)前遺傳學(xué)咨詢,并進行羊水或臍帶血穿刺做染色體核型分析。2.胎兒重度肺動脈瓣狹窄具有典型的聲像圖特征,輕度和中度單純性肺動脈狹窄因圖像特征不明顯容易漏診,因此胎兒單純性肺動脈狹窄需結(jié)合肺動脈內(nèi)徑、肺動脈瓣回聲和開放情況、右室大小及室壁厚度、三尖瓣反流、動脈導(dǎo)管有無逆流等情況進行綜合評價同時需通過產(chǎn)前和產(chǎn)后動態(tài)隨訪觀察和評估預(yù)后,提高產(chǎn)前超聲診斷準確率,為臨床提供最佳決策和治療方案。
[Abstract]:Background fetal congenital heart disease is the most common malformation of fetal system screening. It also belongs to one of the major birth defects. It accounts for about 4~13 per thousand in live births. About 20% neonates and 50% of infants die from congenital heart disease, and the incidence of fetal intrauterine fetal death is 4 to 5 times more than that of normal fetus. Therefore, the accurate prenatal diagnosis of fetal congenital heart disease is of important clinical significance in perinatal period and in the management of newborns. It plays an early warning role. Pulmonary artery stenosis is one of the common congenital heart diseases, which accounts for 10%~20% of all congenital heart disease. It can occur alone and become simple. Pulmonary stenosis can also be accompanied by other internal and external malformations of the heart or chromosomal abnormalities. Due to the special hemodynamic characteristics of the cardiovascular circulatory system in the fetus, the image characteristics and hemodynamic changes of the pulmonary artery stenosis in the fetus are not typical and easy to be missed, but the pulmonary stenosis in a part of the fetus will increase with the increase of the gestational age. The degree of stenosis has been progressed continuously, from mild fetal lesions to severe pulmonary stenosis of the newborn, which can cause right ventricular hypertrophy, narrow right ventricle cavity, even pulmonary atresia, and serious symptoms and signs. Therefore, how to evaluate the fetus accurately by echocardiography as early as possible fetal pulmonary stenosis and other related diseases The prognosis and clinical outcome of pulmonary stenosis provide the basis for the targeted intervention treatment. It has always been a difficult and hot issue in the field of prenatal ultrasound diagnosis. Objective 1. the structure of the fetal pulmonary artery was observed by echocardiography, and the ratio of two quantitative measurements (including the pulmonary artery (valve) / aortic (valve) [PA (PV) /AO (AV)) ratio was used. Z score of pulmonary artery and pulmonary artery stenosis diagnosis of fetal pulmonary stenosis. The characteristics of prenatal echocardiography in fetal pulmonary stenosis and its complication of intra and extra cardiac abnormalities were systematically analyzed and analyzed. The correlation of chromosomal abnormalities and.2. was used to investigate the ultrasonic image characteristics of fetal simple pulmonary stenosis, and follow up the development of the whole pregnancy and its birth. Postoperative prognosis and clinical outcomes. 1. a retrospective analysis of 30382 fetal data for prenatal ultrasound screening in the First Affiliated Hospital of Third Military Medical University in December ~2016 January 2014, and 127 cases of fetal pulmonary stenosis diagnosed by prenatal echocardiography. The fetal heart sequence system scanning method was used to keep the images of each fetal heart cut, and the 3D/4D time spatial correlation imaging (STIC) was used to keep the real-time dynamic map. The anatomical structure and hemodynamic changes of the fetal heart were measured, and the size of the atrioventricular, pulmonary artery (valve), and the internal diameter of the aorta were measured. The ratio, the Z score of the pulmonary artery was calculated, the ratio of the Z score of the pulmonary artery to the ratio of the pulmonary artery (valve) / aorta (valve) was compared, and the difference between the two indexes in the diagnosis of fetal pulmonary stenosis was analyzed, and the correlation of the intra cardiac, extra cardiac malformation and chromosome abnormality was analyzed in 127 cases of pulmonary artery diagnosed by.2. prenatal ultrasound. In the narrow fetus, 23 cases of simple pulmonary stenosis were selected to investigate and analyze the changes in the sound image of simple pulmonary stenosis. Compared with postpartum echocardiography, the changes of hemodynamics were observed before and after prenatal and postpartum. In the 1.30382 fetus, 127 cases of fetal pulmonary stenosis were diagnosed by prenatal ultrasound. There were 105 cases of pulmonary stenosis with or after postpartum echocardiography, 13 cases were lost, 6 cases had normal pulmonary artery diameter and flow velocity, 3 autopsy confirmed that the aorta was widened, and the positive rate of.PA (PV) /AO (AV) ratio in the pulmonary artery diameter was higher than that of the pulmonary artery Z score, but the pulmonary artery Z score was higher than that of the pulmonary artery stenosis. The diagnostic coincidence rate was significantly higher than that of PA (PV) /AO (AV) ratio in.105 of 32 cases of pulmonary stenosis (30.48%) and 73 cases of pulmonary artery stenosis (69.52%). In the combination of malformation, 85 cases (80.95%) were combined with cardiac malformation, ventricular septal defect, tetralogy of Fallot's tetralogy and right ventricle double exit were the most common, and all cases of pulmonary artery stenosis were found in all cases. 37 cases (35.24%) with external malformation (37 cases) with single umbilical artery, urinary system malformation and nervous system malformation were the most common. There were no other malformations in 20 cases (19.05%). There were 7 cases of chromosomal abnormalities (6.67%) in.105 cases with simple pulmonary stenosis, 3 were pulmonary stenosis and 4 were pulmonary artery stem stenosis.2 23 cases of simple pulmonary stenosis were diagnosed by prenatal ultrasound, including 20 cases of simple pulmonary stenosis (86.96%), 3 cases of simple pulmonary artery stenosis (13.04%), 16 cases of normal delivery and 7 cases of pulmonary valve stenosis selected for induction of labor in.7 cases, of which 5 cases were confirmed to be severe stenosis of pulmonary valve and 1 cases of chromosomal abnormality in.16 cases. Postpartum echocardiography of newborns in childbirth confirmed that 13 cases had different degrees of pulmonary stenosis: 9 mild stenosis, 1 moderate stenosis and 3 severe stenosis. Among them, the prognosis of mild and moderate pulmonary stenosis was better, 5 cases of mild stenosis were followed up with little change of pulmonary artery cross valve pressure; 2 cases with mild stenosis at birth increased with age. The pulmonary artery cross valve pressure decreased and even returned to normal. The other 2 cases were mild stenosis at birth, and the pulmonary artery cross valve pressure difference increased to moderate stenosis at the later stage. Severe pulmonary stenosis was poor in prognosis. Severe cardiac insufficiency could lead to mild pulmonary artery stenosis in.3 cases, and postnatal echocardiogram confirmed pulmonary movement. Conclusion the internal diameter and flow velocity are normal. Conclusion 1. quantitative methods should be evaluated comprehensively to improve the diagnostic accuracy of the pulmonary stenosis and its degree in the fetus. The pulmonary stenosis is easy to combine with the great vascular malformation and external malformation of the heart, especially the stenosis of the pulmonary artery, and the pulmonary stenosis is also associated with the chromosome abnormality. The abnormal rate of fetal chromosomes in both internal and external malformations is higher than that of the unincorporated fetus. The rate of pulmonary stenosis with chromosome abnormality is higher than that of the pulmonary artery stenosis. Therefore, prenatal genetic counseling should be carried out routinely when prenatal ultrasound is used to diagnose pulmonary stenosis, especially with internal and external malformation, and amniotic fluid or umbilical cord blood puncture should be performed. Body karyotype analysis of.2. fetal severe pulmonary stenosis has typical image features. Mild to moderate simple pulmonary stenosis is not easily missed because of the image characteristics. Therefore, the fetal simple pulmonary stenosis needs to be combined with the diameter of the pulmonary artery, the echo and opening of the pulmonary valve, the size of the right ventricle and the thickness of the ventricular wall, and the reverse flow of the three tip valve. It is necessary to evaluate the prognosis, improve the accuracy of prenatal ultrasound diagnosis and provide the best decision and treatment plan for clinical practice.
【學(xué)位授予單位】：第三軍醫(yī)大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R540.45;R714.5

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