超声心动图在胎儿肺动脉狭窄诊断及出生后转归评估中的临床价值

发布时间：2018-06-11 20:17

本文选题：肺动脉狭窄 + 胎儿　；参考：《第三军医大学》2017年硕士论文

【摘要】：背景胎儿先天性心脏病是胎儿系统筛查最常见的畸形,也属于重大出生缺陷之一,在活产儿中约占4~13‰,大约有20%新生儿和50%的婴儿因患先天性心脏病死亡,患有该疾病的胎儿宫内死胎的发生率较正常胎儿提高4到5倍,这给患儿家庭带来严重的精神和经济负担。因此,胎儿先天性心脏病准确的产前诊断在围产期、新生儿管理中具有重要的临床意义,起着早期预警的作用。肺动脉狭窄是常见的先天性心脏病之一,占所有先天性心脏病的10%~20%,它可以单独发生、成为单纯性肺动脉狭窄,也可以伴发其他心内外畸形或染色体异常。由于胎儿期心血管循环系统具有特殊的血流动力学特点,肺动脉狭窄在胎儿期的图像特征和血流动力学改变不典型,容易漏诊,但是一部分胎儿的肺动脉狭窄会随着孕周的增加,狭窄的程度呈持续性进展,由胎儿期的轻微病变演变为新生儿重度肺动脉狭窄,可致右室肥厚、右室腔狭小,甚至肺动脉闭锁,出现严重的症状和体征。因此,如何通过超声心动图尽早发现胎儿肺动脉狭窄及其它相关疾病,准确评估胎儿肺动脉狭窄的预后和临床转归,为针对性地干预治疗提供依据,一直是产前超声诊断领域研究的难点和热点问题。研究目的1.应用超声心动图观察胎儿肺动脉的结构,采用两种定量测量指标(包括肺动脉(瓣)/主动脉(瓣)[PA(PV)/AO(AV)]比值和肺动脉的Z评分)诊断胎儿肺动脉狭窄,探讨并系统分析胎儿肺动脉狭窄的产前超声心动图表现特点及其并发心内外畸形情况、与染色体异常的相关性。2.探讨胎儿单纯性肺动脉狭窄的超声图像特征,随访整个孕期的发展变化及其出生后的预后和临床转归。研究方法1.回顾性分析2014年1月~2016年12月在第三军医大学第一附属医院超声科进行产前超声系统畸形筛查的30382例胎儿资料,产前超声心动图诊断胎儿肺动脉狭窄共127例。首先进行胎儿生物测量来确定超声孕周,采用胎儿心脏序列切面系统扫查方法常规留取各个胎儿心脏切面图像,同时采用3D/4D时间-空间关联成像技术(STIC)留取实时动态图。对胎儿心脏解剖结构和血流动力学改变进行系统检测,测量各房室大小,肺动脉(瓣)、主动脉(瓣)内径及其比值,计算肺动脉的Z评分,将肺动脉的Z评分与肺动脉(瓣)/主动脉(瓣)的比值进行比较,分析这两种指标在诊断胎儿肺动脉狭窄的差异,并对合并心内、心外其他畸形及染色体异常的相关性进行分析。2.上述产前超声诊断的127例肺动脉狭窄的胎儿中,选取23例单纯性肺动脉狭窄病例的资料,探讨和分析胎儿单纯性肺动脉狭窄的声像图变化,并与产后超声心动图进行对比,观察产前产后血流动力学变化规律。结果1.30382例胎儿中,产前超声诊断胎儿肺动脉狭窄127例,经尸检或产后超声心动图证实肺动脉狭窄共105例,失访13例,6例产后超声心动图证实肺动脉内径及流速正常,3例尸检证实主动脉增宽,肺动脉内径正常。PA(PV)/AO(AV)比值在轻度肺动脉狭窄中的阳性检出率高于肺动脉的Z评分,但肺动脉Z评分的诊断符合率明显高于PA(PV)/AO(AV)比值。105例胎儿中肺动脉瓣狭窄32例(30.48%),肺动脉主干狭窄73例(69.52%)。在合并畸形方面,合并心内畸形85例(80.95%),以室间隔缺损、法洛氏四联症和右室双出口最多见,同时发现肺动脉干狭窄的病例中均合并心内外畸形;合并心外畸形37例(35.24%),以单脐动脉、泌尿系统畸形和神经系统畸形最常见;未合并其他畸形20例(19.05%),均为单纯性肺动脉瓣狭窄。105例肺动脉狭窄病例中,7例染色体异常(6.67%),其中3例为肺动脉瓣狭窄,4例为肺动脉干狭窄。2.产前超声诊断23例单纯性肺动脉狭窄,其中20例为单纯性肺动脉瓣狭窄(86.96%);3例为单纯性肺动脉干狭窄(13.04%)。23例中有16例正常分娩和7例肺动脉瓣狭窄选择引产。7例引产中5例尸检证实为肺动脉瓣重度狭窄和1例染色体异常。16例正常分娩的新生儿产后超声心动图证实13例有不同程度的肺动脉瓣狭窄:9例轻度狭窄,1例中度狭窄,3例重度狭窄。其中,轻中度肺动脉瓣狭窄预后较好,5例轻度狭窄后期随访肺动脉跨瓣压变化不大;2例出生时轻度狭窄的患儿随着年龄的增加,肺动脉跨瓣压差降低、甚至恢复正常;另外2例出生时为轻度狭窄,后期随访肺动脉跨瓣压差增加至中度狭窄。重度肺动脉瓣狭窄者预后差,可致严重心功能不全。3例产前诊断为单纯性肺动脉干狭窄均为轻度,出生后超声心动图证实肺动脉内径及流速正常。结论1.两种定量方法应进行综合评估,才能提高胎儿时期肺动脉狭窄及其程度的诊断准确性。肺动脉狭窄容易合并心内大血管畸形及心外畸形,尤其是肺动脉干狭窄;肺动脉狭窄与染色体异常也有一定的相关性,合并有心内外畸形的胎儿染色体异常率高于无合并畸形的胎儿,肺动脉瓣狭窄伴染色体异常率高于肺动脉干狭窄。因此,当产前超声诊断出肺动脉狭窄尤其合并有心内外畸形时,应常规进行产前遗传学咨询,并进行羊水或脐带血穿刺做染色体核型分析。2.胎儿重度肺动脉瓣狭窄具有典型的声像图特征,轻度和中度单纯性肺动脉狭窄因图像特征不明显容易漏诊,因此胎儿单纯性肺动脉狭窄需结合肺动脉内径、肺动脉瓣回声和开放情况、右室大小及室壁厚度、三尖瓣反流、动脉导管有无逆流等情况进行综合评价同时需通过产前和产后动态随访观察和评估预后,提高产前超声诊断准确率,为临床提供最佳决策和治疗方案。
[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.
【学位授予单位】：第三军医大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R540.45;R714.5

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