Fetal lung volume and pulmonary artery changes in congenital heart disease with decreased pulmonary blood flow: Quantitative ultrasound analysis

Background

It has been reported that congenital heart disease with decreased pulmonary blood flow (CHD‐DPBF) may affect postnatal lung morphogenesis and function. However, there has been a lack of information regarding the impact of CHD‐DPBF on prenatal fetal lung development.

Methods

Fifty‐four fetuses with CHD‐DPBF were compared with 110 controls. Fetal lung volume (FLV) was estimated using three‐dimensional ultrasonography (3D‐US). Estimated fetal weight (EFW) and McGoon index (MGI) were estimated using two‐dimensional ultrasonography (2D‐US).

Results

FLV/EFW and MGI values measured using sonography for the CHD‐DPBF group were significantly reduced compared to those of the control group (P < .05). Pearson correlation analysis indicated that the summed diameter of the right pulmonary artery and left pulmonary artery (RPA + LPA) measured by 2D‐US correlated well with FLV measured by 3D‐US.

Conclusions

In our study, FLV/EFW and MGI values for fetuses with CHD‐DPBF tended to be decreased and FLV was associated with RPA + LPA. We concluded that CHD‐DPBF might delay fetal lung development. The summed diameter of the RPA + LPA measurement could be used as an alternative to FLV for assessing fetal lung development.     

时间-空间相关成像技术诊断胎儿心脏畸形的应用研究

目的:探讨应用STIC技术诊断胎儿心脏畸形的应用价值。方法:对1 286例胎儿进行超声心动图顺序检查,检查过程中应用STIC技术采集心脏容积数据,数据存盘后进行图像后处理并脱机分析,对心脏畸形的胎儿超声心动图结果,与出生后复查超声心动图及引产后病理结果进行对照分析。结果:STIC技术结合胎儿超声心动图检出胎儿心脏异常189例,其中心脏复杂畸形36例,合计91处心脏畸形,STIC技术产前诊断胎儿心脏畸形的灵敏度(97.4%),特异度(99.6%),误诊率(0.4%),漏诊率(2.6%),总的符合率(99.2%)。结论 :STIC技术结合胎儿超声心动图诊断胎儿心脏畸形具有较高的灵敏度、特异度,对胎儿心脏畸形可以明确诊断。     

Can 3D Ultrasound and Doppler Angiography of Great Arteries Be Included in Second Trimester Ecocardiographic Examination? A Prospective Study on Low‐Risk Pregnancy Population

The introduction of new applications such as the spatiotemporal image correlation (STIC) and the tomographic ultrasound imaging (TUI) has greatly enhanced the study of cardiac and vessels anatomy. The aim of our study was to assess the feasibility of including real-time volumetric 3D reconstruction of heart anatomy and Doppler angiography of great vessels during routine second trimester pregnancy scan, in a low-risk population. Visualization of the pulmonary veins, ductus venosus and inferior vena cava was significantly superior with 3D ultrasound. Therefore, STIC makes it possible to store heart beats for a mean time of 10 s, to replay acquired volumes in slow motion, to study the heart atrium along three axes and retrace volumetric image storage inversely from the apex to the base of the heart, making reconstruction of the four-chamber view and great artery outflow tract much easier and faster. Furthermore, the use of digital communication allow operators to store and send volume of images that can be postprocessed by external consultant specialists worldwide. Although 3D echocardiography is less time-consuming and independent of fetal positioning in utero when compared with 2D it may be premature to include 3D and 3D Doppler angiography among routine examination. However, the study of the outflow tracts by 2D echocardiography should be implemented in order to improve the diagnostic accuracy of CHDs. At present, only "targeted" cases in which a routine 2D scan has raised diagnostic doubts should undergo a second level sonography for a thorough 2D-3D examination of cardiac and vessels anatomy.     

Growth of the great vessels in the normal human fetus and in the fetus with cardiac defects

BACKGROUND. There is a paucity of quantitative anatomic data regarding human great vessel development that could be useful as a reference for fetal echocardiographers who must distinguish abnormal from normal cardiac development at early stages. METHODS AND RESULTS. To determine normal growth patterns, we plotted the diameters of the aortic and pulmonary valves, ductus arteriosus, aortic isthmus, and descending aorta in 274 autopsy specimens from nonselected spontaneous abortuses of normal karyotype. There was a linear increase in the diameters of these structures within the developmental period studied (10-26 weeks). A relative narrowing of the aorta at the isthmus compared with the aortic valve and descending aorta probably indicates that the majority of fetal left heart output goes to the developing heart and brain. In contrast to previous studies of late gestation and neonatal animals, however, we found that the diameter of the aortic isthmus was larger than that of the ductus arteriosus, suggesting substantial isthmic blood flow in these midtrimester fetuses. Among nineteen other hearts with diverse defects, both of two hearts with a narrow isthmus had an enlarged ductus arteriosus and one heart with pulmonary atresia/intact septum had a narrow ductus and increased aortic valve diameter. CONCLUSIONS. During midgestation, the normal heart may have substantial aortic isthmic blood flow that diminishes due to rerouting in late gestation when increased requirements of the fetal brain and other organs prevail. Although fetal shunts may explain some vessel abnormalities, the majority of cardiac defects in this study were not associated with abnormal growth of the great vessels within this developmental age range.     

4D fetal echocardiography—An update

With the introduction of the electronic 4‐dimensional and spatial‐temporal image Correlation (e‐STIC), it is now possible to obtain large volume datasets of the fetal heart that are virtually free of artifact. This allows the examiner to use a number of imaging modalities when recording the volumes that include two‐dimensional real time, power and color Doppler, and B‐flow images. Once the volumes are obtained, manipulation of the volume dataset allows the examiner to recreate views of the fetal heart that enable examination of cardiac anatomy. The value of this technology is that a volume of the fetal heart can be obtained, irrespective of the position of the fetus in utero, and manipulated to render images for interpretation and diagnosis. This article presents a summary of the various imaging techniques and provides clinical examples of its application used for prenatal diagnosis of congenital heart defects and abnormal cardiac function.     

胎儿心脏超声智能导航技术检查胎儿心脏先天性畸形的临床应用价值

目的 探讨胎儿心脏超声智能导航技术检查胎儿心脏先天性畸形的临床应用价值.方法 选择2018-07～2019-12于成都市第一人民医院经胎儿超声心动图检查诊断为胎儿心脏先天性畸形的单胎11例.于孕18.4～30.0(24.7±2.7)周应用胎儿心脏超声智能导航技术进行检查,分析先天性心脏畸形胎儿的心脏容积数据及9个胎儿心...     

胎儿先天性心脏病的超声诊断及漏诊分析

目的探讨超声诊断胎儿先天性心脏病的可行性和临床价值。方法采用彩色多普勒超声对6000例孕20~40周的孕妇常规行胎儿四腔心,左、右室流出道,主动脉弓及动脉导管的观察。结果发现心脏畸形24例;经尸检及出生后心超证实。合关心外畸形7例。漏诊9例。结论超声诊断胎儿先天性心脏病是可行的,值得推广。     

Complete Atrioventricular Septal Defect with Truncus Arteriosus and Interrupted Aortic Arch in the Fetus

Complete atrioventricular septal defect is generally diagnosed on the four‐chamber view of the fetal heart. This defect can be accompanied by additional outflow tract lesions, including truncus arteriosus. Although truncus arteriosus in isolation can be associated with interrupted aortic arch, we describe a fetal case with the rare association of all three diagnoses: complete atrioventricular septal defect with truncus arteriosus and interrupted aortic arch. This case points to the importance of evaluating the complete fetal heart even after one congenital cardiac abnormality is noted. The complexity of heart disease has implications for prenatal counseling and postnatal management.     

Clinical Features of the Complete Closure of the Ductus Arteriosus Prenatally

Objective. Prenatal constriction of the ductus arteriosus associated with maternal drug ingestion was reported several decades ago. There are fewer reports of the complete closure of the ductus arteriosus; therefore, the clinical features of the latter are poorly understood. The aim of this study is to clarify the clinical features of complete ductal closure and postnatal pulmonary hypertension by performing echocardiography of the fetus. Patients. We diagnosed four fetuses with complete ductal closure by performing fetal echocardiography and reviewed the prenatal and postnatal medical records of the mother and fetus. Results. One mother each had bronchial asthma, ulcerative colitis, and idiopathic thrombocytopenic purpura, and they had received nonsteroidal anti‐inflammatory drugs and/or corticosteroids during pregnancy. The fourth mother did not have basal disease and had not ingested any drugs. Fetal diagnosis was performed at 32–38 weeks of gestation. All fetuses had right heart dilatation with tricuspid regurgitation in the absence of any cardiac defects, and Doppler echocardiography indicated that the right ventricular pressure was elevated. Two of the fetuses had fetal hydrops, which suggested severe right heart dysfunction. All fetuses were delivered by emergent cesarean delivery. After birth, all the infants developed persistent pulmonary hypertension and required oxygen inhalation. Of these, three required mechanical ventilation, and two, nitric oxide inhalation. All infants improved within 2 weeks, and they had no neurological and cardiac complications after discharge. Conclusion. Right heart dilatation and severe tricuspid regurgitation in the absence of a cardiac defect in the fetus strongly suggested ductal dysfunction. Careful evaluation of ductal patency and right ventricular function can lead to precise early diagnosis and good prognosis.     

Congenital heart disease in low birth weight infants.

In this study of low birth weight infants (less than 2500 g), we compared the birth weight distribution of patients with patent ductus arteriosus associated with prematurity with that of patients having congenital heart disease other than patent ductus arteriosus. Among 1436 low birth weight infants 37 infants had congenital heart disease other than patent ductus arteriosus and 198 had isolated ductus arteriosus. Infants with congenital heart disease had a mean birth weight of 2018 grams (standard deviation = 370 g) which was significantly greater than that of infants with patent ductus arteriosus. Four of the 37 infants with congenital heart disease other than ductus arteriosus were among 1150 low birth weight infants born in this institution, an incidence of 3.5/1000 live low birth weight infants. Seventy-eight of the 198 infants with patent ductus arteriosus were among 1150 low birth weight infants born in this institution, an incidence of 70/1000 live low birth weight infants. Twenty-one of the 37 infants with congenital heart disease were of appropriate weight for gestational age and 16 were small for gestational age.     

Heart malformations in trisomy 13 and trisomy 18]

Congenital heart diseases were studied in children diagnosed of trisomy 13 and trisomy 18 in our hospital between January 1973 and July 1990. Twenty patients with trisomy 18 were diagnosed (18 females and two males). All had cardiac malformations. The findings were: ventricular septal defect in 16 cases (80%), valvular anomalies in 12 (63%), patent ductus arteriosus in nine (47%) and atrial septal defect or patent foramen ovale in 7 cases (36%). We found some complex congenital cardiac diseases: one atrioventricular canal, one tetralogy of Fallot, one hypoplastic left ventricle with mitral atresia and double outlet right ventricle, one case of univentricular heart with aortic outlet from a rudimentary cavity, a right ventricular atresia with pulmonary and tricuspid valves atresia. Nine cases of trisomy 13 were diagnosed (seven females and two males). We found: ventricular septal defect in 7 cases (77%), valvular disease in five (100% of the necropsy studies), secundum atrial septal defect in 4 patients (80%) and patent ductus arteriosus in two. Two cases presented hypoplastic left ventricle with aortic arch hypoplasia, one of them had subaortic stenosis and left superior vena cava being connected to the right atrium via coronary sinus; one case showed fibroelastosis. Our results have been similar to the previously reported and confirm the invariably presence of cardiac malformations in these syndromes. These malformations are an important sign of suspicion in fetal ultrasonography.     

Right atrial isomerism diagnosed by STIC-HD live flow and autopsy: A case report

Rationale:Right atrial isomerism (RAI) is one of the most severe forms of congenital heart disease. This case of RAI was so complex that it incorporated 7 heart defects. It can be challenging to display the spatial relationship between different anatomical structures using conventional two-dimensional and color ultrasound (2D-Doppler imaging); therefore, we used spatio-temporal image correlation (STIC) and high definition live flow imaging technology to vividly display this case of RAI in a stereoscopic mode.Patient concerns:A 24-year-old woman was referred to our tertiary center at 24 weeks of gestation. The woman had difficult conceiving. Once pregnant, she was opposed to abortion, even if there was a possibility of deformity.Diagnosis:The fetus presented with an atrioventricular septal defect, persistent left superior vena cava, supra-cardiac total anomalous pulmonary venous connection (TAPVC), double outlet right ventricle, right ductus arteriosus, right aortic arch (RAA) with mirror image branching, and aortic arch dysplasia.Interventions:After consulting a pediatric cardiologist, the woman requested an abortion and consented to an autopsy.Outcomes:Autopsy supported the echocardiographic findings.Lessons:Accurate diagnosis of RAI is essential for clinical and parent decision making. 2D-Doppler imaging combined with STIC-HD live flow can be used to visualize the spatial morphology of blood vessels, including the cardiac chambers and great vessels of the fetal heart, and smaller peripheral vessels.     

Use of casts in the necropsy diagnosis of fetal congenital heart disease.

OBJECTIVE--To evaluate a casting technique in the interpretation of fetal cardiac anatomy. DESIGN--In 32 fetuses, the echocardiographic and cast features were compared and correlated. PATIENTS--Three normal fetal heart specimens from spontaneous abortuses and 32 specimens from spontaneous or induced abortions with congenital heart malformation. RESULTS--There was close correlation between the echocardiographic and anatomical features in 32 abnormal fetuses studied. In some, additional features of diagnosis could be displayed on the cast and the relative sizes of the cardiac structures could be appreciated and defined. CONCLUSIONS--With increasing echocardiographic detection of congenital heart disease in early prenatal life, an increasing number of fetal heart specimens of small size are dissected for pathological confirmation. The use of silicone rubber casts to reproduce the internal anatomy proved a useful addition to dissection, providing a three dimensional model of the cardiac defect.     

Screening performance of congenital heart defects in first trimester using simple cardiac scan,nuchal translucency,abnormal ductus venosus blood flow and tricuspid regurgitation

Objective: The objective of this study was to analyze if the addition of simple cardiac scan in cases with increased nuchal translucency (NT) and/or abnormal ductus venosus (DV) blood flow, and/or tricuspid regurgitation (TCR) can improve detection of congenital heart defects (CHD) in chromosomally normal fetuses without non‐ cardiac defects at 11‐13 + 6 gestational weeks in a population of singleton pregnancies.
Methods: During the 10 years period, all singleton pregnancies at 11‐13 + 6 weeks were routinely scanned for NT, DV blood flow and TCR assessment and, if a sin‐ gle of these parameters was abnormal, simple cardiac scan with 2D gray scale and color and/or directional power Doppler in 4‐chamber (4‐CV) and 3 vessel and trachea views (3VTV) was performed.
Results: The sensitivity and specificity of NT ≥ 95th + DV R/A a‐wave + TCR in detecting CHD were 77% and 97%, respectively, and of simple cardiac scan, 67% and 98%, respectively. Area under the curve of receiver operating characteristic curve of NT ≥ 95th + DV R/A a‐wave + TCR was 0.838, and of NT ≥ 95th + DV R/A a‐wave + TCR + simple cardiac scan was 0.915.
Conclusions: In chromosomally normal fetuses without non‐cardiac anomalies, addition of simple cardiac scan to the combined first trimester screening parameters improves detection of major CHD during first trimester.     

Spectrum of congenital heart defects and extracardiac malformations associated with chromosomal abnormalities: results of a seven year necropsy study.

OBJECTIVE: To analyse the spectrum of congenital heart malformations, the frequency of extracardiac malformations, and the proportion of chromosome aberrations among fetuses sent for necropsy. MATERIAL: Necropsies were performed on 815 fetuses-448 induced abortions (55%), 220 spontaneous abortions (27%), and 147 stillbirths (18%)-during a seven year period (1991-97) in the department of pathology of the Charité Medical Centre in Berlin. A congenital heart defect was identified in 129 cases (16%). For all 129 fetuses, karyotyping and an ultrasound examination had been performed. RESULTS: Congenital heart defects were present in 22% of induced abortions (99 cases), 9% of spontaneous abortions (20 cases), and 7% of stillbirths (10 cases). The heart malformations were classified into 13 categories. A fetus with more than one defect was included only in the category of the most serious defect. The malformations in order of frequency were: ventricular septal defect (VSD) (28%), atrioventricular septal defect (AVSD) (16%), hypoplastic left heart (HLH) (16%), double outlet right ventricle (DORV) (12%), coarctation of the aorta (CoA) (6%), transposition of the great arteries (TGA) (4%), aortic valve stenosis (AoVS) (4%), tetralogy of Fallot (TOF) (3%), truncus arteriosus communis (TAC) (3%), pulmonary valve stenosis/pulmonary valve atresia (PaVS/PaVA) (3%), tricuspid atresia (TA) (3%), single ventricle (SV) (1.5%), and atrial septal defect (ASD) (0.5%). The most common congenital heart defects were VSD, AVSD, HLH, and DORV, which made up 72% of all the cases. In 11 cases the heart defect was isolated (no other cardiovascular or extracardiac malformations present), 85 cases (66%) were associated with additional cardiac malformations, 85 cases (66%) were associated with extracardiac malformations, and chromosome anomalies were detected in 43 cases (33%). CONCLUSIONS: Fetal congenital heart malformations are common. These defects are often associated with other cardiovascular and extracardiac malformations, as well as with chromosome anomalies. Complex heart defects such as AVSD, HLH, and DORV are frequent in fetuses, as they often lead to spontaneous abortion or stillbirth or, after prenatal diagnosis, to deliberate termination of pregnancy.     

Three-dimensional rotational digital angiography in a complicated case of patent ductus arteriosus transcatheter closure.

There has been no report so far as to the usefulness of 3D rotational angiography in congenital heart disease interventions. We present a case of a difficult patent ductus arteriosus in which 3D rotational angiography was the key to successful closure. A 64-year-old woman who had been operated on many years previously for a patent ductus arteriosus came to our attention for a heart murmur on the left upper sternal border. Echocardiography revealed a still patent duct with moderate shunt. The patient underwent cardiac magnetic resonance imaging, which confirmed patency of the duct, but its diameter and length were difficult to measure due to the particular orientation and morphology of the previously legated duct. It was decided to attempt device closure. Three-dimensional rotational digital subtraction technique enabled us to find the most useful projection for measuring the duct and selecting the best implant device (coil or Amplatzer device). The exact oblique and cranio-caudal degree of the selected projection on rotational 3D reconstruction was replicated to obtain the best standard digital subtraction angiographic view of the duct. Thus, a 5/4-mm Amplatzer Duct Occluder was selected and successfully implanted with excellent results. This case suggests that 3D rotational angiography may be an important imaging tool to guide congenital heart disease interventions such as patent ductus arteriosus closure or aortic coarctation repair.     

时间-空间相关成像技术初步评价正常胎儿心脏心室容积及心室收缩功能

目的 探讨时间-空间相关成像技术(STIC技术)在正常胎儿心脏心室容积及心室收缩功能方面的应用.方法 选取进行胎儿超声心动检查的正常妊娠孕妇136例,按孕周分为22～周、23～周、24～周、25～周、26～周、27～周、28～周7组.运用时间-空间相关成像技术后处理软件获得容积参数,包括舒张末期容积(EDV)、收缩末期容积(ESV)及心室收缩功能参数(EF值)等,分析胎儿心室容积发育与孕周的关系,比较左右心室容积和收缩功能的差异.结果 胎儿左右心室容积参数EDV、ESV与孕周间均有线性相关关系,随孕周增长而增加,而收缩功能参数EF与孕周之间无明显线性关系,随孕周增长无明显变化;容积参数EDV、ESV左右心室之间差异无统计学意义,而EF左右心室间差异有统计学意义(P＜0.05).结论 时间-空间相关成像技术对胎儿心内膜清晰成像时能够获得心室腔容积,进而评价心脏收缩功能,有助于进一步研究胎儿心脏容积发育和收缩功能变化.     

Accuracy of electrical impedance cardiography for measuring cardiac output in children with congenital heart defects

This study determined whether noninvasive electrical impedance cardiography accurately measures systemic blood flow (cardiac output) in children with congenital heart defects. A total of 37 patients ranging in age from 2 to 171 months underwent complete right- and left-sided heart catheterizations that included simultaneous Fick and impedance measurement of cardiac output. Based on the diagnosis, 4 groups were formed consisting of a control group (n = 11) with no shunts, a group with intracardiac left-to-right shunting and an atrial septal defect (n = 7), another with a ventricular septal defect (n = 12) and an extracardiac left-to-right shunting with patent ductus arteriosus group (n = 7). Impedance values for systemic blood flow were compared with systemic and pulmonary blood flow obtained by the direct Fick method with measured oxygen consumption. The difference between impedance and Fick systemic blood flow was less than or equal to 5% in each of the 4 groups. The highest correlation between impedance and Fick systemic blood flow was with the atrial septal defect group (r = 0.89) and lowest with the ventricular septal defect and control (r = 0.69) groups. Fick pulmonary blood flow was significantly greater than impedance or Fick systemic flow in all 3 shunt groups. Impedance cardiography accurately measured systemic blood flow in children without shunts or valvular insufficiency. Likewise, systemic blood flow was accurately measured by impedance in the presence of intracardiac left-to-right shunts (atrial and ventricular septal defects) and extracardiac left-to-right shunts (patent ductus arteriosus).(ABSTRACT TRUNCATED AT 250 WORDS)     

Maternal and fetal outcome in operated vs non-operated cases of congenital heart disease cases in pregnancy

ObjectivesTo study pregnancy outcomes in operated vs non-operated cases of congenital heart disease cases during pregnancy.Materials and methodsA total of 55 patients of congenital heart disease who delivered in the authors unit in last 10 years were taken in this retrospective study. These were divided into two groups Group 1:29 (52.7%) patient who had no cardiac surgery and Group 2: 26(47.2%) who had cardiac surgery to correct their cardiac defect before pregnancy. All patients were evaluated for cardiac complications and outcome during pregnancy. Obstetric complications, mode of delivery and fetal outcome was compared in the two groups using statistical analysis.ResultThe commonest lesion was atrial septal defect (ASD) seen in 22(40%) patients followed by ventricular septal defect (VSD) in 16(29%) .Congenital valvular disease 8(14.5%) and patent ductus arteriosus in 4(7.2%) cases. The mean age was 25.9 ± 3.15 years in Group 1 and 26.3 ± 4.53 years in Group 2. The baseline characteristics were similar in the two groups. There was no difference in cardiac complications, NYHA deterioration and need of cardiac drugs in the two groups. Obstetric complications and mode of delivery were also similar in the two groups.Mean birth weight was 2516.65 ± 514.04 gm in Group 1 and 2683.00 ± 366.00 gm in Group 2 and was similar. APGAR < 8, stillbirth rate and other neonatal complications were also similar in two groups.ConclusionThe maternal and fetal outcome was excellent in patients with congenital heart disease and was similar in unoperated and operated cases.     

Fetal cardiovascular morphology of truncus arteriosus with or without truncal valve insufficiency in the rat

BACKGROUND. Recent advances in fetal echocardiography have necessitated further study on fetal in situ cardiovascular morphology of truncus arteriosus and the effects of truncal valve insufficiency. METHODS AND RESULTS. We studied 55 fetal rats with truncus arteriosus among 300 fetuses from 40 virgin females treated with 200 mg fertilysin on the 10th day of pregnancy. After rapid whole-body freezing on the 21st day, the fetuses were studied by means of serial cross-sectional photographs of the frozen thorax. Thirty-five fetuses with a normal heart treated with fertilysin served as controls. Truncus arteriosus was characterized by a large ventricular septal defect, a solitary artery (truncus arteriosus) overriding the ventricular septum, the right and left pulmonary arteries originating from the truncus arteriosus with or without a common trunk (main pulmonary artery), and absent ductus arteriosus. Fetuses with truncal valve insufficiency had thick truncal valves, a large truncus arteriosus, and large ventricles. The subgroup of 12 fetuses with a large truncus (truncal diameter greater than 160% of the ascending aorta diameter in the controls) showed significantly greater values for right ventricular volume (200% of control) and mass (120% of control), left ventricular volume (170% of control) and mass (110% of control), right (120% of control) and left (110% of control) atrial volume, and pericardial fluid (140% of control) than the controls. These changes were less prominent and ventricular volumes were not increased in the remaining subgroup with a truncal diameter of 160% or less of aorta diameter in the controls. CONCLUSIONS. In fetal truncus arteriosus, truncal valve insufficiency was associated with increased ventricular volume load and incomplete cardiac compensation in rats.