Cardiac function in congenital diaphragmatic hernia.

Cardiac function is known to play critical role in the pathophysiological progression and ultimate clinical outcome of patients with congenital diaphragmatic hernia (CDH). While often anatomically normal, the fetal and neonatal heart in CDH can suffer from both right and left ventricular dysfunction. Here we explore the abnormal fetal heart, early postnatal right and left ventricular dysfunction, the interplay between cardiac dysfunction and pulmonary hypertension, evaluation and echocardiographic assessment of the heart, and therapeutic strategies for managing and supporting the pathophysiologic heart and CDH. Further, we take a common clinical scenario and provide clinically relevant guidance for the diagnosis and management of this complex process.

Prediction of fetal growth restriction and small for gestational age by ultrasound cardiac parameters.

OBJECTIVE: Prediction of fetal growth restriction (FGR) and small of gestational age (SGA) infants by using various ultrasound cardiac parameters in a logistic regression model. METHODS: In this retrospective study we obtained standardized ultrasound images of 357 fetuses between the 20th and 39th week of gestation, 99 of these fetuses were between the 3rd and 10th growth percentile, 61 smaller than 3rd percentile and 197- appropriate for gestational age over the 10th percentile (control group). Several cardiac parameters were studied. The cardiothoracic ratio and sphericity of the ventricles was calculated. A binary logistic regression model was developed for prediction of growth restriction using the cardiac and biometric parameters. RESULTS: There were noticeable differences between the control and study group in the sphericity of the right ventricle (p = 0.000), left and right longitudinal ventricle length (pright = 0.000, pleft = 0.000), left ventricle transverse length (p = 0.000), heart diameter (p = 0.002), heart circumference (p = 0.000), heart area (p = 0.000), and thoracic diameter limited by the ribs (p = 0.002). There was no difference of the cardiothoracic ratio between groups. The logistic regression model achieved a prediction rate of 79.4 % with a sensitivity of 74.5 % and specificity of 83.2 %. CONCLUSION: The heart of growth restricted infants is characterized by a more globular right ventricle, shorter ventricle length and smaller thorax diameter. These parameters could improve prediction of FGR and SGA.

Review of speckle tracking analysis to measure the size, shape, and contractility of the fetal heart in fetuses with congenital heart defects.

Evaluation of the fetal heart involves two approaches. The first describes a screening protocol in which the heart is imaged in transverse planes that includes the four-chamber view (4CV), left and right outflow tracts, and the 3-vessel-tracheal view. The second approach is a fetal echocardiogram that requires additional cardiac images as well as evaluating ventricular function using diagnostic tools such as M-mode and pulsed Doppler ultrasound. Speckle tracking analysis of the ventricular and atrial endocardium of the fetal heart has focused primarily on computing longitudinal global strain. However, the technology enabling this measurement to occur has recently been adapted to enable the clinician to obtain numerous additional measurements of the size, shape, and contractility of the ventricles and atrial chambers. By using the increased number of measurements derived from speckle tracking analysis, we have reported the ability to screen for tetralogy of Fallot, D-transposition of the great arteries (D-TGA), and coarctation of the aorta by only imaging the 4CV. In addition, we have found that measurements derived from speckle tracking analysis of the ventricular and atrial chambers can be used to compute the risk for emergent neonatal balloon atrial septostomy in fetuses with D-TGA. The purpose of this review is to consolidate our experience in one source to provide perspective on the benefits of speckle tracking analysis to measure the size, shape, and contractility of the ventricles and atria imaged in the 4CV in fetuses with congenital heart defects.

Ultrasound parameters of arteries and heart in normal fetuses.

BACKGROUND: Currently, no normal ultrasound data of the fetuses during the 20-40 gestation have been obtained for references of fetal growth and development. If such ultrasound data existed for prenatal diagnosis of possible diseases and abnormalities, neonates would be able to get timely treatment immediately after birth. This study was thus performed to obtain ultrasound parameters of normal fetuses during the 20-40 week gestation and the distribution of ultrasound parameters with the gestational age for references of detecting potential fetal diseases and abnormalities. METHODS: Normal fetuses without any abnormalities were enrolled, and the ultrasound parameters of the general biology, arteries, and aorta were measured and analyzed. RESULTS: 417 normal fetuses were enrolled. A significant (P < 0.05) negative correlation with the gestational age was detected in the peak systolic velocity/peak diastolic velocity (S/D), pulsatility index (PI) and resistance index (RI) of the umbilical artery (UA). A relatively stable relationship with the gestational age was detected in the fetal weight%, S/D, PI and RI of the middle cerebral artery (MCA), peak systolic velocity (PSV) and velocity time integral (VTI) of the intra-abdominal UA, fetal heart to chest ratio, mitral valve (MV)- and tricuspid valve (TV)-E/A peak flow velocity, aortic isthmic Z-score and displacement, distance between the brachiocephalic artery-left common carotid artery (BA-LCCA) and LCCA-left subclavian artery (LSA), Z-score of aorta, ascending aorta (AAO), pulmonary artery (PA), main pulmonary artery (MPA), and descending aorta (DAO). A significant (P < 0.05) positive correlation with the gestational age was detected in the fetal biological data, MCA PSV and VTI, free-UA PSV and VTI and cardio-thoracic ratio, cardiac parameters, ductus arteriosus (DA) and isthmus diameter, aortic parameters, PA and MPA diameter, MPA PSV and VTI, isthmus flow volume and velocity and PA flow volume, DA and BA parameters, and LCCA and LSA parameters (flow volume, PSV, and VTI). CONCLUSION: A certain correlation and distribution trend is detected in the ultrasound parameters of normal fetuses, and the ratios among different parameters remain relative stable. These findings can be used for determination of abnormal growth of the fetuses in prenatal ultrasound scan.

Prenatal diagnosis of criss-cross heart - case series and review of the literature.

OBJECTIVE: This study aimed to improve the accuracy of prenatal diagnosis by analyzing fetal echocardiographic features of criss-cross heart (CCH), to provide an effective basis for the development of management strategies and improve the prognosis of patients. METHODS: A retrospective analysis was performed on CCH cases diagnosed prenatally at our center between July 2016 and June 2022. Clinical data and prenatal fetal echocardiographic images were reviewed. Literature on prenatal diagnosis of CCH was searched from January 2000 to December 2023 in the PubMed database. RESULTS: Fourteen (0.03%) CCH cases were diagnosed from a database of fetal echocardiograms of 41354 cases at our center. The prenatal genetic testing results were normal in 10 cases and 4 cases didn't check. All cases underwent termination of pregnancy. All cases showed crossed ventricular inflow tracts and combined with other cardiac structural abnormalities. A total of eight articles containing 25 cases were found in the literature review and all cases were associated with other cardiac structural abnormalities. CONCLUSION: Prenatal echocardiography is the primary tool for fetal diagnosis of CCH. Continuous scanning helps avoid missing data and misdiagnosis.

How automated techniques ease functional assessment of the fetal heart: Applicability of two-dimensional speckle-tracking echocardiography for comprehensive analysis of global and segmental cardiac deformation using fetalHQ®.

BACKGROUND: Prenatal echocardiographic assessment of fetal cardiac function has become increasingly important. Fetal two-dimensional speckle-tracking echocardiography (2D-STE) allows the determination of global and segmental functional cardiac parameters. Prenatal diagnostics is relying increasingly on artificial intelligence, whose algorithms transform the way clinicians use ultrasound in their daily workflow. The purpose of this study was to demonstrate the feasibility of whether less experienced operators can handle and might benefit from an automated tool of 2D-STE in the clinical routine. METHODS: A total of 136 unselected, normal, singleton, second- and third-trimester fetuses with normofrequent heart rates were examined by targeted ultrasound. 2D-STE was performed separately by beginner and expert semiautomatically using a GE Voluson E10 (FetalHQ®, GE Healthcare, Chicago, IL). Several fetal cardiac parameters were calculated (end-diastolic diameter [ED], sphericity index [SI], global longitudinal strain [EndoGLS], fractional shortening [FS]) and assigned to gestational age (GA). Bland-Altman plots were used to test agreement between both operators. RESULTS: The mean maternal age was 33 years, and the mean maternal body mass index prior to pregnancy was 24.78 kg/m2. The GA ranged from 16.4 to 32.0 weeks (average 22.9 weeks). Averaged endoGLS value of the beginner was -18.57% ± 6.59 percentage points (pp) for the right and -19.58% ± 5.63 pp for the left ventricle, that of the expert -14.33% ± 4.88 pp and -16.37% ± 5.42 pp. With increasing GA, right ventricular endoGLS decreased slightly while the left ventricular was almost constant. The statistical analysis for endoGLS showed a Bland-Altman-Bias of -4.24 pp ± 8.06 pp for the right and -3.21 pp ± 7.11 pp for the left ventricle. The Bland-Altman-Bias of the ED in both ventricles in all analyzed segments ranged from -.49 mm ± 1.54 mm to -.10 mm ± 1.28 mm, that for FS from -.33 pp ± 11.82 pp to 3.91 pp ± 15.56 pp and that for SI from -.38 ± .68 to -.15 ± .45. CONCLUSIONS: Between both operators, our data indicated that 2D-STE analysis showed excellent agreement for cardiac morphometry parameters (ED and SI), and good agreement for cardiac function parameters (EndoGLS and FS). Due to its complexity, the application of fetal 2D-STE remains the domain of scientific-academic perinatal ultrasound and should be placed preferably in the hands of skilled operators. At present, from our perspective, an implementation into clinical practice "on-the-fly" cannot be recommended.

Utility of the novel fetal heart quantification (fetal HQ) technique in diagnosing ventricular interdependence and biventricular dysfunction in a case of prenatally diagnosed Uhl's anomaly.

Uhl's anomaly is characterized by complete or partial absence of right ventricular myocardium. We describe a case of prenatally diagnosed Uhl's anomaly with biventricular dysfunction which was quantified with speckle tracking echocardiography using a novel fetal heart quantification (fetal HQ) technique.

Fetal Cardiovascular MR Imaging.

Prenatal diagnosis of congenital heart disease allows for appropriate planning of delivery and an opportunity to inform families about the prognosis of the cardiac malformation. On occasion, prenatal therapies may be offered to improve perinatal outcomes. While ultrasound is the primary diagnostic method, advances have led to interest in fetal MRI for its potential to aid in clinical decision-making. This review explores technical innovations and the clinical utility of fetal cardiovascular magnetic resonance (CMR), highlighting its role in diagnosing and planning interventions for complex heart conditions. Future directions include the prediction of perinatal physiology and guidance of delivery planning.

Investigation on ultrasound images for detection of fetal congenital heart defects.

Congenital heart defects (CHD) are one of the serious problems that arise during pregnancy. Early CHD detection reduces death rates and morbidity but is hampered by the relatively low detection rates (i.e., 60%) of current screening technology. The detection rate could be increased by supplementing ultrasound imaging with fetal ultrasound image evaluation (FUSI) using deep learning techniques. As a result, the non-invasive foetal ultrasound image has clear potential in the diagnosis of CHD and should be considered in addition to foetal echocardiography. This review paper highlights cutting-edge technologies for detecting CHD using ultrasound images, which involve pre-processing, localization, segmentation, and classification. Existing technique of preprocessing includes spatial domain filter, non-linear mean filter, transform domain filter, and denoising methods based on Convolutional Neural Network (CNN); segmentation includes thresholding-based techniques, region growing-based techniques, edge detection techniques, Artificial Neural Network (ANN) based segmentation methods, non-deep learning approaches and deep learning approaches. The paper also suggests future research directions for improving current methodologies.

Automatic segmentation of 15 critical anatomical labels and measurements of cardiac axis and cardiothoracic ratio in fetal four chambers using nnU-NetV2.

BACKGROUND: Accurate segmentation of critical anatomical structures in fetal four-chamber view images is essential for the early detection of congenital heart defects. Current prenatal screening methods rely on manual measurements, which are time-consuming and prone to inter-observer variability. This study develops an AI-based model using the state-of-the-art nnU-NetV2 architecture for automatic segmentation and measurement of key anatomical structures in fetal four-chamber view images. METHODS: A dataset, consisting of 1,083 high-quality fetal four-chamber view images, was annotated with 15 critical anatomical labels and divided into training/validation (867 images) and test (216 images) sets. An AI-based model using the nnU-NetV2 architecture was trained on the annotated images and evaluated using the mean Dice coefficient (mDice) and mean intersection over union (mIoU) metrics. The model's performance in automatically computing the cardiac axis (CAx) and cardiothoracic ratio (CTR) was compared with measurements from sonographers with varying levels of experience. RESULTS: The AI-based model achieved a mDice coefficient of 87.11% and an mIoU of 77.68% for the segmentation of critical anatomical structures. The model's automated CAx and CTR measurements showed strong agreement with those of experienced sonographers, with respective intraclass correlation coefficients (ICCs) of 0.83 and 0.81. Bland-Altman analysis further confirmed the high agreement between the model and experienced sonographers. CONCLUSION: We developed an AI-based model using the nnU-NetV2 architecture for accurate segmentation and automated measurement of critical anatomical structures in fetal four-chamber view images. Our model demonstrated high segmentation accuracy and strong agreement with experienced sonographers in computing clinically relevant parameters. This approach has the potential to improve the efficiency and reliability of prenatal cardiac screening, ultimately contributing to the early detection of congenital heart defects.

Four-Dimensional Ultrasound Imaging of Spatial Relationship of Great Arteries in Fetuses With Transposition of Great Arteries.

The great artery relationship in transposition of great arteries (TGA) is based on the degree of resorption and growth of subsemilunar conus. In transposition of great arteries, the spatial relationship of the great arteries can be variable. Commonly, the aorta will be related right and anterior to pulmonary artery (D-TGA). Less commonly, the aorta can be related left and anterior (SDL-TGA), side-by-side, and antero-posterior to pulmonary artery. Four fetuses with concordant atrioventricular and discordant ventriculoarterial connection with various great arteries relationship were presented in this imaging essay.

Clinical implementation of first trimester screening for congenital heart defects.

OBJECTIVE: To examine the feasibility and performance of implementing a standardized fetal cardiac scan at the time of a routine first-trimester ultrasound scan. METHOD: A retrospective, single-center study in an unselected population between March 2021 and July 2022. A standardized cardiac scan protocol consisting of a four-chamber and 3-vessel trachea view with color Doppler was implemented as part of the routine first-trimester scan. Sonographers were asked to categorize the fetal heart anatomy. Data were stratified into two groups based on the possibility of evaluating the fetal heart. The influence of maternal and fetal characteristics and the detection of major congenital heart disease were investigated. RESULTS: A total of 5083 fetuses were included. The fetal heart evaluation was completed in 84.9%. The proportion of successful scans increased throughout the study period from 76% in the first month to 92% in the last month. High maternal body mass index and early gestational age at scan significantly decreased the feasibility. The first-trimester detection of major congenital heart defects was 7/16, of which four cases were identified by the cardiac scan protocol with no false-positive cases. CONCLUSION: First-trimester evaluation of the fetal heart by a standardized scan protocol is feasible to implement in daily practice. It can contribute to the earlier detection of congenital heart defects at a very low false positive rate.

Prenatal diagnosis of interrupted aortic arch using high-definition flow render mode and spatiotemporal image correlation.

OBJECTIVES: To evaluate the clinical utility of two dimensional (2D) ultrasound combined with spatiotemporal image correlation (STIC) in diagnosing interrupted aortic arch (IAA) in fetal life. METHODS: A total of 53 cases of fetal IAA were diagnosed using 2D ultrasound combined with STIC, and 53 normal fetuses of the same gestational week were selected. These cases were retrospectively analyzed to assess the utility of employing 2D ultrasound combined with STIC in the diagnosis of IAA. RESULTS: 2D ultrasound combined with STIC detected 22 cases of type A IAA, 24 cases of type B IAA, and seven cases of type C IAA. Furthermore, combining 2D ultrasound with STIC enabled dynamic visualization of the IAA, aiding in prenatal diagnosis. The diagnostic coincidence rate of IAA was found to be higher in the HD-flow combined with STIC than that in the 2D combined with HD-flow. CONCLUSION: HD-flow combined with STIC can assist in diagnosing fetal IAA, and this technique has important clinical value.

Transforming congenital heart disease management: Advances in fetal cardiac interventions.

This review addresses the transformative advancements in fetal cardiac interventions (FCI) for congenital heart diseases (CHD), with a particular focus on aortic stenosis with evolving hypoplastic left heart syndrome, pulmonary atresia with an intact ventricular septum, and HLHS with an intact atrial septum (HLHS-IAS). We outline the specific FCI techniques employed, the refined criteria for selecting appropriate fetal and maternal candidates, and the promising yet varied outcomes associated with these procedures. Procedural strategies and clinical decision-making are examined as we take into account the fetal pathophysiology and the benefits and risks of early intervention. We highlight the role of multidisciplinary teams in improving technical success and managing immediate procedural complications, which have led to significant improvements in procedural outcomes. Additionally, the review discusses the long-term outcomes, challenges, and future research directions in FCI, emphasizing the necessity for continuous innovation and collaboration across specialties to advance the management of CHD. The integration of new technologies and research findings holds the promise of further enhancing FCI success rates and patient outcomes.

Enhancing parental understanding of congenital heart disease through personalized prenatal counseling with 3D printed hearts.

OBJECTIVES: In addition to a correct prenatal diagnosis of congenital heart disease (CHD), comprehensive parental counseling is crucial to ensure that parents are well-informed about the condition of the fetus. This study aims to investigate whether there is a significant difference in the information acquired by parents through traditional counseling, utilizing 2-dimensional (2D) illustrations and images, compared to an advanced approach utilizing personalized three-dimensional (3D) printed models of the fetal heart developed from 3D ultrasound imaging. METHODS: This study, designed as a pilot randomized control trial, enrolled pregnant women with gestational ages greater than 18 weeks, whose fetuses were diagnosed with CHD and referred to our center between November, 2020 and June, 2021. Two groups of patients were included in the study. The first group received standard medical counseling with 2D images and illustrations, while the second group underwent advanced counseling with 3D-printed patient-specific heart models. Both groups were then required to complete the same survey in which the knowledge of the CHD was investigated. The 3D models were created from 3D ultrasound imaging and printed using resin materials in both 1:1 and 5:1 scale. RESULTS: A comparison of the scores obtained from the two groups revealed that 3D visualization of the fetus's heart has the potential to increase parental knowledge about CHD and the required surgical procedures. Furthermore, all couples expressed interest in receiving a 1:1 scale model of their baby's heart. CONCLUSION: Personalized prenatal counseling with 3D-ultrasound-based heart models positively impacts parents' understanding of CHD. The use of 3D models provides a more comprehensive and accessible representation of the condition, contributing to an increased knowledge gain, and potentially helping to support informed decisions regarding their child's care.

Evaluating fetal tricuspid and mitral annular plane systolic excursion (TAPSE and MAPSE) using spatiotemporal image correlation (STIC) M-mode.

OBJECTIVES: This study aimed to evaluate the feasibility and reliability of tricuspid and mitral annular plane systolic excursion measurements (TAPSE and MAPSE) applying M-mode spatiotemporal image correlation (STIC) technology in low-risk pregnancies. METHODS: An initial retrospective pilot study was carried out to assess repeatability, followed by a larger mixed cross-sectional and prospective longitudinal study, both evaluating low-risk singleton pregnancies between 22+6 and 39+1 weeks. As only annuli capture was necessary, STIC acquisition parameters were set to the minimum volume angle of 15° and acquisition time of 7.5 s. RESULTS: A total of 330 volumes were analysed offline applying STIC M-mode. Acquisition rates were 96.9 % for TAPSE and 93.7 % MAPSE in the pilot study (n=32) and 98.0 % for both in the longitudinal study (n=102). Both study designs revealed good repeatability for both sides of the heart, with higher intraclass correlation coefficients (ICCs) for TAPSE (0.84-0.94) compared to MAPSE (0.80-0.88). Good repeatability was demonstrated for both sides of the heart, more so for TAPSE than MAPSE, with ICCs for TAPSE 0.84-0.91 and MAPSE 0.75-0.76, comparable to prior ICCs for STIC repeatability. CONCLUSIONS: Modified STIC acquisition settings specifically tailored for capturing the longitudinal annular displacement may improve STIC TAPSE and MAPSE acquisition rates, optimising image quality for precise measurement and potentially bringing these modalities closer to clinical application.