Evaluation of cardiac findings using speckle tracking in fetuses with hemoglobin Bart's disease.

OBJECTIVE: Hemoglobin (Hb) Bart's disease is a severe manifestation of alpha thalassemia, resulting in fetal tissue hypoxia and severe anemia. There is limited research available on assessing fetal speckle tracking analysis as a response to fetal anemia caused by Hb Bart's disease and its utility as a sonographic predictor for Hb Bart's disease. This study aimed to assess the diagnostic performance of fetal cardiac parameters derived from speckle tracking echocardiography for distinguishing between affected and unaffected fetuses in pregnancies at risk of Hb Bart's disease during the 17-24 gestational weeks. METHODS: A total of 115 pregnant women at risk for fetal Hb Bart's disease, who underwent either amniocentesis or cordocentesis at Siriraj Hospital, Bangkok Thailand, were included. Speckle tracking analysis was performed on the 4-chamber view (4CV) of the fetal heart, assessing heart size, shape, ventricular contractility, and left ventricular function prior to invasive prenatal testing. Logistic regression analysis determined significant cardiac predictors and calculated the probability of a fetus having Hb Bart's disease. RESULTS: Among the cohort, 38 fetuses (33%) were diagnosed with Hb Bart's disease, and 9 cases (7.8%) exhibited frank hydropic signs. In comparison to the control group, affected fetuses displayed a notable enlargement of the 4CV and a more globular shape specifically in the right ventricular chamber. Additionally, there were significant differences in the left global and longitudinal contractility between affected and unaffected fetuses. However, at mid-gestation, no significant distinctions were observed in terms of transverse contractility and left ventricular function between the two groups. Based on logistic regression analysis, combined cardiac parameters derived from speckle tracking analysis as a function of head circumference, could differentiate non-hydropic fetuses with Hb Bart's disease from unaffected fetuses, achieving a maximum sensitivity of 100%, specificity of 98.7%, and overall accuracy of 99.06%. CONCLUSIONS: Speckle tracking echocardiography has the potential to accurately identify early fetal heart changes in individuals at risk of developing Bart's anemia during the second trimester. This not only offers a novel predictive marker for Hb Bart's disease but also helps address the question of the underlying mechanisms of heart failure associated with anemia. This article is protected by copyright. All rights reserved.

Improved detection of coarctation of the aorta using speckle-tracking analysis of fetal heart on last examination prior to delivery.

OBJECTIVE: The false-positive rate for prenatal diagnosis of coarctation of the aorta (FP-CoA) commonly exceeds 50%, with an accurate detection rate of < 50%. This study was conducted to determine if the sensitivity for prenatal detection of true CoA and the FP-CoA rate could be improved by evaluating the fetal epicardial size and shape in the four-chamber view (4CV) and the endocardial right (RV) and left (LV) ventricular size, shape and contractility. METHODS: We analyzed retrospectively Digital Imaging and Communications in Medicine (DICOM) clips of the 4CV from the last examination prior to delivery in a series of 108 fetuses with CoA suspected prenatally by pediatric cardiologists using traditional diagnostic criteria. Postnatal evaluation distinguished those fetuses which subsequently required CoA surgery (true positives; true CoA) from those that were FP-CoA. Postnatal cardiac abnormalities were identified for each group. For the prenatal evaluation, we measured the 4CV end-diastolic epicardial area, circumference, length, width and global sphericity index. Speckle-tracking analysis was used to compute the endocardial RV and LV end-diastolic area, length, 24-segment sphericity index, 24-segment transverse width and the following functional parameters: fractional area change; global longitudinal, free-wall and septal-wall strain; basal-apical-length, basal free-wall and basal septal-wall fractional shortening; septal-wall annular plane systolic excursion; 24-segment transverse-width fractional shortening; and LV end-diastolic and end-systolic volumes, stroke volume, cardiac output and ejection fraction. In addition, the RV/LV end-diastolic area ratio was computed. Using a control group of 200 normal fetuses, the mean and SD for each of the above cardiac measurements was used to compute the Z-scores for each measurement in each of the 108 study fetuses. Logistic regression analysis was then performed on the Z-score values to identify variables that separated the true CoA group from the FP-CoA group. RESULTS: Of the 108 study fetuses, 54 were confirmed postnatally to have true CoA and 54 were FP-CoA. Right/left area disproportion > 90th centile was present in 80% (n = 43) of the true-CoA fetuses and 76% (n = 41) of the FP-CoA fetuses. Fetuses with true CoA had a significantly greater number of associated cardiac abnormalities (93%, n = 50) compared with the FP-CoA fetuses (61%, n = 33) (P < 0.001). The most common associated malformations were bicuspid aortic valve (true CoA, 46% (n = 25) vs FP-CoA, 22% (n = 12); P < 0.01), aortic arch hypoplasia (true CoA, 31% (n = 17) vs FP-CoA, 11% (n = 6); P < 0.01), ventricular septal defect (true CoA, 33% (n = 18) vs FP-CoA, 11% (n = 6); P < 0.05) and mitral valve abnormality (true CoA, 30% (n = 16) vs FP-CoA, 4% (n = 2); P < 0.01). Logistic regression analysis identified 28 variables that correctly identified 96% (52/54) of the fetuses with true CoA, with a false-positive rate of 4% (2/54) and a false-negative rate of 4% (2/54). These variables included the epicardial size in the 4CV, size and shape of RV and LV, and abnormal contractility of RV and LV. The area under the receiver-operating-characteristics curve was 0.98 (SE, 0.023; 95% CI, 0.84-1). There was no significant difference in the percent of fetuses with RV/LV area disproportion between those with CoA and those that were FP-CoA. CONCLUSIONS: Speckle-tracking analysis of multiple ventricular measurements may be helpful to refine the diagnosis in fetuses that are suspected to have CoA prenatally. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Quantitative evaluation of fetal right and left ventricular fractional area change using speckle-tracking technology.

OBJECTIVES: To measure, using speckle-tracking technology, the fractional area change (FAC) of the right and left ventricles in normal fetal hearts between 20 and 40 weeks of gestation. METHODS: The four-chamber view of the fetal heart was obtained in 200 normal fetuses between 20 and 40 weeks of gestation. FAC was computed from the ventricular areas (((end-diastolic area - end-systolic area)/end-diastolic area) × 100) for the right and left ventricles, and regressed against seven independent biometric and age variables. FAC was correlated with longitudinal fractional shortening (LFS) (((end-diastolic longitudinal length - end-systolic longitudinal length)/end-diastolic longitudinal length) × 100) obtained from the mid-ventricular basal-apical lengths of the right and left ventricular chambers and with transverse fractional shortening (TFS) (((end-diastolic transverse length - end-systolic transverse length)/end-diastolic transverse length) × 100) from three transverse positions (base, mid, apical) located within each ventricular chamber. To evaluate potential clinical utility, FAC, LFS and TFS results were examined in nine fetuses with a congenital heart defect (CHD). RESULTS: Regression analysis demonstrated significant associations between FAC and the independent biometric and age variables (R2  = 0.13-0.15). FAC was significantly correlated with LFS (R2  = 0.18-0.28) and TFS (R2  = 0.13-0.33). Examination of the fetuses with CHD revealed that six of the nine had abnormal FAC Z-score values for the index pathological ventricle. When abnormal LFS and TFS values were compared to the FAC in these fetuses, the FAC was either abnormally low or normal. CONCLUSIONS: This study reports results from measuring the FAC of the right and left ventricles, and demonstrates a correlation with LFS and TFS. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Prenatally Diagnosed Ventricular Inversion, Restrictive Ventricular Septal Defect, Pulmonary Stenosis, Hypertensive Left Ventricle and Double Outlet Right Ventricle: Case Report and Literature Review.

We herein describe, for the first time, the fetal presentation of a case of ventricular inversion, restrictive ventricular septal defect, pulmonary stenosis, hypertensive left ventricle and double outlet right ventricle at 34 weeks of gestational age. Postnatal echocardiography confirmed the prenatal diagnosis. The patient was subsequently successfully palliated with a left ventricle to pulmonary artery conduit. This report illustrates the importance of detailed fetal echocardiography to ensure appropriate delivery and neonatal management, and to optimize outcome.

24-segment sphericity index: a new technique to evaluate fetal cardiac diastolic shape.

OBJECTIVE: Because of parallel circulation in the fetus and the differential effect that various disease states may have on the shape of the right and left ventricles, this study was conducted to evaluate the sphericity index (SI) of 24 transverse segments distributed from the base to the apex of each of the ventricular chambers. METHODS: Two hundred control fetuses were examined between 20 and 40 weeks of gestation. The displacement of the ventricular endocardium during the cardiac cycle was computed using offline speckle-tracking software. From the ASCII output of the analysis, we analyzed 24 end-diastolic transverse segments, distributed from the base to the apex of each ventricle, as well as the end-diastolic mid-basal-apical length. The SI was computed for each of the 24 segments by dividing the mid-basal-apical length by the transverse length for each segment. Regression analysis was performed against biometric measurements and gestational age according to last menstrual period and ultrasound. Eight fetuses, in which the four-chamber view appeared subjectively to demonstrate chamber disproportion, were evaluated as examples to demonstrate the utility of this technology. RESULTS: The SI for each segment was independent of gestational age and fetal biometric measurements. The SI of the right ventricle was significantly (P < 0.001) lower than that of the left ventricle for segments 1-18, suggesting that the right ventricle was more globular in shape than was the left ventricle at the base, mid and a portion of the apical segments of the chamber. Fetuses with various cardiac structural abnormalities and abnormal fetal growth had abnormal SI values that reflected either a more globular or a more flattened ventricular chamber. CONCLUSION: Determination of SI for each of 24 segments of the fetal right and left ventricles provides a comprehensive method to examine the shape of the ventricular chambers. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Prenatal diagnosis of cor triatriatum sinister in association with hypoplastic left heart syndrome.

Cor triatriatum sinister is a rare congenital heart defect related to incomplete common pulmonary vein resorption into the primitive heart. This lesion usually presents with pulmonary venous obstruction and can occur in association with left-sided obstructive lesions such as hypoplastic left heart syndrome (HLHS). In the context of HLHS, the presence of cor triatriatum sinister carries additional surgical and prognostic implications. Fetal diagnosis can enable appropriate counseling of the family and guide optimal peri- and postnatal management. The reported case represents the first prenatal description of cor triatriatum sinister in association with HLHS.

Hypoplastic left heart syndrome: attitudes among pediatric residents and nurses towards fetal and neonatal management.

OBJECTIVE: To determine the attitudes of pediatric residents and nurses towards fetal/neonatal management of hypoplastic left heart syndrome (HLHS), and their basis. METHODS: Pediatric residents and nurses from three cardiac centers completed a survey with hypothetical scenarios in which their own fetuses or newborns had HLHS. While Institution A performs many HLHS surgeries, Institution C performs very few. RESULTS: A total of 43% of residents and 50% of nurses would terminate an affected pregnancy. More experience (4 to 7 years, p = 0.04; >7 years, p = 0.05) and employment at institution C (p = 0.04) predicted termination. Expected better quality of life (QOL) (p = 0.02) and five-year survival >50% (p = 0.06) predicted not terminating. Postnatally, 48% of residents and 68% of nurses would choose, or seriously consider, comfort care. Marriage (p = 0.04) and more experience (4 to 7 years, p = 0.04; >7 years, p = 0.02) predicted choosing comfort care. Asian/Pacific Islander descent (p = 0.01) and expected 5-year survival >50% (p = 0.02) predicted choosing surgery. CONCLUSIONS: Approximately one-half of the pediatric residents and nurses surveyed would choose termination of pregnancy or seriously consider declining neonatal surgery, if their own fetus or infant had HLHS. These attitudes reflect perceptions of long-term QOL and survival. These attitudes may be of interest to caregivers who care for HLHS patients.

Chest radiography and the evaluation of the neonate for congenital heart disease.

We assessed the utility of the chest film as a screening tool for term and preterm neonates with suspected structural heart disease. Three independent observers, blinded to patient diagnosis, retrospectively evaluated the initial chest film for each of 128 consecutive neonates with suspected heart disease (gestational age, 23-42 weeks; birth weight, 500-4,621 g) who had undergone both chest radiography and echocardiography. These evaluations were subsequently compared with the results of the respective echocardiograms, read by board-certified pediatric cardiologists blinded to the chest film and chest film interpretation. Kappa statistics demonstrated moderate correlation (0.42-0.48) among different observers in their interpretation of the chest films but poor correlation (0.15-0.34) between chest radiography and echocardiography. The chest film had a low sensitivity for structural heart disease (26-59%), with a negative predictive value of 46-52%. Among neonates less than 2 kg or younger than 35 weeks of gestation, the chest film had still lower sensitivity for detecting heart disease. Despite agreement among observers in chest film interpretation, the chest film does not function as a screening test for neonates with suspected heart disease, particularly in small or premature neonates. In neonates with suspected heart disease, echocardiography should be considered, even in patients with chest films that do not suggest congenital heart disease.

Prenatal screening for congenital heart disease using real-time three-dimensional echocardiography and a novel 'sweep volume' acquisition technique.

OBJECTIVES: Conventional prenatal screening for congenital heart disease (CHD) involves a time-consuming and highly operator-dependent acquisition of the four-chamber view and outflow tracts. By acquiring the entire fetal heart instantaneously as a single volume, real-time three-dimensional echocardiography (RT3DE) may facilitate fetal cardiac screening. METHODS: Four reviewers, each experienced with fetal cardiac imaging, blindly and independently evaluated a single cardiac volume from each of 18 fetuses (11 normal, seven with CHD). Two-dimensional echocardiography served as the gold standard. Three-dimensional evaluation of each fetus included a series of volume acquisitions lasting 2-6 s each. A 'sweep volume' technique was developed to fit larger hearts into a single non-gated volume. RESULTS: RT3DE had a high sensitivity for detecting CHD (93%), with only a single case being missed by two observers. Specificity for CHD was low (45%), with a high rate of 'cannot determine' responses and false positive artifacts. CONCLUSIONS: These preliminary results suggest that RT3DE has the potential to function as a screening tool for fetal heart disease. However, artifacts must be recognized and minimized, resolution must improve, and substantial training will be necessary prior to widespread clinical use.

Advances in fetal cardiac imaging.

During the past 25 years, two-dimensional imaging of the fetal heart has evolved into a sophisticated and widely practiced clinical tool, but most heart disease still goes undetected until sometime after birth, despite routine fetal ultrasound evaluations. Over the next 25 years, tremendous advances in fetal cardiac imaging, including three-dimensional imaging, promise to revolutionize both the prenatal detection and diagnosis of congenital heart disease. Image resolution continues to improve year after year, allowing earlier (10-15 week) visualization of the fetal heart, as well as the detection of subtle valvar abnormalities that may progress to serious forms of ventricular hypoplasia at term. However, fetal cardiac imaging remains constrained by limited sonographic windows. To improve the prenatal detection of congenital heart disease, outflow tracts are increasingly included along with the routine screening four-chamber view. However, while the four-chamber view resides within a single plane, lending itself to tomographic evaluation with two-dimensional ultrasound, the outflow tracts (and most forms of congenital heart disease) do not reside within a single plane. For these and other reasons, three-dimensional imaging of the fetal heart ultimately may improve the detection of outflow tract abnormalities, and facilitate comprehension of complex forms of congenital heart disease. Finally, other imaging modalities, including but not limited to Doppler tissue imaging and magnetic resonance imaging, continue to evolve and to complement two- and three-dimensional sonographic imaging of the fetal heart. As a result of these ongoing advances in the prenatal detection and assessment of congenital heart disease, these are exciting and glorious times for the field of fetal cardiac imaging.

The 'spin' technique: a new method for examination of the fetal outflow tracts using three-dimensional ultrasound.

BACKGROUND AND OBJECTIVE: The prenatal detection of congenital heart defects remains one of the most difficult challenges for the sonologist/sonographer when performing the second- or third-trimester screening examination. The four-chamber view has been used for a number of years as the primary screening image for detection of heart defects, but the inclusion of the right and left outflow tracts increases the detection of cardiac malformations. One of the difficulties, however, is obtaining and interpreting two-dimensional images of the outflow tracts. This paper reviews a new technique using three-dimensional (3D) multiplanar imaging that allows the examiner to identify the outflow tracts within a few minutes of acquiring the 3D volume dataset by rotating the volume dataset around the x- and y-axes. METHODS: 3D multiplanar imaging of the fetal heart using static 3D or spatio-temporal image correlation (STIC) imaging allows the examiner to obtain a volume of data that can be manipulated along the x- and y-axes using reference points from the four-chamber view, five-chamber view, three-vessel view at the level of the bifurcation of the pulmonary arteries, and three-vessel view at the level of the transverse aortic arch and trachea. RESULTS: The full length of the main pulmonary artery, ductus arteriosus, aortic arch and superior vena cava could be identified easily in the normal fetus by rotating the volume dataset along the x- and y-axes. The vessels were identified using the four-chamber view, the five-chamber view, and the two three-vessel views. The technique was useful in identification of d-transposition of the great vessels and evaluation of the outflow tracts in hypoplastic left heart syndrome. CONCLUSION: 3D multiplanar evaluation of the fetal heart allows the examiner to identify the outflow tracts using a simple technique that requires only rotation around x- and y-axes from reference images obtained in a transverse sweep through the fetal chest.

Spatio-temporal image correlation (STIC): new technology for evaluation of the fetal heart.

Spatio-temporal image correlation (STIC) is a new approach for clinical assessment of the fetal heart. It offers an easy to use technique to acquire data from the fetal heart and to aid in visualization with both two-dimensional and three-dimensional (3D) cine sequences. The acquisition is performed in two steps: first, images are acquired by a single, automatic volume sweep. Second, the system analyzes the image data according to their spatial and temporal domain and processes an online dynamic 3D image sequence that is displayed in a multiplanar reformatted cross-sectional display and/or a surface rendered display. The examiner can navigate within the heart, re-slice, and produce all of the standard image planes necessary for a comprehensive diagnosis. The advantages of STIC for use in evaluation of the fetal heart are as follows: the technique delivers a temporal resolution which corresponds to a B-mode frame rate of approximately 80 frames/s; it provides the examiner with an unlimited number of images for review; it allows for correlation between image planes that are perpendicular to the main image acquisition plane; it may shorten the evaluation time when complex heart defects are suspected; it enables the reconstruction of a 3D rendered image that contains depth and volume which may provide additional information that is not available from the thin multiplanar image slices (e.g. for pulmonary veins, septal thickness); it lends itself to storage and review of volume data by the examiner or by experts at a remote site; it provides the examiner with the ability to review all images in a looped cine sequence.

Ambiguity and mistakes in the pediatric cardiology vernacular.

The pediatric cardiology vernacular is replete with ambiguous and erroneous terminology. This article discusses several examples in a plea to bring the accuracy and precision of the scientific method to the clinical pediatric cardiology vernacular.

Reptilian echocardiography: insights into ontogeny and phylogeny?

We describe the echocardiographic findings in a large reptile-the carpet python. If ontogeny recapitulates phylogeny, the study of reptilian hearts may provide insights into human cardiac development. In addition, the reptilian heart has unique structural and physiological adaptations that may broaden our perspective on evolutionary cardiac adaptation.

Neonatal euthanasia: moral considerations and criminal liability.

Despite tremendous advances in medical care for critically ill newborn infants, caregivers in neonatal intensive care units still struggle with how to approach those patients whose prognoses appear to be the most grim, and whose treatments appear to be the most futile. Although the practice of passive neonatal euthanasia, from a moral perspective, has been widely (albeit quietly) condoned, those clinicians and families involved in such cases may still be found legally guilty of child abuse or even manslaughter. Passive neonatal euthanasia remains both a moral dilemma and a legal ambiguity. Even the definition of passive euthanasia remains unclear. This manuscript reviews the basic moral and legal considerations raised by the current practice of neonatal euthanasia, and examines the formal position statements of the American Medical Association and the American Academy of Pediatrics. The paper concludes by emphasising the need, at least in the United States, to clarify the legal status of this relatively common medical practice.

Balloon dilatation of native aortic coarctation in two patients with Turner syndrome.

We report successful balloon dilatation of native coarctation of the aorta in two patients with Turner syndrome, with favorable endovascular remodeling demonstrated by intravascular ultrasound imaging and angiography 1 year after the procedure.