Ebstein anomaly: 3D virtual and physical models from obstetrical ultrasound data.

3D virtual and physical models from ultrasound scan data allow a 3D spatial view of congenital heart anomalies, interactive discussion among a multidisciplinary team, and improved parental counseling. To the best of our knowledge, this is the first description of 3D physical and virtual models of a fetal Ebstein anomaly.

Prenatal Cardiac Ultrasound Markers of Outcomes in Ebstein's Anomaly: An International Multicenter Case Series.

This study aimed to analyze prenatal cardiac ultrasound markers of outcome in fetuses with Ebstein's anomaly (EA). From a retrospective database, 35 fetuses diagnosed with EA at fetal medicine centers in Brazil, Italy, and Poland were retrieved. The primary outcome was perinatal mortality. We analyzed prenatal cardiac ultrasound markers of outcomes and perinatal follow-up. Gestational age at diagnosis, extracardiac fetal anomalies, spontaneous fetal demise, and gestational age at each event were recorded. In postnatal survivors, data on cardiac surgery and short-term postoperative outcomes were collected. Our study included a cohort of 35 fetuses with EA (mean gestational age of 29.4 weeks), in which 6 fetuses were excluded due to termination of pregnancy (3), pregnancy still ongoing (2), and missed follow-up (1). Of the remaining 29 cases, severe tricuspid regurgitation and absence of anterograde pulmonary flow (pulmonary atresia) were observed in 88%. Significant cardiomegaly accounts for 58% of these data with a mean cardiothoracic ratio of 0.59. The cardiovascular profile (CVS) score ≤ 6 in six patients with one survival (4 fetal deaths, one stillbirth, and one survival). All fetuses with CVS score of 5 had intrauterine demise. Seventeen fetuses were born alive (53.1% of 29 cases). Of the remaining fetuses, one (1%) fetal was a stillbirth, six (20%) fetuses were neonatal deaths, and five (17%) fetuses were fetal deaths. Of the nineteen patients who underwent surgery to correct the cardiac defect, 17 survived after surgery. Among the survivors, biventricular cardiac repair was performed using the cone technique (da Silva's approach) in the majority of cases. We observed 2 abnormal karyotypes among in the remaining 29 fetuses. One of the patients with abnormal karyotype was a fetus with ascites and large for gestational age. The other patient with abnormal karyotype underwent cardiac surgery and progressed to neonatal death. Nine patients (25%) had extracardiac anomalies (genitourinary anomalies and single umbilical artery), being that 2 of them are alive and 4 died (2 had fetal and 2 neonatal death). Fetal EA is associated with high mortality. The most common prenatal marker associated with non-survival was CVP score ≤ 6. Fetuses that survived and underwent postnatal corrective surgery are significantly favorable outcomes.

Impact of overweight and obesity in the fetal cardiac function parameters in the second and third trimesters of pregnancy.

OBJECTIVE: To assess the impact of overweight and obesity in the second and third trimesters of pregnancy on fetal cardiac function parameters. METHODS: We performed a prospective cohort study of 374 singleton pregnant women between 20w0d and 36w6d divided into three groups: 154 controls (body mass index - BMI < 25 kg/m2), 140 overweight (BMI 25-30 kg/m2) and 80 obese (BMI ≥ 30 kg/m2). Fetal left ventricular (LV) modified myocardial performance index (Mod-MPI) was calculated according to the following formula: (isovolumetric contraction time + isovolumetric relaxation time)/ejection time. Spectral tissue Doppler was used to determine LV and right ventricular (RV) myocardial performance index (MPI'), peak myocardial velocity during systole (S'), early diastole (E'), and late diastole (A'). RESULTS: We found significant differences between the groups in maternal age (p < 0.001), maternal weight (p < 0.001), BMI (p < 0.001), number of pregnancies (p < 0.001), parity (p < 0.001), gestational age (p = 0.013), and estimated fetal weight (p = 0.003). Overweight pregnant women had higher LV Mod-MPI (0.046 versus 0.044 seconds, p = 0.009) and LV MPI' (0.50 versus 0.47 seconds, p < 0.001) than the control group. Obese pregnant women had higher RV E' than control (6.82 versus 6.33 cm/sec, p = 0.008) and overweight (6.82 versus 6.46 cm/sec, p = 0.047) groups. There were no differences in 5-min APGAR score < 7, neonatal intensive care unit admission, hypoglycemia and hyperglobulinemia between the groups. CONCLUSIONS: We observed fetal myocardial dysfunction in overweight and obese pregnant women with higher LV Mod-MPI, LV MPI' and RV E' compared to fetuses from normal weight pregnant women.

Fetal tetralogy of Fallot: Three-dimensional virtual and physical models from ultrasound scan data findings.

Three-dimensional reconstructions provide a spatial view of the congenital heart disease with a better understanding of the pathology for parents and allow interactive discussion among the medical team (maternal-fetal medicine specialist, neonatology, pediatric cardiology, and cardiovascular surgeon) and improve both objective knowledge and learner satisfaction for medical students.

Three-dimensional navigation inside a normal fetal heart in a virtual reality environment.

Three-dimensional navigation allows in a virtual reality environment across the fetal heart structures using glasses and joysticks. This technology allows virtual interactive discussions among multidisciplinary teams providing new perspectives on diagnosis and planning possible surgical corrections.

Prenatal diagnosis of fetal left ventricular diverticulum: 3D virtual and physical reconstruction models.

Congenital diverticulum is a very rare congenital heart disease. 3D virtual and physical models from ultrasound scan data provide a spatial visualization and better understanding of congenital heart disease to the parents and support possible surgical procedures.

Assessment of fetal heart aortic and pulmonary valve annulus area by three-dimensional ultrasonography: reference curves and applicability in congenital heart diseases.

OBJECTIVES: To determine reference curves for fetal aortic and pulmonary valve annulus area by three-dimensional ultrasonography using the spatio-temporal image correlation (STIC) in the rendering mode, and to ascertain its applicability in congenital heart disease (CHD). METHODS: We performed a retrospective cross-sectional study of 328 normal fetuses and 42 fetuses with CHD between 20 and 33 weeks 6 days of gestation. The outflow plane view of the great vessels was used to measure the areas of the valvar annuli, and the measurements were performed in systole. A linear regression model adjusted according to the determination coefficient (R2) was utilized to construct the reference intervals. The concordance correlation coefficient (CCC) was used to calculate the reproducibility of the mitral and tricuspid valve areas. RESULTS: The mean ± standard deviation (SD) of the aortic and pulmonary valve annulus areas ranged from 6.6 ± 1.2 to 32.9 ± 1.1 mm2 and 10.7 ± 1.3 to 40.3 ± 1.2 mm2, respectively. We observed a linear relationship and strong positive correlation between the area of the aortic and pulmonary valve annuli with r=0.97 and 0.96, respectively. Good intra (CCC=0.99) and interobserver agreement (CCC=0.98) was observed for the measurement of the aortic valve annulus area. A good intra (CCC=0.99) and interobserver (CCC=0.97) agreement was also observed for the measurement of the pulmonary valve annulus area. The mean ± SD of the difference of the areas of the aortic and pulmonary valve annuli between the normal fetuses and those with CHD were -1.801 ± 1.429 mm2 (p=0.208) and -1.033 ± 1.467 mm2 (p<0.0001), respectively. CONCLUSIONS: The reference curves for the areas of the aortic and pulmonary valve annuli of fetal hearts were determined, and showed good inter and intraobserver reproducibility. The constructed reference curves showed applicability in different types of CHD.

The use of cardiac ultrasound imaging in first-trimester prenatal diagnosis of congenital heart diseases.

This study aims to explore ultrasound (US) cardiac and echocardiographic features that may enable the early diagnosis of various major congenital heart diseases (CHDs). Focusing on providing useful US tools for this assessment, high resolution of US cardiac images of various CHDs, such as hypoplastic left heart syndrome, conotruncal anomalies, and univentricular heart, were evaluated. Results show that early US detection of most major CHDs is feasible during first-trimester ultrasonography cardiac evaluation. Concerns about safety issues, findings on early fetal cardiovascular hemodynamics, and cardiac lesions that can progress during the course of pregnancy were also discussed.

Area of the Fetal Ascending and Descending Aorta by Spatiotemporal Image Correlation in the Rendering Mode: Reproducibility and Comparison with Pregestational Diabetic Mothers.

Background: The objective of this study was to assess the ascending and descending aorta area measurements by three-dimensional (3D) ultrasound using spatiotemporal image correlation (STIC) in the rendering mode comparing these measurements with pregestational diabetic mothers and assessing the reproducibility of the method. Methods: We carried out a retrospective cross-sectional study with 58 normal and nine fetuses from pregestational diabetic mothers between 20 and 33 + 6 weeks of gestation. Fetal heart volumes were acquired at the level of four-chamber view to obtain the reconstructed planes for the ascending and descending aorta areas in the rendering mode. Linear regression was performed to assess the correlation between the fetal aorta areas and gestational age (GA). To assess the intra- and interobserver reproducibility, we used the concordance correlation coefficient (CCC). Results: The mean ascending and descending aorta areas were 0.12 (0.02-0.48) and 0.11 (0.04-0.39) cm2 in normal fetuses, respectively. There was a moderate positive correlation between GA and ascending aorta area measurements (0.005676*GA - 0.01283; r = 0.53, P < 0.0001) and strong positive correlation between GA and descending aorta area (0.01095*GA - 0.1581; r = 0.68, P < 0.0001). We observed a weak intra- and interobserver reproducibility with CCC ranging from 0.05 to 0.91. The mean difference in the ascending and descending aorta area measurements of normal and fetuses of pregestational diabetic mothers was -0.03 cm2 (P = 0.276) and -0.03 cm2 (P = 0.231), respectively. Conclusion: The fetal ascending and descending aorta area measurements obtained by 3D ultrasound using STIC in the rendering mode increased with GA in normal fetuses. The method showed weak intra- and interobserver reproducibility.

References Values of Fetal Heart Myocardial Volume by Three-Dimensional Ultrasound using Spatiotemporal Image Correlation and Virtual Organ Computer-Aided Analysis Methods and Their Applicability in Pregestational Diabetic Women.

OBJECTIVE: To determine reference values for myocardial volume of the fetal heart using three-dimensional ultrasound with spatiotemporal image correlation (STIC) and virtual organ computer-aided analysis (VOCAL) and to assess their applicability in women with pregestational diabetes. STUDY DESIGN: This retrospective cross-sectional study included 177 normal pregnant women with fetuses between 200/7 and 336/7 weeks of age. Fetal cardiac volumes were collected using the STIC method, and myocardial volume was obtained by subtraction of the intracavitary volumes using the VOCAL 30-degree method. Intra- and interobserver reproducibility values were determined using the concordance correlation coefficient (CCC). Sixteen women with pregestational diabetes mellitus were evaluated for validation. RESULTS: There was a strong correlation between fetal myocardial volume and gestational age (R 2 = 0.82). Intra- and interobserver reproducibility values were excellent and moderate, respectively, with CCCs of 0.99 and 0.83, respectively. There was no significant difference in mean fetal myocardial volume between normal pregnant women and those with pregestational diabetes (p = 0.64). CONCLUSION: Reference values for myocardial volume of the fetal heart were determined in normal pregnant women and were not statistically different from those in women with pregestational diabetes mellitus.

Prenatal diagnosis of aortic arch anomalies: Echocardiography, 3D-ultrasonography, and computed tomography angiogram findings. A case-report.

We report the case of a fetus presenting aortic arch anomalies associated with a ventricular septal defect (VSD). This fetus, which was referred at 25 weeks of gestation, was suspected to have coarctation of aorta (CoA) evidenced by enlarged right chambers at the four-chamber view during a routine obstetric ultrasonographic scan. The prenatal diagnosis of CoA remains a challenge. Here, we review the ultrasonographic findings that could contribute to this diagnosis.

Reference ranges for the fetal mitral, tricuspid, and interventricular septum annular plane systolic excursions (mitral annular plane systolic excursion, tricuspid annular plane systolic excursion, and septum annular plane systolic excursion) between 20 and 36 + 6 weeks of gestation.

Objectives This study aimed to establish reference ranges for fetal mitral, tricuspid, and interventricular septum annular plane systolic excursions (MAPSE, TAPSE, and SAPSE) in normal pregnant women between 20 and 36 + 6 weeks of gestation. Methods This prospective and cross-sectional study included 360 low-risk singleton pregnancies between 20 and 36 + 6 weeks of gestation. MAPSE, TAPSE, and SAPSE were measured by M-mode in real time in an apical or basal four-chamber view through placing the cursor at the atrioventricular junction, marked by the valve rings at the tricuspid, mitral, and basal septum, respectively. A regression analysis was done to determine the appropriate polynomial equation model for both measurements and standard deviation (SD) values in relation to gestational age (GA). The intra- and inter-observer reproducibility was evaluated using the concordance correlation coefficient (CCC) and limits of agreement (LoA). Results There was a significant positive correlation between MAPSE (r=0.705, p<0.0001), TAPSE (r=0.804, p<0.0001), and SAPSE (r=0.690, p<0.0001) and GA. The mean of each parameter ranged as follows: 2.87-5.56 mm, MAPSE; 3.98-8.07 mm, TAPSE; and 2.34-4.21 mm, SAPSE. Poor/moderate intra- and inter-observer reliability (CCC between 0.70 and 0.90) and poor/moderate agreement of all the tested parameters were evaluated (LoA between 10 and 50%). Conclusions Reference values were established for the fetal MAPSE, TAPSE, and SAPSE between 20 and 36 + 6 weeks of gestation in low-risk pregnant women. These parameters showed poor/moderate reproducibility.

Fetal Cardiac Function and Ventricular Volumes Determined by Three-Dimensional Ultrasound Using STIC and VOCAL Methods in Fetuses from Pre-gestational Diabetic Women.

To assess the fetal cardiac function and ventricular volumes by three-dimensional (3D) ultrasound using spatio-temporal image correlation (STIC) and virtual organ computer-aided analysis (VOCAL) methods in fetuses from pre-gestational diabetic women. This was a prospective and cross-sectional study that evaluated 53 fetuses from pre-gestational diabetic women and 53 fetuses from healthy mothers between 20 and 34 weeks of gestation. Only fetuses with no structural or genetic abnormalities and singleton pregnant women were included in this study. The fetal cardiac volumes were assessed by STIC and VOCAL methods. The ejection fraction, stroke volume, and cardiac output were calculated from these measurements to evaluate fetal cardiac function. The Mann-Whitney U test was performed to compare the two groups. For calculation of intra- and interobserver reproducibility's, we used concordance correlation coefficients. The mean differences in the right atrial volumes between the diabetic and normal groups ranged from 0.05 mL to 0.1 mL (p = 0.917 and 0.355, respectively). The median of left atrium (LA) volume measurement in pre-gestational diabetic group was significantly lower than healthy mothers (LA: 0.62 vs. 0.68 mL; p < 0.001). The fetal right and left ventricular volumes were similar in both groups. No significant differences in ejection fraction, stroke volume and cardiac output were observed (p value range 0.086-0.815). The majority of fetal atrial/ventricular volumes showed good intra- and interobserver reliabilities. Conversely, the majority cardiac function parameters showed poor intra- and interobserver agreements. STIC and VOCAL methods gave reproducible quantitative results for fetal atrial and ventricular volumes. Significant differences in fetal left atrial volumes were observed between the two groups, which be related to LA atrial dysfunction and /or left ventricle (LV) compliance, reflecting earlier stages of cardiac dysfunction.

Reference range of fetal myocardial area by three-dimensional ultrasonography and its applicability in fetuses of pre-gestational diabetic women.

Objective To determine the reference range for the myocardial area in healthy fetuses using three-dimensional (3D) ultrasonography and validate these results in fetuses of pregnant women with pre-gestational diabetes mellitus (DM). Methods This cross-sectional retrospective study included 168 healthy pregnant women between gestational weeks 20 and 33+6 days. The myocardial area was measured using spatio-temporal image correlation (STIC) in the four-chamber view. Polynomial regression models were used, and the goodness of fit of the models were evaluated by the coefficient of determination (R2). Intra- and inter-observer reproducibility was determined using the concordance correlation coefficient (CCC). Validation was performed in 30 pregnant women with pre-gestational DM. Results There was a strong correlation (R2=0.71, P<0.0001) between myocardial area and gestational age. There was good intra- and inter-observer reproducibility, with a CCC of 0.86 and 0.83, respectively. However, there was no significant difference in the mean myocardial area between healthy fetuses and fetuses of women with pre-gestational DM (0.11 cm2, P=0.55). Conclusion The reference range was determined for the myocardial area in fetuses, and there was no significant difference in this variable between healthy fetuses and the fetuses of women with pre-gestational DM.

Fetal transposition of the great arteries: 3D virtual and physical models from ultrasound datasets.

Transposition of the great arteries (TGA) is a cyanotic congenital heart disease characterized by ventriculoarterial discordance and atrioventricular concordance with the great arteries in a parallel relationship. Prenatal diagnosis of TGA has implications for postnatal outcomes, allowing for planned delivery and perinatal management. Three-dimensional virtual or physical models of fetal TGA allow better understanding of fetal cardiac anomalies by parents and interactive discussion among the multidisciplinary team (obstetricians, pediatricians, maternal-fetal specialists, pediatric cardiologists, and cardiovascular surgeons), as well as continuing medical education.

Evaluation of cardiac contractility of fetuses from pregestational diabetes mellitus pregnancies by three-dimensional ultrasound.

OBJECTIVE: This study aimed to evaluate cardiac contractility in fetuses from pregestational diabetes mellitus pregnancies by three-dimensional ultrasound using spatiotemporal image correlation in rendering mode. METHODS: A retrospective cross-sectional study was performed on 40 fetuses from nondiabetic pregnancies and 28 pregestational diabetic pregnancies between 20 and 33 weeks and 6 days. Cardiac contractility was assessed by measuring the ventricular myocardial area in diastole subtracted from the ventricular myocardial area in systole. RESULTS: Pregestational diabetic pregnancies had a lower maternal age than nondiabetic pregnancies (26.7 vs. 39.9 years, p=0.019). Cardiac contractility in fetuses from diabetic and nondiabetic pregnancies was similar (p=0.293). A moderately positive and significant correlation was observed between gestational age and cardiac contractility (r=0.46, p=0.0004). A 1-week increase in gestational age was responsible for a 0.1386 cm2 increase in cardiac contractility. CONCLUSION: Cardiac contractility as evaluated by three-dimensional ultrasound using spatiotemporal image correlation in rendering mode showed no significant differences across fetuses with and without pregestational diabetes.

Retrospective analysis of percutaneous intervention of the renal artery in transplanted kidneys in children and adolescents at a tertiary public hospital.

BACKGROUND: This study evaluated the long-term effects of percutaneous intervention in children and adolescents with transplant renal artery stenosis (TRAS). METHODS: Twenty patients had significant stenosis (>50%) and underwent percutaneous transluminal angioplasty (PTA/stenting) (TRAS group-intervention); 14 TNS (non-significant group -control) patients did not have significant stenosis (≤50%) and were treated clinically. The combined primary endpoints were death from all causes and late graft failure. The secondary endpoints were serum creatinine (SCr), systolic blood pressure (SBP), and diastolic blood pressure (DBP). RESULTS: No statistically significant difference was found between TRAS-Intervention(N = 20) and TNS groups-Control (N = 14) for these clinical parameters: deaths, 1 (5.0%) vs. 0 (0.0%) (p = 1.000) and graft loss, 4 (20.0%) vs. 2 (14.3%) (p = 1.000). For the secondary endpoints, after 1 month and 1 year the values of SCr, SBP, and DBP were similar between the two groups but not statistically significant. DISCUSSION: In the TRAS group (intervention), the stent implantation was beneficial for treating refractory hypertension and reducing blood pressure (BP) in children and adolescents. Despite the outcomes being similar in the two groups, it can be inferred that the patients in the TRAS group (intervention) would have had a worse outcome without the percutaneous intervention. CONCLUSION: TRAS treatment with stenting can be considered for children and adolescents. Because the sample in the present study comprised of only a specific population, further studies are needed for generalization. TRIAL REGISTRATION: The trial was registered at clinictrials.gov with trial registration number NCT04225338.

Fetal bradycardia and sinus node dysfunction.

The study reported here is a rare case of fetal sinus bradycardia that evolved into symptomatic bradycardia after birth, at which time the implantation of a cardiac pacemaker was indicated. Fetal echocardiography was used to diagnose the type of cardiac rhythm that caused the intra-uterine bradycardia, which enabled the initiation of the appropriate therapy approach and avoided an unnecessary interruption of the pregnancy. However, the details of the sinus bradycardia were impossible to determine in utero in this case due to sinus node dysfunction. After birth, the electrocardiogram results drew attention to a potentially unusual cause of sinus bradycardia, and enabled the diagnosis of this rare disease in this infant.

Elevated lipoprotein(a) in a newborn with thrombosis and a family history of dyslipidemia.

This paper reports a rare case of elevated lipoproteinemia(a) that evolved into thrombosis during the neonatal period. During the first days of life, the patient presented with an intracardiac thrombus, pulmonary thromboembolism and a hemorrhagic stroke. Initially, the results of the blood tests performed to screen for thrombophilic diseases were normal for the patient's age. The maternal dyslipidemia and the family's positive history of thromboembolism drew attention to an underlying, inherited, thrombophilic defect. Upon further investigation of the thrombophilia, the increase in lipoprotein(a) levels found in the mother and infant enabled the diagnosis of hyperlipoprotein(a) and the administration of appropriate therapy.