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.

Prenatal Echo-Sonographic Parameters in Fetuses Wrapped with the Umbilical Cord in the Third Trimester of Pregnancy.

This study constitutes a description of prenatal echo-sonographic parameters in fetuses wrapped with the umbilical cord in the third trimester of pregnancy and demonstrates the practical importance of the umbilical cord collision. Echocardiographic examinations were performed within 6 months, and a group of patients in the third trimester with a mean age of 28.1 ± 0.79 weeks of gestation (p = 0.075) was distinguished. The group included 46 fetuses from single pregnancies with the umbilical cord around the fetal neck and 70 fetuses without the umbilical cord around the fetal neck. The course of the umbilical cord coiling around the fetal neck was recorded by color Doppler. We also conducted a follow-up with the newborns. In the study group, there was an elevated peak systolic velocity of the umbilical artery (UMB PSV) at a level of 44.17 cm/s vs. 38.90 cm/s in the control group (p = 0.004), and caesarean sections were significantly more frequent (54.5% vs. 31.4%). The persistence of the nuchal cord during delivery was observed in 37% of newborns in the study group, while the occurrence of umbilical wrapping during delivery was found in 18.6% of newborns in the control group (p = 0.027). In the studied cases, caesarean sections were 2.62 times more frequent (OR = 2.62), whereas nuchal cords during delivery were 2.57 times more often observed (OR = 2.57). Fetal umbilical cord wrapping in the third trimester of pregnancy does not have a significant hemodynamic impact; however, the UMB PSV might be slightly elevated in this group, and the frequency of umbilical cord collision during delivery and the need to perform a caesarean section at term seem to be more common.

Small prenatal diameter of the ascending aorta is associated with increased mortality risk in neonates with congenital diaphragmatic hernia.

Introduction: The aim of this study was to evaluate whether selected prenatal markers obtained from fetal echocardiography can predict postnatal outcome in congenital diaphragmatic hernia (CDH) patients. We also aimed to verify the prognostic value of lung-to-head ratio (LHR). Material and methods: The study group included 29 fetuses with CDH. We analyzed potentially prognostic parameters measured using fetal echocardiography and fetal ultrasound. The assessed parameters were compared between the group of patients with CDH who survived to discharge (n = 21) and the subset of patients who died before discharge from hospital (n = 8). Results: In survivors, mean z-score for ascending aorta (AAo) diameter was 0.23 ±0.98 vs. - 1.82 ±1.04 in patients who died (t-test, p = 0.0015). In survivors, the main pulmonary artery/ascending aorta ratio was 1.22 ±0.17 vs. 1.46 ±0.21 in patients who died (t-test, p = 0.017). In survivors, the LHR was 1.81 ±0.96 vs. 0.95 ±0.6 in patients who died (t-test, p = 0.019). In survivors, the observed to expected LHR was 57 ±30% vs 30 ±18% in patients who died (t-test, p = 0.018). Conclusions: Narrowing of the ascending aorta in CDH fetuses is a poor prognostic factor associated with increased mortality in neonates. Our study also confirmed the prognostic value of LHR.

Ectopia cordis: prenatal diagnosis, perinatal outcomes, and postnatal follow-up of an international multicenter cohort case series.

OBJECTIVE: This study aimed to analyze prenatal diagnosis, perinatal outcomes, and postnatal follow-up in fetuses with ectopia cordis (EC). METHODS: This retrospective analysis accessed 31 patients with EC who were either diagnosed or referred to a tertiary Fetal Medicine centers for EC diagnosis in Brazil, Germany, Italy, and Poland. We analyzed prenatal diagnosis, perinatal outcomes, and follow-up in these patients. RESULTS: Our study included a cohort of 31 fetuses with EC, 4 and 27 of whom had partial and complete protrusion of the heart through a ventral defect in the thoracoabdominal wall, respectively. EC was diagnosed by fetal echocardiography at a mean gestational age of 20.3 ± 8.6 weeks (range, 8-35 weeks). Of the four cases, in which the karyotype was performed, all of them had a normal result (1 - 46,XX and 3 - 46,XY). Five patients showed conotruncal abnormalities and six ventricular septal defects. Termination of pregnancy (TOP) was performed in 15 cases (48%) and seven pregnant women had spontaneous fetal demise (22.5%). Of the seven fetuses that were born alive, four of them died, and three infants underwent surgery. Among these three infants, all of them survived, one was 5 months, 13 years old and 29 years old at the time of study completion. CONCLUSIONS: Ectopia cordis is associated with high mortality rates and intracardiac/extra-cardiac defects. Ventricular septal defects and conotruncal anomalies were the more common intracardiac defects associated with EC. However, in this cohort of fetuses with EC the incidence of PC was lower than reported in the literature.

Maternal hyperoxygenation during pregnancy as a tool in fetal disease diagnosis and treatment.

Maternal hyperoxygenation (MHO) consists of giving pregnant women (60% to 100%) oxygen through a facemask and using ultrasound assess or monitor the influence on fetal cardiovascular circulation. This review discusses the findings and the utility of acute and chronic MHO in various fetal diseases.

Cardiac Adaptation and Preferential Streaming in Fetuses with Multiple Nuchal Cords.

The echocardiographic monitoring of a fetus with multiple nuchal cords around the fetal neck is important as it may result in cardiac remodeling and preferential streaming, thus affecting the condition of the fetus. The main aim of our study was to assess whether the collision of the umbilical cord around the fetal neck can lead to discrepancies in the size of the pulmonary artery and the aorta in the three-vessel view and to an increase in the size of the heart, which may result from the compression of the carotid arteries caused by the umbilical cord wrapping around the fetal neck. A total number of 854 patients were included in this study and divided into three groups. Group A (control group) included 716 fetuses (84%) without the umbilical cord around the fetal neck. Group B (study group B) included 102 fetuses (12%) with one coil of the umbilical cord around the fetal neck. Group C (study group C) included 32 fetuses (4%) with two coils of the umbilical cord around the fetal neck. The range of the gestational age of the patients considered for this study was 27-40.2 weeks based on the ultrasound biometry and was not statistically different between the analyzed groups A, B and C (p > 0.05). The Pa/Ao index was calculated by dividing the value of the width of the pulmonary artery (in mm) to the width of the aorta (in mm) measured in the ultrasound three-vessel view. We found that fetuses that the fetuses with one and two coils of the umbilical cord around the neck showed significantly higher values of the width of the pulmonary trunk with the unchanged value of the aorta width. Therefore, we also observed significantly higher values of the ratio of the pulmonary trunk to the aorta for the fetuses wrapped with the umbilical cord around the neck compared with the control group without the umbilical cord around the neck (. Moreover, in the fetuses with one and two coils of the umbilical cord around the fetal neck, an increased amount of amniotic fluid was observed, whereas larger dimensions of CTAR in the fetuses with two coils of the umbilical cord around the neck were present (p < 0.05). The wrapping of the fetus with the umbilical cord around the fetal neck may induce the redistribution of blood flow, leading to fetal heart enlargement and disproportion and may be the cause of polyhydramnios.

Normograms in Prenatal Life of Stomach and Urinary Bladder in the Second and Third Trimesters of Pregnancy.

Aim: The aim of this study was to prepare normograms for the fetal stomach, urinary bladder, and stomach to urinary bladder index in healthy fetuses. Material and methods: The study was conducted based on the data extracted from the database of our tertiary center in the years 2016-2019. The study group, comprising 867 fetuses, demonstrated normal biometry and normal heart structure, normal heart function, no extracardiac malformations, and no extracardiac anomalies. The stomach to urinary bladder index was analyzed in the study group. The examinations were performed with the use of the following ultrasound machines: Voluson E10, Philips and Voluson Expert, with convex transabdominal transducers. Linear regression analysis based on Microsoft Excel was used for statistical analysis. Results: The average size of the stomach in healthy fetuses between the 14-40th week of gestation was 18 mm (8-40 mm), the average urinary bladder measurement was 17 mm (15-42 mm), and the fetal stomach to urinary bladder index was constant: 1.26 (0.09-3.93). Conclusions: The normograms for the stomach, urinary bladder and the stomach to urinary bladder index prepared based on our study group can contribute to an improvement in the accuracy of examination and provide an unified organization of the description of fetuses. These normograms constitute an additional marker for the assessment of fetal condition. A clear disproportion in the size of the urinary bladder and stomach can be helpful in terms of paying more attention to fetuses with untypical features in screening centers.

Fetal echocardiography and early neonatal balloon valvuloplasty improved overall survival in prenatally detected aortic stenosis over 25 years of tertiary center experience.

PURPOSE: This article aimed to present the factors determining survival and prognosis in fetuses and newborns with critical prenatal aortic stenosis (AS) and to present 26 years of tertiary center experience. METHODS: Study included 87 fetuses with critical AS requiring surgical intervention during neonatal period. All results were expressed as means ± SD, in numbers and percentages. The statistically significant results were those with p < 0.05. RESULTS: An increase in the number of cases of AS was observed in our center along with a decrease in gestational age of our patients during the first echocardiographic exam. The survival rate of newborns was considerably higher when born in due time (p < 0.05) with body weight > 2500 g (p < 0.05). Balloon valvuloplasty performed in the first days after birth occurred to be an optimal solution in these cases. CONCLUSIONS: Fetal echocardiography and special perinatal care with transplacental maternal pharmacotherapy in selected cases and an early neonatal aortic balloon valvuloplasty have shown improvement in survival rate. The most dangerous for the newborn with AS was the first week of postnatal life. It is vital to refer the fetuses with AS to the reference centers which offer the possibility of invasive cardiac intervention on the first day after birth, and it might be an optimal solution.

An investigation of the optimal inter-pregnancy interval following pregnancy with a fetus with congenital heart disease.

Introduction: Congenital heart defects (CHD) are one of the most commonly diagnosed congenital malformations in fetuses and newborns. The aim of the study was to determine whether inter-pregnancy interval (IPI), maternal age or number of pregnancies had any influence on the recurrence of congenital heart disease in subsequent pregnancies. Material and methods: We found in our database 144 women with subsequent pregnancies after CHD in a previous pregnancy. Each woman was selected according to the eligibility and exclusion criteria. Medical history as well as obstetrics history were recorded. Comparisons of groups with and without a recurrence of CHD were performed. We calculated hazard ratios for recurrence of CHD. We also performed analysis of the impact of confounding variables: maternal age and parity. Missing data were excluded from the analysis. Smoking habits as well as socio-demographic characteristics were not evaluated in this study. Results: A higher risk of recurrence of CHD correlated with a shorter IPI, with a median of 11 months compared with 24 months for the group of healthy fetuses in subsequent pregnancy. The results were statistically significant. Parity was proven to be an important confounder of the study. Multivariable analysis including parity and maternal age did not affect the confidence intervals of hazard ratios for IPI. Conclusions: The optimal IPI to reduce the risk of recurrence of CHD is 24 months. Shorter intervals are related to a higher risk of recurrence of CHD in the next pregnancy and are independent on the age of the woman and parity.

The association of diabetes with all-cause mortality in patients with end-stage renal disease compared to the general population in Poland - a comparative analysis.

Introduction: End-stage renal disease (ESRD) is an important complication of diabetes, which is the leading cause of ESRD worldwide. The aim of the study was a comparative analysis of all-cause mortality in patients with ESRD with diagnosed diabetes mellitus (DM) and no diagnosed DM. Material and methods: Data for the analysis were obtained from the resources of the Polish National Health Fund, and they concerned patients with end-stage renal disease from the entire population of Polish patients in the period from 1.01.2011 to 31.12.2013. In addition, the period from 1.01.2012 to 31.12.2012 was analysed for two subpopulations: diabetic and non-diabetic patients. Results: The all-cause mortality in patients with end-stage renal disease in Poland per 100,000 representatives of the general population was 17.7, 15.9, and 12.50 persons in 2011, 2012, and 2013, respectively. The all-cause mortality rates for patients with ESRD and diabetes in Poland in 2012 were more than 15 times higher, for both men and women, than the all-cause mortality rates for non-diabetic patients with ESRD. Mortality in the study group of diabetic men with ESRD amounted to 147.59 ±29.07/100,000 men, whereas in the study group of diabetic women with ESRD it was 105.13 ±26.77/100,000 women. Regarding non-diabetic men with ESRD and non-diabetic women with ESRD, mortality amounted to 9.58 ±6.29/100,000 and 6.87 ±2.27/100,000 men and women, respectively. Conclusions: The occurrence of diabetes in patients with ESRD significantly increases the risk of death compared to patients with ESRD without diabetes.

Ebstein's anomaly: epidemiological analysis and presentation of different prenatal management.

OBJECTIVES: To present the crucial role of echocardiographic examination in perinatal care and analyze influence of prenatal treatment for neonatal outcome. Furthermore, the attempt to answer the question if there was any relationship between the occurrence of fetal Ebstein's anomaly and environmental risk factors in polish population. METHODS: Forty-five prenatal diagnoses of Ebstein's anomaly were compiled over the 21-year period (1998‒2018) form our single unit. The analysis included the assessment of maternal parameters (age, past obstetric history, and place of residence) and fetal parameters (sex, gestational age, anatomy, the fetal cardiovascular condition assessed by the CVPS, associated extracardiac anomalies or malformations, prenatal treatment, delivery and follow-up). RESULTS: The average age of gravida was 29.5 years (± 5.2 years) and gravidae <35 years of age accounted for 80% . There were 43 singleton pregnancies and 2 cases of multiple pregnancy. Ebstein's anomaly was mostly (averagely) diagnosed at 28th week of gestation. Forty-three fetuses had normal karyotypes and two had trisomy 21. Cardiomegaly was present in 91% (41) of fetuses. The average heart area to chest area ratio was 0.56 (± 0.12). In 21 cases, there was only fetal monitoring - echocardiographic examinations and postnatal mortality was 44.4%. In 5 cases, transplacental digoxin treatment was administered and mortality was: 40%. In another 5 cases, only steroid therapy was applied and postnatal mortality was 100%. Steroids and transplacental digoxin treatment were administered in 11 cases and mortality was 63.6%. In 3 last cases transplacental digoxin treatment, steroids and maternal hyperoxygenation therapy were given and mortality was 0%. Cesarean section rate was 49%. Moreover, due to Ebstein's anomaly regional peak of occurrence benzopyrene was deliberated as environmental risk factor. CONCLUSIONS: Fetal Ebstein's anomaly occurred in our population in healthy young women, expecting their first child and malformation was not related to fetus gender, nor to maternal health condition. Our data can be a new signal for the development of novel treatment strategies in therapy in fetuses with Ebstein's anomaly.

Hypoplastic left heart syndrome with prenatally diagnosed foramen ovale restriction: diagnosis, management and outcome.

OBJECTIVE: Despite advances in prenatal diagnosis and postnatal intervention/surgery methods, patients with Hypoplastic Left Heart Syndrome (HLHS) and coexisting foramen oval restriction still achieve high mortality rates. Our objective was to determine survival predictors and to find answers to, why restriction develops in some, but not others. METHODS: We performed a retrospective analysis of prenatal history and postnatal sequel of 22 patients with HLHS and foramen ovale restriction between 2008 and 2017. RESULTS: There were 11 survivors and 11 nonsurvivors. The most significant difference between the two groups pertained to the average time of foramen ovale restriction diagnosis which was 33 weeks for survivors and 28 weeks for nonsurvivors (p = .0416) and the duration of in-utero restriction (9 versus 5 weeks, p = .0213). Twenty patients (20/22) exhibited possible signs of infection. CONCLUSIONS: (1) Earlier development and longer presence of foramen ovale restriction in the setting of HLHS is associated with higher short-term mortality regardless of the degree of restriction. (2) Ratio of forward pulmonary vein flow to reverse flow (f/r) expressed as a velocity-time integral (VTI) is a good emergent intervention predictor, but it does not correlate with foramen ovale size and maximal velocity, nor does it influence survival rates. (3) Ultrasonographic signs of possible infection of the fetus is a potential risk factor of foramen ovale restriction development in patients with HLHS.

Nomograms of the fetal thymus for clinical practice.

INTRODUCTION: The fetal thymus may be visualized using ultrasonography (USG) and is typically located in the mediastinum. In the past years, the size of the fetal thymus has served not only as a marker of genetic or heart defects but also as a predictive factor for intrauterine growth restriction, premature birth, preeclampsia, chorioamnionitis or even neonatal sepsis. MATERIAL AND METHODS: A total of 410 fetuses were qualified for the study. Fetuses with heart defects were excluded from the study. The fetal thymus was evaluated with ultrasonography between the 14th and 40th week of gestation. After obtaining a standard transverse view encompassing the three great vessels, thymus measurements were attempted, i.e. maximal transverse diameter, circumference and surface area. Linear regression was used for statistical analysis, yielding 3 models, each with a different dependent variable. The confidence interval for each model was set at 80% to aid the comparison with centile grid growth charts for neonates and children. The test was regarded as statistically significant when p < 0.05. RESULTS: From a total of 410 fetuses the thymus transverse diameter, circumference and area were successfully measured in 410, 320 and 330 cases, respectively. The probabilities are lower than 0.0005 for each model, which means that each model is quite statistically significant. CONCLUSIONS: The coverage of healthy thymus nomograms in the fetal population may be the basis for the identification of fetuses at risk of hypoplasia or thymic hyperplasia, which seems particularly important from the point of view of the detection of potential inborn immunological disorders.