Background
Birthweight (BW) is an important prognostic factor in newborns with congenital heart defects (CHD).
Objectives
To give an overview of the literature on BW z‐score in children with isolated ...CHD.
Search strategy
A systematic search was performed on isolated CHD and BW in PubMed, Embase, Web of Science, COCHRANE Library and Emcare.
Selection criteria
Neonates with isolated CHD were included if a BW percentile, BW z‐score or % small‐or‐gestational age (SGA) was reported.
Data collection and analysis
BW z‐score and percentage SGA were pooled with random‐effect meta‐analysis. Quality and risk of bias were assessed using the modified Newcastle Ottawa Scale.
Main results
Twenty‐three articles (27 893 cases) were included. BW z‐scores were retrieved from 11 articles, resulting in a pooled z‐score of −0.20 (95% CI −0.50 to 0.11). The overall pooled prevalence of SGA <10th percentile was 16.0% (95% CI 11.4–20.5; 14 studies). Subgroup analysis of major CHD showed similar results (BW z‐score −0.23 and percentage SGA 16.2%).
Conclusions
Overall BW in isolated CHD is within range of normality but impaired, with a 1.6‐fold higher risk of SGA, irrespective of the type of CHD (major CHD vs all CHD combined). Our findings underline the association between CHD and BW. The use of BW z‐scores provides insight into growth of all fetuses with CHD.
Tweetable
Infants with a congenital heart defect (CHD) have a lower birthweight z‐score and a higher incidence of small‐for‐gestational age (<10th percentile). This was encountered both in the major CHD‐group as well as in all‐CHD combined group analysis. Future research on the association between birthweight and CHD should include all types of CHDs (including mild cardiac defects) and placental‐related disease, such as pre‐eclampsia. We advocate the use of international standardised fetal growth and birthweight charts in CHD research.
Tweetable
Infants with a congenital heart defect (CHD) have a lower birthweight z‐score and a higher incidence of small‐for‐gestational age (<10th percentile). This was encountered both in the major CHD‐group as well as in all‐CHD combined group analysis. Future research on the association between birthweight and CHD should include all types of CHDs (including mild cardiac defects) and placental‐related disease, such as pre‐eclampsia. We advocate the use of international standardised fetal growth and birthweight charts in CHD research.
Impaired placentation is an important contributing factor to intra-uterine growth restriction and pre-eclampsia in fetuses with congenital heart defects (CHD). These pregnancy complications occur ...more frequently in pregnancies with fetal CHD. One of the most important factors influencing the life of children with CHD is neurodevelopmental delay, which seems to start already in utero. Delayed neurodevelopment in utero may be correlated or even (partly) explained by impaired placentation in CHD cases. This systematic review provides an overview of published literature on placental development in pregnancies with fetal CHD. A systematic search was performed and the Newcastle-Ottawa scale was used to access data quality. Primary outcomes were placenta size and weight, vascular and villous architecture, immunohistochemistry, angiogenic biomarkers and/or placental gene expression. A total of 1161 articles were reviewed and 21 studies were included. Studies including CHD with a genetic disorder or syndrome and/or multiple pregnancies were excluded. Lower placental weight and elevated rates of abnormal umbilical cord insertions were found in CHD. Cases with CHD more frequently showed microscopic placental abnormalities (i.e. abnormal villous maturation and increased maternal vascular malperfusion lesions), reduced levels of angiogenic biomarkers and increased levels of anti-angiogenic biomarkers in maternal serum and umbilical cord blood. Altered gene expression involved in placental development and fetal growth were found in maternal serum and CHD placentas. In conclusion, abnormal placentation is found in CHD. More extensive studies are needed to elucidate the contribution of impaired placentation to delayed neurodevelopment in CHD cases.
•Growth restriction and pre-eclampsia are common in fetal congenital heart disease.•Congenital heart disease is associated with delayed fetal brain development.•Macroscopic, microscopic and (angio)genic alterations are found in these placentas.•Impaired placenta might contribute to the delayed brain development.
Fetuses with congenital heart defects (CHD) show delayed neurodevelopment, fetal growth restriction (FGR) and placenta related complications. The neurodevelopmental delay may be, partly, attributed ...to placental factors.
As both placental development and fetal aortic flow/oxygenation influence neurodevelopment, placentas were compared within fetal CHD groups based on aortic oxygenation and flow, aiming to unravel the true effects in the developmental processes.
Placental tissues of pregnancies with fetal CHD and healthy controls were selected from biobanks of two Dutch academic hospitals (LUMC, UMCU). Additionally, biometry and Dopplers were assessed.
CHD cases with reduced oxygenation (RO) towards the fetal brain were compared to cases with reduced flow (RF) in the aortic arch and healthy controls. Genetic abnormalities, termination of pregnancy, fetal demise and/or multiple pregnancies were excluded.
Histological outcomes were related to fetal Dopplers and biometry. A placenta severity score was used to assess the severity of placental abnormalities per case.
In CHD, significantly more delayed maturation, maternal vascular malperfusion, fetal hypoxia and higher placenta severity scores (median 14 in RO, 14 in RF, 5 in controls, p < 0.001) were observed. Doppler abnormalities (PI UA > p90, PI MCA < p10, CPR < p10) and FGR were more often found in CHD. There were no differences in placental abnormalities, fetal growth and fetal Dopplers between cases with RO and RF.
Fetal hemodynamics in the ascending aorta could not be related to placenta characteristics. We hypothesize that placental development influences neurodevelopment in excess of hemodynamics in CHD cases.
•Placental features in fetal CHD could not be related to aortic flow and oxygenation.•Next to hemodynamic alterations, alternative contributing factors might play a role.•Abnormal placental development might influence neurodevelopment in these cases.
Objective
We aimed to assess current prenatal detection rate (DR) of aortic coarctation (CoA) and its impact on neonatal outcome in the Netherlands to evaluate the efficacy of the Dutch screening ...protocol in which the cardiac four‐chamber view, outflow tracts and three‐vessel view are compulsory.
Methods
All prenatally and postnatally diagnosed CoA cases between 2012 and 2021 were extracted from our PRECOR‐registry. Annual DRs were calculated with a focus on the trend over time and attributing factors for detection. Postnatal outcome was compared between prenatally detected and undetected cases.
Results
49/116 cases (42.2%) were detected prenatally. A higher chance of detection was found for cases with extracardiac malformations (71.4%; p = 0.001) and the more severe cases with an aortic arch hypoplasia and/or ventricular septal defect (63.2%; p = 0.001). Time‐trend analysis showed no improvement in DR over time (p = 0.33). Undetected cases presented with acute circulatory shock in 20.9% and were more likely to have severe lactic acidosis (p = 0.02) and impaired cardiac function (p < 0.001) before surgery.
Conclusion
Even in a well‐organized screening program, the DR of CoA still requires improvement, especially in isolated cases. The increased risk of severe lactic acidosis in undetected cases stresses the need for urgent additions to the current screening program, such as implementation of the three‐vessel trachea view and measurement of outflow tracts.
Key points
What's already known about this topic?
Aortic coarctation is known for low prenatal detection rates in prenatal screening programs.
A prenatal diagnosis decreases the chance of severe neonatal morbidity or death.
What does this study add?
Even in a well‐organized screening setting with regular quality monitoring, prenatal detection of aortic coarctation is still low, especially for isolated cases.
The addition of the three‐vessel trachea view and the measurement of the outflow tracts to a prenatal screening program are highly recommended.
Objectives
To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch ...(RAA).
Methods
Fetuses diagnosed with isolated RAA, defined as the absence of intra‐ or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR.
Results
In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false‐positive; therefore, the follow‐up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor.
Conclusions
Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.
Key points
What is already known about this topic?
The detection rate of a fetal right aortic arch (RAA) has increased after the introduction of the three‐vessel view (3VV) and three‐vessel trachea view (3VTV) to fetal cardiac screening.
Suspicion of fetal RAA may require advanced prenatal diagnostics and careful follow‐up as it can coexist with intra‐ and extracardiac anomalies and chromosomal anomalies such as 22q11 deletion.
An isolated RAA may be considered a benign condition, but sometimes requires surgical intervention due to respiratory or feeding problems.
What does this study add?
A surgical intervention was required in 10% and 25% of the infants with a prenatal diagnosis of isolated RAA in the first year and first 5 years of life, respectively.
A neonatal intervention for acute respiratory distress due to tracheal compression is rare, but may be required in cases with a double aortic arch (DAA).
Delivery in a hospital with neonatal intensive care facilities is advised in all cases of suspected DAA.
Aim
We compared neonatal deaths and end‐of‐life decisions in a neonatal intensive care unit (NICU) and paediatric intensive care unit (PICU) in a Dutch tertiary children's hospital.
Subjects
All 235 ...full‐term infants who died within 28 days of life between 2003 and 2013 in the NICU (n = 199) and PICU (n = 36) were retrospectively studied.
Results
The median length of stay was three days in the NICU and seven days in the PICU (p = 0.003). The main reasons for NICU stays were asphyxia (52.8%) and congenital malformations (42.2%), and in the PICU, they were congenital malformations (97.2%) and primarily cardiac problems (83.3%, p < 0.001). The median age of death was three days in the NICU and eight days in the PICU (p < 0.001), and mortality despite full intensive care treatment was 4.0% and 25.0%, respectively. Intensive treatment was discontinued because of poor survival chances in 25.1% of NICU and 52.8% of PICU cases (p < 0.001), and care was redirected because of expected poor quality of life in 70.9% and 22.2%, respectively.
Conclusion
Differences between the age at death and end‐of‐life decisions were found between full‐term infants in the NICU and PICU in the same children's hospital. Underlying disorders and doctors’ attitudes may have played a role.
Studies on long-term sequelae of gastroschisis are scarce. The limited data suggest increased abdominal complaints in young children. To provide proper counseling for both parents and patients, more ...information on long-term outcome is needed. This study aims to evaluate long-term outcome regarding GI function, gastroesophageal reflux (GER), health-related quality of life (HRQoL) and cosmetic satisfaction.
An observational longitudinal cohort study was performed. All patients (N = 43) born between 1982 and 2008 with gastroschisis that were admitted to the University Medical Centre Utrecht, Wilhelmina Children's Hospital were invited to fill in a survey. Data of included patients were compared to validated Dutch reference standards.
Fourteen patients responded to the survey. The median follow-up was 18 years. Abdominal pain on weekly basis was present in two patients (14%) and feeding difficulties were present in one patient. Presence of a complication during gastroschisis treatment led to more GI symptoms (80% versus 22%). One patient experienced moderate complaints of regurgitation or dyspepsia. Although the overall HRQoL was lower in teenage gastroschisis patients as compared to healthy controls (73/100 versus 83/100, respectively), we found no relevant difference in overall HRQoL in the other age groups. Seven patients (50%) were satisfied with the cosmetic result of the scar.
GI function and HRQoL in gastroschisis patients seem similar to healthy controls at adolescent and adult age. Complications during gastroschisis treatment led to an increase of abdominal complaints later in life. The surgical technique had no significant effect on the cosmetic results.
•Gastroschisis patients have normal gastro-intestinal functioning at adolescent and adult age.•Gastroschisis patients with complications during their treatment have more abdominal complaints later in life.•Health-related quality of life is favorable in gastroschisis patients at adolescent and adult age.•Half of the gastroschisis patients is satisfied with the cosmetic result of their scar.
•Fetal demise is more common in fetal congenital heart disease (CHD).•Cardiac failure is the most important cause of fetal demise in CHD.•Placental insufficiency is identified as another important ...cause of stillbirth.•Impaired vascular placental development in fetal CHD is suggested.
Congenital heart defects are the most common congenital anomaly. Despite the increasing survival of these children, there is still an increased incidence of fetal demise, frequently attributed to cardiac failure. Considering that abnormal placental development has been described in congenital heart disease, our hypothesis is that placental insufficiency may contribute to fetal death in congenital heart disease.
This study aimed to assess cases with fetal congenital heart disease and intrauterine demise, and analyze factors that are related to the demise.
All congenital heart disease cases diagnosed prenatally during the period January 2002 to January 2021 were selected from the regional prospective congenital heart disease registry, PRECOR. Multiple pregnancies and pregnancies with fetal trisomy 13 or 18, triploidy, and Turner's syndrome were excluded from the analysis, because fetal demise is attributed to the chromosomal abnormality in these cases. Cases were categorized into 4 groups based on the possible cause of fetal death as follows: cardiac failure, additional (genetic) diagnosis, placental insufficiency, and a group in which no cause was found. A separate analysis was performed for isolated congenital heart disease cases.
Of the 4806 cases in the PRECOR registry, 112 had fetal demise, of which 43 were excluded from the analysis (13 multiple pregnancies, 30 genetic). Of these, 47.8% were most likely related to cardiac failure, 42.0% to another (genetic) diagnosis, and 10.1% to placental insufficiency. No cases were allocated to the group with an unknown cause. Only 47.8% of the cases had isolated congenital heart disease, and in this group 21.2% was most likely related to placental insufficiency.
This study shows that in addition to cardiac failure and other (genetic) diagnoses, placental factors play an important role in fetal demise in congenital heart disease, especially in cases of isolated heart defects. Therefore, these findings support the importance of regular ultrasonographic assessment of fetal growth and placental function in fetal congenital heart disease.
Neonates with congenital heart disease are at risk for impaired brain development in utero, predisposing children to postnatal brain injury and adverse long-term neurodevelopmental outcomes. Given ...the vital role of the placenta in fetal growth, we assessed the incidence of placental pathology in fetal congenital heart disease and explored its association with total and regional brain volumes, gyrification, and brain injury after birth.
Placentas from 96 term singleton pregnancies with severe fetal congenital heart disease were prospectively analyzed for macroscopic and microscopic pathology. We applied a placental pathology severity score to relate placental abnormalities to neurological outcome. Postnatal, presurgical magnetic resonance imaging was used to analyze brain volumes, gyrification, and brain injuries. Placental analyses revealed the following abnormalities: maternal vascular malperfusion lesions in 46%, nucleated red blood cells in 37%, chronic inflammatory lesions in 35%, delayed maturation in 30%, and placental weight below the 10th percentile in 28%. Severity of placental pathology was negatively correlated with cortical gray matter, deep gray matter, brainstem, cerebellar, and total brain volumes (
=-0.25 to -0.31, all
<0.05). When correcting for postmenstrual age at magnetic resonance imaging in linear regression, this association remained significant for cortical gray matter, cerebellar, and total brain volume (adjusted
=0.25-0.47, all
<0.05).
Placental pathology occurs frequently in neonates with severe congenital heart disease and may contribute to impaired brain development, indicated by the association between placental pathology severity and reductions in postnatal cortical, cerebellar, and total brain volumes.
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