Critical Congenital Heart Disease Research Articles

Prenatal Diagnosis Rate of Critical Congenital Heart Disease Remains Inadequate with Significant Racial/Ethnic and Socioeconomic Disparities and Technical Barriers.

Prenatal diagnosis (preDx) of critical congenital heart disease (CCHD) decreases neonatal morbidity and mortality. Obstetrical fetal cardiac imaging guidelines in 2013 aimed to increase preDx. The objectives of this study were to determine the contemporary preDx rate of CCHD and identify maternal-fetal factors and variations in prenatal care that may be potential barriers. This retrospective single center study evaluated maternal demographics and characteristics of infants with CCHD (requiring cardiac catheterization or surgical intervention before 6months-old) between 2016 and 2019. 58% of the 339 infants with CCHD had preDx. Infants with preDx were more likely to have mothers ≥ 35years-old (p = 0.028), family history of CHD (p = 0.017), health insurance (p = 0.002), or anatomic scan with perinatology (p < 0.001). Hispanic infants were less likely to have preDx (45.6%, p = 0.005). PreDx rates were higher in infants with extracardiac/genetic anomalies (p < 0.001) and significantly different between CCHD subtypes (76% for single ventricle, 51% for biventricular/four-chamber view, 59% for proximal outflow tract anomalies, and 48% for distal great artery anomalies; p = 0.024). In infants without preDx, 25% of their mothers had indication for, but did not undergo, fetal echocardiography. PreDx rates of CCHD remains inadequate across subtypes detectable by standard fetal cardiac screening views, particularly in uninsured and Hispanic communities.

High risk of necrotising enterocolitis in term-born neonates with CHD delivered by caesarean section: a case-control study.

Necrotising enterocolitis is linked with altered intestinal microbiota, and caesarean birth is associated with imbalance of newborn intestinal microbiome. We aimed to investigate the role of delivery mode (vaginal or caesarean) and gestational age in the development of necrotising enterocolitis among term-born neonates (≥ 37 weeks) with CHD. Case-control study. We studied all newborns with CHD who underwent cardiac surgery during the neonatal (≤ 28 days of age) period, between 2007 and 2017. Totally, 60 cases of necrotising enterocolitis were matched (by year of birth and type of congenital heart lesion) with 180 controls (1:3 ratio). Multivariable conditional logistic regression was used to assess the study question. The overall prevalence of necrotising enterocolitis was 6.3% in term-born newborns with CHD. Neonates with a left-ventricular outflow tract lesion or single ventricle lesion accounted for 55% (n = 33) of cases. 62% (n = 37) cases were in the modified Bell's stage 2 or more for necrotising enterocolitis classification. In multivariable modelling, gestational age at birth was not associated with the development of necrotising enterocolitis [adjusted odds ratio per week increase, 95% confidence interval: 1.20 (0.90-1.60)]. Birth by caesarean delivery (compared to vaginal) was strongly associated with development of necrotising enterocolitis [adjusted odds ratio (95% confidence interval): 2.64 (1.31-5.29)]. We failed to identify an association between preoperative enteral nutrition and necrotising enterocolitis. This study showed a high risk of necrotising enterocolitis in newborns with critical CHD born via caesarean. This information is important given the high prevalence of planned birth by caesarean in newborns with CHD.

Commentary on “Brain Injury During Transition in the Newborn With Congenital Heart Disease: Hazards of the Preoperative Period”

BRAIN INJURY DURING TRANSITION IN THE NEWBORN WITH CONGENITAL HEART DISEASE: HAZARDS OF THE PREOPERATIVE PERIOD: Jennifer M. Lynch, J. William Gaynor, Daniel J. Licht Seminars in Pediatric Neurology Volume 28, December 2018, Pages 60-65 Infants born with critical congenital heart disease are at risk for neurodevelopmental morbidities later in life. In-utero differences in fetal circulation lead to vulnerabilities which lead to an increased incidence of stroke, white matter injury, and brain immaturity. Recent work has shown these infants may be most vulnerable to brain injury during the early neonatal period when they are awaiting their cardiac surgeries. Novel imaging and monitoring modalities are being employed to investigate this crucial time period and elucidate the precise timing and cause of brain injury in this population.

Electroencephalography as a tool to predict cerebral oxygen metabolism during deep-hypothermic circulatory arrest in neonates with critical congenital heart disease

ObjectivesRecent research suggests that increased cerebral oxygen use during surgical intervention for neonates with congenital heart disease may play a role in the development of postoperative white matter injury. The objective of this study is to determine whether increased cerebral electrical activity correlates with greater decrease of cerebral oxygen saturation during deep hypothermic circulatory arrest. MethodsNeonates with critical congenital heart disease requiring surgical intervention during the first week of life were studied. All subjects had continuous neuromonitoring with electroencephalography and an optical probe (to quantify cerebral oxygen saturation) during cardiac surgical repair that involved the use of cardiopulmonary bypass and deep hypothermic circulatory arrest. A simple linear regression was used to investigate the association between electroencephalography metrics before the deep hypothermic circulatory arrest period and the change in cerebral oxygen saturation during the deep hypothermic circulatory arrest period. ResultsSixteen neonates had both neuromonitoring modalities attached during surgical repair. Cerebral oxygen saturation data from 5 subjects were excluded due to poor data quality, yielding a total sample of 11 neonates. A simple linear regression model found that the presence of electroencephalography activity at the end of cooling is positively associated with the decrease in cerebral oxygen saturation that occurs during deep hypothermic circulatory arrest (P < .05). ConclusionsElectroencephalography characteristics within 5 minutes before the initiation of deep hypothermic circulatory arrest may be useful in predicting the decrease in cerebral oxygen saturation that occurs during deep hypothermic circulatory arrest. Electroencephalography may be an important tool for guiding cooling and the initiation of circulatory arrest to potentially decrease the prevalence of new white matter injury in neonates with critical congenital heart disease.

A multicenter study on two-stage transfer learning model for duct-dependent CHDs screening in fetal echocardiography

Duct-dependent congenital heart diseases (CHDs) are a serious form of CHD with a low detection rate, especially in underdeveloped countries and areas. Although existing studies have developed models for fetal heart structure identification, there is a lack of comprehensive evaluation of the long axis of the aorta. In this study, a total of 6698 images and 48 videos are collected to develop and test a two-stage deep transfer learning model named DDCHD-DenseNet for screening critical duct-dependent CHDs. The model achieves a sensitivity of 0.973, 0.843, 0.769, and 0.759, and a specificity of 0.985, 0.967, 0.956, and 0.759, respectively, on the four multicenter test sets. It is expected to be employed as a potential automatic screening tool for hierarchical care and computer-aided diagnosis. Our two-stage strategy effectively improves the robustness of the model and can be extended to screen for other fetal heart development defects.

Fetal electrocardiography and artificial intelligence for prenatal detection of congenital heart disease.

This study aims to investigate non-invasive electrocardiography as a method for the detection of congenital heart disease (CHD) with the help of artificial intelligence. An artificial neural network was trained for the identification of CHD using non-invasively obtained fetal electrocardiograms. With the help of a Bayesian updating rule, multiple electrocardiographs were used to increase the algorithm's performance. Using 122 measurements containing 65 healthy and 57 CHD cases, the accuracy, sensitivity, and specificity were found to be 71%, 63%, and 77%, respectively. The sensitivity was however 75% and 69% for CHD cases requiring an intervention in the neonatal period and first year of life, respectively. Furthermore, a positive effect of measurement length on the detection performance was observed, reaching optimal performance when using 14 electrocardiography segments (37.5 min) or more. A small negative trend between gestational age and accuracy was found. The proposed method combining recent advances in obtaining non-invasive fetal electrocardiography with artificial intelligence for the automatic detection of CHD achieved a detection rate of 63% for all CHD and 75% for critical CHD. This feasibility study shows that detection rates of CHD might improve by using electrocardiography-based screening complementary to the standard ultrasound-based screening. More research is required to improve performance and determine the benefits to clinical practice.

Perioperative brain injury marker concentrations in neonatal open-heart surgery: a prospective observational study.

Neonates with critical congenital heart defects undergoing open-heart surgery on cardiopulmonary bypass circulation are at risk for white matter brain injury. This article reports on pre- and postoperative plasma concentrations of brain injury markers glial fibrillary acidic protein (GFAP), neurofilament light (NfL) and Tau, and their respective associations with white matter lesions detected on postoperatively performed brain MRI. Forty term newborns with isolated critical congenital heart defects were included in a prospective observational study. Brain injury marker plasma concentrations were determined prior to surgery and at postoperative days 1, 2 and 3. Brain magnetic resonance imaging was performed pre- and postoperatively. Concentrations of brain injury markers were analysed using ultrasensitive single molecule array technology. Absolute pre- and postoperative plasma biomarker concentrations, and postoperative concentrations adjusted for preoperative concentrations were used for subsequent analysis. Plasma concentrations of GFAP, NfL and Tau displayed a well-defined temporal trajectory after neonatal cardiopulmonary bypass circulation. GFAP and Tau reached peak concentrations at postoperative day 2 (median concentrations 170.5 and 67.2 pg/ml, respectively), whereas NfL continued to increase throughout the study period (median concentration at postoperative day 3 191.5 pg/ml). Adjusted Tau at postoperative day 2 was significantly higher in infants presenting with white matter lesions on postoperative MRI compared to infants without white matter injury.

Role of Pulse Oximetry as a Screening Tool for the Detection of Congenital Heart Disease in Newborn Babies

Background: Congenital heart disease (CHD) is one of the commonly seen malformations, with incidence varying from 7 to 8/1000 live birth. Causes are multifactorial. Routine examination of neonates may miss more than 50% of cases. Pulse oximetry is a simple, noninvasive, bedside test which estimates the percentage of oxygen bound to hemoglobin (oxygen saturation [SpO2]). Detection of critical CHD (CCHD) has been possible with SpO2 screening. Many countries included SpO2 as part of newborn screening due to this. As a primary approach, both pre- and post-ductal extremity SpO2 is measured after 24 h of life. Echocardiography (ECHO) will be done on neonates with SpO2 readings &lt;95%. Neonates born in high-altitude regions, studies have suggest to use adjusted threshold values. In India, there are limited studies. Aims: The aim of this study was to determine the usefulness of pulse oximetry as a screening tool for early detection of CHD in otherwise asymptomatic newborns. To determine, the accuracy of SpO2 for detecting clinically unrecognized CCHD in newborns. Materials and Methods: This is a prospective observational study done in the department of pediatrics in a tertiary hospital. The study was conducted over 12 months. During the study period, all neonates born who fulfilled the inclusion criteria were included in the study. After 24 h of life, neonates were examined clinically and the pre- and post-ductal SpO2 was measured. Neonates with SpO2 &lt;90% in room air were excluded from the study. If the SpO2 was between 90% and 94%, clinical examination was repeated, if suspicious of CHD, they were referred for ECHO. If there was no suspicion of CHD, SpO2 was repeated after 6 h and ECHO was done if SpO2 ≤95. The difference of SpO2 &gt;3% between the right upper limb and right lower limb was considered positive. Positive neonates were evaluated with two-dimensional echocardiograph. Inclusion criteria - All hemodynamically stable neonates were born during the study period. Exclusion criteria (1) Antenatally diagnosed cardiac anomalies, (2) Outborn neonates, (3) Parents/guardians are not willing to participate in the study and/or further investigation, (4) Sick neonates and those with SpO2 &lt;90% at birth. Results: 1117 (83.7%) were eligible for the study out of 1333 neonates born. 669 (59.9%) were born by cesarean section and 448 (40.1%) by vaginal delivery. 996 (89.2%) were born at term (≥37 weeks of gestation) and 121 (10.8%) were preterm (&lt;37 weeks of gestation). The male-to-female ratio was 1.03:1. The mean birth weight of the neonates was 2.91 ± 0.46 kg (mean ± standard deviation). The mean SPO2 in the right upper limb was 96.62 ± 1.73, in the right lower limb was 96.87 ± 1.76, in the left upper limb was 96.59 ± 1.90, and in the left lower limb was 97.06 ± 1.74. The average SPO2 difference between the right upper limb and right lower limb was 1.04 ± 1.07. Based on SpO2, 858 (76.8%) cases were not suspected of having CHD and 259 (23.3%) were suspected of having CHD and were evaluated with ECHO. Six (0.5%) neonates had CHD in whom echo was done. In our study, for detecting CCHD, SPO2 cutoff value of ≤90% showed 90% sensitivity, 99.94% specificity, 75% positive predictive value, and 99.98% negative predictive value. Conclusions: This study emphasizes noninvasive SPO2 as reliable and feasible, with good negative predictive value screening for CHD in neonates.

Association Between Prenatal Diagnosis and Age at Surgery for Noncritical and Critical Congenital Heart Defects.

The relationship between the prenatal diagnosis of congenital heart defects (CHDs) and age at CHD surgery is poorly understood, despite the known relationships between age at surgery and long-term outcomes. The objective of this study was to determine the associations between prenatal diagnosis of CHD and age at surgery, and whether these associations differ for critical and noncritical CHDs. This is a cohort analysis of patients aged 0 to 9 years who received their initial cardiac surgery at Ann & Robert H. Lurie Children's Hospital of Chicago between 2015 and 2021 with prenatal diagnosis as the exposure variable. All data were obtained from the locally maintained Society of Thoracic Surgeons Congenital Heart Surgery Database at Lurie Children's Hospital. We used multivariable fixed effects regression models to estimate the strength of the association of prenatal diagnosis with age at surgery among patients with critical (surgery ≤60 days) and noncritical (surgery >60 days) CHDs. Of 1131 individuals who met inclusion criteria, 532 (47%) had a prenatal diagnosis, 428 (38%) had critical CHDs, 533 (47%) were female, and the median age at surgery was 119 days (interquartile range, 11-309 days). After controlling for demographics, comorbidities, and surgical complexity, the mean age at surgery was significantly younger in those with prenatally versus postnatally diagnosed critical CHD (7.1 days sooner, P<0.001) and noncritical CHDs (atrial septal defects [12.4 months sooner, P=0.037], ventricular septal defects [6.0 months sooner, P<0.003], and noncritical coarctation of the aorta [1.8 months sooner, P=0.010]). Younger age at CHD surgery, which is associated with postsurgical neurodevelopmental and physical outcomes, is significantly associated with prenatal CHD diagnosis. This relationship was identified for both critical and noncritical CHDs.

Prognosis and outcome of intrauterine treatment of fetuses with critical congenital heart disease.

Intrauterine valvuloplasty is an innovative therapy, which promotes ventricular growth and function in some congenital heart diseases (CHDs). The technique remains challenging and can only be performed in a few centers. This study aimed to assess the feasibility and mid-term outcomes of fetal cardiac intervention (FCI) in fetuses with critical CHD in an experienced tertiary center. Five fetal aortic valvuloplasty (FAV) or fetal pulmonary valvuloplasty (FPV) procedures were performed in our fetal heart center between August 2018 and May 2022. Technical success was defined as crossing the aortic or pulmonary valve and balloon inflation, followed by evidence of increased blood flow across the valve and/or new regurgitation. Follow-up clinical records and echocardiography were obtained during the prenatal and postnatal periods. Five fetuses received FAV or FPV, including critical aortic stenosis ( n = 2) and pulmonary atresia with intact ventricular septum ( n = 3). The mean maternal age was 33.0 ± 2.6 years. The median gestational age (GA) at diagnosis was 24 weeks (range, 22-26 weeks). The median GA at intervention was 29 weeks (range, 28-32 weeks). All five cases underwent successful or partially successful procedures. One patient had pulmonary valve perforation without balloon dilation. No procedure-related deaths or significant complications occurred. However, one neonatal death occurred due to heart and renal failure. The median follow-up period was 29.5 months (range, 8.0-48.0 months). The four surviving patients had achieved biventricular circulation, exhibited improved valve, and ventricular development at the last follow-up visit. Intrauterine FCI could be performed safely with good prognosis in critical CHD.

The Construction of an Intrauterine Diagnosis and Treatment System and Comprehensive Lifecycle Health Service of Congenital Heart Disease: Xinhua Hospital Model

With the growing influence of slow population growth and population aging, China has established the birth policy and issued a series of documents to promote maternal and fetal health and improve the birth rate. With the increase in prevalence of birth defects, timely diagnosis and intervention in utero provide possibilities to reduce unnecessary abortions and offer better prognosis. Congenital heart disease (CHD), as one of the most common congenital birth defects, is the leading cause of mortality in patients aged &lt;5 years, and brings a heavy burden to both the affected families and society. Fetuses with CHD are associated with an increased risk of pregnancy-related complications and premature birth, and children with CHD typically face growth and developmental problems even after the correction of malformation. Therefore, management including diagnosis, treatment, and rehabilitation throughout the fetal period into childhood and even adulthood is essential for children with CHD. Based on the rapid advances in intrauterine and perinatal medicine and an in-depth collaboration among obstetrics and pediatrics, a novel diagnosis and treatment system has been established for the management of CHD in the past 2 decades in Shanghai Xinhua Hospital. This Intrauterine Diagnosis and Treatment System and Comprehensive Lifecycle Health Service of Congenital Heart Disease model provides prenatal diagnosis, intrauterine intervention, delivery room service and neonatal therapies, and postintrauterine rehabilitation for children with CHD. We have developed a four-dimensional spatiotemporal image correlation echocardiography and a three-dimensional cardiac virtual endoscopy system for the intrauterine diagnosis of CHD, dramatically raising the diagnostic utility. Our innovative and independent newborn-intervention technique has effectively reduced the re-intervention rate in patients with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. In 2018, Xinhua Hospital independently performed the case of fetal aortic valvuloplasty in Asia through a multidepartment collaborative effort. All children treated in this system achieved biventricular circulation and a better long-term postoperative outcome. We also have conducted postoperative rehabilitation therapy to promote the development and health of children with CHD. The practice of Xinhua model has reduced unnecessary abortion of CHD fetuses, reduced the mortality rate associated with critical CHD, and improved the mid- and long-term prognosis in CHD, which is essential to promote the fertility level and children’s health. Furthermore, translational medicine platform and the birth cohort Early Life Plan was constructed to explore the origins of major developmental diseases and establish an early intervention model in CHD. This practice of assessment of the intrauterine system has been expanded to other congenital defects in Xinhua Hospital, and sequential treatment of more than 2,000 cases has been completed to date. Based on practice in intrauterine management of CHD and other diseases, the concept of Intrauterine Pediatrics was proposed as a first to emphasize early prevention and intervention of childhood diseases and promote a comprehensive lifecycle service for children. The development and evolution of this system requires further attention not only from researchers but also from the government and global medical communities.

Utility of Screening Fetal Echocardiograms at aSingle Institution Following Normal Obstetric Ultrasound in Fetuses with Down Syndrome.

Fetal echocardiograms (F-echo) are recommended in all pregnancies when the fetus has Down syndrome (DS) even if there was a prior obstetric scan (OB-scan) that was normal. The utility of a screening F-echo in this high-risk population when an OB-scan is normal is unknown. Goal of this study was to evaluate if any diagnosis of a critical congenital heart disease (CHD) was missed in a fetus with DS who had a normal OB-scan. Secondary goal was to determine if any CHD was missed postnatally when an OB-scan was read as normal. Retrospective chart review of all fetuses that had a F-echo whose indication was DS between 1/1/2010 to 6/30/2022 was performed. Fetuses were included if they had an OB-scan that was read as normal and had a F-echo. Postnatal transthoracic echocardiogram (pTTE) was reviewed when available. Critical CHD was defined as CHD requiring catheterization or surgical intervention < 1month of age. One hundred twenty-two F-echo on fetuses with DS were evaluated, of which 48 met inclusion criteria. OB-scan was performed at 20.4 ± 4.5weeks gestational age and F-echo was performed at 24.0 ± 4.6weeks gestational age. No patient with a normal OB-scan had a diagnosis of a critical CHD by F-echo (n = 48, negative predictive value = 100%). Evaluating those patients that had an OB-scan and a pTTE (n = 38), 14 patients were diagnosed with CHD (muscular ventricular septal defect (VSD) n = 5, perimembraneous VSD n = 3, secundum atrial septal defect (ASD) n = 2, primum ASD n = 1, transitional atrioventricular septal defect (AVSD) n = 2, and aortic valve abnormality n = 1; negative predictive value = 63.2%). F-echo correctly diagnosed 4 of the 14 missed OB-scan CHD (perimembraneous VSD n = 2, muscular VSD n = 1, and transitional AVSD n = 1). Critical CHD was not missed with a normal OB-scan in this high-risk population. F-echo also missed the majority of CHD when an OB-scan was read as normal. The cost/benefit of screening F-echo in fetuses with DS should be evaluated if a normal OB-scan has been performed, considering all these patients would have a pTTE performed per guidelines.

Outcomes of neonatal critical congenital heart disease: results of a prospective registry-based study from South India

ObjectivesCongenital heart disease (CHD) is now a leading contributor of infant and neonatal mortality in many low/middle-income countries including India. We established a prospective neonatal heart disease registry in Kerala...

Impact of Prenatal Diagnosis of Critical Congenital Heart Disease on Preoperative and Postoperative Outcomes.

The objective of this study was to assess the relationship of prenatal diagnosis of critical congenital heart disease (CHD) to preoperative and postoperative patient findings. Retrospective analysis of neonates with critical CHD who underwent cardiothoracic surgery at one of four centers in North Carolina between 2008 and 2013. Surgical data collected by sites for submission to the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the North Carolina CHD Lifespan Database were queried. There were 715 patients with STS records; 558 linked to the NC-CHD database. Patients with prenatal diagnosis had a lower incidence of preoperative risk factors, including need for mechanical ventilation and presence of shock. However, prenatally diagnosed patients had worse short-term outcomes, including higher operative mortality, higher incidence of select postoperative complications, and longer LOS. There was no difference in one-year mortality. Our findings are consistent with current literature which suggests that prenatal diagnosis of critical CHD is associated with a more optimized preoperative clinical status. However, we found that patients with prenatal diagnoses had less favorable postoperative outcomes. This needs to be investigated further, but may be secondary to patient-specific factors, such as CHD disease severity.

Neonatal Depression Is Associated With 1-Year Mortality in Critical Congenital Heart Disease.

Background Low 5-minute Apgar scores (AS) are predictive of term and preterm neonatal mortality but have not been well studied in the critical congenital heart disease (CCHD) population. We analyzed US national vital statistics data to evaluate the association between neonatal depression (AS 0-3) and 1-year mortality in CCHD. Methods and Results We performed a retrospective cohort study using 2014 to 2018 Centers for Disease Control and Prevention cohort-linked birth certificate and infant death records. Five-minute AS were categorized as ≤3, 4 to 6, or ≥7. We calculated birth rates and associated mortality rates by AS group in infants with and without CCHD. Multivariable logistic regression analyzed neonatal, maternal, and pregnancy-related risk factors for neonatal depression and 1-year mortality. Of 11 642 neonates with CCHD (0.06% of all births), the 5.8% with AS 0 to 3 accounted for 23.3% of all 1-year CCHD mortality, with 69.9% of deaths occurring within 1 month of life. Gestational age at birth, growth restriction, extracardiac defects, race, and low maternal education were associated with an increased odds of AS 0 to 3 in neonates with CCHD relative to those with AS 7 to 10 on multivariable analysis. AS 0 to 3 was associated with 1-year CCHD mortality after adjusting for these factors, prenatal care, and delivery location (adjusted odds ratio, 14.57 [95% CI, 11.73-18.10]). Conclusions The AS is a routine clinical measure providing important prognostic information in CCHD. These findings suggest that prenatal and perinatal factors, beyond those included in current risk stratification tools, are important for CCHD outcomes. Multidisciplinary collaboration to understand the pathophysiology underlying neonatal depression may help identify interventions to improve CCHD mortality rates.

Continuous Data-Driven Monitoring in Critical Congenital Heart Disease: Clinical Deterioration Model Development.

Critical congenital heart disease (cCHD)-requiring cardiac intervention in the first year of life for survival-occurs globally in 2-3 of every 1000 live births. In the critical perioperative period, intensive multimodal monitoring at a pediatric intensive care unit (PICU) is warranted, as their organs-especially the brain-may be severely injured due to hemodynamic and respiratory events. These 24/7 clinical data streams yield large quantities of high-frequency data, which are challenging in terms of interpretation due to the varying and dynamic physiology innate to cCHD. Through advanced data science algorithms, these dynamic data can be condensed into comprehensible information, reducing the cognitive load on the medical team and providing data-driven monitoring support through automated detection of clinical deterioration, which may facilitate timely intervention. This study aimed to develop a clinical deterioration detection algorithm for PICU patients with cCHD. Retrospectively, synchronous per-second data of cerebral regional oxygen saturation (rSO2) and 4 vital parameters (respiratory rate, heart rate, oxygen saturation, and invasive mean blood pressure) in neonates with cCHD admitted to the University Medical Center Utrecht, the Netherlands, between 2002 and 2018 were extracted. Patients were stratified based on mean oxygen saturation during admission to account for physiological differences between acyanotic and cyanotic cCHD. Each subset was used to train our algorithm in classifying data as either stable, unstable, or sensor dysfunction. The algorithm was designed to detect combinations of parameters abnormal to the stratified subpopulation and significant deviations from the patient's unique baseline, which were further analyzed to distinguish clinical improvement from deterioration. Novel data were used for testing, visualized in detail, and internally validated by pediatric intensivists. A retrospective query yielded 4600 hours and 209 hours of per-second data in 78 and 10 neonates for, respectively, training and testing purposes. During testing, stable episodes occurred 153 times, of which 134 (88%) were correctly detected. Unstable episodes were correctly noted in 46 of 57 (81%) observed episodes. Twelve expert-confirmed unstable episodes were missed in testing. Time-percentual accuracy was 93% and 77% for, respectively, stable and unstable episodes. A total of 138 sensorial dysfunctions were detected, of which 130 (94%) were correct. In this proof-of-concept study, a clinical deterioration detection algorithm was developed and retrospectively evaluated to classify clinical stability and instability, achieving reasonable performance considering the heterogeneous population of neonates with cCHD. Combined analysis of baseline (ie, patient-specific) deviations and simultaneous parameter-shifting (ie, population-specific) proofs would be promising with respect to enhancing applicability to heterogeneous critically ill pediatric populations. After prospective validation, the current-and comparable-models may, in the future, be used in the automated detection of clinical deterioration and eventually provide data-driven monitoring support to the medical team, allowing for timely intervention.

A Novel, Multi-Directional Framework for Transitioning a Child with Critical Congenital Heart Disease Between Home and Hospital with Concurrent Hospice Services (FR215B)

A Novel, Multi-Directional Framework for Transitioning a Child with Critical Congenital Heart Disease Between Home and Hospital with Concurrent Hospice Services (FR215B)

Study on the value of the prenatal-postnatal integrated management model in the screening, diagnosis, and treatment of fetal heart malformations.

With the development and widespread use of fetal ultrasound and magnetic resonance technology in recent years, approximately 75% of fetuses are diagnosed prenatally with congenital structural malformations, a serious birth defect that endangers the life and health of the newborn. In this study, we aimed to study and analyze the value of the prenatal-postnatal integrated management model in the screening, diagnosis and treatment of fetal heart malformations. All pregnant women who were to undergo delivery in our hospital between January 2018 and December 2021 were recruited as the first subjects in this study, and after excluding those who refused to participate in the study, a total of 3,238 cases were finally included as subjects of this study. All pregnant women were screened for fetal heart malformations using the prenatal-postnatal integrated management model. Maternal files were established for all cases of heart malformations, grading the fetuses according to their heart disease grade, observing and recording their deliveries, treatment results and follow-ups. After screening for heart malformations using the prenatal-postnatal integrated management model, 33 cases of heart malformations were identified, including 5 cases of Grade I (all delivered), 6 cases of Grade II (all delivered), 10 cases of Grade III (1 induced), and 12 cases of Grade IV (1 induced); 2 cases of ventricular septal defect healed spontaneously after delivery, and 18 infants were treated accordingly. The results of the later follow-up showed that 10 children had normalized their heart structure, 7 cases had slight alterations in the heart valves, and 1 case died. The prenatal-postnatal integrated management model is a multidisciplinary cooperation model with certain clinical value in the screening, diagnosis and treatment of fetal heart malformations, which is beneficial to comprehensively improve the ability of hospital physicians in the grading management of heart malformations, detecting fetal heart malformations early and predicting fetal changes after birth. It further reduces the incidence of serious birth defects, conforms to the development trend of the diagnosis and treatment of congenital heart diseases, enables to reduce child mortality with timely treatment, effectively improves the surgical prognosis of critical and complex congenital heart diseases, with a promising application prospect.

Implementation of Newborn Screening for Conditions in the United States First Recommended during 2010-2018.

The Recommended Uniform Screening Panel (RUSP) is the list of conditions recommended by the US Secretary of Health and Human Services for inclusion in state newborn screening (NBS). During 2010-2022, seven conditions were added to the RUSP: severe combined immunodeficiency (SCID) (2010), critical congenital heart disease (CCHD) (2011), glycogen storage disease, type II (Pompe) (2015), mucopolysaccharidosis, type I (MPS I) (2016), X-linked adrenoleukodystrophy (X-ALD) (2016), spinal muscular atrophy (SMA) (2018), and mucopolysaccharidosis, type II (MPS II) (2022). The adoption of SCID and CCHD newborn screening by programs in all 50 states and three territories (Washington, D.C.; Guam; and Puerto Rico) took 8.6 and 6.8 years, respectively. As of December 2022, 37 programs screen for Pompe, 34 for MPS I, 32 for X-ALD, and 48 for SMA. The pace of implementation based on the average additional number of NBS programs per year was most rapid for SMA (11.3), followed by CCHD (7.8), SCID (6.2), MPS I (5.4), Pompe (4.9), and X-ALD (4.7).

Multicentre study protocol comparing standard NRP to deveLoped Educational Modules for Resuscitation of Neonates in the Delivery Room with Congenital Heart Disease (LEARN-CHD)

IntroductionInfants born with critical congenital heart defects (CCHDs) have unique transitional pathophysiology that often requires special resuscitation and management considerations in the delivery room (DR). While much is known about...