Critical Congenital Heart Disease Research Articles

Abstract 4143228: Uncovering Hidden Disparities in Surgical Mortality among Texas Medicaid-Insured Infants with Critical Congenital Heart Disease

Background: Data shows that publicly insured patients with critical congenital heart disease (CCHD) have higher surgical mortality than privately insured patients. However, little is known about disparities within Medicaid-insured cohorts. We hypothesized that disparities in surgical mortality exist within the Medicaid population, with the highest mortality being among the most socially vulnerable patients. Methods: We conducted a retrospective cohort study using Texas Birth Defects Registry records of infants with CCHD born 2010-2014 who underwent cardiac surgery <1 year old (CCHDs). Patients with CCHD as defined by the Centers for Disease Control or ductal dependent anatomy were included, and records for study inclusion were reviewed by a pediatric cardiologist. Cases were linked with death certificates and the Medicaid database, and Medicaid patients were stratified into Primary (PM) and Secondary (SM) cohorts. The PM cohort was comprised of patients with the highest scores (most socioeconomically vulnerable) based on the census tract derived Social Vulnerability Index (SVI). The primary predictor was race/ethnicity and primary outcome was infant mortality. Descriptive sociodemographics were analyzed using chi-square. Infant mortality was analyzed using stratified univariable Cox Proportional-Hazards models. Results: We identified 2525 infants with CCHD S , of whom 14.5% had PM and 56.9% had SM. The Medicaid-cohort had more single ventricle, chromosomal, and extracardiac defects than the Non-Medicaid (NM) cohort (p<0.0001). Compared to the SM cohort, CCHD S infants with PM were more likely to be born preterm, and be born to mothers who are non-White, have < a high school education (p<0.0001, respectively), and who drove further to surgical facility (p<0.005). Cox regression showed no racial/ethnic mortality disparities in the PM cohort. In the SM cohort, infant mortality was 2.2x higher among Black patients (HR 2.21, CI 1.41-3.45). In the NM cohort, Hispanic patients had nearly 2x (HR 1.93, CI 1.12-3.33) and Black patients had 3x (HR 3.01, CI 1.42-5.96) higher risk of infant mortality than White CCHDs infants. Conclusions: We completed one of the largest analyses of Medicaid-insured CCHD S infants using a novel SVI stratification. Despite worse SVI, racial/ethnic disparities in CCHD S mortality were not observed among PM-insured patients. Future database work should include stratified Medicaid populations to accurately interpret outcomes in patients with CCHD.

Abstract 4135333: Peripheral Perfusion Index: Normative Values From Electronically Reported Critical Congenital Heart Disease Screenin

Background: Critical Congenital Heart Disease (CCHD) screening is performed in an effort to reduce morbidity and mortality associated with CCHD by facilitating early detection. This screening consists of pulse oximetry and is most effective at identification of cyanotic cardiac lesions, with less sensitivity for left sided obstructive lesions. Research: Question Peripheral perfusion index (PPI) is obtained from the plethysmographic signal of a pulse oximeter and represents the ratio of pulsatile to non-pulsatile blood flow. As such, it is a surrogate for peripheral perfusion at the site of measurement. PPI has been postulated to be an adjunctive screening method for low perfusion states in neonates, including left sided obstructive lesions. To date, reference ranges of PPI values in neonates have not been published in the United States. This project utilized electronically reported CCHD screening results to establish normative PPI data in healthy neonates. Methods: In Minnesota, CCHD screening results are electronically sent to the Minnesota Department of Health (MDH) and are cross referenced against the Minnesota Birth Information System, which tracks infants who are identified to have a congenital birth defect within the first year of life. These data were provided by the MDH for infants born in Minnesota from Jan 2017 through Dec 2022. Infants were included in the analysis if they did not have a known critical congenital heart defect, were born between 35 and 42 weeks gestation, and were 24-48 hours of life at time of screening. Results: PPI data from 11,179 cases were included in the analysis. Overall, there is a trend towards increasing PPI with gestational age. Pre-ductal PPI trended higher than post-ductal PPI. There was no difference between PPI in males and females. Conclusions: These data reflect trends identified in international data. This is the largest dataset to date of PPI in healthy neonates and the first in the United States. Future studies will compare neonates with left sided obstructive lesions against these normative data. If PPI is found to predict left sided obstructive lesions in neonates, there may be utility in use of PPI in CCHD screening to facilitate earlier detection of these lesions.

Abstract 4139179: Is Prenatal Diagnosis Responsible for Differences in Timing to Postnatal Intervention for Critical Congenital Heart Defects?

Introduction: The relationships between prenatal diagnosis of congenital heart defects (CHDs) and outcomes are inconsistent. Some studies have shown that compared to postnatal diagnosis, prenatal diagnosis is associated with later surgical timing and worse outcomes. These findings could be due to confounding because more severe CHDs are likelier to be diagnosed on prenatal ultrasonography. To bypass this confounding, we evaluated prenatal diagnosis as a mediator. Aim: To evaluate whether prenatal diagnosis of CHD mediates the association between disease severity and timing to postnatal intervention. Hypothesis: Infants with severe CHD receive intervention sooner than those with mild CHD due to a higher prenatal diagnosis prevalence. Methods: This is a retrospective cohort study of all infants who received their initial cardiac catheterization or surgery between 0-60 days (i.e., critical CHD) at Ann&Robert H. Lurie Children’s Hospital of Chicago (2015-2021). Birthing individuals with inadequate prenatal care were excluded. The primary exposure variable was a three-level categorical variable derived from existing literature, categorizing disease severity based on anatomic factors (mild, moderate, and severe CHD). The outcome was age at intervention, i.e., cardiac catheterization or surgery, whichever occurred sooner, in days. The mediator variable was whether prenatal diagnosis occurred. Control variables included birth weight, sex, surgery during the COVID-19 pandemic, race, ethnicity, birth hospital’s level of perinatal care as designated by Illinois state, presence of a syndrome or non-cardiac abnormality, and insurance status. Results: In total, 429 parent-infant pairs met inclusion criteria; 68.3% had prenatal diagnosis and 28.4% had severe CHD. In adjusted analyses, compared to those with the mild CHD, those with moderate CHD had 40.0% higher probability of prenatal diagnosis (p<0.001) and on average, received intervention 14 days sooner (p=0.001). Those with severe CHD had a 59.5% higher probability of prenatal diagnosis (p<0.001), and on average received intervention 19 days sooner (p<0.001). In a mediation analysis, prenatal diagnosis accounted for 27.2% of the difference in surgical timing between moderate and mild disease, and 29.5% of the difference in surgical timing between severe and mild disease ( Figure 1 ). Conclusion: The relationship between CHD severity and timing to postnatal intervention is partly attributable to prenatal diagnosis.

The Role of Newborn Pulse Oximetry Screening for Detecting Critical Congenital Heart Defects: A Narrative Review.

Critical congenital heart diseases are life-threatening, with a high morbidity rate and mortality among newborns; in fact, a newborn discharged from the hospital with undiagnosed heart disease may experience severe complications during the initial days or weeks of their life, necessitating emergency care and even death. Among all kinds of critical congenital heart disease, coarctation of the aorta is one of the most difficult to diagnose because it only becomes noticeable a few days after birth, when patients have already been discharged from the hospital. This underlines the importance of having a reliable diagnostic tool to discover these diseases. The identification of some of these patients can be achieved through newborn screening with pulse oximetry, but only a small number are diagnosed. Hence, the objective of this review was to determine the value of pulse oximetry screening for the early detection of congenital heart defects in newborns, with a focus on coarctation of the aorta. A literature search was conducted between December 2023 and February 2024 using four electronic databases: Pubmed, Google Scholar, Embase, and Scopus to identify studies that evaluated the efficacy of pre- and post-discharge neonatal ductal saturation monitoring for the diagnosis of critical congenital heart defects before discharge. Twenty research studies with a large number of patients, demonstrating moderate sensitivity and high specificity of pulse oximetry test in detecting critical heart defects, especially coarctation of the aorta, were selected and analyzed. Many confirmations have been found of how good the specificity of screening is, reaching an average value of 99.9% in each study analyzed. The problem still lies in the sensitivity of the screening, which is not as good as the specificity, never reaching 90% in any of the studies analysed. So, it is crucial to keep up with efforts to improve the efficacy of the pulse oximetry screening method.

A Comparison for Infantile Mortality of Crucial Congenital Heart Defects in Korea over a Five-Year Period.

Background: Nearly half of congenital heart defects (CHDs) related to mortality occur during infancy although advancements in treatments have increased the survival rates. This study comprehensively examined overall and surgical mortality in CHD infants with the highest mortality rates in an effort to improve our understanding of CHD epidemiology. Methods: Participants were drawn from a dataset of 1,964,691 infants born between 2014 and 2018 in Korea. Crucial CHDs are defined here as including diverse categorical defects and classical critical CHDs but excluding simple shunt defects. Overall mortality (procedural and natural mortality) and procedural mortality (interventional and surgical mortality) for infants were analyzed. Results: The performance rate for multiple procedures in infants with crucial CHDs was 16%. The overall and surgical mortalities of crucial CHDs were 8% and 7%. The mortalities of palliative procedures were relatively high. Procedural mortalities for infants were significantly decreased in the tetralogy of Fallot (TOF), atrioventricular septal defects, and total anomalous pulmonary venous return (TAPVR) compared with overall mortalities for infants. Surgical mortalities for infants involving TOF and TAPVR were significantly lower, but those for infants involving hypoplastic left heart syndrome (HLHS) were higher than those for all ages. Conclusions: Palliative procedural techniques in infants must be improved to obtain better outcomes, particularly in the palliative surgery of HLHS. The infantile procedural outcomes for TOF and TAPVR are excellent and important in order to overcome disastrous circumstances during infancy. This comprehensive study of the overall and procedural mortalities of CHDs may have laid a cornerstone for CHD epidemiology in Korean infants.

Stem cell seeding of decellularised porcine right ventricular outflow tracts to advance repair of congenital heart defects

Abstract Background Replacement of the right ventricular outflow tract (RVOT) is necessary in many critical congenital heart disease subtypes, often when the child is less than one year old. Poor availability of small-diameter cardiac homografts necessitates the use of xenografts in paediatric surgical reconstruction. Currently used xenografts are limited by immunoincompatibility and lack of growth, requiring repeat surgery to replace the defective graft. One approach to tackle acute immune rejection of animal tissue is aldehyde fixation of grafts prior to xenotransplantation. Though masking antigenicity, fixation is associated with multiple issues, including toxicity of fixative remnants and calcification. Decellularisation of biological grafts to remove foreign material is a promising alternative to reduce immunogenicity and dampen the risk of rejection. To combat lack of growth, in vitro cellularisation with stem cells is a promising solution. Purpose Current RVOT substitutes have not proven to be a lifelong solution for paediatric cardiac repair. This research has optimised a novel decellularisation technique for porcine RVOTs to produce a durable and cell-free scaffold with translational capability. Current work is looking to seed mesenchymal stem cells (MSCs) onto the acellular RVOT in vitro to enhance immune tolerance and endow the graft with growth capacity upon implant. Methods First, this decellularisation technique produces acellular RVOT scaffolds by simultaneously utilising three methods: enzymatic, chemical, and mechanical decellularisation. Novel to this method, the latter is realised using a 3D printed flow chamber to utilise the shear forces of continuous fluid flow for cell removal from the external and internal RVOTs structure. Coating the acellular graft with poly-lysine has then enabled recellularisation with MSCs isolated from the umbilical cord. Results Nuclei quantification demonstrates decellularisation of all structures of the RVOT, and elimination of residual nucleic acids is evidenced. Immunohistochemical analysis confirms the xenoantigen Galα1-3Galβ1-4GlcNAc-R is absent from the decellularised tissue. In parallel with removal of typical immunogens associated with the rejection response, the elastin and collagen fibres of the extracellular matrix are maintained, supported by histology and electron microscopy. Importantly, the tensile properties of the decellularised grafts are preserved. Finally, MSCs adhered onto the external pulmonary artery surface in vitro, achieving a uniform monolayer of cells. Conclusion RVOTs have been successfully decellularised and proven to possess seeding potential. Future work aims to characterise the seeded MSCs to assess parameters including proliferative capacity and expression of typical MSC markers. Overall, this work has the potential to produce a non-immunogenic graft possessing growth potential as a surgical alternative for paediatric RVOT replacement.

Correction to: Pulmonary Interstitial Emphysema in an Infant with Critical Congenital Heart Defect Associated with TNNC1 Gene Mutation and 22q11.2 Microdeletion: a Case Report

Correction to: Pulmonary Interstitial Emphysema in an Infant with Critical Congenital Heart Defect Associated with TNNC1 Gene Mutation and 22q11.2 Microdeletion: a Case Report

Evaluating the Modified American Academy of Pediatrics Screening Algorithm for Critical Congenital Heart Disease.

In 2018, an expert panel recommended two key modifications to the most used algorithm for screening neonates for critical congenital heart disease (CCHD). Our aim was to evaluate the outcomes of the modified algorithm compared with those of the original algorithm in a real-world setting. We compared the performance characteristics of an original CCHD algorithm used to screen term neonates at a large hospital system between October 26, 2018, and October 15, 2020, and the recommended modified algorithm used between October 15, 2020, and June 30, 2022. We calculated sensitivity, specificity, false positive rate, proportion of false positives with non-CCHD illness, and error rates of test administration and interpretation for each algorithm. Sensitivity was not significantly different between the modified algorithm compared with the original algorithm (40.00 vs. 12.50%, p ≥0.99), but specificity was lower (and hence false positive rate was higher) in the modified algorithm (99.91 vs. 99.98%, p < 0.001). Despite a higher false positive rate in the modified algorithm, the proportion of false positives with significant non-CCHD illness was similar (36.47 vs. 28.57%, p ≥0.99), a finding that translated to an increase in the number of cases of significant non-CCHD illness detected (11 cases out of 32,178 screens vs. 2 cases out of 32,984 screens). Error rates of test administration and interpretation were similar between the two algorithms. In this limited study, the modified algorithm for CCHD screening using pulse oximetry had a higher false positive rate than that of the original American Academy of Pediatrics algorithm. However, this higher rate led to an increased overall number of cases detected of significant non-CCHD illness. · Experts recommend two changes to the American Academy of Pediatrics-endorsed CCHD screen.. · This study evaluates the new algorithm for screening.. · The new algorithm detects at least as many cases as the original.. · There was no significant difference in test sensitivity for CCHD.. · The new algorithm has a statistically higher false positive rate..

Special considerations for the stabilization and resuscitation of patients with cardiac disease in the Neonatal Intensive Care Unit

Effective resuscitation of neonates with congenital heart disease (CHD) depends on comprehensive planning, thorough understanding of physiology, vigilant monitoring, and interdisciplinary collaboration to achieve the best outcomes. Neonatal heart disease can affect cardiac structure, rhythm, or ventricular function, and may be either congenital or acquired. Critical congenital heart disease (CCHD) can result in inadequate pulmonary blood flow, impaired intracardiac mixing, airway obstruction, or insufficient cardiac output. Tailored resuscitation strategies are important as early as the delivery room, where some CHD lesions may cause immediate cardiovascular instability during the transition from fetal to postnatal circulation. Premature infants with CHD are at higher risk due to their small size and the complications associated with prematurity, affecting both CHD management and overall clinical stability. Addressing both cardiac and non-cardiac causes of decompensation requires a precise understanding of each patient's unique physiology and trajectory from delivery through postintervention intensive care.

The illness severity scores can predict the need for respiratory and inotropic support in newborns with critical congenital heart malformations

Background and objectives. The illness severity scoring systems have been developed and validated to assess mortality risks based on clinical conditions and aid critical patient decision-making. Dextro-transposition of the great arteries (D-TGA) and coarctation of the aorta (CoA) are critical congenital heart defects that require medical treatment from birth and surgical repair in the neonatal period. The study aimed to compare the values of three scoring systems in predicting the need for respiratory and inotropic support for newborns with D-TGA and CoA. Materials and methods. An observational study was conducted on inborn infants diagnosed with D-TGA and CoA admitted to the newborn intensive care unit (NICU) within 12 hours of birth at a referral unit for congenital heart disease between January 2018 and December 2020. Newborns with D-TGA and CoA who died within the first 24 hours of birth, outborn infants, and those with a late diagnosis were excluded. For each patient, the Clinical Risk Index of Babies (CRIB-II), Score for Neonatal Acute Physiology-II (SNAP-II), and Score for Neonatal Acute Physiology-Perinatal Extension-II (SNAPPE-II) were calculated. Results. A total of 69 newborns were included in the current study, 30 (43.48 %) of whom were infants with D-TGA (D-TGA Group) and 39 (56.52 %) with CoA (CoA group). Newborns who required respiratory support had significantly higher CRIBII scores (3.03 ± 0.63 vs. 1.34 ± 0.19; p = 0.001), SNAP-II scores (26.61 ± 13.96 vs. 12.43 ± 9.98; p = 0.001), and SNAPPE-II scores (33.04 ± 2.91 vs. 14.47 ± 12.35; p = 0.001) compared to those who did not require it. Significantly higher CRIB-II (3.22 ± 0.67 vs. 1.56 ± 0.23; p = 0.003), SNAP-II (22.91 ±14.57 vs. 14.28 ± 1.58; p = 0.003), and SNAPPE-II (28.82 ± 17.95 vs. 16.58 ± 13.85; p = 0.003) scores were also associated with the need for inotropic support. When comparing the predictive efficiency of illness severity scores for detecting the risk of needing respiratory and inotropic support, they are more effective at predicting the need for ventilation and less effective at predicting the need for inotropes. Conclusions. The illness severity scores can effectively predict the need for pre-surgery interventions. Their simplicity makes them suitable for routine use.

Cyanotic heart defects in children: neurological aspects of the problem

Background. The goal is to justify the need to monitor neurodevelopment in children with congenital heart defects (CHD) and to study neurological disorders in patients under 3 years of age with cyanotic heart defects. Materials and methods. Modern research on the neurodevelopment of children with CHD were analyzed. Sixty-two patients with cyanotic heart defects who underwent examination and surgical treatment at the Center for Pediatric Cardiology and Cardiac Surgery of the Ministry of Health of Ukraine were studied. They are divided into 2 groups: group 1 are children with critical cyanotic CHD and group 2 — with non-critical cyanotic CHD. The control group consisted of 35 healthy patients. A neurological examination was performed using standardized neurological scales. Data processing was carried out using the Stata 12.1 licensed statistical package. Results. Neurodevelopmental disorders were diagnosed in 33 (53 %) of 62 children with cyanotic CHD: in group 1 — in 29 % of cases and in group 2 — in 24 %. These groups were statistically homogeneous in terms of gestational age (p = 0.492) and body weight at birth, with no significant difference from the control group. Older age is characterized mainly by delays in the stages of motor development (16 % in group 1 and 15 % in group 2) and speech disorders (7 % in group 1 and 3 % in group 2). Acute disorders of cerebral circulation (6 % in group 2) and the presence of epileptic seizures (up to 3 %) were characteristic of children under 1 year. Apgar score was lower in patients with critical cyanotic CHD with neurodevelopmental delay and a longer duration of artificial circulation during surgical treatment. Conclusions. 1. Neurological disorders were diagnosed in 53 % of patients with cyanotic heart defects: in 29 % with critical heart defects and 24 % with non-critical heart defects. 2. Older children are characterized mainly by delays in the stages of motor development and speech disorders. Behavioral disorders are more typical for children with non-critical CHD. Acute disorders of cerebral circulation and the presence of epileptic seizures were characteristic of children under 1 year of age. 3. The influence of body weight and gestational age on neurodevelopmental disorders is excluded. The severe state of children with neurodevelopmental delay at birth and an increase in the duration of artificial circulation during surgical treatment were found. 4. Interdisciplinary examination of patients with cyanotic heart defects, including examination by a pediatric neurologist, as well as a follow-up is necessary for timely referral to the next stages of rehabilitation.

Effects of Prostaglandin E1 and Balloon Atrial Septostomy on Cerebral Blood Flow and Oxygenation in Newborns Diagnosed with Transposition of the Great Arteries.

Dextro-transposition of the great arteries (D-TGA) is a critical congenital heart defect that can impact neurodevelopment due to cerebral perfusion and oxygenation disorders followed by alterations in synaptogenesis, gyrification, sulcation, and the microstructure. Brain injuries can occur both pre-operatively and postoperatively, especially white matter injuries, neuronal loss, and stroke. Materials and Methods: In a retrospective study conducted at a tertiary center between 2016 and 2023, we investigated the early effects of Prostaglandin E1 (PGE1) administration and balloon atrial septostomy (BAS) on cerebral blood flow and oxygenation in inborn neonates with D-TGA. Cerebral Doppler Ultrasound in the anterior cerebral artery (ACA) was performed to assess the resistive index (RI), Peak Systolic Velocity (PSV), and End-Diastolic Velocity (EVD) before PGE1, before the BAS procedure, and 24 h after birth. Cerebral regional saturations of oxygen (crSO2) and cerebral fractional tissue oxygen extraction (cFTOE) were evaluated. D-TGA patients were divided into the PGE1 group and the PGE1 + BAS group. Age-matched healthy controls were used for comparison. Results: All 83 D-TGA newborns received PGE1 within two hours after delivery, of whom 46 (55.42%) underwent BAS. In addition, 77 newborns composed the control group. PGE1 administration increased crSO2 from 47% to 50% in the PGE1 group, but lower than in controls at 24 h of life, while cFTOE remained elevated. The RI increased 24 h after delivery (0.718 vs. 0.769; p = 0.000002) due to decreased EDV (10.71 vs. 8.74; p < 0.0001) following PGE1 treatment. The BAS procedure resulted in a significant increase in crSO2 from 42% to 51% at 24 h of life in the PGE1 + BAS group. Doppler parameters exhibited a similar trend as observed in the PGE1 group. Conclusions: PGE1 treatment and BAS are lifesaving interventions that may improve cerebral perfusion and oxygenation in newborns with D-TGA during the transition period, as reflected by increasing SpO2 and crSO2.

Survival of Children With Critical Congenital Heart Defects in the National Birth Defects Prevention Study.

Critical congenital heart defects (CCHDs) are associated with considerable morbidity and mortality. This study estimated survival of children with nonsyndromic CCHDs and evaluated relationships between exposures of interest and survival by CCHD severity (univentricular or biventricular function). This analysis included 4380 infants with CCHDs (cases) born during 1999-2011 and enrolled in the National Birth Defects Prevention Study, a multisite, population-based case-control study of major birth defects. Cases were linked to state death files. Nonparametric Kaplan-Meier survival functions were used to estimate 1- and 5-year survival probabilities overall and by severity group (univentricular/biventricular) stratified by demographic and clinical exposure variables of interest. The log-rank test was used to determine whether stratified survival curves were equivalent. Survival and 95% confidence intervals (CIs) were also estimated using Cox proportional hazards modeling adjusted for maternal age, education, race/ethnicity, study site, and birth year. One- and five-year survival rates were 85.8% (CI 84.7-86.8) and 83.7% (CI 82.5-84.9), respectively. Univentricular 5-year survival was lower than biventricular case survival [65.3% (CI 61.7-68.5) vs. 89.0% (CI 87.8-90.1; p < 0.001)]. Clinical factors (e.g. preterm birth, low birthweight, and complex/multiple defects) were associated with lower survival in each severity group. Sociodemographic factors (non-Hispanic Black race/ethnicity, <high school education, smoking, and lower household income) were only associated with survival among biventricular cases. Mortality among children with CCHDs occurred primarily in the first year of life. Survival was lower for those with univentricular defects, and social determinants of health were most important in predicting survival for those with biventricular defects.

Racial Disparities in Palliative Care at End-of-Life in Children with Advanced Heart Disease in the South

To examine specialty pediatric palliative care (SPPC) and end-of-life (EOL) care for children with advanced heart disease in Alabama, including rates of and disparities in SPPC involvement. We performed a retrospective study from electronic health records of children (≤21 years at death) who died with advanced heart disease at a single institution between 2012 and 2019 (n=128). The main outcome was SPPC consult; we assessed clinical and sociodemographic factors associated with SPPC. The median age at death was 6 months (IQR=1-25 months) with 80 (63%) ≤1 year; 46% were Black and 45% non-Hispanic White. Seventy (55%) children had critical congenital heart disease, 45 (35%) non-critical congenital heart disease, and 13 (10%) acquired heart disease. Twenty-nine children (22%) received SPPC. Children ≤1 year at time of death and Black children were less likely to receive SPPC (adjusted odds ratio [95% confidence interval]: 0.2 [0.1-0.6], reference >1 year; 0.2 [0.1-0.7], reference non-Hispanic White). SPPC was associated with death while receiving comfort-focused care (30.6 [4.5-210]), do not resuscitate orders (8.2 [2.1-31.3]), and hospice enrollment (no children without SPPC care were enrolled in hospice) but not medically intense EOL care (ICU admission, mechanical ventilation, hemodialysis, or CPR) or death outside the ICU. Children dying with advanced heart disease in Alabama did not have routine SPPC involvement; infants and Black children had lower odds of SPPC. SPPC was associated with more comfort-focused care. Disparities in SPPC utilization for children with advanced heart disease need further examination.

Pulse Oximetry-Based Critical Congenital Heart Disease Screening and Its Differential Performance in Rural America

Pulse Oximetry-Based Critical Congenital Heart Disease Screening and Its Differential Performance in Rural America

Neonatal screening for critical congenital heart diseases in Peru: an urgent call

Congenital heart diseases are the most common congenital malformations worldwide and represent one of the leading causes of neonatal death, in addition to the significant use of human and financial resources by health systems. The purpose of this document is to support the implementation of neonatal screening for critical congenital heart diseases using pulse oximetry according to the different geographical altitudes of Peru. This technology is widely used worldwide and has high sensitivity, specificity, and cost-effectiveness. At many latitudes, it has led to better survival in this group of patients and in the neonatal population in general since its use in the early detection of sepsis, pneumonia, and other conditions that affect the oxygenation of the newborn. Neonatal screening for critical congenital heart disease is applicable at all levels of healthcare at a national level, and its implementation must be a priority to improve neonatal health.

Parent and healthcare professional experiences of critical congenital heart disease in New Zealand to advance health equity

BackgroundHigher odds of survival have been reported in European infants compared to Indigenous Māori and Pasifika infants with critical congenital heart disease in New Zealand. We therefore aimed to understand how to mitigate this disparity by investigating the parent and healthcare professional experiences’ of critical congenital heart disease healthcare in New Zealand.MethodsA prospective qualitative study utilising semi-structured interviews was conducted on a cohort of purposefully sampled parents and health professionals with experience of critical congenital heart disease healthcare in New Zealand. Parents were recruited after a fetal critical congenital heart disease diagnosis and offered two interviews at least three months apart, whilst multidisciplinary fetal and cardiosurgical health professionals were interviewed once. Interviews were recorded and transcribed verbatim before coding, categorization and qualitative analysis.ResultsDuring 2022 and 2023, 45 people participated in 57 interviews (25 parents: 19 mothers, 6 fathers; Indigenous Māori, n = 5; Pasifika, n = 6; Asian, n = 4; European, n = 10; and 20 healthcare professionals: European n = 17). The three lessons learned from participants were: (1) Minoritized groups experience disparate healthcare quality; (2) healthcare systems are under-resourced to provide equitable support for the differential needs of grieving parents; and (3) healthcare systems could engage minoritized families more optimally in shared decision-making.ConclusionsAccording to the experiences of parents and healthcare professionals, persisting inequities in CCHD healthcare quality occur by ethnic group, with the New Zealand healthcare system privileging European families. The concepts from this study could be translated by healthcare leaders, policymakers, and professionals into evidence-based healthcare system improvements to enhance experiences for non-European families more broadly.

Population Pharmacokinetics and Target Attainment of Allopurinol and Oxypurinol Before, During, and After Cardiac Surgery with Cardiopulmonary Bypass in Neonates with Critical Congenital Heart Disease.

The CRUCIAL trial (NCT04217421) is investigating the effect of postnatal and perioperative administration of allopurinol on postoperative brain injury in neonates with critical congenital heart disease (CCHD) undergoing cardiac surgery with cardiopulmonary bypass (CPB) shortly after birth. This study aimed to characterize the pharmacokinetics (PK) of allopurinol and oxypurinol during the preoperative, intraoperative, and postoperative phases in this population, and to evaluate target attainment of the current dosing strategy. Nonlinear mixed-effects modeling was used to develop population PK models in 14 neonates from the CRUCIAL trial who received up to five intravenous allopurinol administrations throughout the postnatal and perioperative periods. Target attainment was defined as achieving an allopurinol concentration >2 mg/L in at least two-thirds of the patients during the first 24h after birth and between the start and 36h after cardiac surgery with CPB. A two-compartment model for allopurinol was connected to a one-compartment model for oxypurinol with an auto-inhibition effect on the conversion, which best described the PK. In a typical neonate weighing 3.5kg who underwent cardiac surgery at a postnatal age (PNA) of 5.6 days, the clearance (CL) of allopurinol and oxypurinol at birth was 0.95 L/h (95% confidence interval 0.75-1.2) and 0.21 L/h (0.17-0.27), respectively, which subsequently increased with PNA to 2.97 L/h and 0.41 L/h, respectively, before CPB. During CPB, allopurinol and oxypurinol CL decreased to 1.38 L/h (0.9-1.87) and 0.12 L/h (0.05-0.22), respectively. Post-CPB, allopurinol CL increased to 2.21 L/h (1.74-2.83), while oxypurinol CL dropped to 0.05 L/h (0.01-0.1). Target attainment was 100%, 53.8%, and 100% at 24h postnatally, 24h after the start of CPB, and 36h after the end of cardiac surgery, respectively. The combined concentrations of allopurinol and oxypurinol maintained ≥ 90% inhibition of xanthine oxidase (IC90XO) throughout the postnatal and perioperative period. The minimal target concentration of allopurinol was not achieved at every predefined time interval in the CRUCIAL trial; however, the dosing strategy used was deemed adequate, since it yielded concentrations well exceeding the IC90XO. The decreased CL of both compounds during CPB suggests influence of the hypothermia, hemofiltration, and the potential sequestration of allopurinol in the circuit. The reduced CL of oxypurinol after CPB is likely attributable to impaired kidney function.

Measuring misclassification and sample bias in passive surveillance systems: Improving prevalence estimates of critical congenital heart defects in state-based passive surveillance systems.

We assessed reporting misclassification for 12 critical congenital heart defects (CCHDs) identified through administrative diagnosis codes within a passive surveillance system. We measured the effect of misclassification on prevalence estimation. Lastly, we investigated a sample-based review strategy to estimate surveillance misclassification resulting from administrative diagnosis codes for case detection. We received 419 reports of CCHDs between 2007 and 2018; 414 were clinically reviewed. We calculated confirmation probabilities to assess misclassification and adjust prevalence estimates. Random samples of reported cases were taken at proportions between 20% and 90% for each condition to assess sample bias. Sampling was repeated 1000 times to measure sample-estimate variability. Misclassification ranged from a low of 19% (n = 4/21) to a high of 84% (n = 21/25). Unconfirmed prevalence rates ranged between one and six cases per 10,000 live births, with some conditions significantly higher than national estimates. However, confirmed rates were either lower or comparable to national estimates. Passive birth defect surveillance programs that rely on administrative diagnosis codes for case identification of CCHDs are subject to misclassification that bias prevalence estimates. We showed that a sample-based review could improve the prevalence estimates of 12 cardiovascular conditions relative to their unconfirmed prevalence rates.

Incidence and Prenatal Detection of Critical and Major Congenital Heart Disease in Western Australia From 2017–2021

Incidence and Prenatal Detection of Critical and Major Congenital Heart Disease in Western Australia From 2017–2021