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Objective: Neurodevelopmental delay is common in children who undergo surgery for Congenital Heart Disease (CHD) in infancy. Cardiac surgery associated acute kidney injury (CS-AKI) occurs frequently in the paediatric cardiac Intensive care unit (ICU). Cardiac surgery associated acute kidney injury (CS-AKI) is associated with worse neurodevelopmental scores and delay in cognitive, language, and motor domains in children with CHD. No known data exist regarding the association of CS-AKI and motor and language subscales. In this study, we explored the relationship between CS-AKI and receptive and expressive language, as well as gross and fine motor delay., Methods: This was a single centre retrospective observational cohort study. Children who underwent surgery for CHD and developed recurrent CS-AKI in the first year of life who had follow-up neurodevelopmental testing using the Bayley Scale of Infant Development Version III were included. Neurodevelopmental delay subscales assessed included: receptive and expressive language, fine and motor skills., Results: The study cohort included 203 children. Recurrent CS-AKI was significantly associated with lower scores in receptive and expressive language, as well as fine and gross motor on unadjusted analyses. On adjusted analyses, recurrent CS-AKI was significantly associated with severe receptive language delay., Conclusion: The independent association of recurrent CS-AKI with severe language delay in children who undergo surgery for CHD in infancy is novel. Our findings may contribute to the understanding of language impairment in this population. Further studies are required to better understand this relationship and any potentially modifiable factors.

Objective: CHD predisposes children to neurodevelopmental delays. Frequent, prolonged hospitalisations during infancy prevent children with heart disease from participating in recommended language and cognitive development programmes, such as outpatient early childhood literacy programmes, and contribute to caregiver stress, a risk factor for adverse developmental outcomes. This study aims to describe the implementation of a single-centre inpatient early childhood literacy programme for hospitalised infants with heart disease and assess its impact on reading practices and patient-family hospital experience., Methods: Admitted infants ≤1 year old receive books, a calendar to track reading frequency, and reading guidance at regular intervals. Voluntary feedback is solicited from caregivers using an anonymous, QR-code survey on books. A prospective survey also assessed programme impact on hospital experience., Results: From February 2021 to November 2023, the Books@Heart programme provided 1,293 books to families of 840 infants, of whom 110 voluntarily submitted feedback. Caregivers reported a significant improvement in access to books (p < 0.001) and increased reading frequency after learning about Books@Heart ( p = 0.003), with the proportion reading to their child daily increasing from 27% to 62%. Among 40 prospective survey responses, caregivers reported feeling a sense of personal fulfillment (60%), self-confidence (30%), connection (98%), and personal well-being (40%) while reading to their child., Conclusion: An inpatient early childhood literacy programme is a well-received intervention for infants with heart disease that promotes development, improves book access, increases reading exposure, and engages families. Further studies are needed to assess its impact on sustained reading practices and neurodevelopmental outcomes.

While significant progress has been made in reducing disparities within the US health care system, notable gaps remain. This article explores existing disparities within pediatric congenital heart disease care. Congenital heart disease, the most common birth defect and a leading cause of infant death, has garnered substantial attention, revealing certain disparities within the US health care system. Factors such as race, ethnicity, insurance coverage, socioeconomic status, and geographic location are all commonalities that significantly affect health disparities in pediatric congenital heart disease. This comprehensive review sheds light on disparities from diverse perspectives in pediatric care, demonstrates the inequities and inequalities leading to these disparities, presents effective solutions, and issues a call to action for providers, institutions, and the health care system. Recognizing and addressing these disparities is imperative for ensuring equitable care and enhancing the long-term well-being of children affected by congenital heart disease. Implementing robust, evidence-based frameworks that promote responsible and safe interventions is fundamental to enduring change.

Background: Infants who undergo surgery for congenital heart disease are at risk of neurodevelopmental delay. Cardiac surgery-associated acute kidney injury (CS-AKI) is common but its association with neurodevelopment has not been explored., Methods: This was a single-center retrospective observational study of infants who underwent cardiac surgery in the first year of life who had neurodevelopmental testing using the Bayley Scale for Infant Development, third edition. Single and recurrent episodes of stages 2 and 3 CS-AKI were determined., Results: Of 203 children with median age at first surgery of 12 days, 31% had one or more episodes of severe CS-AKI; of those, 16% had recurrent CS-AKI. Median age at neurodevelopmental assessment was 20 months. The incidence of delay was similar for patients with and patients without CS-AKI but all children with recurrent CS-AKI had a delay in one or more domains and had significantly lower scores in all three domains, namely, cognitive, language, and motor., Conclusions: This study has assessed the association of CS-AKI with neurodevelopmental delay after surgery for congenital heart disease in infancy. Infants who have recurrent CS-AKI in the first year of life are more likely to be delayed and have lower neurodevelopmental scores., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Oxygen saturation is the percentage of hemoglobin that is saturated with oxygen, converting it to oxyhemoglobin. Oxygen saturation is a critical part of the physical examination of children with congenital heart disease (CHD). The expected oxygen saturation of a patient with CHD depends on their anatomical lesion, their previous surgeries, and any additional pulmonary or systemic pathology that may derange their saturation. Oxygen saturation can be noninvasively measured using pulse oximetry. Pulse oximetry is based on the differential absorption of infrared and red light by oxyhemoglobin and deoxyhemoglobin, with the former absorbing more infrared than the latter. Pulse oximetry readings may be inaccurate in settings of low cardiac output, peripheral vasoconstriction, arrhythmia, hypothermia, and venous pulsations. The use of pulse oximetry in the care of a child with CHD begins with the newborn critical CHD screen. A failed screen indicates a need for further investigation, such as repeated pulse oximetry or echocardiography. The oxyhemoglobin dissociation curve may be used to estimate the partial pressure of oxygen in the blood at various oxygen saturations. It is also a marker of the affinity of hemoglobin for oxygen, with a right-shifted curve indicating a higher oxygen tension needed to saturate hemoglobin. This is a helpful adaptation of the body to situations of stress such as fever, acidosis, and hypercapnia. An understanding of these concepts is paramount for providers caring for patients with known or potential CHD in any setting to appropriately interpret and respond to abnormal saturations for each child., (© American Academy of Pediatrics, 2022. All rights reserved.)

With the substantial growth of pediatric ventricular assist device (VAD) support, there has been an expansion of the target population towards more complex patients, including congenital heart disease (CHD) with single ventricle (SV) physiology. The outcomes of Stage I and Stage II SV-CHD patients on VAD support from the Pedimacs database are poor, with less than 50% survival on VAD by the 3-month mark in both. The primary objective of this article is to describe the current state of VAD support for the failing Stage I and II SV-CHD circulation, to provide insight into potential areas of outcome improvement. We reviewed the published literature in the form of database and registry reports as well as single-center studies to discuss the outcomes of Stage I and Stage II SV-CHD patients on VAD support. Registry-based studies suggest that VAD support for the failing Stage I and Stage II SV-CHD circulations is challenging. However, the more promising outcomes in several single-institutional reports for both Stage I and Stage II SV-VAD indicate that the grim picture from the databases does not reflect the best outcomes that are possible to be achieved, potentially at experienced centers with higher volumes. Areas of future study and potential improvement including timely initiation of VAD support in the cohort of patients expected to not be a candidate for standard SV palliations, pump selection and the benefits of continuous-flow devices, and the decision-making for setting up the optimum circulation for VAD support, be it Fontan completion if feasible or takedown to shunt physiology., (Copyright © 2021. Published by Elsevier Inc.)

Background: Extracardiac birth defects are associated with worse outcomes in congenital heart disease (CHD). The impact of esophageal atresia/trachea-esophageal fistula (EA/TEF) on outcomes after surgery for ductal-dependent CHD is unknown., Methods: Retrospective matched cohort study using the Pediatric Health Information System database from 07/2004 to 06/2015. Hospitalizations with ductal-dependent CHD and EA/TEF, undergoing CHD surgery were included as cases. Admissions with ductal-dependent CHD without EA/TEF were matched 3:1 for age at admission and Risk Adjustment for Congenital Heart Surgery-1 classification. Comparisons were performed using generalized estimating equations., Results: There were 124 cases and 372 controls. Cases included 32 (25.8%) low-risk, 86 (69.3%) intermediate-risk, and 6 (4.8%) high-risk patients. Cases had more females compared to controls (53.2% vs 41.1%, P = .022). Cases were more likely to be premature (28.2% vs 13.7%, P = .001) and low birth weight (29.8% vs 11.8%, P < .001). Cases had a similar frequency of Down syndrome, and DiGeorge/Velocardiofacial syndrome, but a higher frequency of anorectal malformations (4.3% vs 2.4%, P < .001) and renal anomalies (27.4% vs 9.9%, P < .001) than controls. Cases had a higher mortality on univariate (22.0% vs 8.4%, P < .001) and multivariable analysis (odds ratio 2.45, 95%, confidence interval 1.34 - 4.49). Prematurity also was significantly associated with mortality on multivariable analysis. Cases had a longer duration of mechanical ventilation, longer hospital duration of stay, and higher total cost than controls (all P < .001)., Conclusion: In children with ductal-dependent CHD, EA/TEF is associated with increased morbidity, mortality and resource utilization. A majority of patients undergo EA/TEF repair prior to congenital heart disease surgery. (Surgery 2017;160:XXX-XXX.)., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Malawi is one of the poorest nations in the world, ranked 151st among 195 countries by the World Bank, with an under-5-year mortality rate of 63 per 1000 live births. There are no previous studies describing the spectrum of inpatient pediatric cardiology consultations in sub-Saharan Africa. A descriptive cohort study was performed at Kamuzu Central Hospital (KCH), a tertiary care hospital in Lilongwe, Malawi. Demographic, anthropometric, and clinical information for all cardiology consults patients aged 0-18 years admitted to the children's wards over a period of 1 month was reviewed. Seventy-three consults and 69 echocardiograms were performed on 71 patients (35 males, 38 females). The median (IQR) age was 3.1 years (9 months-10.5 years). About 53% (39/73) had failure to thrive, 4.1% (3/73) were sero-reactive for HIV and 100% (73/73) were fully immunized for age per the Expanded Program on Immunization schedule. Seventy-four percent of the echocardiograms were abnormal, with 34.8% (24/69) having congenital heart disease (CHD) and 18.8% (13/69) having acquired heart disease (AHD) with preserved cardiac function. Among CHD, 10.1% (7/69) had cyanotic CHD and 24.6% (17/769 had acyanotic CHD. Among AHD, 10.1% (7/69) had rheumatic heart disease with preserved cardiac function. Symptomatic systolic heart failure (HF) with ejection fraction <50%, was found in 20.3% (14/69), and pulmonary hypertension was diagnosed in 10.1% (7/69). Overall admission mortality was 5.5% (4/73). Three patients left the hospital against medical advice. None of the patients with systolic HF had CHD. There was no significant association of HIV, gender, or failure to thrive on presence of systolic HF. This is the first report describing the spectrum of pediatric cardiology consults in an inpatient setting in Malawi. There was an unexpectedly high proportion of CHD and systolic HF. Further studies should be conducted to explore the implications and potential causes of these findings., (© 2018 Wiley Periodicals, Inc.)

BackgroundThe growth trajectories of common measurements, including estimated fetal weight (EFW), head circumference (HC), and abdominal circumference (AC), in fetuses with congenital heart disease (CHD) have not been described for different cardiac lesions. We hypothesized that (i) fetuses with CHD have differential growth in utero, and (ii) different categories of CHD demonstrate different in utero growth curves.MethodsWe performed a retrospective observational cohort study of pregnancies with known fetal CHD seen from January 2000 to June 2013. For analysis, the infants were divided into single ventricle (SV), biventricular conotruncal, d-transposition of great arteries (d-TGA), biventricular septal defects (SD; including atrial, ventricular, and atrioventricular SD), and all others (Other).ResultsA total of 194 newborns met inclusion criteria. There was significant differential growth of EFW in all CHD types, except d-TGA, starting with low z-scores before 25 weeks gestation, improving toward normal around 30-32 weeks gestation, and then again differential growth with advancing gestation. SV and SD groups had significant differential growth of HC starting early in gestation and linearly progressing negative z-scores with advancing gestation.ConclusionWe observed differences in the fetal growth curves throughout gestation for the major categories of CHD, including significant differential growth in even "simple" CHD, such as SD.