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Neonates with ductal-dependent CHD rely on the patency of the ductus arteriosus to maintain circulation. Alprostadil is utilised to maintain ductal patency, although optimal dosing has not been determined. This study aims to describe alprostadil dosing in neonates with ductal-dependent CHD.
Methods:
This is a single-centre retrospective study including neonatal patients with ductal-dependent CHD who received alprostadil from January 2015 to December 2015 (cohort 1) and January 2021 to December 2021 (cohort 2). The primary objective was to describe alprostadil dosing in the two study periods. Secondary objectives included clinical outcomes and adverse events associated with different alprostadil dosing strategies.
Results:
Sixty-five patients met eligibility for inclusion in this study: thirty-eight patients in cohort 1 and twenty-seven patient s in cohort 2. Baseline demographics were similar between cohorts. Initial alprostadil dosing in cohort 1 and cohort 2 was 0.006 mcg/kg/min and 0.025 mcg/kg/min (p = < 0.001), respectively. Patients in cohort 2 were found to have a higher incidence of apneic events, apneic events requiring respiratory support, and the incidence of fever ≥38 °C.
Conclusions:
In this single-centre study, we report that higher doses of alprostadil were associated with an increased risk of adverse events, which should be validated by prospective multicentre studies.
Persistent pulmonary hypertension of the newborn is a serious disease with significant morbidity and mortality. Magnesium sulphate has been proposed as a potential treatment for persistent pulmonary hypertension of the newborn, but its efficacy remains unclear. The article aims to evaluate the effects of magnesium sulphate on persistent pulmonary hypertension of the newborn.
Methods:
A comprehensive search of PubMed, Web of Science, Embase, and Cochrane Library was conducted to identify relevant studies. The primary outcomes were pulmonary artery pressure and oxygenation index, while secondary outcomes included mean blood pressure, alveolar-arterial oxygen difference, arterial partial pressure of oxygen (PaO2), arterial partial pressure of carbon dioxide (PaCO2), and arterial oxygen saturation. Statistical analysis was performed by Cochrane Review Manager 5.3.
Results:
The study analysed twelve studies involving 380 patients. Results indicated that magnesium sulphate treatment significantly reduced pulmonary artery pressure levels (MD −24.96, 95% CI −28.19 to −21.73, P < 0.0001) and mean blood pressure (MD −3.11, 95% CI −3.91 to −2.32, P < 0.0001) compared to pretreatment. Additionally, it led to a notable decrease in oxygenation index (P < 0.00001) and alveolar-arterial oxygen difference (P < 0.0001), while increasing PaO2 (P < 0.0001) and arterial oxygen saturation (P < 0.001). However, there was no significant effect on PaCO2 levels compared to pretreatment.
Conclusion:
Magnesium sulphate is a valuable therapy for persistent pulmonary hypertension of the newborn. It markedly reduced pulmonary artery pressure, alveolar-arterial oxygen difference, and oxygenation index, while enhancing PaO2, and arterial oxygen saturation, with no impact on PaCO2 levels. Magnesium sulphate may also reduce mean blood pressure following a 2-hour treatment. Additional studies are necessary to further clarify its efficacy and potential side effects.
Registration:
This study was registered with PROSPERO (CRD42024578092).
Total anomalous pulmonary venous drainage associated with hypoplastic aortic arch represents an exceptionally rare and intricate congenital cardiac anomaly. We report the case of a 13-day-old late preterm neonate presenting with infracardiac total anomalous pulmonary venous drainage, severe pulmonary hypertension, and a hypoplastic aortic arch. The patient underwent single-stage surgical correction involving rerouting of the pulmonary venous confluence to the left atrium and aortic arch reconstruction using autologous pericardial patch under cardiopulmonary bypass with selective cerebral perfusion. Postoperative recovery was favourable, marked by successful extubation, resolution of pulmonary hypertension, and absence of residual anatomical obstruction. This case highlights the feasibility and effectiveness of early, comprehensive surgical repair and the necessity of coordinated multidisciplinary perioperative care. Long-term follow-up remains vital for monitoring functional outcomes and detecting late complications.
Haemolysis is developing prominence in the setting of supporting increasingly complex children with heart failure with a ventricular assist device. The goal of this study is to better characterise haemolysis and its implications in children supported with pulsatile ventricular assist devices.
Methods:
This is a single-centre retrospective review of 44 children who were supported by Berlin Heart EXCOR between January 2006 and June 2020. Patients were divided into major haemolysers and non-major haemolysers. Major haemolysers were defined as patients with lactate dehydrogenase > 500U/L (2.5x the upper limits of normal) with either total bilirubin > 2mg/dL (with predominantly indirect hyperbilirubinemia) or anaemia out of proportion to the clinical scenario more than three days following implantation of the ventricular assist device(s). Patient demographics, ventricular assist device factors, and outcomes, including end-organ function and mortality, were compared between major haemolysers and non-major haemolysers.
Main results:
Forty-four patients supported by the Berlin EXCOR were included in the analytic cohort of the study: 27 major haemolysers and 17 non-major haemolysers. Major haemolysis was more common in those supported with single-ventricle ventricular assist device (i.e., VAD in the context of functionally univentricular anatomy) compared to those with biventricular hearts, p = 0.01. There were no patients with an isolated left ventricular assist device or isolated right ventricular assist device in our analytic cohort of 44 patients. Of the 19 patients with single-ventricle ventricular assist device, 84% (16/19) were major haemolysers. Of the 25 patients with a biventricular assist device, 44% (11/25) were major haemolysers. Major haemolysers and non-major haemolysers had a body surface area of 0.28 and 0.40, respectively (p = 0.01). Overall, survival to discharge from the hospital was 66% (n = 29/44). Survival to discharge from the hospital was 52% (14/27) in major haemolysers versus 88% (15/17) in non-major haemolysers, p = 0.02. Only 3 of the 27 with major haemolysis had severe haemolysis, that is, lactate dehydrogenase > 2000 and bilirubin above 10. Non-major haemolysers had a better improvement in creatinine clearance during ventricular assist device support, p < 0.0001. (During the same era of this study, 22 patients who were supported with Berlin Heart were excluded from the analytic cohort because they did not have any recorded measurement of lactate dehydrogenase. Seventeen of these 22 patients had no clinical evidence of haemolysis. Survival to discharge from the hospital in this excluded cohort was 86% [19/22].)
Conclusions:
Major haemolysis in patients with pulsatile ventricular assist device is more likely with single-ventricle ventricular assist device support and smaller body surface area.
Ventricular tachycardia is a rare condition in the fetus and neonate with a paucity of data regarding the management and outcomes.
Methods:
We reviewed the clinical histories, associated CHD and syndromes, diagnostic investigations, management strategies, and outcomes for all fetuses and neonates with ventricular tachycardia encountered between 2005 and 2020.
Results:
Five fetal and 8 neonatal cases of ventricular tachycardia were encountered. Two (40%) fetal cases, compared to 5 (62%) neonatal cases had CHD (p = 0.59), and only 1 fetus had cardiomyopathy with findings suggesting restriction before ventricular tachycardia onset. The median age at ventricular tachycardia presentation was 32 (26–37) weeks for fetal and 11 (1–27) days for neonatal cases. Of the fetal cases, 2 were initially treated trans-placentally with propranolol and 2 with amiodarone. Two fetuses (40%) had ventricular tachycardia suppression prenatally that recurred during the neonatal period necessitating propranolol therapy, 2 (40%) had resolution before birth with no postnatal recurrence, and the cardiomyopathy case never achieved full control, developed hydrops and demised. Of the neonatal cases, 4 received intravenous antiarrhythmics on admission: 3 amiodarone and 1 esmolol, and 2 of these were converted to propranolol and 2 to sotalol. Three other neonates initially received propranolol, with 1 discharged on propranolol, 1 on sotalol, and 1 on mexiletine after failed propranolol and sotalol treatment. The 8th neonate was discharged on no medication.
Conclusion:
Most fetal and neonatal ventricular tachycardia is manageable with pharmacologic therapy. Given its rarity, larger studies are needed to identify optimal management strategies.
In this chapter of Complex Ethics Consultations: Cases that Haunt Us, the authors describe a baby born at 25 weeks gestational age (at a time when survival at that stage was tenuous) to an adolescent mother. The fragile preemie developed necrotizing enterocolitis that was so extensive that definitive surgical resection was impossible. With no definitive treatment and inevitable suffering without it, the recommendation to shift to comfort care was declined and ethics consultants helped to negotiate the conflict.
The iron regulation mechanisms are not exactly the same between adulthood and the early postnatal period. Also, neonatal iron status is different in full-term versus preterm infants because the prenatal/gestational period, when hepatic iron accumulates, is shortened. Newborns, especially premature infants, are at high risk of iron deficiency due to inadequate iron stores, which constitute the primary source of iron to satisfy the neonate’s increasing iron requirements. In addition, frequent blood transfusions and congenital haemochromatosis may induce iron overload in the affected neonate. To understand the cause of neonatal iron deficiency/overload and to promote the development of effective therapeutic interventions in humans, different animal models have been generated by genetic engineering, low-/high-iron diets, phlebotomy/transfusion and surgical manipulation. These models use various laboratory and domestic animals to study iron imbalance. They serve as surrogate models for experiments that are ethically or practically unfeasible to conduct on human neonates. Although an animal model for studying neonatal iron disorders may not fully replicate the complexities of human diseases, it is designed to model specific aspects of these conditions. Combined data from multiple models can help to offset the limitations inherent in each individual model. In this review, we outline approaches to induce neonatal iron disorders, current animal models of full-term and preterm neonates, and recommendations for diagnosis.
Edited by
James Ip, Great Ormond Street Hospital for Children, London,Grant Stuart, Great Ormond Street Hospital for Children, London,Isabeau Walker, Great Ormond Street Hospital for Children, London,Ian James, Great Ormond Street Hospital for Children, London
Intravenous fluids are routinely given to children when the enteral route is not sufficient or it’s not an option, such as during surgery and anaesthesia. Lack of understanding of the composition of fluids and the appropriate rate to administer them has been associated with serious morbidity and mortality in children. Recent evidence has shown that giving children isotonic fluids with a sodium concentration similar to plasma decreases the risk of hyponatraemia without an increase in adverse effects. This has led to a change in guidelines, which now recommend that isotonic fluids are used in children along with regular monitoring of fluid balance and electrolytes. Current evidence supported by several anaesthesia societies across the world recommend that children are allowed and should be encouraged to drink clear fluids up to one hour before elective surgery. Evidence is starting to emerge from enhanced recovery programmes in children of improved outcomes from individualised perioperative fluid therapy and avoidance of prolonged preoperative fasting. Strategies to reduce blood transfusion in children having surgery include treatment of preoperative iron deficiency, acceptance of low transfusion thresholds, cell salvage and tranexamic acid administration.
Edited by
James Ip, Great Ormond Street Hospital for Children, London,Grant Stuart, Great Ormond Street Hospital for Children, London,Isabeau Walker, Great Ormond Street Hospital for Children, London,Ian James, Great Ormond Street Hospital for Children, London
Neonates presenting for specialist neonatal and paediatric surgery range in age and complexity from the extremely premature infant undergoing laparotomy for necrotising enterocolitis to the healthy term neonate undergoing hernia repair. Many patients presenting for repair of congenital abnormalities are born prematurely and/or have congenital cardiac defects, which leads to additional challenges. The most common general surgical conditions presenting in the newborn period are oesophageal atresia (OA) with or without trachea-oesophageal fistula (TOF), congenital diaphragmatic hernia (CDH), intestinal atresias, meconium ileus, malrotation and volvulus, Hirschsprung’s disease, imperforate anus, exomphalos, gastroschisis and necrotising enterocolitis (NEC). Anaesthetic techniques should be tailored to the relevant surgical condition, but most of the key principles of management are similar and require an in-depth knowledge of the anatomical, physiological and pharmacological differences particular to this vulnerable group of patients. In this chapter, we discuss the general considerations for anaesthesia in neonatal surgery and then each specific neonatal condition in relation to their anaesthetic and surgical management.
Cardiac surgery-associated acute kidney injury (CS-AKI) and fluid overload (FO) are common among neonates who undergo cardiopulmonary bypass, and increase mortality risk. Current diagnostic criteria may delay diagnosis. Thus, there is a need to identify urine biomarkers that permit earlier and more accurate diagnosis.
Methods:
This single-centre ancillary prospective cohort study describes age- and disease-specific ranges of 14 urine biomarkers at perioperative time points and explores associations with CS-AKI and FO. Neonates (≤28 days) undergoing cardiac surgery were included. Preterm neonates or those who had pre-operative acute kidney injury were excluded. Urine biomarkers were measured pre-operatively, at 0 to < 8 hours after surgery, and at 8 to 24 hours after surgery. Exploratory outcomes included CS-AKI, defined by the modified Kidney Disease Improving Global Outcomes criteria, and>10% FO, both measured at 48 hours after surgery.
Results:
Overall, α-glutathione S-transferase, β-2 microglobulin, albumin, cystatin C, neutrophil gelatinase-associated lipocalin, osteopontin, uromodulin, clusterin, and vascular endothelial growth factor concentrations peaked in the early post-operative period; over the sampling period, kidney injury molecule-1 increased and trefoil factor-3 decreased. In the early post-operative period, β-2 microglobulin and α-glutathione S-transferase were higher in neonates who developed CS-AKI; and clusterin, cystatin C, neutrophil gelatinase-associated lipocalin, osteopontin, and α-glutathione S-transferase were higher in neonates who developed FO.
Conclusion:
In a small, single-centre cohort, age- and disease-specific urine biomarker concentrations are described. These data identify typical trends and will inform future studies.
Developmental care for newborns with congenital heart disease (CHD) improves cardiac and respiratory patterns. According to the American Heart Association, developmental care in newborns with CHD is important for improving neurodevelopmental outcomes. This study aimed to evaluate the validity and reliability of the Turkish version of the Developmental Care Scale for Neonates with Congenital Heart Disease.
Methods:
This was a methodological, descriptive study conducted with 169 nurses from a tertiary-level NICU. The Demographical Information Form and the Developmental Care Scale for Neonates with Congenital Heart Disease were used to collect the data. The scales’ language and content validity, construct validity, and internal consistency were also assessed.
Results:
The scale consists of 31 items and four subscales. Factor loadings ranged from 0.44 to 0.82 and explained 65% of the total variance. Fit indices indicate that the model is acceptable. Cronbach’s α was 0.95 for the entire instrument, 0.91 for developing the external environment subscale, 0.94 for assessing family well-being, 0.86 for the caregiver activities toward the neonate, and 0.82 for the basic need subscale. Item–total correlations ranged between 0.34 and 0.75, according to the item analysis results.
Conclusions:
The Turkish version of the Developmental Care Scale for Neonates with Congenital Heart Disease is valid and reliable. The use of this scale could improve the performance of neonatal intensive care nurses in providing developmental care to newborns with CHD as well as the quality of care.
Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis usually have to undergo treatment in the neonatal period. Compared to traditional surgical intervention, catheter-based cardiac interventions may achieve similar or superior outcomes for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. However, there is limited literature on anaesthesia techniques, challenges, and risks associated with cardiac catheterisation in this population.
Methods:
This article retrospectively analysed the clinical data of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis neonates who were treated with interventional cardiac catheterisation in our hospital from January 2015 to October 2022. Clinical outcomes considered were haemodynamic or pulse oxygen saturation instability, vasoactive requirements, prolonged intubation (>24 h postoperatively), and cardiovascular adverse events.
Results:
A total of 63 patients met the inclusion criteria. All patients survived the intervention. Among the patients with critical pulmonary stenosis, 40 successfully received percutaneous balloon pulmonary valvuloplasty, while three patients received ductal stenting due to moderate right ventricular dysplasia at the same time. For patients with pulmonary atresia with intact ventricular septum, 17 of the 23 patients successfully underwent percutaneous pulmonary valve perforation and percutaneous balloon pulmonary valvuloplasty. Of these, five patients underwent ductal stenting due to unstable pulmonary blood flow. Three patients only underwent ductal stenting. In addition, three patients received hybrid therapy.
Conclusions:
There are various clinical techniques and risk challenges in the interventional cardiac catheterisation of neonatal pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. However, by mastering the physiological and pathophysiological characteristics of the disease, adequately preparing for the perioperative period, and predicting the procedure process and potential complications, anaesthesia and surgical risks can be effectively managed.
Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe Ebstein’s anomaly following Starnes procedure. The patient’s postoperative course highlights the challenges and complications in simultaneous management of these diagnoses. Transition from shunted single-ventricle physiology to bidirectional cavopulmonary connection improved end-organ function, permitting more aggressive hepatic malignancy treatment.
Pain management is essential in the immediate post-surgical period. We sought to describe the ketorolac dose regimen in neonates and infants following cardiac surgery. Secondary outcomes included renal dysfunction, bleeding, and pain management.
Methods:
We performed a single-centre retrospective cohort study of neonates and infants (aged < 12 months) who received ketorolac following cardiac surgery, from November 2020 through November 2021 (inclusive). Ketorolac was administered at 0.5 mg/kg every 6 hours. Safety was defined by absence of a clinically significant decline in renal function (i.e., increase in serum creatinine [SCr] by ≥ 0.3 mg/dL from baseline within 48 hours and/or urine output ≤ 0.5 mL/kg/hour for 6 hours) and absence of clinically significant bleeding defined as major by International Society on Thrombosis and Hemostasis paediatric criteria or Severe/Fatal Bleeding Events by Nellis et al. Efficacy measures included pain scores and opioid utilisation.
Results:
Fifty-five patients met eligibility criteria. The median (range) dose and duration of ketorolac administration was 0.5 mg/kg/dose for 48 (6–90) hours. Among all patients, there was not a statistically significant difference observed in median SCr within 48 hours of baseline (p > .9). There were no major or severe bleeding events. The median (range) opioid requirements (morphine intravenous equivalents per kg per day) at 48 hours post-ketorolac initiation was 0.1 (0–0.8) mg/kg/day.
Conclusions:
If validated prospectively, these findings suggest that a ketorolac regimen 0.5 mg/kg/dose every 6 hours in neonates and infants post-cardiac surgery may be safe with regard to renal function and bleeding risk, and effective regarding opioid-sparing capacity.
In neonatal vein of Galen aneurysmal malformation, vein of Galen aneurysmal malformation echocardiography remains the mainstay for early detection and explains various haemodynamic changes occurring due to a large systemic arterio-venous shunt. However, there is limited evidence of echocardiography in risk stratifying neonatal vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation. The objective of this study was to identify echocardiographic parameters that could be associated with major outcomes and guide timing of neuro-intervention.
Methods:
In this retrospective chart review, infants < 28 days of age with the diagnosis of vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation were included. Demographic, clinical, and echocardiographic parameters were compared in neonates who survived or died with neonatal presentation. A risk algorithm model based on key echocardiographic parameters was developed to determine those who are at risk of early death.
Results:
Of the 19 neonates included, with median birth weight 3.1 kg (IQR 2.58–3.36), nine (47%) neonates died at median age of 5 days (IQR 4–17). All neonates showed retrograde diastolic flow at the level of descending aorta by colour Doppler on the first post-natal echocardiogram at median age of 2 days (IQR 1–5.5). An aortic antegrade-to-retrograde velocity time integral ratio of < 1.5 and supra-systemic pulmonary artery pressure had 100% positive predictive value of death (p = 0.029), whereas aortic antegrade-to-retrograde velocity time integral ratio of > 1.5 and sub-systemic pulmonary artery pressure had 100% positive predictive value of survival (p = 0.029).
Conclusion:
Combination of aorta antegrade-to-retrograde velocity time integral ratio and degree of pulmonary hypertension on the first post-natal echocardiogram may help stratify the severity of disease and guide optimal timing for neuro-intervention for neonatal vein of Galen aneurysmal malformation.
Through communication with caregivers, infants and toddlers come to orient intentionally to the world and their agency begins to unfold, initially through sharing intentions with adults. We show how the child’s emotional orientation to the world in the first three years of life happens through communication and is characterized by developmental periods that encompass other psychic functions. To understand children’s social situation from the child’s perspective, we have emphasized the need to attend to the child’s relations with others. We have therefore proposed taking a double perspective by focusing on both the child and the adults in their worlds. This is because the experiences of these very young children have to be seen in relation to how they are mediated by their caregivers. When caregivers or researchers orient to children’s perspectives, they never access it as a first perspective but always as a second, a relational, perspective and here we argue for Hedegaard’s formulated approach of the interactive observation. This method advocates a double perspective when researching children’s development, as the observations are mediated through the researcher’s experience.
We describe an outbreak of echovirus 18 infection involving 10 patients in our neonatal intensive care unit (an attack rate of 33%). The mean age at the onset of illness was 26.8 days. Eighty percent were preterm infants. All were discharged home without sequelae. There were no differences in gestation age, birth weight, delivery mode, use of antibiotics, and parenteral nutrition between the enterovirus (EV) group and non-EV group, but the rate of breastfeeding was significantly higher in the EV group. Separation care and reinforcement of hand-washing seemed to be effective in preventing further spread of the virus. Visiting policy, hygiene practice, and handling of expressed breastmilk should be reinforced.
A neonate was seen for an evolving broad QRS complex rhythm initially captured at birth as intermittent escape beats on electrocardiogram. Continuous monitoring recorded features mimicking pre-excitation, but closer analysis revealed a regular broad QRS complex rhythm with isorhythmic atrioventricular dissociation, favouring a ventricular source. Treatment with flecainide and propranolol achieved successful control of the incessant arrhythmia with improvement in cardiac function on echocardiogram.
A patent ductus arteriosus in patients with transposition of the great arteries is usually beneficial to allow shunting between pulmonary and systemic circulations. However, if the duct is too large, it can cause haemodynamic instability, pulmonary oedema and compromised organ perfusion. We present a neonate in whom a 5 mm short and tubular ductus arteriosus was causing significant cardiac failure with necrotising enterocolitis and liver impairment, leaving him too unstable for the arterial switch operation. At day 14 of life (3.1 kg), the baby underwent successful transcatheter closure using an Amplatzer vascular plug (Abbott, Chicago, Il, USA) delivered through a 5Fr catheter from the aortic side. The procedure was uncomplicated and successful as the neonate was extubated 2 days later. He subsequently underwent successful arterial switch surgery.
The culling of injured and non-viable pigs (Sus scrofa) (neonate to breeding stock) is a routine and necessary procedure on most farms. Usually, pigs are culled using one of the following methods: blunt-force trauma (manual and mechanical), captive-bolt stunners, electrical stunning and electrocution or carbon dioxide. Manual blunt-force trauma is one of the most widely used methods due to its low or absent operational and investment costs. However, as a method, it has serious limitations, which include the risk of incomplete concussion, pain, and distress. Manual blunt-force trauma is also aesthetically unpleasant to operators and wider society. To address these issues there has been significant recent research into the development of alternatives to manual blunt-force trauma, these include: captive-bolt stunners, on-farm, gas-based controlled atmosphere systems, low atmospheric pressure systems and electrical stunning. Some of these are currently in commercial use while others are still in the developmental phase. This review brings together the relevant research in this field, evaluating the methods in terms of mechanism of action (mechanical and physiological), effectiveness and animal welfare.