The treatment for proximal aortic arch hypoplasia in paediatric patients is still controversial. While some authors favours direct tissue anastomosis, others state that patch augmentation may also be a good alternative. The aim of this study is to compare the results of arch reconstructions using bovine pericardium with the direct anastomosis technique.

Paediatric patients who underwent arch reconstruction via median sternotomy between 2019 and 2023 were evaluated. Patients were divided into two groups according to the repair method of arch reconstructions: direct native tissue anastomosis and bovine pericardial patch augmentation. Using perioperative data, the relationship between the surgical method and postoperative morbidity, in-hospital mortality, and the risks for early reintervention was investigated.

Between August 2019 and August 2023, 38 paediatric patients underwent arch reconstruction. The average age and weight of the patients were 40 days (15–157.5 days, interquartile) and 3.78 kg (3.2–6.0 kg, interquartile range), respectively. While completely native tissue anastomosis was applied in 18 of the patients (47.4%), bovine pericardial patch was used in arch reconstruction in 20 patients (52.6%). Cross-clamp time was found to be significantly longer in patients using bovine patches (p = .016). No difference was detected between the two surgical methods in terms of postoperative mortality and morbidity factors (p > .05). There was no significant difference between the two surgical procedures in terms of reintervention in the early period after discharge (p = .177).

Although early results of both reconstruction techniques may be promising, their reliability needs to be evaluated in detail with large-scale prospective studies.

This study investigated the prevalence of malnutrition, time to achieve caloric goals, and nutritional risk factors after surgery for CHD in a cardiac ICU.

This retrospective study included patients with CHD (1 month-18 years old) undergoing open-heart surgery (2021–2022). We recorded nutritional status, body mass index-for-age z-score, weight-for-length/height z-score, cardiopulmonary bypass and aortic cross-clamp time, Paediatric Risk of Mortality-3 score, Paediatric Logistic Organ Dysfunction-2 score, vasoactive inotropic score, total duration of mechanical ventilation, length of stay in the cardiac ICU, mortality, and time to achieve caloric goals.

Of the 75 included patients, malnutrition was detected in 17% (n= 8) based on the body mass index-for-age z-score and in 35% (n= 10) based on the weight-for-length/height z-score. Sex, mortality, cardiopulmonary bypass and aortic cross-clamp time, Paediatric Risk of Mortality-3, Paediatric Logistic Organ Dysfunction-2, and vasoactive inotropic score, duration of mechanical ventilation, and length of cardiac ICU stay were similar between patients with and without malnutrition. Patients who achieved caloric goals on the fourth day and those who achieved them beyond the fourth day showed statistical differences in mortality, maximum vasoactive inotropic score, duration of mechanical ventilation, cardiopulmonary bypass and aortic cross-clamp time, Paediatric Risk of Mortality-3, Paediatric Logistic Organ Dysfunction-2, and length of cardiac ICU and hospital stay (p< 0.05). Logit regression analysis indicated that the duration of mechanical ventilation, Paediatric Logistic Organ Dysfunction-2 and Paediatric Risk of Mortality-3 score was a risk factor for achieving caloric goals (p< 0.05).

Malnutrition is prevalent in patients with CHD, and concomitant organ failure and duration of mechanical ventilation play important roles in achieving postoperative caloric goals.

Infants who require cardiopulmonary bypass for surgical repair of CHD are at high risk for secondary infections, which cause significant death and disability. The risk of secondary infection is increased by immune dysfunction. The intestinal microbiome calibrates immune function. Infants with CHD have substantial changes in their intestinal microbiome. We performed this scoping review to describe the current understanding of the relationship between the intestinal microbiome and immune function after pediatric cardiac surgery with cardiopulmonary bypass.

We searched the PubMed, Cumulative Index to Nursing and Allied Health Literature, Cochrane, and Scopus databases with the assistance of a medical librarian. We included trials that analysed intestinal microbiome composition and immune function after cardiac surgery with cardiopulmonary bypass in infants.

We found two observational cohorts and two interventional trials describing composition of intestinal microbiome and some measures of immune function after heart surgery with cardiopulmonary bypass in infants. A total of 114 children were analysed. Three trials were exclusively in infants, and one was in older children and infants. All trials found a differential composition of the intestinal microbiome in infants with CHD compared to those without CHD, and one described a robust correlation between composition of the intestinal microbiome with cytokine profile and adverse outcomes.

Despite robust preclinical data and data from other disease states, there is minimal data about the correlation between immune function and intestinal microbiome composition in infants with CHD after cardiopulmonary bypass.

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.

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.

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.

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.

Coronary artery lesions are the most severe complications of Kawasaki disease. Despite recent advances, evidence of the association between risk factors and coronary artery lesion is lacking. In this study, we demonstrated the potential clinical indicators that could assist to evaluate the prevalence of coronary artery lesion among paediatric patients with Kawasaki disease.

We retrospectively enrolled 260 paediatric patients with Kawasaki disease. Patients with coronary dilation, coronary aneurysm, and intimal thickening of coronary arteries were included in this study. Medical records of each patient were collected. Logistic regression analysis was performed to explore risk factors and the occurrence of coronary artery lesion in patients with Kawasaki disease.

Respectively, 64 (24.6%), 39 (15%), and 56 patients (21.5%) of the participants had coronary dilation, coronary aneurysm, and intimal thickening of coronary arteries. Univariate analysis revealed that age, gender, duration of fever, time of initial use of intravenous immunoglobulin, erythrocyte sedimentation rate, white blood cell counts, time of platelet increase, the maximum value of platelet, albumin, and immunoglobulin G level was associated with coronary artery lesion. In multivariable logistic analysis, those younger and mainly males were associated with all three outcomes of coronary artery lesion, lower serum albumin levels, and later initial use of intravenous immunoglobulin were linked to a higher risk of coronary dilation and coronary aneurysm.

The potential risk factors that could be used to estimate the occurrence of coronary artery lesion in Kawasaki disease patients are young age, male, lower serum albumin lever, and later initial use of intravenous immunoglobulin. However, long-term follow-up and multi-centre studies are required to verify our findings in the future.

Haemodynamic instability is common after surgical repair of CHDs in infants and children. Monitoring cardiac output in addition to traditional circulation parameters could improve the postoperative care of these patients. Echocardiography and transpulmonary thermodilution are the two most common methods for measuring cardiac output in infants.

To compare the results of cardiac output measurements using echocardiography and a transpulmonary thermodilution setup after paediatric cardiac surgery.

Forty children, scheduled for elective repair of a ventricular septal defect or of an atrio-ventricular septal defect using cardiopulmonary bypass, were enrolled in this prospective, observational study. Cardiac output was simultaneously measured using echocardiography and a commercially available transpulmonary thermodilution method (PiCCO™) at 18 h after the end of surgery.

At 18 h after surgery, PiCCO™ gave a mean of 3.0% higher cardiac output than echocardiography. This difference was not statistically significant. 95% of the observations fell within –50.0 to 82.6%.

The methods were found to have a good agreement on average, with no statistically significant difference between them. However, the spread of the results was large. It is questionable whether the methods can be used interchangeably in clinical practice.

An aberrant right subclavian artery represents the most common aortic arch vascular anomaly. Conventional wisdom states that these anomalies do not result in dysphagia, but rather serve as “red herrings”. Clearly, in the vast majority of cases, this holds true. Nonetheless, one should never say never.

Herein, we present a cohort of four children with debilitating dysphagia resulting from an aberrant right subclavian artery. Subclavian reimplantation via a right posterolateral thoracotomy was performed successfully in all cases.

Dysphagia resolved postoperatively, and all patients were able to advance to a normal diet. They were able to gain appropriate weight postoperatively and continue to do well at most recent clinical follow-up.

This case series suggests that aberrant right subclavian artery anatomy should be considered a potential aetiology of dysphagia, albeit rarely. Surgical intervention for select patients can provide dramatic resolution of symptoms.

Microcephalic osteodysplastic primordial dwarfism (MOPD) syndrome type 2, caused by a mutation in the PCNT gene (21q22.3), is a rare autosomal recessive disorder. Patients present with bone dysplasia, insulin resistance, kidney diseases, and cardiac malformations, making them prone to vascular diseases. Cardiomyopathy, hypertension, and coronary diseases are documented. The prognosis is associated with cerebrovascular complications.

We report a case of a patient with MOPD type II who suffered a myocardial infarction in our institution. Informed consent for publishing was obtained.

A 17-year-old female with MPOD II syndrome (20 kg and 86 cm) was referred for chest pain. Thoracic pains had been occurring for over a month, increasing in intensity, with an episode prompting emergency consultation. Initial tests revealed elevated troponin and an inflammatory response. Electrocardiogram (ECG) showed ST-segment depression and elevation. Echocardiography revealed hypokinetic inferior walls with moderate concentric hypertrophy. A coronary CT scan showed subendocardial hypodensity. Diagnostic coronary angiography revealed tri-branch lesions and almost complete stenoses or occlusions on the circumflex artery (Image). No indication for interventional treatment due to diffuse atheromatous lesions. Exclusive medical treatment was initiated.

MPOD II syndrome is associated with cardiac malformations and neurovascular complications, including myocardial infarction. Regular ECG monitoring is advisable. Active surveillance for coronary diseases is necessary from adolescence. Recognising this complication allows for prompt intervention. This case highlights the need for specific monitoring and prompt management of chest pain in patients with MPOD II syndrome. Primary prevention could mitigate the occurrence of coronary events in this high-risk population.