Primary pulmonary vein stenosis is a rare congenital heart condition and carries a poor prognosis. Various surgical techniques have been introduced to treat primary pulmonary vein stenosis. However, there is no consensus on the optimal approach. This study aimed to evaluate the outcomes of surgical treatment of primary pulmonary vein stenosis, identify the risk factors for pulmonary vein restenosis and reintervention, and determine the optimal strategy for primary pulmonary vein stenosis repair.

From December 2008 to December 2023, 64 patients with primary pulmonary vein stenosis undergoing surgical repair in our institution were included [non-sutureless group, n = 42; sutureless group, n = 22]. The Cox proportional hazard model was used to identify risk factors for pulmonary vein restenosis and pulmonary vein restenosis-related reintervention.

There were three in-hospital deaths and one late death. The survival rates at 1, 5, and 15 years were 91.9%, 84.2%, and 69.8%, respectively, in the whole cohort. Compared with the non-sutureless group, the sutureless group did not significantly improve the long-term survival of primary pulmonary vein stenosis patients (P = 0.484). Pulmonary vein restenosis occurred in 15 patients. Multivariable analysis showed that Involvement of the left inferior pulmonary vein (P < 0.001) was the only independent risk factor for pulmonary vein restenosis, while sutureless repair (P = 0.037) was a protective factor. Pulmonary vein restenosis-related reintervention occurred in 12 patients. On multivariate analysis, three or four pulmonary veins involvement (P = 0.001) and preoperative severity score exceeding 5 (P = 0.050) were found to be independent risk factors associated with pulmonary vein restenosis-related reintervention; however, sutureless repair (P = 0.021) was a protective factor.

Management of infants with primary pulmonary vein stenosis is challenging. Surgical repair of primary pulmonary vein stenosis can be safely achieved using different techniques, with similar long-term mortality. Compared with non-sutureless repair, sutureless repair was significantly associated with decreased pulmonary vein restenosis and decreased pulmonary vein restenosis-related reintervention, respectively. Preoperative severity score exceeding 5, 3, or 4 pulmonary veins involvement, and left inferior pulmonary vein involvement are independent risk factors for adverse outcomes.

Congenital heart Disease (CHD) is a significant cause of morbidity and mortality. Pulse-oximetry is a good non-invasive simple tool for critical CHD screening. Implications of this tool may be possible in certain areas and non-practical in others. We aim to report on the preliminary results of a recent ongoing protocol concerning the use of pulse-oximetry in detecting critical CHD in newborn in Egypt.

All neonates born in or transferred to 10 university hospitals during the period between February and November 2023 and fulfilled the criteria of inclusion were screened for critical CHD by pulse-oximetry using Granelli protocol in the first 24–72 hours after birth.

During a 10-month pilot period, a total of 2392 neonates were screened. A total of 549 neonates (23%) tested positive (failed) screening. Among the positive cases, 213 neonates (42%) died during their hospital stay, while the remaining were discharged or scheduled for intervention. The positive cases underwent echocardiography that revealed CHD in the majority of cases (80.3%). Only 40 cases of those cases had cardiac defects that are classified as critical CHD with a prevalence of 16.7 per 1000 live births, while the rest of the cases have either simple (non-critical CHD) or persistent pulmonary hypertension. About 19.7% of positive cases have completely free echocardiograms without cardiac defects.

The prevalence of critical CHD in Egypt is higher than the mean worldwide prevalence. The introduction of pulse-oximetry as a mass screening tool for critical CHD is possible and effective in low-income countries.

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.

Due to the high postoperative mortality, tools for an adaequate risk stratification are important to identify high-risk patients undergoing the Norwood procedure. As a marker of ventricular wall stress, NT-proBNP might be of particular interest in these children.

This study evaluated whether NT-proBNP’s age-adjusted z-score (“zlog-NT-proBNP”) predicts outcomes after stage I Norwood procedure.

Patients who underwent the Norwood procedure between 1 January 2011 and 31 December 2022, with perioperative NT-proBNP measurements available were enrolled. Since reference intervals of NT-proBNP are highly age-dependent, age-adjusted zlog-NT-proBNP was used. Serial zlog-NT-proBNP values were analysed to predict the occurrence of major adverse cardiovascular events after the Norwood procedure. Major adverse cardiovascular events was defined as death, resuscitation, or mechanical circulatory support. Secondary endpoints were re-operation and re-intervention for shunt.

A total of 139 patients underwent the Norwood procedure and had at least one NT-proBNP measurement available. Preoperative zlog-NT-proBNP measurements (median 3.7, interquartile range 3.1–4.19) showed no association with the occurrence of major adverse cardiovascular events or mortality. Zlog-NT-proBNP early after ICU admission (3.2, interquartile range 2.4–3.8) was predictive of mortality but showed no association with the occurrence of major adverse cardiovascular events. Zlog-NT-proBNP before ICU discharge (3.2, interquartile range 2.8–3.8) was significantly associated with the occurrence of both major adverse cardiovascular events (hazard ratio 1.83, 95% confidence interval 1.25–2.67, P = 0.002) and death (hazard ratio 2.1, 95% CI 1.4–3.2, P < 0.001).

High zlog-NT-proBNP levels after the Norwood surgery were strongly associated with the occurrence of major adverse cardiovascular events and death. Therefore, zlog-NT-proBNP has the potential to identify high-risk patients before life-threatening complications occur.

The most common cause of morbidity and mortality in type 1 diabetes mellitus is cardiovascular system involvement. Sudden death has been reported in type 1 diabetes mellitus patients. To analyse the use of the cardiac electrophysiological balance index in predicting ventricular arrhythmias in children with type 1 diabetes mellitus disease.

One hundred type 1 diabetes mellitus paediatric patients and 100 healthy children, divided into three groups according to their haemoglobin A1C levels, were included in the study. All participants were evaluated with transthoracic echocardiography, 12-lead electrocardiography, and 24-h Holter after a detailed physical examination. Systolic and diastolic function parameters, electrocardiography intervals, and heart rate variability parameters were evaluated.

The mean age in the type 1 diabetes mellitus and control groups was 11.52 (± 3.52) and 11.78 (± 3.45) years, respectively. In total, 57% of the type 1 diabetes mellitus group and 56% of the control group were male. The mean haemoglobin A1C value was 9.14% (± 1.79) and the disease duration was 3.71 years (± 3.13). The type 1 diabetes mellitus group had a higher QTc duration, deceleration time duration, A velocity, and a lower E/A ratio than the control group. In the type 1 diabetes mellitus group, Tpe, Tpe/QT ratio, QTc/QRS ratio, Tpe/QRS ratio, Tpe/(QT × QRS) ratio values were significantly higher than in the control group.

Children with type 1 diabetes mellitus are at risk for atrial and ventricular arrhythmias without valvular disease, ischemic heart disease, or diastolic dysfunction despite normal systolic function. No correlation was found between disease duration, metabolic control, autonomic function parameters, and arrhythmia risk; however, it was associated with diastolic function parameters.

Poor weight gain in infants with single ventricle cardiac physiology between stage 1 and stage 2 palliative surgeries is associated with worse outcomes. The growth of infants with single ventricle physiology, enrolled in home monitoring programmes in the United Kingdom, has not been widely described.

To explore the growth of infants with single ventricle physiology supported by a home monitoring programme, at a tertiary centre in the South of England.

A retrospective review of two cohorts, comparing weight gain amongst infants with single ventricle physiology, before and following the implementation of a home monitoring programme. Inclusion was dependent on a diagnosis compatible with single ventricle physiology during the interstage.

Enrolment into a home monitoring programme (cohort 2) was associated with 55% more infants being discharged home during the interstage period (p < 0.05). Interstage mortality did not differ between cohorts. There were no differences in interstage growth velocity between cohorts (cohort 1 23.98 ± 11.7 g/day and cohort 2 23.82 ± 8.3 g/day); however, infants in cohort 2 experienced less growth deceleration early in life, and achieved catch-up growth at 12-23 months. Interstage nasogastric feeding, regardless of the cohort, was associated with worse growth outcomes.

A home monitoring programme for infants with single ventricle physiology provides the opportunity for infants to be safely discharged home to their families and cared for at home during the interstage. Infants in the home monitoring programme experienced better growth, achieving weight restoration at 12–23 months.

Interprofessional teams in the pediatric cardiac ICU consolidate their management plans in pre-family meeting huddles, a process that affects the course of family meetings but often lacks optimal communication and teamwork.

Cardiac ICU clinicians participated in an interprofessional intervention to improve how they prepared for and conducted family meetings. We conducted a pretest–posttest study with clinicians participating in huddles before family meetings. We assessed feasibility of clinician enrollment, assessed clinician perception of acceptability of the intervention via questionnaire and semi-structured interviews, and impact on team performance using a validated tool. Wilcoxon rank sum test assessed intervention impact on team performance at meeting level comparing pre- and post-intervention data.

Totally, 24 clinicians enrolled in the intervention (92% retention) with 100% completion of training. All participants recommend cardiac ICU Teams and Loved ones Communicating to others and 96% believe it improved their participation in family meetings. We exceeded an acceptable level of protocol fidelity (>75%). Team performance was significantly (p < 0.001) higher in post-intervention huddles (n = 30) than in pre-intervention (n = 28) in all domains. Median comparisons: Team structure [2 vs. 5], Leadership [3 vs. 5], Situation Monitoring [3 vs. 5], Mutual Support [ 3 vs. 5], and Communication [3 vs. 5].

Implementing an interprofessional team intervention to improve team performance in pre-family meeting huddles is feasible, acceptable, and improves team function. Future research should further assess impact on clinicians, patients, and families.