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.

Acute rheumatic fever commonly causes carditis in children. It is critical to have biomarkers for diagnostic and prognostic purposes. We aimed to investigate the expressions of miR-101, miR-1183, and miR-1299 in children with acute rheumatic fever and rheumatic heart disease and determine their potential as diagnostic biomarkers.

This cross-sectional study was conducted on 69 paediatric acute rheumatic fever patients and 27 gender and age-matched controls. The clinical findings were noted from medical records, echocardiography was repeated, and plasma miR-101, miR-1183, and miR-1299 expressions were studied using RT-PCR.

Out of 54 patients with carditis, 36 developed rheumatic heart disease with significant left-sided heart involvement. No significant demographic difference was present between rheumatic heart disease patients and controls (p > 0.05). miR-101 and miR-1183 expressions significantly reduced by 4.5-fold in acute rheumatic fever patients compared to controls (p : 0.029 and p : 0.024, respectively). Family history was found to be significantly associated with 9-fold reduced miR-1183 expression in acute rheumatic fever patients (p : 0.005). The expression of miR-1183 in acute rheumatic fever children with carditis in general and severe carditis was significantly reduced 4.5- and 9-fold, respectively (p : 0.024 and p : 0.007, respectively). In acute rheumatic fever cases with moderate-severe carditis and patients who did not develop rheumatic heart disease, miR-1183 expression significantly reduced 8.3-fold and 9-fold, respectively (p : 0.015 and p : 0.019, respectively). The ROC analysis revealed that miR-101 and miR-1183 were statistically significant for acute rheumatic fever diagnosis (AUC: 0.64 for both miRNAs).

Significantly lower plasma expressions of miR-101 and miR-1183 in acute rheumatic fever patients, especially in cases with moderate-severe carditis, might indicate the potential use of these miRNAs as diagnostic biomarkers.

Cases of Fontan failure with normal Fontan pressure have been reported. This study aimed to identify catheterisation-derived haemodynamic predictors of heart transplantation/death, other than Fontan pressure, in late post-Fontan patients.

This retrospective study evaluated post-Fontan patients who underwent cardiac catheterisation at age ≥10 years between 1993 and 2018. The predictive effect of cardiac index-systemic vascular resistance index plot and perfusion pressure on freedom from the primary outcome (heart transplantation/death) was evaluated. Patients were categorised into haemodynamic categories A (cardiac index ≥ 3, systemic vascular resistance index ≥ 13), B (cardiac index < 3, systemic vascular resistance index ≥ 13), C (cardiac index ≥ 3, systemic vascular resistance index < 13), and D (cardiac index < 3, systemic vascular resistance index < 13).

In total, 79 patients (median age: 15.7 [range: 10.1–50.2] years) were included; of them, the primary endpoint occurred in 10 (13%; median follow-up: 1.9 [range: 0.1–18.8] years). Category C patients had significantly shorter freedom from the endpoint than categories A and B patients. Univariate analysis identified significant haemodynamic predictors, including Fontan pressure, pulmonary/systemic vascular resistance index, pulmonary/systemic flow, systemic arterial oxygen saturation, systemic venous oxygen saturation, systemic vascular resistance index, perfusion pressure, perfusion pressure < 53 mmHg, and category C. In multivariable analysis, perfusion pressure < 53 mmHg and category C emerged as predictors of heart transplantation/death alongside Fontan pressure.

Haemodynamic profiling of late post-Fontan patients using the cardiac index-systemic vascular resistance index plot can aid to comprehend the post-Fontan status and predict clinical prognosis.

This study aimed to evaluate veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection.

Patients who underwent staged total cavopulmonary connection between 1995 and 2022 were reviewed. Veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection were depicted using angiograms. The prevalence of veno-venous collaterals, the risks for the development of veno-venous collaterals, and the impact of veno-venous collaterals on outcomes were analysed.

In total, 586 patients were included. Veno-venous collaterals were found in 72 (12.3%) patients. Majority of veno-venous collaterals originated from the superior caval vein and drained into the inferior caval vein. Before bidirectional cavopulmonary shunt, mean pulmonary artery pressure (16.3 vs. 14.5 mmHg, p = 0.018), and trans-pulmonary gradient (9.5 vs. 8.0 mmHg, p = 0.030) were higher in patients with veno-venous collaterals compared to those without. Veno-venous collaterals intervention was performed in 32 (5.5%) patients, in a median of 29 (16–152) days after bidirectional cavopulmonary shunt. Before total cavopulmonary connection, pulmonary artery pressure (10.3 vs. 9.4 mmHg, p = 0.015) and ventricular end-diastolic pressure (8.4 vs. 7.6 mmHg, p = 0.035) were higher, and arterial oxygen saturation (SaO2, 80.6 vs. 82.6 %, p = 0.018) was lower in patients with veno-venous collaterals compared to those without. More palliations before total cavopulmonary connection (p < 0.001, odds ratio: 1.689) were an independent risk for the development of veno-venous collaterals. Veno-venous collaterals did not affect survival after total cavopulmonary connection (92.8 vs. 92.7% at 10 years, p = 0.600).

The prevalence of veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection was 12%. Veno-venous collaterals may be induced by the elevated pulmonary artery pressure and trans-pulmonary gradient, and also by more previous palliations. However, they had no impact on clinical outcomes following total cavopulmonary connection.

Transcatheter closure of large and complex atrial septal defect can pose challenges and complications during device placement. To improve stability, several assistive techniques have been developed.

This retrospective study evaluated the efficacy of the device-assisted device closure technique for large secundum atrial septal defects. Patients who underwent device-assisted device closure of atrial septal defect between December 2023 and August 2024 were analysed.

Twenty patients (mean age 38.69 years) underwent device closure of large secundum atrial septal defect with device-assisted device closure technique. The mean atrial septal defect diameter was 31.9 mm. The average thick-to-thick measurement was 38.3 mm, which determined the device size. The majority (18 cases) had thin, floppy margins and two had deficient inferior rim. Successful closure was achieved in 18 patients (90%), while two patients (10%) required other methods of assistance. Based on fluoroscopic guidance, patients were divided into two groups: Group A (8 patients) used anteroposterior projection, and Group B (12 patients) used left anterior oblique-cranial view. After initial two failures with anteroposterior view, all cases were successfully closed using left anterior oblique-cranial projection. Device sizes ranged from 36 to 50 mm (median 40 mm). Cocoon devices were used for sizes up to 42 mm, and Occlunix for larger devices. No significant procedural complications occurred, although two patients had minor post-procedural events.

Device-assisted device closure technique offers a promising and safe dynamic assistance approach for transcatheter closure of large and challenging atrial septal defects. The left anterior oblique-cranial view showed promising results, though without statistical significance. While results are encouraging, larger prospective studies are needed to validate its effectiveness.

Glenn procedure carries low morbidity and mortality within stages of single-ventricle palliation. However, some patients with Glenn failure need a stage reversal, while others require unanticipated surgical interventions. Our understanding of perioperative factors and outcomes associated with such unexpected interventions is extremely limited.

Patients who underwent unexpected surgery after the Glenn procedure between January 2010 and December 2019 within the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) were identified with a subgroup analysis of those reverting to stage I physiology. Patient’s requiring reversal to stage I palliation were matched 1:5 with controls. Multivariable logistic regression analysis was performed to evaluate risk factors for reintervention.

A total of 16,913 patients underwent Glenn procedure with 1221 (7.2%) requiring unexpected cardiac surgical intervention and 95 (0.56%) patients required takedown to a stage I. Significant clinical and operative risk factors were identified for such unexpected interventions.

The overall mortality after Glenn procedure was 1.2%, while mortality after unexpected reintervention was 6.6% at 30 days and after Glenn takedown was 27.5% at last follow-up. Unexpected surgical intervention and right ventricular dominance were significant risk factors for mortality.

Unexpected reinterventions, including need for takedown after the Glenn procedure, are associated with significantly higher mortality. Further studies should focus on improving our patient selection, understanding the risk factors mechanistically, including impact of the right ventricle as systemic ventricle in order to avoid need for unexpected surgical interventions.

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.

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.

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.

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.

Despite patent ductus arteriosus closure in premature infants is a relatively fast procedure, it involves specific steps for equipment exchange and the use of a 4-Fr catheter, which may be large for tiny infants, potentially causing haemodynamic instability or tricuspid valve regurgitation.

This study aims to describe the early experience with a new technique designed to simplify transcatheter patent ductus arteriosus closure in premature infants. It enables the closure using the KA micro plug device via a single microcatheter without equipment exchanges and contrast exposure. This technique involves a simple modification of the microcatheter by creating a curved tip.

This is a retrospective, single-institution study involving premature infants who underwent attempted transcatheter patent ductus arteriosus closure using a manually modified microcatheter of the KA micro plug device at Stead Family Children’s Hospital.

Five patients underwent transcatheter patent ductus arteriosus closure using a KA micro plug with a modified microcatheter. The procedure was successful in all patients, with no haemodynamic instability. The median (range) for procedural weight, time, and radiation dose were 1055 g (730–1330), 12 minutes (11–19), and 0.49 µGy.m2 (0.14–1), respectively. At a median follow-up of 1.1 months (0.17–2.6), all patients maintained good device position without residual shunt or other concerning echocardiogram findings.

Transcatheter patent ductus arteriosus closure in infants weighing less than 1500 g can be successfully performed using a KA micro plug device through a microcatheter with zero-contrast use, low radiation exposure, and a short procedure time.

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.

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.

The sutureless repair technique has been favoured due to its purported reduction in post-operative pulmonary venous obstruction rates. This study aims to compare the outcomes of conventional versus sutureless repair techniques in Total Anomalous Pulmonary Venous Drainage.

In this retrospective single-centre analysis (2012–2022), we evaluated children who underwent conventional or sutureless repair for isolated total anomalous pulmonary venous drainage, excluding complex cardiac anomalies and incomplete data. Patients were categorised into conventional (Group C, n = 58) and sutureless (Group S, n = 41) groups. Primary outcomes included mortality, morbidity, and post-operative complications. Statistical analysis included Mann–Whitney U, chi-square, and Fisher’s exact tests where appropriate.

Supracardiac type predominated in both groups (53.4% in Group C and 70.7% in Group S), with higher cardiac type frequency in Group C (24.1% versus 2.4%, p = 0.016). Early complications occurred in 58.5% versus 53.4% of cases in Groups S and C, respectively (p = 0.767). The mortality rate (17.2% versus 14.6%, p = 0.944) and post-operative pulmonary venous obstruction (21.2% versus 19.0%, p = 0.809) were higher in Group C, though not significantly. Mean cardiopulmonary bypass times were comparable between groups (105 versus 89 minutes, p = 0.424).

In this comprehensive analysis of paediatric Total Anomalous Pulmonary Venous Drainage repair, both conventional and sutureless techniques demonstrated comparable safety profiles and clinical outcomes. These findings suggest that surgical approach selection should be individualised based on patient characteristics and surgeon expertise. Further prospective studies with larger cohorts are needed to validate these observations.