A patient post-Fontan palliation with a venous collateral unusually arising from the renal vein. Since renal vein oxygen saturations are relatively high, there was not systemic desaturation despite a right-to-left shunt.
A patient post-Fontan palliation with a venous collateral unusually arising from the renal vein. Since renal vein oxygen saturations are relatively high, there was not systemic desaturation despite a right-to-left shunt.
Introduction: This study compared health status and developmental skill acquisition of children aged 3-5 years with and without CHD and identified predictors of special education or early intervention plan.
Materials and methods: Data were analysed from the 2022 National Survey of Children's Health using complex weighted survey data procedures. Chi-square tests compared health status and developmental skill acquisition of children aged 3-5 years with and without CHD. Multivariate logistic regression identified predictors of the need for special education or early intervention plan.
Results: 11,097 National Survey of Children's Health responses pertained to children aged 3-5 years. Children aged 3-5 years with CHD were more likely than heart-healthy peers to be born prematurely, have special healthcare needs, have parent-reported health as "fair" or "poor," be diagnosed with anxiety, depression, or a developmental disorder, and receive special education or an early intervention plan. Children aged 3-5 years with CHD were less likely to have acquired communication, fine motor, personal social, and problem-solving skills than comparators at the time of the survey, even after adjustment for special healthcare needs. Having public plus private insurance, special healthcare needs designation, and a developmental disorder predicted children aged 3-5 years needing special education or an early intervention plan.
Conclusion: Children with predictors of receiving special education or an early intervention plan may benefit from early identification and support. Further research should investigate the impact of systemic disparities on developmental skill acquisition in children with CHD.
Objectives: The bidirectional Glenn surgery is an important staging procedure for patients with single ventricle physiology. Approximately 1000 children are born each year in the United States with this subset of CHD. There is limited data regarding optimal post-operative management for these children. We surveyed paediatric cardiac intensive care providers surrounding their management strategies after the bidirectional Glenn surgery.
Design: An anonymous survey was distributed via email to paediatric cardiac intensive care providers. The survey included anonymised demographic data and focused on post-operative physiologic targets for patients recovering after the bidirectional Glenn surgery.
Subjects: Thirty-five paediatric cardiac intensive care providers responded to an anonymous 12-question survey. Subjects were mostly comprised of paediatric cardiac intensive care attendings (80%), with an average of 7.86 years of training. The respondents primarily practised in settings with medical trainees, and all practised in settings with extracorporeal membrane oxygenation capabilities.
Intervention: Respondents were asked to complete a web-based survey. Five of the survey questions were devoted to background demographic data, and seven questions were aimed at identifying physiologic targets. Two of the seven questions were in relation to a provided clinical vignette.
Measurements and main results: This survey demonstrated that there is a lack of consensus in the management of patients after the bidirectional Glenn surgery. Specifically, granular SpO2, mean arterial pressure, and pH Goals were all less than 75% consensus. This survey highlights the variable practice patterns in providers taking care of patients after the bidirectional Glenn surgery, and further demonstrates the need for physiologic and outcome-driven targets to optimise the post-operative care.
Background: Utilisation of nicardipine in the neonatal and infant period has been historically avoided due to a concern for a more calcium-sensitive myocardium. The aim of this study was to characterise the association between nicardipine and systolic blood pressure in neonates and infants after cardiac surgery.
Methods: In this single-centre, retrospective study, patients under 12 months of age who underwent cardiac surgery and received nicardipine for at least one hour were included (September 2022 to January 2024). Patients were monitored with Etiometry. Variables of interest included haemodynamic parameters, ionised calcium, serum lactate, vasoactive infusion score, and nicardipine dose. A time series regression was conducted with each patient having 5 distinct time points.
Results: One hundred and eighty-five time points were collected across 37 patients with a mean age of 3 months. Of these patients, 22% were neonates and 32% were functionally univentricular. With nicardipine utilisation, a decrease in systolic blood pressure of 14 mmHg after an 8-hour time period was noted (p = 0.017). Heart rate, diastolic blood pressure, cerebral and renal oxygen extraction, ionised calcium, serum lactate, and vasoactive inotrope score did not significantly change over the study period.
Conclusion: Nicardipine utilisation in neonates and infants after cardiac surgery was associated with decreased systolic blood pressure. Indirect markers demonstrate no change in cardiac function. Additional studies are needed to better elucidate nicardipine's role in this patient population.
Introduction: Mahaim ablation poses a significant challenge among accessory pathway ablations. This study aims to present our findings on Mahaim ablation performed using a contact force catheter, emphasizing both the effectiveness and safety of this technique.
Materials and methods: This is a single-centre retrospective cohort study. Twenty-two consecutive patients who underwent Mahaim pathway ablation procedures between January 2017 and January 2025 were included in the study. The EnSite Precision System (Abbott, St. Paul, MN, USA) was used to facilitate mapping and to reduce or eliminate the need for fluoroscopy. Ablation was performed using the TactiCath catheter.
Results: The median age was 11 years (range: 7-18). Antidromic tachycardia was induced in all patients with the left bundle block. All patients underwent successful ablation with the TactiCath catheter. Ablation was performed via the jugular approach in 11 patients and via the femoral region in another 11 patients. Ablation procedures were performed under atrial pacing in four patients regarding catheter stabilization. Locations of the Mahaim signals were at the right anterolateral (n = 6), right lateral (n = 7), and right posterolateral (n = 9) tricuspid annulus. The acute success rate was 100% (22/22). Fluoroscopy was not used on any patient. The median procedure time was 132 minutes (range: 72-411). No major complications were observed. One recurrent patient who presented with a tachycardia attack one month later had Ebstein's anomaly. An ablation procedure was successfully performed from the right lateral region.
Conclusion: In paediatric Mahaim tachycardia ablations, the use of a contact force catheter can be safe and successful.
Introduction: Children with CHD are at risk for neurodevelopmental impairments, and though these are often mild, some children face severe developmental challenges. Both unalleviated pain and exposure to opioids in the neonatal period have detrimental effects on the developing brain.
Method: We developed and implemented a Comfort Curriculum including a standardised sedation pathway, bedside non-pharmacologic reference, and holding guidelines. Our primary aim was to assess the effect of the Comfort Curriculum on opioid exposure. The secondary aim was to assess the effect of the Comfort Curriculum on pain scores in neonates in the first 5 days after surgery. A retrospective cohort study of all cardiac surgical patients ≤30 days of age at the time of their first operation was conducted before and at two points after implementation of the Comfort Curriculum (3 months and 15 months).
Results: We found that initial and maximum opioid infusion rates significantly decreased between the pre-implementation and both post-implementation phases, while pain scores did not increase. The total cumulative opioid doses in the first five post-operative days showed a non-statistically significant decrease in both post-implementation phases compared to the pre-implementation phase, and median pain scores showed a trend towards decreasing in both post-implementation phases.
Discussion: After implementation of the Comfort Curriculum, we found a significant decrease in the initial and maximum opioid doses and a signal towards a reduction in total opioid dose in the first 5 days after neonatal cardiac surgery.
Background: Percutaneous device closure is the preferred method for closing patent ductus arteriosus (PDA), even in preterm infants. We report our experience using the new KONAR-MFO™ ventricular septal defect occluder for transcatheter closure of preterm patent ductus arteriosus as an alternative device in resource-limited centres.
Methodology: Case 1: A preterm baby with Down's syndrome and tracheobronchomalacia was born at 29 weeks, weighing 1.68 kg with multiple cardiac defects, including a 4 mm PDA and a 6 mm mid-muscular ventricular septal defect (VSD), stuck on a ventilator. Case 2: Another preterm baby born at 35 weeks, weighing 1.89 kg, with anorectal malformation and a right inguinal hernia. The echocardiogram revealed a 4 mm PDA with severe PAH. In both cases, the patent ductus arteriosus was occluded using a 6 mm × 4 mm Konar MFO device on day 14 (case 1) and day 20 (case 2).
Results: The baby (case 1) was weaned off the ventilator and discharged on the 28th postnatal day at 2.09 kg. As for case 2, the baby was weaned off the ventilator within 2 days and discharged on day 30 at 2.23 kg. A follow-up echocardiogram of both cases confirmed a well-positioned device with no obstructions. At 18 months, in the follow-up, both babies were gaining weight and thriving.
Conclusions: The KONAR MFO device is a safe and effective option for patent ductus arteriosus closure in preterm infants, even in resource-limited settings. Its versatility accommodates various duct sizes and morphologies, and its self-expandable design ensures easy deployment, addressing the anatomical challenges often seen in preterm infants.
Background: Patients with 22q11.2 deletion are known to have immune abnormalities. Data on the immune profile of non-syndromic patients with conotruncal heart defects are limited.
Methods: A prospective study evaluated the genetic and immunological profiles and early to mid-term postoperative outcomes of patients with conotruncal heart defects.
Results: Infants with 22q11.2 deletion had low leukocyte counts, while low total lymphocyte counts were observed in all patients except infants without a genetic syndrome. Reduced CD3+, CD4+, and CD8+ cells were found in 22q11.2 deletion neonates and infants, as well as in infants without a genetic syndrome. Immunoglobulin G, M, and A abnormalities occurred across all groups. T cell receptor excision circle levels were lowest in patients with complex heart defects. Kappa-deleting recombination excision circle levels were increased in patients without a genetic syndrome. Early postoperative infections were frequent in all groups. Neonates with 22q11.2 deletion had longer ICU stay and higher need for antibiotics and hospital readmission at 3 and 6 months of follow-up.
Conclusion: Neonates and infants with conotruncal heart defects have low preoperative T lymphocyte counts, reduced T cell receptor excision circle and immunoglobulin levels, and high incidence of postoperative infections. Higher kappa-deleting recombination excision circle levels compensated the T cell disbalances in patients without a genetic syndrome. The presence of a 22q11.2 deletion with conotruncal heart defects was associated with prolonged mechanical ventilation, longer ICU length of stay, higher need for antibiotic treatment after discharge from the hospital, and readmission risk in neonates after cardiac surgery.
Objective: Transcatheter closure of secundum atrial septal defects is well-established, but data on very large defects requiring 40-50 mm occluders remain limited. This multicenter retrospective study evaluated the safety and efficacy of percutaneous closure using large atrial septal occluders.
Methods: Patients undergoing atrial septal defect device closure with 40-50 mm occluders from June 2015 to May 2025 at two tertiary centres were analysed. Patients were grouped by device size: Group A (40-42 mm; n = 34) and Group B (44-50 mm; n = 28).
Results: The cohort (n = 62) had a mean age of 42.07 years with female predominance (70.96%). Mean atrial septal defect size was 33.03 mm (thick-to-thick: 38.71 mm), with mean pulmonary artery pressure of 22.33 mmHg and left ventricular end-diastolic pressure of 12.59 mmHg. Aneurysmal septum with thin or deficient posteroinferior rim was common (77.4% and 19.4%, respectively). Procedural success was 93.5%. Device instability or residual flow led to surgical closure in three cases; one had intraprocedural embolisation. Procedural assistance (balloon/device, etc.) was needed in 93.5%. Fenestrated devices were used in four (6.5%) patients-due to severe pulmonary stenosis, diastolic dysfunction, or pulmonary hypertension. "Cobra" configuration and device bulkiness were noted in 9.7% and 6.5%, respectively. Arrhythmias occurred in 25.8%, mostly transient. One patient developed device-related endocarditis, and one late, unrelated death occurred due to intracranial haemorrhage. At a mean follow-up of 29.29 months, outcomes were satisfactory.
Conclusion: Transcatheter closure of large atrial septal defects with 40-50 mm devices is safe and effective with proper anatomical assessment, procedural planning, and vigilant follow-up in selected patients.

