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Background: Extracorporeal membrane oxygenation (ECMO) is used in children with cardiopulmonary failure. While the majority of ECMO centers use unfractionated heparin, other anticoagulants, including factor XI and factor XII inhibitors are emerging, which may prove suitable for ECMO patients. However, before these anticoagulants can be applied in these patients, baseline data of FXI and FXII changes need to be acquired., Objectives: This study aimed to describe the longitudinal profile of FXI and FXII antigenic levels and function before, during, and after ECMO in children., Methods: This is a prospective observational study in neonatal and pediatric patients with ECMO (<18 years). All patients with venoarterial ECMO and with sufficient plasma volume collected before ECMO, on day 1 and day 3, and 24 hours postdecannulation were included. Antigenic levels and functional activity of FXI and FXII were determined in these samples. Longitudinal profiles of these values were created using a linear mixed model., Results: Sixteen patients were included in this study. Mean FXI and FXII antigenic levels (U/mL) changed from 7.9 and 53.2 before ECMO to 6.0 and 34.5 on day 3 and they recovered to 8.8 and 39.4, respectively, after stopping ECMO. Function (%) of FXI and FXII decreased from 59.1 and 59.0 to 49.0 and 50.7 on day 3 and recovered to 66.0 and 54.4, respectively., Conclusion: This study provides the first insights into changes of the contact pathway in children undergoing ECMO. FXI and FXII antigen and function change during ECMO. Results from this study can be used as starting point for future contact pathway anticoagulant studies in pediatric patients with ECMO., (© 2023 The Authors.)

Background: One potential approach for advancing univentricular heart surgical palliation outcomes is by stem cell therapy to augment right ventricular function and muscle mass. Whether the stem cell-inclusive cord blood mononuclear cells (CBMNCs) are safe to perfuse into the coronary vasculature during neonatal cardiopulmonary bypass (CPB) is unknown. We evaluated the acute safety, functional effects, and fate of human CBMNCs in a novel model of coronary vasculature delivery in a lamb model of infant CPB., Methods: Neonatal lambs were randomized in blinded fashion to receive control (n = 5) or human CD45(+) CBMNCs (8 × 10(6) cells/kg body weight, n = 7) treatments during CPB. Aortic cross-clamp time was 40 minutes, with maintenance blood cardioplegia delivered every 10 minutes. Pressure-volume indices were used to measure left ventricular function before CPB and 60 minutes after CPB. CBMNCs were assessed by flow cytometry and immunohistochemistry., Results: CBMNC-treated lambs were hemodynamically stable after CPB, with a decline in left ventricular pressure-volume indices similar to controls. The coronary vasculature was patent on microscopy, without evidence of cell aggregates or clots. Human CD45(+) cells were distributed in high abundance within all cardiac regions, predominantly the right atrium and ventricles, and trafficked beyond endothelial cell layers and between myocytes. CD45(+) cells localized at low incidence in the spleen, liver, lungs, and kidneys, but rarely remained in the circulation (<0.1% of infused cells)., Conclusions: Coronary delivery of human CBMNCs during blood-cardioplegic arrest in a lamb model of CPB results in highly abundant myocardial distribution of cells without acute adverse effects on vascular patency and post-CPB cardiac function., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Outcomes after operations for bicuspid aortic valve disease in pediatric patients were determined., Methods: Between 1977 and 2011, 146 consecutive patients underwent surgical repair of bicuspid aortic valve. Median age at operation was 207 days (range, 5 days to 16 years). Indication for surgery was stenosis in 113, insufficiency in 25, and both in 8. Valve debridement was done in 76 patients, and complex repairs in 70, including 61 who required addition of pericardial patches, consisting of the creation of a neocommissure in 55, cusp extension in 33, and a perforation repair in 6. The valve was made tricuspid in 38 patients (29 cusp extensions)., Results: Twenty-year survival was 88% (95% confidence interval, 73% to 95%). After a mean follow-up of 8 ± 7 years, 35 patients needed a reintervention. Freedom from reintervention at 18 years was 43% (95% confidence interval, 28% to 56%). At the latest follow-up, an additional 13 patients without reoperation had moderate or severe stenosis, and 17 had moderate regurgitation. Seventy-eight patients had an event-free long-term outcome (no reintervention, stenosis, or regurgitation). The only independent predictive factors of an event-free outcome were not having addition of patch material at repair (hazard ratio, 12; p = 0.05) and shorter bypass time (HR, 1.01; p = 0.023). The 10-year freedom from any significant event was 60% (95% confidence interval, 46% to 71%) for those without use of patch material, whereas nearly all those with a patch repair had an adverse event at that time., Conclusions: Outcomes after surgical repair of bicuspid aortic valves in the pediatric population are excellent, especially if the repair can be performed without the addition of patches. Primary repair should be offered because long-lasting results can be achieved if the disease can be relieved by simple procedures., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Outcomes after atrioventricular (AV) valve operations in patients with functional single ventricles are unclear., Methods: From 1988 to 2010, 76 consecutive patients with single ventricles underwent AV valve operations for regurgitation at a single institution. Five replacements, 66 repairs, and 5 valve closures were performed at a median age of 1 year (range, 1 day-14 years) on 43 tricuspid, 9 mitral, and 24 common AV valves., Results: Hospital mortality was 17% (13/76). The follow-up was 100% complete. There were 15 late deaths. There were 48 survivors with a mean follow-up of 8.3±6 years. One- and 10-year Kaplan-Meier survival after AV valve operations was 72% (95% confidence interval [CI], 60%-81%) and 61% (95% CI, 48%-71%), respectively. Independent predictors of overall mortality were presence of a common AV valve (p=0.03), requirement for postoperative mechanical circulatory support (p=0.02), and timing of valve operations between initial palliation and performance of a bidirectional cavopulmonary shunt (BCPS) (p=0.047). Ten-year freedom from valve reoperation and from thromboembolic events of hospital survivors was 56% (95% CI, 38%-70%) and 70% (95% CI, 56%-80%), respectively. At last follow-up, 11 of 48 surviving patients (23%) had moderate to severe regurgitation, and pacemaker implantation was required in 6 patients. Only 34 patients reached the stage of Fontan completion., Conclusions: AV valve regurgitation is a considerable burden for the patient with a single ventricle, especially when appearing at a young age. A quarter of patients died within the first year after operation, and they had considerable morbidity in terms of reoperation, thromboembolic events, and pacemaker implantation. Their chances of reaching Fontan completion seemed decreased., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Perioperative brain injury is common in infants undergoing cardiac surgery. Amplitude-integrated electroencephalography (aEEG) provides real-time neurologic monitoring and can identify seizures and abnormalities of background cerebral activity. We aimed to determine the incidence of perioperative electrical seizures, and to establish the background pattern of aEEG, in neonates undergoing Norwood-type palliations for complex congenital heart disease in relation to outcome at 2 years., Methods: Thirty-nine full-term neonates undergoing Norwood-type operations underwent aEEG monitoring before and during surgery and for 72 hours postoperatively. The perfusion strategy included full-flow moderately hypothermic cardiopulmonary bypass with antegrade cerebral perfusion. Amplitude-integrated electroencephalography tracings were reviewed for seizure activity and background pattern. Survivors underwent neurodevelopmental outcome assessment using the Bayley Scales of Infant Development (3rd edition) at 2 years of age., Results: Thirteen (33%) infants had electrical seizures, including 9 with intraoperative seizures and 7 with postoperative seizures. Seizures were associated with significantly increased mortality, but not with neurodevelopmental impairment in survivors. Delay in recovery of the aEEG background beyond 48 hours was also associated with increased mortality and worse motor development., Conclusions: Perioperative seizures were common in this cohort. Intraoperative seizures predominantly affected the left hemisphere during antegrade cerebral perfusion. Delayed recovery in aEEG background was associated with increased risk of early mortality and worse motor development. Ongoing monitoring is essential to determine the longer-term significance of these findings., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Repair of total anomalous pulmonary venous drainage (TAPVD) in neonates remains a challenge as it is often associated with severe obstruction. We describe a large cohort of neonates who underwent TAPVD repair at a single institution., Methods: From 1973 to 2008, 112 patients underwent simple TAPVD surgery during the first month of life. Data collection occurred retrospectively., Results: Preoperative pulmonary venous obstruction (PVO) occurred in 89 (79.5%) patients. There were 12 (10.7%) early deaths. Significant risk factors were bypass time greater than 65 minutes (p=0.014) and emergent surgery (p=0.005). Hospital mortality was unchanged throughout the 3 eras (1973 to 1988, 1989 to 1998, 1999 to 2008), despite an increase in patients with preoperative acidosis (p=0.004) and severe TAPVD obstruction (p=0.038) during the recent 10 years. There were 6 (6.25%) late deaths within 2 years of repair. Survival at 20 years was 83.4% (95% confidence interval 75 to 89). Risk factors for late death were operative weight 2.5 kg or less (p=0.004) and postoperative pulmonary hypertensive crisis (p=0.02). Reoperation for recurrent PVO was required in 13 patients (11.9%). Risk factors were operative weight 2.5 kg or less (p=0.035) and postoperative pulmonary hypertensive crisis (p=0.002). Follow-up was 96% complete and survivors (n=90) were asymptomatic at a median age of 11.7 years., Conclusions: Hospital mortality remained unchanged over the 36-year period. Survival beyond 2 years offers excellent outcome. Risk factors for mortality were the preoperative clinical status, prolonged bypass time, persisting micro-obstruction, and low operative weight. A reduction in mortality will likely require development of effective medical management for patients who have peripheral PVO not amenable to surgical repair., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)