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Congenital hyperinsulinism is a clinical syndrome of pancreatic β-cell dysfunction characterized by failure to suppress insulin secretion in the presence of hypoglycemia. Here, we describe the concerns, the techniques used to ameliorate these potential problems, and the outcomes for a child with this condition undergoing cardiopulmonary bypass for correction of an atrial septal defect.

Background: Since 1996, ABO-incompatible heart transplantation has been undertaken by performing whole-body plasma exchange to remove isohemagglutinins using the cardiopulmonary bypass (CPB) circuit at the time of transplantation. This requires large volumes of donated blood and blood products, causes hemodynamic instability during the exchange transfusion, and limits practical use to small children. We sought to determine the efficacy of anti-A/B immunoadsorption within the CPB circuit on removal of isohemagglutinins in an ex vivo setting before its use clinically., Methods: An anti-A/B immunoadsorption column was placed into a CPB circuit mimicking a typical ABO-incompatible transplant patient, which had been primed with type O whole human blood. Samples were taken for determination of isohemagglutinin titers following each plasma volume pass through the anti-A/B immunoadsorption column., Results: There was a linear decrease of at least 1 dilution seen in both anti-A and anti-B IgG and IgM antibodies with each plasma volume pass through the column. This predictable removal allowed the formulation of selection criteria for ABO-incompatible heart transplantation given the reciprocal of titer and patient weight. This degree of predictability allowed us to use it successfully in the clinical setting, reducing antibodies to an undetectable level during ABO-incompatible heart transplantation., Conclusions: The incorporation of an anti-A/B immunoadsorption column into the extracorporeal circuit reduces allogeneic blood product requirement for ABO-incompatible heart transplantation, while providing efficacious removal of anti-A and anti-B isohemagglutinins. This can be undertaken within the time period of CPB before graft reperfusion and expands the potential recipient pool to larger patients with higher isohemagglutinin titers., (Crown Copyright © 2018. Published by Elsevier Inc. All rights reserved.)

Beta (β)-thalassemia is a blood disorder with an incidence of 1 in 100,000. 1 This case report outlines a patient with β-thalassemia requiring cardiopulmonary bypass (CPB) and the measures taken to ensure an uneventful procedure.

Objectives: Colloid osmotic pressure (COP) is a major determinant of fluid shift in paediatric patients undergoing corrective surgery for congenital heart disease (CHD) using cardiopulmonary bypass (CPB). However, very few baseline data are available for those patients requiring surgery within the first few weeks and months of life. Our aim was to determine if our CHD population exhibited COP similar to that of other extremely ill subsets of patients and, if so, whether this related to risk stratification model scores., Methods: Thirty consecutive patients under 10 kg underwent cardiac surgery with CPB. Data were collected on COP, albumin concentration, prime composition, post-operative length of ventilation and intensive care unit (ICU) stay as well as risk stratification utilising Risk Adjustment for Congenital Heart Surgery (RACHS-1) and Partial Risk Adjustment in Surgery (PRAiS) scoring systems., Results: The patients had a mean pre-bypass COP of 13.9±2.5 mmHg. A significant negative correlation was observed between pre-operative baseline COP and length of ventilation (r=0.7; p<0.001) and a significant negative correlation between PRAiS (r=0.64; p<0.001) and RACHS-1 (r=0.5; p=0.004) scores and baseline COP was seen., Conclusions: Neonatal and paediatric patients requiring surgical correction of complex congenital cardiac malformations exhibit extremely low baseline COP, comparable to other sick neonatal populations, and are lower than those previously reported. Baseline COP correlates significantly with predicted survival rates and time spent on a ventilator.

Major aortopulmonary collateral arteries (MAPCAs) provide significant issues during cardiopulmonary bypass, including flooding of the surgical field which requires significant blood volumes to be returned to the extracorporeal circuit via handheld suckers. This has been shown to be the major source of gaseous microemboli and is associated with adverse neurological outcome. Use of pH-stat has been previously shown to decrease the shunt through MAPCAs via an unknown mechanism. Here, we report the associated benefits of pH-stat in decreasing sucker usage and gaseous microemboli in a patient with known MAPCAs presenting for repair of tetralogy of Fallot and pulmonary atresia., (© The Author(s) 2014.)

Introduction: Great Ormond Street Children's Hospital undertakes over 500 open heart cardiothoracic procedures requiring cardiopulmonary bypass per year. Data from our centre show that many of our neonatal/paediatric patients require higher cardiac indexes than previously thought. We evaluated the new Pixie oxygenation system, rated from 0.1 L/min to 2 L/min, to determine if it could be used for these patients., Methods: Between 2010 and 2012, 250 Pixie oxygenators were used on consecutive patients requiring correction of congenital cardiac defects. Data were collected on FiO2 requirements, oxygenator pressure drop and gaseous microemboli handling. Retrospective analysis was also undertaken on the procedures and demographics of all patients during 2011-2012 to determine the percentage of patients on whom the Pixie could be used., Results: Analysis of the procedures undertaken at Great Ormond Street Hospital (GOSH) showed that 89% were in patients under 20 kg, requiring a flow rate of <2 L/min (at a base cardiac index of 2.8 L/min/m2). The maximum FiO2 required at 2.5 L/min was 85%. Gaseous microemboli were reduced by 82.5±9.9% and bubble volume was decreased by 94.3±8.4% from the 'venous' pre-oxygenator to the 'arterial' post-oxygenator., Discussion: The Pixie oxygenator proved effective at flows up to 2.5 L/min, with air-handling capabilities comparable with other oxygenators. This represents a single oxygenator that could potentially be used to cover 89% of our surgical procedures. However, we believe that, for the smallest patients (i.e., < 2 kg), a smaller priming oxygenator should be used in order to limit unnecessary haemodilution in this vulnerable group.

We present a series of three children with sickle cell disease aged 3 months, 3 weeks and 18 months, all presenting for cardiac surgery requiring cardiopulmonary bypass. The cardiac lesions were atrioventricular septal defect, transposition of the great arteries and ventricular septal defect, with sickle cell loads of 35%, 11% and 39% respectively at presentation. We calculated that the bypass circuit would provide sufficient volume to decrease sickle cell levels to safe values, so we decided to proceed to bypass without pre-operative exchange transfusion, and modified the bypass technique so as to avoid the likely stimulants of a sickle cell crisis. Haemoglobin S levels after the start of bypass were significantly lower than before bypass, and remained low throughout the case and into the second postoperative day. By adopting this approach, we feel that we achieved a successful outcome with minimal distress to the children and their families.