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OBJECTIVES Several procedures have been described to correct pectus excavatum and carinatum. We have used a modified Ravitch procedure (STRATOS titanium bars) for patients who were unsuitable for the minimally invasive Nuss procedure. The operation produced excellent cosmetic results, but we have noted several fractures and displacements of the STRATOS bars. METHODS We reviewed all our STRATOS patients since first use in 2009 until 2014. We collated the following data of each patient: diagnosis, severity of pectus deformity, comorbidity, previous operations for pectus, age at bar implantation, the number of bars implanted and duration of implantation. Patients with severe comorbidity were excluded. RESULTS Between 2009 and 2014, 39 patients were treated for pectus deformities using the STRATOS bars. Twelve of 39 patients (31%) were identified as having bar(s) broken before removal. There were no significant differences between patients with fractured bars and those without, except for the number of bars implanted (P  = 0.016) CONCLUSIONS This incidence of bar fracture is unexpectedly high, although the literature on this topic is sparse. The high fracture rate is alarming, because of its unpredictability and lack of clear aetiological factors. The operation is done (largely) for psychosocial and cosmetic reasons, and therefore, we must have complete confidence in the quality of the bars used in teenage pectus patients. We raise the issue to warn other units and encourage them to report bar fracture rates. [ABSTRACT FROM AUTHOR]

OBJECTIVES Our study describes and analyses the results from aortopexy for the treatment of airway malacia in children. METHODS Demographic data, characteristics and preoperative, operative and outcome details, including the need for reintervention, were collected for children undergoing aortopexy between 2006 and 2016. RESULTS One hundred patients [median age 8.2 months, interquartile range (IQR) 3.3–26.0 months] underwent aortopexy. Sixty-four (64%) patients had tracheomalacia (TM) only, 24 (24%) patients had TM extending into their bronchus (tracheobronchomalacia) and 11 (11%) patients had bronchomalacia. Forty-one (41%) children had gastro-oesophageal reflux disease, of which 17 (41%) children underwent a Nissen fundoplication. Twenty-eight (28%) children underwent a tracheo-oesophageal fistula repair prior to aortopexy (median 5.7 months, IQR 2.9–17.6 months). The median duration of follow-up was 5.3 years (IQR 2.9–7.5 years). Thirty-five (35%) patients were on mechanical ventilatory support before aortopexy. Twenty-seven (77%) patients could be safely weaned from ventilator support during the same admission after aortopexy (median 2 days, IQR 0–3 days). Fourteen patients required reintervention. Overall mortality was 16%. Multivariable analysis revealed preoperative ventilation (P  = 0.004) and bronchial involvement (P  = 0.004) to be adverse predictors of survival. Only bronchial involvement was a predictor for reintervention (P  = 0.012). CONCLUSIONS Aortopexy appears to be an effective procedure in the treatment of children with severe airway malacia. Bronchial involvement is associated with adverse outcome, and other procedures could be more suitable. For the treatment of severe airway malacia with isolated airway compression, we currently recommend aortopexy to be considered. [ABSTRACT FROM AUTHOR]

OBJECTIVES Jeune syndrome (asphyxiating thoracic dystrophy) is a rare disorder characterized by skeletal dysplasia, reduced diameter of the thoracic cage and extrathoracic organ involvement. Fatal, early respiratory insufficiency may occur. Two-stage lateral thoracic expansion has been reported, addressing each side sequentially over 3–12 months. While staged repair theoretically provides less invasive surgery in a small child with respiratory distress, we utilized a single stage, bilateral procedure aiming to rapidly maximize lung development. Combined bilateral surgery also offered the chance of rapid recovery, and reduced hospital stay. We present our early experience of this modification of existing surgical treatment for an extremely rare condition, thought to be generally fatal in early childhood. METHODS Nine children (6 males, 3 females; median age 30 months [3.5–75]) underwent thoracic expansion for Jeune syndrome in our centre. All patients required preoperative respiratory support (5 with tracheostomy, 8 requiring positive pressure ventilation regularly within each day/night cycle). Two children underwent sequential unilateral (2-month interval between stages) and 7 children bilateral thoracic expansion by means of staggered osteotomies of third to eighth ribs and plate fixation of fourth to fifth rib and sixth to seventh rib, leaving the remaining ribs floating. RESULTS There was no operative mortality. There were 2 deaths within 3 months of surgery, due to pulmonary hypertension (1 following two-stage and 1 following single-stage thoracic expansion). At the median follow-up of 11 months (1–15), 3 children have been discharged home from their referring unit and 2 have significantly reduced respiratory support. One child remains on non-invasive ventilation and another is still ventilated with a high oxygen requirement. CONCLUSION Jeune syndrome is a difficult condition to manage, but bilateral thoracic expansion offers an effective reduction in ventilator requirements in these children. While two-stage repair has been described previously, this is the first report of single-stage bilateral thoracic expansion. Single-stage repair is feasible and may offer better resource management and significant cost savings by potentially reducing theatre usage and overall length of stay (intensive care and hospital) without compromising clinical outcomes. [ABSTRACT FROM AUTHOR]

Objective: To evaluate effects of the material of the cardiopulmonary bypass (CPB) tubes (polyvinyl chloride, PVC) and prime solutions on expression of neutrophil adhesion molecule CD11b and L-selectin. Methods: We carried out a series of experiments using donor blood from 30 healthy adult human volunteers. In all experiments, neutrophil cell surface expressions of CD11b and L-selectin were assayed immediately and serially up to 2 hours, using immune-fluorescence techniques and flow cytometry. Study 1: Effects of PVC were compared with glass and polystyrene (n = 5). Study 2: Blood was mixed with Plasma-lyte (Pl) (prime solution), Hartman solutions, albumin or not altered (control), n = 5. Study 3: The effects of changing pH of the Pl (control, neutralised and acidic solution, n = 5) were examined. Study 4: Haemodilution (undiluted, 1:1, 1:2, and 1:3, vol/vol, prime to blood, n = 5) was carried out using Pl and the subsequent changes in expressions of the adhesion molecules were analysed. Study 5: The combined effect of PVC and Pl was assessed (n = 5). Study 6: We evaluated the effect of increasing plasma water by adding sterile water to whole blood and compared it with control (n = 5). Results: Study 1: PVC, similar to glass, caused more up-regulation of CD11b and down-regulation of L-selectin than polystyrene (238 and 162% vs. 68 increase of CD11b, P < 0.001; 89 and 95% vs. 16% decrease of L-selectin, P < 0.001). Study 2: Pl and Hartman solutions caused more up-regulation of CD11b and down-regulation of L-selectin compared to albumin and control (166 and 188% vs. 26 and 44% increase of CD11b, P < 0.01; 19 and 26% vs. 10 and 6% decrease of L-selectin, P < 0.01, respectively). Study 3: Haemodilution had no effect on these molecules. Study 4: The mean of the difference between the acidic and neutral solution was 208% increase of CD11b and 30% decrease of L-selectin, P < 0.05. Study 5: The combined effect of mixing blood with Pl and exposure to PVC caused marked up-regulation of CD11b (336% increase, P < 0.01) and down-regulation of L-selectin (78% decrease, P < 0.05). Study 6: Water for injection caused marked up-regulation of CD11b and down-regulation of L-selectin. Conclusions: Mixing blood with acidic prime solution and/or exposing it to PVC tubes causes up-regulation of neutrophil adhesion molecule CD11b and down-regulation of L-selectin. Neutralisation of the prime solution reduces the extent of neutrophil activation, whereas haemodilution has no effect. Increasing plasma water is stimulating to the neutrophil. Modulation of prime solutions and the material of CPB tubes may reduce neutrophil activation which may reduce patient morbidity. [ABSTRACT FROM PUBLISHER]

Objective: To examine the role of heparin in modulating neutrophil activation and release of cytokine. Background: Up-regulation of CD11b, down-regulation of l-selectin on neutrophil cell surface and release of IL8 occur in response to extracorporeal circulation (ECC) and were proposed to cause leakage of the capillaries in patients. Design: In a series of experiments, we examined the effect of heparin (4 U/ml) comparing it with ethylenediamine tetra-acetate (EDTA, 1.5 mg/ml) and citrate mixture (100 μ1/ml), heparin dose-response, IL8 (human recombinant IL8) dose-response and protamine (80 μg/ml) neutralisation of heparin (4 U/ml) using donor blood (total of 38). The role of complement component type 3 (C3) was tested. Neutrophils from a patient with complete C3 deficiency were stimulated by using heparin and cobra venom factor (10 μg/ml) and compared with controls (n = 5). CD11b and l-selectin expressions were assayed immediately and serially up to 120 min using immune fluorescence and flow cytometry. Serum concentrations of IL8 were determined by using enzyme-linked immunosorbent assay. Results: The medians of up-regulation of CD11b were 540.2 (range 235.2–653.3) for heparin vs. 186.5 (55.7–207.1) for EDTA and 192.5 (69.2–263.8) for citrate mixture, P < 0.01. The medians of down-regulation of l-selectin were 79 (32–192) for heparin vs. 18.4 (0–188) for EDTA and 36.2 (7.4–135) for citrate mixture, P < 0.05. Up-regulation of CD11b, down-regulation of L-s and release of IL8 were inversely related to heparin concentration (r = 0.87, P < 0.05). Serum concentration of IL8 had a direct relationship to the changes in CD11b and l-selectin expression (r = 0.92). Heparin-protamine complex was less stimulant to expression of CD11b and l-selectin than heparin or protamine (P < 0.05). In blood samples from C3-deficient patients, heparin and cobra venom factor caused up-regulation of CD11b and down-regulation of l-selectin similar to that of controls (P > 0.05). Conclusions: Heparin stimulates up-regulation of neutrophil adhesion molecules CD11b, down-regulation of l-selectin and release of IL8. These effects are inversely related to heparin concentration and are independent of C3 activation. IL8 has a direct relationship to activation of neutrophil adhesion molecules. Increasing heparin dosage reduces neutrophil activation and may reduce the morbidity of patients. [ABSTRACT FROM PUBLISHER]

Objective: Upregulation of neutrophil adhesion molecules (CD11b and L-selectin) and release of a modulating cytokine (IL8) have been reported in vivo and in vitro in adult cardiopulmonary bypass. The aim of this study was to determine whether paediatric bypass preparations have similar influences and whether neutrophil-endothelium interactions are required for IL8 release. Methods: In vitro paediatric cardiopulmonary bypass circuits (n = 15) were constructed (identical to those used clinically), as well as static loops (n = 15) using donor blood. The effects of circulation and temperature (17 °C, 25 °C, 37 °C) on the initiation of acute inflammation were examined. Cellular expressions of neutrophil adhesion molecules CD11b and L-selectin were assayed by immunofluorescence technique, and serum IL8, IL6, TNF-/ga, leucocyte elastase, and terminal complement complex were measured by elisa. Results: In all experiments, an immediate increase in CD11b expression occurred [median values, in relative fluorescence units: 64.9 (range 45.3–212.9) at rest; 365.2 (205–835.4) at 10 min; P < 0.001], along with a decrease in L-selectin expression [153.5 (115.5–220.7) at rest; 42 (12–134) at 10 min; P < 0.01]. Serum concentrations of the following increased gradually and were higher in circulation than in static loops: IL8 [1500 (500–2500) pg·ml−1 in circuit v 600 (180–1500) pg·ml−1 in loop, P < 0.001]; TNF-α [400 (120–1100) v 50 (0–80) pg·ml−1, P < 0.001]; leucocyte elastase [1388 (778–6977) v 833 (175–1800) ng·ml−1, P < 0.05]; and terminal complement complex [25.9 (6.8–120) v4.7 (0–21.6) AU·ml−1, P < 0.01]. Cooling decreased and rewarming increased upregulation of CD11b and downregulation of L-selectin and release of IL8. IL6 was undetectable. Conclusions: In the absence of endothelium, in vitro paediatric cardiopulmonary bypass causes profound acute inflammatory changes in donor blood with release of IL8. These changes were greater than in adult cardiopulmonary bypass. Temperature variation and circulation modulate the responses. [ABSTRACT FROM PUBLISHER]

Abstract: Objective: The Aristotle Score has been proposed as a measure of ‘complexity’ in congenital heart surgery, and a tool for comparing performance amongst different centres. To date, however, it remains unvalidated. We examined whether the Basic Aristotle Score was a useful predictor of mortality following open-heart surgery, and compared it to the Risk Adjustment in Congenital Heart Surgery (RACHS-1) system. We also examined the ability of the Aristotle Score to measure performance. Methods: The Basic Aristotle Score and RACHS-1 risk categories were assigned retrospectively to 1085 operations involving cardiopulmonary bypass in children less than 18 years of age. Multiple logistic regression analysis was used to determine the significance of the Aristotle Score and RACHS-1 category as independent predictors of in-hospital mortality. Operative performance was calculated using the Aristotle equation: performance=complexity×survival. Results: Multiple logistic regression identified RACHS-1 category to be a powerful predictor of mortality (Wald 17.7, p <0.0001), whereas Aristotle Score was only weakly associated with mortality (Wald 4.8, p =0.03). Age at operation and bypass time were also highly significant predictors of postoperative death (Wald 13.7 and 33.8, respectively, p <0.0001 for both). Operative performance was measured at 7.52units. Conclusions: The Basic Aristotle Score was only weakly associated with postoperative mortality in this series. Operative performance appeared to be inflated by the fact that the overall complexity of cases was relatively high in this series. An alternative equation (performance=complexity/mortality) is proposed as a fairer and more logical method of risk-adjustment. [Copyright &y& Elsevier]

In 1998, the first report of the Society of Thoracic Surgery (STS) National Congenital Heart Surgery Database reported the clinical features of 18 congenital heart categories. The report provided a significant amount of important information and also highlighted the strengths and weaknesses of the existing database. Following this report, the STS Congenital Heart Surgery Committee, in cooperation with the European Association of Cardio-Thoracic Surgery and the European Congenital Heart Surgeons Foundation, initiated the International Congenital Heart Surgery and Nomenclature Database Project. The goal was to begin the standardization of nomenclature and reporting strategies and establish the foundations for an international congenital heart surgery database. The first report of the International Congenital Heart Surgery Nomenclature Project was published in the Annals of Thoracic Surgery in April 2000. The current report outlines modifications to the minimal dataset, as well as the diagnosis and procedure short lists. [Copyright &y& Elsevier]

Abstract: Objective: To evaluate the safety and effectiveness of novel biodegradable (BD) stents to treat bronchial anastomotic stenosis in patients after lung transplantation. Methods: Twenty BD stents were implanted endoscopically in six patients (median age 41.5 years (range 35–57 years)) with post-transplant bronchial anastomotic stenoses, between 2006 and 2010. All stents were custom-made from bio-absorbable polydioxanone (PDS). The median stent diameter was 12mm (8–17mm) and median length was 20mm (12–30mm). All patients were evaluated clinically, by bronchoscopy and high-definition computed tomography (CT). Results: The stenosis was initially relieved in all cases. There was no bleeding, perforation or displacement after BD stent implantation. Four patients needed multiple stenting for anastomotic re-stenosis. Median time to any re-stenting was 5 months (2–15 months). There was one sudden death, 1 year after the last BD stent implantation, from a pulmonary embolus. All five survivors are in good clinical condition up to 4 years’ follow-up (median 40 months, range 7–48 months) since first stenting and intervention-free up to 44 months (median 24 months, range 7–44 months). Conclusions: This small pilot study shows that BD stents are a safe, effective and reliable alternative to classical metallic stents in patients with anastomotic stenosis after lung transplantation, and may avoid the need for permanent stenting. [Copyright &y& Elsevier]

Abstract: Objective: We introduced a fast-track program for our cardiac operations requiring adjustment in anaesthesia techniques to facilitate rapid extubation and discharge from the intensive care unit (ICU). Our objective was to investigate the quality of analgesia in fast-track paediatric cardiac patients. Methods: We performed a retrospective review of the records of all patients who were fast-tracked in our institution between January 2006 and January 2007. Data collected included surgical procedure, anaesthesia technique, intra-operative opioids, ventilation time, intensive care stay, postoperative morphine consumption, pain scores, patient-controlled analgesia/nurse-controlled analgesia (PCA/NCA) duration, supplemental analgesia and incidence of vomiting. Results: Fifty-four patients were studied, with a median age of 5.6 years (8 months–18 years), median weight 15.6kg (range: 6.4–101kg), median intensive care unit (ICU) ventilation time 1.1h (range: 0–8h) and median ICU stay of 4.1h (1–52h). All patients received intra-operative fentanyl, median dose of 16.8mcgkg−1 (range: 15–20mcgkg−1). Twenty-three children received a bolus of morphine intra-operatively median dose of 102mcgkg−1 (range: 50–170mcgkg−1). Those patients who did not receive a morphine bolus intra-operatively, received a 100mcgkg−1 loading dose of morphine in the ICU. Twenty-four patients received intravenous paracetamol intra-operatively and five patients were given both paracetamol and diclofenac. Twenty-five children were not given either paracetamol or diclofenac intra-operatively. During the postoperative period, all patients received morphine by infusion administered via either PCA (18%) or NCA)(73%). The median PCA/NCA infusion time was 28.9h. Forty-eight patients received paracetamol and non-steroidal analgesics postoperatively, either diclofenac or ibuprofen. Five patients received only paracetamol and only one patient required no supplemental analgesia. The bedside nurse reported the pain scores on an hourly basis on a 10-point visual analogue score where 0=no pain and 10=strongest pain. Pain scores showed that most patients after day 0 (which was the day of surgery) had only mild pain. Conclusions: Our data showed that our program achieves high-quality analgesia in fast-track paediatric cardiac patients. [Copyright &y& Elsevier]

Abstract: Objective: Fast-track patient pathways for cardiac surgery are used in adult practice and by necessity is a mainstay in the developing world. We aimed to introduce a fast-track protocol for uncomplicated paediatric open-heart surgery cases and to subsequently review the results of this change in practice. Methods: A fast-track protocol co-ordinated by the Advanced Nurse Practitioners was introduced in January 2006 for children aged over 6 months undergoing uncomplicated open-heart procedures. We conducted a review of prospectively collected data on all included patients. The setting was a tertiary paediatric cardiac surgical centre in the UK. The outcome measures for audit were: patient fitness to leave the intensive care unit (ICU) on the day of surgery and hospital length of stay. Results: Included children had a mean age 6 (standard deviation (SD) 4.9) years and mean weight 22.7 (SD 17.6) kg. Of the 194 patients included, 153 (79%) were fit to leave the ICU on the day of surgery. Patients undergoing surgery for ventricular septal defect: odds ratio (OR) 2.8 (95% CI: 1.2–5.6) P =0.01 and left ventricular outflow tract obstruction: OR 5.5 (95% CI: 1.4–21.2) P =0.01, were more likely to be unfit than atrial septal defect and right ventricular outflow tract obstruction. Patients undergoing surgery in the afternoon were more likely to be unfit than those undergoing surgery in the morning: OR 2.3 (95% CI: 1.2–4.8) P =0.03. No relationship was found between age or weight and fitness to fast track. Median length of hospital stay for the whole cohort was 3 (range: 2–11) days. After adjustment for case mix, there was significant evidence that length of hospital stay reduced as experience with the protocol increased over the series of patients RC −0.02 (95% CI: −0.01 to −0.03) P <0.01. Conclusion: A fast-track programme can be implemented safely and effectively if the appropriate support including a step-down ward area is put in place. Greater experience with this type of protocol leads to reductions in the length of hospital stay for children aged over 6 months undergoing uncomplicated open-heart surgery. Fast-track cases should be performed in the morning. [Copyright &y& Elsevier]

Abstract: Objective: The aim of this study was to develop a graphical method of risk-stratified outcome analysis in paediatric cardiac surgery to provide a means of continuous, prospective performance monitoring and allow real-time detection of change in outcomes. Methods: Risk-adjusted survival following open-heart surgery was prospectively measured over a 15-month period (n =460). Outcomes were charted using variable life-adjusted display (VLAD) charts, which indicate the cumulative difference in observed minus expected survival against the cumulative number of cases performed. Risk stratification was based on RACHS-1 (risk adjustment in congenital heart surgery) risk category and age at surgery, using our previously published risk model. The probability of deviation in performance from the expected baseline level was determined using a mathematical model. Results: By the end of the series, observed survival (443/460=96.3%) exceeded that predicted by the risk model (434.5/460=94.5%), equivalent to a one-third reduction in expected mortality. Mathematical modelling indicated a 1–5% likelihood that this difference would have occurred by random variation alone, suggesting the outcomes represented genuine improvement. Conclusions: VLAD charts provide an effective, easily visualised display of surgical performance and can be applied to paediatric cardiac surgery. Early detection of change, whether improvement or deterioration, is important for ongoing quality assurance within a cardiac surgery programme. [Copyright &y& Elsevier]

Objective: The analysis of the second harvest of the STS Congenital Heart Surgery Database produced meaningful outcome data and several critical lessons relevant to congenital heart surgery outcomes analysis worldwide. Methods: This data harvest represents the first STS multi-institutional experience with software utilizing the nomenclature and database requirements adopted by the STS and EACTS (April 2000 Annals of Thoracic Surgery). Members of the STS Congenital Heart Committee analyzed the STS data. Results: This STS harvest includes data from 16 centers (12787 cases, 2881 neonates, 4124 infants). In 2002, the EACTS reported similar outcome data utilizing the same database definitions (41 centers, 12736 cases, 2245 neonates, 4195 infants). Lessons from the analysis include: (1) Death must be clearly defined. (2) The Primary Procedure in a given operation must be documented. (3) Inclusionary and exclusionary criteria for all diagnoses and procedures must be agreed upon. (4) Missing data values remain an issue for the database. (5) Generic terms in the nomenclature lists, that is terms ending in Not Otherwise Specified (NOS), are redundant and decrease the clarity of data analysis. (6) Methodology needs to be developed and implemented to assure and verify data completeness and data accuracy. ‘Operative Mortality’ and ‘Mortality Assigned to this Operation’ were defined by the STS and EACTS; these definitions were not utilized uniformly. ‘Thirty Day Mortality’ was problematic because some centers did not track mortality after hospital discharge. Only ‘Mortality Prior to Discharge’ was consistently reported. Designation of Primary Procedure for a given operation determines its location for analysis. Until Complexity Scores lead to automated methodology for choosing the Primary Procedure, the surgeon must designate the Primary Procedure. Inclusionary and exclusionary criteria for all diagnoses and procedures have been developed in an effort to define acceptable concomitant diagnoses and procedures for each analysis. Improvements in data completeness can be achieved using a variety of techniques including developing more functional techniques of data entry at individual institutions and software improvements. Future versions of the STS Congenital Database will request that the coding of diagnoses and procedures avoid the terms ending in NOS. Conclusions: Lessons from this data harvest should improve congenital heart surgery outcome analysis. [Copyright &y& Elsevier]

Abstract: Objective: The aim of the study is to assess the surgical outcome of slide tracheoplasty in patients with congenital tracheal stenosis and single lung. Methods: Pre, intra- and postoperative data were collected. Anatomy and associated anomalies were described. Results: Seven patients (median age 5 months; range 39 days–1 year) with single lung underwent slide tracheoplasty. Single right lung was present in four patients. Associated anomalies were present in four patients (56%) including vascular ring, left pulmonary artery (LPA) sling, VACTERL (abnormalities of the vertebrae, anus, cardiovascular tree, trachea, oesophagus, renal system, and limb buds) syndrome, atrial septal defect (ASD) and aberrant left subclavian artery. Six patients (85%) needed preoperative ventilation and two (28%) needed preoperative extracorporeal membrane oxygenation (ECMO). Median postoperative ventilation was 7 days (6–35 days). Two patients needed postoperative ECMO, one of whom required preoperative ECMO. Complications occurred in five patients (71%): reintubation due to pneumothorax, pneumonia and several tracheal dilatations due to recurrent tracheal stenosis, which was eventually stented. There was one hospital death in a patient, who could not be weaned off ECMO due to severe distal malacia. At a median follow-up of 16 months (7 days–7 years), all survivors are in good clinical condition and without additional stenting. Conclusion: Slide tracheoplasty can be performed in patients with single lung and tracheal stenosis with a good surgical outcome. [Copyright &y& Elsevier]