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The article focuses on a study which shows that a stem-cell-based, tissue-engineered tracheal transplant is a potential therapeutic option for patients. It mentions that patients treated with autologous stemcell-derived transplants do not need immunosuppression. It reflects that normal airway and lung function, high quality of life, and the elimination of the need for repeated surgical interventions were the end-points of a tracheal replacement in children.

Abstract: The opinions in this paper were originally requested by The Mid-Staffordshire Public Inquiry in the United Kingdom. 1 [1] http://www.midstaffspublicinquiry.com/. This inquiry has highlighted the way in which Boards of Hospitals may fail to use information correctly in several ways; inappropriate collection, poor validation, lack of appropriate analysis and the absence of transparency. Here I outline some personal opinions based on long experience at the coal-face of both medicine and information management. Whilst many of the examples I have chosen reflect life the in the UK National Health Service, they are widely applicable internationally. Data drive change, and I argue that healthcare has become an information economy. Purchasers and users of care can drive the demand for better data, but the underlying infrastructure requires adequate funding. I also argue that outcome measurement alone is not enough, and that quality control offers faster, more effective improvement through the transformation cycle. The technology available to us now gives us fresh and exciting opportunities to engage with our patients and to share information with those who need it. Anyone bringing their precious child to a hospital has a right to know about the quality, safety and outcomes they can expect. We have a duty to give those data to them. [Copyright &y& Elsevier]

Abstract: Disorders of the major airways in children are often difficult to treat. Recent advances in interventional radiology are proving useful, for both assessment of the severity of the problem and treatment. Flexible bronchoscopy and bronchography are essential tools for diagnosis, intervention and follow-up. Echocardiography, computed tomography and magnetic resonance imaging may also be important for the evaluation of cardiovascular anomalies, which are often associated with airway obstruction. Surgery remains the first line of treatment for most congenital abnormalities of the airway and for cardiac anomalies that cause airway compression. Balloon dilatation and stenting are helpful in certain other conditions, as well as in children whose airway problem is not fully corrected by surgery. A multidisciplinary approach is required, with input from pediatric cardiothoracic surgeons, radiologists, radiographers, otolaryngologists, pulmonologists, anesthesiologists, intensivists, physiotherapists and liaison nurses. [Copyright &y& Elsevier]

Reports issues related to medicine. Campaigns for in vitro fertilization; Complications of coronary artery bypass surgery; Treatment for homozygous familial hypercholesterolemia.

Background: This analysis is about practical living bioethics and how law, ethics and sociology understand and respect children's consent to, or refusal of, elective heart surgery. Analysis of underlying theories and influences will contrast legalistic bioethics with living bioethics. In-depth philosophical analysis compares social science traditions of positivism, interpretivism, critical theory and functionalism and applies them to bioethics and childhood, to examine how living bioethics may be encouraged or discouraged. Illustrative examples are drawn from research interviews and observations in two London paediatric cardiac units. This paper is one of a series on how the multidisciplinary cardiac team members all contribute to the complex mosaic of care when preparing and supporting families' informed consent to surgery. Results: The living bioethics of justice, care and respect for children and their consent depends on theories and practices, contexts and relationships. These can all be undermined by unseen influences: the history of adult-centric ethics; developmental psychology theories; legal and financial pressures that require consent to be defined as an adult contract; management systems and daily routines in healthcare that can intimidate families and staff; social inequalities. Mainstream theories in the clinical ethics literature markedly differ from the living bioethics in clinical practices. Conclusion: We aim to contribute to raising standards of respectful paediatric bioethics and to showing the relevance of virtue and feminist ethics, childhood studies and children's rights. [ABSTRACT FROM AUTHOR]

Comments on an article about tracheal occlusion for congenital diaphragmatic hernia treatment in fetus. Processes involved in fetoscopic surgery; Outcome assessment following tracheal occlusion; Factors that need to be considered in fetal surgery.

Discusses the use of non-heart-beating donors for lung transplantation in the United Kingdom. Study published in the March 17, 2001 issue of the periodical 'Lancet;' Benefits of the research, including increased donor supply; Issues raised by the paper, including consensus on the ethics of the procedure; Feeling that units with patients who are potential donors must be prepared to revise their thinking to allow the procedures for organ preservation and retrieval and detailed functional assessment of the organ; Shortcomings of the paper.

Background: Standards generally reported in the literature about informing children and respecting their consent or refusal before elective heart surgery may differ from actual practice. This research aims to summarize the main themes in the literature about paediatric anaesthesia and compare these with research findings on how health professionals counsel young children before elective heart surgery, respect their consent or refusal, and maintain patient-centred care.Methods: This qualitative research involved: literature reviews about children's consent to surgery and major interventions; observations of wards, clinics and medical meetings in two paediatric cardiology departments, October 2019 to February 2020; audio-recorded semi-structured interviews with 45 hospital staff, including 5 anaesthetists, and related experts, November 2019 to April 2021; interviews with 16 families, with children aged 6- to 15-years and their parents shortly after elective heart surgery, and some months later (reported in other papers); thematic data analysis; and research reports on how different professions contribute to children's informed decisions for heart surgery.Results: The medical, ethics and English legal literature tend to assume legal minors cannot refuse major recommended treatment, and cannot consent until they are 12 years or older. Little is said about informing pre-competent children. If children resist, some anaesthetists rely on sedation and distraction, and avoid much informed discussion, aiming to reduce peri-operative anxiety. However, interviewees reported informing young children, and respecting their consent or refusal before elective surgery. They may delay elective surgery and provide further information and support, aiming to reduce fear and promote trust. Six years of age was commonly cited as the threshold for respecting consent to heart transplantation.Conclusion: Differing views about younger children's competence, anxiety and best interests support different reactions to children's consent and refusal before elective heart surgery. This paper reports the zero-restraint policy followed for over a decade in at least one leading surgery centre. The related law and literature need to be updated, to take more account of evidence of actual practice. [ABSTRACT FROM AUTHOR]

Background: Aortopexy is the most effective treatment for severe tracheomalacia associated with esophageal atresia with distal tracheo-esophageal fistula (EA/TOF). In the last few years, the thoracoscopic approach has been proposed, but the number of patients treated is limited. The purpose of this study is to review our initial experience with thoracoscopic aortopexy. Patients and Methods: A retrospective review of medical records was performed on EA/TOF patients undergoing thoracoscopic aortopexy at the Great Ormond Street Hospital for Sick Children (London, United Kingdom) from January 2009 to May 2012. Patient demographics, indication, perioperative course, and long-term results when available were noted. Results: Four patients underwent a successful thoracoscopic aortopexy, with no operation being converted. No morbidity or mortality was associated with the procedure. Length of postoperative stay ranged from 2 to 4 days. All patients were relieved of their symptoms, and no recurrence was noted. Conclusions: Thoracoscopic aortopexy is a feasible and successful treatment for severe tracheomalacia in EA/TOF patients. The complication rate may be lower than after the open procedure and is more satisfactory in terms of cosmetic appearance. However, we need a larger and prospective study with a longer follow-up to confirm these preliminary results. [ABSTRACT FROM AUTHOR]

Care pathways are used increasingly worldwide to organize patient care. However, different views exist about their effectiveness. One of the reasons for this is that pathways are complex interventions. A recent Cochrane review was published which reported positive results, but although the Cochrane team performed excellent work with an enormous commitment, the conclusions may be inappropriate. To fully understand the potential and problems of care pathways, it is important to define (a) exactly what we are talking about (b) whether the study methods are appropriate, and (c) whether we can properly define the outcomes. [ABSTRACT FROM AUTHOR]

To gather detailed data on the incidence of phrenic nerve damage (PND) following cardiac surgery in children, the risk factors for its development, its effect on morbidity and its prognosis. Prospective electrophysiological measurement of phrenic nerve latency in 310 children before and after cardiac surgery. Tertiary paediatric cardiac surgical centre. Our findings were fourfold. Firstly, the incidence of PND in our group of patients was 20%, significantly higher than estimates using indirect methods of assessment. Secondly, PND increased the duration of ventilation by a median of 76 h (20 vs. 96 h; p < 0.001), and late post-operative deaths (before hospital discharge) occurred in 12.9% of patients compared to 2.4% among patients with a normal post-operative phrenic latency. Thirdly, the risk factors that were independently predictive of the development of PND were the site of the surgery and the patient's age. Patients who required surgery at both the lung hilum and the pericardium were more likely to develop PND than patients with only one of those sites, or when neither was involved, and children less than 18 months old were more likely to develop PND than older children. Lastly, the natural history of PND following surgery appears to be good. In our follow-up to 3 months, approximately one third recovered within 1 month and a further third (overall) recovered by 3 months. We conclude that the incidence of PND is much higher than currently recognised, and has a very significant effect on post-operative morbidity and mortality. Most children who survive the post-operative period will recover nerve function within 3 months. [ABSTRACT FROM AUTHOR]

Rationale: Preliminary unpublished studies in our laboratory suggested that the behaviour of Sprague-Dawley (SD) and Dark Agouti (DA) rats was markedly different on both the elevated plus maze and in the open-field apparatus. We wished to confirm and extend this initial finding. Objective: The study was designed to examine the behaviour of SD and DA rats in the elevated plus maze, open-field apparatus and automated activity meters. The response of both strains on the elevated plus maze following diazepam (1 mg/kg and 1.5 mg/kg) administration was subsequently investigated. Results: DA rats showed markedly greater anxiety-like behaviour than SD rats in both the plus maze and open field, with fewer percentage open/total arm entries and percentage time spent on open/total arms in the plus maze and fewer crossings in the open field. Acute handling plus administration of vehicle abolished this difference in anxiety levels, with DA rats showing similar open-arm behaviour to that of SD rats. Both strains demonstrated a clear anxiolytic response to diazepam (1 mg/kg) in terms of percentage time spent on the open arms, but only SD rats had a statistically significant increase in percentage open-arm entries compared with vehicle-injected control animals. Conclusions: While the high level of anxiety-like behaviour of DA rats versus SD rats could prove useful in future ethological studies on anxiety, the fact that acute handling decreased the anxiety-like behaviour on the elevated plus maze may limit the value of this strain for the study of putative anxiolytic drugs. [ABSTRACT FROM AUTHOR]

Rationale: Decreased 5-HT function has been shown to induce behaviour consistent with an "anxiolytic" effect. Administration of a single dose of 3,4-methylenedioxymethamphetamine (MDMA; "ecstasy" 12.5 mg/kg IP) to rats results in prolonged damage to central serotonergic nerve terminals. Thus we wished to assess whether an MDMA-induced lesion may have longer-term behavioural consequences. Objective: The study was designed to examine the behaviour of MDMA-pretreated and control animals in the elevated plus-maze and open field at a number of time-points, up to 80 days, after the administration of a single neurotoxic dose of MDMA (12.5 mg/kg IP). Results: MDMA-pretreated Dark Agouti rats demonstrated a statistically significant reduction in anxiety-related behaviour, compared to saline-pretreated control rats, in both the elevated plus-maze and open field when the rats were tested on day 73 (open field) and day 80 (plus maze) after MDMA administration. Conclusions: The behavioural consequences of a single neurotoxic dose of MDMA can be demonstrated over 2 months after administration of the compound, thereby indicating that long-term adaptive changes occur within the brain following the neurodegeneration of 5-HT neurones produced by this recreationally used drug. [ABSTRACT FROM AUTHOR]

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]

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]

Introduction Slide tracheoplasty is now considered gold standard treatment for long segment congenital tracheal stenosis. Outcomes are typically focused upon airway patency. Dysphagia is often reported in children undergoing cardiothoracic surgery, but not specifically after slide tracheoplasty. This study was carried out to describe the nature and prevalence of dysphagia following slide tracheoplasty for long segment congenital tracheal stenosis. Methods Retrospective case note review was conducted on a series of patients who underwent swallow evaluation following slide tracheoplasty between 2006 and 2014. A clinical swallow assessment was carried out by a Speech and Language Therapist with videofluoroscopic evaluation of swallowing where indicated. Logistic regression assessed the impact of gender, feeding history, weight, tracheal diameter, stenting and co-morbidities on the likelihood of having post-operative dysphagia. Results 43 out of 83 slide tracheoplasty patients underwent swallow evaluation. Dysphagia was identified in 30 (70%) of 43 patients. Videofluoroscopy was undertaken in 22 of these patients. All patients who had a videofluoroscopy presented with altered swallow physiology. Aspiration risk was confirmed in 15 patients with frank aspiration seen in 9. Pre-operative history of dysphagia was present in 9 patients. There were two cases of vocal fold palsy. The presence of a stent was the strongest predictor of post-operative dysphagia with an odds ratio of 10.6 (95% CI 1.2–92.8). Conclusions This study documents a high prevalence of post-operative dysphagia in a pediatric population following slide tracheoplasty. In most cases there was no history suggestive of dysphagia pre-operatively. Swallowing needs to be assessed after slide tracheoplasty and longitudinal studies are required. [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]

This paper aims to update clinicians on "hot topics" in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients. In simple D-TGA: 1) familial recurrence risk is low; 2) children diagnosed pre-natally have improved cognitive skills compared with those diagnosed post-natally; 3) echocardiography helps to identify risk factors; 4) routine use of BAS and prostaglandin E1 may not be indicated in all cases; 5) early ASO improves outcomes and reduces costs with a low mortality; 6) single or intramural coronary arteries remain risk factors; 7) post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; 8) coronary insufficiency and arrhythmias are rare but are associated with sudden death; 9) early- and late-onset ND abnormalities are common; 10) aortic regurgitation and aortic root dilation are well tolerated; and 11) the aging ASO patient may benefit from "exercise-prescription" rather than restriction. Significant strides have been made in understanding risk factors for cardiac, ND, and other important clinical outcomes after ASO. [ABSTRACT FROM AUTHOR]

Abstract: Tissue engineering of autologous lung tissue aims to become a therapeutic alternative to transplantation. Efforts published so far in creating scaffolds have used harsh decellularization techniques that damage the extracellular matrix (ECM), deplete its components and take up to 5 weeks to perform. The aim of this study was to create a lung natural acellular scaffold using a method that will reduce the time of production and better preserve scaffold architecture and ECM components. Decellularization of rat lungs via the intratracheal route removed most of the nuclear material when compared to the other entry points. An intermittent inflation approach that mimics lung respiration yielded an acellular scaffold in a shorter time with an improved preservation of pulmonary micro-architecture. Electron microscopy demonstrated the maintenance of an intact alveolar network, with no evidence of collapse or tearing. Pulsatile dye injection via the vasculature indicated an intact capillary network in the scaffold. Morphometry analysis demonstrated a significant increase in alveolar fractional volume, with alveolar size analysis confirming that alveolar dimensions were maintained. Biomechanical testing of the scaffolds indicated an increase in resistance and elastance when compared to fresh lungs. Staining and quantification for ECM components showed a presence of collagen, elastin, GAG and laminin. The intratracheal intermittent decellularization methodology could be translated to sheep lungs, demonstrating a preservation of ECM components, alveolar and vascular architecture. Decellularization treatment and methodology preserves lung architecture and ECM whilst reducing the production time to 3 h. Cell seeding and in vivo experiments are necessary to proceed towards clinical translation. [Copyright &y& Elsevier]

A letter to the editor and the authors' reply are presented in response to the article "Stem-cell-based, Tissue Engineered Tracheal Replacement in a Child: A 2-year Follow-up Study," by Martin J. Elliott, P. De Coppi, and S. Speggiorin in the September 15, 2012 issue.

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]

Fetal endoscopic tracheal occlusion (FETO) is a promising treatment for severe congenital diaphragmatic hernia, a condition that carries significant morbidity and mortality. It is hypothesised that balloon occlusion of the fetal trachea leads to an improvement in lung growth and development. The major documented complications of FETO to date are related to preterm delivery. To report a series of five infants who developed tracheomegaly following FETO. Review of all children referred with tracheomegaly to the paediatric intensive care and tracheal service at two referral centres. Five neonates presented with features of respiratory distress shortly after birth and were subsequently found to have marked tracheomegaly. Two neonates had tracheomalacia in addition. There are no previous reports in the literature describing tracheomalacia, or more specifically, tracheomegaly, as a consequence of FETO. We propose that the particularly compliant fetal airway is at risk of mechanical damage from in utero balloon occlusion. This observation of a new problem in this cohort suggests a thorough evaluation of the trachea should be performed in children who have had FETO in utero. It may be that balloon occlusion of the trachea earlier in utero (before 26 weeks’ gestation) predisposes to this condition. [ABSTRACT FROM AUTHOR]

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]

Quality-of-care evaluation must take into account variations in “case mix.” This study reviewed the application of two case-mix complexity-adjustment tools in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database: the Aristotle Basic Complexity (ABC) score and the Risk Adjustment in Congenital Heart Surgery (RACHS-1) method. The 2006 STS Congenital Heart Surgery Database Report, the first STS report to incorporate both methods, included 45,635 operations from 47 centers. Each operation was assigned an ABC score in a range from 1.5 (lowest complexity) to 15 (highest complexity), an ABC level in a range from 1 (lowest complexity) to 4 (highest complexity), and a RACHS-1 category in a range from 1 (lowest risk) to 6 (highest risk). The overall discharge mortality was 3.9% (1,222/31,719 eligible cardiac index operations). Of the eligible cardiac index operations, 85.8% (27,202/31,719) were eligible for analysis by the RACHS-1 method, and 94.0% (29,813/31,719) were eligible for analysis by the ABC approach. With both RACHS-1 and ABC, as complexity increases, discharge mortality also increases. The ABC approach allows classification of more operations, whereas the RACHS-1 discriminates better at the higher end of complexity. Complexity stratification is a useful method for analyzing the impact of case mix on pediatric cardiac surgical outcomes. Both the RACHS-1 and ABC methods facilitate complexity stratification in the STS database. [ABSTRACT FROM AUTHOR]

CKD is a major co-morbidity in pediatric lung transplant recipients. We report the prevalence of renal impairment post-lung transplant at a single center, using a modified, age-adjusted eGFR for the best approximation of true GFR, and investigated associations and possible predictors of decline in renal function post-transplant. Renal function was assessed by eGFR pre-transplant, three and 12 months post-transplant, and at last follow-up. Decline in renal function was analyzed as percentage fall in eGFR in two phases (0–3 and 3–12). Furthermore, we investigated impact of gender, age, pre-transplant diagnosis and renal function, transplant type, early post-transplant dialysis, and tacrolimus trough levels on decline in eGFR using multivariate analysis. Over a five-yr period, 30 transplants were performed. Mean eGFR pretransplant was 117 mL/min/1.73 m2 (s.d. 35) with mean decline in eGFR during the first three months post-transplant of 33% (s.d. 31, p < 0.001). Thereafter, mean decline in eGFR was 8% (s.d. 18, p = 0.02). None of the factors assessed were significantly associated with decline in eGFR post-transplant. In conclusion, many children have decline in renal function following lung transplantation, particularly early post-transplant. Unlike in adults, we were unable to detect any predictors of renal impairment in pediatric lung transplant recipients. [ABSTRACT FROM AUTHOR]

Background: We aimed to improve the quality and safety of handover of patients from surgery to intensive care using the analogy of a Formula 1 pit stop and expertise from aviation. Methods: A prospective intervention study measured the change in performance before and after the implementation of a new handover protocol that was developed through detailed discussions with a Formula 1 racing team and aviation training captains. Fifty (23 before and 27 after) postsurgery patient handovers were observed. Technical errors and information omissions were measured using checklists, and teamwork was scored using a Likert scale. Duration of the handover was also measured. Results: The mean number of technical errors was reduced from 5.42 (95% CI ±1.24) to 3.15 (95% CI ±0.71), the mean number of information handover omissions was reduced from 2.09 (95% CI ±1.14) to 1.07 (95% CI ±0.55), and duration of handover was reduced from 10.8 min (95% CI ±1.6) to 9.4 min (95% CI ±1.29). Nine out of twenty-three (39%) precondition patients had more than one error in both technical and information handover prior to the new protocol, compared with three out of twnety-seven (11.5%) with the new handover. Regression analysis showed that the number of technical errors were significantly reduced with the new handover ( t = −3.63, P < 0.001), and an interaction suggested that teamwork ( t = 3.04, P = 0.004) had a different effect with the new handover protocol. Conclusions: The introduction of the new handover protocol lead to improvements in all aspects of the handover. Expertise from other industries can be extrapolated to improve patient safety, and in particular, areas of medicine involving the handover of patients or information. [ABSTRACT FROM AUTHOR]

This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for congenital heart disease. Five areas will be reviewed: (1) common language = nomenclature, (2) mechanism of data collection (database or registry) with an established uniform core data set, (3) mechanism of evaluating case complexity, (4) mechanism to ensure and verify data completeness and accuracy, and (5) collaboration between medical subspecialties. During the 1990s, both the Society of Thoracic Surgeons (STS) and the European Association for Cardiothoracic Surgery (EACTS) created congenital heart surgery outcomes databases. Beginning in 1998, the EACTS and STS collaborated in the work of the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common congenital heart surgery nomenclature, along with a common core minimal data set, were adopted by the EACTS and the STS and published in the Annals of Thoracic Surgery. In 2000, the International Nomenclature Committee for Pediatric and Congenital Heart Disease was established; this committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD). The working component of ISNPCHD is the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group (NWG). By 2005, the NWG cross-mapped the EACTS–STS nomenclature with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology and created the International Paediatric and Congenital Cardiac Code (IPCCC) (). This common nomenclature (IPCCC), and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both EACTS and STS; since 1998, this nomenclature and database have been used by both the STS and EACTS to analyze outcomes of more than 75,000 patients. Two major multi-institutional efforts have attempted to measure case complexity; the Risk Adjustment in Congenital Heart Surgery-1 and the Aristotle Complexity Score. Efforts to unify these two scoring systems are in their early stages but are encouraging. Collaborative efforts involving the EACTS and STS are under way to develop mechanisms to verify data completeness and accuracy. Further collaborative efforts are also ongoing between pediatric and congenital heart surgeons and other subspecialties, including pediatric cardiac anesthesiologists (via the Congenital Cardiac Anesthesia Society), pediatric cardiac intensivists (via the Pediatric Cardiac Intensive Care Society), and pediatric cardiologists (via the Joint Council on Congenital Heart Disease). Clearly, methods of congenital heart disease outcomes analysis continue to evolve, with continued advances in five areas: nomenclature, database, complexity adjustment, data verification, and subspecialty collaboration. [ABSTRACT FROM AUTHOR]

Summary: Objective: To review our strategy for the treatment of patients with Type IV laryngotracheoesophageal cleft—a very rare congenital malformation. Methods: Retrospective review of nine cases of Type IV laryngotracheoesophageal cleft managed between October 1994 and January 2004 at Great Ormond Street Hospital for Children, London. Results: Three children were not operated upon (Group A) because of serious co-morbidity and parental decision not to opt for repair; they died at the ages of 2, 7 and 14 days, respectively. Six cases were repaired (Group B) at ages ranging from 2 to 53 days, all using an anterior cervico-thoracic approach. Two cases were operated upon using conventional ventilation, three using cardiopulmonary bypass and one using extracorporeal membrane oxygenation. There was no intra-operative mortality. The number of operative and diagnostic procedures varied from 4 to 37. Two operated cases expired at the ages of 11 days and 25 months; both were operated upon using cardiopulmonary bypass and had significant cardiac co-morbidity. Post-operative microlaryngoscopy and bronchoscopy showed dehiscence in the cleft repair ranging from 1mm to 2cm in five cases. This was observed at the distal end in three patients and just below the vocal cords in two cases. Three cases underwent further repair which was successful. The most recent case repaired using extracorporeal membrane oxygenation required less heparin when compared with those done on cardiopulmonary bypass and had the best post-operative result. Two patients were finally decannulated. The total duration of diagnostic and operative procedures ranged from 9 to 26h and 30min. The hospital stay in the operated cases varied from 9 days to 2 years 2 months 3 days, and their ICU stay ranged from 9 days to 10 months 7 days. All four of our patients with clefts which ended above the carina are alive after multiple repairs whereas all five cases with clefts extending all the way to the carina died (repair was attempted in two). Conclusion: Early diagnosis and repair are essential for successful treatment of Type IV laryngotracheoesophageal clefts. We believe it is best to repair this defect via an anterior cervico-thoracic approach, with or without a median sternotomy, and extracorporeal membrane oxygenation is now our preferred method of gas exchange during such repair. The longest Type IV clefts extending all the way to the carina have the worst prognosis. The decision to operate or not should be based upon the associated co-morbidity and fully informed parental choice, since treatment entails significant morbidity and mortality. [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]

Background: Elevation in Epstein–Barr virus (EBV) load measured in peripheral blood has been proposed as a marker for development of post-transplant lymphoproliferative disease (PTLD), but there are few published data examining this relationship. We report the longitudinal surveillance of EBV for all recipients of heart (HTx), heart–lung (HLTx) and lung (LTx) transplants at our institution. Methods: The study population included all patients transplanted between January 2003 and July 2004. EBV load was serially measured in peripheral blood by real-time polymerase chain reaction (PCR). Results were correlated with recipient pre-transplant EBV status and development of PTLD. Results: Forty-four transplant operations were performed, including 33 HTx, 6 HLTx and 5 LTx. Thirty-two (73%) of the patients were EBV seropositive pre-transplant. Nineteen (44%) pediatric recipients developed EB viremia, including 17 HTx, 1 HLTx and 1 LTx. Eleven (58%) of these patients were EBV seropositive pre-transplant. EBV was first detected at a median of 30.5 days (range 2 to 81) post-transplant. The median peak EBV load in that group was 10,099 copies/ml (range 5,935 to 255,466) whole blood. One patient with cystic fibrosis post-LTx developed PTLD localized in the colon. This patient was EBV seronegative pre-transplant; peak EBV load was 14,513 copies/ml. Acute infectious mononucleosis was seen in 1 case. Positive pre-transplant EBV status did not predict post-transplant EB viremia (positive predictive value 0.03). Conclusions: Contrary to earlier reports, our data demonstrate that a high EBV load does not lead to PTLD early post-transplant. These results do not support the practice of pre-emptively reducing immunosuppression in patients with raised EBV load. [Copyright &y& Elsevier]

Background: More children are coming to heart transplantation on extracorporeal membrane oxygenation (ECMO), or inotropic support and/or with renal impairment. The use of basiliximab, a chimeric monoclonal antibody against CD25 (interleukin 2 receptor alfa) has not been previously reported in critically ill pediatric heart transplant recipients. Basiliximab has potential advantages in the treatment of patients with renal impairment. Methods: Basiliximab was provided to 29 patients (median age 7.8 years; range 0.4–16 years) on ECMO, with renal impairment or receiving intravenous inotropes at transplantation. Children normally received 2 doses on Day 0 and Day 4 after transplantation. Calcineurin inhibitor was provided in low dose or withheld altogether in patients with renal impairment. Flow cytometry was used to monitor CD25. Results: At transplantation, 11 patients were prescribed cyclosporine; the remaining 18 received tacrolimus. All but 4 patients had subtherapeutic levels of calcineurin inhibitor in the first postoperative week. Excluding these 4, there were 19 patients who had more than 4 consecutive doses of calcineurin inhibitor canceled in the first week (median 8 doses; range 3–40 doses). A total of 71 surveillance biopsies were performed, and 4 episodes of severe acute rejection occurred in the first 6 months. In all but one child, the glomerular filtration rate had returned to, or improved on baseline measurement by 1 month after transplantation. Infections rates were low and acceptable. CD25 was undetectable at first assessment, and in all but 1 patient (on ECMO) for at least 2 to 3 weeks thereafter. There were no adverse effects. Conclusions: Basiliximab was well tolerated in this group of very ill children. In children with pre- or postoperative renal dysfunction, where doses of calcineurin inhibitor were low or canceled, basiliximab was associated with a low incidence of rejection. Posttransplant ECMO may reduce the efficacy of basiliximab. These preliminary results are encouraging and now need confirmation in a large, randomized trial. [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]

Pulmonary artery slings are rare congenital anomalies seen only on the left side. The presence of right pulmonary artery (RPA) sling is extremely rare. We report a case of a RPA sling with a single lung associated with tracheal stenosis and its management. doi: 10.1111/jocs.12240 (J Card Surg 2014;29:256-258) [ABSTRACT FROM AUTHOR]

Airway compression is a common problem in children with certain forms of congenital heart disease. Although various surgical approaches are available to overcome this form of airway obstruction, internal stenting is necessary in a minority of patients. It can be difficult to assess the success of stenting at the time of the procedure, and the interval to successful extubation is usually used as an outcome measure. Measurement of relevant parameters of respiratory physiology with flow-volume and volume-pressure loops permits immediate quantitative assessment of the adequacy of stenting. A 3-month-old infant who underwent bronchial stenting and physiological assessment at the time of the procedure is described. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 11 year old boy who was suffering from asymptomatic atrial septal defect (ASD) and was admitted to a hospital for interventional catheter device closure of the defect. He was also ailing from secundum atrial septal defect. He was diagnosed with a small defect in the oval fossa with an inferior small venosus defect. His medial papillary muscle got damaged due to the multiple percutaneous attempts at retrieval of ASD.

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]