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Postpneumopnectomy syndrome (PPS) is an extreme rotation and malposition of mediastinum causing dynamic and symptomatic central airway compression, arisingafter pneumonectomy or more uncommonly, in congenital single lung physiology. Affected patients present with severe respiratory compromise. Intrathoracic prosthesis placement is an evolving technique in children that mitigate the effects of thoracic dead space. Assessment of clinical recovery and functional benefit in children undergoing placement of intrathoracic prosthesis following pneumonectomy or in congenital single lung situations. Retrospective chart review of patients at Great Ormond Street Hospital from 2010 to 2020 was performed of all patients who underwent intrathoracic tissue expander placement. We summarize the outcomes of twenty four children, including those with both congenital and postpneumonectomy PPS etiology. 24 Children who underwent placement of intrathoracic prosthesis for PPS in the study period with median age of 3.5 months and weight of 5 kg. Single lung etiology was congenital in 15 children (6 agenesis, 9 hypoplasia), and postpneumonectomy in 9 children. In seven patients, there was associated long segment tracheal stenosis. Pre operative ECMO was required in 2 patients, and pre operative ventilation was required in 12 patients all of whom had congenital single lung. Intrathoracic prosthesis placement was concurrent with intracardiac repair in 5 patients. There were no operative deaths, but one early postoperative death related to septicaemia. Median follow up was 75 months with 10 patients on continued respiratory support and 3 on nocturnal support with good quality of life. Two children needed reoperations for replacement of prosthesis. The use of tissue expanders is within the armamentarium of most plastic surgeons' practice. We also therefore advocate for a collaborative team approach involving Plastic and Cardiothoracic Surgery for surgical treatment of these patients. This multidisciplinary strategy has improved management of this rare and debilitating condition of PPS, thereby offering significant improvements in general progress of these sick children having single lung physiology. Evidence is still lacking on functional outcomes in these children and further work is necessary to prove that this is indeed achievable. [ABSTRACT FROM AUTHOR]

Congenital tracheobronchial stenosis is a rare disease characterized by complete tracheal rings that can affect variable lengths of the tracheobronchial tree. It causes high levels of morbidity and mortality both due to the stenosis itself and to the high incidence of other associated congenital malformations. Successful management of this complex condition requires a highly individualized approach delivered by an experienced multidisciplinary team, which is best delivered within centralized units with the necessary diverse expertise. In such settings, surgical correction by slide tracheoplasty has become increasingly successful over the past 2 decades such that long-term survival now exceeds 88%, with normalization of quality of life scores for patients with non-syndrome-associated congenital tracheal stenosis. Careful assessment and planning of treatment strategies is of paramount importance for both successful management and the provision of patients and carers with accurate and realistic treatment counseling. [ABSTRACT FROM AUTHOR]

Objective: Our study describes the results from surgical slide tracheoplasty (STP) in children with long segment tracheal stenosis. Methods: Demographic and preoperative conditions, operative details, and outcome measures, including the need for endoscopic airway intervention and mortality, were collected for children undergoing STP between February 1995 and December 2012. Results: One hundred one patients (median age, 5.8 months; range, 5 days-15 years) underwent STP. Seventy-two patients (71.3%) had associated cardiovascular anomalies. Preoperative ventilation was necessary in 56 patients (55.4%), whereas extracorporeal membrane oxygenation was required in 10 patients (9.9%). Abnormal bronchial arborization was present in 39 children (38.6%), which included 13 patients (12.8%) with an anomalous right upper lobe bronchus and 17 patients (16.8%) with tracheal trifurcation. Airway stenosis extended into 1 or both bronchi in 24 patients (23.7%) and preoperative malacia was present in 24 patients (23.7%). STP was extended into the bronchus in 47 patients (46.5%). Overall survival was 88.2% (mortality in 12 patients). Post-STP balloon dilation was necessary in 45 patients (44.6%) and stenting was required in 22 patients (21.8%). Multivariate analysis revealed preoperative extracorporeal membrane oxygenation (P < .05), preoperative malacia (P < .001), and bronchial stenosis (P < .05) to be adverse predictors of survival. Preoperative malacia was a significant risk factor for stenting (P < .05). Conclusions: STP is a versatile and reliable technique associated with low morbidity and mortality when compared with previous strategies for children with long segment tracheal stenosis. The presence of preoperative bronchomalacia is a significant risk factor for death and postoperative stenting. [Copyright &y& Elsevier]

Background: Recent reports suggest worse outcomes in pediatric orthotopic heart transplantation (OHT) for congenital heart disease (CHD) and restrictive cardiomyopathy (RCM). We examined early outcomes in these diverse groups of patients in comparison with patients with dilatated cardiomyopathy (DCM). Methods: From 2000 to 2011, 209 patients were included: 50 with CHD, 23 with RCM, and 136 with DCM. Early survival was studied, as was the occurrence of acute rejection, donor-specific antibodies (DSAs) and nondonor-specific antibodies (NSDAs), incidence of pulmonary hypertension (PHT), right ventricular failure (RVF), and the need for mechanical circulatory support (MCS). Results: The incidence of preoperative PHT was greatest in the RCM group (χ2 p = 0.0006); the requirement for mechanical support before OHT was greatest in patients with DCM. Thirty-day survival was 92.0%, 97.1%, and 100% for patients with CHD, DCM, and RCM respectively. The incidence of RVF was highest for patients with RCM (43.5%; versus CHD, 26.0%; versus DCM, 14.7%). One-year survival estimates for patients with CHD, DCM, and RCM were 92.0%, 97.8%, and 82.6%, respectively (log-rank p = 0.165). Multivariable analysis revealed 4 significant risk factors for mortality: age, incidence of acute rejection, preoperative PHT, and the presence of NDSAs. The occurrence of DSAs was similar, although there was a significantly higher incidence of NDSAs in the CHD and RCM groups (36.0% and 30.4%, respectively, versus 14.0% in the DCM group; χ2 p = 0.0024). Conclusions: Equivalent outcomes are achievable in pediatric OHT despite marked heterogeneity in anatomic and physiologic complexity in recipients. Physiologic factors such as PHT are likely to be more important than anatomic complexities in determining survival. The potential relevance of NDSAs warrants further investigation. [ABSTRACT FROM AUTHOR]

Abstract: Tracheomalacia associated with esophageal atresia (EA) is a well-known condition. However, complete absence of tracheal rings (TRs) is extremely rare. Our aim is to describe a novel triad of conditions and to discuss the best treatment. An expremature male operated for EA presented with severe respiratory distress. The diagnosis of absent cartilage rings, suspected on bronchoscopy, was confirmed by optical coherence tomography. The absence of TRs was localized to a short tracheal segment, and the carina trifurcated into right upper lobe, right intermediate, and left main bronchus. The patient was treated with resection and anastomosis with a completely satisfactory course. Absence of TRs was previously reported by us in 2 other cases, both with associated EA and trifurcation of the carina. One child was treated with tracheostomy and the other with a stent, but the outcome was far from optimal. The patient with tracheostomy eventually underwent resection and anastomosis with tracheostomy closure. Congenital absence of TRs is extremely rare. Although localized, it is responsible for severe symptoms owing to complete tracheal collapse and may be misdiagnosed as tracheomalacia. In our experience, it has been associated with EA and trifurcated carina. Our limited experience suggests resection of the abnormal segment and tracheal anastomosis as the best treatment. [Copyright &y& Elsevier]

Background: Slide tracheoplasty has become the surgical technique of choice for repair of congenital tracheal stenosis. Despite the initial reluctance regarding the ability of this “reconstructed” trachea to grow, the reduced morbidity and mortality have allowed slide tracheoplasty to be widely adopted. The aim of this study was to evaluate tracheal growth after slide tracheoplasty. Methods: This was a retrospective study. In follow-up bronchography performed 1, 6, 12, 18, and 24 months after slide tracheoplasty, we measured the cross-sectional areas of the midtrachea and distal trachea at each investigation and correlated the measurements with the anthropomorphic factors (body weight, height, and body surface). Results: Fourteen patients were enrolled in this study. The midtracheal and distal tracheal cross-sectional areas significantly increased with time (p ≤ 0.0001). The average rates of midtracheal growth were 21.0 mm2year in the first 6 months and 8.0 mm2/year in the first 2 years, and the distal trachea grew 18.5 mm2/year and 8.4 mm2/year, respectively. Regression analysis showed that both the midtrachea and the distal trachea increase significantly with weight (r 2 = 0.257, p ≤ 0.0001), height (r 2 = 0.376, p ≤ 0.0001), and body surface area (r 2 = 0.315, p ≤ 0.0001). Balloon dilation did not significantly alter the tracheal growth in the first 2 years after slide tracheoplasty. Conclusions: Slide tracheoplasty does not inhibit tracheal growth. The reconstructed trachea grows faster in the first 6 months and slows in the following 18 months. There is a positive correlation between tracheal cross-sectional area and weight, height, and body surface area. [ABSTRACT FROM AUTHOR]

Background: Congenital tracheal stenoses are rare and life-threatening anomalies, associated with considerable variation in both morphology and prognosis. They have been classified previously according to the length of the stenosis or the severity of the symptoms, but not according to bronchial involvement. Methods: Data from patients who underwent slide tracheoplasty for long-segment (>50%) congenital tracheal stenosis were collected. We identified four different types of bronchial arborization (normal, n = 52; tracheal right upper lobe bronchus, n = 10; carina with “trifurcation,” n = 14; and unilateral bronchial and lung agenesis, n = 8). Each type included congenital tracheal (above the carina) or tracheobronchial (extending below the carina) stenosis. Results: Eighty-four children were enrolled in the study. Preoperative ventilation was necessary in 44 patients (52.4%; 75% in patients with a single lung), and preoperative extracorporeal membrane oxygenation was needed in 10 patients (11.9%). Preoperative tracheostomy was present in 3 patients initially treated elsewhere (3.5%), and a left pulmonary artery sling was performed in 44% (37 of 84). The overall mortality was 13% (11 of 84), 7.9% in patients with tracheal stenosis and 28.6% with tracheobronchial stenosis. No deaths occurred in patients with right upper lobe bronchus anatomy. Endoscopic procedures after slide tracheoplasty were required in 34 patients (40.4%). Stents were placed in 18 patients (21.4%), with a higher incidence in those with bronchial trifurcation (42.8%, 6 of 12). Conclusions: This classification appears useful for the morphologic characterization of congenital airway stenosis and could be the benchmark for future prospective studies on the outcome of these patients. [ABSTRACT FROM AUTHOR]

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]

Purpose: We here report our experience with biodegradable polydioxanone stents for tracheal narrowing in children. Description: Eleven custom-made polydioxanone stents were implanted in 4 patients with airway narrowing due to external compression or intrinsic collapse. The median stent diameter was 9 mm (range, 6 to 14 mm) and median length was 15 mm (range, 13 to 70 mm). Evaluation: Narrowing was relieved initially in all cases. There was no bleeding or perforation after polydioxanone stent implantation. Size mismatching was a problem in 2 cases. Three patients needed repeat stenting after stent absorption. There was 1 death, unrelated to the stent implantation. All 3 survivors are in good clinical condition up to 12 months after first stenting. Conclusions: This pilot study shows that polydioxanone stents offer an alternative to metallic or silastic stents for collapse or external compression of the trachea in children. They may avoid the need for permanent stenting and allow subsequent growth of the airway. [ABSTRACT FROM AUTHOR]

The question posed in the title of this article is: “Congenital Heart Surgery Databases Around the World: Do We Need a Global Database?” The answer to this question is “Yes and No”! Yes - we need to create a global database to track the outcomes of patients with pediatric and congenital heart disease. No–we do not need to create a new “global database.” Instead, we need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This review article will achieve the following objectives: (A) Consider the current state of analysis of outcomes of treatments for patients with congenitally malformed hearts. (B) Present some principles that might make it possible to achieve life-long longitudinal monitoring and follow-up. (C) Describe the rationale for the creation of a Global Federated Multispecialty Congenital Heart Disease Database. (D) Propose a methodology for the creation of a Global Federated Multispecialty Congenital Heart Disease Database that is based on linking together currently existing databases without creating a new database. To perform meaningful multi-institutional analyses, any database must incorporate the following six essential elements: (1) Use of a common language and nomenclature. (2) Use of a database with an established uniform core dataset for collection of information. (3) Incorporation of a mechanism to evaluate the complexity of cases. (4) Implementation of a mechanism to assure and verify the completeness and accuracy of the data collected. (5) Collaboration between medical and surgical subspecialties. (6) Standardization of protocols for life-long longitudinal follow-up. Analysis of outcomes must move beyond recording 30-day or hospital mortality, and encompass longer-term follow-up, including cardiac and non-cardiac morbidities, and importantly, those morbidities impacting health-related quality of life. Methodologies must be implemented in our databases to allow uniform, protocol-driven, and meaningful long-term follow-up. We need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This “Global Federated Multispecialty Congenital Heart Disease Database” will not be a new database, but will be a platform that effortlessly links multiple databases and maintains the integrity of these extant databases. Description of outcomes requires true multi-disciplinary involvement, and should include surgeons, cardiologists, anesthesiologists, intensivists, perfusionists, neurologists, educators, primary care physicians, nurses, and physical therapists. Outcomes should determine primary therapy, and as such must be monitored life-long. The relatively small numbers of patients with congenitally malformed hearts requires multi-institutional cooperation to accomplish these goals. The creation of a Global Federated Multispecialty Congenital Heart Disease Database that links extant databases from pediatric cardiology, pediatric cardiac surgery, pediatric cardiac anesthesia, and pediatric critical care will create a platform for improving patient care, research, and teaching related to patients with congenital and pediatric cardiac disease. [Copyright &y& Elsevier]

Abstract: Persistent respiratory symptoms often occur after double aortic arch (DAA) repair but rarely require a second operation. We report 4 children with severe respiratory problems (failure to extubate, 2; severe respiratory distress, 2) caused by severe tracheomalacia and tracheal compression after DAA repair, treated by anterior aortopexy. Aortopexy proved effective and safe in improving symptoms and provides a simple treatment option for children with severe malacia or tracheal compression after DAA repair. [Copyright &y& Elsevier]

Abstract: Long segment congenital tracheal stenosis (LSCTS), associated with complete tracheal rings, is a rare condition, difficult to manage and historically associated with high mortality rate. We report two pairs of identical twins all affected by LSCTS successfully treated by sliding tracheoplasty. All had severe respiratory distress. Three infants had left pulmonary artery (LPA) sling and one intra-cardiac malformation. Slide tracheoplasty was done under cardiopulmonary bypass, and cardiovascular malformations were corrected at the same time. One child needed plication of paralyzed right hemi-diaphragm and another distal tracheal Palmaz stent insertion due severe tracheobronchomalacia. All children are doing well during 6 months follow-up. Slide tracheoplasty seems to produce the same good early results in twins as for singletons with LSCTS. [Copyright &y& Elsevier]

Summary: Congenital heart disease (CHD) is an important clinical problem. Although survival has improved over recent decades, certain children with CHD remain difficult to treat, usually because of severe co-morbidity or uncorrectable defects. Vascular compression of the airway is one such co-morbidity, occurring in approximately 1–2% of children with CHD. It may be caused by congenital anomalies of the configuration of the great vessels, enlargement of otherwise normal structures or as a result of surgery. The anatomical patterns seen in these children may be complex, and as surgical correction is usually required to relieve the compression, the pre-operative imaging assessment should be as complete as possible. Precise diagnosis and therapy are essential because chronic airway compression in childhood carries a significant morbidity and mortality. Airway stenting is currently reserved for rare occasions when surgical correction is not possible. [Copyright &y& Elsevier]

Background: Surgery for congenital heart disease (CHD) has changed considerably during the last three decades. The results of primary repair have steadily improved, to allow treating almost all patients within the pediatric age; nonetheless an increasing population of adult patients requires surgical treatment. The objective of this study is to present the early surgical results of patients who require surgery for CHD in the adult population within a multicentered European study population. Methods: Data relative to the hospital course of 2,012 adult patients (age ≥18 years) who required surgical treatment for CHD from January 1, 1997 through December 31, 2004 were reviewed. Nineteen cardiothoracic centers from 13 European countries contributed to the data collection. Results: Mean age at surgery was 34.4 ± 14.53 years. Most of the operations were corrective procedures (1,509 patients, 75%), followed by reoperations (464 patients, 23.1%) and palliative procedures (39 patients, 1.9%). Six hundred forty-nine patients (32.2%) required surgical closure of an isolated ostium secundum atrial septal defect. Overall hospital mortality was 2%. Preoperative cyanosis, arrhythmias, and NYHA class III-IV, proved significant risk factors for hospital mortality. Follow-up data were available in 1,342 of 1,972 patients (68%) who were discharged home. Late deaths occurred in 6 patients (0.5%). Overall survival probability was 97% at 60 months, which is higher for corrective procedures (98.2%) if compared with reoperations (94.1%) and palliations (86.1%). Conclusions: Surgical treatment of CHD in adult patients, in specialized cardiac units, proved quite safe, beneficial, and low-risk. [Copyright &y& Elsevier]

Background: A serious complication after surgical closure of ventricular septal defect (VSD) is complete heart block. In this retrospective study, we reviewed the incidence of complete heart block after surgical closure of a VSD at Great Ormond Street Hospital from 1976 to 2001 to identify any particular anatomic features that still predisposed patients to surgically-induced complete heart block and to provide anatomic guidelines to avoid this in future. Methods: Data were extracted from our local database for patients having (1) isolated VSD or VSD in the setting of (2) tetralogy of Fallot with pulmonary stenosis or (3) tetralogy of Fallot with pulmonary atresia; (4) absent pulmonary valve syndrome; (5 and 6) coarctation or interruption of the aortic arch; and (7) subaortic fibrous shelf. We carefully reviewed the operative notes from all patients with postoperative complete heart block to discover any predisposing anatomical reasons to explain the complication. Results: Two thousand seventy-nine patients had a VSD closure. Permanent complete heart block developed in 7 of 996 patients (0.7%) with an isolated defect and in 1 of 847 patients (0.1%) with tetralogy of Fallot. Four more patients had postoperative complete heart block. Conclusions: Instances of iatrogenic complete heart block continue to occur after surgical VSD closure, either because of unexpected biological variations or because of unawareness of the disposition of the atrioventricular conduction axis in particular circumstances. This report emphasizes the latter aspect in details and suggests a risk of iatrogenic complete heart block of less than 1%. [Copyright &y& Elsevier]

The most concrete and universal outcome measure used in databases, whether governmental, professional society, research, or third-party payer, is operative mortality. To assure congruous data entry by multiple users of The Society of Thoracic Surgeons and the European Association for Cardiothoracic Surgery congenital heart surgery databases, operative mortality must be clearly defined. Traditionally, operative mortality has been defined as any death, regardless of cause, occurring (1) within 30 days after surgery in or out of the hospital, and (2) after 30 days during the same hospitalization subsequent to the operation. Differing hospital practices result in problems in use of the latter part of the definition (eg, the pediatric hospital that provides longer-term care will have higher mortality rates than one which transfers patients to another institution for such care). In addition, because of the significant number of pediatric multiple operation hospitalizations, issues of assignment of mortality to a specific operation within the hospitalization, calculation of operative mortality rates (operation based vs patient admission based), and discharge other than to home must be addressed and defined. We propose refinements to the definition of operative mortality which specifically meet the needs of our professional societies’ multi-institutional registry databases, and at the same time are relevant and appropriate with respect to the goals and purposes of administrative databases, government agencies, and the general public. [Copyright &y& Elsevier]

Background: After utilizing separate congenital databases in the early 1990s, the Society of Thoracic Surgeons (STS) and the European Association for Cardio-Thoracic Surgery (EACTS) collaborated on several joint database initiatives. Methods: In 1998, the joint EACTS-STS International Congenital Heart Surgery Nomenclature and Database Project Committee was created and a common nomenclature and common core minimum database dataset were adopted and published by the STS and the EACTS. In 1999, the joint EACTS-STS Aristotle Committee was created and the Aristotle Score was adopted and published as a method to provide complexity adjustment for congenital heart surgery. Collaborative efforts involving the EACTS and STS are underway to develop mechanisms to verify data completeness and accuracy. Results: Since 1998, this nomenclature, database, and methodology of complexity adjustment have been used by both the STS and EACTS to analyze outcomes of over 40,000 patients. A huge amount of data have been generated which allow comparison of practice patterns and outcomes analysis between Europe and North America. The aggregate data from the first 5 years of data collection not only make for interesting comparison but also allow examination of regional difference in practice patterns. For example, in the EACTS, out of 4,273 neonates, 885 (20.7%) underwent arterial switch procedures and 297 (6.95%) underwent Norwood stage 1 procedures. In the STS, out of 3,988 neonates, 472 (11.8%) underwent arterial switch procedures and 575 (14.4%) underwent Norwood stage 1 procedures. Conclusions: This analysis of the EACTS-STS multi-institutional outcomes database confirms that in both Europe and North America, case complexity and mortality is highest among neonates, then infants, and then children. Regional differences in practice patterns are demonstrated, with the overall goal being the continued upgrade in the quality of surgery for congenital heart disease worldwide. [Copyright &y& Elsevier]

Background: The analysis of the second harvest (1998–2001) of the Society of Thoracic Surgeons Congenital Heart Surgery Database included the first attempt by the STS to apply a complexity-adjustment method to evaluate congenital heart surgery results. Methods: This data harvest represents the first STS multiinstitutional experience with software utilizing the international nomenclature and database specifications adopted by the STS and the European Association for Cardio-Thoracic Surgery (April 2000 Annals of Thoracic Surgery) and the first STS Congenital Database Report incorporating a methodology facilitating complexity adjustment. This methodology, allowing for complexity adjustment, gives each operation a basic complexity score (1.5 to 15) and level (1 to 4) based upon the work of the EACTS-STS Aristotle Committee, a panel of 50 expert surgeons. The complexity scoring, based on the primary procedure (from the EACTS-STS International Nomenclature Procedures Short List), estimates complexity through three factors: mortality potential, morbidity potential, and technical difficulty. Results: This STS harvest includes data from 16 centers reporting 12,787 cases, with discharge mortality known for 10,246 cases. The basic complexity score has been applied to the outcomes analysis of these cases and a new equation has been proposed to evaluate one aspect of performance: Aristotle Performance Index = Outcome × Complexity = (Survival) × (Mean Complexity Score) Conclusions: The complexity analysis represents a basic complexity-adjustment method to evaluate surgical results. Complexity is a constant precise value for a given patient at a given point in time; performance varies between centers. Future STS congenital data harvests will incorporate a second step, the Comprehensive Aristotle Score, utilizing additional patient specific complexity modifiers to allow a more precise complexity adjustment. [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]

Purpose: We describe the methods we have used to employ digital video techniques in the teaching of surgery for congenital heart disease.Description: This paper describes the hardware and software required to initiate and utilize digital video in surgery for congenital heart defects. Images are collected through a headlight camera and stored on standard digital-video camera tape. Tapes are catalogued using a purpose built database, linked to the hospital data system, and employing the European Pediatric Codes coding system for diagnostic and procedural coding. This permits keyword as well as patient-based searches. Master tapes are then digitally edited using proprietary software to create for example teaching tapes or to explain the operation to the family of the patient.Evaluation: Eighty percent of the procedures performed by one surgeon (M.J.E.) during the last year have been recorded in this way.Conclusions: A working digital archive of pediatric cardiac surgical procedures has been created. Database links to hospital systems create the opportunity for linking full, visual records of procedures to be retained in an electronic patient record. [Copyright &y& Elsevier]

Background: The balance between systemic oxygen consumption (&Vdot;O2) and delivery (DO2) is impaired after cardiopulmonary bypass (CPB) and is related to systemic inflammatory response syndrome. We sought to assess &Vdot;O2 and DO2 and their relationship with proinflammatory cytokines after CPB with the use of modified ultrafiltration (MUF) in infants.Methods: Sixteen infants, aged 1–11.5 months (median, 6.3 months), undergoing hypothermic CPB with MUF were studied during the first 12 hours after arrival in the intensive care unit (ICU). The central temperature was maintained at 36.8–37.1°C using external cooling or warming. &Vdot;O2 was continuously measured using respiratory mass spectrometry. Arterial blood samples for the tumor necrosis factor (TNF), interleukin-6 (IL-6), and interleukin-8 (IL-8) were taken and DO2 was calculated using the Fick principle on arrival at the ICU, and 2, 4, 8, and 12 hours postoperatively. Cytokines were additionally measured after induction of anesthesia and at the end of MUF.Results: &Vdot;O2 significantly decreased by 18.8% during the study period. DO2 was depressed throughout this period and reached a nadir at 8 hours (357.1 ± 136.2 ml · min-1 · m-2). The decrease in cytokines was accompanied with the decrease in &Vdot;O2 despite varied relationships between the levels of each of the cytokines and &Vdot;O2 measurements.Conclusions: Our data indicate an unusual continuous decrease in &Vdot;O2 during the first 12 hours after CPB in infants. Control of body temperature to maintain euthermia in addition to the use of MUF may be beneficial to the balance between &Vdot;O2 and DO2 in the early postoperative period. [Copyright &y& Elsevier]

We describe an unusual case of tracheo-aortic fistula, which occurred after tracheal surgery and tracheal stenting. The management of this complex case and the surgical technique used for repair are discussed and illustrated. Repair of the aortic arch was accomplished using a modified technique of regional low-flow perfusion, similar to that described for neonatal aortic arch reconstruction. This strategy allowed maintenance of cerebral, myocardial, and systemic perfusion during arch repair, thus avoiding total circulatory arrest. [Copyright &y& Elsevier]

Hunter syndrome is a multisystem genetic disease in which a significant proportion of morbidity and mortality arise from respiratory dysfunction. Notably, tracheal abnormalities in Hunter syndrome can compromise clinical stability, and management is primarily supportive. We describe here the first successful implementation of aorto-tracheopexy in a 19-year-old patient as a surgical strategy to resolve progressive respiratory deterioration. [ABSTRACT FROM AUTHOR]

Uhl''s anomaly is a rare condition and surgical techniques are few and have had variable success. We present a novel and successful surgical technique to treat this difficult condition. [Copyright &y& Elsevier]

Long-segment tracheal stenosis with complete tracheal rings is a severe and life-threatening problem in small children. Slide tracheoplasty has largely become the treatment of choice for these patients []. Its use after the failure of other techniques has not been previously reported. We describe a 3-year-old child who underwent a rescue slide tracheoplasty for re-stenosis after initial patch tracheoplasty for long-segment tracheal stenosis and pulmonary artery sling. [Copyright &y& Elsevier]

Slide tracheoplasty has become the preferred technique for repair of long-segment congenital tracheal stenosis with complete tracheal rings. Complex morphological subtypes require technical modifications, which we present as follows. [Copyright &y& Elsevier]

Quadricuspid semilunar valves are very rare, especially in association with other congenital cardiac anomalies. A quadricuspid pulmonary valve has never been described in the setting of transposition of the great arteries. In this brief article we describe one such case, and we discuss the operative strategy during the arterial switch operation with particular reference to the technique of coronary translocation. [Copyright &y& Elsevier]

The first report of The Society of Thoracic Surgeons (STS) National Congenital Heart Surgery Database in 1998 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 Nomenclature and Database Project. The goal was to begin the standardization of nomenclature 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 minimum dataset as well as the diagnoses and procedure short lists. Plans for the next STS National Congenital Heart Surgery Database harvest are also presented. [Copyright &y& Elsevier]

Intraoperative imaging has immense value for teaching and training surgeons by providing part of a future digital medical record of the patient and supportive evidence of good practice and an open attitude to patient safety. We report a case in which intraoperative video recording of an operation allowed us, after an incorrect count, to confidently assert that there was no equipment left behind in the patient. [Copyright &y& Elsevier]

Aortopexy is the treatment of choice for clinically significant tracheobronchomalacia from external vascular compression. When a marked chest depression is present, aortopexy may be less effective. We report 2 patients with pectus excavatum and vascular compression of the trachea who, despite their young age, benefited from combined Nuss bar insertion and aortopexy. [Copyright &y& Elsevier]

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]

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]

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]