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To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2009.08.020 Byline: Aparna Hoskote (a), Catherine Carter (b), Phillip Rees (b), Martin Elliott (c), Michael Burch (b), Katherine Brown (a) Abbreviations: BSA, body surface area; CHD, congenital heart disease; DCM, dilated cardiomyopathy; ECMO, extracorporeal membrane oxygenation; iNO, inhaled nitric oxide; PVR, pulmonary vascular resistance; PVRI, pulmonary vascular resistance index; RCM, restrictive cardiomyopathy; RVF, right ventricular failure; TPG, transpulmonary gradient Abstract: To identify pretransplant factors associated with postprocedural right ventricular failure and the relationship between right ventricular failure and long-term survival in children. Author Affiliation: (a) Cardiac Critical Care Unit, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom (b) Cardiothoracic Transplant Medicine, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom (c) Cardiothoracic Surgery, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom Article History: Received 23 December 2008; Revised 3 July 2009; Accepted 10 August 2009

The successful use of a geographic information system was demonstrated during a hostage rescue exercise at a school. The Upland, California, Police Department took part in the exercise which took place at Upland High School.

Byline: Michael J. Davies, Khan Nguyen, J.William Gaynor, Martin J. Elliott Abstract: Objective: Our objective was to test the hypothesis that use of modified ultrafiltration after cardiopulmonary bypass improves intrinsic left ventricular systolic function in children. Methods: Twenty-one infants undergoing cardiopulmonary bypass were instrumented with ultrasonic dimension transducers, to measure the anteroposterior minor axis diameter, and a left ventricular micromanometer. Patients were randomized to modified ultrafiltration (n = 11, age 226 [+ or -] 355 days, weight 6.7 [+ or -] 3.1 kg) or control (n = 10, age 300 [+ or -] 240 days, weight 7.0 [+ or -] 2.5 kg) (all differences p > 0.05 between groups). Left ventricular systolic function was assessed by means of the slope of the preload-recruitable stroke work index. Myocardial cross-sectional area was measured by echocardiography. Data were acquired immediately after separation from bypass, at steady state, and during transient vena caval occlusion. Data acquisition was repeated after 13 [+ or -] 5 minutes of modified ultrafiltration or after 12 [+ or -] 5 minutes without modified ultrafiltration in the control group. Inotropic drug support was the same at both study points. Results: In the modified ultrafiltration group, the filtrate volume was 363 [+ or -] 262 ml. The hematocrit value increased from 26.0% [+ or -] 2.7% to 36.7% [+ or -] 9.5% (p = 0.018), myocardial cross-sectional area decreased from 3.72 [+ or -] 0.35 cm.sup.2 to 3.63 [+ or -] 0.36 cm.sup.2 (p = 0.04), end-diastolic length increased from 25.6 [+ or -] 9.0 mm to 28.8 [+ or -] 9.9 mm (p = 0.01), and end-diastolic pressure fell from 5.6 [+ or -] 0.8 mm Hg to 4.2 [+ or -] 0.8 mm Hg (p = 0.005), suggesting an improved diastolic compliance. In the control group, the hematocrit value, myocardial cross-sectional area, end-diastolic length, and pressure did not change (all p > 0.05). Mean ejection pressure increased in the ultrafiltration group (p = 0.001) but did not change in the control group (p = 0.22). The slope of the preload-recruitable stroke work index increased after ultrafiltration from 52.3 [+ or -] 52.0 to 74.2 [+ or -] 66.0 (10.sup.3 erg/cm.sup.3) (p = 0.02) but did not change in the control group (p = 0.07). One patient from each group died in the postoperative period. Patients in the ultrafiltration group received less inotropic drug support in the first 24 hours after the operation (156.62 [+ or -] 92.31 [mu]g/kg in 24 hours) than patients in the control group (865.33 [+ or -] 1772.26 [mu]g/kg in 24 hours, p = 0.03). Conclusions: Use of modified ultrafiltration after cardiopulmonary bypass improves intrinsic left ventricular systolic function, improves diastolic compliance, increases blood pressure, and decreases inotropic drug use in the early postoperative period. (J Thorac Cardiovasc Surg 1998;115:361-70) Article History: Received 7 May 1997; Revised 23 October 1997; Accepted 23 October 1997 Article Note: (footnote) [star] Sponsored by Marc R. de Leval, MD, FRCS., [star][star] From the Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom,a and the Childrens Hospital of Philadelphia, Philadelphia, Pa.b , a revisions requested June 9, 1997, aa Address for reprints: Michael J. Davies, FRCS, Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH, United Kingdom., acents 12/6/87224

Byline: Gabriel Chow, Idris G. Roberts, A.David Edwards, Adrian Lloyd-Thomas, Angie Wade, Martin J. Elliott, Fenella J. Kirkham Abstract: Objectives: Neurologic impairment, at least partly ischemic in origin, has been reported in up to 25% of infants undergoing cardiopulmonary bypass, with or without circulatory arrest. Controversy continues about the effect of pump flow, pulsatile or nonpulsatile, on the brain and in particular on cerebral blood flow. This study examines the relationship between pump flow rate and cerebral hemodynamics during pulsatile and nonpulsatile cardiopulmonary bypass. Method: Near-infrared spectroscopy was used to determine cerebral blood flow and cerebral blood volume (measured as concentration change) in a randomized crossover study. Pulsatile and nonpulsatile flow were used for six 5-minute intervals at each of three different pump flow rates (0.6, 1.2, and 2.4 L * m.sup.2 * min.sup.-1) in 40 patients, median age 2 months (range 2 weeks to 20 years 5 months). The relations between pulsatile flow, pump flow rate, cerebral blood flow, hemoglobin concentration change (cerebral blood volume), mean arterial pressure, arterial carbon dioxide tension, and hematocrit value were prospectively examined by means of multivariate analysis. Results: Cerebral blood flow decreased 36% per L * m.sup.-2 * min.sup.-1 decrease in pump flow rate and was associated with changes in mean arterial pressure but did not differ according to pulsatility. Change in hemoglobin concentration was unrelated to changes in pulsatility of pump flow. Conclusion: Cerebral blood flow is related to pump flow rate. Pulsatile flow delivered with a Stockert pump does not increase cerebral blood flow or alter hemoglobin concentration during cardiopulmonary bypass in children. (J Thorac Cardiovasc Surg 1997;114:568-77) Article History: Received 23 September 1996; Revised 19 November 1997; Revised 30 April 1997; Accepted 2 May 1997 Article Note: (footnote) [star] From the Departments of Neurosciences,a Anaesthetics,b Epidemiology and Biostatistics,c and Cardiothoracic Surgery,d Institute of Child Health (UCL)/Great Ormond Street Hospital for Children, Great Ormond Street, and Department of Pediatrics and Neonatal Medicine,e Royal Postgraduate Medical School, Du Cane Rd., London, United Kingdom., [star][star] This work was performed at Great Ormond Street Hospital for Children, London, United Kingdom., a This work was funded by the British Heart Foundation. F.J.K. is funded by the Wellcome Trust., aa Address for reprints: Fenella J. Kirkham, FRCP, Neurosciences Unit, Institute of Child Health (UCL), Mecklenburgh Square, London WC1N 2AP, United Kingdom., acents 0022-5223/97 $5.00 + 0 12/1/83071

Byline: Jeffrey P. Jacobs, Martin J. Elliott, Marcus P. Haw, C.Martin Bailey, Claus Herberhold Abstract: Purpose: Tracheal stenosis can be a life-threatening problem in children. Long-segment tracheal stenosis and recurrent tracheal stenosis are especially problematic. Tracheal homograft reconstruction represents a novel therapeutic modality for these patients. Methods: Cadaveric trachea is harvested, fixed in formalin, washed in thimerosal (Methiolate), and stored in acetone. The stenosed tracheal segment is opened to widely patent segments proximally and distally. The anterior cartilage is excised and the posterior trachealis muscle or tracheal wall remains. A temporary silicone rubber intraluminal stent is placed and absorbable sutures secure the homograft. Regular postoperative bronchoscopic treatment clears granulation tissue. The stent is removed endoscopically after epithelialization over the homograft. Twenty-four children with severe tracheal stenosis (age 5 months to 18 years, mean [+ or -] standard error of the mean = 8.18 [+ or -] 1.21 years) underwent tracheal homograft reconstruction. All but one had had previous unsuccessful reconstructive attempts. Ten lesions were congenital, nine were posttraumatic, and five were due to prolonged intubation. Results: Follow-up ranged from 5 months to 10 years (3.79 [+ or -] 0.70 years). Twenty patients survive (20/24 = 83%), 16 without any airway problems. Four patients are still undergoing treatment. One patient requiring emergency extracorporeal membrane oxygenator support before the operation died 10 days after tracheal homograft reconstruction. Another patient with severe preoperative mediastinal sepsis died 3.5 months after tracheal homograft reconstruction. Two patients with functional airways died late of unrelated problems. Conclusions: Tracheal homograft reconstruction demonstrates encouraging short-term to medium-term results for children with severe recurrent tracheal stenosis. Postoperative bronchoscopic and histologic studies provide evidence of epithelialization and support the expectation of good long-term results. (J Thorac Cardiovasc Surg 1996;112:1549-60) Article History: Received 6 May 1996; Revised 3 June 1996; Revised 25 June 1996; Accepted 1 July 1996 Article Note: (footnote) [star]1 Sponsored by Marc R. de Leval, MD., [star]2 From Great Ormond Street Hospital for Children, London, United Kingdom,a and the University of Bonn, Bonn, Germany.b , [star]3 Read at the Seventy-sixth Annual Meeting of The American Association for Thoracic Surgery, San Diego, Calif., April 28-May 1, 1996., [star]4 Address for reprints: Martin J. Elliott, MD, FRCS, Cardiothoracic Unit, Great Ormond Street Hospital for Children, London, United Kingdom WCIN 3JH., [star]5 *Present institution: Miami Children's Hospital, Miami, Fla., [star]6 **Present institution: Harefield Hospital, London, United Kingdom., [star]7 0022-5223/96 $5.00 + 0, [star]8 12/6/76252

Byline: Ralph E. Delius, Marc A. Rademecker, Marc R. de Leval, Martin J. Elliott, Jaroslav Stark Abstract: Objectives: The aim of this report is to examine the short-and intermediate-term outcome of a complex biventricular repair compared with a single ventricle repair in patients with two functional ventricles. Patient population: Since 1986, 34 patients with atrioventricular concordance or discordance, ventriculoarterial discordance, ventricular septal defect, and pulmonary stenosis or atresia have undergone biventricular repair (group I). Another group of 16 patients (group II) with the same diagnoses have undergone a single ventricle repair consisting of a total cavopulmonary connection because of either a straddling atrioventricular valve (11 patients) or an uncommitted ventricular septal defect (5 patients). Results: The mean length of follow-up was 3.9 years in group I and 3.0 years in group II. Freedom from reoperation at 7 years was 45.5% in group I and 100% in group II (p = 0.014). The actuarial estimate of survival at 7 years was 68.0% in group I and 93.8% in group II (p = 0.048). Conclusion: Short- and intermediate-term morbidity and mortality were greater in patients undergoing a biventricular repair than in a similar group of patients undergoing total cavopulmonary connection. It is unknown whether the long-term results of a total cavopulmonary connection in patients with two ventricles are as good as those obtained with a biventricular approach. However, there may be situations in which the short- and intermediate-term risks of a complex biventricular repair may outweigh the long-term disadvantages of a single ventricle approach. (J Thorac Cardiovasc Surg 1996;112:1561-9) Article History: Received 3 May 1996; Revised 25 June 1996; Revised 26 August 1996; Accepted 27 August 1996 Article Note: (footnote) [star] From the Great Ormond Street Hospital NHS Trust, London, United Kingdom., [star][star] Read at the Seventy-sixth Annual Meeting of The American Association for Thoracic Surgery, San Diego, Calif., April 28-May 1, 1996., a Address for reprints: Marc R. de Leval, MD, FRCS, Cardiothoracic Unit, Great Ormond Street Hospital for Children, London WC1N 3JH, United Kingdom., aa 0022-5223/96 $5.00 + 0, acents 12/6/77573

Byline: Ralph E. Delius, Marc R. de Leval, Martin J. Elliott, Jaroslav Stark Abstract: Ojective: The aim of this report is to review the surgical experience of a single institution with a relatively large series of patients with mixed total pulmonary venous drainage. Patient population: Between January 1, 1971, and December 31, 1994, 232 patients with total pulmonary venous drainage underwent surgical correction. Twenty of these patients (8.6%) had mixed type total pulmonary venous drainage. Ages at operation ranged from 1 day to 46 months, with a median of 2.3 months. Results: Both cardiac catheterization and echocardiography were performed before operation in 12 patients. Four patients underwent only cardiac catheterization, and another four patients underwent only echocardiography. The sensitivity and specificity for catheterization were 94% and 99%, respectively; they were 31% and 100%, respectively, for echocardiography. Severe pulmonary venous obstruction was present in three patients, all of whom underwent emergency operation. Three patients (15%), all of whom had preoperative pulmonary venous obstruction, died after operation. There were two late deaths, one of pulmonary vein stenosis and the other of probable pulmonary hypertension. The actuarial survival at 10 years was 73% for all patients; patients who survived the initial operation had a 10-year survival of 87%. Conclusion: The diagnosis of mixed total pulmonary venous drainage can be difficult to establish by echocardiography or at the time of operation. For patients in stable condition, cardiac catheterization may be considered if fewer than three pulmonary veins are identified by echocardiography. Pulmonary venous obstruction is relatively infrequent in this group of patients but when present impacts patient survival significantly. The long-term results with this lesion are excellent. (J Thorac Cardiovasc Surg 1996;112:1581-8) Article History: Received 3 May 1996; Revised 21 June 1996; Revised 24 July 1996; Accepted 29 July 1996 Article Note: (footnote) [star] From the Great Ormond Street Hospital NHS Trust, London, United Kingdom., [star][star] Read at the Seventy-sixth Annual Meeting of The American Association for Thoracic Surgery, San Diego, Calif., April 28-May 1, 1996., a Reprint requests: Marc R. de Leval, MD, FRCS, Cardiothoracic Unit, Great Ormond Street Hospital for Children, London WC1N 3JH, United Kingdom., aa 0022-5223/96 $5.00 + 0, acents 12/6/76917

The success of cardiac surgery has transformed the prospects of children with congenital heart disease with over 90% now surviving to adulthood. The early pioneering surgeons took on significant risk, whilst current surgical practice emphasises safety and consistency. In this article we review important British contributions to the field and consider challenges for the future, specifically how to better manage and reduce the adverse sequelae of congenital cardiac surgery by continuing to innovate safely.

AbstractSubstantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code(IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases(ICD-11). The most recent publication of the IPCCCwas in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease(ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCCand ICD-11 is the IPCCC ICD-11 Nomenclatureand is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHDthrough 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHOICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHDthought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHDrealize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCCthat was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHDwill again publish updated versions of IPCCC, as IPCCCcontinues to evolve.

BackgroundPrevious research has identified a relationship between income inequality and child abuse and neglect in the USA. This association has received limited exploration outside the USA.MethodsAdministrative data on child protection (CP) in 172 English and Welsh local authorities between 2013 and 2018 were combined with data on deprivation, ethnic density and education from publicly available data sources. Commercial income data were used for Gini coefficient estimation. We tested whether similar evidence for three key findings from a US study could be found in England and Wales. These included whether there was evidence of a relationship between income inequality and child maltreatment, whether this relationship was non-linear and whether this relationship varied dependent on the level of poverty.ResultsThere was a significant non-linear relationship between income inequality and state care rates in England and Wales. Predicted state care rates were higher as income inequality increased, up until around average levels where the effect flattens. However, there was no significant relationship for models predicting CP plan/register rates. Income inequality, income deprivation, ethnic density and higher education were able to explain around 75% of the variance in English and Welsh state care rates.ConclusionsThere is some evidence to support the claim of a relationship between income inequality and child maltreatment beyond the USA in England and Wales, and a case for further comparative research, but there are significant limitations in the comparability of data.

Functional integration of proteins with carbon-based nanomaterials such as nanotubes holds great promise in emerging electronic and optoelectronic applications. Control over protein attachment poses a major challenge for consistent and useful device fabrication, especially when utilizing single/few molecule properties. Here, we exploit genetically encoded phenyl azide photochemistry to define the direct covalent attachment of four different proteins, including the fluorescent protein GFP and a β-lactamase binding protein (BBP), to carbon nanotube side walls. AFM showed that on attachment BBP could still recognize and bind additional protein components. Single molecule fluorescence revealed that on attachment to SWCNTs function was retained and there was feedback to GFP in terms of fluorescence intensity and improved resistance to photobleaching; GFP is fluorescent for much longer on attachment. The site of attachment proved important in terms of electronic impact on GFP function, with the attachment site furthest from the chromophore having the larger effect on fluorescence. Our approach provides a versatile and general method for generating intimate protein–CNT hybrid bioconjugates. It can be potentially applied to any protein of choice; the attachment position and thus interface characteristics with the CNT can easily be changed by simply placing the phenyl azide chemistry at different residues by gene mutagenesis. Thus, our approach will allow consistent construction and modulate functional coupling through changing the protein attachment position.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2006.01.063 Byline: Mazyar Kanani (a), Martin Elliott (a), Andrew Cook (a), Amy Juraszek (b), William Devine (c), Robert H. Anderson (a) Abstract: The mortality following repair of atrioventricular septal defects has fallen dramatically in the last 4 decades, but reoperation for late regurgitation across the left atrioventricular valve has remained disconcertingly stagnant. Seeking potential structural causes, we compared the morphology of the surgically created septal leaflet of the left valve following repair of atrioventricular septal defects to the aortic leaflet of the normal mitral valve. Author Affiliation: (a) Cardiac Unit, Great Ormond Street Hospital for Children, London, UK (b) Cardiac Registry, Department of Pathology, Harvard Medical School, Children's Hospital, Boston, Mass (c) Department of Pathology, University of Pittsburgh Medical School, Children's Hospital of Pittsburgh, Pittsburgh, Pa. Article History: Received 6 July 2005; Revised 13 January 2006; Accepted 30 January 2006 Article Note: (footnote) Funded by the British Heart Foundation.

AbstractThis manuscript is a personal tribute to Professor Marc de Leval from two of his colleagues: Professor Martin J. Elliott and Professor John E. Deanfield. As stated by the authors: “Marc’s career history is presented in the accompanying eulogy from the current Heart & Lung Team at Great Ormond Street Hospital (GOSH), allowing us to highlight Marc’s personal qualities that made him such an inspirational colleague. … Marc was, as we have said, the cardiologist’s surgeon. He was also the surgeon’s cardiologist, bridging the two disciplines and fusing the team. He was delighted by the advent of interventional cardiology and did not see it as a threat or competition, but instead, as appropriate for the well-being of his patients. He recognised how traumatic surgery could be for patients and their families and sought to avoid it whenever possible by alternative treatments. Marc will be remembered with love and admiration by his many patients and their families, whose lives he changed. His technical skill, energy, devotion, humour, intellect and influence will be sorely missed. May he rest in peace.”

The Robert E Gross Lecture was delivered by Professor Martin Elliott of London, at Boston Children’s Hospital June 1st, 2016. Professor Elliott makes a plea for revision to the system of medical publication and academic reward, especially in the field of surgery.

Tracheal replacement for the treatment of end‐stage airway disease remains an elusive goal. The use of tissue‐engineered tracheae in compassionate use cases suggests that such an approach is a viable option. Here, a stem cell‐seeded, decellularized tissue‐engineered tracheal graft was used on a compassionate basis for a girl with critical tracheal stenosis after conventional reconstructive techniques failed. The graft represents the first cell‐seeded tracheal graft manufactured to full good manufacturing practice (GMP) standards. We report important preclinical and clinical data from the case, which ended in the death of the recipient. Early results were encouraging, but an acute event, hypothesized to be an intrathoracic bleed, caused sudden airway obstruction 3 weeks post‐transplantation, resulting in her death. We detail the clinical events and identify areas of priority to improve future grafts. In particular, we advocate the use of stents during the first few months post‐implantation. The negative outcome of this case highlights the inherent difficulties in clinical translation where preclinical in vivo models cannot replicate complex clinical scenarios that are encountered. The practical difficulties in delivering GMP grafts underscore the need to refine protocols for phase I clinical trials. StemCellsTranslationalMedicine2017;6:1458–1464 Here, a stem cell‐seeded, decellularized tissue‐engineered tracheal graft was used on a compassionate basis for a girl with critical tracheal stenosis after conventional reconstructive techniques failed. We report a compassionate use case of tracheal tissue engineering using a cell‐seeded, decellularized donor trachea. The teenage recipient had critical stenosis after conventional reconstructive techniques had failed. The case involved application of full good manufacturing practice standards to create a manufactured advanced medical product for transplantation.

The channel width-to-length ratio is an important transistor parameter for integrated circuit design. Contact diffusion into the channel during fabrication or operation alters the channel width and this important parameter. A novel methodology combining atomic force microscopy and scanning Kelvin probe microscopy (SKPM) with self-consistent modeling is developed for the nondestructive detection of contact diffusion on active devices. Scans of the surface potential are modeled using physically based Technology Computer Aided Design (TCAD) simulations when the transistor terminals are grounded and under biased conditions. The simulations also incorporate the tip geometry to investigate its effect on the measurements due to electrostatic tip–sample interactions. The method is particularly useful for semiconductor– and metal–semiconductor interfaces where the potential contrast resulting from dopant diffusion is below that usually detectable with scanning probe microscopy.

Stem cell-based tissue-engineered tracheas are at an early stage in their product development cycle. Tens of patients have been treated worldwide in predominantly compassionate use settings, demonstrating significant promise. This potentially life-saving treatment is complex, and the cost and its implications for such treatments are yet to be fully understood. The costs are compounded by varying strategies for graft preparation and transplant, resulting in differing clinical and laboratory costs from different research groups. In this study, we present a detailed breakdown of the clinical and manufacturing costs for three of the United Kingdom (UK) patients treated with such transplants. All three patients were treated under Compassionate Use legislation, within the UK National Health Service (NHS) hospital setting. The total costs for the three UK patients treated ranged from $174,420 to $740,500. All three patients were in a state of poor health at time of treatment and had a number of complexities in addition to the restricted airway. This is the first time a cost analysis has been made for a tissue-engineered organ and provides a benchmark for future studies, as well as comparative data for use in reimbursement considerations.

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Postpneumonectomy syndrome is a rare complication following pneumonectomy with its related change in mediastinal configuration. Symptoms range from airway obstruction to esophageal symptoms, leading at times to a persistent requirement for respiratory support. Surgery is often beneficial, which is in the form of placement of a prosthesis, with variable results. We report 2 cases of postpneumonectomy syndrome: one with successful relief, and the other in which the saline-filled prosthesis failed to achieve the desired result.

Introduction:Prosthetic materials, autologous tissues, cryopreserved homografts and allogeneic tissues have thus far proven unsuccessful in providing long-term functional solutions to extensive upper airway disease and damage. Research is therefore focusing on the rapidly expanding fields of regenerative medicine and tissue engineering in order to provide stem cell-based constructs for airway reconstruction, substitution and/or regeneration.Areas covered:Advances in stem cell technology, biomaterials and growth factor interactions have been instrumental in guiding optimization of tissue-engineered airways, leading to several first-in-man studies investigating stem cell-based tissue-engineered tracheal transplants in patients. Here, we summarize current progress, outstanding research questions, as well as future directions within the field.Expert opinion:The complex immune interaction between the transplant and host in vivois only beginning to be untangled. Recent progress in our understanding of stem cell biology, decellularization techniques, biomaterials and transplantation immunobiology offers the prospect of transplanting airways without the need for lifelong immunosuppression. In addition, progress in airway revascularization, reinnervation and ever-increasingly sophisticated bioreactor design is opening up new avenues for the construction of a tissue-engineered larynx. Finally, 3D printing is a novel technique with the potential to render microscopic control over how cells are incorporated and grown onto the tissue-engineered airway.

Stem-cell-based, tissue engineered transplants might offer new therapeutic options for patients, including children, with failing organs. The reported replacement of an adult airway using stem cells on a biological scaffold with good results at 6 months supports this view. We describe the case of a child who received a stem-cell-based tracheal replacement and report findings after 2 years of follow-up.

The redox-active protein cytochrome b562has been engineered to introduce pairs of thiol groups in the form of cysteine residues at specified sites. Successful STM imaging of the molecules adsorbed on a gold surface indicated that one thiol group controls the orientation of the molecule and that the protein maintains its native form under the experimental conditions. Stable protein–gold STM tip electrical contact was directly observed to form viathe second free thiol group in current–voltage and current–distance measurements. Proteins with thiol contacts positioned across the protein’s short axis displayed a conductance of (3.48 ± 0.05) × 10–5G0. However proteins with thiol groups placed along the long axis reproducibly yielded two distinct values of (1.95 ± 0.03) × 10–5G0and (3.57 ± 0.11) × 10–5G0, suggesting that the placement of the asymmetrically located haem within the protein influences electron transfer. In contrast, the unengineered wild-type cytochrome b562had conductance values at least 1 order of magnitude less. Here we show that an electron transfer protein engineered to bind gold surfaces can be controllably oriented and electrically contacted to metallic electrodes, a prerequisite for potential integration into electronic circuits.

Introduction:Prosthetic replacements, autologous tissue transfer and allografts have so far failed to offer functional solutions for the treatment of long circumferential tracheal defects and loss of a functioning larynx. Interest has therefore turned increasingly to the field of tissue-engineering which applies the principles and methods of bioengineering, material science, cell transplantation and life sciences in an effort to develop in vitrobiological substitutes able to restore, maintain or improve tissue and organ function.Areas covered:This article gives an overview of the tissue-engineering approach to airway replacement and will describe the encouraging results obtained so far in tracheal regeneration. The recent advances in the field of tissue-engineering have provided a new attractive approach towards the concept of functional substitutes and may represent an alternative to the shortage of suitable grafts for reconstructive airway surgery. We summarize fundamental questions, as well as future directions in airway tissue engineering.Expert opinion:The replacement of active movement, as would be necessary to replace an entire larynx introduces another order of magnitude of complexity, although progress in this area is starting to bear fruit. In addition, the stem cell field is advancing rapidly, opening new avenues for this type of therapy.

AbstractBackground and aimsSeveral years ago, one of us described the difference in attitude to patients with hypoplastic left heart syndrome in the United States of America and Europe as similar to that between Mars and Venus. Uncertainty remains with regard to the long-term prognosis for patients with hypoplastic left heart syndrome. This prognosis may be considered in terms of survival, functional performance, including exercise capacity and neurodevelopment, as well as psychosocial effects on the patient, family, and siblings. Counselling parents where either an antenatal or postnatal diagnosis of hypoplastic left heart syndrome has been made requires practitioners to give information on these prognostic aspects. We wanted to see how attitudes among European surgeons have changed over the last few years.MethodsWe performed a review of recent European data for hypoplastic left heart syndrome and conducted a survey among surgeons in major European centres to ascertain key aspects of their attitudes to the management of patients with hypoplastic left heart syndrome and how they counsel parents.Results and conclusionsAs of January, 2011, 2392 citations in the PubMed database were available for the search string “hypoplastic left heart”. The majority of these were from the centres from the United States of America and Europe. The European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Database shows an annual increase in the number of Norwood (Stage I) operations for hypoplastic left heart syndrome from 2003 to 2009, with a corresponding reduction in mortality. European rates of antenatal detection vary widely between centres, as do the rates of termination for a prenatal diagnosis of hypoplastic left heart syndrome. We observed a wide variation in the estimates of surgeons for survival and quality of life for surgical palliation of hypoplastic left heart syndrome, as well as in their estimates for actual rates of termination of pregnancy in their centres. Further, there was marked inconsistency in the information given to parents as part of the process of counselling. These issues remain to be resolved if parents are to make a fully informed decision for their child.

Single-center studies have failed to link modest increases in total donor ischemic time to mortality after pediatric orthotopic heart transplant. We aimed to investigate whether prolonged total donor ischemic time is linked to pediatric intensive care morbidity after orthotopic heart transplant.

To define the factors influencing the outcome of aortopexy as management of tracheo-bronchomalacia.

AbstractInterventional cardiology for paediatric and congenital cardiac disease is a relatively young and rapidly evolving field. As the profession begins to establish multi-institutional databases, a universal system of nomenclature is necessary for the field of interventional cardiology for paediatric and congenital cardiac disease. The purpose of this paper is to present the results of the efforts of The International Society for Nomenclature of Paediatric and Congenital Heart Disease to establish a system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease, focusing both on procedural nomenclature and the nomenclature of complications associated with interventional cardiology. This system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease is a component of The International Paediatric and Congenital Cardiac Code. This manuscript is the second part of the two-part series. Part 1 covered the procedural nomenclature associated with interventional cardiology as treatment for paediatric and congenital cardiac disease. Part 2 will cover the nomenclature of complications associated with interventional cardiology as treatment for paediatric and congenital cardiac disease.

AbstractInterventional cardiology for paediatric and congenital cardiac disease is a relatively young and rapidly evolving field. As the profession begins to establish multi-institutional databases, a universal system of nomenclature is necessary for the field of interventional cardiology for paediatric and congenital cardiac disease. The purpose of this paper is to present the results of the efforts of The International Society for Nomenclature of Paediatric and Congenital Heart Disease to establish a system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease, focusing both on procedural nomenclature and on the nomenclature of complications associated with interventional cardiology. This system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease is a component of The International Paediatric and Congenital Cardiac Code. This manuscript is the first part of a two-part series. Part 1 will cover the procedural nomenclature associated with interventional cardiology as treatment for paediatric and congenital cardiac disease. This procedural nomenclature of The International Paediatric and Congenital Cardiac Code will be used in the IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatment) of the National Cardiovascular Data Registry®of The American College of Cardiology. Part 2 will cover the nomenclature of complications associated with interventional cardiology as treatment for paediatric and congenital cardiac disease.

Tremendous progress has been made in the field of pediatric heart disease over the past 30 years. Although survival after heart surgery in children has improved dramatically, complications still occur, and optimization of outcomes for all patients remains a challenge. To improve outcomes, collaborative efforts are required and ultimately depend on the possibility of using a common language when discussing pediatric and congenital heart disease. Such a universal language has been developed and named the International Pediatric and Congenital Cardiac Code (IPCCC). To make the IPCCC more universally understood, efforts are under way to link the IPCCC to pictures and videos. The Archiving Working Group is an organization composed of leaders within the international pediatric cardiac medical community and part of the International Society for Nomenclature of Paediatric and Congenital Heart Disease (www.ipccc.net). Its purpose is to illustrate, with representative images of all types and formats, the pertinent aspects of cardiac diseases that affect neonates, infants, children, and adults with congenital heart disease, using the codes and definitions associated with the IPCCC as the organizational backbone. The Archiving Working Group certifies and links images and videos to the appropriate term and definition in the IPCCC. These images and videos are then displayed in an electronic format on the Internet. The purpose of this publication is to report the recent progress made by the Archiving Working Group in establishing an Internet-based, image encyclopedia that is based on the standards of the IPCCC.

OBJECTIVES To describe a multimodality approach to the management of pediatric head and neck lymphatic malformations using surgery, sclerotherapy, or both and to review the outcomes of these approaches. DESIGN Retrospective case series. SETTING A single pediatric tertiary care referral center. PATIENTS Ninety-seven pediatric patients (aged 1 month to 16 years) diagnosed as having lymphatic malformations of the head and neck during a 7-year period. Follow-up ranged from 3 months to 7 years. INTERVENTIONS All of the patients underwent clinical and radiologic (magnetic resonance imaging) assessment. Treatment modality was selected according to disease location, cyst size, and parental preference. Treatments included surgery (open excision, tongue reduction, electrocautery, and laser treatment), sclerotherapy with OK-432 (Picibanil) or a fibrosing agent (Ethibloc), and a combination of modalities. MAIN OUTCOME MEASURES Clinically determined responses to treatment, complications, and number of treatments required. RESULTS All isolated neck disease had complete or near-complete responses, with no nerve palsies sustained. Although most patients achieved complete or near-complete responses, disease with parotid, laryngopharyngeal, or oral components had poorer outcomes and frequently required multiple treatments. Significant long-term neural injury was sustained in 3 of 6 surgical patients for mediastinal disease and in only 4% (n = 4) of other surgical procedures. CONCLUSIONS Surgery retains an important role in the treatment of pediatric head and neck lymphatic malformations despite the advent of sclerotherapy. Isolated neck disease has an excellent outcome with either modality. Treatment decisions were made via a problem-based approach and were individualized according to anatomical location and disease classification.Arch Otolaryngol Head Neck Surg. 2010;136(3):270-276--

The current practice of slide tracheoplasty for children with long-segment tracheal stenosis is reviewed.

The poem "Anthracite" by Martin Elliott is presented. First Line: For now, I only dream of Anthracite; Last Line: above our own coal face of Anthracite.

The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. At the severe end of the spectrum is found the combination of aortic and mitral atresia, when the left ventricle can be close to non-existent. At the mild end are the patients with hypoplasia of the aortic and mitral valves, but without intrinsic valvar stenosis or atresia, and milder degrees of left ventricular hypoplasia. Although the majority of the patients are suitable only for functionally univentricular repair, a small minority may be candidates for biventricular repair.The nature of the syndrome was a topic for discussion at the second meeting of the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, the Nomenclature Working Group, held in Montreal, Canada, over the period January 17 through 19, 2003. Subsequent to these discussions, the Nomenclature Working Group was able to create a bidirectional crossmap between the nomenclature initially produced jointly on behalf of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and the alternative nomenclature developed on behalf of the Association for European Paediatric Cardiology. This process is a part of the overall efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system of nomenclature for paediatric and congenital cardiac disease, the International Paediatric and Congenital Cardiac Code. In this review, we discuss the evolution of nomenclature and surgical treatment for the spectrum of lesions making up the hypoplastic left heart syndrome and its related malformations. We also present the crossmap of the associated terms for diagnoses and procedures, as recently completed by the Nomenclature Working Group.

Congenitally corrected transposition is a complex cardiac lesion that is often associated with ventricular septal defect, obstruction of the outflow tract of the morphologically left ventricle, and abnormalities of the morphologically tricuspid valve.1,2 Nomenclature for this lesion has been variable and confusing.1 In this review, we define, and hopefully clarify this terminology. The lesion is a combination of discordant union of the atrial chambers with the ventricles, and the ventricles with the arterial trunks.1,2 In rare circumstances, discordant atrioventricular connections can be associated with concordant ventriculo-arterial connections. This malformation has been called “isolated ventricular inversion”. The term is less than precise, and the descriptive approach using the phrase “discordant atrioventricular connections with concordant ventriculo-arterial connections” is preferred, as discussed below.

The nomenclature and classification of patients with a functionally univentricular heart has been debated for decades. We review here the approach taken for dealing with this group of patients by the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Cardiac Disease. We discuss the approach of this Nomenclature Working Group in the context of other historical and contemporary ideas about this topic.

There are many reasons for seeking to create a global database with which to record the outcomes of therapy for congenital heart disease. Such a database can function as a tool to support a variety of purposes:

The abilities for both computer technology, and intra-operative video-imaging, are evolving rapidly. The merger of these two sciences can be very beneficial, both to congenital cardiac surgeons in general, and in facilitating the creation of a cardioscopic database in particular.

The detail of cardiac morphology, with its subtle complexity and variation, is difficult to convey to colleagues and families, and even more time-consuming to learn. The terminology is occasionally unresolved, visual representations challenging, and there is often a mismatch between the presentation of the defect by the morphologist, the echocardiographic impression gained by the cardiologist, and the view obtained by the surgeon in the operating room. It can be said, therefore, that size, complexity and three-dimensional configuration, the three most important concepts to grasp when describing the congenitally malformed heart, are seldom touched upon with traditional illustrations or texts.

As once observed by Tom Spray, “When they invite you to give the history talk, you're finished!” Those words flashed through my mind when I was invited to deliver the version of the lecture celebrating George Daicoff, and forced me to think carefully about what I do, and what I believe about doing it. I have been doing cardiac surgery for 25 years, and paediatric cardiac surgery for 20 of those. Looking back, it would be tempting to think that I am here to follow in the illustrious footsteps of Kirklin, Debakey, Lillehei, Cooley, Ross, Yacoub or Daicoff. But I have to admit that would not be the truth. I was more influenced by Dr Kildare and M.A.S.H., driven by the drama of their lives and the beauty of their nurses. I have not been disappointed.

Recently, the well-known American political commentator, Robert Kagan, reapplied an existing populist statement in describing Americans as being from Mars, and Europeans from Venus. He said “they agree on little and understand one another less and less”. In general, it would be churlish to say that this applied to the medical fraternity, but it has great resonance with intercontinental differences in the management of hypoplastic left heart syndrome. In this review, I offer a personal view of those differences, potential explanations for them, and consider how the situation may change in coming years.

Surgical skills are traditionally assessed through subjective evaluation with experienced surgeons observing and rating trainee activity. However, this process can be expensive, particularly in terms of the time required of expert surgeons. Subjective assessment may also suffer from problems of intersubject variation in the application of evaluation criteria. Computer-based training, in particular systems combining virtual reality interfaces with haptic displays, offers a means of providing both automated and objective assessment of performance. In this paper we propose that, prior to the development of such systems, there is a need to determine measures that can adequately differentiate levels of performance. The paper therefore discusses the evaluation of surgical technique using objective metrics. Two main questions are addressed. How can surgical technique be assessed? What metrics prove useful in defining and modifying surgical skills and techniques?

Objective:The optimal treatment of pediatric empyema remains controversial. The objective of this study is to compare the use of conventional management versus primary thoracoscopic drainage and decortication in children with empyema. Methods:Conventional management has consisted of chest drain insertion under general anesthesia plus intravenous antibiotics. Thoracoscopic drainage and decortication has consisted of primary thoracoscopic drainage and decortication plus antibiotics. The clinical course of 54 patients treated conventionally between 1989 and 1997 was compared with that of 21 patients treated by means of thoracoscopic drainage and decortication between September 2000 and September 2001. Results:Results of the study demonstrated that patients in the drainage-decortication group had fewer invasive interventions per patient than those in the conventional management group (1.0 vs 1.26). Patients undergoing thoracoscopic drainage and decortication also had significantly shorter durations of intravenous antibiotic therapy (7.6 ± 1.2 vs 18.2 ± 7.5 days), chest tube drainage (4.0 ± 0.5 vs 10.2 ± 6.1 days), and hospital stays (7.4 ± 0.8 vs 15.4 ± 7.4). Moreover, there were no open thoracotomies and decortications in the thoracoscopic drainage and decortication group, whereas in the conventional management group 39% (21/54) of patients underwent an open procedure. Conclusion:Although the 2 groups were not prospectively randomized and they were treated in different time periods, the results of this study support the use of thoracoscopic surgery as the primary therapeutic modality in children presenting with pleural empyema. This strategy appears to offer significant benefits over conventional treatment in terms of duration of treatment and the need for more invasive surgery.

Background:Junctional ectopic tachycardia is a major cause of postoperative morbidity after surgery for congenital cardiac disease. To elucidate the mechanism of junctional ectopic tachycardia, surgical correlations were studied in four types of congenital heart defects involving closure of a ventricular septal defect, relief of right ventricular outflow tract obstruction, or both. Methods:Between 1997 and 1999, a total of 343 consecutive patients underwent repair of tetralogy of Fallot (n = 114), common truncus arteriosus (n = 10), ventricular septal defect (n = 161), and atrioventricular septal defect (n = 58). Variables studied included demographic and bypass data, surgical approaches toward ventricular septal defect closure and relief of right ventricular outflow tract obstruction, and resection as opposed to division of muscle bundles. Results:Junctional ectopic tachycardia occurred most frequently after repair of tetralogy of Fallot (n = 25; 21.9%), with no cases occurring after repair of common trunk, 6 occurring after repair of ventricular septal defect (3.7%), and 6 occurring after repair of atrioventricular septal defect (10.3%). Stepwise logistic regression revealed that resection of muscle bundles (P<.0001), higher bypass temperatures (P<.03), and relief of right ventricular outflow tract obstruction through the right atrium (P<.05) significantly and independently predicted postoperative junctional ectopic tachycardia. Conclusions:Relief of right ventricular outflow tract obstruction appears to be more important in the causation of junctional ectopic tachycardia than does ventricular septal defect closure, which may explain the higher incidence of this complication after tetralogy of Fallot repair. Muscular resection seems to be more arrhythmogenic than is simple division. Increased traction through the right atrium for relief of right ventricular outflow tract obstruction would fit the hypothesis that enhanced automaticity of the His bundle, the morphologic substrate for junctional ectopic tachycardia, may result from direct trauma or infiltrative hemorrhage of the conduction system. When feasible, techniques avoiding both extensive muscle resection and excessive traction should be applied during resection of right ventricular outflow tract obstruction.

The rationale for a congenital heart surgery database lies in the organized manner in which information can be compiled to accomplish programmatic evaluation, monitor clinical outcomes, comply with governmental requirements, perform retrospective and prospective clinical studies, and participate in local, national, and global improvement strategies. The task of inaugurating an effective congenital heart surgery database has taken many years and involved concurrent development efforts at multiple sites. Two such efforts took place in North America with the Society of Thoracic Surgeons Congenital Heart Surgery Database, and in Europe with the European Congenital Heart Defects Database. These initial efforts provided the impetus for development of an international congenital heart surgery database that would allow scientific exchanges on an international scale and promote multi-institutional evaluation of congenital heart surgery. Sample outcome templates from the Society of Thoracic Surgeons and the European Association for Cardio-thoracic Surgery's accepted minimum database data set are reviewed for the specific diagnostic entry ventricular septal defect, to familiarize the reader with potential available data summaries and outcome analyses, including risk stratification, when data harvest is performed.In Europe, the agreed upon minimum data set are now collected as part of the Pediatric European Cardiac Surgical Registry, while in North America users of the CardioAccess database and users of the minimum data set downloaded from the Society of Thoracic Surgeons web site are being notified of plans for a 2002 data harvest incorporating the minimum database data set. It is likely that the extant voluntary, minimal data set congenital heart surgery database will be replaced by a mandated system required by states, provinces, and countries. Data collection schemes and data validation programs will become standardized, which will result in improved data quality and uniform congenital heart center participation. As participation with valid data approaches 100% of congenital heart centers, the power and importance of the database increases exponentially. Reliable information can be used to assess residency/fellowship programs, resource allocation, major therapeutic trends, manpower issues, and in the end, all initiatives that will require accurate information. Copyright © 2002 by W.B. Saunders Company

From among 112 patients with cystic fibrosis who were assessed for heart-lung transplantation, 83 were accepted. Twenty-six died while awaiting heart-lung transplantation and 32 had the operation. The management and the outcome of these 32 patients is reported. Survival, infection, and rejection rates among these patients were compared with those of 61 patients without cystic fibrosis who underwent heart-lung transplantation between 1984 and 1990. The cumulative survival rate was 72.29% ± 94.91% at 1 year and 55.59% ± 7.50% at 3 years. The mortality rate was slightly higher in the group with cystic fibrosis during the first year after the operation but it was lower at 3 years. The difference, however, could have been due to chance alone (p = 0.308). The same was true for the prevalence of rejection (up to 6 months: χ2=1-8141, p = 0.17), and infection (up to 6 months: χ2= 2.20, p = 0.14), between the two groups. It is concluded that cystic fibrosis does not constitute an additional risk in terms of survival and morbidity after heart-lung transplantation.

With increasing experience in the surgical management of children with congenital heart disease has come an increasing use of prosthetic valves. They have been employed in a wide variety of conditions ranging from simple congenital stenosis or incompetence to an essential part of repair of more complex disorders. As in adult cardiac surgery, there has been considerable debate over the exact prostheses that should be used in individual circumstances. As we point out in this article, experience has varied both geographically and with time. Our own experience at Great Ormond Street, for example, has been of initial satisfaction with results of mechanical valves followed by enthusiasm for, sequentially, the Hancock and the Ionescu prostheses. Now we have turned full circle and predominantly use mechanical valves, primarily owing to the extraordinary incidence of premature, degenerative calcification of bioprosthetic valves in children. The exact rates of degeneration of these valves are variable; thus it is difficult to relate prosthesis survival to the growth rate of the child. The experience with homograft valve replacements is encouraging, since they do not seem to degenerate so rapidly. Valve replacement in children carries a high risk. The younger the child, the higher the risk, with mitral valve replacement being the most dangerous procedure of all. We conclude that valve replacement in children is a procedure that should not be taken lightly and for which, at present, mechanical valves offer the best prognosis, despite the small risk of anticoagulation.