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Substantial 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 IPCCC was 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 IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and 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 ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought 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 ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.
In present and future magnetic confined fusion devices with metallic plasma-facing components (PFCs) such as JET-ILW and ITER, the calculation of the plasma composition must account for multiple impurities of a wide range of mass and charge, resolve their poloidal asymmetries and account for different central peakings for various elements. Single measurements of radiation and effective charge are not enough to characterize this complex system and a self-consistent analysis of data from multiple diagnostics is required. This contribution describes a method to calculate the plasma composition simultaneously accounting for contributions of up to two low-Z impurities, and two mid-/high-Z impurities. The analysis stems from methodologies explained in Sertoli et al. (Rev. Sci. Instrum., vol. 89 (11), 2018, 113501), expanded to include more impurities and to coherently analyse multiple diagnostics within the same framework. The example Ne-seeded JET-ILW hybrid discharge reported here shows that Be, Ne, Ni and W are necessary to simultaneously explain the observed soft X-ray emission, the W concentration measured by passive vacuum ultra-violet spectroscopy, the line-of-sight integrated measurement of the effective charge, the observed poloidal asymmetry of the soft X-ray (SXR) emission, the Ne density measured by charge-exchange-recombination spectroscopy and the line-of-sight integrals of the total radiation as measured by bolometry. This consistent picture of the elemental composition enables the calculation of the radial profiles of the effective charge, the dilution and total radiation. For the cases analysed up to now, these are often very different from the typical assumptions presently used when modelling JET-ILW discharges. This will affect, among others, the calculation of neutron rates, current density profile and heat transport. These considerations are of course valid for all present and future magnetic-controlled fusion devices which exhibit multi-material plasma-facing components, including ITER.
It is timely, in the 125th anniversary of the initial description by Fallot of the hearts most frequently seen in patients presenting with “la maladie bleu”, that we revisit his descriptions, and discuss his findings in the light of ongoing controversies. Fallot described three hearts in his initial publication, and pointed to the same tetralogy of morphological features that we recognise today, namely, an interventricular communication, biventricular connection of the aorta, subpulmonary stenosis, and right ventricular hypertrophy. In one of the hearts, he noted that the aorta arose exclusively from the right ventricle. In other words, one of his initial cases exhibited double-outlet right ventricle. When we now compare findings in hearts with the features of the tetralogy, we can observe significant variations in the nature of the borders of the plane of deficient ventricular septation when viewed from the aspect of the right ventricle. We also find that this plane, usually described as the ventricular septal defect, is not the same as the geometric plane separating the cavities of the right and left ventricles. This means that the latter plane, the interventricular communication, is not necessarily the same as the ventricular septal defect. We are now able to provide further insights into these features by examining hearts prepared from developing mice. Additional molecular investigations will be required, however, to uncover the mechanisms responsible for producing the morphological changes underscoring tetralogy of Fallot.
Florida is the fourth largest state in the United States of America. In 2004, 218,045 live babies were born in Florida, accounting for approximately 1744 new cases of congenital heart disease. We review the initial experience of The Society of Thoracic Surgeons Congenital Heart Surgery Database with a regional outcomes report, namely the Society of Thoracic Surgeons Florida Regional Report.
Eight centres in Florida provide services for congenital cardiac surgery. The Children’s Medical Services of Florida provide a framework for quality improvement collaboration between centres. All congenital cardiac surgical centres in Florida have voluntarily agreed to submit data to the Society of Thoracic Surgeons Database. The Society of Thoracic Surgeons and Duke Clinical Research Institute prepared a Florida Regional Report to allow detailed regional analysis of outcomes for congenital cardiac surgery.
The report of 2007 from the Society of Thoracic Surgeons Congenital Heart Surgery Database includes details of 61,014 operations performed during the 4 year data harvest window, which extended from 2003 through 2006. Of these operations, 6,385 (10.5%) were performed in Florida. Discharge mortality in the data from Florida overall, and from each Florida site, with 95% confidence intervals, is not different from cumulative data from the entire Society of Thoracic Surgeons Database, both for all patients and for patients stratified by complexity.
A regional consortium of congenital heart surgery centres in Florida under the framework of the Children’s Medical Services has allowed for inter-institutional collaboration with the goal of quality improvement. This experience demonstrates, first, that the database maintained by the Society of Thoracic Surgeons can provide the framework for regional analysis of outcomes, and second, that voluntary regional collaborative efforts permit the pooling of data for such analysis.
This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up.
During the 1990s, both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature, along with a common core minimal dataset, were adopted by The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons, and published in the Annals of Thoracic Surgery. In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. By 2005, the Nomenclature Working Group crossmapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology, and therefore created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET].
This common nomenclature, the International Paediatric and Congenital Cardiac Code, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons. Between 1998 and 2007 inclusive, this nomenclature and database was used by both of these two organizations to analyze outcomes of over 150,000 operations involving patients undergoing surgical treatment for congenital cardiac disease.
Two major multi-institutional efforts that have attempted to measure the complexity of congenital heart surgery are the Risk Adjustment in Congenital Heart Surgery-1 system, and the Aristotle Complexity Score. Current efforts to unify the Risk Adjustment in Congenital Heart Surgery-1 system and the Aristotle Complexity Score are in their early stages, but encouraging. Collaborative efforts involving The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons are under way to develop mechanisms to verify the completeness and accuracy of the data in the databases. Under the leadership of The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease, further collaborative efforts are ongoing between congenital and paediatric cardiac surgeons and other subspecialties, including paediatric cardiac anaesthesiologists, via The Congenital Cardiac Anesthesia Society, paediatric cardiac intensivists, via The Pediatric Cardiac Intensive Care Society, and paediatric cardiologists, via the Joint Council on Congenital Heart Disease and The Association for European Paediatric Cardiology.
In finalising our review, we emphasise that analysis of outcomes must move beyond 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 these databases to allow uniform, protocol driven, and meaningful, long term follow-up.
Persistent patency of the arterial duct represents one of the most common lesions in the field of congenital cardiac disease. The strategies for management continue to evolve. In this review, we focus on management beyond the neonatal period. We review the temporal evolution of strategies for management, illustrate the currently available the techniques for permanent closure of the patent arterial duct, review the expected outcomes after closure, discuss the current controversy over the appropriate treatment of the so-called “silent” duct, and provide recommendations for the current state of management of patients with persistent patency of the arterial duct outside of the neonatal period.
At the Congenital Heart Institute of Florida, we now recommend closure of all patent arterial ducts, regardless of their size. Before selecting and performing the type of procedure, we explain the natural history of the persistently patent arterial duct to the parents or legal guardian of the child. Particular emphasis is placed on the risks of endocarditis, including the recognition that many cases of endocarditis may not be preventable.
The devastating effects of endocarditis, coupled with the perception of more anecdotal reports of endocarditis with the silent duct, as well as the low risk of interventions, has led us to recommend closure of the patent arterial duct in these situations. We now recommend intervention, after informed consent, for all patients with a patent arterial duct regardless of size, including those in which the patent duct is “silent”. We recognize, however, that this remains a controversial topic, especially given the new recommendations for endocarditis prophylaxis from American Heart Association.
Since 2003, our strategy for closure of the patent arterial duct has changed subsequent to the availability of the Amplatzer occluder. This new device has allowed significantly larger patent arterial ducts to be closed with interventional catheterization procedures that in the past would have been closed at surgery. During the interval between 2002 and 2006 inclusive, the overall surgical volume at our Institute has been stable. Over this period, the number of patients undergoing surgical ligation of the patent arterial duct has decreased, with this decline in volume most notable for the subgroup of patients weighing more than five kilograms. This decrease has been especially notable in thoracoscopic procedures and is attributable to the increased ability to close larger ducts using the Amplatzer occluder.
For infants with symptomatic pulmonary overcirculation weighing less than 5 kilograms, our preference is for the surgical approach. For patients who have ductal calcification, significant pleural scarring, or “window-like” arterial ducts, video-assisted ligation is not an option and open surgical techniques are used. When video-assisted ligation is possible, the approach is based on family and surgeon preference. When open thoracotomy is selected, we usually use a muscle-sparing left posterolateral thoracotomy.
For patients weighing more than 5 kilograms, we currently recommend percutaneous closure for all patent arterial ducts as the first intervention, reserving surgical treatment for those cases that are not amenable to the percutaneous approach. For symptomatic infants weighing greater than 5 kilogram with large ducts, we prefer to use the Amplatzer occluder. In rare instances, the size of the required ductal occluder is so large that either encroachment into the aorta or pulmonary arteries is noted, and the device is removed. The child is then referred for surgical closure. We can now often predict via echocardiography that a duct is too large for transcatheter closure, even with the Amplatzer occluder, and refer these patients directly to surgery.
For patients with an asymptomatic patent arterial duct, we prefer to wait until the weight is from 10 to 12 kilograms, or they are closer to 2 years of age. If the patent arterial duct is greater than 2.0 to 2.5 millimetres in diameter, our preference is to use the Amplatzer occluder. For smaller ducts, we typically use stainless steel coils. Using this strategy, we close all patent arterial ducts, regardless of their size.
It was in 1971, Fontan and Baudet1 published their experience using a surgical procedure to restore a physiologic circulation in three patients with tricuspid atresia. Of the three patients, two survived, thus ushering a new era in the treatment of children with complex forms of congenital cardiac disease. This operation itself, however, was the culmination of a series of experimental and clinical observations that underlined the clinical possibility totally to bypass the right side of the heart.
After repair of tetralogy of Fallot, many patients present in need of reoperative surgical reconstruction of the right ventricular outflow tract. The predominant physiologic lesion is pulmonary insufficiency, but there may also be varying degrees of obstruction of the right ventricular outflow tract. In the past, it has been felt that patients tolerate pulmonary insufficiency reasonably well. In some patients, however, the long-term effects of pulmonary insufficiency and subsequent right ventricular dilation and dysfunction are associated with poor exercise tolerance and increased incidence of arrhythmias and sudden death.1,2 Numerous studies support replacement of the pulmonary valve as treatment for pulmonary insufficiency in order to improve performance, optimize hemodynamics, and better control arrhythmias.3–10 The indications for reconstruction of the right ventricular outflow tract in this setting, nonetheless, as well as the operative strategy, continue to evolve. There are multiple surgical options for replacement of the pulmonary valve for these patients, including aortic and pulmonary homografts, stented and stentless porcine valves, porcine valved conduits, bovine jugular venous conduits, and even mechanical valves and mechanical valved conduits.11–32 It was a less than ideal experience with these currently available options that stimulated our interest into employing alternative materials and techniques. Favorable experimental and clinical experience with valves made of a polytetrafluoroethylene monoleaflet33–36 encouraged us to consider a new method of reconstruction with this material, using a bifoliate polytetrafluoroethylene valve. In this work, we review our indications for replacement of the pulmonary valve after repair of tetralogy of Fallot, the surgical options available, and our experience reconstructing the right ventricular outflow tract with a new surgically created bifoliate polytetrafluoroethylene valve.
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