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Intraoperative imaging determines the integrity of surgical repairs. Transoesophageal echocardiography represents standard care for intraoperative imaging in CHD. However, some conditions preclude its use, and epicardial echocardiography is used alternatively. Minimal literature exists on the impact of epicardial echocardiography versus transoesophageal echocardiography. We aimed to evaluate accuracy between the two modalities and hypothesised higher imaging error rates for epicardial echocardiography.
We retrospectively reviewed all epicardial echocardiograms performed over 16 years and compared them to an age- and procedure-matched, randomly selected transoesophageal echocardiography cohort. We detected un- or misidentified cardiac lesions during the intraoperative imaging and evaluated patient outcomes. Data are presented as a median with a range, or a number with percentages, with comparisons by Wilcoxon two-sample test and Fisher’s exact test.
Totally, 413 patients comprised the epicardial echocardiography group with 295 transoesophageal echocardiography matches. Rates of imaging discrepancies, re-operation, and incision infection were similar. About 13% of epicardial echocardiography patients had imaging discrepancies versus 16% for transoesophageal (p = 0.2352), the former also had smaller body sizes (p < 0.0001) and more genetic abnormalities (33% versus 19%, p < 0.0001). Death/mechanical support occurred more frequently in epicardial echocardiography patients (16% versus 6%, p < 0.0001), while hospitalisations were longer (25 versus 19 days, p = 0.0003).
Diagnostic accuracy was similar between patients undergoing epicardial echocardiography and transoesophageal echocardiography, while rates of death and mechanical support were increased in this inherently higher risk patient population. Epicardial echocardiography provides a reasonable alternative when transoesophageal echocardiography is not feasible.
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.
The Pediatric Heart Network Normal Echocardiogram Database Study had unanticipated challenges. We sought to describe these challenges and lessons learned to improve the design of future studies.
Challenges were divided into three categories: enrolment, echocardiographic imaging, and protocol violations. Memoranda, Core Lab reports, and adjudication logs were reviewed. A centre-level questionnaire provided information regarding local processes for data collection. Descriptive statistics were used, and chi-square tests determined differences in imaging quality.
For the 19 participating centres, challenges with enrolment included variations in Institutional Review Board definitions of “retrospective” eligibility, overestimation of non-White participants, centre categorisation of Hispanic participants that differed from National Institutes of Health definitions, and exclusion of potential participants due to missing demographic data. Institutional Review Board amendments resolved many of these challenges. There was an unanticipated burden imposed on centres due to high numbers of echocardiograms that were reviewed but failed to meet submission criteria. Additionally, image transfer software malfunctions delayed Core Lab image review and feedback. Between the early and late study periods, the proportion of unacceptable echocardiograms submitted to the Core Lab decreased (14 versus 7%, p < 0.01). Most protocol violations were from eligibility violations and inadvertent protected health information disclosure (overall 2.5%). Adjudication committee reviews led to protocol changes.
Numerous challenges encountered during the Normal Echocardiogram Database Study prolonged study enrolment. The retrospective design and flaws in image transfer software were key impediments to study completion and should be considered when designing future studies collecting echocardiographic images as a primary outcome.
An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many “short list” versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various “short lists”. In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the “short list” for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.
The long-term outcome of patients with congenitally malformed hearts involving abnormal right ventricular morphology and haemodynamics is variable. In most instances, the patients are at risk for right ventricular failure, in part due to morphological differences between the right and left ventricles and their response to chronic volume and pressure overload. In patients after repair of tetralogy of Fallot, and after balloon valvotomy for valvar pulmonary stenosis, pulmonary regurgitation is the most significant risk factor for right ventricular dysfunction. In patients with a dominant right ventricle after Fontan palliation, and in those with systemic right ventricles in association with surgically or congenitally corrected transposition, the right ventricle is not morphologically capable of dealing with chronic exposure to the high afterload of the systemic circulation. In patients with Ebstein’s malformation of the tricuspid valve, the degree of atrialisation of the right ventricle determines how well the right ventricle will function as the pump for the pulmonary vascular bed.
We are researching the interaction between the rule and the ontology layers of the Semantic Web, by comparing two options: 1) using OWL and its rule extension SWRL to develop an integrated ontology/rule language, and 2) layering rules on top of an ontology with RuleML and OWL. Toward this end, we are developing the SWORIER system, which enables efficient automated reasoning on ontologies and rules, by translating all of them into Prolog and adding a set of general rules that properly capture the semantics of OWL. We have also enabled the user to make dynamic changes on the fly, at run time. This work addresses several of the concerns expressed in previous work, such as negation, complementary classes, disjunctive heads, and cardinality, and it discusses alternative approaches for dealing with inconsistencies in the knowledge base. In addition, for efficiency, we implemented techniques called extensionalization, avoiding reanalysis, and code minimization.
How best to analyse and describe the features of the situation commonly known as “visceral heterotaxy” remains controversial. Much of the disagreement devolves on how to deal with the concept of isomerism. In the opinion of some, the concept of bilateral right-sidedness and bilateral left-sidedness, while useful in helping to remember which abnormalities are likely to occur in asplenia or polysplenia, should not be granted the status of a specific “situs”, since there are numerous examples of exceptions to these patterns. On the other hand, those who favour the concept of isomerism point out that, when describing only the heart, and taking the structure of the atrial appendages as the starting point for analysis, basing this on the extent of the pectinate muscles relative to the atrioventricular junctions, then the only possible arrangements for the appendages are the usual one, its mirror-image, and the two situations in which appendages of comparable morphology are found on both sides of the heart, these being the arrangements of right or left isomerism. It is certainly the case that the arrangement of the organs is not always in harmony with the arrangement of the atrial appendages, but those circumstances, in which there is disharmony, can readily be described by paying specific attention to each series of organs. On this basis, in this review, we describe the approach to heterotaxy, and isomerism of the atrial appendages, in terms of the genetic background, the diagnosis, and outcomes after cardiac surgery. Attention is given to the various diagnostic modalities, including fetal and postnatal echocardiography, recent tomographic and magnetic resonance imaging techniques, and the time-honoured approach using angiography.
Temporary pacing wires have been associated with serious postoperative complications. Recommendations for their routine use after open heart surgery are decades old, and may not reflect current surgical techniques and outcomes.
The electronic web-enabled medical records of all patients undergoing congenital cardiac surgery from February, 2002, through December, 2005, were reviewed, excluding patients undergoing implantation of pacemakers as a primary procedure, or those undergoing ligation of a patent arterial duct.
There were 1193 surgical procedures performed, 1041 with cardiopulmonary bypass. Median age of the patients was 5.8 months, with a range from 0 days to 54 years, weighing 6.2 kilograms, with a range from 1 to 114 kilograms. Mortality prior to discharge was 2.5%, and median postoperative stay was 6 days. No deaths were attributed to arrhythmias. Temporary pacing wires were placed 14 times (1.2%). Indications for placement included sinus nodal dysfunction in 8 patients, preoperative in 4 and intraoperative in 4, high degree atrioventricular block in 4 patients, and intraoperative atrial flutter in 2 patients. Of these patients, 4 (0.3%) eventually underwent permanent implantation of a pacemaker, 2 for persistent sinus nodal dysfunction, and 2 for persistent atrioventricular block. Postoperative junctional ectopic tachycardia requiring antiarrhythmic therapy occurred in 9 patients (0.8%). All recovered without incident, and none were treated with temporary pacing.
The diminished risk of unexpected postoperative arrhythmias in the current era alleviates the necessity for routine placement of temporary pacing wires. Those institutions with experienced surgical and cardiac critical care teams may be able to predict the need for temporary pacing wires preoperatively or intraoperatively.
The Helex Septal Occluder is a new device used to close atrial septal defects via interventional catheterization. In order to study the role of echocardiography during its use, and to describe the morphologic variants of defects suitable for closure with this occluder, we evaluated all patients undergoing intended closure of an atrial septal defect with the Helex occluder. A combination of transthoracic, transesophageal, three-dimensional, and intracardiac echocardiography were used before, during, and after the procedure to characterize anatomy, assess candidacy for closure, guide the device during its deployment, and evaluate results. Among the 60 candidates included in the study, 11 were excluded because of transesophageal echocardiographic and/or catheterization data obtained in the laboratory. Attempts at closure were successful in 46 patients, and unsuccessful in 3. We successfully treated four types of defects. These were defects positioned centrally within the oval fossa with appreciable rims along the entire circumference of the defect, defects with deficient or absent segments of the rim, defects with aneurysm of the primary atrial septum, and defects with multiple fenestrations. Follow-up transthoracic echocardiograms taken at a median of 7 months demonstrated no residual defects in 21, trivial residual defects in 17, and small residual defects in 8 patients. In 20 patients, three-dimensional reconstructions were used to characterize the morphology of the defect and the position of the device. Because transesophageal echocardiography was often limited by acoustic interference from the device, intracardiac echocardiography was utilized in 3 cases to overcome this limitation.
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