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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.
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.
In critically ill children, multi-organ-system disease can influence the choice of antiarrhythmic medication. Intravenous therapy is often necessary. There is a scarcity of paediatric critical-care cases demonstrating the dosing, monitoring, and efficacy of intravenous sotalol. This case demonstrates the effective use of intravenous sotalol in an adolescent with renal, hepatic, and haematological dysfunctions.
Adults with high premature ventricular contraction burden can develop left ventricular dilation, dysfunction, and strain, consistent with a cardiomyopathy, which is reversible with radiofrequency ablation of the premature ventricular contractions. Evidence in children with similar ectopy burden is limited. We performed a single-centre retrospective review to examine the prevalence of premature ventricular contraction-induced cardiomyopathy, natural history of ventricular ectopy, and progression to ventricular tachycardia in children with frequent premature ventricular contractions.
Children aged between 6 months and 18 years, with premature ventricular contractions comprising at least 20% of rhythm on 24-hour Holter monitor, were included in our study. Those with significant structural heart disease, ventricular tachycardia greater than 1% of rhythm at the time of premature ventricular contraction diagnosis, or family history of cardiomyopathy – except tachycardia-induced – were excluded. Cardiomyopathy was defined by echocardiographic assessment.
A total of 36 children met the study criteria; seven patients (19.4%, 95% CI 6.2–32.6%) met the criteria for cardiomyopathy, mostly at initial presentation. Ectopy decreased to <10% of beats without intervention in 16.7% (95% CI 4.3–29.1%) of the patients. No patient progressed to having ventricular tachycardia as more than 1% of beats on follow-up Holter. Radiofrequency ablation was performed in three patients without cardiomyopathy.
Our study demonstrates a higher prevalence of cardiomyopathy among children with high premature ventricular contraction burden than that previously shown. Ectopy tended to persist throughout follow-up. These trends suggest the need for a multi-centre study on frequent premature ventricular contractions in children. In the interim, regular follow-up with imaging to evaluate for cardiomyopathy is warranted.
The Florida Children’s Medical Services (CMS) has a long-standing history of ensuring that providers of multiple paediatric subspecialties abide by the highest standards. The cardiac sub-committee has written quality standard documents that participating programmes must meet or exceed. These standards oversee paediatric cardiology services including surgery, catheterisations, and outpatient services. On April, 2012, the cardiac sub-committee decided to develop similar standards in paediatric electrophysiology. A task force was created and began this process. These standards include a catalogue of required and optional equipment, as well as staff and physician credentials. We sought to establish expectations of procedural numbers by practitioner and facility. The task force surveyed the members of the Pediatric and Congenital Electrophysiology Society. Finding no consensus, the task force is committed to generate the data by requiring that the CMS participating programmes enrol and submit data to the Multicenter Pediatric and Adult Congenital EP Quality (MAP-IT™) Initiative. This manuscript details the work of the Florida CMS Paediatric Electrophysiology Task Force.
QTc prolongation has been reported in adults following cardiopulmonary bypass; however, this phenomenon has not been studied in children with congenital cardiac disease. This study's aim was to formally assess QTc in children undergoing cardiac surgery.
Pre-operative and post-operative electrocardiograms during hospital stays were prospectively analysed on 107 consecutive patients under 18 years of age undergoing cardiac surgery. QTc was measured manually in leads II, V4, and V5. Measurements of 440 and 480 milliseconds were used to categorise patients. Peri-procedural data included bypass and cross-clamp time, medications, and electrolyte measurements. Outcome data included arrhythmias, length of mechanical ventilation, and hospital stay. Patients with post-operative new bundle branch block or ventricularly paced rhythm were excluded.
In all, 59 children were included, out of which 26 had new QTc over 440 milliseconds and 6 of 59 had new QTc over 480 milliseconds post-operatively. The mean increase in post-operative QTc was 25 milliseconds, p=0.0001. QTc over 480 was associated with longer cross-clamp time, p=0.003. Other risk factors were not associated with post-operative QTc prolongation. This phenomenon was transient with normalisation occurring in 67% of patients over 60 hours on average. One patient with post-operative QTc over 440 milliseconds developed ventricular tachycardia. There was no correlation between prolonged QTc and duration of mechanical ventilation, or hospital stay.
A significant number of children undergoing cardiac surgery showed transient QTc prolongation. The precise aetiology of QT prolongation was not discerned, though new QTc over 480 milliseconds was associated with longer cross-clamp time. In this cohort, transient QTc prolongation was not associated with adverse sequela.
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