RESUMO
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.
Assuntos
Cardiopatias Congênitas , Classificação Internacional de Doenças , Criança , Feminino , Humanos , Sistema de Registros , Sociedades Médicas , Organização Mundial da SaúdeRESUMO
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.
Assuntos
Cardiopatias Congênitas , Classificação Internacional de Doenças , Criança , Feminino , Humanos , Sistema de Registros , Sociedades MédicasRESUMO
Large carnivores are currently disappearing from many world regions because of habitat loss, prey depletion, and persecution. Ensuring large carnivore persistence requires safeguarding and sometimes facilitating the expansion of their populations. Understanding which conservation strategies, such as reducing persecution or restoring prey, are most effective to help carnivores to reclaim their former ranges is therefore important. Here, we systematically explored such alternative strategies for the endangered Persian leopard (Panthera pardus saxicolor) in the Caucasus. We combined a rule-based habitat suitability map and a spatially explicit leopard population model to identify potential leopard subpopulations (i.e., breeding patches), and to test the effect of different levels of persecution reduction and prey restoration on leopard population viability across the entire Caucasus ecoregion and northern Iran (about 737,000 km2 ). We identified substantial areas of potentially suitable leopard habitat (~120,000 km2 ), most of which is currently unoccupied. Our model revealed that leopards could potentially recolonize these patches and increase to a population of >1,000 individuals in 100 yr, but only in scenarios of medium to high persecution reduction and prey restoration. Overall, reducing persecution had a more pronounced effect on leopard metapopulation viability than prey restoration: Without conservation strategies to reduce persecution, leopards went extinct from the Caucasus in all scenarios tested. Our study highlights the importance of persecution reduction in small populations, which should hence be prioritized when resources for conservation are limited. We show how individual-based, spatially explicit metapopulation models can help in quantifying the recolonization potential of large carnivores in unoccupied habitat, designing adequate conservation strategies to foster such recolonizations, and anticipating the long-term prospects of carnivore populations under alternative scenarios. Our study also outlines how data scarcity, which is typical for threatened range-expanding species, can be overcome with a rule-based habitat map. For Persian leopards, our projections clearly suggest that there is a large potential for a viable metapopulation in the Caucasus, but only if major conservation actions are taken towards reducing persecution and restoring prey.
Assuntos
Conservação dos Recursos Naturais , Panthera , Animais , Ecossistema , HumanosRESUMO
Coronary ostial atresia seen with pulmonary atresia and coronary-cameral fistulae or, more rarely, in isolation manifested as left main coronary artery atresia, is well described. We describe the clinical course and post-mortem findings in a neonate who suffered a fatal cardiac arrest and was found to have congenital absence of both coronary ostia in a single/common coronary system.
Assuntos
Anomalias dos Vasos Coronários/diagnóstico , Parada Cardíaca/etiologia , Anomalias dos Vasos Coronários/complicações , Evolução Fatal , Parada Cardíaca/diagnóstico por imagem , Parada Cardíaca/patologia , Humanos , Recém-NascidoRESUMO
We report a case of interrupted aortic arch type C with aortic atresia and a ventricular septal defect with two well-developed ventricles, who underwent a successful single-stage biventricular repair with the modified Yasui procedure and arch reconstruction. Angiography done during conduit revision showed bilateral brachiocephalic trunks with high branching. The child is doing well six years after the initial operation.
Assuntos
Aorta/anormalidades , Coartação Aórtica/cirurgia , Comunicação Interventricular/cirurgia , Ventrículos do Coração/cirurgia , Anormalidades Múltiplas/cirurgia , Aorta/cirurgia , Angiografia Coronária , Humanos , Recém-Nascido , Masculino , Estudos RetrospectivosRESUMO
The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.
Assuntos
Cardiopatias Congênitas/classificação , Comunicação Interventricular/classificação , Melhoria de Qualidade , Terminologia como Assunto , Pré-Escolar , Consenso , Feminino , Cardiopatias Congênitas/cirurgia , Comunicação Interventricular/cirurgia , Humanos , Lactente , Recém-Nascido , Classificação Internacional de Doenças , Masculino , Pediatria , Sociedades MédicasRESUMO
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.
Assuntos
Cardiopatias Congênitas/classificação , Classificação Internacional de Doenças/história , Pediatria , Sociedades Médicas/normas , Terminologia como Assunto , Cardiopatias Congênitas/diagnóstico , História do Século XIX , História do Século XX , História do Século XXI , Humanos , Organização Mundial da SaúdeAssuntos
Cardiologia/normas , Registros Eletrônicos de Saúde/normas , Controle de Formulários e Registros/normas , Cardiopatias Congênitas , Pediatria/normas , Consenso , Confiabilidade dos Dados , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Humanos , Terminologia como AssuntoRESUMO
We report the case of a neonate with pulmonary atresia with intact ventricular septum and coronary cameral fistulae despite having a subsystemic right ventricle. We review the literature on coronary cameral fistulae in this disease and right ventricle-dependent coronary circulation. We discuss the potential consequences of this physiology, including risk of adverse cardiovascular events that may impact risk stratification and surgical palliation.
Assuntos
Seio Coronário/cirurgia , Fístula/cirurgia , Cardiopatias Congênitas/diagnóstico por imagem , Ventrículos do Coração/anormalidades , Atresia Pulmonar/diagnóstico por imagem , Septo Interventricular/cirurgia , Angiografia Coronária , Ecocardiografia , Humanos , Recém-Nascido , MasculinoRESUMO
BACKGROUND: Anomalous origin of a coronary artery from the contralateral sinus of Valsalva is associated with exercise-induced ischemia and sudden death. That is thought to be due to aortic enlargement in patients with an elliptical ostium. We hypothesize that virtual angioscopy can identify abnormal coronary ostial morphology in these patients. METHODS: We retrospectively analyzed 55 consecutive pediatric coronary artery magnetic resonance imaging studies from January 2006 to January 2010 with the diagnosis of anomalous right (n = 20), or left (n = 7) coronary artery, or normal coronary origins (n = 28). One postmortem heart specimen with anomalous left coronary artery was imaged and analyzed to validate our technique. Virtual angioscopy analysis was used for visualization and measurement of the coronary ostia. RESULTS: Distinct aortic origins of the right and left coronaries were seen in all 55 studies. An elliptical orifice with a longer superior-inferior dimension was seen in all anomalous ostia, in contrast to a circular ostium in all normal origins. That was quantified in anomalous ostia with a long-axis to short-axis ratio of 2.5 ± 0.5 (right) and 2.4 ± 0.5 (left) compared with 1.1 ± 0.2 (right) and 1.0 ± 0.3 (left) in controls (p < 0.001 for right and left ostia comparisons). Ostial morphology was confirmed in all 9 patients who underwent operative repair and in 1 patient at autopsy. CONCLUSIONS: Virtual angioscopy identifies abnormal ostial morphology in anomalous coronary artery patients, which is important for characterizing the diagnosis of patients who may be at risk for sudden death.
Assuntos
Anormalidades Múltiplas/patologia , Angioscopia , Aorta Torácica/anormalidades , Aorta Torácica/patologia , Anomalias dos Vasos Coronários/complicações , Anomalias dos Vasos Coronários/patologia , Adolescente , Criança , Feminino , Humanos , Masculino , Estudos RetrospectivosAssuntos
Cardiologia/educação , Diagnóstico por Imagem , Técnicas de Diagnóstico Cardiovascular , Bolsas de Estudo/normas , Pediatria/educação , Adolescente , Criança , Pré-Escolar , Competência Clínica , Educação Baseada em Competências/normas , Currículo/normas , Ecocardiografia/métodos , Avaliação Educacional , Coração Fetal/diagnóstico por imagem , Objetivos , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Ensino , Ultrassonografia Pré-NatalAssuntos
Comitês Consultivos , Técnicas de Imagem Cardíaca/métodos , Cardiologia/educação , Internato e Residência/métodos , Pediatria/educação , Sociedades Médicas , Comitês Consultivos/normas , Técnicas de Imagem Cardíaca/normas , Cardiologia/normas , Competência Clínica/normas , Humanos , Internato e Residência/normas , Pediatria/normas , Sociedades Médicas/normasRESUMO
We describe management of a patient with a prenatal diagnosis of absent pulmonary valve, tricuspid atresia, ventricular septal defect, and congenital heart block. Initial treatment consisted of temporary pacemaker implantation, and subsequent palliation included a central shunt during the neonatal period and placement of a permanent pacemaker. At seven months of age, a bidirectional Glenn anastomosis was performed. Cardiac catheterization revealed high cavopulmonary pressures and ventricular dysfunction precluding Fontan completion. Heart transplantation was performed at 3.75 years of age. The patient is alive and well 26 months posttransplantation.
Assuntos
Anormalidades Múltiplas/cirurgia , Bloqueio Cardíaco/congênito , Comunicação Interventricular/cirurgia , Valva Pulmonar/anormalidades , Atresia Tricúspide/cirurgia , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/terapia , Cateterismo Cardíaco , Pré-Escolar , Terapia Combinada , Feminino , Técnica de Fontan , Bloqueio Cardíaco/diagnóstico , Bloqueio Cardíaco/cirurgia , Bloqueio Cardíaco/terapia , Comunicação Interventricular/diagnóstico , Comunicação Interventricular/terapia , Transplante de Coração , Humanos , Lactente , Recém-Nascido , Marca-Passo Artificial , Gravidez , Diagnóstico Pré-Natal , Valva Pulmonar/cirurgia , Atresia Tricúspide/diagnóstico , Atresia Tricúspide/terapiaRESUMO
OBJECTIVE: Anomalous left coronary artery from the inappropriate aortic sinus with intraseptal course is generally benign but can be confused on imaging studies with the potentially lethal interarterial, intramural anomalous left coronary artery. The purpose of this study was to assess normal ostial morphologic features and intraseptal course using cardiac MRI and CT in pediatric patients with intraseptal anomalous left coronary artery. MATERIALS AND METHODS: A retrospective review was conducted of the medical records of 14 children with the diagnosis of intraseptal anomalous left coronary artery between November 2009 and March 2013. Coronary artery origin and course were evaluated with cardiac MRI or CT, and 3D assessment of coronary ostial morphologic features was performed with virtual angioscopy. RESULTS: The patient ages ranged from 5 to 18 years at diagnosis; 10 (71.4%) were boys. The right and left coronary origins were the right sinus of Valsalva as a common origin (n = 9) or a single coronary artery (n = 5). Anomalous intraseptal left main coronary was found in 13 patients, and one patient had anomalous left anterior descending with retroaortic circumflex coronary artery. Anomalous coronary ostia were round and without stenosis in all studies. The anomalous vessel was identified with echocardiography, but the anomalous left coronary artery was not delineated, and a normal ostium was not adequately portrayed in any instance. CONCLUSION: By use of cardiac MRI and CT, the anomalous course of round coronary ostia was confirmed and visualized in a pediatric cohort with intraseptal anomalous left coronary artery. The data provide the basis for understanding the benign clinical course and showing that surgery is unnecessary for this coronary anomaly.
Assuntos
Angiografia Coronária/métodos , Anomalias dos Vasos Coronários/diagnóstico , Vasos Coronários/patologia , Angiografia por Ressonância Magnética/métodos , Tomografia Computadorizada por Raios X/métodos , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Sensibilidade e EspecificidadeRESUMO
Hypoplastic left ventricle with scimitar syndrome is a rare combination of anatomic lesions. Five patients with this anatomy have been followed up at our institution. Four of these patients are alive: 2 after heart transplant, 1 underwent a Norwood operation, and 1 has survived staged palliation, culminating in a Fontan operation.
Assuntos
Anormalidades Múltiplas , Técnica de Fontan/métodos , Transplante de Coração/métodos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/métodos , Síndrome de Cimitarra/cirurgia , Pré-Escolar , Feminino , Seguimentos , Humanos , Recém-Nascido , Masculino , Cuidados Paliativos/métodosRESUMO
This study aimed to identify the anatomic and pathologic structural cardiac abnormalities in conjoined twins and to focus on those that have prevented the successful separation of conjoined hearts. A retrospective review was undertaken to examine consecutive cases of thoracopagus conjoined twins with conjoined hearts evaluated at The Children's Hospital of Philadelphia from 1 January 1980 through 6 October 2008. The records included autopsy and surgical findings as well as clinical reports. The study group included nine sets of conjoined twins with a mean gestational age at birth of 33.8 ± 5.5 weeks. Three twin pairs were stillborn. Five twin pairs died afterward. One pair died of cardiopulmonary failure. The median age at death was 22 days (range, 0-345 days). Major congenital heart disease was present in 94.4% (17/18) of the hearts, and 72.2% (13/18) of the hearts had single-ventricle physiology. Total anomalous pulmonary venous return occurred in 39% (7/18) of the cases. The clinical outcome for thoracopagus twins with conjoined hearts remains poor because of inability to separate conjoined and single ventricles. Surgical nonintervention and palliative care should be strongly considered for these patients.
Assuntos
Doenças em Gêmeos , Cardiopatias Congênitas/patologia , Tórax/anormalidades , Gêmeos Unidos/fisiopatologia , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Recém-Nascido , Estudos Retrospectivos , Gêmeos Unidos/patologiaRESUMO
BACKGROUND: Mitral valve dysplasia syndrome is a unique form of left-sided heart disease characterized by aortic outflow hypoplasia, dilated left ventricle, dysplastic/incompetent mitral valve, and a restrictive/intact atrial septum. Patients with this constellation of abnormalities have been managed in a variety of ways with overall poor outcomes. METHODS: We performed a retrospective review of all patients with mitral valve dysplasia syndrome to identify fetal echocardiographic markers predictive of outcomes. RESULTS: Mitral valve dysplasia syndrome was identified in 10 fetuses. Fetal left heart dilation and abnormal pulmonary venous flow were associated with increased mortality. Seven fetuses had abnormal pulmonary venous Doppler patterns; 3 had a unique "double-reversal" flow pattern. Severe fetal left heart dilation (left heart/right heart area ratio > 1.5) was present in 5. Prenatal intervention was performed on 3 fetuses: balloon aortic valvuloplasty (n = 2) and balloon atrial septostomy (n = 1). Of the 3, one died in utero and neither survivor underwent a 2-ventricle repair. Five patients required an immediate postnatal intervention to open the atrial septum. The overall mortality was 50%. CONCLUSIONS: Mitral valve dysplasia syndrome is a unique form of congenital heart disease with severe aortic stenosis but normal or enlarged left ventricle secondary to primary mitral valve disease. Increased left heart size and pulmonary vein Doppler patterns are predictive of postnatal outcome. Despite the presence of a dilated left ventricle, postnatal management with staged single ventricle palliation may be the most effective strategy.