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
Data are limited on outcomes associated with mitral valve surgery in infants. Prior studies report high mortality and increased risk for late cardiac failure particularly for those with mitral stenosis. We sought to evaluate outcomes in patients with mitral stenosis (MS) or regurgitation (MR) who had mitral valvuloplasty or replacement in the first year of life. A retrospective analysis of all patients in a single institution who underwent mitral valvuloplasty or replacement in their first year of life from 2004 to 2016 (n = 25), excluding patients with single ventricle pathology or those undergoing surgery for atrioventricular canal defect, was carried out. Median age and weight at surgery were 76.5 days (range 2-329) and 4.5 kg (range 3.0-10.1), respectively. The primary mitral pathology was MR in 16 and MS in 9 patients. Median follow-up among living patients was 4 years (range 106 days-12.3 years). Overall survival was 96% at 30 days and 87.8% at 1, 5, and 10 years. There were three early deaths (12%), all within 6 weeks of surgery. There were no late deaths. Three patients required valve replacement, 1 of which had a primary mitral valve replacement and died within 30 days of surgery. Re-intervention-free survival (surgical and catheter based) was 83.8%, 73.3%, and 48.9% at 1, 5, and 10 years per Kaplan-Meier estimates. There was no difference in re-intervention-free survival between patients with MR versus MS. No risk factors for death or re-intervention were identified. Mitral valvuloplasty and replacement can be performed in infants under 1 year of age with acceptable survival and need for re-intervention.
Assuntos
Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Insuficiência da Valva Mitral/cirurgia , Estenose da Valva Mitral/cirurgia , Procedimentos Cirúrgicos Cardíacos/mortalidade , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
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
The thymus, an organ responsible for T cell development, is one of the more stress-sensitive tissues in the body. Stress, in the form of infections, radiation exposure, and steroids, impairs thymic epithelial cell (TEC) functions and induces the programmed cell death of immature thymocytes. MicroRNAs are small noncoding RNAs involved in tissue repair and homeostasis, with several supporting T cell development. We report that miR-205, an epithelial-specific miR, maintains thymopoiesis following inflammatory perturbations. Thus, the activation of diverse pattern recognition receptors in mice causes a more severe thymic hypoplasia and delayed T cell recovery when miR-205 is conditionally ablated in TECs. Gene expression comparisons in the TECs with/without miR-205 revealed a significant differential regulation of chemokine/chemokine receptor pathways, antigen processing components, and changes in the Wnt signaling system. This was partly a consequence of reduced expression of the transcriptional regulator of epithelial cell function, Forkhead Box N1 (Foxn1), and its two regulated targets, stem cell factor and ccl25, following stress. miR-205 mimics supplemented into miR-205-deficient fetal thymic organ cultures restored Foxn1 expression along with ccl25 and stem cell factor A number of putative targets of miR-205 were up-regulated in TECs lacking miR-205, consistent with an important role for this miR in supporting T cell development in response to stress.
Assuntos
Diferenciação Celular , Quimiocinas CC/metabolismo , Fatores de Transcrição Forkhead/genética , MicroRNAs/metabolismo , Fator de Células-Tronco/metabolismo , Linfócitos T/citologia , Linfócitos T/metabolismo , Animais , Células Cultivadas , Quimiocinas CC/genética , Feminino , Fatores de Transcrição Forkhead/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , MicroRNAs/genética , Fator de Células-Tronco/genética , Timócitos/citologia , Timócitos/metabolismo , Timo/citologia , Timo/crescimento & desenvolvimento , Timo/metabolismoRESUMO
Despite substantial improvements in survival after pediatric heart transplantation, refractory rejection remains a major cause of morbidity and mortality. We have utilized ALE (Campath-1H) in six consecutive patients with refractory rejection. These rejection episodes persisted despite conventional treatment, which included intravenous methylprednisolone, rituximab, immunoglobulin G, and antithymocyte globulin. In our series, after ALE therapy, LV SF increased from 22%±5% to 33%±5% (P=.01). However, in our series, ALE therapy neither led to persistent LV function recovery nor could it prevent subsequent antibody-mediated rejection.
Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Rejeição de Enxerto/prevenção & controle , Transplante de Coração , Adolescente , Alemtuzumab , Anticorpos Monoclonais/administração & dosagem , Soro Antilinfocitário/administração & dosagem , Basiliximab , Criança , Pré-Escolar , Feminino , Humanos , Imunoglobulina G/administração & dosagem , Lactente , Infusões Intravenosas , Masculino , Metilprednisolona/administração & dosagem , Ácido Micofenólico/administração & dosagem , Prednisona/administração & dosagem , Proteínas Recombinantes de Fusão/administração & dosagem , Estudos Retrospectivos , Rituximab/administração & dosagem , Tacrolimo/administração & dosagem , TransplantadosRESUMO
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 SaúdeRESUMO
OBJECTIVE: This study's objective was to evaluate insertion techniques and device fit of the pediatric version of the Impella ventricular assist device in swine which had similar sized carotids and left ventricles (LVs) as children weighing 10-20 kg. BACKGROUND: Options for minimally invasive circulatory support in children are limited. A modified device based on the current Impella 2.5 platform was created in an effort to provide minimally invasive circulatory support for children. METHODS: Animal studies (n = 10) were performed to determine technical feasibility of device implant via the right common carotid artery (RCCA) in swine with a carotid and LV size similar to children with a BSA < 1 m2 and weight 10-20 kg. The RCCA diameter was measured on pre-implant ultrasound and the LV length was measured at necropsy. The animals were supported for 4 hr and the device explanted. Blood samples and post-explant necropsy was performed to evaluate for device related complications. RESULTS: All animals underwent successful device insertion. Mean carotid artery diameter by ultrasound was 3.5 ± 0.3 mm. There was no LV or aortic/mitral valve damage with a minimum LV length of 5.4 cm. CONCLUSIONS: Minimally invasive circulatory support is needed in small children. Limitations are primarily related to vessel and chamber size. The Impella Pediatric catheter was safely and successfully implanted in carotid arteries similar in size to children weighing 10-20 kg with minimal complications. © 2016 Wiley Periodicals, Inc.
Assuntos
Ventrículos do Coração/anatomia & histologia , Coração Auxiliar , Implantação de Prótese/instrumentação , Função Ventricular Esquerda , Animais , Superfície Corporal , Peso Corporal , Artéria Carótida Primitiva/anatomia & histologia , Artéria Carótida Primitiva/diagnóstico por imagem , Remoção de Dispositivo , Estudos de Viabilidade , Teste de Materiais , Miniaturização , Modelos Animais , Desenho de Prótese , Implantação de Prótese/efeitos adversos , Suínos , Fatores de TempoRESUMO
The HeartWare HVAD has been used as a bridge to cardiac transplantation in the pediatric population. We describe outcomes following HeartWare HVAD implantation at a single center. A retrospective chart review was performed of all HeartWare HVAD implants performed at our institution between May 2013 and March 2015. Eight children between the ages of 9 and 17 years underwent HVAD implantation as a bridge to transplant (N = 7 cardiomyopathy, N = 1 complex single ventricle). There was one operative death in the complex single ventricle patient. Seven patients (87%) were successfully bridged to transplant. Median time of support was 24.5 days (range, 6-91 days). All transplanted patients are alive and well at a median follow-up of 448 days. Our results demonstrated that mechanical support with HeartWare HVAD is feasible in patients of varying sizes (from older children to adolescents).
Assuntos
Insuficiência Cardíaca/terapia , Transplante de Coração , Coração Auxiliar , Função Ventricular Esquerda , Adolescente , Fatores Etários , Criança , Estudos de Viabilidade , Feminino , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Desenho de Prótese , Recuperação de Função Fisiológica , Estudos Retrospectivos , Texas , Fatores de Tempo , Resultado do TratamentoRESUMO
Cardiac rhabdomyomas in patients with tuberous sclerosis complex are usually detected antenatally or during infancy, with subsequent stabilisation or spontaneous regression. Development of a new cardiac rhabdomyoma beyond infancy is very rare. We report a male child who needed resection of a large rhabdomyoma in neonatal life, and then developed a new-onset rhabdomyoma at 2 years of age in a different location, needing another resection. Routine surveillance for cardiac rhabdomyomas in asymptomatic patients with tuberous sclerosis is essential.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Neoplasias Cardíacas/diagnóstico por imagem , Rabdomioma/diagnóstico por imagem , Esclerose Tuberosa/complicações , Ecocardiografia , Seguimentos , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/cirurgia , Humanos , Recém-Nascido , Masculino , Rabdomioma/complicações , Rabdomioma/cirurgiaRESUMO
Although nesiritide has been used in adults with left heart failure, the experience in the paediatric population is limited. We reviewed and analysed our experience with continuous nesiritide infusion as adjunct therapy in children with biventricular dysfunction due to diverse aetiologies and suffering from oliguria despite intravenous diuretics and inotropic therapies for heart-failure management.
Assuntos
Insuficiência Cardíaca/tratamento farmacológico , Natriuréticos/uso terapêutico , Peptídeo Natriurético Encefálico/uso terapêutico , Disfunção Ventricular/tratamento farmacológico , Adolescente , Criança , Estado Terminal , Insuficiência Cardíaca/complicações , Humanos , Lactente , Masculino , Oligúria/etiologia , Disfunção Ventricular/etiologiaRESUMO
Left atrial (LA) decompression in infants with thick atrial septum placed on veno-arterial extracorporeal membrane oxygenation (ECMO) can be reliably achieved with transcatheter atrial septal (AS) stent placement. However, with myocardial recovery and ECMO decannulation the stent causes a permanent left to right shunt and surgical removal of the AS stent is usually accomplished. Transcatheter removal of AS stent is an attractive option as the advantages of LA decompression would then be coupled with the advantage of avoiding a sternotomy for surgical removal of the stent. To our knowledge, this is the first reported case of successful transcatheter retrieval of AS stent used for LA decompression in a 13-month-old child.
Assuntos
Cateterismo Cardíaco/métodos , Oxigenação por Membrana Extracorpórea/métodos , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/terapia , Stents , Disfunção Ventricular Esquerda/terapia , Descompressão Cirúrgica/métodos , Remoção de Dispositivo/métodos , Serviço Hospitalar de Emergência , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido Prematuro , Medição de Risco , Resultado do Tratamento , Ultrassonografia , Disfunção Ventricular Esquerda/diagnóstico por imagemRESUMO
BACKGROUND: Recent data suggest that the Berlin Heart EXCOR Pediatric ventricular assist device is superior to extracorporeal membrane oxygenation for bridge to heart transplantation. Published data are limited to 1 in 4 children who received the device as part of the US clinical trial. We analyzed outcomes for all US children who received the EXCOR to characterize device outcomes in an unselected cohort and to identify risk factors for mortality to facilitate patient selection. METHODS AND RESULTS: This multicenter, prospective cohort study involved all children implanted with the Berlin Heart EXCOR Pediatric ventricular assist device at 47 centers from May 2007 through December 2010. Multiphase nonproportional hazards modeling was used to identify risk factors for early (<2 months) and late mortality. Of 204 children supported with the EXCOR, the median duration of support was 40 days (range, 1-435 days). Survival at 12 months was 75%, including 64% who reached transplantation, 6% who recovered, and 5% who were alive on the device. Multivariable analysis identified lower weight, biventricular assist device support, and elevated bilirubin as risk factors for early mortality and bilirubin extremes and renal dysfunction as risk factors for late mortality. Neurological dysfunction occurred in 29% and was the leading cause of death. CONCLUSIONS: Use of the Berlin Heart EXCOR has risen dramatically over the past decade. The EXCOR has emerged as a new treatment standard in the United States for pediatric bridge to transplantation. Three-quarters of children survived to transplantation or recovery; an important fraction experienced neurological dysfunction. Smaller patient size, renal dysfunction, hepatic dysfunction, and biventricular assist device use were associated with mortality, whereas extracorporeal membrane oxygenation before implantation and congenital heart disease were not.
Assuntos
Transplante de Coração , Coração Auxiliar , Tamanho Corporal , Causas de Morte , Criança , Pré-Escolar , Comorbidade , Ensaios de Uso Compassivo , Desenho de Equipamento , Oxigenação por Membrana Extracorpórea/estatística & dados numéricos , Feminino , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/cirurgia , Cardiopatias/sangue , Cardiopatias/cirurgia , Transplante de Coração/estatística & dados numéricos , Hemorragia/epidemiologia , Humanos , Hiperbilirrubinemia/epidemiologia , Lactente , Nefropatias/epidemiologia , Hepatopatias/epidemiologia , Masculino , Mortalidade , Insuficiência de Múltiplos Órgãos/epidemiologia , Modelos de Riscos Proporcionais , Risco , Acidente Vascular Cerebral/epidemiologia , Taxa de Sobrevida , Resultado do Tratamento , Listas de EsperaRESUMO
Percutaneous circulatory support is widely available for use in adult patients. Options remain limited in the pediatric population due to limitations of vessel and patient size, resulting in the use of more invasive means of circulatory support. We present a case series of pediatric patients in whom the Impella 2.5 axial flow pump was able to be placed utilizing a hybrid approach that allowed for adequate distal perfusion while providing successful short-term hemodynamic support.
Assuntos
Insuficiência Cardíaca/terapia , Coração Auxiliar , Hemodinâmica , Função Ventricular Esquerda , Fatores Etários , Tamanho Corporal , Criança , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Desenho de Prótese , Resultado do TratamentoRESUMO
Hypoplastic left heart syndrome (HLHS) with intact (IAS) or highly restrictive atrial septum (RAS) has extremely poor outcomes largely related to pulmonary pathology. At birth, immediate left atrial (LA) decompression is required to remain viable, but there is a tradeoff between residual increase in LA pressure and pulmonary overcirculation, either of which exacerbates the pulmonary status. From August 2010 to April 2013, a retrospective chart review was performed on consecutive patients with a prenatal diagnosis of HLHS with IAS/RAS presenting to a single center. The management strategy was immediate LA decompression followed by placement of bilateral pulmonary artery bands (bPAB) and subsequent conventional Norwood procedure. Six patients were born with HLHS with IAS/RAS during this time period with this planned management strategy. Four patients underwent LA decompression and subsequently developed low cardiac output with pulmonary overcirculation. bPAB were used with improvement in cardiac output and pronounced diuresis. These patients all survived the Norwood and subsequent Glenn procedures and remain alive [median follow-up 2.2 years (range 11 months-2.7 years)]. Two patients did not survive with therapy being withdrawn before the Norwood procedure. It is hypothesized that a strategy of total LA decompression followed by bPAB maximizes preoperative systemic perfusion and minimizes ongoing injury to the pulmonary system. This may enhance patient candidacy for the Norwood procedure and long-term survival.
Assuntos
Átrios do Coração/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Artéria Pulmonar/cirurgia , Septo Interatrial , Peso ao Nascer , Débito Cardíaco , Descompressão Cirúrgica , Feminino , Técnica de Fontan , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Procedimentos de Norwood , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
The PHTS was founded in 1991 as a not-for-profit organization dedicated to the advancement of the science and treatment of children during listing for and following heart transplantation. Now, 21 yr later, the PHTS has contributed significantly to the field, most notably in the form of outcomes analyses and risk factor assessment, in addition to amassing the most detailed dataset on pediatric heart transplant recipients worldwide. The purpose of this report is to review the last decade of pediatric patients listed for heart transplantation (January 1, 2000-December 31, 2009) and summarize the changes, trends, outcomes, and lessons learned.
Assuntos
Bases de Dados Factuais , Transplante de Coração/tendências , Sistema de Registros , Adolescente , Criança , Pré-Escolar , Feminino , Transplante de Coração/mortalidade , Transplante de Coração/estatística & dados numéricos , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Multicêntricos como Assunto , Avaliação de Resultados em Cuidados de Saúde , Complicações Pós-Operatórias , Doadores de Tecidos/estatística & dados numéricos , Estados Unidos , Listas de Espera/mortalidadeRESUMO
BACKGROUND: The Society of Thoracic Surgeons Workforce on Congenital Surgery performed a practice survey to analyze contemporary data. METHODS: An electronic survey was sent to congenital heart surgeons in North America. Details on demographics, training paradigm, clinical practice, and work satisfaction were queried, tabulated, and analyzed. RESULTS: Of 312 unique contacts, 201 (64.4%) responded. Of these, 178 (89%) were practicing. The median age was 52 years (interquartile range, 43, 59 years), and 157 (88%) were male. The number of female respondents increased from 12 (7%) in 2015 to 18 (11%) at present. Practice composition was predominantly mixed pediatric and adult (141; 79%), although 15 (8%) surgeons practiced exclusively pediatric surgery. Most surgeons (154; 87%) reported performing the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 4 and 5 procedures. One-fourth (42; 24%) reported performing fewer than 50 pediatric cases per year, and 18 (10%) stated that their primary role was as a surgical assistant. Individual surgeon case volume was most commonly 100 to 149 total cases (29%). Although one-half (91; 51%) reported their volume as being "just right," 74 (42%) reported that their case volume was "too small." Seventy-six (43%) reported too many surgeons in their region. Of the 201 practicing surgeons, 30 (14.9%) plan retirement in the next 5 years. Most described career satisfaction, with 102 (57%) being very satisfied and 48 (27%) somewhat satisfied. CONCLUSIONS: Although most congenital heart surgeons in North America are satisfied with their careers, more than 40% believe that their caseload is inadequate and that there are too many surgeons in their region. Further analysis is warranted regarding career dissatisfaction and diversity.
Assuntos
Cardiopatias Congênitas , Cirurgiões , Cirurgia Torácica , Procedimentos Cirúrgicos Torácicos , Adulto , Humanos , Masculino , Feminino , Criança , Pessoa de Meia-Idade , Inquéritos e Questionários , Cirurgia Torácica/educação , Cardiopatias Congênitas/cirurgiaRESUMO
Children with heart failure unresponsive to medical therapy are left with few options for survival. Ventricular assist devices (VADs) are life-saving options for such patients, allowing for bridge to transplantation or cardiac recovery. Retrospective review of cases from May 2006 to October 2010 was undertaken. Fourteen patients underwent implantation of VADs for refractory heart failure. Mean age was 9 years (range 1-17 years), and weight was 41 kg (range 9.7-71 kg). Indications for support: end-stage cardiomyopathy (n = 8), myocarditis (n = 3), univentricular failure (n = 2), and congenital heart disease/postcardiotomy (n = 1). Level of limitation at time of implant included critical cardiogenic shock in six (43%) and progressive decline in eight (57%). Extracorporeal membrane oxygenation was used as a bridge to VAD in five (36%) patients. Preimplant variables: 86% of patients requiring mechanical ventilation (mean 10.3 days), hyperbilirubinemia in 75%, and acute renal insufficiency in 79%. Device selection was systemic VAD in 11 (79%) and biventricular assist device in three (21%). Berlin Heart EXCOR was used in eight patients, while six patients received a Thoratec implantable VAD or paracorporeal VAD. Mean duration of support was 68 days (range 8-363 days). Overall survival was 79%. Ten patients (71%) were successfully bridged to transplantation, three (21%) died while on a device, one remains on support, and no patients were weaned from VAD. Children supported for single ventricle heart failure had a 50% survival with none currently bridged to transplantation. Complications included bleeding requiring reoperation in 21% (n = 3), stroke in 29% (n = 4), and driveline infections in 7% (n = 1). In two patients, a total of six pump exchanges were performed for thrombus formation. Survival for pediatric patients of all ages is excellent using current device technology with a majority of patients being successfully bridged to transplantation. Morbidity is acceptably low considering the severity of illness. Significant challenges exist with long-term extracorporeal support due to lack of donor availability and the high incidence of preformed alloantibodies especially in the failing single ventricle.
Assuntos
Insuficiência Cardíaca/cirurgia , Coração Auxiliar , Choque Cardiogênico/cirurgia , Adolescente , Criança , Pré-Escolar , Oxigenação por Membrana Extracorpórea , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Resultado do TratamentoRESUMO
There are minimal data regarding chronic management of single-ventricle ventricular assist device (VAD) patients. This study aims to describe our center's multidisciplinary team management of single-ventricle patients supported long term with the Berlin Heart EXCOR Pediatric VAD. Patient #1 was a 4-year-old with double-outlet right ventricle with aortic atresia, L-looped ventricles, and heart block who developed heart failure 1 year after Fontan. She initially required extracorporeal membrane oxygenation support and was transitioned to Berlin Heart systemic VAD. She was supported for 363 days (cardiac intensive care unit [CICU] 335 days, floor 28 days). The postoperative course was complicated by intermittent infection including methicillin-resistant Staphylococcus aureus, intermittent hepatic and renal insufficiencies, and transient antithrombin, protein C, and protein S deficiencies resulting in multiple thrombi. She had a total of five pump changes over 10 months. Long-term medical management included anticoagulation with enoxaparin, platelet inhibition with aspirin and dipyridamole, and antibiotic prophylaxis using trimethoprim/sulfamethoxazole. She developed sepsis of unknown etiology and subsequently died from multiorgan failure. Patient #2 was a 4-year-old with hypoplastic left heart syndrome who developed heart failure 2 years after bidirectional Glenn shunt. At systemic VAD implantation, he was intubated with renal insufficiency. Post-VAD implantation, his renal insufficiency resolved, and he was successfully extubated to daytime nasal cannula and biphasic positive airway pressure at night. He was supported for 270 days (CICU 143 days, floor 127 days). The pump was upsized to a 50-mL pump in May 2011 for increased central venous pressures (29 mm Hg). Long-term medical management included anticoagulation with warfarin and single-agent platelet inhibition using dipyridamole due to aspirin resistance. He developed increased work of breathing requiring intubation, significant anasarca, and bleeding from the endotracheal tube. The family elected to withdraw support. Although both patients died prior to heart transplantation, a consistent specialized multidisciplinary team approach to the medical care of our VAD patients, consisting of cardiothoracic surgeons, heart transplant team, hematologists, pharmacists, infectious disease physicians, psychiatrists, specialty trained bedside nursing, and nurse practitioners, allowed us to manage these patients long term while awaiting heart transplantation.
Assuntos
Oxigenação por Membrana Extracorpórea , Insuficiência Cardíaca/cirurgia , Coração Auxiliar , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Complicações Pós-Operatórias/diagnóstico , Anticoagulantes/uso terapêutico , Berlim , Pré-Escolar , Oxigenação por Membrana Extracorpórea/efeitos adversos , Feminino , Técnica de Fontan , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/etiologia , Humanos , Masculino , Staphylococcus aureus Resistente à Meticilina/isolamento & purificação , Insuficiência de Múltiplos Órgãos/etiologia , Complicações Pós-Operatórias/microbiologia , Complicações Pós-Operatórias/terapia , Insuficiência Renal/terapia , Infecções Estafilocócicas/diagnóstico , Infecções Estafilocócicas/terapiaRESUMO
Congenital arteriovenous malformations are a well-described cause of neonatal heart failure. Fistulous connections are typically intrahepatic or intracranial. We present a case of a neonate with an intrathoracic arteriovenous malformation between the subclavian artery and superior vena cava resulting in florid neonatal heart failure. This unusual fistulous connection has only rarely been reported in the literature, and in those reports, it has not resulted in neonatal heart failure.
Assuntos
Malformações Arteriovenosas/complicações , Insuficiência Cardíaca/etiologia , Artéria Subclávia/anormalidades , Veia Cava Superior/anormalidades , Tronco Braquiocefálico , Humanos , Recém-Nascido , Imagem Cinética por Ressonância Magnética , Masculino , Ultrassonografia , Veia Cava Superior/diagnóstico por imagemRESUMO
Bioprosthetic aortic valve replacement (bAVR) in patients with congenital heart disease is challenging due to age, size and complexity. Our objective was to assess survival and identify predictors of re-operation. Data were retrospectively collected for 314 patients undergoing bAVR at 8 centers from 2000-2014. Kaplan-Meier estimation of time to re-operation and Cox regression were utilized. Average age was 45.2 years (IQR 17.8-71.1) and 30% were <21. Indications were stenosis (48%), regurgitation (28%) and mixed (18%). Twenty-eight (9%) underwent prior AVR. Median valve size was 23mm (IQR 21, 25). Implanted valves included CE (Carpentier-Edwards) Perimount (47%), CE Magna/Magna Ease (29%), Sorin Mitroflow (9%), St Jude (2%) and other (13%). Median follow-up was 2.9 (IQR 1.2, 5.7) years. Overall, 11% required re-operation, 35% of whom had a Mitroflow and 65% were <21 years old. Time to re-operation varied among valve type (p=0.020). Crude 3-year rate was 20% in patients ≤21. Smaller valve size indexed to BSA was associated with re-operation (21.7 vs. 23.5 mm/m2). Predictors of reintervention by multivariable analysis were younger age (29% increase in hazard per 5-year decrease, p<0.001), Mitroflow (HR=4 to 8 versus other valves), and smaller valve size (20% increase in hazard per 1 mm decrease, p=0.002). The overall 1, 3 and 5-year survival rates were 94%, 90% and 85% without differences by valve (p=0.19). A concerning reduction in 5-year survival after bAVR is shown. Re-operation is common and varies by age and valve type. Further research is needed to guide valve choice and improve survival.