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1.
Pediatr Transplant ; 25(3): e13990, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33666316

RESUMO

Right heart failure (RHF) is a vexing problem in children after left ventricular assist device (LVAD) implantation that can negatively impact transplant candidacy and survival. Anticipation, prevention, early identification and appropriate medical and device management of RHF are important to successful LVAD outcomes. However, there is limited pediatric evidence to guide practice. This pediatric-focused review summarizes the relevant literature and describes the harmonized approach to RHF from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION). This review seeks to improve RHF outcomes through the sharing of best practices and experience across the pediatric VAD community.


Assuntos
Insuficiência Cardíaca/cirurgia , Coração Auxiliar , Criança , Insuficiência Cardíaca/diagnóstico , Humanos
2.
Pediatr Cardiol ; 42(4): 934-941, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33585998

RESUMO

Increased circulating catecholamines are associated with worse exercise performance in adult heart failure patients. Patients with Fontan physiology have increased circulating catecholamines and theoretically could benefit from beta blockade. We hypothesized that carvedilol would improve exercise performance in Fontan patients. A double-blind, placebo-controlled, crossover trial of carvedilol was performed. Single ventricle patients between the ages of 10 and 35 years with a previous Fontan operation who were able to complete a maximal exercise test (respiratory exchange ratio > 1.0) were included. Two 12-week treatment arms were separated by a 6-week washout period. Exercise testing was performed at beginning and end of each treatment arm. Primary endpoint was improvement in peak oxygen consumption/kg (pVO2) from baseline. Of the 26 subjects enrolled, 23 completed the study. Four subjects did not reach goal maximum carvedilol dose, vs. 1 for placebo (p = 0.14). The mean change in pVO2 between treatments was not different (carvedilol = - 2.1 mL/kg/min v. placebo = - 1.42, p = 0.28). Carvedilol therapy decreased peak heart rate by 4.2 ± 20.2 bpm, (p < 0.01) leading to an increase in peak oxygen pulse (p < 0.01). Serum N-terminal-proBNP increased with carvedilol therapy (mean change of + 23.77 pg/mL) compared to placebo (mean change of - 5.37 pg/mL, p = 0.03). There were no serious adverse events related to study drug. Carvedilol was not associated with improved exercise performance and was associated with mildly increased N-terminal-proBNP. This study does not support the routine administration of carvedilol to healthy Fontan patients.Clinical Trials Registration ClinicalTrials.gov Identifier: NCT02946892. Registered October 27, 2016. Retrospectively Registered. https://clinicaltrials.gov/ct2/show/NCT02946892.


Assuntos
Antagonistas Adrenérgicos beta/uso terapêutico , Carvedilol/uso terapêutico , Exercício Físico , Técnica de Fontan/métodos , Insuficiência Cardíaca/tratamento farmacológico , Adolescente , Adulto , Criança , Estudos Cross-Over , Método Duplo-Cego , Teste de Esforço/métodos , Feminino , Insuficiência Cardíaca/cirurgia , Frequência Cardíaca , Humanos , Masculino , Peptídeo Natriurético Encefálico/análise , Consumo de Oxigênio , Fragmentos de Peptídeos/análise , Resultado do Tratamento , Adulto Jovem
3.
Pediatr Transplant ; 24(4): e13688, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32112495

RESUMO

Ischemic cardiomyopathy with resultant refractory HF may occur in patients with WBS, often as the result of coronary involvement with SVAS. The rapid development of arteriopathy at a young age raises concerns regarding transplant candidacy due to progressive stenoses at other arterial sites with potential detrimental impact on long-term heart graft function. We report a 2-month-old male infant diagnosed with mild aortic stenosis during the neonatal period, but subsequently developed rapidly progressive supravalvar and coronary artery stenoses leading to cardiogenic shock due to myocardial ischemia. The presentation led to the diagnosis of WBS. He required prolonged CPR including ECMO therapy. He subsequently underwent LVAD implantation as bridge to transplant and 4 days later heart transplantation. His post-operative course was complicated by prolonged mechanical ventilation and extended intensive care unit and hospital stays. However, at follow-up 18 months post-transplant he continues to have normal graft function with mild, non-progressive residual coarctation of aorta and non-progressive moderately hypoplastic pulmonary arteries.


Assuntos
Transplante de Coração , Isquemia Miocárdica/cirurgia , Pré-Escolar , Progressão da Doença , Humanos , Masculino , Isquemia Miocárdica/etiologia , Fatores de Tempo , Síndrome de Williams/complicações
4.
Pediatr Transplant ; 24(6): e13764, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32536034

RESUMO

Significant inter- and intra-center practice variability is present in pediatric donor heart acceptability. This may contribute to variation in the donor refusal rate and may impact waitlist time, morbidity, mortality, and transplant rates. In order to reduce practice variability, our center developed and implemented a comprehensive strategy regarding donor acceptance in September 2017. The aim of this study was to assess the impact of this strategy on waitlist time and outcomes as well as early post-transplant outcomes. We performed a single-center, retrospective analysis of all pediatric (<18 years) patients listed for single-organ heart transplant at our center from September 2015 to September 2018. Patients were divided into those listed before (Group 1) and after implementation of the comprehensive strategy (Group 2). The primary end-point was waitlist time. Secondary end-points included waitlist removal due to death or clinical deterioration, donor refusals per listed patient, early post-transplant outcomes (graft failure, mechanical ventilation time, inotropic support, length of hospital stay) and 1-year post-transplant survival. Of 78 listed patients, 54 were transplanted (29 in Group 1), 9 were removed due to death or clinical deterioration (7 in Group 1) and 15 were removed due to clinical improvement (12 in Group 1). The waitlist time was significantly shorter in Group 2 (17 days, IQR 7-53) vs Group 1 (90 days, IQR 14-162); P = .006. The number of donor refusals was lower in Group 2 (1, IQR 0-2.2) vs Group 1 (4, IQR 2-19); P < .001. The percentage of refused donors with normal function (Left ventricular ejection fraction > 50%) was lower in Group 2 vs Group 1 (53% vs 84%; P < .001). Difference in removal from the waitlist for death or deterioration in Group 2 vs Group 1 (n = 2, 7% vs n = 7, 20%, P = .18) did not reach statistical significance. There was no difference in post-transplant outcomes between groups. The waitlist time and donor refusals significantly decreased after implementation of a comprehensive donor acceptance strategy without impacting transplant outcomes. This analysis supports the need for a comprehensive approach to donor organ acceptance within a pediatric transplant center.


Assuntos
Insuficiência Cardíaca/cirurgia , Transplante de Coração/métodos , Tempo de Internação , Doadores de Tecidos , Listas de Espera , Criança , Pré-Escolar , Feminino , Sobrevivência de Enxerto , Humanos , Lactente , Masculino , Aceitação pelo Paciente de Cuidados de Saúde , Pediatria , Respiração Artificial , Estudos Retrospectivos , Volume Sistólico , Obtenção de Tecidos e Órgãos , Resultado do Tratamento , Função Ventricular Esquerda
5.
Pediatr Transplant ; 22(4): e13190, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29878688

RESUMO

This study aims to compare 2 common induction strategies, basiliximab and ATG. Analysis of the ISHLT transplant registry was performed. The database was queried for pediatric heart transplants from January 1, 2000, to June 30, 2015, who had received induction with basiliximab or ATG. Primary end-point was graft survival. Secondary end-points included 1-year survival and 1-year conditional survival. There were 3158 heart transplants who received induction with basiliximab or ATG. The ATG cohort was younger, more likely to have congenital heart disease or be a retransplant, have a higher PRA, longer ischemic time, and been transplanted earlier in the study period (all P<.01). There was no difference in graft loss in the basiliximab cohort compared to the ATG cohort (HR 1.18 P=.06). On conditional 1-year survival analysis, basiliximab induction was associated with graft loss (HR=1.35 95% CI 1.1-1.7, P<.01), and in the propensity-matched cohort, the basiliximab cohort was more likely to experience rejection prior to discharge (P=.04). Infection prior to discharge was more common in the antithymocyte cohort. Induction with ATG is associated with improved late graft survival compared to basiliximab.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Soro Antilinfocitário/uso terapêutico , Rejeição de Enxerto/prevenção & controle , Transplante de Coração , Imunossupressores/uso terapêutico , Quimioterapia de Indução/métodos , Proteínas Recombinantes de Fusão/uso terapêutico , Adolescente , Basiliximab , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Seguimentos , Sobrevivência de Enxerto , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento
7.
J Heart Lung Transplant ; 43(5): 787-796, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38199514

RESUMO

BACKGROUND: To date, no pediatric studies have highlighted the impact of center's ventricular assist device (VAD) volumes on post implant outcomes. METHODS: Children (age <19) enrolled in Pedimacs undergoing initial left ventricular assist device implantation from 2012 to 2020 were included. Center volume was analyzed as a continuous and categorical variable. For categorical analysis, center volumes were divided as: low volume (1-15 implants), medium volume (15-30 implants), and high volume (>30 implants) during our study period. Patient characteristics and outcomes were compared by center's VAD volumes. RESULTS: Of 44 centers, 16 (36.4%) were low, 11 (25%) were medium, and 17 (38.6%) were high-volume centers. Children at high-volume centers were least likely intubated, sedated, or paralyzed, and most likely ambulating preimplant (p < 0.05 for all). Center's VAD volumes were not a significant risk factor for mortality post implant when treated as a continuous or a categorical variable (p > 0.05). Compared to low volume, children at high-volume centers had fewer early neurological events. Compared to medium volume, those at high-volume centers had fewer late bleeding events (p < 0.05 for all). There were no significant differences in survival after an adverse event by hospital volumes (p > 0.05). CONCLUSIONS: Although hospital volume does not affect post-VAD implant mortality, pediatric centers with higher VAD volumes have fewer patients intubated, sedated, paralyzed pre implant, and have lower adverse events. Failure to rescue was not significantly different between low, medium, and high-volume VAD centers.


Assuntos
Insuficiência Cardíaca , Coração Auxiliar , Hospitais com Alto Volume de Atendimentos , Humanos , Masculino , Criança , Feminino , Pré-Escolar , Insuficiência Cardíaca/terapia , Insuficiência Cardíaca/cirurgia , Adolescente , Estudos Retrospectivos , Estados Unidos/epidemiologia , Resultado do Tratamento , Lactente , Hospitais com Baixo Volume de Atendimentos/estatística & dados numéricos , Bases de Dados Factuais , Taxa de Sobrevida/tendências
8.
Circ Heart Fail ; 16(2): e010123, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36786204

RESUMO

Individuals with Fontan circulation are at risk of late mortality from both cardiac and noncardiac causes. Despite the known risk of mortality, referral indications for advanced heart failure care vary between centers, and many individuals die from Fontan circulation-related complications either after late consideration for advanced heart failure therapies or having never seen a heart failure specialist. There is a critical need for guidelines to direct appropriately timed referral for advanced heart failure consultation. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Fontan Committee has developed recommended thresholds for advanced heart failure referral to guide primary cardiologists. These recommendations are divided into 4 categories of clinical Fontan circulatory dysfunction including (1) cardiac/systemic ventricular dysfunction, (2) Fontan pathway dysfunction, (3) lymphatic dysfunction, and (4) extracardiac dysfunction.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Insuficiência Cardíaca , Disfunção Ventricular , Humanos , Cardiopatias Congênitas/cirurgia , Disfunção Ventricular/complicações , Ventrículos do Coração
9.
J Heart Lung Transplant ; 42(2): 246-254, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36270923

RESUMO

PURPOSE: Cardiac disease results in significant morbidity and mortality in patients with muscular dystrophy (MD). Single centers have reported their ventricular assist device (VAD) experience in specific MDs and in limited numbers. This study sought to describe the outcomes associated with VAD therapy in an unselected population across multiple centers. METHODS: We examined outcomes of patients with MD and dilated cardiomyopathy implanted with a VAD at Advanced Cardiac Therapies Improving Outcomes Network (ACTION) centers from 9/2012 to 9/2020. RESULTS: A total of 19 VADs were implanted in 18 patients across 12 sites. The majority of patients had dystrophinopathy (66%) and the median age at implant was 17.2 years (range 11.7-29.5). Eleven patients were non-ambulatory (61%) and 6 (33%) were on respiratory support pre-VAD. Five (28%) patients were implanted as a bridge to transplant, 4 of whom survived to transplant. Of 13 patients implanted as bridge to decision or destination therapy, 77% were alive at 1 year and 69% at 2 years. The overall frequencies of positive outcome (transplanted or alive on device) at 1 year and 2 years were 84% and 78%, respectively. Two patients suffered a stroke, 2 developed sepsis, 1 required tracheostomy, and 1 experienced severe right heart failure requiring right-sided VAD. CONCLUSIONS: This study demonstrates the potential utility of VAD therapies in patients with muscular dystrophy. Further research is needed to further improve outcomes and better determine which patients may benefit most from VAD therapy in terms of survival and quality of life.


Assuntos
Insuficiência Cardíaca , Coração Auxiliar , Distrofias Musculares , Humanos , Criança , Adulto Jovem , Adolescente , Adulto , Resultado do Tratamento , Qualidade de Vida , Insuficiência Cardíaca/cirurgia , Distrofias Musculares/terapia , Sistema de Registros , Estudos Retrospectivos
10.
Am Heart J ; 163(4): 595-600, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22520525

RESUMO

BACKGROUND: Biomarkers have been proposed to augment or replace endomyocardial biopsy (EMB) to diagnose acute transplant rejection (AR). A new, highly sensitive assay for troponin T detects levels of cardiac troponin T (cTnT) 10- to 100-fold lower than standard assays but has not been investigated in transplant patients. N-terminal pro-brain natriuretic peptide (NT-proBNP) has not been evaluated in pediatric transplant patients. The purpose of this pilot study was to evaluate the association of cTnT and NT-proBNP with AR in pediatric cardiac transplant patients. METHODS: Plasma was obtained at the time of EMB from pediatric patients ≥ 1 year old. N-terminal pro-brain natriuretic peptide was measured in fresh plasma at the time of biopsy, and cTnT was measured from frozen, stored samples using the highly sensitive assay for troponin T. Biomarker data were correlated with EMB results. Cellular AR was defined as an International Society for Heart and Lung Transplantation biopsy score of grade ≥ 2R. RESULTS: Fifty-three blood samples were obtained from 42 patients (mean age 11 years). Seven episodes of AR occurred in 5 patients. Biopsies with vs without AR were associated with higher cTnT (median [interquartile range {IQR}] 66 [45-139] vs 7 [2-13] pg/mL, P = .001) and NT-proBNP (median [IQR] 11,169 [280-23,317] vs 334 [160-650] pg/mL, P < .01). After successful treatment of AR in 5 patients, cTnT fell markedly (median [IQR] 53.5 [44.8-66.5] to 10.7 [1.5-16.4], P = .05). CONCLUSION: In this pilot study, we found marked elevation of cTnT and NT-proBNP among children with AR. Moreover, reduction in cTnT levels after treatment paralleled improvement in EMB results. If these findings are confirmed in larger prospective studies, monitoring with these biomarkers may obviate surveillance EMB.


Assuntos
Rejeição de Enxerto/diagnóstico , Transplante de Coração/imunologia , Peptídeo Natriurético Encefálico/sangue , Fragmentos de Peptídeos/sangue , Troponina I/sangue , Criança , Feminino , Humanos , Imunoensaio/métodos , Masculino , Projetos Piloto , Curva ROC , Sensibilidade e Especificidade
11.
J Heart Lung Transplant ; 41(9): 1248-1257, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-36123003

RESUMO

BACKGROUND: Heart transplantation (HT) is the gold standard for managing end-stage heart failure. Multiple quality metrics, including length of stay (LOS), have been used in solid organ transplantation. However, limited data are available regarding trends and factors influencing LOS after pediatric HT. We hypothesized that various donor, peri-transplant and recipient factors affect LOS after pediatric HT. METHODS: We analyzed patients <18years at time of HT from January 2005 to December 2018 in the Pediatric Heart Transplant Society database, and examined LOS trends, defined prolonged LOS (PLOS = LOS>30days after HT), identified factors associated with PLOS and assessed outcomes. RESULTS: Of 4827 patients undergoing HT, 4414 patients were discharged and included for analysis. Overall median LOS was 19days[13,34]. Median LOS was longer in patients with congenital heart disease(CHD = 25days[15,43] than with cardiomyopathy(CM = 17days[12,27] across all ages. Median LOS in age <1year was 26-days[16,45.5] and in age >10year was 16days[11,26]. PLOS was seen in 1313 patients(30%). Patients with PLOS were younger, smaller and had longer CPB times. There was no difference in utilization of VAD at HT between groups, however, ECMO use at listing(8.45% vs 2.93%,p < 0.05) and HT was higher in the PLOS group(9.22% vs 1.58%,p < 0.05). PLOS was more common in patients with previous surgery, CHD, single ventricle physiology, recipient history of cardiac arrest or CPR, end organ dysfunction, lower GFR, use of mechanical ventilation at HT and Status 1A at HT. CONCLUSION: We present novel findings of LOS distribution and define PLOS after pediatric HT, providing a quality metric for individual programs to utilize and study in their practice.


Assuntos
Transplante de Coração , Criança , Hospitais , Humanos , Tempo de Internação , Modelos Logísticos , Aprendizado de Máquina , Estudos Retrospectivos
12.
Transplant Proc ; 53(8): 2435-2437, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34301402

RESUMO

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has become a highly prevalent infectious disease. Currently, organs are not being transplanted from donors who are SARS-CoV-2 positive. It remains unclear as to how to differentiate active from recovered patients. We report our recent experience of a 3-month-old deceased organ donor who died as the result of an anoxic brain injury after a cardiopulmonary arrest (presumed sudden infant death syndrome). The child was born to a mother presumed to have coronavirus disease 2019. The donor tested negative for SARS-CoV-2 reverse transcriptase-polymerase chain reaction and positive for SARS-CoV-2 immunoglobulin A antibodies. We suspect this is the first known report of its kind and noteworthy for the organ donation and transplantation community.


Assuntos
Anticorpos Antivirais/isolamento & purificação , COVID-19 , Doadores de Tecidos , COVID-19/diagnóstico , COVID-19/imunologia , Humanos , Lactente , Transplante de Órgãos , SARS-CoV-2/imunologia , Obtenção de Tecidos e Órgãos
13.
Ann Thorac Surg ; 112(3): e185-e188, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-33482162

RESUMO

Mechanical support of patients with superior cavopulmonary connection is challenging; multiple factors contribute to failure: elevated pulmonary vascular resistance, aortopulmonary collateral flow, venovenous collaterals, ventricular dysfunction, and atrioventricular valve regurgitation. We report 2 cases of conversion from a single ventricle circulation to biventricular mechanical support by reestablishing caval continuity. Both patients have demonstrated recovery of end-organ function and participation in rehabilitation. This method of support results in improved systemic venous pressures and pulmonary blood flow compared with systemic mechanical circulatory support with a cavopulmonary connection and transfers some of the complexity of the transplant to the ventricular assist device implant.


Assuntos
Técnica de Fontan , Insuficiência Cardíaca/terapia , Coração Auxiliar , Complicações Pós-Operatórias/terapia , Pré-Escolar , Feminino , Humanos , Lactente , Masculino
14.
Int Labour Rev ; 2021 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-34548681

RESUMO

Digital labour platforms have been widely promoted as a solution to the unemployment crisis sparked by the COVID-19 pandemic. However, the pandemic has also highlighted the harms to gig workers-who are exposed either to income loss, or to infection while carrying out essential work, but excluded from labour protections. We examine the COVID-19 policies of 191 platforms in 43 countries to understand how the crisis has shifted the conventions of the gig economy. Using a typology of "fair platform work" we report the introduction of some positive worker protections, but also significant shortfalls, including entrenchment of precarious work as platforms leverage the opportunities arising from the crisis.

15.
Circ Res ; 103(4): 388-95, 2008 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-18635823

RESUMO

The ductus arteriosus is a specialized blood vessel containing highly differentiated and contractile vascular smooth muscle, derived largely from neural crest cells, that is essential for fetal life but typically closes after birth. Impaired development of the ductus arteriosus or disruption of signaling pathways that initiate postnatal closure can result in persistent patency of the ductus arteriosus, the third most common congenital heart defect. We found that Tfap2beta, a transcription factor associated with patent ductus arteriosus in humans, was uniquely expressed in mouse ductal smooth muscle. Endothelin-1 and the hypoxia-induced transcription factor, Hif2alpha were also highly enriched in ductal smooth muscle at embryonic day 13.5 and were dependent on Tfap2beta for their expression in this domain. Hif2alpha functioned as a negative regulator of Tfap2beta-induced transcription by disrupting protein-DNA interactions, suggesting a negative feedback loop regulating Tfap2beta activity. Our data indicate that Tfap2beta, Et-1, and Hif2alpha act in a transcriptional network during ductal smooth muscle development and that disruption of this pathway may contribute to patent ductus arteriosus by affecting the development of smooth muscle within the ductus arteriosus.


Assuntos
Fatores de Transcrição Hélice-Alça-Hélice Básicos/metabolismo , Permeabilidade do Canal Arterial/metabolismo , Canal Arterial/metabolismo , Endotelina-1/metabolismo , Fator de Transcrição AP-2/metabolismo , Transcrição Gênica/fisiologia , Animais , Diferenciação Celular , Modelos Animais de Doenças , Canal Arterial/fisiologia , Permeabilidade do Canal Arterial/etiologia , Permeabilidade do Canal Arterial/fisiopatologia , Feto/metabolismo , Humanos , Camundongos , Camundongos Knockout , Músculo Liso Vascular/citologia , Músculo Liso Vascular/embriologia , Músculo Liso Vascular/metabolismo , Mutação , Transdução de Sinais/fisiologia , Fator de Transcrição AP-2/genética , Transfecção
16.
J Thorac Cardiovasc Surg ; 160(5): 1301-1308.e2, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-31948738

RESUMO

OBJECTIVE: Patients with biventricular assist devices (BiVADs) have worse outcomes than those with left ventricular assist devices (LVADs). It is unclear whether these outcomes are due to device selection or patient factors. We used propensity score matching to reduce patient heterogeneity and compare outcomes in pediatric patients supported with BiVADs with a similar LVAD cohort. METHODS: The Pedimacs registry was queried for patients who were supported with BiVAD or LVAD. Patients were analyzed by BiVAD or LVAD at primary implant and the 2 groups were compared before and after using propensity score matching. RESULTS: Of 363 patients who met inclusion criteria, 63 (17%) underwent primary BiVAD support. After propensity score matching, differences between cohorts were reduced. Six months after implant, in the BiVAD cohort (LVAD cohort) 52.5% (42.5%) had been transplanted; 32.5% (40%) were alive with device, and 15% (10%) had died. Survival was similar between cohorts (P = .31, log-rank), but patients with BiVADs were more likely to experience a major adverse event in the form of bleeding (P = .04, log-rank). At 1 week and 1 and 3 months' postimplant, the percentage of patients on mechanical ventilation, on dialysis, or with elevated bilirubin was similar between the 2 groups. CONCLUSIONS: When propensity scores were used to reduce differences in patient characteristics, there were no differences in survival but more major adverse events in the patients with BiVADs, particularly bleeding. Differences in unmatched patient outcomes between LVAD and BiVAD cohorts likely represent differences in severity of illness rather than mode of support.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Coração Auxiliar , Implantação de Prótese , Adolescente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/instrumentação , Procedimentos Cirúrgicos Cardíacos/mortalidade , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Coração Auxiliar/efeitos adversos , Coração Auxiliar/estatística & dados numéricos , Humanos , Masculino , Pontuação de Propensão , Implantação de Prótese/efeitos adversos , Implantação de Prótese/instrumentação , Implantação de Prótese/mortalidade
17.
ASAIO J ; 66(8): 939-945, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32740356

RESUMO

Thrombotic and bleeding complications have historically been major causes of morbidity and mortality in pediatric ventricular assist device (VAD) support. Standard anticoagulation with unfractionated heparin is fraught with problems related to its heterogeneous biochemical composition and unpredictable pharmacokinetics. We sought to describe the utilization and outcomes in children with paracorporeal VAD support who are treated with direct thrombin inhibitors (DTIs) antithrombosis therapy. Retrospective multicenter review of all pediatric patients (aged <19 years) treated with a DTI (bivalirudin or argatroban) on paracorporeal VAD support, examining bleeding and thrombotic adverse events. From May 2012 to 2018, 43 children (21 females) at 10 centers in North America, median age 9.5 months (0.1-215 months) weighing 8.6 kg (2.8-150 kg), were implanted with paracorporeal VADs and treated with a DTI. Diagnoses included cardiomyopathy 40% (n = 17), congenital heart disease 37% (n = 16; single ventricle n = 5), graft vasculopathy 9% (n = 4), and other 14% (n = 6). First device implanted included Berlin Heart EXCOR 49% (n = 21), paracorporeal continuous flow device 44% (n = 19), and combination of devices in 7% (n = 3). Adverse events on DTI therapy included; major bleeding in 16% (n = 7) (2.6 events per 1,000 patient days of support on DTI), and stroke 12% (n = 5) (1.7 events per 1,000 patient days of support on DTI). Overall survival to transplantation (n = 30) or explantation (n = 8) was 88%. This is the largest multicenter experience of DTI use for anticoagulation therapy in pediatric VAD support. Outcomes are encouraging with lower major bleeding and stroke event rate than that reported in literature using other anticoagulation agents in pediatric VAD support.


Assuntos
Antitrombinas/uso terapêutico , Coração Auxiliar/efeitos adversos , Resultado do Tratamento , Adolescente , Arginina/análogos & derivados , Arginina/uso terapêutico , Criança , Pré-Escolar , Feminino , Hemorragia/etiologia , Hemorragia/prevenção & controle , Hirudinas , Humanos , Lactente , Masculino , América do Norte , Fragmentos de Peptídeos/uso terapêutico , Ácidos Pipecólicos/uso terapêutico , Proteínas Recombinantes/uso terapêutico , Estudos Retrospectivos , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/prevenção & controle , Sulfonamidas/uso terapêutico , Trombose/etiologia , Trombose/prevenção & controle
18.
ASAIO J ; 66(4): 441-446, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32224822

RESUMO

Improving the outcomes of pediatric patients with congenital heart disease with end-stage heart failure depends on the collaboration of all stakeholders; this includes providers, patients and families, and industry representatives. Because of the rarity of this condition and the heterogeneity of heart failure etiologies that occur at pediatric centers, learnings must be shared between institutions and all disciplines to move the field forward. To foster collaboration, excel discovery, and bring data to the bedside, a new, collaborative quality improvement science network-ACTION (Advanced Cardiac Therapies Improving Outcomes Network)-was developed to meet the needs of the field. Existing gaps in care and the methods of improvement that will be used are described, along with the mission and vision, utility of real-world data for regulatory purposes, and the organizational structure of ACTION is described.


Assuntos
Atenção à Saúde/organização & administração , Colaboração Intersetorial , Aprendizagem , Melhoria de Qualidade , Criança , Humanos
19.
J Heart Lung Transplant ; 39(6): 573-579, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32111350

RESUMO

BACKGROUND: The HeartMate 3 ventricular assist device (VAD) is a newer centrifugal continuous-flow VAD used for bridge-to-transplant and destination therapy in adults. However, there is limited experience regarding its use in children and adults with complex congenital heart disease (CHD). METHODS: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a multicenter learning network comprised of pediatric hospitals implanting VADs in children and adults with complex CHD. We examined the outcomes of patients undergoing HeartMate 3 implantation at an ACTION center between December 2017 and September 2019. RESULTS: The HeartMate 3 was implanted in 35 patients at 9 ACTION centers, with a median age of 15.7 (8.8-47.3) years, median weight of 65.7 (19.1-114.1) kg, and median body surface area (BSA) of 1.74 (0.78-2.36) m2. Of the cohort, 14 patients (40%) weighed <60 kg. Diagnoses included dilated cardiomyopathy (63%), dilated cardiomyopathy in neuromuscular disease (20%), and CHD (17%). Of those with CHD, most had a Fontan circulation. With a median 78 days of follow-up, there was 1 death on device (97% survival); 20 out of 35 (57%) underwent transplantation with no post-transplantation mortality. There were no episodes of stroke or pump thrombosis. CONCLUSIONS: Use of the HeartMate 3 in ACTION centers was associated with a low incidence of mortality and adverse events. Patients as small as 19 kg (BSA 0.78 m2) were successfully implanted and supported, indicating that this device may be appropriate for older children and small adults.


Assuntos
Cardiopatias Congênitas/cirurgia , Coração Auxiliar , Sistema de Registros , Adolescente , Adulto , Criança , Feminino , Seguimentos , Transplante de Coração/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Adulto Jovem
20.
Ann Thorac Surg ; 107(4): 993-1004, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30817920

RESUMO

BACKGROUND: The influence of ventricular assist device (VAD) use in the care of children with end-stage heart failure is growing rapidly through increasing numbers, new devices, expanding indications, and improving outcomes. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Institutes of Health-sponsored United States database, provides a platform to understand this emerging population. METHODS: Between September 19, 2012, and December 31, 2017, 30 hospitals implanted 508 devices in 423 patients aged younger than 19 years. This past year was one of evolution for the database as its management was transitioned to The Society of Thoracic Surgery; therefore, this report does not include data from institutions not under contract by August 1, 2018. RESULTS: Of the 423 patients, the diagnosis was cardiomyopathy in 261 (62%), myocarditis in 48 (11%), other in 28 (7%), and congenital heart disease (CHD) in 86 (20%), with 52 of these patients having single-ventricle physiology. The two most common support strategies included left VAD in 342 (81%) and biventricular assist device in 64 (15%). Positive outcome (alive on device or bridge to transplantation/recovery) was 80% at 6 months (overall mortality of 20%). The patient cohort for implantable continuous-flow (IC) pumps (n = 197; age at implant, 13.4 ± 3.8 years; 19% Interagency Registry for Mechanically Assisted Circulatory Support [Intermacs] profile 1, 21% intubated at implant, and 12% with CHD) was significantly different from the paracorporeal continuous-flow (PC) pump cohort (n = 79; age, 3.9 ± 5.2 years; 49% Intermacs profile 1, 86% intubated at implant, and 38% with CHD) and the paracorporeal pulsatile (PP) pump cohort (n = 121; age, 3.3 ± 3.9 years; 41% Intermacs profile 1, 77% intubated at implant, and 21% with CHD). Consistent with their cohort composition, device type positive outcomes at 6 months were PC, 63%; PP, 77%; and IC, 92%. Parametric hazard modeling showed an early hazard for death was associated with Intermacs profile 1, biventricular assist device, percutaneous devices, PC devices, small-volume institutions, low age, and low weight, whereas a constant hazard was associated with intubation and liver dysfunction at time of implant. CONCLUSIONS: IC VADs are the most common VAD type placed in children. The positive outcomes for the IC VADs exceed 90% at 6 months, which may represent our field's maturation in both patient selection and timing of implantation. The PP/PC devices are currently limited to supporting our most challenging patients, those weighing less than 20 kg and those with CHD. The introduction of new devices and our communities' commitment to shared learning and improvement will lead to more pediatric lives saved by VAD support and will also focus on improving the quality of life of children supported with VADs.


Assuntos
Insuficiência Cardíaca/cirurgia , Transplante de Coração/mortalidade , Coração Auxiliar/estatística & dados numéricos , Sistema de Registros , Cirurgia Torácica , Adolescente , Criança , Pré-Escolar , Feminino , Insuficiência Cardíaca/diagnóstico , Transplante de Coração/métodos , Hospitais Pediátricos , Humanos , Masculino , Pediatria , Cuidados Pré-Operatórios/métodos , Medição de Risco , Sociedades Médicas , Taxa de Sobrevida , Resultado do Tratamento , Estados Unidos
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