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1.
N Engl J Med ; 387(23): 2138-2149, 2022 12 08.
Artigo em Inglês | MEDLINE | ID: mdl-36342116

RESUMO

BACKGROUND: Although perioperative prophylactic glucocorticoids have been used for decades, whether they improve outcomes in infants after heart surgery with cardiopulmonary bypass is unknown. METHODS: We conducted a multicenter, prospective, randomized, placebo-controlled, registry-based trial involving infants (<1 year of age) undergoing heart surgery with cardiopulmonary bypass at 24 sites participating in the Society of Thoracic Surgeons Congenital Heart Surgery Database. Registry data were used in the evaluation of outcomes. The infants were randomly assigned to receive prophylactic methylprednisolone (30 mg per kilogram of body weight) or placebo, which was administered into the cardiopulmonary-bypass pump-priming fluid. The primary end point was a ranked composite of death, heart transplantation, or any of 13 major complications. Patients without any of these events were assigned a ranked outcome based on postoperative length of stay. In the primary analysis, the ranked outcomes were compared between the trial groups with the use of odds ratios adjusted for prespecified risk factors. Secondary analyses included an unadjusted odds ratio, a win ratio, and safety outcomes. RESULTS: A total of 1263 infants underwent randomization, of whom 1200 received either methylprednisolone (599 infants) or placebo (601 infants). The likelihood of a worse outcome did not differ significantly between the methylprednisolone group and the placebo group (adjusted odds ratio, 0.86; 95% confidence interval [CI], 0.71 to 1.05; P = 0.14). Secondary analyses (unadjusted for risk factors) showed an odds ratio for a worse outcome of 0.82 (95% CI, 0.67 to 1.00) and a win ratio of 1.15 (95% CI, 1.00 to 1.32) in the methylprednisolone group as compared with the placebo group, findings suggestive of a benefit with methylprednisolone; however, patients in the methylprednisolone group were more likely than those in the placebo group to receive postoperative insulin for hyperglycemia (19.0% vs. 6.7%, P<0.001). CONCLUSIONS: Among infants undergoing surgery with cardiopulmonary bypass, prophylactic use of methylprednisolone did not significantly reduce the likelihood of a worse outcome in an adjusted analysis and was associated with postoperative development of hyperglycemia warranting insulin in a higher percentage of infants than placebo. (Funded by the National Center for Advancing Translational Sciences and others; STRESS ClinicalTrials.gov number, NCT03229538.).


Assuntos
Procedimentos Cirúrgicos Cardíacos , Metilprednisolona , Humanos , Metilprednisolona/efeitos adversos , Estudos Prospectivos , Insulina
2.
Perfusion ; : 2676591241236640, 2024 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-38400702

RESUMO

Cannula stabilization for extracorporeal membrane oxygenation (ECMO) is important for patient mobilization and rehabilitation. Limitations to mobilization on ECMO include staff discomfort and cannula instability. We utilized the technique of negative pressure therapy for ECMO cannula stabilization to improve mobilization. Negative pressure therapy for ECMO cannula stabilization can be utilized safely for a variety of cannulation sites in any patient age from newborns to adults. This wound management strategy may facilitate patient mobilization and rehabilitation therapies in addition to extending cannula site duration.

3.
Heart Fail Clin ; 20(2): 199-208, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38462324

RESUMO

Surgical intervention is often used in the management of heart failure in patients with adult congenital heart disease. This review addresses anatomic variations and complications due to prior surgical interventions, including sternal reentry, collateral vessels, and the neo-aortic root after the Damus-Kaye-Stansel procedure. Surgical considerations for systemic atrioventricular valvular surgery, Fontan revision, and advanced heart failure therapies including ventricular assist devices, heart transplant, and combined heart-liver transplant are discussed, with a focus on unique patient populations including those with systemic right ventricles and those with Fontan circulation.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Insuficiência Cardíaca , Humanos , Adulto , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar , Aorta/cirurgia , Insuficiência Cardíaca/cirurgia , Ventrículos do Coração
4.
Pediatr Cardiol ; 43(2): 426-434, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34609534

RESUMO

Minimal data exist about the incidence and risk factors for arch intervention after comprehensive stage II (CSII). Goal of this study was to document incidence of arch interventions after CSII and determine if any differences existed between those who underwent an arch intervention (aiCSII) versus those did not have an intervention. Single-center retrospective chart review of all hypoplastic left heart syndrome patients who underwent a CSII between 6/1/2005 and 2/1/2020 was performed. Univariate analysis was conducted in addition to principal components analysis (PCA). One hundred patients were evaluated. Sixteen patients underwent 24 arch interventions. Age at initial arch reintervention was 1.3 ± 1.2 years (median 1.0 years, range 0.5-2.2 years). Univariate analysis showed that the aiCSII group were more likely to be female, to have had a retrograde arch intervention post-hybrid procedure, and to be younger at time of CSII. On echocardiograms, aiCSII group had significantly higher pre-CSII patent ductus arteriosus velocities, arch velocities on their 1st post-operative and discharge study post-CSII, and arch velocities pre-Fontan. Gradients were higher in the aiCSII via pre-Fontan catheterization. With PCA, echocardiographic and catheterization data remained significantly associated with aiCSII versus those who did not undergo an arch intervention (OR = 4.5 (1.9, 19.8), p = 0.008). Incidence of arch intervention post-CSII was 16%. Echocardiographic arch velocities during the CSII hospitalization were the strongest predictors for subsequent aortic arch interventions. Further studies are needed to determine any modifiable variables that may reduce the incidence of arch interventions.


Assuntos
Coartação Aórtica , Síndrome do Coração Esquerdo Hipoplásico , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Coartação Aórtica/cirurgia , Pré-Escolar , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Incidência , Lactente , Masculino , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
5.
Echocardiography ; 38(8): 1210-1217, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-34184329

RESUMO

AIMS: Cylinder mitral valve construct (cMVC) is new technique for replacing the mitral valve compared to more traditional mitral valve replacement (MVR) procedures. Goal of this study was to describe echocardiographic changes over time in patients undergoing a cMVC. Secondary goal was to compare echocardiographic changes in patients that underwent a cMVC to a group of patients that underwent a MVR. METHODS: Retrospective analysis of patients undergoing a cMVC was performed. Demographics, discharge echocardiogram, and recent echocardiogram vales were evaluated. Age matched patients undergoing a MVR were assessed. Discharge and recent echocardiographic parameters were compared within the cMVC group. cMVC and MVR values were compared between groups. RESULTS: Five cMVC patients were studied. Age at surgery for the cMVC was 4.3 ± 4.2 years (median 2.2, .8-10.3 years). Time interval from hospital discharge echocardiogram to the most recent echocardiogram was 1.2 ± .7 years (median 1.0, .6-2.0 years). Mean mitral valve gradient significantly increased over time (3.6 ± 3.0 mm Hg vs 7.6 ± 2.9 mm Hg). There were significant improvements in left ventricular diameters, systolic sphericity index, shortening fraction, and ejection fraction over time. There were no significant differences in demographics, discharge echocardiogram values, and follow up echocardiogram values between the cMVC and MVR groups. CONCLUSION: In conclusion, echocardiographic indices of left ventricular function improved over time in patients undergoing cMVC. In addition, there were no significant differences between cMVC and MVR patients in echocardiographic values. Studies with a larger patient sample with longer follow up are needed to determine if cMVC continues to have comparable echocardiographic results to MVR.


Assuntos
Implante de Prótese de Valva Cardíaca , Insuficiência da Valva Mitral , Ecocardiografia , Humanos , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
6.
Biochemistry ; 59(11): 1202-1215, 2020 03 24.
Artigo em Inglês | MEDLINE | ID: mdl-32133840

RESUMO

The heterodimeric actin capping protein (CP) is regulated by a set of proteins that contain CP-interacting (CPI) motifs. Outside of the CPI motif, the sequences of these proteins are unrelated and distinct. The CPI motif and surrounding sequences are conserved within a given protein family, when compared to those of other CPI-motif protein families. Using biochemical assays with purified proteins, we compared the ability of CPI-motif-containing peptides from different protein families (a) to bind to CP, (b) to allosterically inhibit barbed-end capping by CP, and (c) to allosterically inhibit interaction of CP with V-1, another regulator of CP. We found large differences in potency among the different CPI-motif-containing peptides, and the different functional assays showed different orders of potency. These biochemical differences among the CPI-motif peptides presumably reflect interactions between CP and CPI-motif peptides involving amino acid residues that are conserved but are not part of the strictly defined consensus, as it was originally identified in comparisons of sequences of CPI motifs across all protein families [Hernandez-Valladares, M., et al. (2010) Structural characterization of a capping protein interaction motif defines a family of actin filament regulators. Nat. Struct. Mol. Biol. 17, 497-503; Bruck, S., et al. (2006) Identification of a Novel Inhibitory Actin-capping Protein Binding Motif in CD2-associated Protein. J. Biol. Chem. 281, 19196-19203]. These biochemical differences may be important for conserved distinct functions of CPI-motif protein families in cells with respect to the regulation of CP activity and actin assembly near membranes.


Assuntos
Proteína de Capeamento de Actina CapZ/química , Proteína de Capeamento de Actina CapZ/metabolismo , Actinas/química , Actinas/metabolismo , Regulação Alostérica , Motivos de Aminoácidos , Animais , Proteína de Capeamento de Actina CapZ/genética , Dimerização , Eucariotos/classificação , Eucariotos/genética , Eucariotos/metabolismo , Humanos , Cinética , Peptídeos/química , Peptídeos/metabolismo , Filogenia , Ligação Proteica , Conformação Proteica , Domínios e Motivos de Interação entre Proteínas
7.
Addict Biol ; 25(2): e12743, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-30945801

RESUMO

Preclinical studies have shown effects of chronic exposure to addictive drugs on glutamatergic-mediated neuroplasticity in frontostriatal circuitry. These initial findings have been paralleled by human functional magnetic resonance imaging (fMRI) research demonstrating weaker frontostriatal resting-state functional connectivity (rsFC) among individuals with psychostimulant use disorders. However, there is a dearth of human imaging literature describing associations between long-term prescription opioid use, frontostriatal rsFC, and brain morphology among chronic pain patients. We hypothesized that prescription opioid users with chronic pain, as compared with healthy control subjects, would evidence weaker frontostriatal rsFC coupled with less frontostriatal gray matter volume (GMV). Further, those opioid use-related deficits in frontostriatal circuitry would be associated with negative affect and drug misuse. Prescription opioid users with chronic pain (n = 31) and drug-free healthy controls (n = 30) underwent a high-resolution anatomical and an eyes-closed resting-state functional scan. The opioid group, relative to controls, exhibited weaker frontostriatal rsFC, and less frontostriatal GMV in both L.NAc and L.vmPFC. Frontostriatal rsFC partially mediated group differences in negative affect. Within opioid users, L.NAc GMV predicted opioid misuse severity. The current study revealed that prescription opioid use in the context of chronic pain is associated with functional and structural abnormalities in frontostriatal circuitry. These results suggest that opioid use-related abnormalities in frontostriatal circuitry may undergird disturbances in affect that may contribute to the ongoing maintenance of opioid use and misuse. These findings warrant further examination of interventions to treat opioid pathophysiology in frontostriatal circuitry over the course of treatment.


Assuntos
Afeto/efeitos dos fármacos , Analgésicos Opioides/efeitos adversos , Dor Crônica/tratamento farmacológico , Corpo Estriado/efeitos dos fármacos , Corpo Estriado/fisiopatologia , Lobo Frontal/efeitos dos fármacos , Lobo Frontal/fisiopatologia , Adulto , Analgésicos Opioides/uso terapêutico , Dor Crônica/fisiopatologia , Corpo Estriado/diagnóstico por imagem , Feminino , Lobo Frontal/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade
8.
Pediatr Cardiol ; 41(7): 1458-1465, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32607741

RESUMO

Mitral valve replacement (MVR) in children under 2 years is associated with significant morbidity and mortality. Decellularized porcine intestinal submucosa is a commercially available formulation of an extracellular matrix (ECM) with an indication for cardiac tissue repair. The present study reports our experience using ECM cylinder valves in patients for MVR. A retrospective review of patients under 2 years who underwent ECM custom-made cylinder mitral valve (ECM-MV) replacement was performed. Clinical, demographic, operative and post-operative follow-up data, including serial echocardiographic data are presented. Eight patients (age 5.6 ± 1.6 months; weight: 6.0 ± 1.1 kg) were identified who underwent ECM-MVR. There was one in-hospital death and no major neurological events. Six patients underwent replacement of their cylinder valve with either a Melody valve inside the ECM-MVR (n = 3), a mechanical valve (n = 2), or a decellularized bovine pericardial cylinder valve (n = 1). The mean time to replacement surgery was 8.4 ± 2.6 months after ECM-MV. The indications for replacement of ECM-MV included mitral stenosis/regurgitation (n = 4) or dehiscence (n = 2). One remaining patient is 24 months from ECM-MV, with trivial regurgitation and no stenosis. Mitral valve creation using ECM is an option for MVR in pediatrics, avoiding anticoagulation, and provides a suitable construct for later placement of a Melody valve, extending surgical and non-surgical options. However, the durability of the native ECM-MV in the mitral position is concerning considering the high re-intervention rate in a relatively short time period. Further studies are needed to determine the longer-term outcomes of this valve in this complex patient population.


Assuntos
Implante de Prótese de Valva Cardíaca/métodos , Insuficiência da Valva Mitral/cirurgia , Estenose da Valva Mitral/cirurgia , Ecocardiografia , Matriz Extracelular/transplante , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento
9.
Circ Res ; 121(8): 963-969, 2017 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-28729354

RESUMO

RATIONALE: The objective of this autopsy study was to determine whether gastrointestinal angiodysplasia develops during continuous-flow left ventricular assist device (LVAD) support. OBJECTIVE: LVAD support causes pathologic degradation of von Willebrand factor (vWF) and bleeding from gastrointestinal angiodysplasia at an alarming rate. It has been speculated that LVAD support itself may cause angiodysplasia. The relationship to abnormal vWF metabolism is unknown. We tested the hypothesis that abnormal gastrointestinal vascularity develops during continuous-flow LVAD support. METHODS AND RESULTS: Small bowel was obtained from deceased humans, cows, and sheep supported with a continuous-flow LVAD (n=9 LVAD, n=11 control). Transmural sections of jejunum were stained with fluorescein isothiocyanate-conjugated isolectin-B4 for endothelium to demarcate vascular structures and quantify intestinal vascularity. Paired plasma samples were obtained from humans before LVAD implantation and during LVAD support (n=41). vWF multimers and degradation fragments were quantified with agarose and polyacrylamide gel electrophoresis and immunoblotting. Abnormal vascular architecture was observed in the submucosa of the jejunum of human patients, cows, and sheep supported with a continuous-flow LVAD. Intestinal vascularity was significantly higher after LVAD support versus controls (5.2±1.0% versus 2.1±0.4%, P=0.004). LVAD support caused significant degradation of high-molecular-weight vWF multimers (-9±1%, P<0.0001) and accumulation of low-molecular-weight vWF multimers (+40±5%, P<0.0001) and vWF degradation fragments (+53±6%, P<0.0001). CONCLUSIONS: Abnormal intestinal vascular architecture and LVAD-associated vWF degradation were consistent findings in multiple species supported with a continuous-flow LVAD. These are the first direct evidence that LVAD support causes gastrointestinal angiodysplasia. Pathologic vWF metabolism may be a mechanistic link between LVAD support, abnormal angiogenesis, gastrointestinal angiodysplasia, and bleeding.


Assuntos
Angiodisplasia/etiologia , Coração Auxiliar/efeitos adversos , Doenças do Jejuno/etiologia , Jejuno/irrigação sanguínea , Implantação de Prótese/efeitos adversos , Implantação de Prótese/instrumentação , Função Ventricular Esquerda , Adulto , Idoso , Angiodisplasia/metabolismo , Angiodisplasia/patologia , Animais , Autopsia , Bovinos , Modelos Animais de Doenças , Hemorragia Gastrointestinal/etiologia , Humanos , Doenças do Jejuno/metabolismo , Doenças do Jejuno/patologia , Jejuno/metabolismo , Jejuno/patologia , Pessoa de Meia-Idade , Peso Molecular , Desenho de Prótese , Proteólise , Carneiro Doméstico , Fator de von Willebrand/metabolismo
10.
J Pediatr ; 198: 36-45.e3, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29650415

RESUMO

OBJECTIVE: To quantify outcomes of infants (<1 year of age) diagnosed with pulmonary vein stenosis (PVS). STUDY DESIGN: MEDLINE (PubMed), Scopus, and Web of Science were searched through February 1, 2017, with no language restrictions. Publications including infants diagnosed with primary PVS, defined as the absence of preceding intervention(s), were considered. The study was performed according to Meta-analysis of Observational Studies in Epidemiology guidelines, the Systematic Reviews, and Meta-Analysis checklist, and registered prospectively. The quality of selected reports was critically examined. Data extraction was independently performed by multiple observers with outcomes agreed upon a priori. Data were pooled using an inverse variance heterogeneity model with incidence of mortality the primary outcome of interest. RESULTS: Forty-eight studies of 185 infants were included. Studies were highly diverse with regards to the participants, interventions, and outcomes reported. The median (range) age at diagnosis was 5.0 (0.1-11.6) months. Pooled mortality was 58.5% (95% CI 49.8%-67.0%, I2 = 21.4%). We observed greater mortality incidence among infants with 3 or 4 vein stenoses than in those with 1 or 2 vein stenoses (83.3% vs 36.1%; P < .01). We observed greater mortality among infants with bilateral than unilateral disease (78.7% vs 26.0%; P < .01). CONCLUSIONS: Studies of primary PVS during infancy are highly variable in their methodological quality and estimates of clinical outcomes; therefore, estimates of prognosis remain uncertain. Multicenter, interdisciplinary collaborations, including alignment of key outcome measurements, are needed to answer questions beyond the scope of available data.


Assuntos
Estenose de Veia Pulmonar/diagnóstico , Estenose de Veia Pulmonar/terapia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Avaliação de Resultados em Cuidados de Saúde , Estenose de Veia Pulmonar/mortalidade
11.
J Biol Chem ; 291(3): 1076-91, 2016 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-26578515

RESUMO

CARMILs regulate capping protein (CP), a critical determinant of actin assembly and actin-based cell motility. Vertebrates have three conserved CARMIL genes with distinct functions. In migrating cells, CARMIL2 is important for cell polarity, lamellipodial assembly, ruffling, and macropinocytosis. In cells, CARMIL2 localizes with a distinctive dual pattern to vimentin intermediate filaments and to membranes at leading edges and macropinosomes. The mechanism by which CARMIL2 localizes to membranes has not been defined. Here, we report that CARMIL2 has a conserved membrane-binding domain composed of basic and hydrophobic residues, which is necessary and sufficient for membrane localization, based on expression studies in cells and on direct binding of purified protein to lipids. Most important, we find that the membrane-binding domain is necessary for CARMIL2 to function in cells, based on rescue expression with a set of biochemically defined mutants. CARMIL1 and CARMIL3 contain similar membrane-binding domains, based on sequence analysis and on experiments, but other CPI motif proteins, such as CD2AP, do not. Based on these results, we propose a model in which the membrane-binding domain of CARMIL2 tethers this multidomain protein to the membrane, where it links dynamic vimentin filaments with regulation of actin assembly via CP.


Assuntos
Membrana Celular/metabolismo , Filamentos Intermediários/metabolismo , Proteínas dos Microfilamentos/metabolismo , Podossomos/metabolismo , Vimentina/metabolismo , Proteínas Adaptadoras de Transdução de Sinal/química , Proteínas Adaptadoras de Transdução de Sinal/genética , Proteínas Adaptadoras de Transdução de Sinal/metabolismo , Sequência de Aminoácidos , Linhagem Celular , Movimento Celular , Sequência Conservada , Proteínas do Citoesqueleto/química , Proteínas do Citoesqueleto/genética , Proteínas do Citoesqueleto/metabolismo , Bases de Dados de Proteínas , Humanos , Interações Hidrofóbicas e Hidrofílicas , Lipossomos , Proteínas Luminescentes/genética , Proteínas Luminescentes/metabolismo , Proteínas dos Microfilamentos/antagonistas & inibidores , Proteínas dos Microfilamentos/química , Proteínas dos Microfilamentos/genética , Mutação , Fragmentos de Peptídeos/antagonistas & inibidores , Fragmentos de Peptídeos/química , Fragmentos de Peptídeos/genética , Fragmentos de Peptídeos/metabolismo , Domínios e Motivos de Interação entre Proteínas , Isoformas de Proteínas/antagonistas & inibidores , Isoformas de Proteínas/química , Isoformas de Proteínas/genética , Isoformas de Proteínas/metabolismo , Transporte Proteico , Interferência de RNA , Proteínas Recombinantes de Fusão/química , Proteínas Recombinantes de Fusão/metabolismo
12.
Artif Organs ; 41(1): 66-70, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27896819

RESUMO

Outcomes for extracorporeal membrane oxygenation (ECMO) have been described for patients with single ventricle physiology (SVP) undergoing cavopulmonary connection (Glenn procedure). An alternative surgical pathway for patients with SVP consists of an initial hybrid procedure followed by a comprehensive Stage II procedure. No data exist describing the outcomes of patients requiring ECMO after the comprehensive Stage II procedure. The goal of this study is to describe the outcomes for patients who required ECMO after the comprehensive Stage II procedure. Data from the Extracorporeal Life Support Organization (ELSO) registry from 2001 to 2015 for children undergoing the comprehensive Stage II procedure older than 3 months of age were retrospectively analyzed. Demographics and ECMO characteristics were recorded. A total of six children required ECMO support after the comprehensive Stage II procedure (2 males, 4 females). Four patients had the diagnosis of hypoplastic left heart syndrome and two patients had the diagnosis of an unbalanced atrioventricular septal defect. Bypass time was 242.8 ± 110.9 min and cross-clamp time was 91.2 ± 46.2 min for the surgical procedure. Weight was 5.8 ± 1.3 kg and age was 150.2 + 37.9 days at time of ECMO. ECMO duration was 276.0 ± 218.1 h. Complications during the ECMO run included hemorrhage in four patients (67%), renal dysfunction in two patients (33%), and neurologic injury in two patients (33%). Four patients (67%) were discharged alive after ECMO decannulation. Despite being a much more extensive surgical procedure, the morbidity and mortality after ECMO in patients undergoing the comprehensive Stage II procedure are similar to those in patients undergoing the Glenn procedure. If needed, ECMO support is reasonable for patients after the comprehensive Stage II procedure.


Assuntos
Oxigenação por Membrana Extracorpórea , Defeitos dos Septos Cardíacos/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Oxigenação por Membrana Extracorpórea/efeitos adversos , Oxigenação por Membrana Extracorpórea/métodos , Feminino , Técnica de Fontan , Defeitos dos Septos Cardíacos/patologia , Ventrículos do Coração/patologia , Ventrículos do Coração/cirurgia , Hemorragia/etiologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/patologia , Lactente , Nefropatias/etiologia , Masculino , Estudos Retrospectivos , Resultado do Tratamento
13.
Thorac Cardiovasc Surg ; 65(1): 36-42, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27595243

RESUMO

Background Pre-lung transplant (LTx) panel reactive antibody (PRA) levels are associated with adverse outcomes in adult LTx recipients, but their impact in pediatric LTx recipients is unknown. Methods The United Network for Organ Sharing registry was queried from 2004 to 2013 to compare survival between pediatric LTx recipients with PRA class I and II levels = 0 versus > 0. Results Overall, 333 pediatric LTx recipients had data on class I or II PRA and were included in the analysis. Univariate analysis demonstrated that PRA > 0 was not associated with survival benefit for class I (hazard ratio [HR] = 0.985; 95% confidence interval [CI]: 0.623, 1.555; p = 0.947) or class II (HR = 1.080; 95% CI: 0.657, 1.774; p = 0.762) PRA. Multivariate Cox models confirmed no significant association with mortality hazard for both class I (HR = 1.230; 95% CI: 0.641, 2.363; p = 0.533) and class II (HR = 0.847; 95% CI: 0.359, 1.997; p = 0.704) PRA. Multivariate logistic regression models identified no association between class I or class II and acute rejection within 3 years of LTx. Conclusions Pretransplant class I and II PRA levels > 0 were not associated with mortality or acute rejection in pediatric LTx recipients.


Assuntos
Teste de Histocompatibilidade , Histocompatibilidade , Isoanticorpos/sangue , Transplante de Pulmão , Doença Aguda , Adolescente , Biomarcadores/sangue , Criança , Pré-Escolar , Feminino , Rejeição de Enxerto/imunologia , Sobrevivência de Enxerto , Humanos , Estimativa de Kaplan-Meier , Modelos Logísticos , Transplante de Pulmão/efeitos adversos , Transplante de Pulmão/mortalidade , Masculino , Análise Multivariada , Valor Preditivo dos Testes , Modelos de Riscos Proporcionais , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Obtenção de Tecidos e Órgãos , Resultado do Tratamento , Estados Unidos
14.
Echocardiography ; 34(12): 1956-1958, 2017 12.
Artigo em Inglês | MEDLINE | ID: mdl-28942609

RESUMO

Mitral valve construction using extracellular matrix (ECM) is a relatively new procedure. In this case, a 15-month-old boy with a history of severe mitral valve regurgitation secondary to endocarditis underwent mitral valve surgery. Mitral valve repair was not possible, and thus, a 17 mm extracellular matrix cylinder valve (ECM-CV) was constructed for valve replacement. The ECM-CV is clearly imaged using echocardiography, especially three-dimensional imaging, that helped define valve function. As the use of ECM for valve construction increases, echocardiography will play an essential role in evaluating the function and mechanics of these novel valves.


Assuntos
Ecocardiografia/métodos , Matriz Extracelular/transplante , Próteses Valvulares Cardíacas , Insuficiência da Valva Mitral/cirurgia , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Humanos , Lactente , Masculino
15.
Pediatr Cardiol ; 38(2): 280-288, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27882424

RESUMO

Transplant center expertise improves survival after heart transplant (HTx) but it is unknown whether center expertise ameliorates risk associated with mechanical circulatory support (MCS) bridge to transplantation. This study investigated whether center HTx volume reduced survival disparities among pediatric HTx patients bridged with extracorporeal membrane oxygenation (ECMO), left ventricular assist device (LVAD), or no MCS. Patients ≤18 years of age receiving first-time HTx between 2005 and 2015 were identified in the United Network of Organ Sharing registry. Center volume was the total number of HTx during the study period, classified into tertiles. The primary outcome was 1 year post-transplant survival, and MCS type was interacted with center volume in Cox proportional hazards regression. The study cohort included 4131 patients, of whom 719 were supported with LVAD and 230 with ECMO. In small centers (≤133 HTx over study period), patients bridged with ECMO had increased post-transplant mortality hazard compared to patients bridged with LVAD (HR 0.29, 95% CI 0.12, 0.71; p = 0.006) and patients with no MCS (HR 0.33, 95% CI 0.19, 0.57; p < 0.001). Interactions of MCS type with medium or large center volume were not statistically significant, and the same differences in survival by MCS type were observed in medium- or large-volume centers (136-208 or ≥214 HTx over the study period). Post-HTx survival disadvantage of pediatric patients bridged with ECMO persisted regardless of transplant program volume. The role of institutional ECMO expertise outside the transplant setting for improving outcomes of ECMO bridge to HTx should be explored.


Assuntos
Competência Clínica , Oxigenação por Membrana Extracorpórea , Insuficiência Cardíaca/cirurgia , Transplante de Coração/estatística & dados numéricos , Coração Auxiliar , Adolescente , Criança , Pré-Escolar , Feminino , Sobrevivência de Enxerto , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Modelos de Riscos Proporcionais , Sistema de Registros , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Estados Unidos
16.
Clin Transplant ; 30(10): 1332-1339, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-27490992

RESUMO

BACKGROUND: Research on induction immunosuppression in patients undergoing combined heart-lung transplantation (HLTx) is limited. METHODS: The United Network for Organ Sharing database was queried from 2000 to 2013 to examine the influence of induction immunosuppression for combined HLTx in adult (≥18 years) and adolescent (≥12 and <18 years) recipients. RESULTS: Of 394 eligible combined HLTx cases (361 adults, 33 adolescents), 384 were included in univariate Cox analysis and 116 in the multivariate Cox model. Univariate analysis demonstrated no differences in survival by induction medication and no difference among the most common maintenance immunosuppression regimens. Adjusting for use of corticosteroids, multivariate analysis demonstrated no benefit of basiliximab (HR=3.582; 95% CI: 0.966, 13.279; P=.056), thymoglobulin/antilymphocyte globulin (ALG)/antithymocyte globulin (ATG) (HR=0.808; 95% CI: 0.134, 4.888; P=.817), alemtuzumab (HR=0.369; 95% CI: 0.087, 1.563; P=.176), or other induction medications (HR=1.511; 95% CI: 0.146, 15.610; P=.729), compared to no induction medication, with respect to mortality hazard post-HLTx. There were also no differences in treated acute rejection episodes by type of induction immunosuppression. CONCLUSIONS: Induction immunosuppression with contemporary agents does not improve survival after combined HLTx.


Assuntos
Rejeição de Enxerto/prevenção & controle , Transplante de Coração-Pulmão/mortalidade , Terapia de Imunossupressão/métodos , Imunossupressores/uso terapêutico , Quimioterapia de Indução/métodos , Adolescente , Adulto , Idoso , Criança , Bases de Dados Factuais , Quimioterapia Combinada , Feminino , Seguimentos , Rejeição de Enxerto/mortalidade , Humanos , Estimativa de Kaplan-Meier , Quimioterapia de Manutenção/métodos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Adulto Jovem
17.
Pediatr Transplant ; 19(1): 87-93, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25425268

RESUMO

Limited data exist on ECMO at the time of LTx in children. The UNOS database was queried from 2000 to 2013 for pediatric lung transplant recipients (<18 yr) to assess post-transplant survival of patients on ECMO at the time of LTx. Of 587 pediatric recipients with 17 on ECMO, 585 were used for univariate and Kaplan-Meier function analysis, 535 for multivariate Cox models, and 24 for propensity score matching. Univariate Cox (HR = 1.777; 95% CI: 0.658, 4.803; p = 0.257) and Kaplan-Meier function (log-rank test: chi-square (df = 1): 1.32, p = 0.250) analyses did not identify a survival difference between ECMO and non-ECMO, while multivariate Cox models (HR = 1.821; 95% CI: 0.654, 5.065; p = 0.251) did not demonstrate an increased risk for death. Propensity score matching analysis (HR = 1.500; 95% CI: 0.251, 8.977; p = 0.657) also failed to demonstrate a significantly increased hazard ratio. Using a contemporary cohort of pediatric lung transplant recipients, the use of ECMO at the time of lung transplantation did not negatively impact survival.


Assuntos
Oxigenação por Membrana Extracorpórea/mortalidade , Transplante de Pulmão , Adolescente , Criança , Feminino , Humanos , Masculino , Análise Multivariada , Estudos Retrospectivos , Análise de Sobrevida
18.
Paediatr Anaesth ; 25(5): 477-86, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25581204

RESUMO

BACKGROUND: The transfusion of blood products in the setting of uncontrolled bleeding is unquestionably lifesaving. However, in many instances, the decision to transfuse is based on physician gestalt rather than medical evidence. When indications for transfusion are unclear, the benefits of blood products must be balanced against their significant risks and associated costs. As our institution is a referral center for patients of Jehovah's Witness faith, this population has pushed our development of techniques to achieve the goal of bloodless surgery. Our practices in caring for this population have become our standard practice for managing all patients undergoing congenital cardiac surgery. OBJECTIVES: To evaluate our success in minimizing the use of blood products during pediatric cardiac surgery. METHODS: After IRB approval, we retrospectively reviewed all patients who underwent cardiac surgery utilizing cardiopulmonary bypass (CPB), for biventricular repair procedures. The study was conducted at a single institution (Nationwide Children's Hospital (NCH)) during the period: January 1, 2013 and December 31, 2013. RESULTS: A total of 209 patients were included. Overall, 81 patients (38.8%) and 81 of 136 (59.6%) weighing more than 6 kg received no blood products (bloodless) during their entire hospital stay. Bloodless surgery was most successful in patients weighing more than 18 kg, followed by patients weighing 6-18 kg. All 73 patients who weighed <6 kg received blood transfusion during their hospitalization. CONCLUSION: The techniques that we have developed to initially care for our Jehovah's Witness families may be applied to other pediatric and adult surgical procedures.


Assuntos
Transfusão de Sangue/estatística & dados numéricos , Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Cardiopatias Congênitas/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Testemunhas de Jeová , Tempo de Internação/estatística & dados numéricos , Masculino , Religião e Medicina , Estudos Retrospectivos , Adulto Jovem
19.
Transpl Int ; 27(2): 211-8, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24236829

RESUMO

There is an increasing trend in the use of induction immunosuppression in children undergoing lung transplantation (LTx). To evaluate the effect of this practice on survival, the United Network for Organ Sharing (UNOS) was queried from 1987 to 2012, restricting analysis to transplant patients 6-17 years old from 2001 to 2012, who received no induction (NONE) or induction (INDUCED) with the contemporary agents of basiliximab, alemtuzumab, thymoglobulin, antilymphocyte globulin (ALG), or antithymocyte globulin (ATG). Of 23 951 lung transplants, 330 met inclusion criteria with 177 (54%) being INDUCED. Of the INDUCED agents, 121 (68%) were basiliximab, 3 (2%) alemtuzumab, and 53 (30%) ALG/ATG/thymoglobulin. The mean patient age was 13.6 (SD = 3.2) and 14 (SD = 3.0) years for the INDUCED and NONE groups, respectively. The median survival in the INDUCED group was 77.4 months (95% CI: 46.1, 125.6) compared with 50.8 months (95% CI: 42.9, 61.3) for the NONE (log-rank P-value = 0.3601). The most common cause of death was due to allograft failure or pulmonary complications with only one patient dying from post-transplant lymphoproliferative disorder. The estimated hazard ratio for INDUCED versus NONE was 0.859 (95% CI: 0.620, 1.191; P = 0.3618); there were no significant confounders or effect modifiers among the demographic and clinical variables. In conclusion, antibody-based induction immunosuppression with contemporary agents had a trend toward a protective, but not statistically significant, effect in 6- to 17-year-old patients.


Assuntos
Terapia de Imunossupressão/métodos , Transplante de Pulmão/métodos , Adolescente , Alemtuzumab , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Soro Antilinfocitário/uso terapêutico , Basiliximab , Cadáver , Criança , Feminino , Rejeição de Enxerto , Sobrevivência de Enxerto , Humanos , Imunossupressores/uso terapêutico , Transplante de Pulmão/mortalidade , Masculino , Modelos de Riscos Proporcionais , Proteínas Recombinantes de Fusão/uso terapêutico , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento
20.
J Intensive Care Med ; 29(4): 238-42, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-23753242

RESUMO

BACKGROUND: In recent years, the continuous noninvasive hemoglobin measurement has been offered by devices using advanced pulse oximetry technology. Accuracy has been established in healthy adults as well as in surgical and intensive care unit patients but not in the setting of acute hemorrhage. In this study, we evaluated the accuracy of such a device in the clinical setting of preoperative phlebotomy thereby mimicking a scenario of acute blood loss. METHODS: This prospective study included patients undergoing surgical repair of congenital heart disease (CHD) for whom preoperative phlebotomy was planned. Blood was removed after the induction of anesthesia and prior to the start of the surgical procedure. Replacement with crystalloid was guided by hemodynamic variables and cerebral oxygenation measured by near-infrared spectroscopy. Hemoglobin was measured by bedside whole blood analysis (total hemoglobin [tHb]) before and after phlebotomy, and concurrent measurements from the pulse co-oximeter (noninvasive, continuous, or spot-check testing of total hemoglobin [SpHb]) were recorded. RESULTS: The study cohort included 45 patients ranging in age from 3 months to 50 years. Preoperative phlebotomy removed an average of 9.2 mL/kg of blood that was replaced with an average of 7.2 mL/kg of crystalloid. The pre- and postphlebotomy tHb values were 13.0 ± 1.9 and 12.4 ± 1.8 g/dL, respectively. The absolute difference between the tHb and SpHb (▵Hb) was 1.2 ± 0.1 g/dL. Bland-Altman analysis revealed a bias of 0.1 g/dL, a precision of 1.5 g/dL, and 95% limits of agreement of -2.8 to 3.1 g/dL. In 52.2% of the sample sets, the SpHb was within 1 g/dL of the actual hemoglobin value (tHb), and in 80% of the sample sets, the SpHb was within 2 g/dL. No variation in the accuracy of the deviation was noted based on the patient's age, weight, or type of CHD (cyanotic versus acyanotic). CONCLUSION: The current study demonstrates that the accuracy of continuous, noninvasive hemoglobin measurement was not affected by acute blood loss simulated by preoperative phlebotomy. Although the device provided a clinically acceptable correlation with the actual hemoglobin value and offers the value of a continuous trend monitor, given the precision of the device, it does not appear that actual transfusion decisions can be based on the device alone.


Assuntos
Perda Sanguínea Cirúrgica , Hemoglobinas/análise , Oximetria , Flebotomia , Adolescente , Adulto , Criança , Pré-Escolar , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Pessoa de Meia-Idade , Cuidados Pré-Operatórios , Estudos Prospectivos , Adulto Jovem
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