83 research outputs found

    Impact of prior hospital mortality versus surgical volume on mortality following surgery for congenital heart disease

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    ObjectiveOur objective was to assess the relationships of a hospital’s past adjusted in-hospital mortality and surgical volume with future in-hospital mortality after surgery for congenital heart disease.MethodsUsing the Pediatric Health Information Systems database, we (1) calculated hospital surgical volume and standardized mortality ratio (= observed number of deaths/expected number of deaths adjusted for surgery type) for January 2004 through June 2006 for children (0-18 years) after surgery for congenital heart disease at 38 hospitals and (2) assessed the relationship between these values and subsequent mortality during July 2006 through December 2008. We constructed Poisson regression models to estimate risk of mortality, adjusting for age, race, sex, genetic syndrome, insurance type, and surgery type (using the Risk Adjustment in Congenital Heart Surgery method).ResultsThere were 49,792 hospital encounters during 2004 through 2008 for pediatric patients having surgery for congenital heart disease, with an overall in-hospital mortality of 3.45%. For the 24,112 eligible encounters during July 2006 through December 2008, a hospital’s prior standardized mortality ratio was significantly associated with postoperative in-hospital mortality (P < .0001), and a hospital’s prior surgical volume had only borderline significance (P = .0792). On stratified analysis, past standardized mortality ratio was associated with mortality for both lower- and higher-risk surgical risk categories (P = .0105 and .0015, respectively). Hospital surgical volume was not significantly associated with mortality for lower-risk categories (P = .4122), but it was borderline significant for higher-risk categories (P = .0678).ConclusionsIn this data set, prior hospital surgical volume tended to be associated with improved mortality after higher-risk operations in pediatric patients with congenital heart disease, whereas prior hospital postoperative mortality was significantly associated with mortality across all risk strata of congenital heart surgery

    Association of Digoxin With Interstage Mortality: Results From the Pediatric Heart Network Single Ventricle Reconstruction Trial Public Use Dataset

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    Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/139107/1/jah31279.pd

    Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children

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    Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction. The surviving patient had developmental delays. On the basis of this series, we suggest mitigating hypoxemia, and thus the risk of stroke, in patients who have sickle cell disease and cyanotic congenital heart disease. Potential therapies include chronic blood transfusions, hydroxyurea, earlier surgical correction to reduce the duration of hypoxemia, and heart or bone marrow transplantation

    Statistical Modeling of Extracellular Vesicle Cargo to Predict Clinical Trial Outcomes For Hypoplastic Left Heart Syndrome

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    Cardiac-derived c-kit+ progenitor cells (CPCs) are under investigation in the CHILD phase I clinical trial (NCT03406884) for the treatment of hypoplastic left heart syndrome (HLHS). The therapeutic efficacy of CPCs can be attributed to the release of extracellular vesicles (EVs). to understand sources of cell therapy variability we took a machine learning approach: combining bulk CPC-derived EV (CPC-EV) RNA sequencing and cardiac-relevan

    Features of portal hypertension are associated with major adverse events in Fontan patients: The VAST study

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    BACKGROUND: Chronic congestive hepatopathy is known to cause hepatic fibrosis and portal hypertension in patients post-Fontan operation for single ventricle palliation. The clinical significance of these findings is not clear. We hypothesized that features of portal hypertension would be significantly related to major adverse events. METHODS: A retrospective review of 73 adult and pediatric post-Fontan patients referred for a liver evaluation from 2001-2011 was performed. The relationship between features of portal hypertension (VAST score ≥2, 1 point each for Varices, Ascites, Splenomegaly or Thrombocytopenia) and a major adverse event (death, need for transplant, or hepatocellular carcinoma) was examined using logistic regression. RESULTS: 73 post-Fontan patients (30% female, 73% Caucasian, 66% systemic left ventricle (SLV), mean age 24 ±11 years, mean interval from Fontan 17 ±6 years) were included in analysis. Features of portal hypertension (VAST score ≥2) were present in 26 (36%), and there were 19 major adverse events: death (n=12), transplant (n=6), HCC (n=1). A significant relationship was found between VAST score ≥2 and major adverse events (OR=9.8, 95% CI [2.9-32.7]). After adjusting for time since Fontan, SLV, age, hemoglobin and type of failure, VAST score ≥2 remained significant (OR=9.1, 95% CI [1.4-57.6]). CONCLUSION: Fontan patients with features of portal hypertension have a 9-fold increased risk for a major adverse event. Therapies targeted to manage clinical manifestations of portal hypertension, and early referral to heart transplant may help delay major adverse events. Future prospective studies are needed to confirm these findings

    Satellite communications systems and technology. Executive Summary

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    NASA and the National Science Foundation (NSF) commissioned a panel of US experts to study the international status of satellite communications systems and technology. The study covers emerging systems concepts, applications, services, and the attendant technologies. The panel members travelled to Europe, Japan, and Russia to gather information first-hand. They visited 17 sites in Europe, 20 sites in Japan, and four in Russia. These included major manufacturers, government organizations, service providers, and associated R&D facilities. The panel's report was reviewed by the sites visited, by the panel, and by representatives of US industry. The report details the information collected and compares it to US activities

    Information and communication technology

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    NASA and the National Science Foundation (NSF) commissioned a panel of U.S. experts to study the international status of satellite communications systems and technology. The study covers emerging systems concepts, applications, services, and the attendant technologies. The panel members traveled to Europe, Japan, and Russia to gather information firsthand. They visited 17 sites in Europe, 20 in Japan, and 4 in Russia. These included major manufacturers, government organizations, service providers, and associated research and development facilities. The panel's report was reviewed by the sites visited, by the panel, and by representatives of U.S. industry. The report details the information collected and compares it to U.S. activities

    Hospital Costs Related to Early Extubation after Infant Cardiac Surgery

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    Background The Pediatric Heart Network Collaborative Learning Study (PHN CLS) increased early extubation rates after infant Tetralogy of Fallot (TOF) and coarctation (CoA) repair across participating sites by implementing a clinical practice guideline (CPG). The impact of the CPG on hospital costs has not been studied. Methods PHN CLS clinical data were linked to cost data from Children’s Hospital Association by matching on indirect identifiers. Hospital costs were evaluated across active and control sites in the pre- and post-CPG periods using generalized linear mixed effects models. A difference-in-difference approach was used to assess whether changes in cost observed in active sites were beyond secular trends in control sites. Results Data were successfully linked on 410/428 (96%) of eligible patients from 4 active and 4 control sites. Mean adjusted cost/case for TOF repair was significantly reduced in the post-CPG period at active sites (42,833vs.42,833 vs. 56,304, p<0.01) and unchanged at control sites (47,007vs.47,007 vs. 46,476, p=0.91), with an overall cost reduction of 27% in active vs. control sites (p=0.03). Specific categories of cost reduced in the TOF cohort included clinical (-66%, p<0.01), pharmacy (-46%, p=0.04), lab (-44%, p<0.01), and imaging (-32%, p<0.01). There was no change in costs for CoA repair at active or control sites. Conclusions The early extubation CPG was associated with a reduction in hospital costs for infants undergoing repair of TOF, but not CoA repair. This CPG represents an opportunity to both optimize clinical outcome and reduce costs for certain infant cardiac surgeries

    Hypoplastic Left Heart Syndrome Current Considerations and Expectations

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    In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients

    Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology

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    OBJECTIVES: To determine if neurodevelopmental deficits in children with single-ventricle physiology change with age and early developmental scores predict 6-year outcomes. METHODS: In the Single Ventricle Reconstruction Trial, Bayley Scales of Infant Development, Second Edition, were administered at 14 months of age, and parents completed the Behavior Assessment System for Children, Second Edition (BASC-2) annually from the ages of 2 to 6 years. Scores were classified as average, at risk, or impaired. We calculated sensitivities, specificities, and positive and negative predictive values of earlier tests on 6-year outcomes. RESULTS: Of 291 eligible participants, 244 (84%) completed the BASC-2 at 6 years; more Single Ventricle Reconstruction participants than expected on the basis of normative data scored at risk or impaired on the BASC-2 Adaptive Skills Index at that evaluation (28.7% vs 15.9%; P < .001). Children with Adaptive Skills Composite scores <2 SD below the mean at the age of 6 were more likely to have had delayed development at 14 months, particularly on the Psychomotor Development Index (sensitivity of 79%). However, the positive predictive value of the 14-month Mental Development Index and Psychomotor Development Index for 6-year BASC-2 Adaptive Scores was low (44% and 36%, respectively). Adaptive Skills Composite score impairments at the age of 6 were poorly predicted by using earlier BASC-2 assessments, with low sensitivities at the ages of 3 (37%), 4 (48%), and 5 years (55%). CONCLUSIONS: Many children with hypoplastic left heart syndrome who have low adaptive skills at the age of 6 years will not be identified by screening at earlier ages. With our findings, we highlight the importance of serial evaluations for children with critical congenital heart disease throughout development
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