32 results on '"Fuller, Stephanie"'
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2. High-Risk Anatomic Subsets in Hypoplastic Left Heart Syndrome
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Geoffrion, Tracy R. and Fuller, Stephanie M.
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Despite overall improvements in outcomes for patients with hypoplastic left heart syndrome, there remain anatomic features that can place these patients at higher risk throughout their treatment course. These include severe preoperative obstruction to pulmonary venous return, restrictive atrial septum, coronary fistulae, severe tricuspid regurgitation, smaller ascending aorta diameter (especially if <2 mm), and poor ventricular function. The risk of traditional staged palliation has led to the development of alternative strategies for such patients. To further improve the outcomes, we must continue to diligently examine and study anatomic details in HLHS patients.
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- 2022
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3. Impact of Transcatheter Pulmonary Artery Intervention Following Superior Cavopulmonary Connection on Pulmonary Artery Growth
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Chaszczewski, Kasey J., Huang, Jing, Fuller, Stephanie, Smith, Christopher L., Dori, Yoav, Glatz, Andrew C., Gillespie, Matthew J., Rome, Jonathan J., and O’Byrne, Michael L.
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Introduction: Balloon and stent angioplasty of the pulmonary arteries (PAs) are frequently performed following superior cavopulmonary connection (SCPC), not only to normalize the caliber of the affected PA but also in hopes of maximizing downstream growth over time. There are limited data on the impact on subsequent PA growth prior to total cavopulmonary connection (TCPC).Methods: A single-center, retrospective cohort study was performed on children who underwent transcatheter (TC) PA intervention following SCPC between January 1, 2010, and December 31, 2018. Growth of treated and contralateral PAs was measured at the lobar bifurcation (distal branch PA [DBPA]) and in the proximal lower lobe (lower lobe branch [LLB]) on serial angiograms. Growth rate was evaluated using a mixed-effect model clustered by individual patient with an interaction term for treated PA and time to evaluate for differential growth rates between treated and contralateral PAs.Results: Thirty-five patients underwent TC PA intervention following SCPC, at a median of 70 days (interquartile range: 19-297 days) postoperatively. Significant growth was seen at both DBPA and LLB for raw (0.8 mm/year, 95% CI: 0.6-1.0, P< .001 for both) and body surface area (BSA) adjusted measures (8.4mm/m2/year, 95% CI: 5.6-11.2, P< .001; 7.9 mm/m2/year, 95% CI: 5.5-10.2, P< .001). The growth rate of the treated vessel was not significantly different from that of the contralateral vessel at the DBPA or LLB positions for raw (P= .71, .70) or BSA-adjusted measurements (P= .86, .64).Conclusion: Transcatheter PA intervention was associated with normal distal PA growth rate relative to the untreated side.
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- 2021
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4. I was videotaped by my landlord
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Fuller, Stephanie and Smith, Jennifer L.
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Voyeurism -- Personal narratives ,Fashion and beauty ,Health - Abstract
The author describes her shock when she discovered that her landlord had hidden a video camera above her bed.
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- 2002
5. A Technique for Safe Redo Sternotomy in Patients with Aortic Proximity to the Sternum
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Mavroudis, Constantine D., Smood, Benjamin, Grasty, Madison A., Fuller, Stephanie, and Desai, Nimesh D.
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The risk of redo sternotomy is greatly elevated in the setting of aortic proximity to the sternum. Current strategies to avoid catastrophic neurologic injury upon sternal reentry include establishment of peripheral bypass with the use of deep hypothermia and low-flow bypass, both of which may increase risk of neurologic complications. Here, we describe a technique for safe sternal reentry and illustrate its successful use in a patient with close proximity of the aorta to the sternum. With this technique, peripheral cardiopulmonary bypass is established prior to sternal reentry via cannulation of the right axillary artery and femoral vein, and the patient is cooled as the innominate artery is dissected, mobilized, and controlled. This permits the rapid institution of selective antegrade cerebral perfusion (SACP) in the event of aortic injury during sternal reentry. Once the innominate artery is isolated and SACP is initiated, one can safely complete the redo sternotomy, dissection, and distal ascending aortic cross-clamping to continue the operation without interruption in cerebral blood flow. This technique offers a safe approach in select patients and should be utilized in similar high-risk cases.
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- 2022
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6. Surgery for Adult Congenital Heart Disease
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Geoffrion, Tracy and Fuller, Stephanie
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Technical and medical improvements for congenital cardiac disease in children have contributed to an increasing population of patients who survive into adulthood. These patients may be prone to progression of their native palliated disease or suffer from sequelae of their childhood repair that requires repeat surgical intervention. Surgery for adult congenital cardiac disease poses unique challenges and risks.
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- 2020
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7. Too little too late? Communication with patients with congenital heart disease about challenges of adult life
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Deng, Lisa X., Gleason, Lacey P., Awh, Katherine, Khan, Abigail May, Drajpuch, David, Fuller, Stephanie, Goldberg, Leah A., Mascio, Christopher E., Partington, Sara L., Tobin, Lynda, Kovacs, Adrienne H., and Kim, Yuli Y.
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To investigate the experiences and communication preferences of adult patients with congenital heart disease (CHD) in the domains of employment, insurance, and family planning. Patients ≥ 18 years of age completed a questionnaire about experiences and communication preferences regarding employment, health insurance, and family planning. Of 152 patients (median age = 33 years, 50% female, 35% with CHD of great complexity), one in four reported work‐related problems due to CHD and a quarter also recalled a previous gap in health insurance. Of females, 29% experienced an unplanned pregnancy. The median importance of discussion ratings (on a 0‐10 scale) were 3.5 (employment), 6.0 (insurance), and 8.0 (family planning). Few patients recalled discussions about employment (19%) or health insurance (20%). Over half recalled discussions about family planning, although males were less likely to have had these discussions than females (24% vs 86%, P< .001). Across the three domains, patients identified 16‐18 years as the most appropriate age to initiate discussion, although for patients who recalled discussions, they typically occurred between 20 and 25 years. Adults with CHD commonly face employment, health insurance, and family planning challenges. However, discussions about these matters occur with less frequency than recommended and at older ages than patients would prefer. Communication about such issues should be incorporated into a comprehensive educational curriculum for adolescents during the process of transition to adult care.
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- 2019
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8. Psychological distress in adults with congenital heart disease: focus beyond depression
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Gleason, Lacey P., Deng, Lisa X., Khan, Abigail M., Drajpuch, David, Fuller, Stephanie, Ludmir, Jonathan, Mascio, Christopher E., Partington, Sara L., Tobin, Lynda, Kim, Yuli Y., and Kovacs, Adrienne H.
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AbstractBackgroundAdults with congenital heart disease face psychological challenges although an understanding of depression vs. anxiety symptoms is unclear. We analyzed the prevalence of elevated symptoms of anxiety and depression and explored associations with demographic and medical factors as well as quality of life.MethodsAdults with congenital heart disease enrolled from an outpatient clinic completed the Hospital Anxiety and Depression Scale and two measures of quality of life: the Linear Analogue Scale and the Satisfaction with Life Scale. Medical data were obtained by chart review.ResultsOf 130 patients (median age = 32 years; 55% female), 55 (42%) had elevated anxiety symptoms and 16 (12%) had elevated depression symptoms on subscales of the Hospital Anxiety and Depression Scale. Most patients with elevated depression symptoms also had elevated anxiety symptoms (15/16; 94%). Of 56 patients with at least one elevated subscale, 37 (66%) were not receiving mental health treatment. Compared to patients with 0 or 1 elevated subscales, patients with elevations in both (n=15) were less likely to be studying or working (47% vs. 81%; p=0.016) and reported lower scores on the Linear Analogue Scale (60 vs. 81, p<0.001) and the Satisfaction with Life Scale (14 vs. 28, p<0.001).ConclusionsAmong adults with congenital heart disease, elevated anxiety symptoms are common and typically accompany elevated depressive symptoms. The combination is associated with unemployment and lower quality of life. Improved strategies to provide psychosocial care and support appropriate engagement in employment are required.
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- 2019
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9. Risk factors for mortality in paediatric cardiac ICU patients managed with extracorporeal membrane oxygenation
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Achuff, Barbara-Jo, Elias, Matthew D., Ittenbach, Richard F., Ravishankar, Chitra, Nicolson, Susan C., Spray, Thomas L., Fuller, Stephanie, Gaynor, J. William, and O’Connor, Matthew J.
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AbstractBackgroundVeno-arterial extracorporeal membrane oxygenation is frequently used in patients with cardiac disease. We evaluated short-term outcomes and identified factors associated with hospital mortality in cardiac patients supported with veno-arterial extracorporeal membrane oxygenation.MethodsA retrospective review of patients supported with veno-arterial extracorporeal membrane oxygenation at a university-affiliated children’s hospital was performed.ResultsA total of 253 patients with cardiac disease managed with extracorporeal membrane oxygenation were identified; survival to discharge was 48%, which significantly improved from 39% in an earlier era (1995–2001) (p=0.01). Patients were categorised into surgical versus non-surgical groups on the basis of whether they had undergone cardiac surgery before or not, respectively. The most common indication for extracorporeal membrane oxygenation was extracorporeal cardiopulmonary resuscitation: 96 (51%) in the surgical group and 45 (68%) in the non-surgical group. In a multiple covariate analysis, single-ventricle physiology (p=0.01), duration of extracorporeal membrane oxygenation (p<0.01), and length of hospital stay (p=0.03) were associated with hospital mortality. Weekend or night shift cannulation was associated with mortality in non-surgical patients (p=0.05).ConclusionWe report improvement in survival compared with an earlier era in cardiac patients supported with extracorporeal membrane oxygenation. Single-ventricle physiology continues to negatively impact survival, along with evidence of organ dysfunction during extracorporeal membrane oxygenation, duration of extracorporeal membrane oxygenation, and length of stay.
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- 2019
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10. Pulmonary hypertension: Barrier or just a bump in the road in transplanting adults with congenital heart disease
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Menachem, Jonathan N., Birati, Edo Y., Zamani, Payman, Owens, Anjali T., Atluri, Pavan, Bermudez, Christian A., Drajpuch, David, Fuller, Stephanie, Kim, Yuli Y., Mascio, Christopher E., Palanivel, Vikram, Rame, J. Eduardo, Wald, Joyce, Acker, Michael A., and Mazurek, Jeremy A.
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Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Identification of disease progression and timing of referral for advanced therapies is often delayed. However, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience. However, in some cases, PH is a result of elevated systemic filling pressures and low output. We describe the approach used to successfully transplant an ACHD patient with severe pre‐HT PH performing HT alone. We review the literature and describe the one patient's journey from primarily palliative, to a combined heart‐lung transplant candidate, to successful HT patient. We discuss the methodology used to successfully transplant a patient, with significantly elevated pulmonary pressures and an initial pulmonary vascular resistance (PVR) > 13 Wood units. There are a number of complexities associated with the ACHD population and it is of utmost importance to carefully identify the underlying hemodynamic milieu and inform the appropriate treatment course in order to have successful transplant outcomes.
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- 2018
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11. Thoracic Surgery Considerations in the Child and Young Adult
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Atkins, Marvin D. and Fuller, Stephanie
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Vascular rings and slings may represent life-threatening compression of the esophagus and trachea. Such anatomic variants, although rare, are encountered by all thoracic surgeons in the scope of their practice at some time. The thoracic surgeon, whether treating such patients in the practice of congenital heart surgery, or in the practice of adult cardiac or thoracic surgery must have a requisite understanding of such anatomic variants, their diagnostic workup and radiologic interpretation, as well as their surgical management. Primary tracheobronchial disorders are also frequently encountered in the scope of a thoracic surgical practice and also are discussed.
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- 2018
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12. Long-Term Outcomes of Pediatric Cardiac Patients Supported by Extracorporeal Membrane Oxygenation
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Elias, Matthew D., Achuff, Barbara-Jo, Ittenbach, Richard F., Ravishankar, Chitra, Spray, Thomas L., Fuller, Stephanie, Montenegro, Lisa M., Gaynor, J. William, and O’Connor, Matthew J.
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- 2017
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13. Successful cardiac transplantation outcomes in patients with adult congenital heart disease
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Menachem, Jonathan N, Golbus, Jessica R, Molina, Maria, Mazurek, Jeremy A, Hornsby, Nicole, Atluri, Pavan, Fuller, Stephanie, Birati, Edo Y, Kim, Yuli Y, Goldberg, Lee R, and Wald, Joyce W
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ObjectivesThe purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT).BackgroundHeart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT.MethodsThe Penn Congenital Transplant Database comprises a cohort of patients with ACHD who underwent OHT between March 2010 and April 2016. We performed a retrospective cohort study of the 20 consecutive patients. Original cardiac diagnoses include single ventricle palliated with Fontan (n=8), dextro-transposition of the great arteries after atrial switch (n=4), tetralogy of Fallot (n=4), pulmonary atresia (n=1), Ebstein anomaly (n=1), unrepaired ventricular septal defect (n=1) and Noonan syndrome with coarctation of the aorta (n=1).ResultsEight patients required pretransplant inotropes and two required pretransplant mechanical support. Nine patients underwent heart–liver transplant and three underwent heart–lung transplant. Three patients required postoperative mechanical circulatory support. Patients were followed for an average of 38 months as of April 2016, with 100% survival at 30 days and 1 year and 94% overall survival (19/20 patients).ConclusionsACHD–OHT patients require highly specialised, complex and multidisciplinary healthcare. The success of our programme is attributed to using team-based, patient-centred care including our multidisciplinary staff and specialists across programmes and departments.
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- 2017
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14. A Comparison of Perioperative Management of Anomalous Aortic Origin of a Coronary Artery Between an Adult and Pediatric Cardiac Center
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Herrmann, Jeremy L., Goldberg, Leah A., Khan, Abigail M., Partington, Sara L., Brothers, Julie A., Mascio, Christopher E., Spray, Thomas L., Kim, Yuli Y., and Fuller, Stephanie
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Background: Anomalous aortic origin of a coronary artery (AAOCA) presents in varying age-groups. Assuming management algorithms differ between pediatric and adult institutions, we compared the perioperative management of patients with AAOCA at two such centers.Methods: A retrospective review was conducted at a pediatric and an adult institution of patients 14 years or older who underwent surgical repair of AAOCA between January 2000 and May 2014.Results: Twenty patients from the pediatric center (median age: 16.5 years, range: 14-18 years) and nine patients from the adult center (median age: 40 years, range: 37-52 years) were included. An anomalous aortic origin of a right coronary artery was the most frequent pathology at each institution. Chest pain was the most common presenting symptom at both institutions. Preoperative echocardiography was performed in 95% patients at the pediatric center and in 100% of patients at the adult center. Cardiac catheterization was utilized more frequently at the adult center, and cardiac magnetic resonance imaging more commonly employed at the pediatric center. Isolated coronary unroofing was performed in 19 of 20 cases at the pediatric center and in only 2 (22%) cases at the adult institution, both by congenitally trained cardiac surgeons. More concomitant cardiac procedures were performed at the adult center with associated longer operative times and hospital stays.Conclusion: Management strategies for AAOCA vary depending on both patient-specific factors and expertise of the managing team. Further studies are needed to optimally standardize diagnostic and treatment pathways regardless of location venue.
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- 2016
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15. Congenital Heart Surgery in Adults: The Challenge of Estimation of Risk of Mortality
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Fuller, Stephanie and Jacobs, Jeffrey P.
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- 2016
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16. Total Airway Reconstruction in the Neonate: Combined Mandibular Distraction and Slide Tracheoplasty for Multiple Level Airway Obstruction
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Basta, Marten N., Mudd, Pamela A., Fuller, Stephanie M., Javia, Luv R., and Taylor, Jesse A.
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- 2015
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17. Diuretic use in the adult Fontan
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Andrade, Lauren, Ortega-Legaspi, Juan M., Awh, Katherine, Fuller, Stephanie, Patel, Bhavesh, Tobin, Lynda, Wald, Joyce, and Kim, Yuli Y.
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To explore diuretic use in Fontan patients and its association with clinical features and adverse outcomes.
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- 2022
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18. Surgical revisions and mechanical support of the failing Fontan
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Fuller, Stephanie
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AbstractDespite its many anatomic modifications, Fontan failure remains prevalent in adult patients living with single-ventricle physiology. Although the Fontan operation has created a generation of survivors of complex single-ventricle disease, recognition of the limitation of this circulation is increasing. Most commonly, survivors suffer from ventricular dysfunction, protein-losing enteropathy, plastic bronchitis and arrhythmias. Although several medical therapies exist to palliate these long-term complications, surgical treatments are becoming increasingly useful, including the introduction of mechanical devices and cardiac transplantation. Currently, there are a variety of options available for mechanical support of the failing Fontan. Further developments in mechanical support technology will be required to tailor-make specific devices for this unique population. Owing to the fact that availability of donor hearts for transplantation is limited, mechanical support will play an increasingly important role in the management of complications of the Fontan circulation.
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- 2013
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19. Repair of Anomalous Left Coronary Artery From Pulmonary Artery in an Infant With Respiratory Syncytial Virus Bronchiolitis
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Kirsch, Roxanne E., DiMaria, Michael, Quartermain, Michael D., Meaney, Peter A., and Fuller, Stephanie
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In anomalous left coronary artery from the pulmonary artery (ALCAPA), infants 6 to 12 weeks will often present with symptoms consistent with reflux or bronchiolitis. In those infants diagnosed with both ALCAPA and concomitant active respiratory syncytial virus (RSV) bronchiolitis, others have reported delaying revascularization therapy until resolution of the RSV bronchiolitis. Here, we report the case of a three-month-old infant, diagnosed with ALCAPA and active RSV bronchiolitis, who underwent successful myocardial revascularization within 24 hours of presentation and diagnosis.
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- 2012
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20. Neonatal surgical reconstruction and peri-operative care for hypoplastic left heart syndrome: current strategies
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Fuller, Stephanie, Ghanayem, Nancy, Chai, Paul, Nigro, John, Kleisli, Thomas, and Tabbutt, Sarah
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AbstractThe management of newborns with hypoplastic left heart syndrome has changed significantly over the past three decades, with an associated dramatic improvement in outcomes. The aim of this paper is to discuss current peri-operative and palliative surgical strategies. Owing to the fact that comparative outcomes for these strategies have been addressed in a limited number of prospective trials and extractions from multi-centred databases, the primary focus of this review is descriptive.
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- 2011
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21. Abstract 11786: Heart Transplant in Congenital Heart Disease: Trends in Mortality With Ventricular Assist Devices
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Kamsheh, Alicia M, Burstein, Danielle, Zhang, Xuemei, Ampah, Steve, Edelson, Jonathan, Lin, Kimberly, O'Connor, Matthew J, Ravishankar, Chitra, Wittlieb-Weber, Carol, Maeda, Katsuhide, Nuri, Muhammad, Gaynor, James W, Chen, Jonathan, Fuller, Stephanie M, and Rossano, Joseph
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Introduction:Patients with congenital heart disease (CHD) may require cardiac transplantation for either primary management or failed surgical palliation. This study describes 1-year mortality of patients with CHD listed for transplant, trends in 1-year mortality over three decades, and use of mechanical support in this population.Methods:The United Network for Organ Sharing database was queried to identify patients aged 0-30 years with CHD listed for transplant from 1990 to 2019. VAD status at listing and 1-year mortality after listing were examined using the Cochran-Armitage trend test.Results:Overall, 9327 patients with CHD aged 0-30 years were listed for transplant between 1990 and 2019 with a total 1-year mortality rate of 26% (n=2466). For non-survivors to 1-year after listing, 66% (n=1619) died before transplant and 34% (n=847) after transplant. Patients <1 year of age were the most frequent age group listed for transplant (n=3678, 39%) and experienced the highest 1-year mortality after listing (n=1264, 34%). The 1-year overall mortality decreased over the study period (1990-1999: 33% vs 2000-2009: 30% vs 2010-2019: 19%, p<0.0001), including in infants <1 year of age (1990-1999: 41% vs 2000-2009: 37% vs 2010-2019: 26%, p<0.0001). Overall, few patients were supported with VAD at listing (3.1%). However, VAD use increased (1990-1999: 1.0% vs 2000-2009: 2.8% vs 2010-2019: 5.0%, p< 0.0001) and the 1-year mortality for patients with VAD at listing decreased over time (1990-1999: 57% vs 2000-2009: 35% vs 2010-2019: 20%, p<0.0001).Conclusions:Survival after listing for heart transplant is improving, however mortality amongst infants remains high. While there has been >50% improvement in survival in patients of all ages with CHD supported with a VAD at listing, VADs are used rarely in patients with CHD. Further study is needed to understand which patients may benefit from mechanical circulatory support, especially among infants.
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- 2021
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22. Abstract 12768: Development of a Clinical 3D Modeling Program to Guide Pediatric Cardiothoracic Surgery and Intervention
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Ghosh, Reena M, Jolley, Matthew, Mascio, Christopher, Chen, Jonathan M, Fuller, Stephanie M, Rome, Jonathan J, Silvestro, Elizabeth, and Whitehead, Kevin K
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Introduction:Surgical and catheter-based interventions for congenital heart disease require precise understanding of complex anatomy. The use of three-dimensional (3D) visualization techniques to enhance visuospatial understanding has been well documented, but integration of these methods into routine clinical practice has not been well described. We report the growth and development of a clinical 3D modeling service to inform procedural planning within a high-volume pediatric heart center.Methods:Clinical 3D modeling was performed using cardiac magnetic resonance (CMR) or computed tomography (CT) derived data. Image segmentation and post-processing was performed using FDA-approved software. Patient-specific anatomy was visualized using 3D printed models, digital flat screen models and virtual reality. Surgical repair options were digitally designed using proprietary and open-source computer aided design (CAD) based modeling tools.Results:From 2018 to 2020 there were 112 individual 3D modeling cases performed, 16 for educational purposes and 96 clinically utilized for procedural planning. Over the 3-year period, demand for clinical modeling tripled and in 2020, 3D modeling was requested in more than one-quarter of STAT category 3, 4 and 5 cases. The most common indications for modeling were complex biventricular repair (n = 30, 31%) and repair of multiple ventricular septal defects (VSD) (n=11, 12%).Conclusions:Using a multidisciplinary approach, clinical application of 3D modeling can be seamlessly integrated into pre-procedural care for patients with congenital heart disease. Rapid expansion and increased demand for utilization of these tools within a high-volume center demonstrate the high value conferred on these techniques by surgeons and interventionalists alike.
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- 2021
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23. Reproductive Hormones Modulate Oxidative Stress in Alzheimer's Disease
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Barron, Anna M., Fuller, Stephanie J., Verdile, Giuseppe, and Martins, Ralph N.
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Alzheimer's disease (AD) is a neurodegenerative disease characterized by gradual cognitive decline, impairments in speech and language, and dysfunction in the sensorimotor systems, culminating in complete reliance on nursing care. Oxidative stress, caused by an imbalance in the pro-oxidant/antioxidant mechanisms in the body, has been implicated in AD pathogenesis, as in many other age-associated diseases such as atherosclerosis, Parkinson disease, and amyotrophic lateral sclerosis. Although the hormones estrogen, progesterone, testosterone, and luteinizing hormone are best known for their roles in reproduction, many studies show these hormones have other roles, including neuroprotection. Changes in the levels of these hormones that occur in reproductive senescence are hypothesized to increase risk of AD, as a result of reduced protection against oxidative insults. The Aβ peptide, overproduction of which is thought to be a key pathogenic event in the development of AD, is neurotoxic, most likely due to its ability to promote oxidative stress. The reproductive hormones are known to influence Aβ metabolism, and this review discusses the beneficial and detrimental effects these hormones have on Aβ production and oxidative stress, and their relevance in potential AD therapies.
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- 2006
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24. The amyloid precursor protein of Alzheimer disease in human brain and blood
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Li, Qiao‐Xin, Fuller, Stephanie J., Beyreuther, Konrad, and Masters, Colin L.
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Studies of the metabolism and function of the amyloid precursor protein (APP) and its proteolytic fragment Aβ in cultured cells, transgenic mice, and post‐mortembrain tissue have advanced our understanding of Alzheimer disease (AD). However, the molecular pathogenesis of the disease is still not clear, and we are a long way from finding a cure for the disease. Studies carried out on human platelets and leukocytes have also helped shed light on APP and Aβ metabolism and function. Platelet and leukocyte APP isoforms are processed using mechanisms similar to those in neuronal cells to generate Aβ and soluble forms of APP. The activation of platelets and leukocytes leads to the secretion of APP and Aβ, resulting in higher levels of these proteins in serum. APP and Aβ in the circulation may be involved in the regulation of platelet function and in the modulation of immune responses. Because human platelets and lymphocytes produce all forms of APP and secrete amyloidogenic Aβ peptides, these tissues may be useful in monitoring responses to therapeutic interventions directed at APP metabolism. Although not of neuronal origin, further studies on the more accessible ex vivotissues, including platelets and leukocytes and other blood components, may reveal potential peripheral markers for AD and will further our understanding of the molecular pathogenesis of AD. J. Leukoc. Biol.66: 567–574; 1999.
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- 1999
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25. Soluble pool of Aβ amyloid as a determinant of severity of neurodegeneration in Alzheimer's disease
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McLean, Catriona A., Cherny, Robert A., Fraser, Fiona W., Fuller, Stephanie J., Smith, Margaret J., Vbeyreuther, Konrad, Bush, Ashley I., and Masters, Colin L.
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Genetic evidence strongly supports the view that Aβ amyloid production is central to the cause of Alzheimer's disease. The kinetics, compartmentation, and form of Aβ and its temporal relation to the neurodegenerative process remain uncertain. The levels of soluble and insoluble Aβ were determined by using western blot techniques, and the findings were assessed in relation to indices of severity of disease. The mean level of soluble Aβ is increased threefold in Alzheimer's disease and correlates highly with markers of disease severity. In contrast, the level of insoluble Aβ (also a measure of total amyloid load) is found only to discriminate Alzheimer's disease from controls, and does not correlate with disease severity or numbers of amyloid plaques. These findings support the concept of several interacting pools of Aβ, that is, a large relatively static insoluble pool that is derived from a constantly turning over smaller soluble pool. The latter may exist in both intracellular and extracellular compartments, and contain the basic forms of Aβ that cause neurodegeneration. Reducing the levels of these soluble Aβ species by threefold to levels found in normal controls might prove to be a goal of future therapeutic intervention.
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- 1999
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26. Gelatinase A possesses a β-secretase-like activity in cleaving the amyloid protein precursor of Alzheimer's disease
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LePage, Rex N., Fosang, Amanda J., Fuller, Stephanie J., Murphy, Gillian, Evin, Geneviève, Beyreuther, Konrad, Masters, Colin L., and Small, David H.
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The ability of the 72 kDa gelatinase A to cleave the amyloid protein precursor (APP) was investigated. HeLa cells were transfected with an APP 695plasmid. The cells were incubated with gelatinase A, which cleaved the 110 kDa cell-surface APP, releasing a 100 kDa form of the protein. A peptide homologous to the β-secretase site was cleaved by gelatinase A adjacent to a glutamate residue at position −3 (βA4 numbering system). A peptide homologous to the α-secretase site was not cleaved. The results demonstrate that 72 kDa gelatinase A is not an α-secretase, but that it may have a β-secretase activity.
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- 1995
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27. Abstract 11629: Risk Factors for Complications Following Cardiac Surgery at Children?s Hospitals in Adults With Congenital Heart Disease
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Salciccioli, Katherine B, Guffey, Danielle, Ghanayem, Nancy, Lasa, Javier J, Gaies, Michael G, Fuller, Stephanie M, Kim, Francis, Smith, Andrew, Cotts, Timothy, Causey, Jamie, Ermis, Peter R, and Jain, Parag
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Background:Increasing numbers of adult congenital heart disease (ACHD) patients undergo cardiac surgery in children?s hospitals. Surgical outcomes data for ACHD patients at pediatric hospitals are limited.Objective:To identify predictors of prolonged cardiac intensive care unit (CICU) length of stay (pLOS) and major postoperative complications (mComp) following ACHD cardiac surgery at pediatric hospitals.Methods:Surgical encounters of patients >18 years in the Pediatric Cardiac Critical Care Consortium (PC4) registry (8/2014-1/2019; 34 hospitals) were included. Primary outcomes included pLOS (defined as LOS ? 90thpercentile) and mComp (cardiac arrest, ECMO, arrhythmia requiring intervention, stroke, renal replacement therapy, infection, reoperation/reintervention).Results:A total of 1773 surgical encounters were analyzed. The pLOS cutoff was >7 days. Eighteen patients (1.0%) died during hospitalization, 9 (0.5%) of whom died before the pLOS cutoff and were excluded from analysis. Of 1764 encounters, 8.8% (n=156) had pLOS and 23.3% (n=413) had >1 mComp. Predictors of primary outcomes identified in multivariable analysis are shown in Figure 1. STAT 4/5 operation, >3 previous sternotomies, and preoperative renal dysfunction/dialysis were significant risk factors for both pLOS and complications. Preoperative ventilation increased odds of pLOS while preoperative arrhythmias increased odds of mCOMP.Conclusions:In this analysis of postoperative ACHD care in pediatric CICUs contributing data to PC4, preoperative arrhythmias, renal dysfunction, and respiratory failure are potentially modifiable factors associated with pLOS and/or mComp. STAT 4/5 procedures and >3 previous sternotomies were also associated with both pLOS and mComp. Future quality improvement initiatives focused on preoperative optimization and implementation of adult-specific perioperative protocols may mitigate morbidity in this growing patient population.
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- 2019
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28. Abstract 14367: Diuretic Use is Associated With Worse Transplant-Free Survival in Adult Fontan Patients
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Ortega-Legaspi, Juan M, Awh, Katherine, Patel, Bhavesh, Ramachandran, Abhinay, Wald, Joyce, Denduluri, Srinivas, Archer, Alexandra, Drajpuch, David, Fuller, Stephanie, Mascio, Christopher, Ruckdeschel, Emily, Tobin, Lynda, and Kim, Yuli Y
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Background:Single ventricle physiology can lead to heart failure necessitating diuretics, yet diuretic use and its prognostic value has not been fully explored in the adult Fontan patient. We aim to examine diuretic use in this population and its association with adverse outcomes.Methods:Single center retrospective review of Fontan patients ? 18 years from 2005-2017. Diuretic use at most recent outpatient clinic visit was recorded. Outcomes of interest were heart transplantation and death. Comparison of Fontan patients by diuretic use was performed using Chi-square and Wilcoxon rank-sum tests. Kaplan-Meier survival analysis stratified by diuretic use was performed to examine time to transplant or death. Cox Proportional Hazards models examined risk of outcome based on diuretic use.Results:Of 167 Fontan patients (age 30 [range 21 - 60] years, 49% female), 69 (41%) were taking diuretics at their last outpatient visit. Patients on diuretics were older, symptomatic, underwent Fontan surgery at an older age, and had more comorbidities (Table 1). During a median follow-up of 4.3 years (0.1 - 14.8), 16 patients underwent transplant or died (9.6%). Kaplan-Meier survival analysis showed significantly lower transplant-free survival for Fontan patients taking diuretics (Figure 1). Diuretic use was associated with a 17-fold increased risk of transplant or death (HR 17.4, 95% CI 2.3 - 131.9; p=0.006).Conclusion:Diuretic use is seen in older, symptomatic adult Fontan patients with comorbidities and is associated with worse transplant-free survival. Use of diuretics signals the onset of clinical heart failure and is prognostic of poor outcomes.
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- 2019
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29. Abstract 15190: Predictors for Seizures in Neonates Following Surgery With Cardiopulmonary Bypass
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Naim, Maryam Y, Massey, Shavonne L, Costarino, Andrew T, Fuller, Stephanie, Gaynor, J. William, Kimmel, Stephen E, Licht, Daniel J, Mascio, Christopher E, Nicolson, Susan C, Putt, Mary, and Abend, Nicholas S
- Abstract
Introduction:Electrographic seizures (ES) are an important predictor of neurodevelopmental outcome in neonates undergoing cardiopulmonary bypass (CPB). Following implementation of postoperative continuous electroencephalography (CEEG) in 161 neonates, we previously reported an 8% ES incidence, 85% were EEG-only, and ES were associated with deep hypothermic circulatory arrest (DHCA) and delayed sternal closure. Now we aim to identify predictors of ES to permit evidence-based targeted CEEG.Methods:A retrospective study of neonates who underwent surgery with CPB from 2012 through 2018 was performed. Potential ES predictors were evaluated using logistic regression. Model performance was assessed using receiver operating characteristic (ROC) curves.Results:Of 739 of neonates monitored with CEEG, 59 (8%) had ES that started at a median of 25 hours postoperatively (Table). ES were EEG-only in 49 (83%), and constituted status epilepticus in 13 (22%). Patients with ES had higher mortality (16 of 59, 27% vs. 31 of 646, 5%; p<0.001). In univariable analyses gestational age, head circumference, prenatal diagnosis, cardiac defect, CPB duration, DHCA, regional cerebral perfusion, extracorporeal membrane oxygenation, cardiac arrest, inhaled nitric oxide (iNO) and delayed sternal closure predicted ES (p <0.1). In multivariable analysis iNO and delayed sternal closure were significant predictors. The area under the ROC curve for this model was 0.76. Using a cut-point to emphasize negative predictive value (NPV) and sensitivity, 519 of 729 (71%) would have been selected for CEEG, including 56 of 59 (95%) with ES (NPV 99%, positive predictive value of 11%, sensitivity 95%, specificity 32%).Conclusions:ES occurred in 8% of neonates following CPB. ES were associated with iNO use and delayed sternal closure. Since no preoperative or operative factors predicted ES, and the multivariable model had only fair performance, CEEG of all neonates following CBP is recommended.
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- 2019
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30. Making in metal.
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Fuller, Stephanie
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METALWORKING education , *ADULT education finance - Abstract
Presents a selective survey of metal working courses to examine the changes that had taken place in the provision of part-time learning opportunities following the restructuring of funding for adult education. Reason why metalworking crafts are vulnerable to financial pressures; How the survey was conducted; Reasons why adult students participate in metalworking classes; Several common points of metalworking courses.
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- 1998
31. Surgical and Catheter-Based Reinterventions Are Common in Long-Term Survivors of the Fontan Operation
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Downing, Tacy E., Allen, Kiona Y., Goldberg, David J., Rogers, Lindsay S., Ravishankar, Chitra, Rychik, Jack, Fuller, Stephanie, Montenegro, Lisa M., Steven, James M., Gillespie, Matthew J., Rome, Jonathan J., Spray, Thomas L., Nicolson, Susan C., Gaynor, J. William, and Glatz, Andrew C.
- Abstract
Supplemental Digital Content is available in the text.
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- 2017
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32. Of hemorrhagic shock, spherical cows and Aloe vera
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Gutierrez, Guillermo and Fuller, Stephanie
- Abstract
The central question explored in this commentary is whether the beneficial effects of an Aloe vera derived drag-reducing polymer during hemorrhagic shock is due to its O2radical scavenging properties or to changes in blood rheology.
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- 2004
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