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Preston T.J.,The Heart Center at Nationwide Childrens Hospital | Olshove Jr. V.F.,The Heart Center at Nationwide Childrens Hospital | Chase M.,Ohio State University
Journal of Extra-Corporeal Technology | Year: 2012

The successful use of prolonged extracorporeal life support with a heart-lung machine was first performed in 1972, as described by Hill et al., on a young man with post-traumatic respiratory failure. The first successful use of extracorporeal membrane oxygenation (ECMO) was 1976 by Bartlett et al. Since this time, the use of ECMO for neonatal and pediatric pulmonary support has become a standard of care in many children's hospitals. The use of ECMO, being a very invasive procedure, is not without risk. In our experience, most patients require multiple transfusions of the different blood components (packed red blood cells, plasma, platelets, and cryoprecipitate). Exposure to one or more blood products often occurs with connection to the ECMO circuit, as the circuit is generally primed with blood products or whole blood. Jehovah's Witnesses (JWs) are known best in the medical community for their refusal of blood products, even at the risk of death, which presents challenges for health care providers. This belief stems from the biblical passages that have been quoted as forbidding transfusion: Genesis 9:3-4, Leviticus 17:13-14, and Acts 15:19- 21. This refusal of blood poses even greater challenges when treating the pediatric JW population. When a blood product is deemed medically necessary for the JW patient, the healthcare provider must either seek legal intervention, or support the patient's/family's wishes and associated outcome. This ethical dilemma may be further complicated in the setting of therapies, which may pose additional risks and potentially less clear benefit such as with ECMO. Bloodless cardiac surgery with cardiopulmonary bypass has been reported in the JW population in adults and pediatrics, including neonates. After a thorough search of the literature, no published report of a JW patient being supported on ECMO without blood or blood component utilization was identified. This case report will present our experience with multiple day, bloodless ECMO support of a 17-yearold male patient of the JW faith. © 2011 AmSECT. Source


Ratliff T.M.,The Heart Center at Nationwide Childrens Hospital | Hodge A.B.,The Heart Center at Nationwide Childrens Hospital | Preston T.J.,The Heart Center at Nationwide Childrens Hospital | Galantowicz M.,Ohio State University | And 2 more authors.
Journal of Extra-Corporeal Technology | Year: 2014

Patients and parents of Jehovah's Witness (JW) faith present multiple challenges to a medical team, especially in the neonatal and pediatric population. The medical team must balance honoring the parents' request of not receiving blood products and fulfilling our commitment as advocates for the child's wellbeing. A multidisciplinary approach to cardiac surgery must be embraced for bloodless cardiopulmonary bypass (CPB) to be successful. At our institution, we have developed strategies and techniques for blood conservation that are used preoperatively, intraoperatively, and postoperatively for every CPB case with the goal of a bloodless procedure. These protocols include: preoperative erythropoietin, preoperative iron administration, selection of a CPB circuit specific to the patient's height and weight, acute normovolemic hemodilution, retrograde autologous prime and venous autologous prime, tranexamic acid administration, zerobalance ultrafiltration, flushing of the pump suckers post-CPB, modified ultrafiltration, and cell salvage.We present an 8-day-old, 3.2-kg patient of JW faith with aortic valve stenosis and regurgitation and a patent foramen ovale who underwent a bloodless left ventricle-to-aorta tunnel repair and aortic valve repair on CPB. Source


Barnette D.N.,Molecular and Cellular Pharmacology Graduate Program | Barnette D.N.,Center for Cardiovascular and Pulmonary Research at Nationwide Childrens Hospital Research Institute | Barnette D.N.,The Heart Center at Nationwide Childrens Hospital | Hulin A.,University of Liege | And 6 more authors.
Journal of Molecular and Cellular Cardiology | Year: 2013

Mature heart valves are complex structures consisting of three highly organized extracellular matrix layers primarily composed of collagens, proteoglycans and elastin. Collectively, these diverse matrix components provide all the necessary biomechanical properties for valve function throughout life. In contrast to healthy valves, myxomatous valve disease is the most common cause of mitral valve prolapse in the human population and is characterized by an abnormal abundance of proteoglycans within the valve tri-laminar structure. Despite the clinical significance, the etiology of this phenotype is not known. Scleraxis (Scx) is a basic-helix-loop-helix transcription factor that we previously showed to be required for establishing heart valve structure during remodeling stages of valvulogenesis. In this study, we report that remodeling heart valves from Scx null mice express decreased levels of proteoglycans, particularly chondroitin sulfate proteoglycans (CSPGs), while overexpression in embryonic avian valve precursor cells and adult porcine valve interstitial cells increases CSPGs. Using these systems we further identify that Scx is positively regulated by canonical Tgfβ2 signaling during this process and this is attenuated by MAPK activity. Finally, we show that Scx is increased in myxomatous valves from human patients and mouse models, and overexpression in human mitral valve interstitial cells modestly increases proteoglycan expression consistent with myxomatous mitral valve phenotypes. Together, these studies identify an important role for Scx in regulating proteoglycans in embryonic and mature valve cells and suggest that imbalanced regulation could influence myxomatous pathogenesis. © 2013 Elsevier Ltd. Source


Hodge A.B.,The Heart Center at Nationwide Childrens Hospital | Yeager C.J.,Medical University of South Carolina | Preston T.J.,The Heart Center at Nationwide Childrens Hospital | Savage A.J.,Medical University of South Carolina | And 2 more authors.
Journal of Extra-Corporeal Technology | Year: 2013

Acute right ventricular failure post heart transplantation in the pediatric population has not been well documented. Treatment using medical therapies including inotropes and nitric oxide are often inefficient for pediatric patients. Extracorporeal membrane oxygenation has been traditionally used in children until a long-term decision can be made. As a result of the emergence of smaller assist devices, pediatric practitioners now have more options available to treat this patient population. We describe the successful use of the Thoratec® CentriMag® in a pediatric patient posttransplantation with acute right ventricular failure. Source


Tao G.,Molecular Cell and Developmental Biology Graduate Program | Tao G.,Center for Cardiovascular and Pulmonary Research | Tao G.,The Heart Center at Nationwide Childrens Hospital | Miller L.J.,Center for Cardiovascular and Pulmonary Research | And 5 more authors.
Developmental Dynamics | Year: 2013

Background: Formation of the epicardium requires several cellular processes including migration, transformation, invasion, and differentiation in order to give rise to fibroblast, smooth muscle, coronary endothelial and myocyte cell lineages within the developing myocardium. Snai1 is a zinc finger transcription factor that plays an important role in regulating cell survival and fate during embryonic development and under pathological conditions. However, its role in avian epicardial development has not been examined. Results: Here we show that Snai1 is highly expressed in epicardial cells from as early as the proepicardial cell stage and its expression is maintained as proepicardial cells migrate and spread over the surface of the myocardium and undergo epicardial-to-mesenchymal transformation in the generation of epicardial-derived cells. Using multiple in vitro assays, we show that Snai1 overexpression in chick explants enhances proepicardial cell migration at Hamburger Hamilton Stage (HH St.) 16, and epicardial-to-mesenchymal transformation, cell migration, and invasion at HH St. 24. Further, we demonstrate that Snai1-mediated cell migration requires matrix metalloproteinase activity, and MMP15 is sufficient for this process. Conclusions: Together our data provide new insights into the multiple roles that Snai1 has in regulating avian epicardial development. Developmental Dynamics 242:699-708, 2013. © 2013 Wiley Periodicals, Inc.. Source

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