The Heart Center at Nationwide Childrens Hospital

Columbus, OH, United States

The Heart Center at Nationwide Childrens Hospital

Columbus, OH, United States

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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 Liège | 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.


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.


Burnside J.,The Heart Center at Nationwide Childrens Hospital | Gomez D.,The Heart Center at Nationwide Childrens Hospital | Preston T.J.,The Heart Center at Nationwide Childrens Hospital | Olshove Vincent F. V.F.,The Heart Center at Nationwide Childrens Hospital | And 2 more authors.
Journal of Extra-Corporeal Technology | Year: 2011

During the course of extracorporeal membrane oxygenation, patients are at constant risk of exposure to air emboli. Air emboli may enter the circuit during routine lab sampling, medication administration, air entrainment through the venous cannula, or via a circuit disruption. Circuit components have been designed and positioned to minimize the quantity of air that travels through the arterial line to the patient. The purpose of this study was to assess the air handling of a newer generation extracorporeal life support circuit. The extracorporeal life support circuit consisted of an open hard-shell venous reservoir, Better Bladder (BB14) or silicone bladder (R-14), and Quadrox D ® oxygenator or 0800 silicone oxygenator. Air emboli detection sensors were placed in the extracorporeal life support circuit: post bladder, post oxygenator, and post heat exchanger if applicable. Air was injected as a 1 mL/min for 5 minutes injection or as a single 5 mL bolus. Emboli detection was recorded continuously during and for 3 minutes post air injection at two blood flow rates (Q b) (.5 and 1.2 L/min). All tests were performed in triplicate with each condition. All tested components reduced the embolic volume transmitted through the circuit. The quantity of this reduction was dependent on both the Q b and the air injection condition. During this in-vitro testing, air emboli passing through any of the components tested was decreased. Furthermore, the emboli delivery was reduced post component with the slower Q b (.5 L/min).


Tao G.,Molecular Cell and Developmental Biology Graduate Program | Tao G.,Nationwide Childrens Hospital | Tao G.,The Heart Center at Nationwide Childrens Hospital | Miller L.J.,Nationwide Childrens Hospital | 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..


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.


PubMed | The Heart Center at Nationwide Childrens Hospital
Type: | Journal: Congenital heart disease | Year: 2016

Enteral feeding is associated with decreased infection rates, decreased mechanical ventilation, decreased hospital length of stay, and improved wound healing. Enteral feeding difficulties are common in congenital heart disease. Our objective was to develop experience-based newborn feeding guidelines for the initiation and advancement of enteral feeding in the cardiothoracic intensive care unit.This is a retrospective analysis of a quality improvement project.This quality improvement project was performed in a cardiothoracic intensive care unit.Newborns admitted to the cardiothoracic intensive care unit for cardiac surgery from January 2011 to May 2015 were retrospectively reviewed.Newborn feeding guidelines for the initiation and advancement of enteral feeding were implemented in January 2012.Guideline compliance and clinical variables before and after guideline implementation were reviewed.Compliance with the guidelines increased from 83% in 2012 to 100% in the first two quarters of 2015. Preguidelines (January 2011-December 2011): 45 newborns underwent cardiac surgery; 8 deaths prior to discharge; 1 patient discharged from NICU, therefore, N=36. Postguidelines (January 2012-May 2015): 131 newborns with 12 deaths, 12 admitted from home, 8 in the NICU, 3 on the floor preop, and 3 back transferred, therefore, N=93. No difference in feeding preop (post 75% vs pre 69%; P=.5) or full po feeds at discharge (post 78% vs pre 89%; P=.2). Mesenteric ischemia was not statistically different postguidelines (post 6% vs pre 14%; P=.14). Length of hospital stay decreased postguidelines (post 27+17d vs pre 34+42d; P<.001).Implementation of experience-based newborn feeding guidelines for initiation and advancement of enteral feeding in the cardiothoracic intensive care unit was successful in reducing practice variation supported by increasing guideline compliance. Percentage of patients full oral feeding at discharge did not change. Length of hospital stay was reduced although cannot be fully attributed to feeding guideline implementation.


PubMed | The Heart Center at Nationwide Childrens Hospital, Ohio State University and Nationwide Childrens Hospital
Type: Journal Article | Journal: The journal of extra-corporeal technology | Year: 2016

This study assesses the effects of transfusion of autologous or allogeneic blood on cerebral and tissue oxygenation during spinal surgery. Packed red blood cell transfusions are indicated to improve oxygen delivery to tissues. There are limited data demonstrating changes in tissue oxygenation with blood administration. Tissue (deltoid) and cerebral oxygenation were monitored using near-infrared spectroscopy during spinal surgery in patients. As indicated, cell saver or allogeneic blood was administered. Tissue and cerebral oxygenation were recorded before and after transfusion. The study enrolled 50 patients, 33 of whom (17 males and 16 females) received allogeneic blood (


PubMed | The Heart Center at Nationwide Childrens Hospital
Type: Case Reports | Journal: The 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 childrens 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. Jehovahs 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 patients/familys 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-year-old male patient of the JW faith.


PubMed | The Heart Center at Nationwide Childrens Hospital, Nationwide Childrens Hospital Research Center, University of Washington and Ohio State University
Type: Journal Article | Journal: American journal of perinatology | Year: 2016

ObjectivesPulmonary vein stenosis (PVS) is a rare, often lethal anomaly associated with poor outcomes. Given the association between bronchopulmonary dysplasia (BPD) and cardiovascular complications, we tested the hypotheses that (1) a subgroup of neonates with severe BPD develop PVS (BPD-PVS) and have worse outcomes than do neonates with severe BPD alone (BPD); (2) among a cohort of neonates with severe BPD-associated pulmonary hypertension (BPD-PH), PVS is an additional risk factor for adverse outcomes and mortality. Study DesignWe performed a retrospective review of neonates with severe BPD, based on the Eunice Kennedy Shriver National Institute of Child Health and Development (NICHD) criteria, at our institution between June 1, 2009, and June 30, 2013. PVS was determined based on serial review of echocardiograms performed during their hospitalization. Neonates with congenital heart disease or chromosomal anomalies were excluded. ResultsOf 213 patients with severe BPD, 10 (4.7%) were found to have PVS (BPD-PVS). Neonates with BPD-PVS had lower birth weight (634178 vs. 767165g; p<0.01) and were more likely to be intrauterine growth restricted (80 vs. 11%; p<0.01) than neonates with BPD alone. Time on mechanical ventilation and length of hospitalization were longer in the BPD-PVS group than BPD group. Survival was lower in the BPD-PVS group than BPD group (5/10 [50%] vs. 196/203 [97%]; log-rank test p<0.01). Among a subgroup of neonates with BPD-PH, survival was lower among infants with PVS than those without PVS (5/9 [56%] vs. 26/30 [86%]; log-rank test p=0.01). ConclusionsCompared with neonates with severe BPD alone, those with acquired PVS are at increased risk for worse outcomes, including higher mortality. Evidence-based recommendations regarding screening protocols and surveillance are needed in this high-risk subgroup of BPD neonates.


PubMed | The Heart Center at Nationwide Childrens Hospital
Type: Journal Article | Journal: Congenital heart disease | Year: 2012

Percutaneous pulmonary valve implantation (PPVI) is an emerging therapy for pulmonary valve dysfunction. Minimal data on the midterm effects of PPVI on ventricular function exist. We describe the effects of PPVI on right and left ventricular (RV, LV) function with speckle tracking echocardiography.Patients who met the inclusion criteria of the Food and Drug Administration Phase 1 Feasibility Clinical Trial PPVI were identified. Patients were studied with echocardiograms at baseline, post-PPVI (day of discharge), 3 months, and at 6 months. Patients were studied by cardiac magnetic resonance at baseline and at 6 months. Longitudinal strain was measured at the basal, mid, and apical portions of the RV, interventricular septum (IVS), and LV. Global RV and LV strain and strain rates were recorded. Paired t-tests were used for analysis.Ten patients were analyzed: nine patients were a variant of tetralogy of Fallot and one patient had complex LV outflow obstruction requiring a Ross and RV-pulmonary atresia conduit. Mean age was 24.4 7.6 years. Indication for PPVI was pulmonary regurgitation in six patients, stenosis in two patients, and stenosis/regurgitation in two patients. After PPVI, both RV systolic pressure and RV to pulmonary artery pressure gradient significantly decreased. Cardiac magnetic resonance RV end-diastolic volume significantly decreased. IVS-mid, IVS-apical, and LV-global strain significantly increased and RV-basal decreased immediately after PPVI. Global RV a strain rate significantly increased immediately after PPVI. However, RV, IVS, and LV strain/strain rate values between baseline and the 6 month echocardiographic study were either similar or significantly decreased.Despite improvement in RV hemodynamics, there was a decrease or no improvement in RV and LV function as measured by strain echocardiographic values at midterm follow-up. Larger studies with longer follow-up are needed to determine if these results remain consistent.

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