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Jeffrey Robbins, PhD Director and Endowed Chair, Molecular Cardiovascular Biology
Director and Endowed Chair, Molecular Cardiovascular Biology
Executive Co-Director, Heart Institute
Associate Chair, Children's Hospital Research Foundation
Professor, UC Department of Pediatrics
Structure function relationships for the contractile proteins; cardiac-specific gene manipulation in transgenic rabbits; the contractile protein myosin, and human heart failure; molecular studies of human valve disease
Visit the Robbins Lab.
Gupta MK, Robbins J. Post-translational control of cardiac hemodynamics through myosin binding protein C. Pflugers Arch: European journal of physiology. 2014 Feb;466(2):231-6.
Mun JY, Previs MJ, Yu HY, Gulick J, Tobacman LS, Beck Previs S, Robbins J, Warshaw DM, Craig R. Myosin-binding protein C displaces tropomyosin to activate cardiac thin filaments and governs their speed by an independent mechanism. Proc Natl Acad Sci U S A. 2014 Feb 11;111(6):2170-5.
Sandri M, Robbins J. Proteotoxicity: An underappreciated pathology in cardiac disease. J Mol Cell Cardiol. 2014 Jun;71c:3-10.
Wang X, Robbins J. Proteasomal and lysosomal protein degradation and heart disease. J Mol Cell Cardiol. 2014 Jun;71c:16-24.
James J, Robbins J. Ablating a cardiac protein: Causality at last. Circ Res. 2013;112:1415-1419.
Razzaque MA, Gupta M, Osinska H, Gulick J, Blaxall BC, Robbins J. An endogenously produced fragment of cardiac myosin-binding protein C is pathogenic and can lead to heart failure. Circ Res. 2013 Aug 16;113(5):553-61.
Gupta MK, Gulick J, James J, Osinska H, Lorenz JN, Robbins J. Functional dissection of myosin binding protein C phosphorylation. J Mol Cell Cardiol. 2013;64C:39-50.
Bhuiyan MS, Pattison JS, Osinska H, James J, Gulick J, McLendon PM, Hill JA, Sadoshima J, Robbins J. Enhanced autophagy ameliorates cardiac proteinopathy. J Clin Invest. 2013;123(12):5284-97.
Pattison JS, Robbins J. Desmin and heart disease. In: Kavallaris M, ed. Cytoskeleton and human disease. New York, NY: Humana Press; 2012:411-424.
Bhuiyan MS, Gulick J, Osinska H, Gupta M, Robbins J. Determination of the critical residues responsible for cardiac myosin binding protein C's interactions. J Mol Cell Cardiol. 2012;53:838-847.
Cardiac Signaling in the Normal and Abnormal Heart. Principal Investigator. National Heart, Lung, and Blood Institute. Sep 2013-May 2018. #P01 HL69779.
Proteotoxicity: an underappreciated factor in cardiac disease. North American Coordinator. Leducq Transatlantic Alliance For Cardiovascular Disease. 2011-2016.
Thrombospondin 4 Regulates Adaptive ER Stress Response. Co-Principal Investigator. National Heart, Lung, and Blood Institute. Jan 2011-Dec 2014. #R01 HL105924.
Cardiac hypertrophy intracellular signaling pathways. Co-Principal Investigator. National Heart, Lung, and Blood Institute. Jan 2009-Dec 2014. #R01 HL1062927.
Jeffrey A. Towbin, MD, FAAP, FACC, FAHA Director, Heart Institute Diagnostic Laboratory
is a leading expert in cardiomyopathy and had authored numerous papers on cardiomyopathy and heart failure.
Director, Heart Institute Diagnostic Laboratory
BS: Biology, University of Cincinnati, 1974.MS: Cell Biology, University of Cincinnati, 1977.MD: University of Cincinnati, 1982.
Residency: Pediatrics, Cincinnati Children's Hospital Medical Center, 1985.
Fellowship: Pediatric Cardiology, Baylor College of Medicine, Texas Children's Hospital, 1989.
McBride KL, Zender GA, Fitzgerald-Butt SM, Seagraves NJ, Fernbach SD, Zapata G, Lewin M, Towbin JA, Belmont JW. Association of common variants in ERBB4 with congenital left ventricular outflow tract obstruction defects. Birth Defects Res A Clin Mol Teratol. 2011 Mar;91(3):162-8.
Fakhro KA, Choi M, Ware SM, Belmont JW, Towbin JA, Lifton RP, Khokha MK, Brueckner M. Rare copy number variations in congenital heart disease patients identify unique genes in left-right patterning. Proc Natl Acad Sci U S A. 2011 Feb 15;108(7):2915-20.
Goldenberg I, Horr S, Moss AJ, Lopes CM, Barsheshet A, McNitt S, Zareba W, Andrews ML, Robinson JL, Locati EH, Ackerman MJ, Benhorin J, Kaufman ES, Napolitano C, Platonov PG, Priori SG, Qi M, Schwartz PJ, Shimizu W, Towbin JA, Vincent GM, Wilde AA, Zhang L. Risk for life-threatening cardiac events in patients with genotype-confirmed long-QT syndrome and normal-range corrected QT intervals. J Am Coll Cardiol. 2010 Dec 28;57(1):51-9.
Towbin JA, Ware SM, Jefferies JL. Heart transplants in pediatric patients: viral infection as a loss predictor. Future Cardiol. 2010 Nov;6(6):735-41.
Foerster SR, Canter CE, Cinar A, Sleeper LA, Webber SA, Pahl E, Kantor PF, Alvarez JA, Colan SD, Jefferies JL, Lamour JM, Margossian R, Messere JE, Rusconi PG, Shaddy RE, Towbin JA, Wilkinson JD, Lipshultz SE. Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood: an outcomes study from the Pediatric Cardiomyopathy Registry. Circ Heart Fail. 2010 Nov 1;3(6):689-97.
Purevjav E, Varela J, Morgado M, Kearney DL, Li H, Taylor MD, Arimura T, Moncman CL, McKenna W, Murphy RT, Labeit S, Vatta M, Bowles NE, Kimura A, Boriek AM, Towbin JA. Nebulette mutations are associated with dilated cardiomyopathy and endocardial fibroelastosis. J Am Coll Cardiol. 2010 Oct 26;56(18):1493-502. Towbin JA. Left ventricular noncompaction: a new form of heart failure. Heart Fail Clin. 2010 Oct;6(4):453-69 Towbin JA. Preface: Heart failure in children. Heart Fail Clin. 2010 Oct;6(4):xvii-iii.
Wilkinson JD, Landy DC, Colan SD, Towbin JA, Sleeper LA, Orav EJ, Cox GF, Canter CE, Hsu DT, Webber SA, Lipshultz SE. The pediatric cardiomyopathy registry and heart failure: key results from the first 15 years. Heart Fail Clin. 2010 Oct;6(4):401-13 Towbin JA. Hypertrophic cardiomyopathy. Pacing Clin Electrophysiol. 2009 Jul;32 Suppl 2:S23-31.
Jeffrey B. Anderson, MD, MPH
is an electrophysiologist with a clinical interest in heart rhythm abnormalities and syncope in pediatric patients. His has a research interest in quality improvement and outcomes in infants and children with congenital heart disease. He is currently working on projects to improve medical management of pediatric patients presenting with syncope as well as to improve nutrition and growth in infants with complex congenital heart disease.
Assistant Professor, UC Department of Pediatrics
Pediatric electrophysiology; Syncope; Quality improvement and outcomes
Syncope, nutrition and growth in congenital heart disease, large database analysis, quality improvement
Jeffrey B. Anderson MD, MPH, is an assistant professor of pediatrics with the University of Cincinnati College of Medicine and holds a joint appointment with the Heart Institute and the James M. Anderson Center for Clinical Excellence at Cincinnati Children’s Hospital Medical Center.
Dr. Anderson obtained both his medical degree and his Masters in Public Health from the University of Utah School of Medicine. He completed an internship in internal medicine and pediatrics at the North Carolina Memorial Hospital followed by a pediatrics residency at the North Carolina Children’s Hospital in Chapel Hill, NC. Following a year serving as pediatric chief resident in Chapel Hill, he completed his pediatric cardiology fellowship and advanced training in electrophysiology at Cincinnati Children’s Hospital Medical Center.
Dr. Anderson’s research interests include nutrition in congenital heart disease, syncope in the pediatric population and application of quality improvement methodology to improve outcomes in congenital heart disease. Dr. Anderson has spent the last several years working with infants with congenital heart disease with nutritional failure to better understand the etiology of their growth problems and to design interventions to improve their growth.
Following cardiology fellowship, Dr. Anderson helped establish the Syncope Clinic in the Heart Institute at Cincinnati Children’s Hospital Medical Center, and he continues to direct the work in this clinic.
Anderson JB, Kalkwarf HJ, Kehl JE, Eghtesady P, Marino BS. Low Weight-for-Age Z-Score and Infection Risk After the Fontan Procedure. Ann Thorac Surg. 2011 May;91(5):1460-6.
Anderson JB, Marino BS, Irving SY, García-España JF, Ravishankar C, Stallings VA, Medoff-Cooper B. Poor post-operative growth in infants with two-ventricle physiology. Cardiol Young. 2011 Mar 9:1-9.
Anderson JB, Benson DW. Genetics of sick sinus syndrome. Card Electrophysiol Clin. 2010 Dec 1;2(4):499-507.
Czosek RJ, Anderson JB, Cao J, Knilans TK. Assessment of T-wave oversensing in an infant with an implanted defibrillator. Heart Rhythm. 2010 Oct;7(10):1516-7.
Anderson JB, Beekman RH 3rd, Eghtesady P, Kalkwarf HJ, Uzark K, Kehl JE, Marino BS. Predictors of poor weight gain in infants with a single ventricle. J Pediatr. 2010 Sep;157(3):407-13, 413.e1. Dixon CA, Anderson JB, Ruddy RM, Cripe LH. Infantile-onset Pompe disease: a diagnosis not to miss. Pediatr Emerg Care. 2010 Apr;26(4):293-5. Anderson JB, Beekman RH 3rd, Border WL, Kalkwarf HJ, Khoury PR, Uzark K, Eghtesady P, Marino BS. Lower weight-for-age z score adversely affects hospital length of stay after the bidirectional Glenn procedure in 100 infants with a single ventricle. J Thorac Cardiovasc Surg. 2009 Aug;138(2):397-404.e1.
Anderson J, Kocis K. Echocardiographic evaluation of pericardial effusions and cardiac tamponade in children. Pediatric Ultrasound Today. 2006; 11(8): 137-52. Anderson JB, Fuller TC, Hawkins JA, Brinkman MK, Profaizer T, Shaddy MD. Long-term reduction of panel reactive antibodies in children receiving mycophenolate mofetil after valved allograft placement. Transplantation. 2005 Aug 15;80(3):414-6. Shaddy RE, Fuller TC, Anderson JB, Lambert LM, Brinkman MK, Profaizer T, Hawkins JA. Mycophenolic mofetil reduces the HLA antibody response of children to valved allograft implantation. Ann Thorac Surg. 2004 May;77(5):1734-9.
Robert H. Beekman III, MD
is a recognized leader in pediatric cardiology and interventional cardiology. He’s currently researching participation in multicenter trials of stent therapy for coarctation (COAST), covered stents for coarctation and aortic aneurysms, and the C3PO Registry. Dr. Beekman is also chair of the JCCHD National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) and a member of the Steering Committee of the ACC/NCDR IMPACT Registry.
Cardiac catheterization; intervention for congenital heart disease; quality improvement
Robert H. Beekman III, MD, is currently a professor of pediatric cardiology at Children's Hospital Medical Center of Cincinnati, Ohio. Dr. Beekman's subspecialty interests involve cardiac catheterization and interventional cardiology, and clinical quality improvement.
A native of California, Dr. Beekman graduated from Occidental College in Los Angeles, and earned his MD from Duke University in Durham, NC. Dr. Beekman completed his pediatric internship and residency at UCLA Medical Center, and his pediatric cardiology fellowship at the University of Michigan Medical Center, Ann Arbor, MI. Dr. Beekman also obtained a master's degree in statistical analysis and research design from the University of Michigan. From 1985 to 1996, Dr. Beekman was a member of the Division of Pediatric Cardiology at the University of Michigan School of Medicine. From 1996-2006 he was director of Cardiology at Children's Hospital Medical Center of Cincinnati, and professor of pediatrics at the University of Cincinnati School of Medicine.
Dr. Beekman's academic interests relate to cardiac catheterization, and he has made numerous scholarly contributions in the field of hemodynamic assessment and transcatheter intervention for congenital heart disease. He has published more than 170 manuscripts in cardiology journals.
Dr. Beekman has been active in the cardiology community, locally and nationally. He is chair of the Section on Cardiology and Cardiac Surgery of the American Academy of Pediatrics. Beekman also is the chair of the JCCHD National Quality Improvement Collaborative for pediatric cardiology. Dr. Beekman has been a member of the Executive Committee and the Training Committee of the AHA Council on Cardiovascular Disease in the Young, and of the Congenital Heart Committee of the ACC. He is currently on the editorial board of the Journal of Interventional Cardiology, a contributing editor of theHeart.org, an Internet cardiology journal, and an associate editor of Congenital Heart Disease.
MD: Duke University School of Medicine, Durham, NC, 1976.
MS: Clinical Research Design and Statistics, University of Michigan, Ann Arbor, MI, 1986.Residency: Pediatrics, UCLA Medical Center, Los Angeles, CA, 1976 to 1979.Fellowship: Pediatric Cardiology, CS Mott Children's Hospital, University of Michigan, Ann Arbor, MI, 1979 to 1982.Certification: Pediatrics, 1981; Pediatric Cardiology, 1983.
Anderson JB, Beekman RH 3rd, Eghtesady P, Kalkwarf HJ, Uzark K, Kehl JE, Marino BS. Predictors of poor weight gain in infants with a single ventricle. J Pediatr. 2010 Sep;157(3):407-13, 413.e1. Sutherell JS, Hirsch R, Beekman RH 3rd. Pediatric interventional cardiology in the United States is dependent on the off-label use of medical devices. Congenit Heart Dis. 2010 Jan;5(1):2-7.
Anderson JB, Beekman RH 3rd, Border WL, Kalkwarf HJ, Khoury PR, Uzark K, Eghtesady P, Marino BS. Lower weight-for-age z score adversely affects hospital length of stay after the bidirectional Glenn procedure in 100 infants with a single ventricle. J Thorac Cardiovasc Surg. 2009 Aug;138(2):397-404.e1. Beekman RH 3rd, Duncan BW, Hagler DJ, Jones TK, Kugler JD, Moore JW, Jenkins KJ; Workgroup on Pediatric Cardiac Devices, Section on Cardiology and Cardiac Surgery, American Academy of Pediatrics. Pathways to approval of pediatric cardiac devices in the United States: challenges and solutions. Pediatrics. 2009 Jul;124(1):e155-62. Bennett MR, Ravipati N, Ross G, Nguyen MT, Hirsch R, Beekman RH, Rovner L, Devarajan P. Using proteomics to identify preprocedural risk factors for contrast induced nephropathy. Proteomics Clin Appl. 2008;2(7-8):1058-1064.
Cooper DS, Jacobs JP, Chai PJ, Jaggers J, Barach P, Beekman RH, Krogman O, Manning P. Pulmonary complications associated with the treatment of patients with congenital heart disease: Consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease. Cardiology in the Young. 2008;18:215-221.
Jenkins KJ, Beekman RH, Bergersen LJ, Everett AD, Forbes TJ, Franklin RCG, Klitzner TS, Krogman ON, Martin GR, Webb CL. Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac disease: The perspective of cardiology. Cardiology in the Young. 2008;18:116-123, 2008.
Mays WA, Border WL, Knecht SK, Gerdes YM, Pfriem H, Claytor RP, Knilans TK, Hirsch R, Mone SM, Beekman RH. Exercise capacity improves after transcatheter closure of the Fontan fenestration in children. Congenital Heart Disease. 2008;3: 254-261.
Gudausky TM, Hirsch R, Khoury PR, Beekman RH. Comparison of two transcatheter device strategies for occlusion of the patent ductus arteriosus. Catheter Cardiovasc Interven. 2008;72: 675-680.
Gruenstein DH, Beekman RH, Spicer RL. Ductal stent and cavo-atrial sac occlusion in an adult with profound cyanosis after palliated cyanotic congenital heart disease. residual superior vena caval-right atrial shunt in an adult with palliated complex congenital heart disease. J Invasive Cardiol. 2008;20: E41-43.
Burns C. Blaxall, PhD, FAHA Director of Translational Science, Heart Institute
is interested in understanding the molecular mechanisms associated with the development and progression of heart failure. We are particularly interested in developing novel heart failure therapeutics targeting myocardial function and fibrosis. To this end, we also seek to understand the pathologic role of cardiac myocyte and non-myocyte (i.e.. fibroblast) intercellular communication.
Director of Translational Science, Heart Institute
Molecular and signaling mechanisms of heart failure; cardiac fibrosis; drug and therapeutic discovery
Visit the Blaxall lab.
Dr. Burns C. Blaxall has focused his entire career on understanding the molecular mechanisms of heart failure. He received his PhD in pharmacology from the University of Colorado Health Sciences Center, and postdoctoral training at Duke University Medical Center. He then rose through the faculty ranks at the University of Rochester Medical Center and the Aab Cardiovascular Research Institute, where he also directed the Howard Hughes Medical Institute Med-into-Grad Fellowship in Cardiovascular Science.
In 2012, he was recruited to the Heart Institute of Cincinnati Children’s Hospital Medical Center, where he is director of Translational Science. Dr. Blaxall has received many academic honors, including the Early Career Investigator Award from the American Heart Association (AHA), the Outstanding Achievement Award from the Founder’s AHA Affiliate, the Merit Award for Research Achievement from Mended Hearts, and election as fellow of the AHA.
PhD: University of Colorado HSC, Denver, CO, 1999.
Fellowship: Duke University Medical Center, Durham, NC.
Martin ML, Blaxall BC. Cardiac intercellular communication: are myocytes and fibroblasts fair-weather friends? J Cardiovasc Transl Res. 2012.
Jaffré F, Friedman AE, Hu Z-Y, Mackman N and Blaxall BC. Beta-adrenergic receptor stimulation transactivates Protease-Activated Receptor 1 via MMP-13 in heart. Circulation. 2012;125(24):2993-3003.
Kamal FA, Smrcka AV, Blaxall BC. Taking the heart failure battle inside the cell: Small molecule targeting of Gβγ subunits. J Mol Cell Cardiol. 2011;51(4):462-7.
Ram R, Mickelsen DM, Theodoropoulos C, Blaxall BC. New approaches in small animal echocardiography: imaging the sounds of silence. Amer J Pathol Heart Circ Physiol. 2011;301(5):H1765-80.
Belmonte S, Blaxall BC. G-protein coupled receptor kinases as therapeutic targets in cardiovascular disease. Circ Res. 2011;109(3):309-19.
Aguilar F, Casey LM, Belmonte S, Noujaim SF, Maekawa N, Dunaevsky O, Protak TL, Noujaim S, Jalife J, Berk BC, Gertler FB, Blaxall BC. Cardiac dysfunction in Mena knockout mice. Amer J Pathol Heart Circ Phys. 2011;300(5):H1841-52.
Casey LM, Pistner AR, Belmonte S, Jaffre F, Migdalovic D, Stolpnik O, Nwakanma F, Vorobiof G, Dunaevsky O, Smrcka AV, Blaxall BC. Small molecule targeting of G beta gamma prevents heart failure progression. Circ Res. 2010;107(4):532-9.
Jaffré F, Bonnin P, Callebert J, Debbabi H, Setola V, Doly S, Monassier L, Mettauer, Blaxall BC, Launay JM, Maroteaux L. Serotonin and angiotensin receptors in cardiac fibroblasts coregulate adrenergic-dependent cardiac hypertrophy. Circ Res. 2009;104;113-123.
Bullard TA, Protack TL, Aguilar F, Bagwe S, Massey HT, Blaxall BC. Identification of Nogo as a novel indicator of heart failure. Physiolog Genom. 2008;32(2):182-9.
R, Hampton CR, Casey LM, Bullard TA, Tencati M, Pedersen B, Andrade-Gordon P, Sayen MR, Gottlieb RA, Pohlman TH, Verrier ED, Blaxall BC, Mackman N. Par-1 in Cardiac Remodeling and Heart Failure. Circulation. 2007;116(20):2298-306.
James F. Cnota, MD
is a pediatric cardiologist with clinical focus in echocardiography and fetal cardiology. His research activities are in two areas: 1) fetal cardiology and perinatal risk factors in congenital heart disease and 2) multicenter clinical trials in pediatric cardiovascular disease.
Associate Professor, UC Department of Pediatrics
Fetal cardiology including fetal echocardiography
BS: Molecular and Cellular Biology, University of Arizona, Tucson, Arizona, 1991
MD: Tulane University School of Medicine, New Orleans, Louisiana, 1995
Residency: University of Arizona Health Sciences Center, Tucson, Arizona, 1998
Fellowship: Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, 2001
MS: Clinical Epidemiology and Health Services Research, Wake Forest University School of Medicine, Winston-Salem, North Carolina, 2005
Divanović A, Hor K, Cnota J, Hirsch R, Kinsel-Ziter M, Michelfelder E. Prediction and perinatal management of severely restrictive atrial septum in fetuses with critical left heart obstruction: Clinical experience using pulmonary venous Doppler analysis. J Thorac Cardiovasc Surg. 2010 Dec 2. Crombleholme TM, Lim FY, Habli M, Polzin W, Jaekle R, Michelfelder E, Cnota J, Liu C, Kim MO. Improved recipient survival with maternal nifedipine in twin-twin transfusion syndrome complicated by TTTS cardiomyopathy undergoing selective fetoscopic laser photocoagulation. Am J Obstet Gynecol. 2010 Oct;203(4):397.e1-9. Habli M, Cnota J, Michelfelder E, Salisbury S, Schnell B, Polzin W, Lim FY, Crombleholme TM. The relationship between amniotic fluid levels of brain-type natriuretic peptide and recipient cardiomyopathy in twin-twin transfusion syndrome. Am J Obstet Gynecol. 2010 Oct;203(4):404.e1-7. Hambrook JT, Kimball TR, Khoury P, Cnota J. Disparities exist in the emergency department evaluation of pediatric chest pain. Congenit Heart Dis. 2010 May-Jun;5(3):285-91. Vuletin JF, Lim FY, Cnota J, Kline-Fath B, Salisbury S, Haberman B, Kingma P, Frischer J, Crombleholme T. Prenatal pulmonary hypertension index: novel prenatal predictor of severe postnatal pulmonary artery hypertension in antenatally diagnosed congenital diaphragmatic hernia. J Pediatr Surg. 2010 Apr;45(4):703-8. Hor KN, Gottliebson WM, Carson C, Wash E, Cnota J, Fleck R, Wansapura J, Klimeczek P, Al-Khalidi HR, Chung ES, Benson DW, Mazur W. Comparison of magnetic resonance feature tracking for strain calculation with harmonic phase imaging analysis. JACC Cardiovasc Imaging. 2010 Feb;3(2):144-51. Land MH, Rouster-Stevens K, Woods CR, Cannon ML, Cnota J, Shetty AK. Lactobacillus sepsis associated with probiotic therapy. Pediatrics. 2005 Jan;115(1):178-81.
Grace BE, Cnota JF, Hines MH, Payne RM, Ririe DO. Traumatic Coronary Artery Fistula in a Child. Circulation 2005;1 II :e272-e273.
Allison A. Divanovic, MD Co-Director, Medical Student/Resident Education, Heart Institute
is a pediatric cardiologist who specializes in noninvasive imaging, specifically transthoracic and fetal echocardiography. Her research interests include the impact of Twin-twin transfusion syndrome on the fetal heart as well as the management of fetuses with congenital heart defects, in particular, hypoplastic left heart syndrome.
Co-Director, Medical Student/Resident Education, Heart Institute
Pediatric cardiology; fetal and neonatal cardiology; transthoracic, transesophageal and fetal echocardiography
Villa CR, Habli M, Votava-Smith JK, Cnota JF, Lim FY, Divanovic AA, Wang Y, Michelfelder EC. Assessment of Fetal Cardiomyopathy in Early Stage Twin-Twin Transfusion Syndrome: Comparison between Commonly Reported Cardiovascular Assessment Scores. Ultrasound Obstet Gynecol. 2013 Oct 21. Epub ahead of print.
Cnota JF, Hangge PT, Wang Y, Woo JG, Hinton AC, Divanovic AA, Michelfelder EC, Hinton RB. Somatic Growth Trajectory in the Fetus with Hypoplastic Left Heart Syndrome. Pediatr Res. 2013 Sep;74(3):284-9.
Hangge P, Cnota JF, Woo JG, Hinton AC, Divanovic AA, Manning PB, Ittenbach RF, Hinton RB. Microcephaly is Associated with Early Adverse Neurologic Outcomes in Hypoplastic Left Heart Syndrome. Pediatr Res. 2013 Jul;74(1):61-7.
Statile CJ, Cnota JF, Gomien S, Divanović AA, Crombleholme T, Michelfelder E. Estimated Cardiac Output and Cardiovascular Profile Score in Fetuses with High Cardiac Output Lesions. Ultrasound Obstet Gynecol. 2013 Jan:41(1):54-8.
Divanović A, Witte D, Michelfelder E. A Fetus with Hypoplastic Left Heart Syndrome and Tricuspid Stenosis with Evolving Hydrops Fetalis. Pediatr Cardiol. 2012 Oct;33(7):1210-12.
Sheridan RM, Michelfelder EC, Choe KA, Divanovic A, Ware S, Liu C, Stanek JW. Ductus Arteriosus Aneurysm with Massive Thrombosis of Pulmonary Artery. Pediatr Dev Pathol. 2012 Jan-Feb;15(1):79-85.
Divanović A, Cnota J, Ittenbach R, Tan X, Border W, Crombleholme T, Michelfelder E. Characterization of Diastolic Dysfunction in Twin-Twin Transfusion Syndrome: Association between Doppler Findings and Ventricular Hypertrophy. J Am Soc Echocardiogr. 2011 Aug;24(8):834-40.
Divanović A, Hor K, Cnota J, Hirsch R, Kinsel-Ziter M, Michelfelder E. Prediction and Perinatal Management of Severely Restrictive Atrial Septum in Fetuses with Critical Left Heart Obstruction: Clinical Experience Using Pulmonary Venous Doppler Analysis. J Thorac Cardiovasc Surg. 2011 Apr;141(4):988-94.
Gudausky TM, Pearl J, Dent CL, Kim E, Divanović A, Spicer R, Beekman RH 3rd. Open-chest Epicardial Approach to Transcatheter Pulmonary Artery Stenting Following Heart Transplantation in an Infant. Congenital Heart Dis. 2007; Vol 2(1):64-9.
Stuart L. Goldstein, MD Director, Center for Acute Care Nephrology
focuses on all aspects of acute kidney injury (AKI) spanning from translational work in AKI biomarker validation to long-term AKI epidemiology and outcomes. He is specifically interested in improving outcomes in the critically ill child with or at risk for AKI.
Director, Center for Acute Care Nephrology
Acute kidney injury; continuous renal replacement therapy; end-stage renal disease; health-related quality of life
Stuart L. Goldstein, MD, has been an active investigator in the field of pediatric acute kidney injury (AKI) since 2000. Dr. Goldstein’s main research foci include: AKI epidemiology and outcomes, acute renal replacement therapy provision and investigation of novel urinary AKI biomarkers in the pediatric population. Dr. Goldstein has established a strong record of interdisciplinary and inter-institutional collaboration with cardiologists, intensivists and emergency center physicians, which is evidenced by his establishment and directing of the Prospective Pediatric Continuous Renal Replacement Therapy Registry from 2001 to 2012, and the Prospective Pediatric AKI Research Group (ppAKI-RG) in 2012. The ppAKI-RG is comprised of 39 centers from around the world with the goal of improving outcomes for the child with or at-risk for, AKI.
Dr. Goldstein has led initial efforts to develop a standardized definition for pediatric AKI, assess novel AKI biomarkers in heterogeneous populations, and conceiving and validating stratification tools to identify patients at risk for AKI. Building up these findings, Dr. Goldstein embarked on establishing the ppAKI-RG consortium to focus on multi-centered research studies that are dedicated to understanding and treating AKI in pediatric patients. Currently, the ppAKI-RG has initiated three major, and unprecedented, national and international studies (AWARE, NINJA and DIRECT) to reduce AKI and improve patient outcomes. Dr. Goldstein is also a recognized educator; he has developed the only pediatric specific acute care nephrology sub-specialty fellowship with graduates who are now leaders in the field of pediatric AKI.
Goldstein SL, Devarajan P. Acute kidney injury in childhood: should we be worried about progression to CKD? Pediatr Nephrol. 2011 Apr;26(4):509-22.
Du Y, Zappitelli M, Mian A, Bennett M, Ma Q, Devarajan P, Mehta R, Goldstein SL. Urinary biomarkers to detect acute kidney injury in the pediatric emergency center. Pediatr Nephrol. 2011 Feb;26(2):267-74.
Moffett BS, Goldstein SL, Adusei M, Kuzin J, Mohan P, Mott AR. Risk factors for postoperative acute kidney injury in pediatric cardiac surgery patients receiving angiotensin-converting enzyme inhibitors. Pediatr Crit Care Med. 2011 Feb 10.
Zappitelli M, Moffett BS, Hyder A, Goldstein SL. Acute kidney injury in non-critically ill children treated with aminoglycoside antibiotics in a tertiary healthcare centre: a retrospective cohort study. Nephrol Dial Transplant. 2011 Jan;26(1):144-50.
Koralkar R, Ambalavanan N, Levitan EB, McGwin G, Goldstein S, Askenazi D. Acute Kidney Injury Reduces Survival in Very Low Birth Weight Infant. Pediatr Res. 2010 Dec 20.
Goldstein SL. Continuous renal replacement therapy: mechanism of clearance, fluid removal, indications and outcomes. Curr Opin Pediatr. 2010 Dec 21.
Goldstein SL. Urinary kidney injury biomarkers and urine creatinine normalization: a false premise or not? Kidney Int. 2010 Sep;78(5):433-5.
Goldstein SL, Chawla L. Renal Angina. Clin J AmSoc Nephrol. 2010 May;5(5):943-9.
Sutherland SM, Zappitelli M, Alexander SR, Chua AN, Brophy PD, Bunchman TE, Hackbarth R, Somers MJG, Baum M, Symons JM, Flores FX, Benfield M, Askenazi D, Chand D, Fortenberry JD, Mahan JD, McBryde K, Blowey D, Goldstein SL. Fluid Overload and Mortality in Children Receiving Continuous Renal Replacement Therapy: The Prospective Pediatric Continuous Renal Replacement Therapy Registry. Am J Kidney Dis. 2010 Feb;55(2):316-25.
Jessica Graus Woo, MHSA, PhD
is a molecular epidemiologist with particular research interest in the developmental pathways leading to pediatric obesity. She is specifically interested in how genetics and early life influences, especially early diet, may impact a child’s likelihood of developing obesity and specific metabolic complications of obesity, such as insulin resistance and dyslipidemia.
James D. Gulick, MS
Instructor, UC Department of Pediatrics
Understanding how certain mutations in contractile protein genes are able to alter the function of the heart
Michalek AJ, Howarth JW, Gulick J, Previs MJ, Robbins J, Rosevear PR, Warshaw DM. Phosphorylation modulates the mechanical stability of the cardiac Myosin-binding protein C motif. Biophys J. 2013 Jan 22;104(2):442-52.
Bhuiyan MS, Gulick J, Osinska H, Gupta M, Robbins J. Determination of the critical residues responsible for cardiac myosin binding protein C's interactions. J Mol Cell Cardiol. 2012 Dec;53(6):838-47.
Weith AE, Previs MJ, Hoeprich GJ, Previs SB, Gulick J, Robbins J, Warshaw DM. The extent of cardiac myosin binding protein-C phosphorylation modulates actomyosin function in a graded manner. J Muscle Res Cell Motil. 2012 Dec;33(6):449-59.
Previs MJ, Previs SB, Gulick J, Robbins J, Warshaw DM. Molecular mechanics of cardiac myosin binding protein C in native thick filaments. Science. 2012 Sept 7; 337 (6099): 1215-8.
Weith A, Sadayappan S, Gulick J, Previs MJ, Vanburen P, Robbins J, Warshaw DM. Unique single molecule binding of cardiac myosin binding protein-C to actin and phosphorylation-dependent inhibition of actomyosin motility requires 17 amino acids of the motif domain. J Mol Cell Cardiol. 2012 Jan;52(1):219-27.
Tranter M, Liu Y, He S, Gulick J, Ren X, Robbins J, Jones WK, Reineke TM. In vivo delivery of nucleic acids via glycopolymer vehicles affords therapeutic infarct size reduction in vivo. Mol Ther. 2012 Mar;20(3):601-8.
Jeyaraj D, Haldar SM, Wan X, McCauley MD, Ripperger JA, Hu K, Lu Y, Eapen BL, Sharma N, Ficker E, Cutler MJ, Gulick J, Sanbe A, Robbins J, Demolombe S, Kondratov RV, Shea SA, Albrecht U, Wehrens XH, Rosenbaum DS, Jain MK. Circadian rhythms govern cardiac repolarization and arrhythmogenesis. Nature. 2012 Mar 1;483(7387):96-9.
Robert Bruce Hinton, MD Director, Heart Institute BioRepository (HIBR)
is a pediatric cardiologist who has basic and translational research programs. His laboratory studies the genetic and developmental basis of pediatric heart disease with a focus on cardiovascular malformations and valve disease.
Director, Heart Institute BioRepository (HIBR)
Cardiovascular genetics; echocardiography
Dr. Hinton graduated from Bucknell University with degrees in art history and philosophy. He earned his medical degree from the Medical University of South Carolina in Charleston SC. He completed his pediatric residency training at Memorial Health University Medical Center in Savannah GA and his pediatric cardiology fellowship at Cincinnati Children’s Hospital Medical Center. Dr. Hinton went on to pursue a postdoctoral fellowship in cardiovascular genetics and molecular cardiology at Cincinnati Children’s. He has been an attending staff member of the Division of Cardiology since 2006.
Dr. Hinton’s clinical interests relate to cardiovascular genetics and echocardiography. He is a member of the cardiovascular genetics service, and staffs the echocardiography laboratory. Dr. Hinton’s academic interests focus on translational research efforts using mouse models of human disease to identify new therapeutic targets. Dr. Hinton is a member of the American Academy of Pediatrics, the American Heart Association, the American Society of Bioethics and Humanities, and the American Society of Matrix Biology. He was elected to the Society for Pediatric Research in 2007.
Krishnamurthy VK, Opoka A, Kern CB, Guliak F, Narmoneva DA, Hinton RB. Regional Maladaptive Matrix Remodeling and Biomechanical Dysfunction in a Mouse Model of Aortic Valve Disease. Matrix Biology. 2012; 31(3):197-205. PMID: 22265892.
Kindel SJ, Miller EM, Gupta R, Cripe LH, Hinton RB, Spicer RL, Towbin JA, Ware SM. Pediatric Cardiomyopathy: Importance of Genetic and Metabolic Evaluation. Journal of Cardiac Failure. 2012; 18(5):396-403. PMID: 22555271.
Ryan TD, Ware SM, Lucky AW, Towbin JA, Jefferies JL, Hinton RB. Left Ventricular Noncompaction Cardiomyopathy and Aortopathy in a Patient with Recessive Dystrophic Epidermolysis Bullosa. Circulation Heart Failure. 2012; 5(5):81-82. PMID: 22991407.
Georg-Abraham JK, Zimmerman SL, Hinton RB, Marino BS, Witte D, Hopkin RJ. Tetrasomy 15q25-qter Identified with SNP Microarray in a Patient with Multiple Anomalies including Complex Cardiovascular Malformation. American Journal of Medical Genetics. 2012; 158A(8):1971-1976. PMID: 22711292.
Hinton RB. Bicuspid Aortic Valve and Thoracic Aortic Aneurysm: Three Patient Populations, Two Disease Phenotypes, One Shared Genotype. Cardiology Research and Practice. 2012. 926975. PMID: 22970404.
Krishnamurthy VK, Guliak F, Narmoneva DA, Hinton RB. Regional structure-function relationships in mouse aortic valve tissue. J Biomech. Jan 2011;44(1):77-83.
Wirrig EE, Hinton RB, Yutzey KE. Differential expression of cartilage and bone-related proteins in pediatric and adult diseased aortic valves. J Mol Cell Cardiol. Mar 2011;50(3):561-569.
Calloway TJ, Martin LJ, Zhang X, Tandon A, Benson DW, Hinton RB. Risk factors for aortic valve disease in bicuspid aortic valve: a family-based study. Am J Med Genet A. May 2011;155A(5):1015-1020.
Hinton RB, Yutzey KE. Heart valve structure and function in development and disease. Annu Reb Physiol. Mar 2011;73:29-46.
Martin LJ, Hinton RB, Zhang X, Cripe LH, Benson DW. Aorta measurements are heritable and influenced by bicuspid aortic valve. Frontiers in Genetics. 2011;2(61).
Twist1 regulation of valve progenitors. Co-Investigator. National Heart, Lung and Blood Institute (NIH/NHLBI). Jul 2010-Jun 2015.
Trial of Beta Blocker Therapy (Atenolol) vs. Angiotensin II Receptor Blocker Therapy (Losartan). Co-Investigator. National Heart, Lung and Blood Institute (NIH/NHLBI) Pediatric Heart Network. Sept 2006-Aug 2016.
Jeanne M. James, MD
Cardiovascular genetics, echocardiography, congenital heart disease, medical education, molecular cardiology, ventricular remodeling, cardiac hypertrophy.
Visit the James Lab web site.
Jeanne James, MD, is an associate professor of pediatrics at University of Cincinnati College of Medicine and is currently the director of the Cardiology Fellowship Training Program at Cincinnati Children's Hospital Medical Center.
A native of West Virginia, Dr. James earned her bachelor and medical degrees from West Virginia University in Morgantown, WV. She completed her pediatric residency and pediatric cardiology fellowship at Vanderbilt University Medical Center in Nashville, TN. Dr. James has been an attending staff member of the Division of Cardiology at Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine since 1995.
Dr. James' academic interests are in translational research on heart muscle disease, cardiovascular genetics and echocardiography. She has made numerous scholarly contributions to the field with publications describing both laboratory-based and clinical research. She serves as a grant reviewer for the American Heart Association and is a peer-reviewer for a number of scientific and medical journals.
Dr. James has established collaborative relationships with laboratory researchers at Cincinnati Children’s Hospital, the University of Cincinnati College of Medicine and investigators across the United States. As the director of the Mouse Echocardiography Core, she uses non-invasive imaging to evaluate phenotypes of transgenic mice, including embryonic mice.
Dr. James serves as an attending physician in clinical echocardiography laboratory as well as the inpatient cardiology ward and consult team. Dr. James is a member of the Cardiovascular Genetics (CVG) service and has significant responsibilities in the CVG outpatient clinic. Dr. James is a member of the American Society of Echocardiography, the American Heart Association and the Society for Pediatric Research.
Cheek J, Wirrig E, Alfieri C, James J, Yutzey K. Differential activation of valvulogenic, chondrogenic and osteogenic pathways in mouse models of myxomatous and calcific aortic valve disease. In press.
Sadayappan S, Gulick J, Martin L, Osinska H, Barefield D, Cuello F, Avkiran M, Lasko V, Lorenz J, Maillet M, Martin J, Heller-Brown J, Bers D, Molkentin J, James J, Robbins J. A critical function for Ser-282 in cardiac myosin binding protein-C phosphorylation and cardiac function. Circ Res. 2011; 109(2):141-50.
James J, Kinnett K, Ittenbach R, Wang Y, Benson D, Cripe L. Electrocardiographic abnormalities in very young Duchenne muscular dystrophy patients precede the onset of cardiac dysfunction. Neuromuscul Disord. 2011; 21(7):462-7.
Combs M, Braitsch C, Lange A, James J, Yutzey K. NFATc1 promotes epicardium-derived cell (EPDC) invasion into myocardium. Development. 2011; 138(9):1747-57.
James J, Robbins J. Signaling and myosin binding protein C. J Biol Chem. 2011;286(12):9913-9.
Stanley BA, Graham DR, James J, Mitsak M, Tarwater PM, Robbins J, Van Eyk JE. Altered myofilament stoichiometry in response to heart failure in a cardioprotective a-myosin heavy chain transgenic rabbit model. Proteomics Clin Appl. 2011; 5(3-4):147-58.
Acehan D, Vaz F, Houtkooper R, James J, Moore V, Tokunaga C, Kulik W, Wansapura J, Toth M, Strauss A, Khuchua Z. Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome. J Biol Chem. 2010; 286(2):899-908.
Hinton R, Adelman-Brown J, Witt S, Krishnamurthy V, Gruber M, Osinska H, Sakthivel B, James J, Narmoneva D, Mecham R, Benson D. Elastin haploinsufficiency results in latent progressive aortic valve disease in a mouse model. Circ Res. 2010; 107(4):549-57.
James J, Hor K, Moga M, Martin L, Robbins J. Effects of myosin heavy chain manipulation in experimental heart failure. J Mol Cell Cardiol. 2010; 48(5):999-1006.
Suzuki T, Palmer B, James J, Wang Y, Chen V, VanBuren P, Maughan D, Robbins J, LeWinter M. Effects of cardiac myosin isoform variation on myofilament function and cross-bridge kinetics in transgenic rabbits. Circ Heart Fail. 2009; 2(4):334-41.
Pattison J, Waggoner J, James J, Martin L, Gulick J, Osinska H, Klevitsky R, Kranias E, Robbins J. Phospholamban overexpression in transgenic rabbits. Transgenic Research. 2008; 17(2):157-70.
John Lynn Jefferies, MD, MPH, FAAP, FACC Director, Advanced Heart Failure and Cardiomyopathy
is a clinical cardiologist who has translational and clinical research programs. His research involves cardiomyopathy and cardiovascular genetics with specific interest in dystrophinopathies and aortopathies in children and adults. He also has clinical research projects investigating novel biomarkers of heart failure, chemotherapy induced cardiotoxicity, and the cardiorenal syndrome.
Director, Advanced Heart Failure and Cardiomyopathy
Dr. Jefferies, an associate professor of pediatric cardiology and adult cardiovascular diseases, is the director of Advanced Heart Failure / Cardiomyopathy within the Heart Institute at Cincinnati Children's Hospital Medical Center.
He completed his combined pediatric and adult cardiology training at the Baylor College of Medicine in Houston, Texas at the Texas Children's Hospital and the Texas Heart Institute. He has authored or co-authored over 100 peer-reviewed manuscripts and book chapters on cardiomyopathy, cardiovascular genetics, and adults with congenital heart disease.
His current research interests include heritable causes of vascular disease, novel drug therapies for advanced heart failure, novel gene discovery in cardiomyopathy, characterization and management of left ventricular noncompaction (LVNC), and early diagnosis and management of chemotherapy induced cardiotoxicity.
He is on the editorial board of the Texas Heart Institute Journal and is an active member of numerous professional organization including the Heart Failure Society of America, the American College of Cardiology and the American Heart Association.
Jefferies JL, Towbin JA. Dilated cardiomyopathy. Lancet. 2010 Feb;375(9716):752-62. Breinholt JP, Moulik M, Dreyer WJ, Denfield SW, Kim JJ, Jefferies JL, Rossano JW, Gates CM, Clunie SK, Bowles KR, Kearney DL, Bowles NE, Towbin JA. Viral epidemiologic shift in inflammatory heart disease: the increasing involvement of parvovirus B19 in the myocardium of pediatric cardiac transplant patients. J Heart Lung Transplant. 2010 Jul;29(7):739-46. Rossano JW, Morales DL, Zafar F, Denfield SW, Kim JJ, Jefferies JL, Dreyer WJ. Impact of antibodies against human leukocyte antigens on long-term outcome in pediatric heart transplant patients: An analysis of the United Network for Organ Sharing database. J Thorac Cardiovasc Surg. 2010 May 26.
Carlson P, Jefferies JL, Kearney D, Russell H. Refractory dilated cardiomyopathy associated with metastatic neuroblastoma. Pediatr Blood Cancer. 2010 Jun 29.
Shah AM, Jefferies JL, Rossano JW, Decker JA, Cannon BC, Kim JJ. Electrocardiographic abnormalities and arrhythmias are strongly associated with the development of cardiomyopathy in muscular dystrophy. Heart Rhythm. 2010 Jun 18. Moulik M, Breinholt JP, Dreyer WJ, Kearney DL, Price JF, Clunie SK, Moffett BS, Kim JJ, Rossano JW, Jefferies JL, Bowles KR, O'Brian Smith E, Bowles NE, Denfield SW, Towbin JA. Viral Endomyocardial Infection Is an Independent Predictor and Potentially Treatable Risk Factor for Graft Loss and Coronary Vasculopathy in Pediatric Cardiac Transplant Recipients. J Am Coll Cardiol. 2010 56:582-592.Shchelochkov OA, Li FY, Wang J, Zhan H, Towbin JA, Jefferies JL, Wong LJ, Scaglia F. Milder Clinical Course of Type IV 3-Methylglutaconic Aciduria Due to a Novel Mutation in TMEM70. Mol Genet Metab. 2010 101:282-285. Foerster SR, Canter CE, Cinar A, Sleeper LA, Webber SA, Pahl E, Kantor PF, Alvarez JA, Colan SD, Jefferies JL, Lamour JM, Margossian R, Messere JE, Rusconi PG, Shaddy RE, Towbin JA, Wilkinson JD, Lipshultz SE. Ventricular Remodeling and Survival are More Favorable for Myocarditis than for Idiopathic Dilated Cardiomyopathy in Childhood: An Outcomes Study from the Pediatric Cardiomyopathy Registry. Circ Heart Fail. 2010. Jefferies JL, Price JF, Morales DL. Mechanical support in childhood heart failure. Heart Fail Clin. 2010 Oct;6(4):559-73. Jefferies JL, Hoffman TM, Nelson DP. Heart failure treatment in the intensive care unit in children. Heart Fail Clin. 2010 Oct;6(4):531-58.
Zaza Khuchua, PhD
research Interests are mitochondrial structure, function, biogenesis and recycling in normal and pathological heart muscle. More specifically we are interested in defects in cardiac lipid and phospholipid metabolism. We employ genetically engineered mice to model human genetic disorders.
Mitochondrial function; structure and dynamics in cardiac cells in normal and pathological conditions; role of mitochondrial phospholipids in aerobic metabolism in heart; role of lipid molecules in cell signaling systems
Acehan D, Vaz F, Houtkooper RH, James J, Moore V, Tokunaga C, Kulik W, Wansapura J, Toth MJ, Strauss A, Khuchua Z. Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome. J Biol Chem. 2011 Jan 14;286(2):899-908.
Acehan D, Khuchua Z, Houtkooper RH, Malhotra A, Kaufman J, Vaz FM, Ren M, Rockman HA, Stokes DL, Schlame M. Distinct effects of Tafazzin deletion in differentiated and undifferentiated mitochondria. Mitochondrion.
Tchekneva EE, Khuchua Z, Davis LS, Kadkina V, Dunn SR, Bachman S, Ishibashi K, Rinchik EM, Harris RC, Dikov MM, Breyer MD. Single amino acid substitution in aquaporin 11 causes renal failure. J Am Soc Nephrol. 2008 10:1955-64.
Thomas R. Kimball, MD Medical Director, Heart Institute
is a clinical cardiologist who has clinical research interests in the impact of systemic diseases (including renal, pulmonary and hematologic) on cardiovascular structure and function. He has studied the impact of pediatric obesity on both cardiac and arterial anatomy and physiology and directs the Cardiovascular Imaging Core Research Laboratory which is active in using echocardiography to cardiac phenotype transgenic mice models.
Medical Director, Heart Institute
Congential heart disease; echocardiography; cardiac imaging
Thomas R. Kimball, MD, is a professor of pediatrics with the University of Cincinnati College of Medicine and is currently the medical director of the Heart Institute at Cincinnati Children's Hospital Medical Center.
A native of California, Dr. Kimball graduated with Distinction and Honors from Stanford University. He earned his medical degree from New York University, New York, NY. He completed his pediatric internship and residency at Children's Hospital of Los Angeles and his pediatric cardiology fellowship at Cincinnati Children's Hospital Medical Center.
Dr. Kimball has been an attending staff member of the Division of Cardiology at Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine since 1988.
Dr. Kimball's academic interests relate to echocardiography and he has made numerous scholarly contributions in the field of ventricular function utilizing echocardiography, particularly in the field of hypertension and obesity.
He has established collaborative relationships with basic scientists at the University of Cincinnati College of Medicine using echocardiography to evaluate phenotypes of transgenic mice, including embryonic mice.
Dr. Kimball has been active in the cardiology community. He is a member of the American Society of Echocardiography, American Heart Association, American Academy of Pediatrics, American College of Cardiology and an investigator in the NIH Pediatric Heart Network. He was also a member of the first Family Advisory Council at Cincinnati Children's and received the 2006 Family Advisory Council Award of Excellence. In 2010, he was made the medical director of the Heart Institute at Cincinnati Children's.
Shah AS, Urbina EM, Khoury PR, Kimball TR. Lipids and lipoprotein ratios: The contribution to carotid intima media thickness in adolescents and young adults with type 2 diabetes mellitus. J Clin Lipidol. 2013 Sep-Oct;7(5):441-5.
Uzark K, King E, Spicer R, Beekman R, Kimball T, Varni JW. The clinical utility of health-related quality of life assessment in pediatric cardiology outpatient practice. Congenit Heart Dis. 2013 May;8(3):211-8.
Potts JE, Alghamdi M, De Souza AM, White CT, Potts MT, Warady BA, Furth SL, Sandor GG, Kimball TR. Echo-Doppler assessment of the biophysical properties of the aorta in children with chronic kidney disease. Pediatr Cardiol. 2013 Jun;34(5):1218-25.
Madueme PC, Khoury PR, Urbina EM, Kimball TR. Predictors of exaggerated exercise-induced systolic blood pressures in young patients after coarctation repair. Cardiol Young. 2013 Jun;23(3):416-22.
Urbina EM, Khoury PR, McCoy CE, Dolan LM, Daniels SR, Kimball TR. Triglyceride to HDL-C ratio and increased arterial stiffness in children, adolescents and young adults. Pediatrics. 2013 April;131(4):E1082-90.
Edwards NM, Daniels SR, Claytor RP, Khoury PR, Dolan LM, Kimball TR, Urbina EM. Physical activity is independently associated with multiple measures of arterial stiffness in adolescents and young adults. Metabolism. 2012 Jun;61(6)869-72.
Shah AS, Dolan LM, Gao Z, Kimball TR, Urbina EM. Racial differences in arterial stiffness among adolescents and young adults with type 2 diabetes. Pediatr Diabetes. 2012 Mar;13(2):170-5.
Urbina EM, Khoury PR, McCoy C, Daniels SR, Kimball TR, Dolan LM. Cardiac and vascular consequences of pre-hypertension in youth. J Clin Hypertens. 2011 May;13(5)332-42.
Crowley DI, Khoury PR, Urbina EM, Ippisch HM, Kimball TR. Cardiovascular impact of pediatric obesity epidemic: higher left ventricular mass related to higher body mass index. J Pediatr. 2011 May;158(5):709-14.
Urbina EM, Dolan LM, McCoy CE, Khoury PR, Daniels SR, Kimball TR. Relationship between elevated arterial stiffness and increased left ventricular mass in adolescents and young adults. J Pediatr. 2011 May;158(5)715-21.
Shelley Kirk, PhD, RD, LD Director, HealthWorks!, Center for Better Health and Nutrition, Heart Institute
is a clinical dietitian specializing in the assessment and treatment of obese youth and their families. With her doctorate in epidemiology, her research interests are focused on evaluating the effectiveness and efficacy of the dietary component as part of a comprehensive pediatric weight management intervention offered in a clinical setting.
Director, HealthWorks!, Center for Better Health and Nutrition, Heart Institute
Hoelscher DM, Kirk S, Cunningham-Sabo L, Ritchie L. Position of the Academy of Nutrition and Dietetics: Interventions for the prevention and treatment of pediatric overweight and obesity. Journal of the Academy of Nutrition and Pediatrics. 2013 (In press).
Hampl S, Demeule M, Eneli I, Frank M, Hawkins MJ, Kirk S, Sallinen B, Santos M, Schmidt A, Ward W, Rhodes S. Parent perspectives on attrition from tertiary care pediatric weight management programs. Clinical Pediatrics. 2013; 52(6):509-515.
Kirk S, Brehm B, Saelens BE, Woo JG, Kissel E, D’Alessio D, Bolling C, Daniels SR. Role of carbohydrate modification in weight management among obese children: A randomized clinical trial. Journal of Pediatrics. 2012; 161(2): 320-327.
Beebe DW, Miller N, Kirk S, Daniels SR, Amin R. The association between obstructive sleep apnea and dietary choices among obese individuals during middle to late childhood. Sleep Medicine. 2011; 12(8):797-799.
Sung V, Beebe DW, Vandyke R, Fenchel MC, Crimmins NA, Kirk S, Amin R, Hiscock H, Wake M. Does sleep duration predict metabolic risk in obese adolescents attending tertiary services? A cross- sectional study. Sleep. 2011; 34(7):891-898.
Siegel RM, Neidhard MS, Kirk S. A comparison of low glycemic index and staged portion-controlled diets in improving BMI of obese children in a pediatric weight management program. Clinical Pediatrics. 2011; 50(5):459-461.
Kirk S, Bolling C. Practical Strategies in a Clinical Setting for Promoting Lifestyle Changes in Overweight Youth. Obesity Management. December 2007; 3(6):272-282.
Lawson ML, Kirk S, Mitchell T, Chen MK, Loux TJ, Daniels SR, Harmon CM, Clements RH. One-year outcomes of Roux-en-Y gastric bypass for morbidly obese adolescents: a multi-center study from the Pediatric Bariatric Study Group. Journal of Pediatric Surgery. 2006; 41(1):137-43.
Kirk S, Scott B, Daniels SR. Supplement to the Journal: Pediatric obesity epidemic: Treatment options. Journal of the American Dietetic Association. 2005; 105(5 Suppl 1):S44-51.
Kirk S, Zeller MH, Claytor R, Santangelo M, Khoury PR, Daniels SR. The relationship of health outcomes to improvement in body mass index in children and adolescents. Obes Res. 2005; 13:876-882.
Timothy K. Knilans, MD Director, Clinical Cardiac Electrophysiology and Pacing
is a clinical cardiac electrophysiologist with a large clinical practice in management of arrhythmia and syncope. He has extensive experience in non-invasive electrocardiographic methods and invasive therapeutic modalities including catheter and surgical ablation and implanted electrical device therapy. His research interests center on evaluation and treatment of syncope and prevention of sudden cardiac death.
Director, Clinical Cardiac Electrophysiology and Pacing
Cardiac electrophysiology and pacing; radio frequency ablation; tilt table testing
Czosek RJ, Anderson JB, Marino BS, Mellion K, Knilans TK. Noninvasive risk stratification techniques in pediatric patients with ventricular preexcitation. Pacing Clin Electrophysiol. 2011 May;34(5):555-62.
Eghtesady P, Michelfelder EC, Knilans TK, Witte DP, Manning PB, Crombleholme TM. Fetal surgical management of congenital heart block in a hydropic fetus: Lessons learned from a clinical experience. J Thorac Cardiovasc Surg. 2011 Mar;141(3):835-7.
Czosek RJ, Anderson J, Marino BS, Connor C, Knilans TK. Linear lesion cryoablation for the treatment of atrioventricular nodal re-entry tachycardia in pediatrics and young adults. Pacing Clin Electrophysiol. 2010 Nov;33(11):1304-11.
Mays WA, Border WL, Knecht SK, Gerdes YM, Pfriem H, Claytor RP, Knilans TK, Hirsch R, Mone SM, Beekman RH 3rd. Exercise capacity improves after transcatheter closure of the Fontan fenestration in children. Congenit Heart Dis. 2008 Jul;3(4):254-61.
Knecht SK, Mays WA, Gerdes YM, Claytor RP, Knilans TK. Exercise evaluation of upper- versus lower-extremity blood pressure gradients in pediatric and young-adult participants. Pediatr Exerc Sci. 2007 Aug;19(3):344-8.
Wright KN, Knilans TK, Irvin HM. When, why, and how to perform cardiac radiofrequency catheter ablation. J Vet Cardiol. 2006 Nov;8(2):95-107.
Knilans TK. Multielectrode coronary artery catheterization: still an effective tool in ablation of right free-wall accessory pathways. J Cardiovasc Electrophysiol. 2004 Nov;15(11):1244-5.
Markham LW, Knecht SK, Daniels SR, Mays WA, Khoury PR, Knilans TK. Development of exercise-induced arm-leg blood pressure gradient and abnormal arterial compliance in patients with repaired coarctation of the aorta. Am J Cardiol. 2004 Nov 1;94(9):1200-2.
Beery TA, Dyment M, Shooner K, Knilans TK, Benson DW. A candidate locus approach identifies a long QT syndrome gene mutation. Biol Res Nurs. 2003 Oct;5(2):97-104.
Andreas W. Loepke, MD, PhD Staff Anesthesiologist, Division of Cardiac Anesthesia
Staff Anesthesiologist, Division of Cardiac Anesthesia
Associate Professor, UC Department of Anesthesia
UC Department of Pediatrics
Anesthesia for neonates; congenital heart surgery; single-ventricle physiology
Visit the Loepke Lab.
Andreas W. Loepke, MD, PhD, has been practicing pediatric cardiac anesthesiology since 2000, first at the Children's Hospital of Philadelphia, and since 2003 at Cincinnati Children's Hospital Medical Center.
Dr. Loepke’s research laboratory investigates the potential use of anesthetics for neurological protection during brain ischemia as well as their possible adverse effects on brain development. His research group discovered in animals that anesthetics differentially affect brain regions, dependent on the age during exposure. Related to these findings, his lab made the discovery that neurons are susceptible to the anesthetics’ toxic effects during a specifically vulnerable stage of their development.
MD: University of Dusseldorf, Germany, 1993.PhD: University of Dusseldorf, Germany, 1995.
Internship: General Surgery, Pennsylvania Hospital, Philadelphia, PA, 1996.Residency: Anesthesiology, Thomas Jefferson Medical College, Philadelphia, PA, 1999.Fellowship: Pediatric Anesthesia and Pediatric Cardiac Anesthesia, Children's Hospital of Philadelphia, Philadelphia, PA, 2000.Certification: Anesthesiology, 2000.Maintenance of Certification: Anesthesiology, 2010.
Hofacer RD, Deng M, Ward CG, Joseph B, Hughes EA, Jiang C, Danzer SC, Loepke AW. Cell-age specific vulnerability of neurons to anesthetic toxicity. Ann Neurol. 2013.
Istaphanous GK, Ward CG, Nan X, Hughes EA, McCann JC, McAuliffe JJ, Danzer, SC, Loepke AW. Characterization and Quantification of Isoflurane-Induced Developmental Apoptotic Cell Death In Mouse Cerebral Cortex. Anes Analg. 2013; 116(4): 845-54.
Mintz CD, Wagner M, Loepke AW. Preclinical research into the effects of anesthetics on the developing brain: promises and pitfalls. J Neurosurg Anesthesiol. 2012; 24(4): 362-7.
Istaphanous GK, Howard J, Nan X, Hughes EA, McCann JC, McAuliffe JJ, Danzer SC, Loepke AW. Comparison of the Neuroapoptotic Properties of Equipotent Anesthetic Concentrations of Desflurane, Isoflurane, or Sevoflurane in Neonatal Mice. Anesthesiology. 2011; 114(3): 578-87.
Loepke AW. Developmental Neurotoxicity of Sedatives and Anesthetics – A Concern For Neonatal and Pediatric Critical Care Medicine? Pediatr Crit Care Med. 2010; 11(2): 217-226.
McAuliffe JJ, Loepke AW, Miles L, Joseph B, Hughes E, Vorhees CV. Desflurane, Isoflurane and Sevoflurane Provide Limited Neuroprotection Against Neonatal Hypoxia-Ischemia in a Delayed-Preconditioning Paradigm. Anesthesiology. 2009; 111(3): 533-46.
Loepke AW, Istaphanous GK, McAuliffe JJ, Miles L, Hughes EA, McCann JC, Harlow KE, Kurth CD, Williams MT, Vorhees CV, Danzer SC. The Effects of Neonatal Isoflurane Exposure in Mice on Brain Cell Viability, Adult Behavior, Learning, and Memory. Anesth Analg. 2009; 108(1): 90-104.
Loepke AW, Soriano SG. An Assessment of the Effects Of General Anesthetics on Developing Brain Structure and Neurocognitive Function. Anesth Analg. 2008; 106: 1681-1707.
Loepke AW, McCann JC, Kurth CD, McAuliffe JJ. Physiologic Effects of Isoflurane Anesthesia in Neonatal Mice. Anesth Analg. 2006; 102: 75-80.
Loepke AW, Priestley MA, Schultz SE, McCann JC, Kurth CD. Desflurane Improves Outcome After Low-Flow Cardiopulmonary Bypass in Newborn Pigs. Anesthesiology. 2002; 97: 1521-7.
Angela Lorts, MD Director, Ventricular Assist Device Program
Director, Ventricular Assist Device Program
Heart failure; cardiopulmonary bypass mediated myocardial dysfunction
Lorts A, Burroughs T, Shanley TP. Elucidating the role of reversible protein phosphorylation in sepsis-induced myocardial dysfunction. Shock. 2009 Jul;32(1):49-54. Review.
Lorts A, Schwanekamp JA, Elrod JW, Sargent MA, Molkentin JD. Genetic manipulation of periostin expression in the heart does not affect myocyte content, cell cycle activity, or cardiac repair. Circ Res. 2009 Jan 2;104(1):e1-7.
Oka T, Xu J, Kaiser RA, Melendez J, Hambleton M, Sargent MA, Lorts A, Brunskill EW, Dorn GW 2nd, Conway SJ, Aronow BJ, Robbins J, Molkentin JD. Genetic manipulation of periostin expression reveals a role in cardiac hypertrophy and ventricular remodeling. Circ Res. 2007 Aug 3;101(3):313-21.
McLean KM, Lorts A, Pearl JM. Current treatments for congenital aortic stenosis. Curr Opin Cardiol. 2006 May;21(3):200-4. Review.
Marjorie Maillet, PhD
Signaling pathways; cellular biology; cardiac hypertrophy and heart failure
PhD: Paris XI University, Orsay, France, 2003.
Fellowship: Cincinnati Children’s Hospital, Cincinnati, Ohio.
van Berlo JH, Maillet M, Molkentin JD. Signaling effectors underlying pathologic growth and remodeling of the heart. J Clin Invest. 123, 37-45. 2013.
Maillet M, van Berlo JH, Molkentin JD. Molecular basis of physiological heart growth: fundamental concepts and new players. Nat Rev Mol Cell Biol. 14, 38-48. 2012.
Lynch JM, Maillet M, Vanhoutte D, Schloemer A, Sargent MA, Blair NS, Lynch KA, Okada T, Aronow BJ, Osinska H, Prywes R, Lorenz JN, Mori K, Lawler J, Robbins J, Molkentin JD. A thrombospondin-dependent pathway for a protective ER stress response. Cell. 149, 1257-68. 2012.
Le Grand F, Grifone R, Mourikis P, Houbron C, Gigaud C, Pujol J, Maillet M, Pages G, Rudnicki M, Tajbakhsh S, Maire P. Six1 regulates stem cell repair potential and self-renewal during skeletal muscle regeneration. J Cell Biol. 198, 815-32. 2012.
Drawnel FM, Wachten D, Molkentin JD, Maillet M, Aronsen JM, Swift F, Sjaastad I, Liu N, Catalucci D, Mikoshiba K, Hisatsune C, Okkenhaug H, Andrews SR, Bootman MD, Roderick HL. Mutual antagonism between IP(3)RII and miRNA-133a regulates calcium signals and cardiac hypertrophy. J Cell Biol. 199, 783-98. 2012.
Davis J, Maillet M, Miano JM, Molkentin JD. Lost in transgenesis: a user's guide for genetically manipulating the mouse in cardiac research. Circ Res. 111, 761-77. 2012.
Sadayappan S, Gulick J, Osinska H, Barefield D, Cuello F, Avkiran M, Lasko VM, Lorenz JN, Maillet M, Martin JL, Brown JH, Bers DM, Molkentin JD, James J, Robbins J. A critical function for ser-282 in cardiac Myosin binding protein-C phosphorylation and cardiac function. Circ Res. 109, 141-50. 2011.
Qian L, Wythe JD, Liu J, Cartry J, Vogler G, Mohapatra B, Otway RT, Huang Y, King IN, Maillet M, Zheng Y, Crawley T, Taghli-Lamallem O, Semsarian C, Dunwoodie S, Winlaw D, Harvey RP, Fatkin D, Towbin JA, Molkentin JD, Srivastava D, Ocorr K, Bruneau BG, Bodmer R. Tinman/Nkx2-5 acts via miR-1 and upstream of Cdc42 to regulate heart function across species. J Cell Biol. 193, 1181-96. 2011.
Kehat I, Davis J, Tiburcy M, Accornero F, Saba-El-Leil MK, Maillet M, York AJ, Lorenz JN, Zimmermann WH, Meloche S, Molkentin JD. Extracellular signal-regulated kinases 1 and 2 regulate the balance between eccentric and concentric cardiac growth. Circ Res. 2011 Jan 21;108(2):176-83.
Nakayama H, Bodi I, Maillet M, DeSantiago J, Domeier TL, Mikoshiba K, Lorenz JN, Blatter LA, Bers DM, Molkentin JD. The IP3 receptor regulates cardiac hypertrophy in response to select stimuli. Circ Res. 2010 Sep 3;107(5):659-66
Erik C. Michelfelder, MD Co-Director, Cardiac Imaging Services, Heart Institute
is a clinical cardiologist who has clinical research programs in fetal cardiology and echocardiography. Within the Fetal Heart Program, the fetal echocardiography lab studies anatomic and physiologic predictors of cardiac function, perinatal physiology, and clinical outcomes in fetuses with both structural and function cardiovascular abnormalities. General echocardiographic research conducted by Dr. Michelfelder also included echocardiographic assessment of cardiac systolic and diastolic function in infants, children, and young adults with a variety of structural and function cardiac abnormalities.
Co-Director, Cardiac Imaging Services, Heart Institute
Director, Fetal Heart Program, Heart Institute
Erik C. Michelfelder Sr., MD, is a professor of pediatrics with the University of Cincinnati College of Medicine. He is currently director of the Fetal Heart Program, and co-director of Cardiac Imaging Services for the Heart Institute at Cincinnati Children’s Hospital Medical Center.
Dr. Michelfelder graduated with a degree in biology from Bucknell University and earned his MD from the Penn State University College of Medicine. He completed his residency in pediatrics at St. Christopher’s Hospital for Children in Philadelphia, and his cardiology fellowship at C.S. Mott Children’s Hospital at the University of Michigan in Ann Arbor.
Dr. Michelfelder has been a member of the Division of Cardiology at Cincinnati Children’s Hospital Medical Center since 1998. His clinical and academic focus relate to fetal cardiology and echocardiography.
He is a member of the American Society of Echocardiography, The American Heart Association, The American College of Cardiology, The American Academy of Pediatrics, and the Society for Pediatric Research.
BS: Bucknell University, Lewisburg, PA, 1986.
MD: Penn State University College of Medicine, Hershey, Pennsylvania, 1990.Residency: St. Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, Pennsylvania, 1990-1993.Fellowship: C.S. Mott Children's Hospital, University of Michigan Medical Center, Ann Arbor, Michigan, 1993-1996.Certification: Pediatrics 1991, Pediatric Cardiology 1998.
Ngamprasertwong P, Michelfelder EC, Arbabi S, Choi YS, Statile C, Ding L, Boat A, Eghtesady P, Holland K, Sadhasivam S. Anesthetic Techniques for Fetal Surgery: Effects of Maternal Anesthesia on Intraoperative Fetal Outcomes in Sheep Model. Anesthesiology. 2013 Apr;118(4):796-808.
Statile C, Cnota J, Gomien S, Divanovic A, Crombleholme T, Michelfelder EC. Estimated Cardiac Output and Cardiovascular Profile Score in Fetuses with High Cardiac Output Lesions. Ultrasound Obstet Gynecol. 2013 Jan;41(1):54-8.
Williams IA, Fifer C, Jaeggi E, Levine J, Michelfelder EC, Garfinkel R, Szwast AL. The Association of Fetal Cerebrovascular Resistance with Early Neurodevelopment in Single Ventricle Congenital Heart Disease. Am Heart J. 2013;165(4):544-50.
Habli M, Michelfelder EC, Cnota J, Wall D, Polzin W, Lewis D, Lim FY, Crombleholme TM. Prevalence and Progression of Recipient-twin Cardiomyopathy in Early-stage Twin-twin Transfusion Syndrome. Ultrasound Obstet Gynecol. 2012 Jan;39(1):63-8.
Divanovic A, Cnota JF, Ittenbach R, Tan X, Border WL, Crombleholme T, Michelfelder E. Characterization of Diastolic Dysfunction in Twin-Twin Transfusion Syndrome: Association between Doppler Findings and Ventricular Hypertrophy. J Am Soc Echocardiogr. 2011 Aug;24(8):834-40.
Divanovic A, Hor K, Cnota JF, Hirsch R, Kinsel-Ziter M, Michelfelder EC. Prediction and Perinatal Management of Severely Restrictive Atrial Septum in Fetuses With Critical Left Heart Obstruction: Clinical Experience Using Pulmonary Venous Doppler Analysis. J Thorac Cardiovasc Surg. 2011 Apr;141(4):988-94.
Kinsel-Ziter ML, Cnota JF, Crombleholme TM, Michelfelder EC. Twin-Reversed Arterial Perfusion Sequence: Pre- and Post- Operative Cardiovascular Findings in the Pump Twin. Ultrasound Obstet Gynecol. 2009 Nov;34(5):550-5.
Shah AD, Border WL, Crombleholme TM, Michelfelder EC. Initial Fetal Cardiovascular Profile Score Predicts Recipient Twin Outcome in Twin-Twin Transfusion Syndrome. J Am Soc Echocardiogr. 2008 Oct; 21(10):1105-8.
Michelfelder EC, Gottliebson WM, Border WL, Kinsel ML, Polzin WJ, Livingston JC, Khoury P, Crombleholme TM. Early Manifestations and Spectrum of Recipient Twin Cardiomyopathy in Twin-Twin Transfusion Syndrome: Relation to Quintero Stage. Ultrasound Obstet Gynecol. 2007 Dec;30(7):965-71.
Michelfelder EC, Gomez CA, Border W, Gottliebson W, Franklin C. Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle. Circulation. 2005 Nov 8;112(19):2974-79.
Douglas Millay, PhD
BS: Northern Kentucky University, Highland Heights, KY, 2002.
PhD: University of Cincinnati, Cincinnati, OH, 2008.
Fellowship: University of Texas Southwestern, Dallas, TX, 2014.
Millay DP, O’Rourke JR, Sutherland LB, Bezprozvannaya S, Shelton JM, Bassel-Duby R, Olson EN. Myomaker is a membrane activator of myoblast fusion and muscle formation. Nature. 2013 Jul 18;499 (7458).
Millay DP, Olson EN. Making muscle or mitochondria by selective splicing of PGC1α. Cell Metab. 2013 Jan 8;17(1):3-4.
Hatley ME, Tang W, Garcia MR, Finkelstein D, Millay DP, Liu N, Graff J, Galindo RL, Olson EN. A mouse model of rhabdomyosarcoma originating from the adipocyte lineage. Cancer Cell. 2012 Oct 16;22 (4): 536-46.
Zeve D, Seo J, Suh JM, Stenesen D, Tang W, Berglund ED, Wan Y, Williams LJ, Lim A, Martinez MJ, McKay RM, Millay DP, Olson EN, Graff JM. Wnt signaling activation in adipose progenitors promotes insulin-independent muscle glucose uptake. Cell Metab. 2012 Apr 4;15(4):492-504.
Pei J, Millay DP, Olson EN, Grishin NV. CREST--a large and diverse superfamily of putative transmembrane hydrolases. Biol Direct. 2011 Jul 6;6:37.
Goonasekera SA, Lam CK, Millay DP, Sargent MA, Hajjar RJ, Kranias EG, Molkentin JD. Mitigation of muscular dystrophy in mice by SERCA overexpression in skeletal muscle. J Clin Invest. 2011 Mar;121(3):1044-52.
Wissing ER, Millay DP, Vuagniaux G, Molkentin JD. Debio-025 is more effective that prednisone in reducing muscular pathology in mdx mice. Neuromuscul Disord. 2010 Nov;20(11):753-60.
Millay DP, Goonasekera SA, Sargent MA, Molkentin JD. Calcium influx is sufficient to induce muscular dystrophy through a TRPC-dependent mechanism. Proc Natl Acad Sci USA. 2009 Nov 10;106(45)19023-8.
Millay DP, Sargent MA, Roche JA, Maillet M, McNally EM, Bloch RJ, Molkentin JD. Genetic manipulation of dysferlin expression in skeletal muscle: Novel insights into muscular dystrophy. Am J Pathol. 2009 Nov;175(5):1817-23.
Millay DP, Sargent MA, Osinska H, Baines CP, Barton ER, Vuagniaux G, Sweeney HL, Robbins J, Molkentin JD. Genetic and pharmacologic inhibition of mitochondrial-dependent necrosis attenuates muscular dystrophy. Nat Med. 2008 Apr;14(4):442-7.
Jeffery D. Molkentin, PhD Professor | Howard Hughes Medical Institute Investigator
Professor | Howard Hughes Medical Institute Investigator
Davis J, Burr AR, Davis GF, Birnbaumer L, Molkentin JD. A TRPC6-dependent pathway for myofibroblast transdifferentiation and wound healing in vivo. Dev. Cell 2012. 23:705-715.
Auger-Messier M, Accornero F, Goonasekera SA, Bueno OF, Lorenz JN, van Berlo JH, Willette RN, Molkentin JD. Unrestrained p38 MAPK Activation in Dusp1/4 Double Null Mice Induces Cardiomyopathy. Circ Res. 2012. In Press.
Lorts A, Schwanekamp JA, Baudino TA, McNally EM, Molkentin JD. Deletion of periostin reduced muscular dystrophy and fibrosis in mice by modulating the transforming growth factor-b pathway. Proc Natl Acad Sci USA. 2012. 109:10978-10983
Lynch JM, Maillet M, Vanhoutte D, Schloemer A, Sargent MA, Blair NS, Lynch KA, Okada T, Aronow BJ, Osinska H, Prywes R, Lorenz JN, Mori K, Lawler J, Robbins J, Molkentin JD. A thrombospondin-dependent pathway for a protective ER stress response. Cell. 2012. 149,1257-1268
Liu Q, Chen Y, Auger-Messier M, Molkentin JD. Interaction Between NFkB and NFAT Coordinates Cardiac Hypertrophy and Pathological Remodeling. Circ Res. 2012. 110:1077-1086
Goonasekera SA, Molkentin JD. Unraveling the secrets of a double life: Contractile versus signaling Ca(2+) in a cardiac myocyte. J Mol Cell Cardiol. 52:317-322. 2012.
Goonasekera SA, Hammer K, Auger-Messier M, Bodi I, Chen X, Zhang H, Reiken S, Elrod JW, Correll RN, York AJ, Sargent MA, Hofmann F, Moosmang S, Marks AR, Houser SR, Bers DM, Molkentin JD. Decreased cardiac L-type Ca2+ channel activity induces hypertrophy and heart failure in mice. J. Clin Invest. 2012. 122:280-290
David L. S. Morales, MD Chief of Cardiovascular Surgery, Heart Institute, Cardiothoracic Surgery
Chief of Cardiovascular Surgery, Heart Institute, Cardiothoracic Surgery
Clark-Helmsworth Chair of Cardiovascular Surgery
UC Division of Surgery
Congenital heart surgery; heterotaxy syndrome; pediatric heart and lung transplantation; mechanical circulatory support
Clinical and translational research related to mechanical circulatory support and tissue engineering
MD: Yale University School of Medicine, New Haven, CT.
Residency: General and cardiothoracic surgery, New York-Presbyterian Medical Center, Columbia University, New York, NY.Fellowships: Congenital heart surgery, Texas Children’s Hospital, Baylor University, Waco, TX.
Board certifications: Surgery, thoracic surgery, congenital heart surgery.
Nick Pratap, MB BChir, MRCPCH, FRCA Congenital Cardiac Anesthesiologist, Department of Anesthesia
investigates the use of monitoring in the operating room and cardiac intensive care unit. The goal of his research is to improve clinical outcomes after open heart surgery in young infants. He is also working on novel ways of partnering with families to prepare their children for surgery.
Congenital Cardiac Anesthesiologist, Department of Anesthesia
Attending Cardiac Intensivist, Heart Institute
Assistant Professor, UC Department of Anesthesia
Congenital cardiac anesthesia; general pediatric anesthesia; pediatric liver transplant anesthesia; cardiac intensive care medicine
MA (Cantab): University of Cambridge, UK, 1998.
MB BChir: University of Cambridge, UK, 1998.
Pediatric Residency: Royal College of Paediatrics and Child Health, London, UK, 2002.
Anesthesia Residency: Royal College of Anaesthetists, London, UK, 2006.
Pediatric Anesthesia Fellowship: Great Ormond Street Hospital, London, UK, 2008.
Congenital Cardiac Anesthesia Fellowship: Royal Brompton Hospital, London, UK, 2009.
Pediatric Intensive Care Fellowship: Birmingham Children's and Great Ormond Street Hospitals, UK, 2010.
Pratap JN, Varughese AM, Adler E, Kurth CD. Getting started with the model for improvement: psychology and leadership issues in quality improvement. Br J Hosp Med (Lond). 2013 Feb;74(2):104-8.
Pratap JN, Varughese AM, Adler E, Kurth CD. Getting started with the model for improvement: the model in practice. Br J Hosp Med (Lond). 2013 Jan;74(1):42-6.
Pratap JN, Varughese AM, Adler E, Kurth CD. Getting started with the model for improvement: introduction and understanding variation. Br J Hosp Med (Lond). 2012 Dec;73(12):701-5.
Pratap JN, Wilmshurst S. Anaesthetic management of children with in situ Berlin Heart EXCOR. Pediatr Anesth. 2010 Sep;20(9):812-20.
Pratap JN, McEwan AI. Failure to wean from cardiopulmonary bypass due to left atrial compression by periaortic hematoma. Anesth Analg. 2009 Jul;109(1):35-7.
Pratap JN, Harding L. Critical overfilling of a vaporizer. Eur J Anaesthesiol. 2009 Jan;26(1): 90-1.
Pratap JN, Sekhri C, Lloyd-Thomas AR. Anaesthetic management for adenotonsillectomy of a child with severe obesity due to homozygous melanocortin-4 receptor gene mutations. Paediatr Anaesth. 2009 Feb;19(2):195-6.
Pratap JN, Shankar RK, Goroszeniuk T. Co-injection of clonidine prolongs the analgesic effect of lidocaine skin infiltration by a peripheral action. Anesth Analg. 2007 Apr;104(4):982-3.
Pratap JN, Down JF. Posterior reversible encephalopathy syndrome: a report of a case with atypical features. Anaesthesia. 2008 Nov;63(11):1245-8.
Pratap JN, Clements E, Levy D. Prevalence of obesity and undiagnosed glucose intolerance in a UK district general hospital day surgery unit. Practical Diabetes International. 2006 23(9):408-12.
Enkhsaikhan Purevjav, MD, PhD Research Assistant Professor
focuses on identifying and screening the potential genes responsible for inherited and acquired cardiac diseases, creating in vitro and in vivo cardiomyopathy models and performing functional studies of mutations identified. Additionally, she studies the effects of factors such as viral infections, drugs (ACE inhibitors and beta-blockers) and mechanical stress (cyclic mechanical stretch, acute and chronic exercise) on cardiac function.
Research Assistant Professor
Cardiac disease; genetic abnormalities; cardiac mechanosensing; cardiomyopathy
Enkhsaikhan Purejav's, MD, PhD is focused on screening the potential genes responsible for inherited and acquired cardiac diseases and performing functional studies of mutations in these genes by creating in vitro and in vivo models. In addition, she investigates the effects of factors such as viral infections, drugs including ACE inhibitors and beta-blockers, and mechanical stress (cyclic mechanical stretch, acute and chronic exercise) on cardiac function in these models.
MD: Leningrad Pediatric Medical Institute, Russia, 1989
Residency: Saint’s Petersburg Pediatric Medical Academy, Russia
Certification: Pediatric Cardiology, 1994
PhD: Shimane Medical University, Japan, 2003
Purevjav E, Arimura T, Augustin S, Huby AC, Takagi K, Nunoda S, Kearney DL, Taylor MD, Terasaki F, Bos JM, Ommen SR, Shibata H, Takahashi M, Itoh-Satoh M, McKenna WJ, Murphy RT, Labeit S, Yamanaka Y, Machida N, Park JE, Alexander PM, Weintraub RG, Kitaura Y, Ackerman MJ, Kimura A, Towbin JA. Molecular basis for clinical heterogeneity in inherited cardiomyopathies due to myopalladin mutations. Hum Mol Genet. 2012 May 1;21(9):2039-53.
Purevjav E, Varela J, Morgado M, Kearney DL, Li H, Taylor MD, Arimura T, Moncman CL, McKenna W, Murphy RT, Labeit S, Vatta M, Bowles NE, Kimura A, Boriek AM, Towbin JA. Nebulette mutations are associated with dilated cardiomyopathy and endocardial fibroelastosis. J Am Coll Cardiol. 2010 Oct 26;56(18):1493-502.
Samani K, Wu G, Ai T, Shuraih M, Mathuria NS, Li Z, Sohma Y, Purevjav E, Xi Y, Towbin JA, Cheng J, Vatta M. A novel SCN5A mutation V1340I in Brugada syndrome augmenting arrhythmias during febrile illness. Heart Rhythm. 2009 Sep;6(9):1318-26.
Wu G, Ai T, Kim JJ, Mohapatra B, Xi Y, Li Z, Abbasi S, Purevjav E, Samani K, Ackerman MJ, Qi M, Moss AJ, Shimizu W, Towbin JA, Cheng J, Vatta M. alpha-1-syntrophin mutation and the long-QT syndrome: a disease of sodium channel disruption. Circ Arrhythm Electrophysiol. 2008 Aug;1(3):193-201.
Purevjav E, Nelson DP, Varela JJ, Jimenez S, Kearney DL, Sanchez XV, DeFreitas G, Carabello B, Taylor MD, Vatta M, Shearer WT, Towbin JA, Bowles NE. Myocardial Fas ligand expression increases susceptibility to AZT-induced cardiomyopathy. Cardiovasc Toxicol. 2007;7(4):255-63.
Hardarson HS, Baker JS, Yang Z, Purevjav E, Huang CH, Alexopoulou L, Li N, Flavell RA, Bowles NE, Vallejo JG. Toll-like receptor 3 is an essential component of the innate stress response in virus-induced cardiac injury. Am J Physiol Heart Circ Physiol. 2007 Jan;292(1):H251-8.
Purevjav E, Kimura M, Takusa Y, Ohura T, Tsuchiya M, Hara N, Fukao T, Yamaguchi S. Molecular study of electron transfer flavoprotein alpha-subunit deficiency in two Japanese children with different phenotypes of glutaric acidemia type II. Eur J Clin Invest. 2002 Sep;32(9):707-12.
Robert M. Siegel, MD, FAAP Medical Director, Center for Better Health and Nutrition
Medical Director, Center for Better Health and Nutrition
Pediatric obesity prevention and treatment
Robert M. Siegel is a professor of Clinical Pediatrics at the University of Cincinnati and medical director of the Center for Better Health and Nutrition of the Heart Institute. He graduated Magna cum laude from Hunter College of the City University of New York and earned his MD from New York University. Dr. Siegel did his pediatric residency at Cincinnati Children’s Hospital. He was a general pediatrician for more than twenty years before joining the Heart Institute and was president of the Medical Staff at Cincinnati Children’s from 2008 to 2010. Dr. Siegel’s research interests include dietary and physical activity interventions in obese children.
MD: New York University, School of Medicine, New York, NY, 1984.
Residency: Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 1984-87.
Fellowship: Infectious Diseases, Cincinnati Children’s Hospital Medical Center, 1992. American Board of Pediatrics, 1988.
Certification: Pediatrics, 1988.
Kanetzke EE, Lynch J, Prows CA, Siegel RM, Myers MF. Perceived Utility of Parent-Generated Family Health History as a Health Promotion Tool in Pediatric Practice. Clinical Pediatrics. 2011;50:720-8.
Siegel RM, Neidhard MS, Kirk S. A Comparison of Reduced Glycemic Load and Staged Portion-Controlled Diets in Improving BMI and Other Health Outcomes of Obese Children in a Pediatric Weight Management Program. Clinical Pediatrics. 2011;50:459-461.
Siegel RM, Rich W, Khoury J. An Office-Based Low Carbohydrate Intervention in Teens: One Year Follow-up of a Six-Month Intervention. Clinical Pediatrics. 2011;50:459-461.
Siegel RM. Acute Otitis Media Guidelines, Antibiotic Use, and Shared Medical Decision-Making. Pediatrics. 2010;125:384–386.
Siegel RM, Rich W, Joseph EC, Linhardt J, Knight K, Khoury J, Daniels SR. A Six Month Office-Based Low Carbohydrate Diet Intervention in Obese Teens. Clinical Pediatrics. 2009;48:745-749.
Siegel RM, Schubiner J, Sammarco VJ. A non-operative technique for peroneal tendon subluxation. Clinical Pediatrics. 2008;47(3):300-1.
Olson L, Radecki L, Frintner MP, Weiss K, Siegel R. At what age can children report their asthma health status? Pediatrics. Jan 2007;119:e93-e102.
Siegel RM, Bien J, Lichtenstein P, Davis J, Khoury J, Knight J, Kiely M, Bernier J. A safety-net prescription for otitis media: the effects of a PBRN study on patients and practitioners. Clinical Pediatrics. 2006;45:518-524.
Zink T, Siegel RM, Chen L, Levin L, Pabst S, Putnam F. Physician Knowledge and Management of Children Exposed to Domestic Violence in Ohio: A Comparison of Pediatricians and Family Physicians. Clinical Pediatrics. 2005;44:211-219b.
Siegel RM. Antibiotics and acute otitis media: Reducing overuse and drug resistance. Fam Prac Recertification. 2004;26:92-97.
James P. Spaeth, MD Director, Cardiac Anesthesia
is a pediatric cardiac anesthesiologist and the Director of Cardiac Anesthesia. His clinical research interests include the effect of congenital cardiac disease and cardiac surgery on neurodevelopmental outcomes, and the use of cerebral monitoring during the perioperative period. He is also involved in Quality Improvement work focused on improving the safety of pediatric anesthesia.
Director, Cardiac Anesthesia
MD: University of Virginia School of Medicine, Charlottesville, VA, 1993.
Internship: Internal Medicine, University of Virginia Medical Center, Charlottesville, VA, 1994.
Residency: Anesthesiology, University of California, San Diego Medical Center, San Diego, CA, 1997.
Fellowship: Pediatric Anesthesiology, Children's Hospital of Philadelphia, Philadelphia, PA, 1998.
Certification: Anesthesiology, 1998.
Gunter JB, McAuliffe JJ, Beckman EC, Wittkugel EP, Spaeth JP, Varughese AM. A factorial study of ondansetron, metoclopramide, and dexamethasone for emesis prophylaxis after adenotonsillectomy in children. Paediatr Anaesth. 2006 Nov;16(11):1153-65.
Dent CL, Spaeth JP, Jones BV, Schwartz SM, Glauser TA, Hallinan B, Pearl JM, Khoury PR, Kurth CD. Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion. J Thorac Cardiovasc Surg. 2006 Jan;131(1):190-7. Aronson LA, Spaeth JP. Frontiers in pediatric anesthesia: cardiac anesthesia. Int Anesthesiol Clin. 2006 Winter;44(1):33-49. Dent CL, Spaeth JP, Jones BV, Schwartz SM, Glauser TA, Hallinan B, Pearl JM, Khoury PR, Kurth CD. Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion. J Thorac Cardiovasc Surg. 2005 Dec;130(6):1523-30.
Loepke AW, Spaeth JP. Glucose and Heart Surgery: Neonates Are Not Just Small Adults. Anesthesiology. 2004 Jun;100(6):1345-52.Baker RC, Schubert CJ, Kirwan KA, Lenkauskas SM, Spaeth JT. After-hours telephone triage and advice in private and nonprivate pediatric populations. Arch Pediatr Adolesc Med. 1999 Mar;153(3):292-6.
Michael D. Taylor, MD Director, Advanced Imaging Innovation
is a non-invasive cardiologist interested in applications of imaging to problems of congenital and acquired heart disease. His primary research interest is cardiac MRI and its application in evaluating myocardial function and disease. He has a translational program that uses multi-modality imaging to characterize mouse models of inherited and acquired cardiomyopathies.
Director, Advanced Imaging Innovation
Cardiac MRI; Cardiac CT; and echocardiography
Myocardial metabolism; animal models of heart failure; positron emission tomography
Michael Taylor, MD joined the Heart Institute in July, 2010 as the director of Advanced Imaging Innovation. He was previously the director of cardiac magnetic resonance at Texas Children’s Hospital in Houston.
Dr. Taylor has expertise in cardiac magnetic resonance imaging, positron emission tomography and computed tomography. He has a dual appointment in the Cincinnati Children's Hospital Medical Center Imaging Research Center, an interdisciplinary facility of advanced imaging faculty with dedicated clinical and pre-clinical imaging equipment.
Dr Taylor's primary interests include myocardial metabolism in heart failure, animal models of cardiac pathology, and cardiac magnetic resonance imaging of congenital and acquired heart disease.
MD: University of Wisconsin, Madison, WI; 2001.PhD: Medical Physics, University of Wisconsin, Madison, WI; 2001.
Residency: Pediatrics, Baylor College of Medicine, Houston, TX; 2003.Fellowship: Pediatric Cardiology, Baylor College of Medicine, Houston, TX; 2006.Certification: Pediatrics; 2005.Certification: Pediatric Cardiology; 2006.
Mazur W, Hor KN, Germann JT, Fleck RJ, Al-Khalidi HR, Wansapura JP, Chung ES, Taylor MD, Jefferies JL, Woodrow Benson D, Gottliebson WM. Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain. Int J Cardiovasc Imaging. 2011 Jan 8. Purevjav E, Varela J, Morgado M, Kearney DL, Li H, Taylor MD, Arimura T, Moncman CL, McKenna W, Murphy RT, Labeit S, Vatta M, Bowles NE, Kimura A, Boriek AM, Towbin JA. Nebulette mutations are associated with dilated cardiomyopathy and endocardial fibroelastosis. J Am Coll Cardiol. 2010 Oct 26;56(18):1493-502.
LP Browne, D Kearney, MD Taylor, T Chung, TC Slesnick, AC Nutting, R Krishnamurthy. ALCAPA: the role of myocardial viability studies in determining prognosis . Pediatr Radiol. 2010;40(2):163-167. JL Jefferies, MD Taylor, J Rossano, JW Belmont, WJ Craigen. Novel cardiac findings in periventricular nodular heterotopia . Am J Med Genet. 2009; 152A(1): 165-168. JW Rossano, MD Taylor, EO Smith, CD Fraser, ED McKenzie, JF Price, HA Dickerson, DP Nelson, AR Mott. Glycemic profile in infants who have undergone the arterial switch operation: hyperglycemia is not associated with adverse events . J Thorac Cardiovasc Surg. 2008; 135(4): 739-745.
Elaine M. Urbina, MD, MS Director, Preventive Cardiology
is a pediatric preventive cardiologist who is interested in how CV risk factors affect vascular function. Her lab uses a variety of non-invasive techniques to measure how vascular structure (carotid intima-media thickness), arterial stiffness (pulse wave velocity) and endothelial function (brachial flow mediated dilation) are affected by a variety of pediatric diseases.
Director, Preventive Cardiology
Elaine M. Urbina, MD, graduated from the Medical College of Georgia in 1988. She subsequently trained in pediatrics at the University of California at San Francisco. She completed her pediatric cardiology fellowship at Tulane University in New Orleans, Louisiana, in 1994 where she accepted an assistant professor position.
In addition to practicing clinical pediatric cardiology at Tulane, she was also director of preventive cardiology and the Pediatric Echocardiography Laboratory. Her research interests led her to join the world-renowned Bogalusa Heart Study, one of the longest running epidemiologic studies of CV risk factors in children. As co-investigator under the tutelage of Dr. Gerald S. Berenson, she initiated the non-invasive imaging program and supervised collection of CV data for over a decade.
Dr. Urbina joined Cincinnati Children's Hospital Medical Center as associate professor in October of 2003 and became director of preventive cardiology in 2006. This new opportunity allowed her to concentrate her clinical practice on the diagnosis and treatment of high blood pressure and cholesterol. She continues her research studies concentrating on non-invasive imaging methods to measure atherosclerosis with a focus on vascular function abnormalities using the modalities of:
Her current research includes the application of these new techniques to study the roles that pre- and post-natal nutrition, overweight, high blood pressure, cholesterol and diabetes play in the development of hardening of the arteries.
MD: Medical College of Georgia, Augusta, GA, 1988.
Residency: University of California, San Francisco, CA, 1990; Tulane University, New Orleans, LA 1991 (Pediatrics).
Fellowship: Tulane University, New Orleans, LA, 1994 (Pediatric Cardiology).
Certification: Pediatrics 1991; Pediatric Cardiology 1994.
Urbina EM, Khoury PR, McCoy C, Daniels SR, Kimball TR, Dolan LM. Cardiac and vascular consequences of pre-hypertension in youth. J Clin Hypertens (Greenwich). May 2011;13(5):332-342.
Urbina EM, Dolan LM, McCoy CE, Khoury PR, Daniels SR, Kimball TR. Relationship between elevated arterial stiffness and increased left ventricle mass in adolescents and young adults. J Pediatr. May 2011;158(5):715-721.
Urbina EM, Kimball TR, Khoury PR, Daniels SR, Dolan LM. Increased arterial stiffness is found in adolescents with obesity or obesity-related type 2 diabetes mellitus. J Hypertens. Aug 2010;28(8):1692-1698.
Urbina EM, Wadwa RP, Davis C, Snively BM, Dolan LM, Daniels SR, Hamman RF, Dabelea D. Prevalence of increased arterial stiffness in children with type 1 diabetes mellitus differs by measurement site and sex. J Pediatr. May 2010;156(5):731-737.
Urbina EM, Kimball TR, McCoy CE, Khoury PR, Daniels SR, Dolan LM. Youth with obesity and obesity-related type 2 diabetes mellitus demonstrate abnormalities in carotid structure and function. Circulation. Jun 2009;119(22):2913-2919.
Urbina EM, Bean JA, Daniels SR, D’Alessio D, Dolan LM. Overweight and hyperinsulinemia provide individual contributions to comprises in brachial artery distensibility in healthy adolescents and young adults: brachial distensibility in children. J Am Soc Hypertens. Jun 2007;1(3):200-207.
Urbina EM, Kieltkya L, Tsai J, Srinivasan SR, Berenson GS. Impact of multiple cardiovascular risk factors on brachial artery distensibility in young adults: The Bogalusa Heart Study. Am J Hypertens. Jun 2005;18:767-771.
Urbina EM, Srinivasan SR, Kieltyka RL, Tang R, Bond MG, Chen W, Berenson GS. Correlates of carotid artery stiffness in young adults: The Bogalusa Heart Study. Atherosclerosis. Sep 2004;176(1):157-164.
Urbina EM, Srinivasan SR, Tang R, Bond MG, Kieltyka L, Berenson GS. Impact of multiple coronary risk factors on the intima-media thickness of different segments of carotid artery in healthy young adults: The Bogalusa Heart Study. Am J Cardiol. Nov 2002;90(9):953-958.
Urbina EM, Gidding SS, Bao W, Pickoff AS, Berdusis K, Berenson GS. Effect of body size, ponderosity and blood pressure of the left ventricular growth in children and young adults in Bogalusa Heart Study. Circulation. May 1995;91(9):2400-2406.
Accelerated CV Aging in Youth Related to CV Risk Factor Clusters. Principal Investigator. National Heart, Lung and Blood Institute. Jan 2011-Dec 2016.
SEARCH 3: Cohort Study - Ohio site. Co-Investigator. Centers for Disease Control & National Institutes of Health. Sept 2010-Oct 2015.
Gruschen R. Veldtman, FRCP, MBChB Director of Inpatient ACHD Services, Heart Institute
is a clinician scientist who conducts research in the areas of single ventricle and Fontan physiology and pathophysiology; Eisenmenger syndrome and arrhythmia; contractile reserve in tetralogy of Fallot; and hepatopathy in the Fontan circulation and heart failure.
Director of Inpatient ACHD Services, Heart Institute
Spectrum of adult congenital heart disease: the Fontan circulation; interventional cardiology; pregnancy cardiology; pulmonary hypertension in congenital heart disease
Gruschen R. Veldtman, FRCP, MBChB, is appointed in the Heart Institute at Cincinnati Children's Hospital Medical Center. His major achievement has been the establishment of patient and science centered clinical programs that serve adult patients with various complexity of congenital heart conditions. His major academic contributions have been in the field of liver disease as it relates to the Fontan circulation.
General Paediatrics and Neonates, Royal Hospital for Sick Children, Glasgow, 1995.
Paediatric Cardiology, Leeds General Infirmary, 1999.
Fellowship: Adult Congenital Heart Disease, Toronto 2000.
Research Fellowship: Hospital For Sick Children Toronto, 2001.
Fellowship: Adult Congenital Heart Disease, Mayo Clinic, 2002.
Fellowship: Paediatric Interventional Cardiology, 2003.
Joshua S. Waxman, PhD Assistant Professor
Understanding the molecular underlying nature of congenital heart defects; cardiomyocyte formation
Visit the Waxman Lab.
D’Aniello, E., Rydeen, A., Anderson, J., Mandal, A., and Waxman, J.S.Depletion of retinoic acid receptors initiates a novel positive feedback mechanism that promotes teratogenic increases in retinoic acid. PLoS Genet. Aug. 9(8); e1003689. 2013.
Sorrell, M.R., Dohn T.E., D’Aniello, E., Waxman, J.S. Tcf7l1 proteins cell autonomously restrict cardiomyocyte and promote endothelial specification in zebrafish. Dev Biol. 380(2); 199-210. 2013.
Mandal, A., Rydeen, A., Anderson, J., Sorrell, M.R., Zygmunt, T., Torres-Vázquez, J. and Waxman,J.S. Transgenic retinoic acid sensor lines in zebrafish indicate regions of available embryonic retinoic acid. Developmental Dynamics. Aug. 242(8); 989-1000. 2013.
Dohn TE, Waxman JS. Distinct phases of Wnt/β-catenin signaling direct cardiomyocyte formation in zebrafish. Dev Biol. Jan 15;361(2):364-76. 2012.
Sorrell MR, Waxman JS. Restraint of Fgf8 signaling by retinoic acid signaling is required for proper heart and forelimb formation. Dev. Biol. Oct 1, 2011. ;358(1):44-55. Epub Jul 22, 2011.
Waxman JS, Yelon D. Zebrafish retinoic acid receptors function as context-dependent transcriptional activators. Dev Biol. 352:128-40. 2011.
Feng L, Hernandez RE, Waxman JS, Yelon D, Moens CB. Dhrs3a regulates retinoic acid biosynthesis through a feedback inhibition mechanism. Dev Biol. 338(1):1-14. Feb 1, 2010.
Waxman JS, Yelon D. Increased Hox activity mimics the teratogenic effects of excess retinoic acid signaling. Dev Dyn. 238(5):1207-13. May, 2009.
Linville A, Radtke K, Waxman JS, Yelon D, Schilling TF. Combinatorial roles for zebrafish retinoic acid receptors in the hindbrain, limbs and pharyngeal arches. Dev Biol. 325(1):60-70. Jan 1, 2009.
Waxman JS, Keegan BR, Roberts RW, Poss KD, Yelon D. Hoxb5b acts downstream of retinoic acid signaling in the forelimb field to restrict heart field potential in zebrafish. Dev Cell. 15(6):923-34. Dec, 2008.
Ivan Wilmot, MD Heart Failure, Transplant, VAD Physician
focuses on care of children with advanced heart failure requiring mechanical circulatory support bridge to transplant; quality of life in children with heart failure, transplant, and mechanical circulatory support (MCS); and advanced strain imaging in evaluation of ventricular function in pediatric heart transplant patients.
Heart Failure, Transplant, VAD Physician
Heart failure, transplantation, and mechanical circulatory support.
Ivan Wilmot, MD, graduated summa cum laude from Louisiana State University with a Bachelor's of Science in zoology.
In 1998 he began medical school at Emory University. His general pediatrics residency training took place at the Emory Affiliated Hospitals from 2002 to 2006. He completed his pediatric cardiology fellowship training from 2006 to 2009, and advanced fellowship in heart failure and transplant from 2009 to 2010.
His clinical and research interests focus on heart failure, cardiac transplantation and mechanical circulatory support (MCS).
BS: Louisiana State University, 1998.
MD: Emory University School of Medicine, Atlanta, GA, 2002.
Residency: Emory Affiliated Hospitals, Atlanta, GA, 2005; Chief Resident: Emory Affiliated Hospitals, Atlanta, GA, 2006.
Fellowship: Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, 2009.
Advanced Fellowship: Heart Failure and Transplant, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, 2010.
Certification: American Board of Pediatrics; American Board of Pediatrics Sub-board of Pediatric Cardiology.
Stapleton GE, Wilmot I, Suh EJ. Cardiac catheterisation of patients with common arterial trunk and transposition of the great arteries. Cardiol Young. 2012 Dec:22(6), 687–690.
Dadlani GH, Braley K, Perez-Colon E, Stapleton G, Crawford M, Turpin D, Wilmot I, Freire G, Decker J, Martinez R. Long-term management of patients with hypoplastic left heart syndrome: the diagnostic approach at All Children's Hospital. Cardiol Young. 2011 Dec:21 Suppl 2, 80–87.
Asante-Korang A, Jacobs JP, Ringewald J, Carapellucci J, Rosenberg K, McKenna D, McCormack J, Wilmot I, Gjeldum A, Lopez-Cepero M, Sleasman J. Management of children undergoing cardiac transplantation with high Panel Reactive Antibodies. Cardiol Young. 2011 Dec:21 Suppl 2, 124–132.
Wilmot I, Morales DL, Price JF, Rossano JW, Kim JJ, Decker JA, McGarry MC, Denfield SW, Dreyer, WJ, Towbin JA, Jeffries JL. Effectiveness of mechanical circulatory support in children with acute fulminant and persistent myocarditis. J Card Fail. 2011 June:17(6), 487–494.
Perez-Colon E, Dadlani GH, Wilmot I, Miller M. Mesalamine-induced myocarditis and coronary vasculitis in a pediatric ulcerative colitis patient: a case report. Case Rep Peditr. 2011; 524364.
Sharef S, Coleman R, Rivenes S, Wilmot I, Altman C, Madden-Fuentes R, Salazar J. Stentless xenograft implantation in aortic position in a 7-year-old girl. Tex Heart Inst J. 2010; 37(3), 347–349.
Wilmot I, Kanter KR, Vincent RN, Berg AM, Mahle WT. OKT3 treatment in refractory pediatric heart transplant rejection. J Heart Lung Transplant. 2005 Nov;24(11):1793-7.
Katherine Yutzey, PhD
is focused on the molecular mechanisms of heart development and disease. Particular emphasis is on signaling pathways and transcription factors that control heart valve development as well as contribute to pediatric and adult valve disease. Additional projects address the development of coronary vasculature, cardiac fibrosis and maturation of cardiac muscle after birth.
Visit the Yutzey Lab.
Molecular regulation of heart development; valve development and disease mechanisms; cardiomyocyte proliferation, cardiac connective tissue lineages.
Visit Dr. Yutzey's Lab Web Site.
Katherine E. Yutzey, PhD, is a professor who joined the Division of Molecular Cardiovascular Biology at Cincinnati Children's Hospital Medical Center in 1995.
Dr. Yutzey is the first recipient of the Fifth Third Bank/Charlotte R. Schmidlapp Women Scholars Award and was also a recipient of a Children's Hospital Medical Center Trustee Award. Her work is also supported by grants from National Institutes of Health (NIH) and the American Heart Association.
The focus of Dr. Yutzey's research program is the regulation of normal and abnormal heart development. Congenital heart defects represent one of the most common classes of human birth defects. Increasing evidence exists for a genetic basis of certain instances of congenital heart disease.
Dr. Yutzey’s lab also studies molecular mechanisms of cardiac connective tissue cell lineage development and disease. This work focuses on heart valve development and disease as well as mechanisms of cardiac fibrosis.
BA: Oberlin College, Oberlin, OH, 1986.
PhD: Purdue University, West Lafayette, IN,1992
Fellowship: Cornell University Medical College, New York, NY,1992-1995.
Sengupta A, Kalinichenko VV, Yutzey KE. FoxO and FoxM1 transcription factors have antagonistic functions in neonatal cardiomyocyte cell cycle withdrawal and IGF1 gene regulation. Circ. Res. 112:267-277. 2013.
Carruthers CA, Alfieri CM, Joyce EM, Watkins SC, Yutzey KE, Sacks MS. Gene expression and collagen fiber micromechanical interactions of the semilunar heart valve interstitial cell. Cell. Mol. Bioeng. 5:254-265.
Mead TJ, Yutzey KE. Notch pathway regulation of neural crest cell development in vivo. Dev. Dyn. 2012:241:376-389.
Chakraborty S, Yutzey KE. Tbx20 regulation of cardiac cell proliferation and lineage specialization during embryonic and fetal development in vivo. Dev. Biol. 2012:363:234-246.
Cheek JD, Wirrig EE, Alfieri CM, James JF, Yutzey KE. Differential activation of valvulogenic, chondrogenic and osteogenic pathways in mouse models of myxomatous and calcific aortic valve disease. J. Mol. Cell. Cardiol. 2012:52:689-700.
Le TT, Conley KW, Mead TJ, Rowan S, Yutzey KE, Brown NL. Requirements for Jag1-Rbpj mediated Notch signaling during early mouse lens development. Dev. Dyn. 2012:241:493-504.
Sengupta A, Chakraborty S, Paik J, Yutzey KE, Evans-Anderson HJ. FoxO1 is required in endothelial but not myocardial cell lineages during cardiovascular development. Dev. Dyn. 2012:241:803-813.
Fujimoto KL, Tobita K, Guan J, Hashizume R, Takanari K, Alfieri CM, Yutzey KE, Wagner WR. Placement of an elastic biodegradable cardiac patch on a subacute infarcted heart leads to cellularization with early developmental cardiomyocyte characteristics. J. Card. Fail. 2012:18:585-595.
Braitsch CM, Combs MD, Quaggin SE, Yutzey KE. Pod1/Tcf21 is regulated by retinoic acid signaling and inhibits differentiation of epicardium derived cells into smooth muscle in the developing heart. Dev. Biol. 2012:368:345-357.
Hinton RB, Yutzey KE. Heart valve structure and function in development and disease. Ann Rev. Physiol. 2011:73:29-36.
Twist1 regulation of valve progenitors.wist1 regulation of valve. National Institutes of Health. 2010-2015. R01 HL082716.
Wnt signaling in heart valve development and disease. Principal Investigator. National Institutes of Health. 2012-2016. #R01 HL094319.
Cell signaling mechanisms of calcific aortic valve disease. Principal Investigator. National Institutes of Health. 2012-2016. #R01 HL114682.
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