Clinical Interests

Congenital heart surgery; heterotaxy syndrome; pediatric heart and lung transplantation; mechanical circulatory support

Research Interests

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.

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.

 

Maloyan A, Sayegh J, Osinska H, Chua BH, Robbins J. Manipulation of death pathways in desmin-related cardiomyopathy. Circ Res. 2010 May 14;106(9):1524-32.

Terrell D, Robbins J. Protein conformation-based disease: getting to the heart of the matter. Annu Rev Physiol. 2010 Mar 17;72:15-7.

Maillet M, Davis J, Auger-Messier M, York A, Osinska H, Piquereau J, Lorenz JN, Robbins J, Ventura-Clapier R, Molkentin JD. Heart-specific deletion of CnB1 reveals multiple mechanisms whereby calcineurin regulates cardiac growth and function. J Biol Chem. 2010 Feb 26;285(9):6716-24.

Nakayama H, Bodi I, Correll RN, Chen X, Lorenz J, Houser SR, Robbins J, Schwartz A, Molkentin JD. alpha1G-dependent T-type Ca2+ current antagonizes cardiac hypertrophy through a NOS3-dependent mechanism in mice. J Clin Invest. 2009 Dec;119(12):3787-96. doi: 10.1172/JCI39724.

James J, Hor K, Moga MA, Martin LA, Robbins J. Effects of myosin heavy chain manipulation in experimental heart failure. J Mol Cell Cardiol. 2010 May;48(5):999-1006.

Heineke J, Wollert KC, Osinska H, Sargent MA, York AJ, Robbins J, Molkentin JD. Calcineurin protects the heart in a murine model of dilated cardiomyopathy. J Mol Cell Cardiol. 2010 Jun;48(6):1080-7.

Nakamura T, Gulick J, Pratt R, Robbins J. Noonan syndrome is associated with enhanced pERK activity, the repression of which can prevent craniofacial malformations. Proc Natl Acad Sci U S A. 2009 Sep 8;106(36):15436-41.

Nakamura T, Gulick J, Colbert MC, Robbins J. Protein tyrosine phosphatase activity in the neural crest is essential for normal heart and skull development. Proc Natl Acad Sci U S A. 2009 Jul 7;106(27):11270-5.

Gulick J, Robbins J. Cell-type-specific transgenesis in the mouse. Methods Mol Biol. 2009;561:91-104.

Nicolaou P, Rodriguez P, Ren X, Zhou X, Qian J, Sadayappan S, Mitton B, Pathak A, Robbins J, Hajjar RJ, Jones K, Kranias EG. Inducible expression of active protein phosphatase-1 inhibitor-1 enhances basal cardiac function and protects against ischemia/reperfusion injury. Circ Res. 2009 Apr 24;104(8):1012-20.

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. 

Clinical Interests

Pediatric electrophysiology; Syncope; Quality improvement and outcomes

Research Interests

Syncope, nutrition and growth in congenital heart disease, large database analysis, quality improvement

 

MD: University of Utah, Salt Lake City, UT

Residency: University of North Carolina, Chapel Hill, NC

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.

Cardiac catheterization; intervention for congenital heart disease; quality improvement

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.

Clinical Interests

Genetic basis of pediatric heart disease

Research Interests

Cardiovascular disease in the young;  congenital heart defects; cardiomyopathy; disorders of heart rhythm (arrhythmias); heart malformation or dysfunction

MS: Emory University, 1967.

PhD: University of North Carolina, 1970.

MD: Duke University, 1972.

Residency: Pediatrics, Duke University.

Fellowship: Cardiology, Duke University.

Certification: Pediatrics, 1976, 1993; Cardiology, 1977, 1993.

Hinton RB, Adelman-Brown J, Witt S, Krishnamurthy VK, Osinska H, Sakthivel B, James JF, Li DY, Narmoneva DA, Mecham RP, Benson DW. Elastin haploinsufficiency results in progressive aortic valve malformation and latent valve disease in a mouse model. Circ Res. 2010 Aug 20;107(4):549-57.

Wansapura JP, Hor KN, Mazur W, Fleck R, Hagenbuch S, Benson DW, Gottliebson WM. Left ventricular T2 distribution in Duchenne muscular dystrophy. J Cardiovasc Magn Reson. 2010 Mar 18;12:14.

Beery TA, Shah MJ, Benson DW. Genetic characterization of familial CPVT after 30 years. Biol Res Nurs. 2009 Jul;11(1):66-72.

Hinton RB, Martin LJ, Rame-Gowda S, Tabangin ME, Cripe LH, Benson DW. Hypoplastic left heart syndrome links to chromosomes 10q and 6q and is genetically related to bicuspid aortic valve. J Am Coll Cardiol. 2009 Mar 24;53(12):1065-71. 

Markham LW, Kinnett K, Wong BL, Benson DW, Cripe LH. Corticosteroid treatment retards development of ventricular dysfunction in Duchenne muscular dystrophy. Neuromuscul Disord. 2008;18:365-370.

Hinton RB, Jr, Andelfinger G, Sekar P, Hinton AC, Gendron RL, Michelfelder EC, Robitaille Y, Benson DW. Prenatal head growth and white matter injury in hypoplastic left heart syndrome. Ped Res 2008;64:364-369.

Hinton RB, Martin LJ, Rame-Gowda SR, Tabangin ME, Cripe LH, Benson DW. Hypoplastic left heart syndrome links to chromosomes 10q and 6q and is genetically related to bicuspid aortic valve. J Am Coll Cardiol. 2008; in press.  

Martin LJ, Ramachandran V, Cripe LH, Hinton RB, Andelfinger G, Tabangin M, Shooner K, Keddache M, Benson DW. Evidence in favor of linkage to human chromosomal regions 18q, 5q and 13q for bicuspid aortic valve and associated cardiovascular malformations. Hum Genet. 2007;121:275-284.

Zhao B, Etter L, Hinton Jr. RB, Benson DW. BMP and FGF regulatory pathways in semilunar valve precursor cells. Dev Dyn. 2007;236:971-980.

Viswanathan S, Burch JBE, Fishmann GI, Moskowitz IP, Benson DW. Immunohistochemical characterization of sinoatrial node in four atrioventricular conduction system marker mice. J Mol Cell Cardiol. 2007;42:946-953.

Hinton RB Jr, Martin LJ, Tabangin ME, Mazwi M, Cripe LH, Benson DW. Hypoplastic left heart syndrome is heritable. J Am Coll Cardiol. 2007; 50:1590-1595.

Clinical Interests

Fetal cardiology including fetal echocardiography

Research Interests

Impact of insulin sensitivity on left ventricular mass in healthy teens; blood pressure in offspring of preeclamptic mothers; fetal programming of cardiovascular outcomes; multicenter trials in pediatric heart disease

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.

MD: Univeristy of Cincinnati, Cincinnati, OH

Residency: Cincinnati Children's Hospital Medical Center, Cincinnati, OH

Fellowship: Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

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.

Gudausky TM, Pearl J, Dent CL, Kim E, Divanovic A, Spicer RL, Beekman RH 3rd. Open-chest epicardial approach to transcatheter pulmonary artery stenting following heart transplantation in an infant. Congenit Heart Dis. 2007 Jan;2(1):64-9.

Clinical Interests

Acute kidney injury; continuous renal replacement therapy; end-stage renal disease; health-related quality of life

Research Interests

Acute kidney injury epidemiology and biomarkers; health-related quality of life in ESRD; cardiovascular complications and inflammation in ESRD

MD: Columbia College of Physicians and Surgeons, New York, NY, 1990.

Residency: Baylor College of Medicine, Houston, TX.

Fellowship: Pediatric Nephrology, Children's Hospital, Boston, MA.

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.

Molecular epidemiology;  pediatric obesity;  genetics and early life influences on obesity;  insulin resistance; dyslipidemia
 

BA: Cornell University, Ithaca, NY 1993.

MHSA: University of Michigan, Ann Arbor, MI 1995.

PhD: University of Cincinnati, Cincinnati, OH 2004.

Krawczeski CD, Vandevoorde RG, Kathman T, Bennett MR, Woo JG, Wang Y, Griffiths RE, Devarajan P. Serum cystatin C is an early predictive biomarker of acute kidney injury after pediatric cardiopulmonary bypass. Clin J Am Soc Nephrol. 2010 Sep;5(9):1552-7.

Elder DA, Woo JG, D'Alessio DA. Impaired beta-cell sensitivity to glucose and maximal insulin secretory capacity in adolescents with type 2 diabetes. Pediatr Diabetes. 2010 Aug;11(5):314-21.

Martin LJ, Woo JG, Morrison JA. Evidence of shared genetic effects between pre- and postobesity epidemic BMI levels. Obesity (Silver Spring). 2010 Jul;18(7):1378-82.

Tabangin ME, Woo JG, Martin LJ. The effect of minor allele frequency on the likelihood of obtaining false positives. BMC Proc. 2009 Dec 15;3 Suppl 7:S41.

Woo JG, Guerrero ML, Altaye M, Ruiz-Palacios GM, Martin LJ, Dubert-Ferrandon A, Newburg DS, Morrow AL. Human milk adiponectin is associated with infant growth in two independent cohorts. Breastfeed Med. 2009 Jun;4(2):101-9.

Lomenick JP, Reifschneider KL, Lucky AW, Adams D, Azizkhan RG, Woo JG, Backeljauw PF. Prevalence of adrenal insufficiency following systemic glucocorticoid therapy in infants with hemangiomas. Arch Dermatol. 2009 Mar;145(3):262-6.

Woo JG, Zeller MH, Wilson K, Inge T. Obesity identified by discharge ICD-9 codes underestimates the true prevalence of obesity in hospitalized children. J Pediatr. 2009 Mar;154(3):327-31.

Martin LJ, Gao G, Kang G, Fang Y, Woo JG. Improving the signal-to-noise ratio in genome-wide association studies. Genet Epidemiol. 2009;33 Suppl 1:S29-32.

Woo JG. Using body mass index Z-score among severely obese adolescents: a cautionary note. Int J Pediatr Obes. 2009;4(4):405-10.

Hugo ER, Brandebourg TD, Woo JG, Loftus J, Alexander JW, Ben-Jonathan N. Bisphenol A at environmentally relevant doses inhibits adiponectin release from human adipose tissue explants and adipocytes. Environ Health Perspect. 2008 Dec;116(12):1642-7.

Understanding how certain mutations in contractile protein genes are able to alter the function of the heart
 

MS: University of Missouri-Columbia, Columbia, Mo, 1983

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.

Clinical Interests

Cardiovascular genetics; echocardiography

Research Interests

Valve and aorta disease using human genetics and molecular developmental biology approaches

BA: Bucknell University, Lewisburg, PA.

MD: Medical University of South Carolina, Charleston, SC.

Residency: Memorial Health University Medical Center, Savannah, GA.

Fellowship: Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

Fellowship: Cincinnati Children's  Research Foundation, Cincinnati, OH.

Certification: Pediatrics, Pediatric Cardiology.

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).

Cardiovascular genetics, echocardiography, congenital heart disease, medical education, molecular cardiology, ventricular remodeling, cardiac hypertrophy.

Visit the James Lab.

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.

MD: West Virginia University, Morgantown, WV, 1987.

Residency: Vanderbilt University Medical Center, Nashville, TN, 1987-90.

Fellowship: Vanderbilt University Medical Center, Nashville, TN, 1991-94.

Certification: Pediatrics, 1990 - present

Certified: Pediatric Cardiology, 1996 - present

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. 

BS: University of Tennessee, Knoxville, TN, 1992.

MD: University of Tennessee, Knoxville, TN, 1996.

Residency: University of Kentucky, Lexington, KY, 2000.

MPH: University of Kentucky, Lexington, KY, 2001 Fellowship: Baylor College of Medicine, Houston, TX, 2006.

Certification: Internal Medicine, 2000; Pediatrics, 2001

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.

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

MS: Moscow State University, 1981

PhD: All Union Cardiology Research Center, Moscow Russia, 1987

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.

Clinical Interest

Congential heart disease; echocardiography; cardiac imaging

Research Interests

Ventricular function; transgenic mice phenotyping; cardiovascular changes with obstructive sleep apnea; cardiovascular changes with renal disease; pediatric obesity; cardiovascular changes in fit and unfit children; echocardiography quality assurance

BS: Stanford University with Honors and Distinction, Stanford, CA, 1978.

MD: New York University School of Medicine, New York City, NY, 1982. 

Residency: Children's Hospital of Los Angeles, Los Angeles, CA, 1985. 

Fellowship: Children's Hospital Medical Center, Cincinnati, OH, 1988. 

Certification: Pediatrics, 1986; Pediatric Cardiology, 1991 (recertification, 1997, 2004).

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). 2011 May;13(5):332-42. 

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.

Shah AS, Dolan LM, Gao Z, Kimball TR, Urbina EM. Clustering of risk factors: a simple method of detecting cardiovascular disease in youth. Pediatrics. 2011 Feb;127(2):e312-8.

Crowley DI, Khoury PR, Urbina EM, Ippisch HM, Kimball TR. Cardiovascular Impact of the Pediatric Obesity Epidemic: Higher Left Ventricular Mass is Related to Higher Body Mass Index. J Pediatr. 2010 Dec 11.

Shah AS, Khoury PR, Dolan LM, Ippisch HM, Urbina EM, Daniels SR, Kimball TR. The effects of obesity and type 2 diabetes mellitus on cardiac structure and function in adolescents and young adults. Diabetologia. 2010 Nov 18.

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. 2010 Aug;28(8):1692-8.

Sekar P, Border WL, Kimball TR, Hirsch R, Manning PB, Khoury PR, Beekman Iii RH. Aortic arch recoarctation after the Norwood stage I palliation: the comparative accuracy of blood pressure cuff and echocardiographic Doppler gradients in detecting significant obstruction. Congenit Heart Dis. 2009 Nov;4(6):440-7.

Warden CN, Bernard PK, Kimball TR. The efficacy and safety of oral pentobarbital sedation in pediatric echocardiography. J Am Soc Echocardiogr. 2010 Jan;23(1):33-7.

Ippisch HM, Kimball TR. Anomalous origin of the right coronary artery from the left ventricle. J Am Soc Echocardiogr. 2010 Feb;23(2):222.e1-2.

Shah AS, Dolan LM, Kimball TR, Gao Z, Khoury PR, Daniels SR, Urbina EM. Influence of duration of diabetes, glycemic control, and traditional cardiovascular risk factors on early atherosclerotic vascular changes in adolescents and young adults with type 2 diabetes mellitus. J Clin Endocrinol Metab. 2009 Oct;94(10):3740-5.

Developing effective interventions for overweight and obese children and adolescents

BS: Rutgers University, New Brunswick, NJ, 1976.

MS: University of Minnesota, St. Paul, MN, 1978; University of Southern California, Los Angeles, CA, 1988.

PhD: University of Cincinnati, Cincinnati, OH, 2003.

Inge T, Wilson KA, Gamm K, Kirk S, Garcia VF, Daniels SR. Preferential loss of central (trunk) adiposity in adolescents and young adults after laparoscopic gastric bypass. Surg Obes Relat Dis. 2007 Mar-Apr;3(2):153-8.

Lawson ML, Kirk S, Mitchell T, Chen MK, Loux TJ, Daniels SR, Harmon CM, Clements RH, Garcia VF, Inge TH; Pediatric Bariatric Study Group. One-year outcomes of Roux-en-Y gastric bypass for morbidly obese adolescents: a multicenter study from the Pediatric Bariatric Study Group. J Pediatr Surg. 2006 Jan;41(1):137-43; discussion 137-43.

Kirk S, Zeller M, Claytor R, Santangelo M, Khoury PR, Daniels SR. The relationship of health outcomes to improvement in BMI in children and adolescents. Obes Res. 2005 May;13(5):876-82.

Kirk S, Scott BJ, Daniels SR. Pediatric obesity epidemic: treatment options. J Am Diet Assoc. 2005 May;105(5 Suppl 1):S44-51. Review.

Zeller MH, Saelens BE, Roehrig H, Kirk S, Daniels SR. Psychological adjustment of obese youth presenting for weight management treatment. Obes Res. 2004 Oct;12(10):1576-86.

Zeller M, Kirk S, Claytor R, Khoury P, Grieme J, Santangelo M, Daniels S. Predictors of attrition from a pediatric weight management program. J Pediatr. 2004 Apr;144(4):466-70.

Cote MP, Byczkowski T, Kotagal U, Kirk S, Zeller M, Daniels S. Service quality and attrition: an examination of a pediatric obesity program. Int J Qual Health Care. 2004 Apr;16(2):165-73.

Inge TH, Garcia V, Daniels S, Langford L, Kirk S, Roehrig H, Amin R, Zeller M, Higa K. A multidisciplinary approach to the adolescent bariatric surgical patient. J Pediatr Surg. 2004 Mar;39(3):442-7; discussion 446-7.

Hipsky J, Kirk S. HealthWorks! Weight management program for children and adolescents. J Am Diet Assoc. 2002 Mar;102(3 Suppl):S64-7.

Clinical Interests

Cardiac electrophysiology and pacing; radio frequency ablation; tilt table testing

Research Interests

Cardiac arrest; sudden cardiac death

MD: University of Cincinnati, Cincinnati OH, 1983.

Residency: Cincinnati Children's Hospital, Cincinnati OH 1983-1986.

Fellowship (Pediatric Cardiology): Cincinnati Children's Hospital, Cincinnati OH 1986-1989.

Fellowship (Cardiac Electrophysiology): St. Vincent Hospital, Indianapolis IN 1990-1991.

Fellowship (Pediatric cardiac Electrophysiology): Texas Children's Hospital, Houston TX 1991.

Certification: Pediatrics, 1989 (Recertified 1999); Pediatric Cardiology, 1992 (Recertified 1998).

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.

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.

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.

Clinical Interests

Cardiac intensive care; heart failure; heart transplantation; pulmonary hypertension; single ventricle physiology

Research Interests

Acute kidney injury after cardiopulmonary bypass; urinary biomarkers of renal ischemia; intensive care outcomes; cardiopulmonary bypass mediated injury

MD: University of Missouri, Kansas City, Missouri, 1991.

Residency: Washington University, St. Louis Children's Hospital, St. Louis, Missouri.

Fellowships: Washington University, St. Louis Children's Hospital, St. Louis, Missouri.

Certification: American Board of Pediatrics 1995, recertification 2002; American Board of Pediatrics, Sub-board of Pediatric Critical Care 2000, recertification 2007; American Board of Pediatrics, Sub-board of Pediatric Cardiology 2002.

Krawczeski CD, Goldstein SL, Woo, JG, Wang Y, Piyaphanee N, Ma Q, Bennett M, Devarajan P. Temporal relationship and predictive value of urinary acute kidney injury biomarkers after pediatric cardiopulmonary bypass. J Am Coll Cardiol. 2011 Nov 22;58(22):2301-2309.

Zappitelli M, Krawczeski C, Devarajan P, Wang Z, Sint K, Thiessen-Philbrook H, Li S, Bennett M, Ma Q, Shlipak M, Garg A, Parikh C for the TRIBE-AKI Consortium. Early post-operative serum cystatin C predicts severe AKI after pediatric cardiac surgery: a multi-center prospective study. Kidney International. 2011, Sept;80:6555-662.

Parikh CR, Coca SG, Devarajan P, Shlipak MG, Thiessen-Philbrook H, Edelstein CL, Wang Z, Zappitelli M, Koyner J, Krawczeski CD, Patel UD, Swaminathan M, Passik CS, Garg AX; for the TRIBE-AKI Consortium. Post-operative biomarkers predict acute kidney injury and poor outcomes after cardiac surgery. J Am Soc Nephr. 2011, Sept;22:1748-1757.

Parikh CR, Devarajan P, Zappitelli M, Sint K, Thiessen-Philbrook H, Li S, Kim R, Koyner J,  Coca SG, Edelstein CL, Shlipak MG, Garg AX, Krawczeski CD; for the TRIBE-AKI Consortium. Early post-operative urine and serum biomarkers predict acute kidney injury and poor outcomes after pediatric cardiac surgery. J Am Soc Nephr. 2011, Sept;22:1737-1747.

Krawczeski CD, Woo JG, Wang Y, Bennett MR, Ma Q, Devarajan P. Neutrophil gelatinase-associated lipocalin concentrations predict development of acute kidney injury in neonates and children after cardiopulmonary bypass. J Pediatr. 2011 Jun;158(6):1009-1015.e1

Haase M, Devarajan P, Haase-Fielitz A, Bellomo R, Cruz DN, Wagener G, Krawczeski CD, Koyner JL, Murray J, Zappitelli M, Goldstein SL, Makris K, Ronco C, Martensson J, Marthing C-R, Venge P, Siew E, Ware LB, Ikizler A, Mertens PR. The outcome of neutrophil gelatinase-associated lipocalin (NGAL)-positive subclinical acute kidney injury: A multi-center pooled analysis of prospective studies. J Am Coll Cardio. 2011 Apr;57:1752-1761

Krawczeski CD, Vandevoorde RG, Kathman T, Bennett MR, Woo JG, Wang Y, Griffiths RE, Devarajan P. Serum cystatin C is an early predictive biomarker of acute kidney injury after pediatric cardiopulmonary bypass. Clin J Am Soc Nephrol. 2010 Sep;5(9):1552-7.

Ohye RG, Sleeper LA, Mahony L, Newburger JW, Pearson GD, Lu M, Goldberg CS, Tabbutt S, Frommelt FC, Ghanayem NS, Laussen PC, Rhodes JF, Lewis AB, Mital S, Ravishankar C, Williams IA, Dunbar-Masterson C, Atz AM, Colan S, Minich LL, Pizarro, C, Kanter KR, Jaggars J, Jacobs J, Krawczeski CD, Pike N, McCrindle BW, Virzi L, Gaynor JW; for the Pediatric Heart Network Investigators. Outcomes of the Norwood procedure in infants randomized to a modified Blalock-Taussig versus right ventricle-to-pulmonary artery shunt. New Engl J Med. 2010 May;362:1980-92.

Dent CL, Ma Q, Dastrala S, Bennett M, Mistnefes M, Barasch J, Devarajan P. Plasma neutrophil gelatinase-associated lipocalin predicts acute kidney injury, morbidity, and mortality after cardiac surgery: a prospective study. Criti Care. 2007 Dec;11(6):R127

Dent CL, Spaeth JP, Jones BV, Schwartz SM, Glauser TA, Hallinan B, Pearl JM, Khoury PR, Kurth CD. Brain magnetic resonance imagining abnormalities following the Norwood procedure using regional cerebral perfusion. J Thorac Cardiovas Surg. 2006 Jan;131(1):190-197.

Clinical Interests

Neonatal anesthesia; neonatal cardiac anesthesia; neonatal brain injury related to congenital heart disease; effects of anesthetics on the developing brain

Research Interests

Neonatal brain injury and protection

Visit the Loepke Lab.

MD: University of Dusseldorf, Germany, 1993.

PhD: University of Dusseldorf, Germany, 1995.

Residency: Thomas Jefferson Medical College, Philadelphia, PA, 1999.

Fellowship: Children's Hospital of Philadelphia, Philadelphia, PA, 2000.

Certification: Anesthesiology, 2000.

Maintenance of Certification: Anesthesiology 2010.

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 Feb 2

Boat AC, Sadhasivam S, Loepke AW, Kurth CD. Outcome for the extremely premature neonate: how far do we push the edge? Paediatr Anaesth. 2011 Jan 10.

Murphy BL, Pun RY, Yin H, Faulkner CR, Loepke AW, Danzer SC. Heterogeneous integration of adult-generated granule cells into the epileptic brain. J Neurosci. 2011 Jan 5;31(1):105-17.

Istaphanous GK, Ward CG, Loepke AW. The impact of the perioperative period on neurocognitive development, with a focus on pharmacological concerns. Best Pract Res Clin Anaesthesiol. 2010 Sep;24(3):433-49. Review.

Loepke AW. Developmental neurotoxicity of sedatives and anesthetics: a concern for neonatal and pediatric critical care medicine? Pediatr Crit Care Med. 2010 Mar;1 1(2):217-26. Review.

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 Sep;111(3):533-46.

Istaphanous GK, Loepke AW. General anesthetics and the developing brain. Curr Opin Anaesthesiol. 2009 Jun;22(3):368-73. Review.

Kurth CD, McCann JC, Wu J, Miles L, Loepke AW. Cerebral oxygen saturation-time threshold for hypoxic-ischemic injury in piglets. Anesth Analg. 2009 Apr;108(4):1268-77.

Danzer SC, He X, Loepke AW, McNamara JO. Structural plasticity of dentate granule cell mossy fibers during the development of limbic epilepsy. Hippocampus. 2010 Jan;20(1):113-24.

Loepke AW, Istaphanous GK, McAuliffe JJ 3rd, 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 Jan;108(1):90-104.

Clinical Interests

Heart failure; cardiopulmonary bypass mediated myocardial dysfunction

Research Interests

Myocardial remodeling

MD: Creighton University Medical School, Omaha, Nebraska, 1998.

Residency: The Children’s Hospital, Denver, Colorado, 2001.

Fellowship: Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, 2004. University of Michigan Medical Center, Ann Arbor, Michigan, 2006.

Certifications: American Board of Pediatrics, 2001; American Board of Pediatrics, Sub-Board of Pediatric Cardiology, 2004; American Board of Pediatrics, Sub-Board of Pediatric Critical Care, 2006.

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.

Signaling pathways, Cellular Biology, Cardiac Hypertrophy & Heart Failure

PhD, Paris XI University, Orsay, France, 2003

Fellowship, Cincinnati Children’s Hospital, Cincinnati, Ohio

van Berlo, J. H., Maillet, M. & Molkentin, J. D. Signaling effectors underlying pathologic growth and remodeling of the heart. J Clin Invest 123, 37-45. 2013.

Maillet, M., van Berlo, J. H. & Molkentin, J. D. Molecular basis of physiological heart growth: fundamental concepts and new players. Nat Rev Mol Cell Biol 14, 38-48. 2012.

Lynch, J. M., Maillet, M., Vanhoutte, D., Schloemer, A., Sargent, M. A., Blair, N. S., Lynch, K. A., Okada, T., Aronow, B. J., Osinska, H., Prywes, R., Lorenz, J. N., Mori, K., Lawler, J., Robbins, J. & Molkentin, J. D. 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, F. M., Wachten, D., Molkentin, J. D., Maillet, M., Aronsen, J. M., Swift, F., Sjaastad, I., Liu, N., Catalucci, D., Mikoshiba, K., Hisatsune, C., Okkenhaug, H., Andrews, S. R., Bootman, M. D. & Roderick, H. L. 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, J. M. & Molkentin, J. D. 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, V. M., Lorenz, J. N., Maillet, M., Martin, J. L., Brown, J. H., Bers, D. M., Molkentin, J. D., 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, J. D., Liu, J., Cartry, J., Vogler, G., Mohapatra, B., Otway, R. T., Huang, Y., King, I. N., Maillet, M., Zheng, Y., Crawley, T., Taghli-Lamallem, O., Semsarian, C., Dunwoodie, S., Winlaw, D., Harvey, R. P., Fatkin, D., Towbin, J. A., Molkentin, J. D., Srivastava, D., Ocorr, K., Bruneau, B. G. & 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

Clinical Interests

Cardiac intensive care; single ventricle physiology; aortic and mitral valve disease; cardiopulmonary interaction

Research Interests

Intensive care outcomes; quality of life assessment in children and adolescents with congenital and acquired heart disease; aortic valve and mitral valve surgical outcomes; cardiopulmonary interaction (shock and cardiac arrest).

MD: Harvard Medical School, Boston, MA, 1994.

MPP: Harvard University/John F. Kennedy School of Government (Masters in Public Policy - Health Care Policy), Boston, MA, 1994.

MSCE: University of Pennsylvania/Center for Clinical Epidemiology and Biostatistics, (Master of Science in Clinical Epidemiology), Philadelphia, PA, 2005.

Residency: Pediatrics, Johns Hopkins Hospital, Baltimore, MD, 1997.

Fellowships: Combined Pediatric Cardiology and Pediatric Critical Care Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA, 2002.

Certification:
American Board of Pediatrics 1997, Recertification 2004;
Sub-Board of Pediatric Cardiology 2000;
Sub-Board of Pediatric Critical Care Medicine 2002.

Goldberg D, French B, Szwast AL, McBride MG, Marino BS, Mirarchi N, Hanna BD, Wernovsky G, Paridon SM, Rychik J. Impact of sildenafil on echocardiographic indices of myocardial performance after the Fontan operation. Pediatric Cardiology 2012 (In Press)

Anderson J, Czosek RJ, Uzark K, Knilans TK, Marino BS.  The effect of pediatric syncope on quality of life. Cardiol Young 2012 (In Press)

Wray J, Franklin R, Brown K, Blyth J, Marino BS.  Linguistic validation of a disease-specific quality of life measure for children and teenagers with cardiac disease. Cardiol Young 2012; 22(1):13-17.

Wray J, Brown K, Rodney F, Marino BS. Medical test results don’t tell the whole story: health-related quality of life offers a patient perspective on outcomes. WJPCHS2011; 2(4):566-575.

Metzger A, Matsuura T, McKnite S, Marino BS, Nadkarni V, Lurie K, Yannopoulos D. Intrathoracic pressure regulation improves 24-hour survival in a pediatric porcine model of hemorrhagic shock. Pediatric Research 2011; 70(3):267-271.

Goldberg DJ, French B, McBride MG, Marino BS, Mirarchi N,  Hanna BD, Wernovsky G, Paridon SA, Rychik J.  Impact of oral sildenafil on exercise performance in children and young adults after Fontan operation: a randomized, double-blind, placebo controlled, crossover trial. Circulation 2011; 123:1185-1193.

Anderson JB, Kalkwarf HJ, Kehl JE, Eghtesady P, Marino BS.  Low weight-for-age Z-score and infection risk following Fontan procedure. Annals Thorac Surg 2011; 91(5):1460-1466.

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, 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 Mar 4.

Goldberg DJ, French B, McBride MG, Marino BS, Mirarchi N, Hanna BD, Wernovsky G, Paridon SA, Rychik J. Impact of Oral Sildenafil on Exercise Performance in Children and Young Adults After Fontan Operation: A Randomized, Double-Blind, Placebo Controlled, Crossover Trial. Circulation. 2011 Mar 7.

Marino BS, Drotar D, Cassedy A, Davis R, Tomlinson RS, Mellion K, Mussatto K, Mahony L, Newburger JW, Tong E, Cohen MI, Helfaer MA, Kazak AE, Wray J, Wernovsky G, Shea JA, Ittenbach R. External Validity of the Pediatric Cardiac Quality of Life Inventory. Quality of Life Research. 2011 Mar;20(2):205-14.

Manning PB, Rutter MJ, Lisec A, Gupta R, Marino BS. One slide fits all: the versatility of slide tracheoplasty with cardiopulmonary bypass support for airway reconstruction in children. J Thorac Cardiovasc Surg. 2011 Jan;141(1):155-61.

Marino BS, Tomlinson RS, Wernovsky G, Drotar D, Newburger JW, Mahony L, Mussatto K, Tong E, Cohen M, Andersen C, Shera D, Khoury P, Wray J, Gaynor WJ, Helfaer MA, Kazak AE, Shea JA. Evaluation of Health-related Quality of Life in Children and Adolescents with Congenital or Acquired Heart Disease: Validation of the Pediatric Cardiac Quality of Life Inventory. Pediatrics. 2010;126:1-11.

Kleinman ME, Chameides L, Schexnayder SM, Samson RA, Hazinski MF, Atkins DL, Berg MD, de Caen A, Fink E, Freid E, Hickey R, Marino BS, Nadkarni VM, Proctor L, Qureshi F, Sartorelli K, Topjian A, van der Jagt E, Zaritsky AL. Pediatric Advance Life Support. Circulation. 2010;122(18 Suppl 3):S876-S908. 

Marino BS, Tomlinson RS, Drotar D, Claybon ES, Aguirre A, Ittenbach R, Welkom JS, Helfaer MA, Wernovsky G, Shea JA. Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease. Pediatrics. 2009;123(4):e708-15.

Marino BS, Shera D, Wernovsky G, Tomlinson RS, Aguirre A, Gallagher M, Lee A, Cho C, Stern W, Davis L, Tong E, Teitel D, Mussatto K, Ghanayem N, Gleason, M, Gaynor JW, Wray J, Helfaer MA, Shea JA. The development of the Pediatric Cardiac Quality of Life Inventory: a quality of life measure for children and adolescents with heart disease. Quality of Life Research. 2008;17(4):613-626.

Marino BS, Kruge LE, Cho CJ, Tomlinson RS, Shera D, Weinberg PM, Gaynor JW, Rychik J. Parachute mitral valve: morphologic descriptors, associated lesions, and outcomes after biventricular repair. J Thorac Cardiovasc Surg. 2008;137:385-393.

Echocardiography; fetal cardiology; pediatric cardiology

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.

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. Journal of Thoracic and Cardiovascular Surgery. [in press]

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.

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.

Boat A, Mahmoud M, Michelfelder EC, Lin E, Ngamprasertwong P, Schnell B, Kurth CD, Crombleholme TM, Sadhasivam S. Supplementing desflurane with intravenous anesthesia reduces fetal cardiac dysfunction during open fetal surgery. Paediatr Anaesth. 2010 Aug;20(8):748-56.

Hinton RB, Michelfelder EC, Marino BS, Bove KE, Ware SM. A fetus with hypertrophic cardiomyopathy, restrictive, and single-ventricle physiology, and a beta-myosin heavy chain mutation. J Pediatr. 2010 Jul;157(1):164-6. Epub 2010 Apr 14.

Kinsel-Ziter ML, Cnota JF, Crombleholme TM, Michelfelder EC. Twin-reversed arterial perfusion sequence: pre- and postoperative cardiovascular findings in the 'pump' twin. Ultrasound Obstet Gynecol. 2009 Nov;34(5):550-5.

Michelfelder E, Polzin W, Hirsch R. Hypoplastic left heart syndrome with intact atrial septum: Utilization of a hybrid catheterization facility for cesarean section delivery and prompt neonatal intervention. Catheter Cardiovasc Interv. 2008 Dec 1;72(7):983-7.

Habli M, Michelfelder E, Livingston J, Harmon J, Lim FY, Polzin W, Crombleholme T. Acute effects of selective fetoscopic laser photocoagulation on recipient cardiac function in twin-twin transfusion syndrome. Am J Obstet Gynecol. 2008 Oct;199(4):412.e1-6.

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.

Visit the Molkentin Lab.

BS Marquette University, Milwaukee WI, 1989.

PhD: Medical College of Wisconsin, 1994.

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

Congenital heart disease including adult congenital heart disease and "hybrid" approaches to congenital heart disease; heart transplantation and mechanical circulatory support

MD: College of Physicians & Surgeons of Columbia University, New York, NY, 1994

Residency: Surgical Intern, Department of Surgery, The New York Hospital, New York, NY, July 1994 - June 1995.

Monreal G, Youtz DJ, Phillips AB, Eyman ME, Gorr MW, Velten C, Lucchesi PA, Wold LE, Gerhardt MA. Right ventricular remodeling in restrictive ventricular septal defect. J Mol Cell Cardiol. 2010 Oct;49: 699-706.

Olshove VF, Preston T, Gomez D, Phillips A, Galantowicz M. Perfusion techniques toward bloodless pediatric open heart surgery. J Extra Corpor Technol. 2010 Jun;42(2):122-7.

Holzer R, Marshall A, Kreutzer J, Hirsch R, Chisolm J, Hill S, Galantowicz M, Phillips A, Cheatham J, Bergerson L. Hybrid procedures: adverse events and procedural characteristics -- results of a multi-institutional registry. Congenit Heart Dis. 2010 May-Jun;5(3):233-42.

Stoica SC, Philips AB, Egan M, Rodeman R, Chisolm J, Hill S, Cheatham JP, Galantowicz ME. The retrograde aortic arch in the hybrid approach to hypoplastic left heart syndrome. Ann Thorac Surg. 2009 Dec;88(6):1939-46; discussion 1946-7.

Holzer RJ, Sisk M, Chisolm JL, Hill SL, Olshove V, Phillips A, Cheatham JP, Galantowicz M. Completion angiography after cardiac surgery for congenital heart disease: complementing the intraoperative imaging modalities. Pediatr Cardiol. 2009 Nov;30(8):1075-82.

Phillips AB, Green J, Bergdall V, Yu J, Monreal G, Gerhardt M, Cheatham JP, Galantowicz M, Holzer RJ. Teaching the "hybrid approach": a novel swine model of muscular ventricular septal defect. Pediatr Cardiol. 2009 Feb;30(2):114-8.

Holzer RJ, Green J, Bergdall V, Chisolm JL, Hill SL, Galantowicz M, Cheatham JP, Phillips A. An animal model for hybrid stage I palliation of hypoplastic left heart syndrome. Pediatr Cardiol. 2009 Oct;30(7):922-7.

Monreal G, Nicholson LM, Han B, Joshi MS, Phillips AB, Wold LE, Bauer JA, Gerhardt MA. Cytoskeletal remodeling of desmin is a more accurate measure of cardiac dysfunction than fibrosis or myocyte hypertrophy. Life Sci. 2008 Dec 5;83(23-24):786-94.

Galantowicz M, Cheatham JP, Phillips A, Cua CL, Hoffman TM, Hill SL, Rodeman R. Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg. 2008 Jun;85(6):2063-70.

Phillips AB, Ko W. Effects of ischemic preconditioning and adenosine pretreatment on myocardial function and energetics in a clinically relevant model. Life Sci. 2007 Oct 13;81(17-18):1355-61.

Cardiac disease; genetic abnormalities; cardiac mechanosensing; cardiomyopathy

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.

Pediatric obesity prevention and treatment

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, Scubiner J, Samarmco VJ. A non-operative technique for peroneal tendon subluxation. Clinical Pediatrics. 2008;47(3):300-1.

Olson L, Radecki L, Frinter 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.

Cardiac anesthesia; neurologic injury in children with congenital heart disease; improving perioperative safety

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.

Clinical Interests

Cardiac MRI; Cardiac CT; and echocardiography

Research Interests

Myocardial metabolism; animal models of heart failure; positron emission tomography 

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.
 

Pediatric high blood pressure and cholesterol, diet and exercise to prevent adult heart disease

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.

Clinical genetics; cardiovascular genetics; cardiomyopathy; cardiovascular development

MD, PhD: University of Cincinnati College of Medicine, Cincinnati, OH,1997.

Residency: Pediatrics, Baylor College of Medicine, 2002.

Fellowship: Medical Genetics, Baylor College of Medicine, 2002.

American Board of Pediatrics, 2000, 2007.

American Board of Medical Genetics in Clinical Genetics, 2002.

Tariq M, Belmont JW, Lalani S, Smolarek T, Ware SM. SHROOM3 is a novel candidate for heterotaxy identified by whole exome sequencing. Genome Biol. 2012. Epub ahead of print.

Bedard JEJ, Haaning AM, Ware SM. Identification of a novel ZIC3 isoform and mutation screening in patients with heterotaxy and congenital heart disease patients. PLoS One. 2011;6(8):e23755.

Czosek RJ, Haaning A, Ware SM. A mouse model of conduction system patterning abnormalities in heterotaxy syndrome. Pediatr Res. 2010;68:275-280.

Sutherland M, Ware SM. Disorders of left-right asymmetry: heterotaxy and situs inversus. Am J Med Genet C Semin Med Genet. 2009 Nov 15;151C(4):307-17.

Ware SM*, El-Hassan N, Kahler SG, Zhang Q, Ma Y-M, Miller E, Wong B, Spicer RL, Craigen WJ, Kozel BA, Grange DK, Wong L-J. Infantile cardiomyopathy caused by a mutation in the overlapping region of mitochondrial ATPase 6 and 8 genes. J Med Genet. 2009;46: 308-314.  *corresponding author

Kogan JM, Miller E, Ware SM. High resolution SNP based microarray mapping of mosaic supernumerary marker chromosomes 13 and 17: delineating novel loci for apraxia. Am J Med Genet. 2009;149A: 887-893.

Mohapatra B, Casey B, Li H, Ho-Dawson T, Smith L, Fernbach SD, Molinari L, Niesh SR, Jefferies JL, Craigen WJ, Towbin JA, Belmont JW, Ware SM. Identification and functional characterization of NODAL rare variants in heterotaxy and isolated cardiovascular malformations. Hum Mol Genet. 2009;18: 861-871.

Ware SM*, Quinn M, Ballard ET, Miller E, Uzark K, Spicer RL. Pediatric restrictive cardiomyopathy associated with a mutation in beta-myosin heavy chain. Clin Genet. 2008;73: 165-170.         *corresponding author 

Bedard JEJ, Purnell JD, Ware SM. Nuclear import and export signals are essential for proper cellular trafficking and function of ZIC3. Hum Mol Genet. 2007;16: 187-198.

Ware SM, Harutyunyan KG, Belmont JW. Heart defects in X linked heterotaxy: evidence for a genetic interaction of Zic3 with the Nodal signaling pathway. Dev Dyn. 2006 Jun;235:1631-1637.

Understanding the molecular underlying nature of congenital heart defects; cardiomyocyte formation

Visit the Waxman Lab.

BA: New College, Sarasota, FL,1999.

PhD: University of Washington, Seattle, WA, 2004.

Postdoctoral Fellow: Skirball Institute/NYU School of Medicine, New York, NY, 2004-2009.

Dohn TE, Waxman JS. Distinct phases of Wnt/β-catenin signaling direct cardiomyocyte formation in zebrafish. Dev Biol. Epub ahead of print. Nov 4, 2011.

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. 2011;352:128-40.

Feng L, Hernandez RE, Waxman JS, Yelon D, Moens CB. Dhrs3a regulates retinoic acid biosynthesis through a feedback inhibition mechanism. Dev Biol. 2010 Feb 1;338(1):1-14.

Waxman JS, Yelon D.  Increased Hox activity mimics the teratogenic effects of excess retinoic acid signaling. Developmental Dynamics. 2009;238:1207-13.

Linville A, Radkte K, Waxman JS, Yelon D, Schilling T.  Combinatorial roles for zebrafish retinoic acid receptors in the hindbrain, limbs and pharyngeal arches. Developmental Biology. 2009;325:60-70.

Waxman JS, Keegan BR, Roberts RW, Poss KD, Yelon D.  Hoxb5b acts downstream of retinoic acid signaling in the forelimb field to restrict cardiac cell number in zebrafish. Developmental Cell. 2008;15:923-34. 

Waxman JS, Yelon D.  Comparison of the expression patterns of newly identified zebrafish retinoic acid and retinoid X receptors. Developmental Dynamics. 2007;236:587-95.

Molecular regulation of heart development; valve development and disease mechanisms; cardiomyocyte proliferation, cardiac connective tissue lineages.

Visit the Yutzey Lab.

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. V.V. Kalinichenko, K.E. Yutzey. 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, C.A., C.M. Alfieri, E.M. Joyce, S.C. Watkins, K.E. Yutzey, M.S. Sacks 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, and 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.