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. 

Pediatric cardiology; pediatric heart transplantation

MD: University of South Florida College of Medicine, Tampa, Florida, 1996.

MPH: University of South Florida College of Public Health, Tampa, Florida, 1999.

Residency: All Children’s Hospital, University of South Florida, St. Petersburg, FL.

Fellowships: Cardiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH; Critical Care, Children’s Medical Center Dallas, UT Southwestern, Dallas, Texas.

Certifications: American Board of Pediatrics; American Board of Pediatrics, Sub-board of Pediatric Cardiology; American Board of Pediatrics, Sub-board of Pediatric Critical Care.

Cooper DS, Charpie JR, Flores FX, Gaynor JW, Salvin JW, Devarajan P, Krawczeski CD. Acute kidney injury and critical cardiac disease. World J Ped Congen Heart Surg. 2011;2:411-423.

Jacobs JP, Asante-Korang A, O'Brien SM, Chai PJ, Dadlani GH, Rodriguez-Fazzi GL, Vu D, McCormack J, McKenna DE, Boucek RJ Jr, Cooper DS, Saltiel A, Carapellucci J, van Gelder HM, Daicoff GR, Quintessenza JA. Lessons learned from 119 consecutive cardiac transplants for pediatric and congenital heart disease. Ann Thorac Surg. 2011 Apr;91(4):1248-54.

Pilchard J, Dadlani G, Andropoulos D, Jacobs JP and Cooper DS. Intensive care and perioperative management of patients with complete atrioventricular septal defect . World J Ped Congen Heart Surg. 2010 Apr;1:105-111.

Scanlon MC, Ghanayem NS, Atz AM, Cooper DS. Innovation in congenital and paediatric cardiac critical care. Cardiol Young. 2009 Nov;19 Suppl 2:85-9.

Cooper DS, Costello JM, Bronicki RA, Stock AC, Jacobs JP, Ravishankar C, Dominguez TE, Ghanayem NS. Current challenges in cardiac intensive care: optimal strategies for mechanical ventilation and timing of extubation. Cardiol Young. 2008 Dec;18 Suppl 3:72-83.

Cooper DS, Jacobs JP, Chai PJ, Jaggers J, Barach P, Beekman RH, Krogmann O, Manning P. Pulmonary complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease. Cardiol Young. 2008 Dec;18 Suppl 2:215-21.

Bacha EA, Cooper D, Thiagarajan R, Franklin RC, Krogmann O, Deal B, Mavroudis C, Shukla A, Yeh T, Barach P, Wessel D, Stellin G, Colan SD. Cardiac complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease. Cardiol Young. 2008 Dec;18 Suppl 2:196-201.

Cooper DS, Jacobs JP, Moore L, Stock A, Gaynor JW, Chancy T, Parpard M, Griffin DA, Owens T, Checchia PA, Thiagarajan RR, Spray TL, Ravishankar C. Cardiac extracorporeal life support: state of the art in 2007. Cardiol Young. 2007 Sep;17 Suppl 2:104-15.

Cooper, D.S. and Nichter M.A. Advances in cardiac intensive care. Curr Opin Ped. 2006 Oct;18(5):503-11.

Cooper DS, Schwartz SM, Raake JL, Beam AR, Nelson DP. Supplemental inhaled gases alter tidal volume delivery and measurement. Pediatr Crit Care Med. 2005 Mar;6(2):150-3.

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

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.

Correction of complex cardiac defects in the newborn period, tracheal reconstruction, and blood conservation in open-heart surgery.

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.

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.

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.

Giuliano JS Jr, Lahni PM, Bigham MT, Manning PB, Nelson DP, Wong HR, Wheeler DS. Plasma angiopoietin-2 levels increase in children following cardiopulmonary bypass.Intensive Care Med. 2008 Oct;34(10):1851-7.

Nguyen MT, Dent CL, Ross GF, Harris N, Manning PB, Mitsnefes MM, Devarajan P. Urinary aprotinin as a predictor of acute kidney injury after cardiac surgery in children receiving aprotinin therapy.Pediatr Nephrol. 2008 Aug;23(8):1317-26.

Bigham MT, Brady PW, Manning PB, Jacobs BR, Kimball TR, Wong HR. Therapeutic application of intrapericardial tissue plasminogen activator in a 4-month-old child with complex fibropurulent pericarditis.Pediatr Crit Care Med. 2008 Jan;9(1):e1-4.

Ashcraft TM, Jones K, Border WL, Eghtesady P, Pearl JM, Khoury PR, Manning PB. Factors affecting long-term risk of aortic arch recoarctation after the Norwood procedure. Ann Thorac Surg. 2008 Apr;85(4):1397-401; discussion 1401-2.

Manning PB, Rutter MJ, Border WL. Slide tracheoplasty in infants and children: risk factors for prolonged postoperative ventilatory support.Ann Thorac Surg. 2008 Apr;85(4):1187-91; discussion 1191-2.

Wheeler DS, Dent CL, Manning PB, Nelson DP. Factors prolonging length of stay in the cardiac intensive care unit following the arterial switch operation.Cardiol Young. 2008 Feb;18(1):41-50.

Giuliano JS Jr, Sekar P, Dent CL, Border WL, Hirsch R, Manning PB, Wheeler DS. Unilateral pulmonary edema and acute rheumatic fever. Eur J Pediatr. 2008 Apr;167(4):465-7.

Electronic medical record applications for clinical research

Clinical Interests

Neurodevelopmental outcomes; family-centered care; cardiopulmonary interactions; physiology of non-conventional circulations; endocrine abnormalities in cardiac intensive care; management of acute decompensated heart failure; cardio-renal syndrome; end of life care

Research Interests

Quality of life and neurodevelopmental outcomes in children with acquired and congenital heart disease; etiology of adrenal dysfunction in cardiac intensive care inflammatory organ system injury after congenital heart surgery

Moffett BS, McDade E, Rossano JW, Dickerson HA, Nelson DP. Enteral potassium supplementation in a pediatric cardiac intensive care unit: Evaluation of a practice change.Pediatr Crit Care Med. 2011 Feb 3.

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, ix-x. Review.

Carlo WF, Nelson DP. Inhaled nitric oxide improves oxygen saturation in children with pulmonary arteriovenous malformations after the Fontan procedure.Pediatr Crit Care Med. 2010 Aug 5.

Giuliano JS Jr, Lahni PM, Bigham MT, Manning PB, Nelson DP, Wong HR, Wheeler DS. Plasma angiopoietin-2 levels increase in children following cardiopulmonary bypass.Intensive Care Med. 2008 Oct;34(10):1851-7.

Moffet, BS, AR Mott, DP Nelson, KD Gurwitch. Medication dosing and renal insufficiency in a pediatric cardiac intensive care unit: impact of pharmacist consultation. Pediatr Cardiol. 2008 29(4):744-8.  

Wheeler DS, Dent CL, Manning PB, Nelson DP. Factors prolonging length of stay in the cardiac intensive care unit following the arterial switch operation.Cardiol Young. 2008 Feb;18(1):41-50.

Price JF, AR Mott, DP Nelson, AC Chang, E O'Brian Smith, JA Towbin, WJ Dreyer, SW Denfield, SL Goldstein. Worsening renal function in children hospitalized with decompensated heart failure: evidence for a pediatric cardiorenal syndrome. Pediatr Crit Care Med. 2008 9(3):279-84. 

Moffett, BS, AR Mott, D.P. Nelson, SL Goldstein, JL Jefferies. Renal effects of fenoldopam in critically ill pediatric patients: A retrospective review. Pediatr Crit Care Med. 2008 9(4):403-406. 

Rossano JW, MD Taylor, EO Smith, CD Fraser, Jr., 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):739-45. 

Wheeler, DS, CL Dent, PB Manning, DP Nelson. Factors prolonging length of stay in the cardiac intensive care unit following the arterial switch operation. Cardiol Young. 2008 18(1):41-50. 

Wheeler, DS, JM Pearl, PB Manning, SM Schwartz, CL Dent, P Khoury, DP Nelson. Factors Prolonging Cardiac Intensive Care Unit Stay after neonatal congenital heart surgery. Pediatr Crit Care Med. 2008 18(1):41-50. 

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.

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.

 Transplant; quality of life; and outcomes research

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.

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

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 mycophonolate 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.

Critical cardiac care

MD: University of Cincinnati, Cincinnati, Ohio, 1958.

Residency: Pediatrics, Children's Hospital Medical Center, Cincinnati, Ohio.

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

Certification: Pediatrics, 1963; Pediatric Cardiology, 1964.

Clinical Interests

Pediatric Pulmonary Hypertension

MSN: University of Cincinnati, OH, 2006

BSN: University of Cincinnati, OH, 2003

Certifications: American Nurses Creditialing Center, Certified Pediatric Nurse Practitioner, 2006; Ohio Board of Nursing, 2003

Bagby M, Zussman M, Hirsch R, Gupta, R, Benson, D. Pulmonary Vascular Resistance in Repaired Congenital Diaphragmatic Hernia vs. Age Matched Controls. Pediatr Res. 2012; doi:10.1038/pr.2012.16

Bagby M, Zussman M, Benson W. Gupta R. Hirsch R. Fenestrated Atrial Septal Defect Closure in a Child with Idiopathic Pulmonary Hypertension and Exercise Desaturation. Congenit Heart Dis. 2011;6:162-166.

Abstracts

Bagby M, Zussman M, Hirsch R. Bosentan Use is Safe in Neonates and Infants with Congenital Diaphragmatic Hernia and Severe Pulmonary Hypertension. Accepted for Presentation, The Pulmonary Hypertension Association’s 9th International PH Conference and Scientific Sessions, June 2010.

Bagby M, Hirsch R. De-Novo Initiation of Sub-Cutaneous Treprostinil in a Young Pediatric Patient. Accepted for Presentation, The Pulmonary Hypertension Association’s 9th International PH Conference and Scientific Sessions, June 2010.

Zussman M, Bagby M, Hirsch R. Pulmonary Vascular Resistance is Higher in Patients with Repaired Congenital Diaphragmatic Hernia Compared to Age Matched Controls. Accepted for Presentation, The Pulmonary Hypertension Association’s 9th International PH Conference and Scientific Sessions, June 2010.

Sawyer J, Bagby M, Hirsch R. Transitioning Pediatric Patients from Intravenous Epoprostenol to Subcutaneous Treprostinil: Model of a Pediatric Pulmonary Hypertension Service. Accepted for Poster Presentation, Children’s Hospital of Philadelphia Critical Care Conference, February 2009.

Bagby M, Giver J, Hirsch R. Methods of Increasing Comprehensive and Efficient Patient Care: The Enhancement of a Pediatric Pulmonary Hypertension Program. Accepted for Presentation, The Pulmonary Hypertension Association’s 8th International PH Conference and Scientific Sessions, June 2008.

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.

BS: University of Georgia, 1997

MD: Medical College of of Georgia, 2001

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

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

Fellowship Subspecialty Training: Pediatric Pacing and Electrophysiology, Boston Children's Hospital, Boston, MA.

Walsh MA, Grenier MA, Jefferies JL, Towbin JA, Lorts A, Czosek RJ. Conduction Abnormalities in Pediatric Patients with Restrictive Cardiomyopathy. Circ Heart Fail. 2012 Jan 19. [Epub ahead of print]

Walsh MA, Anderson JB, Knilans TK, Cottrill CM, Czosek RJ.Ventricular Oversensing in a Biventricular Pacemaker. Pacing Clin Electrophysiol. 2012 Jan 13 [Epub ahead of print]

Czosek RJ, Meganathan K, Anderson JB, Knilans TK, Marino BS, Heaton PC. Cardiac rhythm devices in the pediatric population: Utilization and complications. Heart Rhythm. 2012 Feb;9(2):199-208.

Czosek RJ, Anderson JB, Marino BS, Mellion K, Knilans TK. Noninvasive risk stratification techniques in pediatric patients with ventricular preexcitation. Pacing Clin Electrophysiol. 2011;34:555-562.

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

Czosek RJ, Atallah J, Emani S, Hasan B, del Nido P, Berul CI. Electrical dyssynchrony and endocardial fibroelastosis resection in the rehabilitation of hypoplastic left cardiac syndrome. Cardiol Young. 2010;20:516-52.

Czosek RJ, Anderson JB, Cao J, Knilans TK. Assessment of t-wave oversensing in an infant with an implanted defibrillator. Heart Rhythm. 2010;7:1516-1517

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;33:1304-131.

Czosek RJ, Anderson J, Connor C, Knilans T. Nodoventricular pathway associated with twin AV nodes: Complexity of ablation in single ventricle physiology. J Cardiovasc Electrophysiol. 2010;21:936-939.

Czosek RJ, Berul CI. Congenital long-QT syndrome concealed by hypercalcemia in Williams syndrome. J Cardiovasc Electrophysiol. 2008;19:1322-1324.

Clinical Interests

Cheri Franklin, RN, MSN, CNP, has special interest in fetal cardiology / fetal cardiac intervention and congenital heart defects / surgical intervention.

Research Interests

Fetal cardiac intervention
 

Cardiac catheterization; intervention for congenital heart disease; transcatheter valve implantation; single ventricle physiology

MD: Boston University School of Medicine, Boston, MA, 2004.

Residency: Pediatrics, Children’s Hospital Boston, Boston, MA, 2007. 

Fellowship: Pediatric Cardiology, C.S. Mott Children’s Hospital, Ann Arbor, MI, 2010.

Advanced Fellowship: Interventional Cardiology, C.S. Mott Children’s Hospital, Ann Arbor, MI, 2011.

Certifications: Pediatrics, 2007; Pediatric Cardiology, 2010. 

Goldstein BH, Golbus JR, Sandelin AM, Warnke N, Gooding L, King KK, Donohue JE, Yu S, Gurney JG, Goldberg CS, Rocchini AP, Charpie JR.  Impact of Vitamin C on Endothelial Function and Exercise Capacity in Patients with a Fontan Circulation.  Congenit Heart Dis. 2011.

Goldstein BH, Aiyagari R, Bocks ML, Armstrong AK.  Hydrogel Expandable Coils for Vascular Occlusion in Congenital Cardiovascular Disease: A Single Center Experience.  Congenit Heart Dis. 2011.

Goldstein BH, Fifer CG, Armstrong AK, Gelehrter SK, Treadwell MC, van de Ven C, Rocchini AP. Use of a Pressure Guidewire in Fetal Cardiac Intervention for Critical Aortic Stenosis.Pediatrics. 2011 Aug 15.

Schumacher KR, Cools M, Goldstein BH, Ioffe-Dahan V, King K, Gaffney D, Russell MW. Oral Budesonide Treatment for Protein Losing Enteropathy in Fontan-Palliated Patients.Pediatr Cardiol. 2011 Oct;32(7):966-71.

Goldstein BH, Golbus JR, Sandelin AM, Warnke N, Gooding L, King KK, Donohue JE, Gurney JG, Goldberg CS, Rocchini AP, Charpie JR. Usefulness of Peripheral Vascular Function to Predict Functional Health Status in Patients with a Fontan Circulation.Am J Cardiol. 2011 Aug 1;108(3):428-34.

Goldstein BH, Bergersen L, Powell AJ, Graham D, Bacha E, Lang P. Long-Term Outcome of Surgically Repaired Unilateral Anomalous Pulmonary Artery Origin.Pediatr Cardiol. 2010 Oct;31(7):944-51. 

Goldstein BH, Connor C, Gooding L, Rocchini AP. Relation of Systemic Venous Return, Diastolic Dysfunction and Pulmonary Vascular Resistance to Exercise Capacity in Patients with Single Ventricle Receiving Fontan Palliation.Am J Cardiol. 2010 Apr 15;105(8):1169-75.

Clinical Interests

Heart failure and congenital heart disease imaging and treatment

Research Interests

Imaging assessment of heart failure in children; myocardial deformation; clinician/parent interactions in heart failure; clinician practices in heart failure; myocardial mechanics

Grenier MA, Osganian SK, Cox GF, Towbin JA, Sleeper LA, Lurie PR, Orav EJ, Lipshultz SE. Design and implementation of the North American pediatric Cardiomyopathy Registry. Am Heart J. Feb 2000;139(2 Pt 3):S86-95.

Knowlton AA, Grenier M, Kirchoff SR, Salfity M.  Phosphorylation at tyrosine 524 influences nuclear HSP72 with heat stress. Am J Physiol Heart Circ Physio. 2000; 278(6):H2143-9.

Podoll AS, Grenier MA, Feig DI.  Inaccuracy in standard blood pressure measurement in a pediatric clinic. Pediat.2007;119(3):e538-543.

Rossano JW, Grenier M, Dreyer WJ, Kim JJ, Price JF, Jeffries JL, Smith EO, Clunie SK, Moulik M, Decker JA, Breinholt JP, Morales DL, McKenzie ED, Towbin JA, Denfield SW. Effect of body mass index on outcome in pediatric heart transplant patients. J Heart Lung Transplant. Jul 2007;26(7):718-723.

Walsha MA, Greiner MA, Jefferies JL, Towbin JA, Lorts A, Czosek RJ. Conduction abnormalities in pediatric patients wit restrictive cariomyopathy. Circ Heart Fail. Jan 2012.

Clinical Interests

Echocardiography

Research Interests

Dyssynchrony; quality improvement

Clinical Interests

Cardiovascular genetics; echocardiography

Research Interests

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

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).

Hinton RB. Genetic contribution to bicuspid aortic valve morphology. Am J Med Genet. 2011;155(11):2899-2900.

Acharya A, Hans CP, Koenig SN, Nichols HA, Galindo CL, Garner HR, Merrill WH, Hinton RB, Garg V. Inhibitory role of Notch1 in calcific aortic valve disease. PLoS One. 2011;6(11):e27743.

Snider P, Hinton RB, Moreno-Rodriguez RA, Wang J, Rogers R, Lindsley A, Li F, Ingram D, Menick D, Field LJ, Firulli AB, Molkentin JD, Markwald RR, Conway SJ.  Periostin is required for maturation and extracellular matrix stabilization of noncardiomyocyte lineages of the heart.  Circ Res. Apr 2008;102(7):752-760.

Hinton RB, Alfieri C, Witt S, Glascock B, Khoury PR, Benson DW, Yutzey KE.  Mouse heart valve structure and function: echocardiographic and morphometric analyses from the fetus through the aged adult.  Am J Physio Heart Circ Physiol. 2008;294(6):H2480-2488.

Hinton RB, 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.  Pediatr Res.  Oct 2008;64(4):364-369.

Interventional cardiac catheterization; congenital cardiac imaging (CT and MR) with three dimensional reconstruction / myocardial injury; three dimensional imaging; device closure of intra-cardiac defects; pulmonary hypertension; myocardial injury

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.

Mack EH, Wheeler DS, Hirsch R. Endovascular treatment of near-fatal neonatal superior vena cava syndrome. Pediatr Crit Care Med. 2010 Nov 24.

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.

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.

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.

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.

Gudausky TM, Hirsch R, Khoury PR, Beekman RH 3rd. Comparison of two transcatheter device strategies for occlusion of the patent ductus arteriosus.Catheter Cardiovasc Interv. 2008 Nov 1;72(5):675-80.

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.

Hirsch R, Gottliebson W, Crotty E, Fleck R, Strife J. Computed tomography angiography with three-dimensional reconstruction for pulmony venous definition in high-risk infants with congenital heart disease.Congenit Heart Dis. 2006 May;1(3):104-10.

Hirsch R. The hybrid cardiac catheterization laboratory for congenital heart disease: From conception to completion.Catheter Cardiovasc Interv. 2008 Feb 15;71(3):418-28.

Clinical Interests

Cardiac magnetic resonance imaging; echocardiography (TTE, TEE and fetal); congenital heart disease

Research Interests

Cardiac MRI and echocardiography (application in clinical and basic science research)

Kim HK, Gottliebson W, Hor K, Backeljauw P, Gutmark-Little I, Salisbury SR, Racadio JM, Helton-Skally K, Fleck R. Cardiovascular anomalies in turner syndrome: spectrum, prevalence, and cardiac MRI findings in a pediatric and young adult population.AJR Am J Roentgenol. 2011 Feb;196(2):454-60.

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.

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.

Hagenbuch SC, Gottliebson WM, Wansapura J, Mazur W, Fleck R, Benson DW, Hor KN. Detection of progressive cardiac dysfunction by serial evaluation of circumferential strain in patients with Duchenne muscular dystrophy.Am J Cardiol. 2010 May 15;105(10):1451-5.

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.

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.

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.

Hor KN, Wansapura J, Markham LW, Mazur W, Cripe LH, Fleck R, Benson DW, Gottliebson WM. Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study.J Am Coll Cardiol. 2009 Apr 7;53(14):1204-10.

Hor KN, Border WL, Cripe LH, Benson DW, Hinton RB. The presence of bicuspid aortic valve does not predict ventricular septal defect type.Am J Med Genet A. 2008 Dec 15;146A(24):3202-5.

Puchalski MD, Williams RV, Askovich B, Sower CT, Hor KH, Su JT, Pack N, Dibella E, Gottliebson WM. Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?Int J Cardiovasc Imaging. 2009 Jan;25(1):57-63.

Clinical Interests

Echocardiography; congenital heart disease; preventive cardiology; pediatric obesity

Research Interests

Non-Invasive cardiac imaging modalities and techniques; cardiovascular effects of pediatric obesity; cardiovascular effects of exercise

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.

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.

Ippisch HM, Inge TH, Daniels SR, Wang B, Khoury PR, Witt SA, Glascock BJ, Garcia VF, Kimball TR. Reversibility of cardiac abnormalities in morbidly obese adolescents.J Am Coll Cardiol. 2008 Apr 8;51(14):1342-8.

Ippisch HM, Kimball TR. The impact of evolving hand-carried echocardiographic technology on outpatient physical examination accuracy in pediatric cardiology.Congenit Heart Dis. 2007 May;2(3):170-8.

Hao M, Ippisch HM, Cook RS, Perry DJ, Gottliebson WM, Hirsch R, Kimball TR. Implementation of an objective testing system in noninvasive cardiac imaging for evaluation of pediatric cardiology fellows.J Am Soc Echocardiogr. 2007 Oct;20(10):1211-8.

Ippisch HM, Border WL, Witt SA, Glascock BJ, Khoury PR, Kimball TR. Diastolic function in obese children assessed by tissue Doppler imaging.52nd Annual Scientific Sessions of the American College of Cardiology. Chicago, IL. March 31, 2003.

Ippisch HM, Kimball TR. Hand-carried cardiac ultrasound compared to conventional ultrasound for the evaluation of congenital heart disease.14th Annual Scientific Sessions of the American Society of Echocardiography. Las Vegas, NV. June 13, 2003.

Ippisch HM, Witt SA, Border WL, Glascock BJ, Khoury PR, Daniels SR, Kimball TR. Does a Weight Loss Reduction Program Improve Cardiac Dysfunction in Obese Children?American Heart Association's Scientific Sessions. Orlando, Florida. November 12th, 2003.

Ippisch HM, Witt SA, Glascock BJ, Mays W, Knecht S, Forment Y, Claytor R, Knilans TK, Daniels SR, Kimball TR. Do Cardiac Function and Exercise Performance Change In Obese Children Following Participation In A Weight Loss Program?15th Annual Scientific Sessions of the American Society of Echocardiography.  San Diego, CA. June 28th, 2004.

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

Visit the James Lab.

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. 

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.

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

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

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

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

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.

Outpatient clinical management of cardiac disease; echocardiography

BA: Molecular, Cellular and Developmental Biology, University of Colorado, Boulder, CO, 1989.

MD: University of Cincinnati College of Medicine, Cincinnati, Ohio, 1993.

Residency: Cincinnati Children's Hospital, Cincinnati, Ohio, 1993-1996.

Fellowships: Pediatric Resident, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 1994-1996. Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan 1997-2000; Pediatric Echocardiography, Cincinnati Children's Hospital, Cincinnati, Ohio, 2000-2001.

Certification: Pediatrics, 1996; Pediatric Cardiology, 2000.

Lee LA. Innocent murmurs. Presented to the family practice residents and St. Elizabeth Medical Center, Edgewood, KY. January 10, 2003

Lee LA. Stress echocardiography in children. Pediatric Ultrasound Today.  2001 Volume 6, Lesson 4.

Lee LA, Goya G, Witt SA, Flascock BJ, Mays W, Kimball TR. Resting predictors of latent stress-induced left ventricular outflow tract obstruction in young patients with hypertrophic cardiomyopathy. J Am Coll Cardiology. 2001 Oct.

Lee LA. Evaluation and management of hypertension in children. Presented to nurse practitioner students, University of Michigan School of Nursing, Ann Arbor, MI. March 2000. 

Lee LA, Simon C, Bove EL, Mosca RS, Ebbini ES, Abrams GD, Ludomirsky A. High-intensity focused ultrasound effect on cardiac tissues: potential for clinical application. Echocardiography. 2000 Aug;
17(6 Pt 1):563-6.

Ohye RG, Kagisaki K, Lee LA, Mosca RS, Goldberg CS, Bove EL. Biventricular repair for aortic atresia or

hypoplasia and ventricular septal defect. J Thorac Cardiovasc Surg. 1999;118(4):648-53.

Lee LA, Kimball TR, Daniels SR, Khoury P, Meyer RA. Left ventricular mechanics in the preterm infant and their effect on the measurement of cardiac performance. J Pediatr. 1992 Jan;120(1):114-9..

Clinical Interests

Heart failure; cardiopulmonary bypass mediated myocardial dysfunction

Research Interests

Myocardial remodeling

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.

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.

Clinical Interests

Exercise evaluation of pediatric patients with congenital and acquired cardiopulmonary disease; exercise therapy / rehabilitation in pediatric patients with congenital and acquired cardiopulmonary disease and morbidly obese pediatric patients; electrocardiographic and electrophysiologic evaluation of pediatric patients with cardiac arrhythmias

Research Interests

Exercise evaluation of pediatric patients with single ventricle physiology; exercise evaluation of pediatric patient with syncope; exercise evaluation of pediatric patients with congenital or acquire prolonged QT interval; use of exercise therapy / rehabilitation in the treatment of pediatric patients with complex congenital heart disease

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.

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.

Kimball TR, Reynolds JM, Mays WA, Khoury P, Claytor RP, Daniels SR. Persistent hyperdynamic cardiovascular state at rest and during exercise in children after successful repair of coarctation of the aorta.J Am Coll Cardiol. 1994 Jul;24(1):194-200.

Kimball TR, Mays WA, Khoury PR, Mallie R, Claytor RP. Echocardiographic determination of left ventricular preload, afterload, and contractility during and after exercise. J Pediatr. 1993 Jun;122(6):S89-94.

Atzinger CL, Meyer RA, Khoury PR, Gao Z, Tinkle BT. Cross-Sectional and Longitudinal Assessment of Aortic Root Dilation and Valvular Anomalies in Hypermobile and Classic Ehlers-Danlos Syndrome.J Pediatr. 2010 Dec 28.

Kwiatkowski D, Hagenbuch S, Meyer R. A teenager with Marfan syndrome and left ventricular noncompaction.Pediatr Cardiol. 2010 Jan;31(1):132-5.

Mak GZ, Hardy AR, Meyer RA, Kagan RJ. Reversible cardiomyopathy after severe burn injury. J Burn Care Res. 2006 Jul-Aug;27(4):482-6.

Gottliebson WM, Border WL, Franklin CM, Meyer RA, Michelfelder EC. Accuracy of fetal echocardiography: a cardiac segment-specific analysis.Ultrasound Obstet Gynecol. 2006 Jul;28(1):15-21.

Meyer RA. History of ultrasound in cardiology. J Ultrasound Med. 2004 Jan;23(1):1-11.

Wenstrup RJ, Meyer RA, Lyle JS, Hoechstetter L, Rose PS, Levy HP, Francomano CA. Prevalence of aortic root dilation in the Ehlers-Danlos syndrome. Genet Med. 2002 May-Jun;4(3):112-7.

Schratz LM, Meyer RA, Schwartz DC. Serial intracoronary ultrasound in children: feasibility, reproducibility, limitations, and safety.J Am Soc Echocardiogr. 2002 Aug;15(8):782-90.

Mitsnefes MM, Daniels SR, Schwartz SM, Meyer RA, Khoury P, Strife CF. Severe left ventricular hypertrophy in pediatric dialysis: prevalence and predictors.Pediatr Nephrol. 2000 Sep;14(10-11):898-902.

Stanek J, Bove KE, Bofinger M, Needham D, Saldana LR, Mutema GK, Meyer R. Premature closure of foramen ovale and renal vein thrombosis in a stillborn twin homozygous for methylene tetrahydrofolate reductase gene polymorphism: a clinicopathologic case study.J Perinat Med. 2000;28(1):61-8.

Siddiqi TA, Miodovnik M, Meyer RA, O'Brien WD Jr. In vivo ultrasonographic exposimetry: human tissue-specific attenuation coefficients in the gynecologic examination.Am J Obstet Gynecol. 1999 Apr;180(4):866-74.

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.

Diploma in Nursing: Christ Hospital School Of Nursing, Cincninnati, Ohio, 1984.

BSN: University of Cincinnati, Cincinnati, Ohio 1992.

MSN: University of Cincinnati, Cincinnati, Ohio 1995.

Certification: Pediatric Nurse Practitioner 1995

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, 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.

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.

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.

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.

Cardiac MRI; transthoracic and transesophageal echocardiography; myocardial mechanics

MD:  University of Washington School of Medicine, Seattle, WA,  2003.

Residency:  Pediatrics, University of California San Diego, San Diego, CA,  2006.

Fellowship:  Pediatric Cardiology, University of Colorado, Denver, CO,  2010.

Advanced Fellowship:  Non-invasive Cardiac Imaging, University of Colorado, Denver, CO,  2011.

Certification:  Pediatrics, 2007.

Liebig M, Schymik I, Mueller M, Wendel U, Mayatepek E, Ruiter J, Strauss AW, Wanders RJ, Spiekerkoetter U. (2006) Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels. Pediatrics 118:1065-9.

Khuchua Z, Yue Z, Batts L, Strauss AW. (2006) A zebrafish model of human Barth syndrome reveals the essential role of tafazzin in cardiac development and function. Circ Res 99:201-8.

Exil VJ, Gardner CD, Rottman JN, Sims H, Bartelds B, Khuchua Z, Sindhal R, Ni G, Strauss AW. (2006) Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase. Am J Physiol Heart Circ Physiol 290:H1289-97.

Bennett MJ, Russell LK, Tokunaga C, Narayan SB, Tan L, Seegmiller A, Boriack RL, Strauss AW. (2006) Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase. Mol Genet Metab 89:74-9.

Strauss AW. (2005) Surprising? Perhaps not. Long-chain fatty acid oxidation during human fetal development. Pediatr Res 57:753-4.

Spiekerkoetter U, Tokunaga C, Wendel U, Mayatepek E, Ijlst L, Vaz FM, van Vlies N, Overmars H, Duran M, Wijburg FA, Wanders RJ, Strauss AW. (2005) Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. Pediatr Res 57:760-4.

Shekhawat PS, Matern D, Strauss AW. (2005) Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and management. Pediatr Res 57:78R-86R.

Djouadi F, Aubey F, Schlemmer D, Ruiter JP, Wanders RJ, Strauss AW, Bastin J. (2005) Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders. Hum Mol Genet 14:2695-703.

Browning MF, Larson C, Strauss A, Marsden DL. (2005) Normal acylcarnitine levels during confirmation of abnormal newborn screening in long-chain fatty acid oxidation defects. J Inherit Metab Dis 28:545-50.

Strauss AW. (2004) Tandem mass spectrometry in discovery of disorders of the metabolome. J Clin Invest 113:354-6.

Spierkerkoetter U, Khuchua Z, Yue Z, Strauss AW. (2004) The early-onset phenotype of mitochondrial trifunctional protein deficiency: a lethal disorder with multiple tissue involvement. J Inherit Metab Dis 27:294-6.

Spiekerkoetter U, Tokunaga C, Wendel U, Mayatepek E, Exil V, Duran M, Wijburg FA, Wanders RJ, Strauss AW. (2004) Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress. Eur J Clin Invest 34:191-6.

Spiekerkoetter U, Khuchua Z, Yue Z, Bennett MJ, Strauss AW. (2004) General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover. Pediatr Res 55:190-6.

Spiekerkoetter U, Bennett MJ, Ben-Zeev B, Strauss AW, Tein I. (2004) Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein. Muscle Nerve 29:66-72.

McKinney JT, Longo N, Hahn SH, Matern D, Rinaldo P, Strauss AW, Dobrowolski SF. (2004) Rapid, comprehensive screening of the human medium chain acyl-CoA dehydrogenase gene. Mol Genet Metab 82:112-20.

Strauss A, Lock JE. (2003) Pediatric cardiomyopathy--a long way to go. N Engl J Med 348:1703-5.

Spiekerkoetter U, Sun B, Zytkovicz T, Wanders R, Strauss AW, Wendel U. (2003) MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency. J Pediatr 143:335-42.

Spiekerkoetter U, Sun B, Khuchua Z, Bennett MJ, Strauss AW. (2003) Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations. Hum Mutat 21:598-607.

Shekhawat P, Bennett MJ, Sadovsky Y, Nelson DM, Rakheja D, Strauss AW. (2003) Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases. Am J Physiol Endocrinol Metab 284:E1098-105.

Khuchua Z, Wozniak DF, Bardgett ME, Yue Z, McDonald M, Boero J, Hartman RE, Sims H, Strauss AW. (2003) Deletion of the N-terminus of murine map2 by gene targeting disrupts hippocampal ca1 neuron architecture and alters contextual memory. Neuroscience 119:101-11.

Exil VJ, Roberts RL, Sims H, McLaughlin JE, Malkin RA, Gardner CD, Ni G, Rottman JN, Strauss AW. (2003) Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice. Circ Res 93:448-55.

Zytkovicz TH, Fitzgerald EF, Marsden D, Larson CA, Shih VE, Johnson DM, Strauss AW, Comeau AM, Eaton RB, Grady GF. (2001) Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program. Clin Chem 47:1945-55.

Ibdah JA, Paul H, Zhao Y, Binford S, Salleng K, Cline M, Matern D, Bennett MJ, Rinaldo P, Strauss AW. (2001) Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death. J Clin Invest 107:1403-9.

Barycki JJ, O'Brien LK, Strauss AW, Banaszak LJ. (2000) Sequestration of the active site by interdomain shifting. Crystallographic and spectroscopic evidence for distinct conformations of L-3-hydroxyacyl-CoA dehydrogenase. J Biol Chem 275:27186-96.

Ibdah JA, Bennett MJ, Rinaldo P, Zhao Y, Gibson B, Sims HF, Strauss AW. (1999) A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med 340:1723-31.

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.
 

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. 

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 Bodulusa 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.

Webb G. The long road to better ACHD care. Congenit Heart Dis. 2010 May-Jun;5(3):198-205.

Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J, Valente AM, Earing MG, Lui G, Gersony DR, Cook S, Ting JG, Nickolaus MJ, Webb G, Landzberg MJ, Broberg CS; Alliance for Adult Research in Congenital Cardiology (AARCC). Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010 Aug 31;122(9):868-75. 

Valente AM, Bhatt AB, Cook S, Earing MG, Gersony DR, Aboulhosn J, Opotowsky AR, Lui G, Gurvitz M, Graham D, Fernandes SM, Khairy P, Webb G, Gerhard-Herman M, Landzberg MJ; AARCC (Alliance for Adult Research in Congenital Cardiology) Investigators. The CALF (Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients) study. J Am Coll Cardiol. 2010 Jul 6;56(2):144-50.

Silversides CK, Kiess M, Beauchesne L, Bradley T, Connelly M, Niwa K, Mulder B, Webb G, Colman J, Therrien J. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Can J Cardiol. 2010 Mar;26(3):e80-97. 

Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet. Oct 2009; 374(9699):1462-71.

Opotowsky AR, Siddiqi OK, Webb, GD. Trends in hospitalizations for adults with congenital heart disease in the U.S.J Am Coll Cardiol. 2009 Jul; 54(5):460-7.

Schwerzmann M, Salehian O, Harris L, Siu SC, Williams WG, Webb GD, Colman JM, Redington A, Silversides CK. Ventricular arrhythmias and sudden death in adults after a Mustard operations for transposition of the great arteries. Eur Heart J. 2009 Aug; 30(15):1873-9.

Hickey EJ, Veldtman G, Bradley TJ, Gengsakul A, Manlhiot C, Williams WG, Webb GD, McCrindle BW. Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades. European Journal of Cardio-thoracic Surgery. 2009 Jan;35(1):156-64.

Tzemos N, Silversides CK, Colman JM, Therrien J, Webb, GD, Mason J, Cocoara E, Sermer M, Siu SC. Late cardiac outcomes after pregnancy in women with congenital aortic stenosis. American Heart Journal. 2009;157(3):474-480.

Opotowsky AR, Landzberg MJ, Kimmel SE, Webb GD. Research Letter: Trends in the use of percutaneous closure of patent foramen ovale and atrial septal defect in adults, 1998-2004. JAMA. 2008;299(5):521-522.

Clinical molecular genetics; molecular genetic diagnosis of heritable cardiovascular disease; molecular diagnosis of viral infection of the heart

MD, MS: Peking University Health Science Center, Beijing, China.

PhD: Rutgers University, New Brunswick, NJ.

Fellowship: Clinical Molecular Genetics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH.

Certification: American Board of Medical Genetics in Clinical Molecular Genetic,  2011.

RN: University of Cincinnati, Cincinnati, Ohio 1994.

MS: Nursing, University of Cincinnati, Cincinnati, Ohio 1997.

Certification: Pediatric Nurse Practitioner, 1997.
 

Correction of complex cardiac defects in the newborn period, tracheal reconstruction, and blood conservation in open-heart surgery.

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.

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.

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.

Giuliano JS Jr, Lahni PM, Bigham MT, Manning PB, Nelson DP, Wong HR, Wheeler DS. Plasma angiopoietin-2 levels increase in children following cardiopulmonary bypass.Intensive Care Med. 2008 Oct;34(10):1851-7.

Nguyen MT, Dent CL, Ross GF, Harris N, Manning PB, Mitsnefes MM, Devarajan P. Urinary aprotinin as a predictor of acute kidney injury after cardiac surgery in children receiving aprotinin therapy.Pediatr Nephrol. 2008 Aug;23(8):1317-26.

Bigham MT, Brady PW, Manning PB, Jacobs BR, Kimball TR, Wong HR. Therapeutic application of intrapericardial tissue plasminogen activator in a 4-month-old child with complex fibropurulent pericarditis.Pediatr Crit Care Med. 2008 Jan;9(1):e1-4.

Ashcraft TM, Jones K, Border WL, Eghtesady P, Pearl JM, Khoury PR, Manning PB. Factors affecting long-term risk of aortic arch recoarctation after the Norwood procedure. Ann Thorac Surg. 2008 Apr;85(4):1397-401; discussion 1401-2.

Manning PB, Rutter MJ, Border WL. Slide tracheoplasty in infants and children: risk factors for prolonged postoperative ventilatory support.Ann Thorac Surg. 2008 Apr;85(4):1187-91; discussion 1191-2.

Wheeler DS, Dent CL, Manning PB, Nelson DP. Factors prolonging length of stay in the cardiac intensive care unit following the arterial switch operation.Cardiol Young. 2008 Feb;18(1):41-50.

Giuliano JS Jr, Sekar P, Dent CL, Border WL, Hirsch R, Manning PB, Wheeler DS. Unilateral pulmonary edema and acute rheumatic fever. Eur J Pediatr. 2008 Apr;167(4):465-7.

Clinical

Pediatric cradiopulmonary bypass and support techniques; circuit miniaturization; bloodless cardiac surgical techniques

Research

Pediatric cradiopulmonary bypass and support techniques; circuit miniaturization; bloodless cardiac surgical techniques; role of ATIII in inflammatory response

Burnside J, Gomez D, Preston TJ, Olshove VF Jr, Phillips A. In-vitro quantification of gaseous microemboli in two extracorporeal life support circuits. J Extra Corpor Technol. 2011 Sep;43(3):123-9.

Hershenson JA, Ro PS, Miao Y, Tobias JD, Olshove V, Naguib AN. Changes in hemodynamic parameters and cerebral saturation during supraventricular tachycardia. Pediatr Cardiol. 2012 Feb;33(2):286-9.

Sawdy JM, Kempton TM, Olshove V, Gocha M, Chisolm JL, Hill SL, Kirk A, Cheatham JP, Holzer RJ. Use of a dose-dependent follow-up protocol and mechanisms to reduce patients and staff radiation exposure in congenital and structural interventions. Catheter Cardiovasc Interv. 2011 Jul 1;78(1):136-42. Erratum in: Catheter Cardiovasc Interv. 2011 Sep 1;78(3):491.

Zaidi AN, Bauer JA, Michalsky MP, Olshove V, Boettner B, Phillips A, Cook SC. The impact of obesity on early postoperative outcomes in adults with congenital heart disease. Congenit Heart Dis. 2011 May-Jun;6(3):241-6. 

Naguib AN, Corridore M, Phillips A, Olshove V, Galantowicz M, Tobias JD. Perioperative Care of an Infant With an Anomalous Left Innominate Artery Arising From the Main Pulmonary Artery. J Intensive Care Med. 2011 Feb 23.

Naguib AN, Winch P, Ro PS, Olshove V, Tobias JD. Changes in near-infrared spectroscopy and the bispectral index during tilt-table examination. Pediatr Cardiol. 2011 Feb;32(2):234-6.

Preston TJ, Ratliff TM, Gomez D, Olshove VE Jr, Nicol KK, Sargel CL, Chicoine LG. Modified surface coatings and their effect on drug adsorption within the extracorporeal life support circuit. J Extra Corpor Technol. 2010 Sep;42(3):199-202.

Birnbaum B, Berger G, Fenstermaker B, Rowland DG, Boettner B, Olshove V, Galantowicz M, Cheatham JP, Cua CL. Echocardiographic parameters that predict outcome in aortic atresia patients undergoing comprehensive stage II procedure. Congenit Heart Dis. 2010 Sep-Oct;5(5):409-15.

Olshove VF Jr, 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.

Preston TJ, Gomez D, Olshove VF Jr, Phillips A, Galantowicz M. Clinical gaseous microemboli assessment of an oxygenator with integral arterial filter in the pediatric population. J Extra Corpor Technol. 2009 Dec;41(4):226-30.

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.

BSN: University of Louisville, Louisville, KY, 2006.

MSN: Spalding University, Louisville, KY, 2008.

Certifications: Certified Surgical First Assistant, 1998; Family Nurse Practitioner, 2008; Pediatric Nurse Practitioner, 2009.

MD: Boston University, Boston, MA, 1981.

Residency: Anesthesiology, Massachusetts General Hospital, Boston, MA, 1984

Fellowship: Pediatric Anesthesia, Chief Anesthesia Fellow, The Children's Hospital, Boston, MA, 1985.

Certification: Anesthesiology, 1978, 1986; PALS, 2007; ACLS, 2008.

Clinical Interests

Cardiac anesthesia; regional anesthesia; anesthesia for airway surgery

MD: University of Illinois, Chicago, IL, 1994.

Residency: Anesthesiology, University of North Carolina Hospitals, Chapel Hill, NC, 2000.

Residency: Pediatrics, Children's Hospital Medical Center, Cincinnati, OH, 1997.

Fellowship: Pediatric Anesthesiology, University of North Carolina Hospitals, Chapel Hill, NC.

Certification: Pediatrics, 1997; Anesthesiology, 2001; Cardiac Anesthesia.

 Lin EP, Aronson LA. Successful resuscitation of bupivacaine-induced cardiotoxicity in a neonate. Paediatr Anaesth. 2010 Oct;20(10):955-7.

Aronson, LA; Spaeth, J.P.: Frontiers in Pediatric Anesthesia: Cardiac Anesthesia. International Anesthesiology Clinics: Frontiers in Pediatric Anesthesiology. Winter 2006; 44(1):33-49.

Aronson, LA; Dent, C.L.: Postoperative Respiratory Function and Its Management. Pediatric Cardiac Anesthesia. 2005.

Book Chapters

Aronson LA. Pediatrics and Neonatology: Hypoxemia. Complications in Anesthesia. Ed: John L Atlee, 2nd ed, 2007.

Clinical Interests

Anesthesia for congenital cardiac disease; anesthesia equipment and monitoring

Research Interests

Anesthesia equipment and monitoring; regional anesthesia in children; outcomes after anesthesia in children
 

Hoffmann CO, Samuels PJ, Beckman E, Hein EA, Shackleford TM, Overbey E, Berlin RE, Wang Y, Nick TG, Gunter JB. Insufflation vs intubation during esophagogastroduodenoscopy in children. Paediatr Anaesth. 2010 Sep;20(9):821-30.

Mahmoud M, Radhakrishman R, Gunter J, Sadhasivam S, Schapiro A, McAuliffe J, Kurth D, Wang Y, Nick TG, Donnelly LF. Effect of increasing depth of dexmedetomidine anesthesia on upper airway morphology in children. Paediatr Anaesth. 2010 Jun;20(6):506-15.

Sadhasivam S, Cohen LL, Hosu L, Gorman KL, Wang Y, Nick TG, Jou JF, Samol N, Szabova A, Hagerman N, Hein E, Boat A, Varughese A, Kurth CD, Willging JP, Gunter JB. Real-time assessment of perioperative behaviors in children and parents: development and validation of the perioperative adult child behavioral interaction scale. Anesth Analg. 2010 Apr 1;110(4):1109-15.

Mahmoud M, Gunter J, Donnelly LF, Wang Y, Nick TG, Sadhasivam S. A comparison of dexmedetomidine with propofol for magnetic resonance imaging sleep studies in children. Anesth Analg. 2009 Sep;109(3):745-53.

Mahmoud M, Gunter J, Sadhasivam S. Ciné MRI airway studies in children with sleep apnea: optimal images and anesthetic challenges.Pediatr Radiol. 2009 Oct;39(10):1034-7.

Sadhasivam S, Cohen LL, Szabova A, Varughese A, Kurth CD, Willging P, Wang Y, Nick TG, Gunter J. Real-time assessment of perioperative behaviors and prediction of perioperative outcomes.Anesth Analg. 2009 Mar;108(3):822-6.

Gunter JB, Ball J, Than-Win S. Preparation of the Dräger Fabius anesthesia machine for the malignant-hyperthermia susceptible patient. Anesth Analg. 2008 Dec;107(6):1936-45.

Varughese AM, Nick TG, Gunter J, Wang Y, Kurth CD. Factors predictive of poor behavioral compliance during inhaled induction in children. Anesth Analg. 2008 Aug;107(2):413-21.

Mahmoud M, Bryan Y, Gunter J, Kreeger RN, Sadhasivam S. Anesthetic implications of undiagnosed late onset central hypoventilation syndrome in a child: from elective tonsillectomy to tracheostomy.Paediatr Anaesth. 2007 Oct;17(10):1001-5.

Cohen MB, Gunter JB. How safe is intravenous sedation with midazolam and fentanyl for pediatric gastrointestinal endoscopy?Nat Clin Pract Gastroenterol Hepatol. 2007 Oct;4(10):538-9.

Anesthesia for congenital cardiac disease

Kreeger, R.N. Conscious Sedation. Mont Reid Surgical Handbook. 6th ed., 2008. 

Mahmoud M, Bryan Y, Gunter J, Kreeger RN, Sadhasivam S. Anesthetic implications of undiagnosed late onset central hypoventilation syndrome in a child: from elective tonsillectomy to tracheostomy. Paediatr Anaesth. 2007 Oct;17(10):1001-5.

Kreeger RN, Hilvano A. Anesthetic options for the parturient with a neural tube defect. Int Anesthesiol Clin. 2005 Fall;43(4):65-80. Review.

Cardiac anesthesia

Cardiac anesthesiology

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.

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.

Pediatric cardiac anesthesiology

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

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.

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.

Sadayappan S, Gulick J, Klevitsky R, Lorenz JN, Sargent M, Molkentin JD, Robbins J. Cardiac myosin binding protein-C phosphorylation in a {beta}-myosin heavy chain background. Circulation. 2009 Mar 10;119(9):1253-62.

Krenz M, Gulick J, Osinska HE, Colbert MC, Molkentin JD, Robbins J. Role of ERK1/2 signaling in congenital valve malformations in Noonan syndrome. Proc Natl Acad Sci U S A. 2008 Dec 2;105(48):18930-5.

Gulick J, Robbins J. Regulation of transgene expression using tetracycline. Curr Protoc Mol Biol. 2005 Aug;Chapter 23:Unit 23.12.

Pattison JS, Waggoner JR, James J, Martin L, Gulick J, Osinska H, Klevitsky R, Kranias EG, Robbins J. Phospholamban overexpression in transgenic rabbits. Transgenic Res. 2008 Apr;17(2):157-70.

Maloyan A, Gulick J, Glabe CG, Kayed R, Robbins J. Exercise reverses preamyloid oligomer and prolongs survival in alphaB-crystallin-based desmin-related cardiomyopathy. Proc Natl Acad Sci U S A. 2007 Apr 3;104(14):5995-6000

Sadayappan S, Gulick J, Osinska H, Martin LA, Hahn HS, Dorn GW 2nd, Klevitsky R, Seidman CE, Seidman JG, Robbins J. Cardiac myosin-binding protein-C phosphorylation and cardiac function. Circ Res. 2005 Nov 25;97(11):1156-63.

Sanbe A, Osinska H, Villa C, Gulick J, Klevitsky R, Glabe CG, Kayed R, Robbins J. Reversal of amyloid-induced heart disease in desmin-related cardiomyopathy. Proc Natl Acad Sci U S A. 2005 Sep 20;102(38):13592-7. 

Clinical Interests

Cardiovascular genetics; echocardiography

Research Interests

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

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).

Hinton RB. Genetic contribution to bicuspid aortic valve morphology. Am J Med Genet. 2011;155(11):2899-2900.

Acharya A, Hans CP, Koenig SN, Nichols HA, Galindo CL, Garner HR, Merrill WH, Hinton RB, Garg V. Inhibitory role of Notch1 in calcific aortic valve disease. PLoS One. 2011;6(11):e27743.

Snider P, Hinton RB, Moreno-Rodriguez RA, Wang J, Rogers R, Lindsley A, Li F, Ingram D, Menick D, Field LJ, Firulli AB, Molkentin JD, Markwald RR, Conway SJ.  Periostin is required for maturation and extracellular matrix stabilization of noncardiomyocyte lineages of the heart.  Circ Res. Apr 2008;102(7):752-760.

Hinton RB, Alfieri C, Witt S, Glascock B, Khoury PR, Benson DW, Yutzey KE.  Mouse heart valve structure and function: echocardiographic and morphometric analyses from the fetus through the aged adult.  Am J Physio Heart Circ Physiol. 2008;294(6):H2480-2488.

Hinton RB, 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.  Pediatr Res.  Oct 2008;64(4):364-369.

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

Visit the James Lab.

Mitochondrial function; structure and dynamics in cardiac cells in normal and pathological conditions; role of mitochondrial phospholipds 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.

Clinical Interests

Heart failure; cardiopulmonary bypass mediated myocardial dysfunction

Research Interests

Myocardial remodeling

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.

Molkentin JD. The Transcription Factor C/EBP{beta} Serves as a Master Regulator of Physiologic Cardiac Hypertrophy.Circ Res. 2011 Feb 4;108(3):277-8.

Eder P, Molkentin JD. TRPC channels as effectors of cardiac hypertrophy.Circ Res. 2011 Jan 21;108(2):265-72.

Kehat I, Molkentin JD. Molecular pathways underlying cardiac remodeling during pathophysiological stimulation. Circulation. 2010 Dec 21;122(25):2727-35.

Sengupta A, Molkentin JD, Paik JH, Depinho RA, Yutzey KE. FoxO transcription factors promote cardiomyocyte survival upon induction of oxidative stress.J Biol Chem. 2010 Dec 15.

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.

Liu Q, Molkentin JD. Protein kinase Cα as a heart failure therapeutic target. J Mol Cell Cardiol. 2010 Oct 16.

Wansapura JP, Millay DP, Dunn RS, Molkentin JD, Benson DW. Magnetic resonance imaging assessment of cardiac dysfunction in δ-sarcoglycan null mice. Neuromuscul Disord. 2011 Jan;21(1):68-73.

Elrod JW, Wong R, Mishra S, Vagnozzi RJ, Sakthievel B, Goonasekera SA, Karch J, Gabel S, Farber J, Force T, Brown JH, Murphy E, Molkentin JD. Cyclophilin D controls mitochondrial pore-dependent Ca(2+) exchange, metabolic flexibility, and propensity for heart failure in mice.J Clin Invest. 2010 Oct 1;120(10):3680-7. doi: 10.1172/JCI43171.

van Berlo JH, Elrod JW, van den Hoogenhof MM, York AJ, Aronow BJ, Duncan SA, Molkentin JD. The transcription factor GATA-6 regulates pathological cardiac hypertrophy.Circ Res. 2010 Oct 15;107(8):1032-40.

Heineke J, Auger-Messier M, Correll RN, Xu J, Benard MJ, Yuan W, Drexler H, Parise LV, Molkentin JD. CIB1 is a regulator of pathological cardiac hypertrophy.Nat Med. 2010 Aug;16(8):872-9.

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.

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.