Heart Institute

Division Details

Division Data Summary

Research and Training Details

Number of Faculty61
Number of Joint Appointment Faculty2
Number of Research Fellows36
Number of Research Students19
Number of Support Personnel266
Direct Annual Grant Support$8,257,154
Direct Annual Industry Support$32,236
Peer Reviewed Publications190

Clinical Activities and Training

Number of Clinical Staff6
Number of Clinical Fellows14
Number of Other Students1
Inpatient Encounters1961
Outpatient Encounters18493

Division Photo

Heart Institute

Row 1: E Purevjav, D Morales, J Towbin, J Robbins, B Blaxall, A Lorts, C Chin, L Lee

Row 2: S Kirk, C Castleberry, A Divanovic, R Beekman, B Goldstein, J Sticka, K Yutzey, M Maillet

Row 3: S Ware, J Waxman, J Storey, H Ippisch, J Jefferies, C Learn, J James, J Gulick, J Anderson

Row 4: J Molkentin, B Marino, R Hinton, R Czosek, C Madueme, N Madsen, I Wilmot, S Hanke, E Michelfelder, T Ryan, C Statile, M Taylor

Significant Accomplishments

Significant Accomplishments

New Program Focuses on the Links Between the Mind and Heart

Research shows that children with complex congenital and acquired heart disease are at a greater risk for neurodevelopment issues. The Heart Institute-Kindervelt Neurodevelopmental and Educational Clinic (HI NDEC) at Cincinnati Children’s Hospital Medical Center is a new, innovative program established by Bradley Marino, MD, MPP, MSCE, and now headed by Haleh Heydarian, MD.  It provides individualized neurodevelopmental follow-up care designed to help each child reach their full potential by optimizing their neurodevelopmental capabilities and psychosocial outcome. The multidisciplinary HI NDEC team provides a comprehensive neurodevelopmental evaluation for infants, toddlers, children and adolescents. It provides families with the appropriate surveillance, screening, evaluation and treatment resources needed to ensure the best outcome possible for their child. The HI NDEC staff performs developmental assessments on infants, toddlers, and preschool children to maximize school preparedness, and assesses for difficulties in school-age children. Providers evaluate for problems with attention, task management and organization, and for behavioral and emotional problems that may impact relationships with friends and family. The HI NDEC has experts that span an array of specialties, including: Pediatric Cardiology, Developmental-Behavioral Pediatrics, Pediatric Psychology, Education, Pediatric Neurology, Occupational/Physical Therapy, Pediatric Nutrition, Cardiac Nursing, and Social Work. The clinic was prominently featured in the Health Section of the Wall Street Journal on July 31, 2013.

Gene Discovery Could Lead to Treatment to Prevent Thickening of the Heart Muscle

Cardiac fibrosis refers to the thickening or “scarring” of muscle tissue in the heart. Uncontrolled or prolonged accumulation of fibrotic material in the heart contributes to worsening heart disease and leads to heart failure and death. Thus, a better understanding of the genes and mechanisms that promote cardiac fibrosis are critical as we develop new therapies to stop or slow this process. Cardiac fibrosis is controlled primarily by a cell type known as the fibroblast. In select disease states, these fibroblasts become activated and change into a cell type known as the myofibroblast, which can generate proteins that cause fibrosis. In a recent paper in a premier scientific journal, Developmental Cell, Jeffery Molkentin, PhD, and colleagues showed that a novel gene, TRPC6, underlies the conversion of fibroblasts into myofibroblasts, thereby promoting cardiac fibrosis and disease. Molkentin showed that mice lacking TRPC6 had defective generation of myofibroblasts, with a lessening of the fibrotic response in vivo. The lab also showed how TRPC6 was activated in fibroblasts to begin this process, and identified how TRPC6 works to actually convert these cells into myofibroblasts. Their work suggests new therapies with existing experimental drugs might have an anti-fibrotic effect, possibly reducing the progression of heart failure in select disease states.

Heart Institute Sets Sights on Becoming Worldwide Training Center for Pediatric Mechanical Circulatory Support

Mechanical circulatory support for children is an emerging and quickly changing field, and, as such, no institute in the world has established itself as a training and resource center for the multiple initiatives by several worldwide companies creating pediatric ventricular assist devices and total artificial hearts. The Heart Institute is poised to become this international reference center. We have formed relationships with global companies interested in developing ventricular assist devices for pediatric patients. Many of these companies are leading the way to develop new devices that can serve as bridges to heart transplant while patients wait for a heart to become available. These companies include SynCardia Systems, HeartWare, Thoratec, Berlin Heart, and Levitronix. In the past year, we trained physicians from Japan and South America, and were invited to host training symposia for several of these companies at Cincinnati Children’s. As a direct result of these collaborations, the Heart Institute’s surgical team is now the Primary Investigator and Coordinating Center for the first pediatric total artificial heart trial. Additionally, we have created (in concert with our transportation team) an Extracorporeal Membrane Oxygenation (ECMO) transport program, which enables us to treat critically ill children while transferring them to Cincinnati Children’s. This program gives children access to care that they would not have been able to receive elsewhere. The Heart Institute is the only program in the country to provide third-party ECMO transport. We hope by the end of this year to have firmly established the Heart Institute and Cincinnati Children’s as the premier worldwide training and pediatric resource center for mechanical circulatory support.

Division Publications

Heart Institute Publications

  1. Alghamdi M, De Souza AM, White CT, Potts MT, Warady BA, Furth SL, Kimball TR, Potts JE, Sandor GG. Echo-Doppler assessment of the biophysical properties of the aorta in children with chronic kidney disease. Pediatr Cardiol. 2013; 34:1218-25.
  2. Allen KY, Goldstein BH, Pahl E, Schumacher KR, Gajarski RJ, Backer CL, Wax DF, Kaushal S, Gossett JG. Non-cameral coronary artery fistulae after pediatric cardiac transplantation: a multicenter study. J Heart Lung Transplant. 2012; 31:744-9.
  3. Almond CS, Morales DL, Blackstone EH, Turrentine MW, Imamura M, Massicotte MP, Jordan LC, Devaney EJ, Ravishankar C, Kanter KR, Holman W, Kroslowitz R, Tjossem C, Thuita L, Cohen GA, Buchholz H, St Louis JD, Nguyen K, Niebler RA, Walters HL, 3rd, Reemtsen B, Wearden PD, Reinhartz O, Guleserian KJ, Mitchell MB, Bleiweis MS, Canter CE, Humpl T. Berlin Heart EXCOR Pediatric Ventricular Assist Device for Bridge to Heart Transplantation in US Children. Circulation. 2013; 127:1702-11.
  4. Anderson JB, Czosek RJ, Cnota J, Meganathan K, Knilans TK, Heaton PC. Pediatric syncope: national hospital ambulatory medical care survey results. J Emerg Med. 2012; 43:575-83.
  5. Anderson JB, Czosek RJ, Knilans TK, Marino BS. The effect of paediatric syncope on health-related quality of life. Cardiol Young. 2012; 22:583-8.
  6. Anderson JB, Czosek RJ, Knilans TK, Meganathan K, Heaton P. Postoperative Heart Block in Children with Common Forms of Congenital Heart Disease: Results from the KID Database. J Cardiovasc Electrophysiol. 2012; 23:1349-54.
  7. Anderson JB, Iyer SB, Schidlow DN, Williams R, Varadarajan K, Horsley M, Slicker J, Pratt J, King E, Lannon C. Variation in growth of infants with a single ventricle. J Pediatr. 2012; 161:16-21 e1; quiz 21 e2-3.
  8. Antoniak S, Owens AP, 3rd, Baunacke M, Williams JC, Lee RD, Weithäuser A, Sheridan PA, Malz R, Luyendyk JP, Esserman DA, Trejo J, Kirchhofer D, Blaxall BC, Pawlinski R, Beck MA, Rauch U, Mackman N. PAR-1 contributes to the innate immune response during viral infection. J Clin Invest. 2013; 123:1310-22.
  9. Aprikyan AA, Khuchua Z. Advances in the understanding of Barth syndrome. Br J Haematol. 2013; 161:330-8.
  10. Ashley EA, Hershberger RE, Caleshu C, Ellinor PT, Garcia JG, Herrington DM, Ho CY, Johnson JA, Kittner SJ, Macrae CA, Mudd-Martin G, Rader DJ, Roden DM, Scholes D, Sellke FW, Towbin JA, Van Eyk J, Worrall BB. Genetics and cardiovascular disease: a policy statement from the American Heart Association. Circulation. 2012; 126:142-57.
  11. Askenazi DJ, Selewski DT, Paden ML, Cooper DS, Bridges BC, Zappitelli M, Fleming GM. Renal replacement therapy in critically ill patients receiving extracorporeal membrane oxygenation. Clin J Am Soc Nephrol. 2012; 7:1328-36.
  12. Auger-Messier M, Accornero F, Goonasekera SA, Bueno OF, Lorenz JN, van Berlo JH, Willette RN, Molkentin JD. Unrestrained p38 MAPK activation in Dusp1/4 double-null mice induces cardiomyopathy. Circ Res. 2013; 112:48-56.
  13. Averin K, Uzark K, Beekman RH, 3rd, Willging JP, Pratt J, Manning PB. Postoperative assessment of laryngopharyngeal dysfunction in neonates after norwood operation. Ann Thorac Surg. 2012; 94:1257-61.
  14. Bal NC, Maurya SK, Sopariwala DH, Sahoo SK, Gupta SC, Shaikh SA, Pant M, Rowland LA, Goonasekera SA, Molkentin JD, Periasamy M. Sarcolipin is a newly identified regulator of muscle-based thermogenesis in mammals. Nat Med. 2012; 18:1575-9.
  15. Bartos DC, Anderson JB, Bastiaenen R, Johnson JN, Gollob MH, Tester DJ, Burgess DE, Homfray T, Behr ER, Ackerman MJ, Guicheney P, Delisle BP. A KCNQ1 mutation causes a high penetrance for familial atrial fibrillation. J Cardiovasc Electrophysiol. 2013; 24:562-9.
  16. Beekman RH, 3rd. Coarctation of the Aorta. In: HD Allen, DJ Driscoll, RE Shaddy, TF Feltes, eds. Moss & Adams' Heart Disease in Infants, Children and Adolescents: Including the Fetus and Young Adult. Philadelphia, PA: Lippincott Williams & Wilkins; 2012:1044-1060.
  17. Beekman RH, 3rd. FDA Clearance of Cardiac Devices for Children: A Primer and Call to Action. Congenital Cardiology Today. 2012; 10:1-7.
  18. Beekman RH, 3rd, Lannon C. The National Pediatric Cardiology Quality Improvement Collaborative. Neonatology Today. 2013; 8:1-5.
  19. Belmonte SL, Blaxall BC. Conducting the G-protein Coupled Receptor (GPCR) Signaling Symphony in Cardiovascular Diseases: New Therapeutic Approaches. Drug Discov Today Dis Models. 2012; 9:e85-e90.
  20. Belmonte SL, Blaxall BC. G protein-coupled receptor kinase 5: exploring its hype in cardiac hypertrophy. Circ Res. 2012; 111:957-8.
  21. Berger KA, Lynch J, Prows CA, Siegel RM, Myers MF. Mothers' perceptions of family health history and an online, parent-generated family health history tool. Clin Pediatr (Phila). 2013; 52:74-81.
  22. Bhuiyan MS, Gulick J, Osinska H, Gupta M, Robbins J. Determination of the critical residues responsible for cardiac myosin binding protein C's interactions. J Mol Cell Cardiol. 2012; 53:838-47.
  23. Blaxall BC, Baudino TA, Kirshenbaum LA. Cardiac fibroblasts and cellular cross talk in heart failure. J Cardiovasc Transl Res. 2012; 5:737-8.
  24. Bolte C, Zhang Y, York A, Kalin TV, Schultz Jel J, Molkentin JD, Kalinichenko VV. Postnatal ablation of Foxm1 from cardiomyocytes causes late onset cardiac hypertrophy and fibrosis without exacerbating pressure overload-induced cardiac remodeling. PLoS One. 2012; 7:e48713.
  25. Braitsch CM, Combs MD, Quaggin SE, Yutzey KE. Pod1/Tcf21 is regulated by retinoic acid signaling and inhibits differentiation of epicardium-derived cells into smooth muscle in the developing heart. Dev Biol. 2012; 368:345-57.
  26. Brescia ST, Rossano JW, Pignatelli R, Jefferies JL, Price JF, Decker JA, Denfield SW, Dreyer WJ, Smith O, Towbin JA, Kim JJ. Mortality and sudden death in pediatric left ventricular noncompaction in a tertiary referral center. Circulation. 2013; 127:2202-8.
  27. Bridges BC, Selewski DT, Paden ML, Cooper DS, Zappitelli M, Fleming GM, Askenazi DJ. Acute Kidney Injury in Neonates Requiring ECMO. NeoReviews. 2012; 13:e428-433.
  28. Brown KL, Ichord R, Marino BS, Thiagarajan RR. Outcomes Following Extracorporeal Membrane Oxygenation in Children With Cardiac Disease. Pediatric Critical Care Medicine. 2013; 15:S73-S83.
  29. Buchaklian AH, Helbling D, Ware SM, Dimmock DP. Recessive deoxyguanosine kinase deficiency causes juvenile onset mitochondrial myopathy. Mol Genet Metab. 2012; 107:92-4.
  30. Burns KC, Nagarajan R, O. SF, Marino BS. Oncology. In: BS Marino, KS Fine, eds. Blueprints: Pediatrics. Philadelphia, PA: Lippincott Williams & Wilkins; 2013:205-223.
  31. Cabrera AG, Khan MS, Morales DL, Chen DW, Moffett BS, Price JF, Dreyer WJ, Denfield SW, Jeewa A, Fraser CD, Jr., Vallejo JG. Infectious complications and outcomes in children supported with left ventricular assist devices. J Heart Lung Transplant. 2013; 32:518-24.
  32. Carruthers CA, Alfieri CM, Joyce EM, Watkins SC, Yutzey KE, Sacks MS. Gene Expression and Collagen Fiber Micromechanical Interactions of the Semilunar Heart Valve Interstitial Cell. Cell Mol Bioeng. 2012; 5:254-265.
  33. Cassedy A, Drotar D, Ittenbach R, Hottinger S, Wray J, Wernovsky G, Newburger JW, Mahony L, Mussatto K, Cohen MI, Marino BS. The impact of socio-economic status on health related quality of life for children and adolescents with heart disease. Health Qual Life Outcomes. 2013; 11:99.
  34. Chen H, Zhang W, Sun X, Yoshimoto M, Chen Z, Zhu W, Liu J, Shen Y, Yong W, Li D, Zhang J, Lin Y, Li B, Vandusen NJ, Snider P, Schwartz RJ, Conway SJ, Field LJ, Yoder MC, Firulli AB, Carlesso N, Towbin JA, Shou W. Fkbp1a controls ventricular myocardium trabeculation and compaction by regulating endocardial Notch1 activity. Development. 2013; 140:1946-57.
  35. Chin C. Cardiac antibody-mediated rejection. Pediatr Transplant. 2012; 16:404-12.
  36. Cnota JF, Allen KR, Colan S, Covitz W, Graham EM, Hehir DA, Levine JC, Margossian R, McCrindle BW, Minich LL, Natarajan S, Richmond ME, Hsu DT, Pediatric Heart Network I. Superior cavopulmonary anastomosis timing and outcomes in infants with single ventricle. J Thorac Cardiovasc Surg. 2013; 145:1288-96.
  37. Cohen M, Ladinsky M, Marino BS. Gastroenterology. In: BS Marino, KS Fine, eds. Blueprints: Pediatrics. Philadelphia, PA: Lippincott Williams & Wilkins; 2013:134-153.
  38. Cooper DS, Hirsch JC, Jacobs JJ. Pediatric Cardiac Extracorporeal Life Support. In: GM Annich, WR Lynch, G MacLaren, JM Wilson, RH Bartlett, eds. ECMO: Extracorporeal Cardiopulmonary Support in Critical Care. Ann Arbor: Extracorporeal Life Support Organization; 2012.
  39. Cooper DS, Pretre R. Clinical management of pediatric ventricular assist devices. Pediatr Crit Care Med. 2013; 14:S27-36.
  40. Cooper DS, Thiagarajan RR, Almond CS, VanderPluym CJ, Buchholz H, Jacobs JP. Ventricular Assist Devices in Children. In: GM Annich, WR Lynch, G MacLaren, JM Wilson, RH Bartlett, eds. ECMO: Extracorporeal Cardiopulmonary Support in Critical Care. Ann Arbor: Extracorporeal Life Support Organization; 2012.
  41. Correll RN, Molkentin JD. CaMKII does it again: even the mitochondria cannot escape its influence. Circ Res. 2013; 112:1208-11.
  42. Czosek RJ, Anderson J, Khoury PR, Knilans TK, Spar DS, Marino BS. Utility of ambulatory monitoring in patients with congenital heart disease. Am J Cardiol. 2013; 111:723-30.
  43. Czosek RJ, Anderson JB, Heaton PC, Cassedy A, Schnell B, Cnota JF. Staged palliation of hypoplastic left heart syndrome: trends in mortality, cost, and length of stay using a national database from 2000 through 2009. Am J Cardiol. 2013; 111:1792-9.
  44. Czosek RJ, Bonney WJ, Cassedy A, Mah DY, Tanel RE, Imundo JR, Singh AK, Cohen MI, Miyake CY, Fawley K, Marino BS. Impact of cardiac devices on the quality of life in pediatric patients. Circ Arrhythm Electrophysiol. 2012; 5:1064-72.
  45. Davis J, Burr AR, Davis GF, Birnbaumer L, Molkentin JD. A TRPC6-dependent pathway for myofibroblast transdifferentiation and wound healing in vivo. Dev Cell. 2012; 23:705-15.
  46. Davis J, Maillet M, Miano JM, Molkentin JD. Lost in Transgenesis: A User's Guide for Genetically Manipulating the Mouse in Cardiac Research. Circ Res. 2012; 111:761-77.
  47. Dhawan L, Liu B, Pytlak A, Kulshrestha S, Blaxall BC, Taubman MB. Y-Box Binding Protein 1 and RNase UK114 Mediate Monocyte Chemoattractant Protein 1 mRNA Stability in Vascular Smooth Muscle Cells. Mol Cell Biol. 2012; 32:3768-75.
  48. Divanovic A, Witte D, Michelfelder E. A fetus with hypoplastic left heart syndrome and tricuspid stenosis with evolving hydrops fetalis. Pediatr Cardiol. 2012; 33:1210-2.
  49. Drawnel FM, Wachten D, Molkentin JD, Maillet M, Aronsen JM, Swift F, Sjaastad I, Liu N, Catalucci D, Mikoshiba K, Hisatsune C, Okkenhaug H, Andrews SR, Bootman MD, Roderick HL. Mutual antagonism between IP(3)RII and miRNA-133a regulates calcium signals and cardiac hypertrophy. J Cell Biol. 2012; 199:783-98.
  50. Elder D, Marino BS. Endocrinology. In: BS Marino, KS Fine, eds. Blueprints: Pediatrics. Philadelphia, PA: Lippincott Williams & Wilkins; 2013:241-455.
  51. Elrod JW, Molkentin JD. Physiologic functions of cyclophilin D and the mitochondrial permeability transition pore. Circ J. 2013; 77:1111-22.
  52. Fleming GM, Askenazi DJ, Bridges BC, Cooper DS, Paden ML, Selewski DT, Zappitelli M. A multicenter international survey of renal supportive therapy during ECMO: the Kidney Intervention During Extracorporeal Membrane Oxygenation (KIDMO) group. ASAIO J. 2012; 58:407-14.
  53. Frank LH, Bradshaw E, Beekman R, Mahle WT, Martin GR. Critical congenital heart disease screening using pulse oximetry. J Pediatr. 2013; 162:445-53.
  54. Frommelt PC, Gerstenberger E, Baffa J, Border WL, Bradley TJ, Colan S, Gorentz J, Heydarian H, John JB, Lai WW, Levine J, Lu JC, McCandless RT, Miller S, Nutting A, Ohye RG, Pearson GD, Wong PC, Cohen MS, Pediatric Heart Network I. Doppler flow patterns in the right ventricle-to-pulmonary artery shunt and neo-aorta in infants with single right ventricle anomalies: impact on outcome after initial staged palliations. J Am Soc Echocardiogr. 2013; 26:521-9.
  55. Fujimoto KL, Tobita K, Guan J, Hashizume R, Takanari K, Alfieri CM, Yutzey KE, Wagner WR. Placement of an elastic biodegradable cardiac patch on a subacute infarcted heart leads to cellularization with early developmental cardiomyocyte characteristics. J Card Fail. 2012; 18:585-95.
  56. Gao H, Wang F, Wang W, Makarewich CA, Zhang H, Kubo H, Berretta RM, Barr LA, Molkentin JD, Houser SR. Ca(2+) influx through L-type Ca(2+) channels and transient receptor potential channels activates pathological hypertrophy signaling. J Mol Cell Cardiol. 2012; 53:657-67.
  57. George-Abraham JK, Zimmerman SL, Hinton RB, Marino BS, Witte DP, Hopkin RJ. Tetrasomy 15q25.2-->qter identified with SNP microarray in a patient with multiple anomalies including complex cardiovascular malformation. Am J Med Genet A. 2012; 158A:1971-6.
  58. Ghanayem NS, Allen KR, Tabbutt S, Atz AM, Clabby ML, Cooper DS, Eghtesady P, Frommelt PC, Gruber PJ, Hill KD, Kaltman JR, Laussen PC, Lewis AB, Lurito KJ, Minich LL, Ohye RG, Schonbeck JV, Schwartz SM, Singh RK, Goldberg CS. Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg. 2012; 144:896-906.
  59. Goldenberg P, Marino BS. Genetic Disorders. In: BS Marino, KS Fine, eds. Blueprints: Pediatrics. Philadelphia, PA: Lippincott Williams & Wilkins; 2013:315-326.
  60. Goldstein BH, Hirsch R, Zussman ME, Vincent JA, Torres AJ, Coulson J, Ringel RE, Beekman RH, 3rd. Percutaneous balloon-expandable covered stent implantation for treatment of traumatic aortic injury in children and adolescents. Am J Cardiol. 2012; 110:1541-5.
  61. Grenier MA. Looking into an athlete's heart: Panacea or Pandora's box?. Future Cardiol. 2012; 8:805-7.
  62. Griffin AC, Strauss AW, Bennett MJ, Ernst LM. Mutations in long-chain 3-hydroxyacyl coenzyme a dehydrogenase are associated with placental maternal floor infarction/massive perivillous fibrin deposition. Pediatr Dev Pathol. 2012; 15:368-74.
  63. Guha IN, Bokhandi S, Ahmad Z, Sheron N, Cope R, Marshall C, Veldtman G. Structural and functional uncoupling of liver performance in the Fontan circulation. Int J Cardiol. 2013; 164:77-81.
  64. Gupta R, Marino BS, Cnota JF, Ittenbach RF. Finding the right distribution for highly skewed zero-inflated clinical data. Epidemiology Biostatistics and Public Health. 2013; 10:e8732-1-15.
  65. Gurvitz M, Valente AM, Broberg C, Cook S, Stout K, Kay J, Ting J, Kuehl K, Earing M, Webb G, Houser L, Opotowsky A, Harmon A, Graham D, Khairy P, Gianola A, Verstappen A, Landzberg M, Alliance for Adult Research in Congenital C, Adult Congenital Heart A. Prevalence and Predictors of Gaps in Care Among Adult Congenital Heart Disease Patients: HEART-ACHD (The Health, Education, and Access Research Trial). J Am Coll Cardiol. 2013; 61:2180-4.
  66. Hampl S, Demeule M, Eneli I, Frank M, Hawkins MJ, Kirk S, Morris P, Sallinen BJ, Santos M, Ward WL, Rhodes E. Parent perspectives on attrition from tertiary care pediatric weight management programs. Clin Pediatr (Phila). 2013; 52:513-9.
  67. Hanke SP, Gardner AB, Lombardi JP, Manning PB, Nelson DP, Towbin JA, Jefferies JL, Lorts A. Left ventricular noncompaction cardiomyopathy in barth syndrome: an example of an undulating cardiac phenotype necessitating mechanical circulatory support as a bridge to transplantation. Pediatr Cardiol. 2012; 33:1430-4.
  68. Harris LL, Krishnamurthy R, Browne LP, Morales DL, Friedman EM. Left main bronchus obstruction after patent ductus arteriosus ligation: an unusual complication. Int J Pediatr Otorhinolaryngol. 2012; 76:1855-6.
  69. Hayes D, Jr., DiPaola N, Baker PB, Kirkby S, Phillips AB, Nicol KK. Antibody-mediated rejection in a lung transplant recipient after acute stroke. Transpl Immunol. 2012; 27:171-4.
  70. Hinton RB. Genetics of Bicuspid Aortic Valve and Calcific Aortic Valve Disease. In: E Aikawa, ed. Calcific Aortic Valve Disease. Rijeka, Croatia: InTech; 2013:173-204.
  71. Hinton RB. Genetic and environmental factors contributing to cardiovascular malformation: a unified approach to risk. J Am Heart Assoc. 2013; 2:e000292.
  72. Hinton RB. Bicuspid aortic valve and thoracic aortic aneurysm: three patient populations, two disease phenotypes, and one shared genotype. Cardiol Res Pract. 2012; 2012:926975.
  73. Hollander SA, Addonizio LJ, Chin C, Lamour JM, Hsu DT, Bernstein D, Rosenthal DN. Abdominal complaints as a common first presentation of heart failure in adolescents with dilated cardiomyopathy. Am J Emerg Med. 2013; 31:684-6.
  74. Jacobs JP, O'Brien SM, Shahian DM, Edwards FH, Badhwar V, Dokholyan RS, Sanchez JA, Morales DL, Prager RL, Wright CD, Puskas JD, Gammie JS, Haan CK, George KM, Sheng S, Peterson ED, Shewan CM, Han JM, Bongiorno PA, Yohe C, Williams WG, Mayer JE, Grover FL. Successful linking of the Society of Thoracic Surgeons Database to Social Security data to examine the accuracy of Society of Thoracic Surgeons mortality data. J Thorac Cardiovasc Surg. 2013; 145:976-83.
  75. James J, Robbins J. Ablating a cardiac protein: causality at last. Circ Res. 2013; 112:1415-9.
  76. Jayakumar C, Ranganathan P, Devarajan P, Krawczeski CD, Looney S, Ramesh G. Semaphorin 3A is a new early diagnostic biomarker of experimental and pediatric acute kidney injury. PLoS One. 2013; 8:e58446.
  77. Jefferies JL, Goldstein SL. Cardiorenal syndrome: an emerging problem in pediatric critical care. Pediatr Nephrol. 2013; 28:855-62.
  78. Jefferies JL, Morales DL. Mechanical circulatory support in children: bridge to transplant versus recovery. Curr Heart Fail Rep. 2012; 9:236-43.
  79. Jodele S, Hirsch R, Laskin B, Davies S, Witte D, Chima R. Pulmonary arterial hypertension in pediatric patients with hematopoietic stem cell transplant-associated thrombotic microangiopathy. Biol Blood Marrow Transplant. 2013; 19:202-7.
  80. Johnson EK, Zhang L, Adams ME, Phillips A, Freitas MA, Froehner SC, Green-Church KB, Montanaro F. Proteomic analysis reveals new cardiac-specific dystrophin-associated proteins. PLoS One. 2012; 7:e43515.
  81. Kamal FA, Travers JG, Blaxall BC. G protein-coupled receptor kinases in cardiovascular disease: why "where" matters. Trends Cardiovasc Med. 2012; 22:213-9.
  82. Karch J, Molkentin JD. Is p53 the long-sought molecular trigger for cyclophilin D-regulated mitochondrial permeability transition pore formation and necrosis?. Circ Res. 2012; 111:1258-60.
  83. Khan MS, Heinle JS, Samayoa AX, Adachi I, Schecter MG, Mallory GB, Morales DL. Is lung transplantation survival better in infants? Analysis of over 80 infants. J Heart Lung Transplant. 2013; 32:44-9.
  84. Khan MS, Mery CM, Zafar F, Adachi I, Heinle JS, Cabrera AG, Fraser CD, Jr., Morales DL. Is mechanically bridging patients with a failing cardiac graft to retransplantation an effective therapy? Analysis of the United Network of Organ Sharing database. J Heart Lung Transplant. 2012; 31:1192-8.
  85. Kimball TR, Michelfelder EC. Echocardiography: Basic Principles and Imaging. In: HD Allen, DJ Driscoll, RE Shaddy, TF Feltes, eds. Moss & Adams' Heart Disease in Infants, Children and Adolescents: Including the Fetus and Young Adult. Philadelphia, PA: Lippincott Williams & Wilkins; 2012:134-171.
  86. Kirk S, Brehm B, Saelens BE, Woo JG, Kissel E, D'Alessio D, Bolling C, Daniels SR. Role of carbohydrate modification in weight management among obese children: a randomized clinical trial. J Pediatr. 2012; 161:320-7 e1.
  87. Knowles R, Veldtman G, Hickey EJ, Bradley T, Gengsakul A, Webb GD, Williams WG, McCrindle BW. Functional health status of adults with tetralogy of Fallot: matched comparison with healthy siblings. Ann Thorac Surg. 2012; 94:124-32.
  88. Knudson JD, Neish SR, Cabrera AG, Lowry AW, Shamszad P, Morales DL, Graves DE, Williams EA, Rossano JW. Prevalence and outcomes of pediatric in-hospital cardiopulmonary resuscitation in the United States: An analysis of the Kids' Inpatient Database*. Crit Care Med. 2012; 40:2940-4.
  89. Kreeger RN, Ramamoorthy C, Nicolson SC, Ames WA, Hirsch R, Peng LF, Glatz AC, Hill KD, Hoffman J, Tomasson J, Kurth CD. Evaluation of pediatric near-infrared cerebral oximeter for cardiac disease. Ann Thorac Surg. 2012; 94:1527-33.
  90. Lalani SR, Shaw C, Wang X, Patel A, Patterson LW, Kolodziejska K, Szafranski P, Ou Z, Tian Q, Kang SH, Jinnah A, Ali S, Malik A, Hixson P, Potocki L, Lupski JR, Stankiewicz P, Bacino CA, Dawson B, Beaudet AL, Boricha FM, Whittaker R, Li C, Ware SM, Cheung SW, Penny DJ, Jefferies JL, Belmont JW. Rare DNA copy number variants in cardiovascular malformations with extracardiac abnormalities. Eur J Hum Genet. 2013; 21:173-81.
  91. Le Grand F, Grifone R, Mourikis P, Houbron C, Gigaud C, Pujol J, Maillet M, Pages G, Rudnicki M, Tajbakhsh S, Maire P. Six1 regulates stem cell repair potential and self-renewal during skeletal muscle regeneration. J Cell Biol. 2012; 198:815-32.
  92. Learn CP, Holzer RJ, Daniels CJ, Torres AJ, Vincent JA, Moore JW, Armsby LB, Landzberg MJ, Bergersen L. Adverse events rates and risk factors in adults undergoing cardiac catheterization at pediatric hospitals-results from the C3PO. Catheter Cardiovasc Interv. 2013; 81:997-1005.
  93. Lorts A, Krawczeski CD, Marino BS. Cardiology. In: BS Marino, KS Fine, eds. Blueprints: Pediatrics. Philadelphia, PA: Lippincott Williams & Wilkins; 2013:86-133.
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Grants, Contracts, and Industry Agreements

Cardiology Grants

Cardiology

Grant and Contract AwardsAnnual Direct

Cnota, J / Krawczeski, C

Pediatric Heart Network Prairieland Consortium
U10 HL 10967309/01/11-06/30/16$367,500
The Relationship of Family Factors to Developmental and Psychosocial Outcomes in Children with Hypoplastic Left Heart Syndrome
U01 HL 06827001/09/12-01/08/14$1,200
Trial of Beta Blocker vs Angiotensin II Receptor Blocker Therapy in Individuals with Marfan Syndrome
U01 HL 06827005/05/11-12/31/14$1,570

Ippisch, H

Effects of Dietary Fat and Exercise Challenges in Obese Children
K23 HL 09117409/01/08-07/31/13$124,500

Jefferies, j

Heart Failure Pediatric Cardiology Fellowship Program
07/01/12-06/30/12$15,000

Kimball, T

Chronic Kidney Disease in Children
U01 DK 06614308/01/08-07/31/13$60,996

Marino, B

Transitional Telehealth Homecare: REACH
R01 NR 00209309/21/11-06/30/16$86,360
Understanding Mechanisms of Fontan Failure and Key Predictors for Patient Outcome
R01 HL 09825202/01/10-01/31/14$16,667

Michelfelder, E

Hypoplastic Left Heart Syndrome: Expression of RHD in the Fetus
R01 HL 09863405/15/11-12/31/14$34,672

Moore, V

Quantitative Non-Invasive Assessment of Right Ventricular Function Using Global Longitudinal Strain and TAPSE in a Pre-Clinical Setting
06/01/13-05/31/14$22,727

Siegel, R

The Health Effects of a Blueberry Enriched Diet on Obese Children: A Feasibility Study
WBANA-Siegel11/01/12-10/31/13$30,000

Towbin, J

Biomarkers in Pediatric Cardiomyopathy
R01 HL 10909008/06/12-07/31/16$34,637

Urbina, E

Accelerated CV Aging in Youth Related to CV Risk Factor Clusters
R01 HL 10559101/01/11-12/31/15$339,301
Current Year Direct$1,135,130
Industry Contracts

Beekman, R

$1,186

Chin, C

$24,623

Hirsch, R

$6,427
Current Year Direct Receipts$32,236
Total$1,167,366

Molecular Cardiovascular Biology Grants

Molecular Cardiovascular Biology

Grant and Contract AwardsAnnual Direct

Accornero, F

PGF Role in Regulating Cardiac Remodeling
11POST753003507/01/11-06/30/13$45,000

Bhuiyan, S

Functional Significance and Regulation of cMyBP-C Binding to Actin
11POST759018107/01/11-06/30/13$45,000

Blaxall B

A Role for Mena in the Heart
R01 HL 0898802/22/13-01/31/14$87,612
Extracellular Matrix Remodeling and Fibrosis
R01 GM 09734708/15/12-11/30/15$45,991
Small Molecule Targeting of MLK3 for Heart Failure
13IRG1467007901/01/13-12/31/14$68,183
Targeting of B-AR/GBy Signaling in the Heart with Small Molecules
R01 HL 09147505/31/13-05/30/14$235,620

Dohn, T

Training in Cardiovascular Biology
T32 HL 00738201/01/13-12/31/13$14,688

Gupta, M

Role of Myosin Binding Protein-C Phosphorylation in Cardiac Function
11POST759002607/01/11-06/30/13$45,000

Khuchua, Z

A Mouse Model of Barth Syndrome, a Mitochondrial Cardiolipin Disorder
R01 HL 10886707/07/11-03/31/15$238,000

Kwong, J

Defining the Role of SLC25a35 as a Regulator of the Mitochondrial Permeability Transition Pore and Cardiomyocyte Death
12POSTDOC1195000007/01/12-06/30/14$47,000

Lee, M

Differential Twist1 Dimer Function and Gene Regulation in Valve Progenitor Cells
11PRE724002307/01/11-06/30/13$23,000

McLendon, P

The Role of Impaired Protein Degradation Pathways in CryABR120G-Mediated Desmin-R
F32 HL 11255801/11/12-03/11/13$8,990

Molkentin, J

Ca Signaling Domains Programming Cardiac Hyprotrophy
P01 HL 08010108/01/11-05/31/16$271,987
Cardiac Hypertrophic Intracellular Signaling Pathways
R01 HL 06292702/01/09-12/31/13$222,750
Improving Cardiac Function after Myocardial Infarction
P01 HL 10880605/07/12-03/31/17$255,000
Molecular Pathways Controlling Cardiac Gene Expression
R37 HL 06056207/01/08-06/30/13$247,500
Mechanisms of TGF Regulated Fibrosis in Muscular Dystrophy
P01 NS 07202707/01/11-06/30/16$250,000
Thrombospondin 4 Regulates Adaptive ER Stress Response
R01 HL 10592401/01/11-12/31/14$312,920

Rajan, S

Translational and Post-translational Regulation of Tropomyosin in Normal and Cardiomyopathic Hearts
11SDG498002908/01/11-12/31/14$70,000

Robbins , J

A TG Rabbit Model for the Functional Effects of FHC Mutations in B-Cardiac Myosin
R21 HL 11184707/15/12-06/30/14$9,600
Cardiac Myosin Binding Protein-C: Structure, Function and Regulation
P01 HL 05940802/01/10-01/31/15$307,959
Proteotoxicity: An Unappreciated Mechanism of Heart Disease
10/01/11-09/30/16$247,636
Signaling Processes Underlying Cardiovascular Function
P01 HL 06977901/11/08-12/31/12$1,219,260

Schulz, E

Training in Cardiovascular Biology
T32 HL 00738202/01/13-01/31/14$22,904

Sengupta, A

FOXO Function in Cardiomyocytes During Development and Disease
11POST721002607/01/11-06/30/13$45,000

Tariq, M

Identification of Novel Human X-Linked Heterotaxy Genes
12POSTDOC1037000207/01/12-06/30/14$45,000

van Berlo, J

Functional Relevance and Extent of Endogenous Cardiac Regeneration by C-Kit Positive Stem Cells
K99 HL 11285206/04/12-03/31/17$121,660

Ware, S

Genetic and Epigenetic Mechanisms in Cardiomyopathy
13EIA1346000101/01/13-12/31/17$72,727
Genetic Registry for Pediatric Heart Disease: The CCVM Consortium
06/01/13-05/31/16$65,509
Genotype-Phenotype Association in Pediatric Cardiomyopathy
R01 HL 11145904/01/12-03/31/16$987,826
Uncovering Novel Genetic Causes and Risk in Congenital Heart Disease Patients
BWF #100849607/01/09-06/30/15$150,000

Waxman, J

Coup-tf Dependent Mechanisms of Ventricular and Hemangioblast Specification
R01 HL 11289301/15/13-02/28/18$225,000
Illumination of Mechanisms Controlling Atrial Cell Formation
5-FY11-8802/01/11-01/31/13$69,327

Wirrig, E

The Role of COX2 in the Progression of Human Calcific Aortic Valve Disease
F32 HL 11039007/01/12-06/30/15$52,190

Yutzey, K

Cell Signaling Mechanisms of Calcific Aortic Valve Disease
R01 HL 11468208/23/12-06/30/16$250,000
Student Undergraduate Research Fellowship
12UFEL999000002/01/12-01/31/14$20,000
Wnt Signaling in Heart Valve Development and Disease
R01 HL 09431904/15/12-02/28/16$238,000
Twist 1 Regulation of Valve Progenitors
R01 HL 08271607/01/2010-05/31/2015$235,620
Current Year Direct$6,919,459
Total$6,919,459