Division Data Summary

Research and Training Details

Number of Faculty47
Number of Research Fellows25
Number of Research Students14
Number of Support Personnel138
Direct Annual Grant Support$5,786,182
Direct Annual Industry Support$47,585
Peer Reviewed Publications155

Clinical Activities and Training

Number of Clinical Staff4
Number of Clinical Fellows12
Inpatient Encounters1,648
Outpatient Encounters14,231

Division Photo

Heart Institute.

Row 1: B Siegel, K Hor, C Chin, K Yutzey, J Towbin, J Robbins, H Heydarian

Row 2: S Goldstein, J Cnota, M Maillet, C Liu, B Goldstein, J Sticka, A Phillips, R Hirsch, P Manning, C Krawczeski, E Urbina, L Cripe, J James, E Purevjav

Row 3: J Waxman, M Taylor, J Jefferies, Z Khuchua, S Ware, J Molkentin, R Spicer, J Gulick, DW Benson, R Czosek, R Hinton, E Michelfelder, D Cooper, H Ippisch


Significant Publications

Significant Accomplishments

Advanced Heart Failure and Mechanical Circulatory Support Service

The Heart Institute launched the advanced heart failure and mechanical circulatory support service this year to extend the cutting-edge medical therapies we provide to patients of all ages.

A large component of any successful advanced heart failure program is providing mechanical circulatory support specifically in the form of ventricular assist devices (VADs.) Without such devices, many patients who develop end-stage disease would die. We implanted 10 Berlin EXCOR devices within the last year in patients from neonates to adults.

Creating an Environment that Matches Our Level of Care

An extensive renovation of our outpatient clinic area created a more welcoming environment for patients and families. We more than doubled the clinic area to more than 40,000 square feet, adding 16 patient care rooms, separate MRI and echo reading rooms, and separate spaces for pediatric and adult patients with congenital heart disease. New technology allows cardiologists to remotely supervise all echocardiograms.

The new interior features the heart-themed artwork of internationally-known artist Mackenzie Thorpe.  Last fall, Thorpe conducted an art workshop with patients and they co-created the sculpture that resides in our pediatric waiting area.  

Quality Improvement and Research

Our quality improvement and clinical excellence program implemented an “Executive Dashboard” of crucial measures including safety and quality, family satisfaction, clinical functions and resource use.  This dashboard has allowed the Heart Institute to review its status quickly and efficiently with others and has become a model for other institutes and divisions within the hospital. 

In cardiac research, faculty findings published October 2010 in the Journal of the American College of Cardiology showed that mutations in the nebulette protein isoform is associated with lethal cardiac structural abnormalities, including dilated cardiomyopathy and endocardial fibroelastosis. Study authors included Enkhsaikhan Purevjav, MD, PhD, Michael Taylor, MD, PhD, and Jeffrey Towbin, MD.

Our work in molecular cardiovascular biology also progressed significantly this year. Jeffrey Molkentin, PhD, studies intracellular signaling pathways and transcriptional regulatory circuits that control mammalian cell growth and differentiation. Jeffrey Robbins, PhD, established the means to direct the heart to synthesize normal and mutant proteins and to turn these on and off at will. This allows scientists to establish cause-and-effect relationships between mutant proteins and the development of cardiac disease. Stephanie Ware, MD, PhD, continued work with X-linked heterotaxy (HTX-1, MIM 306955), a rare developmental disorder characterized by disturbances in embryonic laterality and other midline developmental defects. Ware uses this genetic defect to study the underpinnings of early axis formation and its importance for cardiogenesis.

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Division Publications

  1. 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. The Journal of biological chemistry. 2011; 286:899-908.
  2. 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. The Journal of pediatrics. 2010; 157:407-13, 413 e1.
  3. Anderson JB, Benson DW. Genetics of sick sinus syndrome. Cardiac electrophysiology clinics. 2010; 2:499-507.
  4. Anderson JB, Kalkwarf HJ, Kehl JE, Eghtesady P, Marino BS. Low weight-for-age z-score and infection risk after the Fontan procedure. The Annals of thoracic surgery. 2011; 91:1460-6.
  5. Atz AM, Travison TG, Williams IA, Pearson GD, Laussen PC, Mahle WT, Cook AL, Kirsh JA, Sklansky M, Khaikin S, Goldberg C, Frommelt M, Krawczeski C, Puchalski MD, Jacobs JP, Baffa JM, Rychik J, Ohye RG. Prenatal diagnosis and risk factors for preoperative death in neonates with single right ventricle and systemic outflow obstruction: screening data from the Pediatric Heart Network Single Ventricle Reconstruction Trial( *). The Journal of thoracic and cardiovascular surgery. 2010; 140:1245-50.
  6. Baker-Smith CM, Neish SR, Klitzner TS, Beekman RH, 3rd, Kugler JD, Martin GR, Lannon C, Jenkins KJ, Rosenthal GL. Variation in postoperative care following stage I palliation for single-ventricle patients: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative. Congenital heart disease. 2011; 6:116-27.
  7. Banchs J, Jefferies JL, Plana JC, Hundley WG. Imaging for cardiotoxicity in cancer patients. Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital. 2011; 38:268-9.
  8. Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, Gatzoulis MA, Gohlke-Baerwolf C, Kaemmerer H, Kilner P, Meijboom F, Mulder BJ, Oechslin E, Oliver JM, Serraf A, Szatmari A, Thaulow E, Vouhe PR, Walma E, Vahanian A, Auricchio A, Bax J, Ceconi C, Dean V, Filippatos G, Funck-Brentano C, Hobbs R, Kearney P, McDonagh T, Popescu BA, Reiner Z, Sechtem U, Sirnes PA, Tendera M, Vardas P, Widimsky P, Swan L, Andreotti F, Beghetti M, Borggrefe M, Bozio A, Brecker S, Budts W, Hess J, Hirsch R, Jondeau G, Kokkonen J, Kozelj M, Kucukoglu S, Laan M, Lionis C, Metreveli I, Moons P, Pieper PG, Pilossoff V, Popelova J, Price S, Roos-Hesselink J, Uva MS, Tornos P, Trindade PT, Ukkonen H, Walker H, Webb GD, Westby J. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). European heart journal. 2010; 31:2915-57.
  9. Beery TA, Smith CR, Kudel I, Knilans T. Measuring sports participation decisional conflict in youth with cardiac pacemakers and/or ICDs. Journal of advanced nursing. 2011; 67:821-8.
  10. Belmont JW, Craigen W, Martinez H, Jefferies JL. Genetic disorders with both hearing loss and cardiovascular abnormalities. Advances in oto-rhino-laryngology. 2011; 70:66-74.
  11. Bergersen L, Gauvreau K, Marshall A, Kreutzer J, Beekman R, Hirsch R, Foerster S, Balzer D, Vincent J, Hellenbrand W, Holzer R, Cheatham J, Moore J, Lock J, Jenkins K. Procedure-type risk categories for pediatric and congenital cardiac catheterization. Circulation. Cardiovascular interventions. 2011; 4:188-94.
  12. 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. Paediatric anaesthesia. 2010; 20:748-56.
  13. Bolli R, Stein AB, Guo Y, Wang OL, Rokosh G, Dawn B, Molkentin JD, Sanganalmath SK, Zhu Y, Xuan YT. A murine model of inducible, cardiac-specific deletion of STAT3: its use to determine the role of STAT3 in the upregulation of cardioprotective proteins by ischemic preconditioning. Journal of molecular and cellular cardiology. 2011; 50:589-97.
  14. 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. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation. 2010; 29:739-46.
  15. Breitbart A, Auger-Messier M, Molkentin JD, Heineke J. Myostatin from the heart: local and systemic actions in cardiac failure and muscle wasting. American journal of physiology. Heart and circulatory physiology. 2011; 300:H1973-82.
  16. Broberg CS, Aboulhosn J, Mongeon FP, Kay J, Valente AM, Khairy P, Earing MG, Opotowsky AR, Lui G, Gersony DR, Cook S, Ting JG, Webb G, Gurvitz MZ. Prevalence of left ventricular systolic dysfunction in adults with repaired tetralogy of fallot. The American journal of cardiology. 2011; 107:1215-20.
  17. Brown DW, Connor JA, Pigula FA, Usmani K, Klitzner TS, Beekman RH, 3rd, Kugler JD, Martin GR, Neish SR, Rosenthal GL, Lannon C, Jenkins KJ. Variation in preoperative and intraoperative care for first-stage palliation of single-ventricle heart disease: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative. Congenital heart disease. 2011; 6:108-15.
  18. Brunetti-Pierri N, Pignatelli R, Fouladi N, Towbin JA, Belmont JW, Craigen WJ, Wong LJ, Jefferies JL, Scaglia F. Dilation of the aortic root in mitochondrial disease patients. Molecular genetics and metabolism. 2011; 103:167-70.
  19. Buber J, Mathew J, Moss AJ, Hall WJ, Barsheshet A, McNitt S, Robinson JL, Zareba W, Ackerman MJ, Kaufman ES, Luria D, Eldar M, Towbin JA, Vincent M, Goldenberg I. Risk of Recurrent Cardiac Events After Onset of Menopause in Women With Congenital Long-QT Syndrome Types 1 and 2. Circulation. 2011; 123:2784-91.
  20. 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. American journal of medical genetics. Part A. 2011; 155A:1015-20.
  21. Carlo WF, Nelson DP. Inhaled nitric oxide improves oxygen saturation in children with pulmonary arteriovenous malformations after the Fontan procedure. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. 2011; 12:e152-4.
  22. Chakraborty S, Wirrig EE, Hinton RB, Merrill WH, Spicer DB, Yutzey KE. Twist1 promotes heart valve cell proliferation and extracellular matrix gene expression during development in vivo and is expressed in human diseased aortic valves. Developmental biology. 2010; 347:167-79.
  23. Ch'en IL, Tsau JS, Molkentin JD, Komatsu M, Hedrick SM. Mechanisms of necroptosis in T cells. The Journal of experimental medicine. 2011; 208:633-41.
  24. Chen X, Nakayama H, Zhang X, Ai X, Harris DM, Tang M, Zhang H, Szeto C, Stockbower K, Berretta RM, Eckhart AD, Koch WJ, Molkentin JD, Houser SR. Calcium influx through Cav1.2 is a proximal signal for pathological cardiomyocyte hypertrophy. Journal of molecular and cellular cardiology. 2011; 50:460-70.
  25. Chio SS, Urbina EM, Lapointe J, Tsai J, Berenson GS. Korotkoff sound versus oscillometric cuff sphygmomanometers: comparison between auscultatory and DynaPulse blood pressure measurements. Journal of the American Society of Hypertension : JASH. 2011; 5:12-20.
  26. Combs MD, Braitsch CM, Lange AW, James JF, Yutzey KE. NFATC1 promotes epicardium-derived cell invasion into myocardium. Development. 2011; 138:1747-57.
  27. Costanzo MR, Dipchand A, Starling R, Anderson A, Chan M, Desai S, Fedson S, Fisher P, Gonzales-Stawinski G, Martinelli L, McGiffin D, Smith J, Taylor D, Meiser B, Webber S, Baran D, Carboni M, Dengler T, Feldman D, Frigerio M, Kfoury A, Kim D, Kobashigawa J, Shullo M, Stehlik J, Teuteberg J, Uber P, Zuckermann A, Hunt S, Burch M, Bhat G, Canter C, Chinnock R, Crespo-Leiro M, Delgado R, Dobbels F, Grady K, Kao W, Lamour J, Parry G, Patel J, Pini D, Towbin J, Wolfel G, Delgado D, Eisen H, Goldberg L, Hosenpud J, Johnson M, Keogh A, Lewis C, O'Connell J, Rogers J, Ross H, Russell S, Vanhaecke J. The International Society of Heart and Lung Transplantation Guidelines for the care of heart transplant recipients. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation. 2010; 29:914-56.
  28. 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. American journal of obstetrics and gynecology. 2010; 203:397 e1-9.
  29. 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. The Journal of pediatrics. 2011; 158:709-714 e1.
  30. Cuneo BF, Fruitman D, Benson DW, Ngan BY, Liske MR, Wahren-Herlineus M, Ho SY, Jaeggi E. Spontaneous rupture of atrioventricular valve tensor apparatus as late manifestation of anti-Ro/SSA antibody-mediated cardiac disease. The American journal of cardiology. 2011; 107:761-6.
  31. Cuneo BF, Lee M, Roberson D, Niksch A, Ovadia M, Parilla BV, Benson DW. A management strategy for fetal immune-mediated atrioventricular block. The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians. 2010; 23:1400-5.
  32. Czosek RJ, Anderson J, Connor C, Knilans T. Nodoventricular pathway associated with twin AV nodes: complexity of ablation in single ventricle physiology. Journal of cardiovascular electrophysiology. 2010; 21:936-9.
  33. 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 and clinical electrophysiology : PACE. 2010; 33:1304-11.
  34. Czosek RJ, Anderson JB, Cao J, Knilans TK. Assessment of T-wave oversensing in an infant with an implanted defibrillator. Heart rhythm : the official journal of the Heart Rhythm Society. 2010; 7:1516-7.
  35. Czosek RJ, Anderson JB, Marino BS, Mellion K, Knilans TK. Noninvasive risk stratification techniques in pediatric patients with ventricular preexcitation. Pacing and clinical electrophysiology : PACE. 2011; 34:555-62.
  36. 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. Cardiology in the young. 2010; 20:516-21.
  37. Czosek RJ, Haaning A, Ware SM. A mouse model of conduction system patterning abnormalities in heterotaxy syndrome. Pediatric research. 2010; 68:275-80.
  38. Das A, Banerjee RK, Gottliebson WM. Right ventricular inefficiency in repaired tetralogy of Fallot: proof of concept for energy calculations from cardiac MRI data. Annals of biomedical engineering. 2010; 38:3674-87.
  39. Das A, Gottliebson WM, Karve M, Banerjee R. Comparison of hemodynamic endpoints between normal subject and tetralogy patient using Womersley velocity profile and MR based flow measurements. Molecular & cellular biomechanics : MCB. 2011; 8:21-42.
  40. Devarajan P, Krawczeski CD, Nguyen MT, Kathman T, Wang Z, Parikh CR. Proteomic identification of early biomarkers of acute kidney injury after cardiac surgery in children. American journal of kidney diseases : the official journal of the National Kidney Foundation. 2010; 56:632-42.
  41. Divanovic 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. The Journal of thoracic and cardiovascular surgery. 2011; 141:988-94.
  42. Duffy JY, Petrucci O, Baker RS, Lam CT, Reed CA, Everman DJ, Eghtesady P. Myocardial function after fetal cardiac bypass in an ovine model. The Journal of thoracic and cardiovascular surgery. 2011; 141:961-8, 968 e1.
  43. Eder P, Molkentin JD. TRPC channels as effectors of cardiac hypertrophy. Circulation research. 2011; 108:265-72.
  44. Eghtesady P, Brar A, Hall M. Seasonality of hypoplastic left heart syndrome in the United States: a 10-year time-series analysis. The Journal of thoracic and cardiovascular surgery. 2011; 141:432-8.
  45. 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. The Journal of thoracic and cardiovascular surgery. 2011; 141:835-7.
  46. 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. The Journal of clinical investigation. 2010; 120:3680-7.
  47. 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. Proceedings of the National Academy of Sciences of the United States of America. 2011; 108:2915-20.
  48. Feltes TF, Bacha E, Beekman RH, 3rd, Cheatham JP, Feinstein JA, Gomes AS, Hijazi ZM, Ing FF, de Moor M, Morrow WR, Mullins CE, Taubert KA, Zahn EM. Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease: A Scientific Statement From the American Heart Association. Circulation. 2011; 123:2607-2652.
  49. 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. Circulation. Heart failure. 2010; 3:689-97.
  50. Force T, Bonow RO, Houser SR, Solaro RJ, Hershberger RE, Adhikari B, Anderson ME, Boineau R, Byrne BJ, Cappola TP, Kalluri R, LeWinter MM, Maron MS, Molkentin JD, Ommen SR, Regnier M, Tang WH, Tian R, Konstam MA, Maron BJ, Seidman CE. Research priorities in hypertrophic cardiomyopathy: report of a Working Group of the National Heart, Lung, and Blood Institute. Circulation. 2010; 122:1130-3.
  51. Froese N, Kattih B, Breitbart A, Grund A, Geffers R, Molkentin JD, Kispert A, Wollert KC, Drexler H, Heineke J. GATA6 promotes angiogenic function and survival in endothelial cells by suppression of autocrine transforming growth factor beta/activin receptor-like kinase 5 signaling. The Journal of biological chemistry. 2011; 286:5680-90.
  52. Gilio K, Harper MT, Cosemans JM, Konopatskaya O, Munnix IC, Prinzen L, Leitges M, Liu Q, Molkentin JD, Heemskerk JW, Poole AW. Functional divergence of platelet protein kinase C (PKC) isoforms in thrombus formation on collagen. The Journal of biological chemistry. 2010; 285:23410-9.
  53. Goldberg DJ, French B, McBride MG, Marino BS, Mirarchi N, Hanna BD, Wernovsky G, Paridon SM, Rychik J. Impact of oral sildenafil on exercise performance in children and young adults after the fontan operation: a randomized, double-blind, placebo-controlled, crossover trial. Circulation. 2011; 123:1185-93.
  54. 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. Journal of the American College of Cardiology. 2011; 57:51-9.
  55. Goonasekera SA, Lam CK, Millay DP, Sargent MA, Hajjar RJ, Kranias EG, Molkentin JD. Mitigation of muscular dystrophy in mice by SERCA overexpression in skeletal muscle. The Journal of clinical investigation. 2011; 121:1044-52.
  56. Gorbea C, Makar KA, Pauschinger M, Pratt G, Bersola JL, Varela J, David RM, Banks L, Huang CH, Li H, Schultheiss HP, Towbin JA, Vallejo JG, Bowles NE. A role for Toll-like receptor 3 variants in host susceptibility to enteroviral myocarditis and dilated cardiomyopathy. The Journal of biological chemistry. 2010; 285:23208-23.
  57. Haase M, Devarajan P, Haase-Fielitz A, Bellomo R, Cruz DN, Wagener G, Krawczeski CD, Koyner JL, Murray P, Zappitelli M, Goldstein SL, Makris K, Ronco C, Martensson J, Martling CR, Venge P, Siew E, Ware LB, Ikizler TA, Mertens PR. The outcome of neutrophil gelatinase-associated lipocalin-positive subclinical acute kidney injury: a multicenter pooled analysis of prospective studies. Journal of the American College of Cardiology. 2011; 57:1752-61.
  58. 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. American journal of obstetrics and gynecology. 2010; 203:404 e1-7.
  59. Harper MT, Molkentin JD, Poole AW. Protein kinase C alpha enhances sodium-calcium exchange during store-operated calcium entry in mouse platelets. Cell calcium. 2010; 48:333-40.
  60. 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. Nature medicine. 2010; 16:872-9.
  61. 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. Circulation research. 2010; 107:549-57.
  62. 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. The Journal of pediatrics. 2010; 157:164-6.
  63. Hinton RB, Yutzey KE. Heart valve structure and function in development and disease. Annual review of physiology. 2011; 73:29-46.
  64. Hirsch R, Bagby MC, Zussman ME. Fenestrated ASD closure in a child with idiopathic pulmonary hypertension and exercise desaturation. Congenital heart disease. 2011; 6:162-6.
  65. Hor KN, Baumann R, Pedrizzetti G, Tonti G, Gottliebson WM, Taylor M, Benson W, Mazur W. Magnetic resonance derived myocardial strain assessment using feature tracking. Journal of visualized experiments : JoVE. 2011; .
  66. Hor KN, Wansapura JP, Al-Khalidi HR, Gottliebson WM, Taylor MD, Czosek RJ, Nagueh SF, Akula N, Chung ES, Benson WD, Mazur W. Presence of mechanical dyssynchrony in Duchenne muscular dystrophy. Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance. 2011; 13:12.
  67. Jain VD, Moghbeli N, Webb G, Srinivas SK, Elovitz MA, Pare E. Pregnancy in women with congenital heart disease: the impact of a systemic right ventricle. Congenital heart disease. 2011; 6:147-56.
  68. James J, Robbins J. Signaling and myosin-binding protein C. The Journal of biological chemistry. 2011; 286:9913-9.
  69. Jefferies JL, Hoffman TM, Nelson DP. Heart failure treatment in the intensive care unit in children. Heart failure clinics. 2010; 6:531-58, ix-x.
  70. Jefferies JL, Price JF, Morales DL. Mechanical support in childhood heart failure. Heart failure clinics. 2010; 6:559-73, x.
  71. Johnson JN, Ansong AK, Li JS, Xu M, Gorentz J, Hehir DA, del Castillo SL, Lai WW, Uzark K, Pasquali SK. Celiac artery flow pattern in infants with single right ventricle following the Norwood procedure with a modified Blalock-Taussig or right ventricle to pulmonary artery shunt. Pediatric cardiology. 2011; 32:479-86.
  72. Kehat I, Accornero F, Aronow BJ, Molkentin JD. Modulation of chromatin position and gene expression by HDAC4 interaction with nucleoporins. The Journal of cell biology. 2011; 193:21-9.
  73. 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. Circulation research. 2011; 108:176-83.
  74. Kehat I, Molkentin JD. Molecular pathways underlying cardiac remodeling during pathophysiological stimulation. Circulation. 2010; 122:2727-35.
  75. 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. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010; 122:868-75.
  76. 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. American journal of roentgenology. 2011; 196:454-60.
  77. Kim JA, Lopes CM, Moss AJ, McNitt S, Barsheshet A, Robinson JL, Zareba W, Ackerman MJ, Kaufman ES, Towbin JA, Vincent M, Goldenberg I. Trigger-specific risk factors and response to therapy in long QT syndrome type 2. Heart rhythm : the official journal of the Heart Rhythm Society. 2010; 7:1797-805.
  78. Kleinman ME, Chameides L, Schexnayder SM, Samson RA, Hazinski MF, Atkins DL, Berg MD, de Caen AR, Fink EL, Freid EB, Hickey RW, Marino BS, Nadkarni VM, Proctor LT, Qureshi FA, Sartorelli K, Topjian A, van der Jagt EW, Zaritsky AL. Part 14: pediatric advanced life support: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Circulation. 2010; 122:S876-908.
  79. Kleinman ME, Chameides L, Schexnayder SM, Samson RA, Hazinski MF, Atkins DL, Berg MD, de Caen AR, Fink EL, Freid EB, Hickey RW, Marino BS, Nadkarni VM, Proctor LT, Qureshi FA, Sartorelli K, Topjian A, van der Jagt EW, Zaritsky AL. Pediatric advanced life support: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Pediatrics. 2010; 126:e1361-99.
  80. Knilans TK, Carboni MP, Czosek RJ, Kanter RJ. Arrhythmias in Children and in Patients with Congenital Heart Disease. Hurst's the heart. New York: McGraw-Hill Medical; 2011.
  81. Korf-Klingebiel M, Kempf T, Schluter KD, Willenbockel C, Brod T, Heineke J, Schmidt VJ, Jantzen F, Brandes RP, Sugden PH, Drexler H, Molkentin JD, Wollert KC. Conditional transgenic expression of fibroblast growth factor 9 in the adult mouse heart reduces heart failure mortality after myocardial infarction. Circulation. 2011; 123:504-14.
  82. 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. Clinical journal of the American Society of Nephrology : CJASN. 2010; 5:1552-7.
  83. 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. The Journal of pediatrics. 2011; 158:1009-1015 e1.
  84. Krieger EV, Landzberg MJ, Economy KE, Webb GD, Opotowsky AR. Comparison of risk of hypertensive complications of pregnancy among women with versus without coarctation of the aorta. The American journal of cardiology. 2011; 107:1529-34.
  85. Krishnamurthy VK, Guilak F, Narmoneva DA, Hinton RB. Regional structure-function relationships in mouse aortic valve tissue. Journal of biomechanics. 2011; 44:77-83.
  86. Lai WW, Vetter VL, Richmond M, Li JS, Saul JP, Mital S, Colan SD, Newburger JW, Sleeper LA, McCrindle BW, Minich LL, Goldmuntz E, Marino BS, Williams IA, Pearson GD, Evans F, Scott JD, Cohen MS. Clinical research careers: reports from a NHLBI pediatric heart network clinical research skills development conference. American heart journal. 2011; 161:13-67.
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  89. Li Z, Ai T, Samani K, Xi Y, Tzeng HP, Xie M, Wu S, Ge S, Taylor MD, Dong JW, Cheng J, Ackerman MJ, Kimura A, Sinagra G, Brunelli L, Faulkner G, Vatta M. A ZASP missense mutation, S196L, leads to cytoskeletal and electrical abnormalities in a mouse model of cardiomyopathy. Circulation. Arrhythmia and electrophysiology. 2010; 3:646-56.
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  102. Meissner M, Weissgerber P, Londono JE, Prenen J, Link S, Ruppenthal S, Molkentin JD, Lipp P, Nilius B, Freichel M, Flockerzi V. Moderate calcium channel dysfunction in adult mice with inducible cardiomyocyte-specific excision of the cacnb2 gene. The Journal of biological chemistry. 2011; 286:15875-82.
  103. Merveille AC, Davis EE, Becker-Heck A, Legendre M, Amirav I, Bataille G, Belmont J, Beydon N, Billen F, Clement A, Clercx C, Coste A, Crosbie R, de Blic J, Deleuze S, Duquesnoy P, Escalier D, Escudier E, Fliegauf M, Horvath J, Hill K, Jorissen M, Just J, Kispert A, Lathrop M, Loges NT, Marthin JK, Momozawa Y, Montantin G, Nielsen KG, Olbrich H, Papon JF, Rayet I, Roger G, Schmidts M, Tenreiro H, Towbin JA, Zelenika D, Zentgraf H, Georges M, Lequarre AS, Katsanis N, Omran H, Amselem S. CCDC39 is required for assembly of inner dynein arms and the dynein regulatory complex and for normal ciliary motility in humans and dogs. Nature genetics. 2011; 43:72-8.
  104. Mital S, Chung WK, Colan SD, Sleeper LA, Manlhiot C, Arrington CB, Cnota JF, Graham EM, Mitchell ME, Goldmuntz E, Li JS, Levine JC, Lee TM, Margossian R, Hsu DT. Renin-Angiotensin-Aldosterone Genotype Influences Ventricular Remodeling in Infants With Single Ventricle. Circulation. 2011; 123:2353-2362.
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  109. Opotowsky AR, Shellenberger D, Dharan V, Pare E, Norwood WI, Webb GD, Donner R. Successful pregnancies in two women with hypoplastic left heart syndrome. Congenital heart disease. 2010; 5:476-81.
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  111. Petrucci O, Baker RS, Lam CT, Reed CA, Duffy JY, Eghtesady P. Fetal right ventricular myocardial function is better preserved by fibrillatory arrest during fetal cardiac bypass. The Annals of thoracic surgery. 2010; 90:1324-31.
  112. 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. Journal of the American College of Cardiology. 2010; 56:1493-502.
  113. Qian L, Wythe JD, Liu J, Cartry J, Vogler G, Mohapatra B, Otway RT, Huang Y, King IN, Maillet M, Zheng Y, Crawley T, Taghli-Lamallem O, Semsarian C, Dunwoodie S, Winlaw D, Harvey RP, Fatkin D, Towbin JA, Molkentin JD, Srivastava D, Ocorr K, Bruneau BG, Bodmer R. Tinman/Nkx2-5 acts via miR-1 and upstream of Cdc42 to regulate heart function across species. The Journal of cell biology. 2011; 193:1181-96.
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  115. Sable C, Foster E, Uzark K, Bjornsen K, Canobbio MM, Connolly HM, Graham TP, Gurvitz MZ, Kovacs A, Meadows AK, Reid GJ, Reiss JG, Rosenbaum KN, Sagerman PJ, Saidi A, Schonberg R, Shah S, Tong E, Williams RG. Best practices in managing transition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a scientific statement from the American Heart Association. Circulation. 2011; 123:1454-85.
  116. Schidlow DN, Anderson JB, Klitzner TS, Beekman RH, 3rd, Jenkins KJ, Kugler JD, Martin GR, Neish SR, Rosenthal GL, Lannon C. Variation in interstage outpatient care after the Norwood procedure: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative. Congenital heart disease. 2011; 6:98-107.
  117. Sengupta A, Molkentin JD, Paik JH, DePinho RA, Yutzey KE. FoxO transcription factors promote cardiomyocyte survival upon induction of oxidative stress. The Journal of biological chemistry. 2011; 286:7468-78.
  118. 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; 127:e312-8.
  119. 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. 2011; 54:722-30.
  120. 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. Molecular genetics and metabolism. 2010; 101:282-5.
  121. Shuhaiber J, Hirsch R. Pulmonary rehabilitation following unifocalization: the role of hybrid approach. Pediatric cardiology. 2011; 32:667-9.
  122. Shuhaiber J, Rehman M, Jenkins K, Fynn-Thompson F, Bacha E. The role of surgical therapy for pulmonary vein atresia in childhood. Pediatric cardiology. 2011; 32:639-45.
  123. Shuhaiber J, Thiagarajan RR, Laussen PC, Fynn-Thompson F, Del Nido P, Pigula F. Survival of children requiring repeat extracorporeal membrane oxygenation after congenital heart surgery. The Annals of thoracic surgery. 2011; 91:1949-55.
  124. Shuhaiber JH, Bergersen L, Pigula F, Emani S, McElhinney D, Marshall A, Fynn-Thompson F, Bacha EA. Intraoperative assessment after pediatric cardiac surgical repair: initial experience with C-arm angiography. The Journal of thoracic and cardiovascular surgery. 2010; 140:e1-3.
  125. Siegel RM, Neidhard MS, Kirk S. A comparison of low glycemic index and staged portion-controlled diets in improving BMI of obese children in a pediatric weight management program. Clinical pediatrics. 2011; 50:459-61.
  126. Spicer RL, Ware SM. Diseases of the Myocardium. Nelson textbook of pediatrics. Philadelphia, PA: Elsevier/Saunders; 2011: 1 online resource (p.). .
  127. Spicer RL, Ware SM. Diseases of the Pericardium. Nelson textbook of pediatrics. Philadelphia, PA: Elsevier/Saunders; 2011: 1 online resource (p.). .
  128. Spicer RL, Ware SM. Tumors of the Heart. Nelson textbook of pediatrics. Philadelphia, PA: Elsevier/Saunders; 2011: 1 online resource (p.). .
  129. Stanley BA, Graham DR, James J, Mitsak M, Tarwater PM, Robbins J, Van Eyk JE. Altered myofilament stoichiometry in response to heart failure in a cardioprotective alpha-myosin heavy chain transgenic rabbit model. Proteomics. Clinical applications. 2011; 5:147-58.
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  133. Sun T, Wu XS, Xu J, McNeil BD, Pang ZP, Yang W, Bai L, Qadri S, Molkentin JD, Yue DT, Wu LG. The role of calcium/calmodulin-activated calcineurin in rapid and slow endocytosis at central synapses. The Journal of neuroscience : the official journal of the Society for Neuroscience. 2010; 30:11838-47.
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Grants, Contracts, and Industry Agreements

Grant and Contract AwardsAnnual Direct / Project Period Direct

Anderson, J

Nutritional Deficiencies in Infants with Single Ventricle
10/01/10-09/30/12$12,500

Benson , W

Musculoskeletal Ancillary Study
07/01/10-06/30/11$3,750
Quality of Life Study-Pediatric Heart Network
U01 HL 06827007/01/10-06/30/11$500
Trial of Beta Blocker vs Angiotensin Receptor Blocker
U01 HL 06827007/01/10-06/30/11$72,010

Braitsch, C

Tbx18 Regulation of Epicardial-Derived Cell Proliferation, Migration and Differentiation in Cardiac Development
07/01/09-06/30/11$23,000

Cole, C

Myocardial Protection during Fetal Bypass: Role of Calcium Cycling
F32 HL 10305407/01/10-06/30/12$54,254

Correll, R

Regulation of Cardiac Gene Expression
F32 HL 09755109/07/09-09/06/12$50,474

Davis, J

The Non-Hypertrophic Role of Calcineurin in Regulating Cardiac Structure-Function
F32 HL 09535312/15/08-12/14/11$52,154

Eghtesady, P

Hypoplastic Left Heart Syndrome: Expression of RHD in the Fetus?
R01 HL 09863401/15/10-12/31/14$269,164

Ippisch, H

Effects of Dietary Fat and Exercise Challenges in Obese Children Effects of Dietary Fat and Exercise Challenges in Obese Children
K23 HL 09117409/01/08-07/31/13$126,085

Karch, J

The Role of Bax and Bak in Necrotic Cell Death
07/01/10-06/30/12$23,000

Khuchua, Z

The shRNA-Mediated Tafazzin Knockdown Mouse Model
01/01/10-12/31/11$16,361

Kimball, T

Chronic Kidney Disease in Children
U01 DK 06614308/01/08-07/31/13$55,222

Krishnamurthy, V

Valve Tissue Mechanics and Cell Phenotype in a Mouse Model of Aortic Valve Disease
07/01/09-06/30/11$23,000

Maillet, M

Role of IP-3 Mediated Calcium Release in Cardiac Hypertrophic Cardiomyopathy
R21 HL 09718604/15/10-03/31/12$125,000

Marino, B

Understanding Mechanisms of Fontan Failure and Key Predictors of Patient Outcome
R01 HL 09825202/01/10-01/31/14$18,488

Molkentin, J

Calcium as a Molecular Signal in the Heart
R01 HL 08931208/15/07-06/30/12$239,303
Cardiac Hypertrophic Intracellular Signaling Pathways
R01 HL 06292702/01/09-12/31/13$250,000
Molecular Pathways Controlling Cardiac Gene Expression
R37 HL 06056207/01/08-06/30/13$250,000
Thrombospondin 4 Regulates Adaptive ER Stress Response
R01 HL 10592401/01/11-12/31/11$315,000

Razzaque, A

Cardiomyopathic Mechanisms in Pediatric Congenital Disease
07/01/10-06/30/12$43,000

Robbins, J

Cardiac Myosin Binding Protein-C: Structure, Function and Regulation
P01 HL 05940802/01/10-01/31/15$356,105
Nikon A1 Confocal Microscope
S10 RR 02701407/01/10-06/30/11$388,205
Signaling Processes Underlying Cardiovascular Function
P01 HL 06977901/11/08-12/31/12$1,219,260

Sengupta, A

URC Postdoctoral Fellow Research Grant
01/01/11-12/31/11$5,000

Siegel, R

Collaborative Community Based Initiative to Improve the Health of Overweight and Obese Children
01/01/11-12/31/11$76,223

Urbina, E

Accelerated CV Aging in Youth Related to CV Risk Factor Clusters
R01 HL 10559101/01/11-12/31/15$383,528
Modifying Dietary Behavior in Adolescents with Elevated Blood Pressure
R01HL08856702/01/11-01/31/12$37,818

van Berlo, J

GATA-6 Function is Crucial for Cardiac Hypertrophy to Prevent Heart Failure
07/01/10-06/30/12$43,000

Ware, S

Role of the Embryonic Node in Cardiac Development and Congenital Heart Disease
R01 HL 08863904/01/07-03/31/12$250,000
Uncovering Novel Genetic Causes and Risk in Congenital Heart Disease Patients
100849607/01/09-06/30/14$75,000
Genetic Causes of Congenital Heart Defects
1FY1040106/01/10-05/31/13$93,866

Waxman, J

Elucidation of Molecular Networks Required to Limit Cardiac Cell Number
R00 HL 09112607/15/10-05/31/13$163,368
Illumination of Mechanisms Controlling Atrial Cell Formation
02/01/11-01/31/13$69,327

Yutzey, K

Notch Signaling in Heart Valve Development and Disease
R01 HL 09431907/01/09-06/30/11$308,217
The Function of Notch1 in Heart Valve Development
07/01/09-06/30/11$23,000
Twist 1 Regulation of Valve Progenitors
R01 HL 08271607/01/10-05/31/15$250,000
Weinstein Cardiovascular Development Conference 2011
04/01/11-09/30/11$2,000
Student Undergraduate Research Fellowships
10UFEL358001606/01/10-05/31/12$20,000
Current Year Direct$5,786,182
Industry Contracts

Beekman. R

$4,177

Hirsch. R

$12,199

Krawczeski. C

$26,208

Yutzey, K

$5,000
Current Year Direct Receipts$47,585
Total$5,833,767
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