2015 Research Annual Report

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Heart InstituteRESEARCH AND TRAINING DETAILS

Faculty 67

Joint Appointment Faculty 2

Research Fellows 33

Research Students 24

Support Personnel 146

Direct Annual Grant Support $11,301,852

Direct Annual Industry Support $227,476

Peer Reviewed Publications 194

CLINICAL ACTIVITIES AND TRAINING

Clinical Staff 187

Staff Physicians 2

Clinical Fellows 21

Other Students 4

Inpatient Encounters 1,972

Outpatient Encounters 19,397

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Division Publications

Division Publications1. Accornero F, Kanisicak O, Tjondrokoesoemo A, Attia AC, McNally EM, Molkentin JD. Myofiber­specific inhibition of

TGFbeta signaling protects skeletal muscle from injury and dystrophic disease in mice. Hum Mol Genet. 2014;23:6903­15.

2. Accornero F, van Berlo JH, Correll RN, Elrod JW, Sargent MA, York A, Rabinowitz JE, Leask A, Molkentin JD.

Genetic Analysis of Connective Tissue Growth Factor as an Effector of Transforming Growth Factor betaSignaling and Cardiac Remodeling. Mol Cell Biol. 2015; 35:2154­64.

3. Adachi I, Khan MS, Guzman­Pruneda FA, Fraser CD, 3rd, Mery CM, Denfield SW, Dreyer WJ, Morales DL, McKenzie

ED, Heinle JS, Fraser CD, Jr.. Evolution and impact of ventricular assist device program on children awaitingheart transplantation. Ann Thorac Surg. 2015; 99:635­40.

4. Adachi I, Morales DS. Implantation of total artificial heart in congenital heart disease. J Vis Exp. 2014; .

5. Aiyagari R, Rhodes JF, Shrader P, Radtke WA, Bandisode VM, Bergersen L, Gillespie MJ, Gray RG, Guey LT, Hill

KD, Hirsch R, Kim DW, Lee KJ, Pelech AN, Ringewald J, Takao C, Vincent JA, Ohye RG, Pediatric Heart Network I.

Impact of pre­stage II hemodynamics and pulmonary artery anatomy on 12­month outcomes in the PediatricHeart Network Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg. 2014; 148:1467­74.

6. Albrecht LV, Zhang L, Shabanowitz J, Purevjav E, Towbin JA, Hunt DF, Green KJ. GSK3­ and PRMT­1­dependentmodifications of desmoplakin control desmoplakin­cytoskeleton dynamics. J Cell Biol. 2015; 208:597­612.

7. Alman AC, Talton JW, Wadwa RP, Urbina EM, Dolan LM, Daniels SR, Hamman RF, D'Agostino RB, Marcovina SM,

Mayer­Davis EJ, Dabelea DM. Cardiovascular health in adolescents with type 1 diabetes: the SEARCH CVDstudy. Pediatr Diabetes. 2014; 15:502­10.

8. Alsaied T, Madueme PC. International medical graduates in cardiology fellowship: brain drain or brain gain?. JAm Coll Cardiol. 2015; 65:507­9; discussion 509­10.

9. Anderson JB, Beekman RH, 3rd. Addressing Nutrition and Growth in Children with Congenital Heart Disease. In:PR Barach, JP Jacobs, SE Lipshultz, PC Laussen, eds. Pediatric and Congenital Cardiac Care: Quality Improvement

and Patient Safety. London, UK: Springer; 2014:153­164.

10. Anderson JB, Beekman RH, 3rd, Kugler JD, Rosenthal GL, Jenkins KJ, Klitzner TS, Martin GR, Neish SR, Darbie L,

King E, Lannon C, National Pediatric Cardiology Quality Improvement C. Use of a learning network to improvevariation in interstage weight gain after the Norwood operation. Congenit Heart Dis. 2014; 9:512­20.

11. Anderson JB, Beekman RH, 3rd, Martin GR, Lannon C. Quality Improvement in Pediatric Cardiology: The NationalPediatric Cardiology Quality Improvement Collaborative. In: PR Barach, JP Jacobs, SE Lipshultz, PC Laussen,eds. Pediatric and Congenital Cardiac Care: Quality Improvement and Patient Safety. London, UK: Springer;

2014:51­68.

12. Anderson JB, Grenier M, Edwards NM, Madsen NL, Czosek RJ, Spar DS, Barnes A, Pratt J, King E, Knilans TK.

Usefulness of combined history, physical examination, electrocardiogram, and limited echocardiogram inscreening adolescent athletes for risk for sudden cardiac death. Am J Cardiol. 2014; 114:1763­7.

13. Apers S, Kovacs AH, Luyckx K, Alday L, Berghammer M, Budts W, Callus E, Caruana M, Chidambarathanu S, Cook

SC, Dellborg M, Enomoto J, Eriksen K, Fernandes SM, Jackson JL, Johansson B, Khairy P, Kutty S, Menahem S,

Rempel G, Sluman MA, Soufi A, Thomet C, Veldtman G, Wang JK, White K, Moons P, consortium A­I, International

Society for Adult Congenital Heart D. Assessment of Patterns of Patient­Reported Outcomes in Adults with

Congenital Heart disease ­ International Study (APPROACH­IS): rationale, design, and methods. Int J Cardiol.2015; 179:334­42.

14. Armsby L, Beekman RH, 3rd, Benson L, Fagan T, Hagler DJ, Hijazi ZM, Holzer R, Ing F, Kreutzer J, Lang P, Levi DS,

Latson L, Moore P, Mullins C, Ruiz C, Vincent R. SCAI expert consensus statement for advanced trainingprograms in pediatric and congenital interventional cardiac catheterization. Catheter Cardiovasc Interv. 2014;84:779­84.

15. Averin K, Lorts A, Connor C. Anaphylactic shock after amiodarone infusion resulting in haemodynamic collapserequiring a temporary ventricular assist device. Cardiol Young. 2015; 25:164­6.

16. Barac A, Murtagh G, Carver JR, Chen MH, Freeman AM, Herrmann J, Iliescu C, Ky B, Mayer EL, Okwuosa TM, Plana

JC, Ryan TD, Rzeszut AK, Douglas PS. Cardiovascular Health of Patients With Cancer and Cancer Survivors: ARoadmap to the Next Level. J Am Coll Cardiol. 2015; 65:2739­46.

17. Barbato E, Lara­Pezzi E, Stolen C, Taylor A, Barton PJ, Bartunek J, Iaizzo P, Judge DP, Kirshenbaum L, Blaxall BC,

Terzic A, Hall JL. Advances in induced pluripotent stem cells, genomics, biomarkers, and antiplatelet therapyhighlights of the year in JCTR 2013. J Cardiovasc Transl Res. 2014; 7:518­25.

18. Benoit S, Antommaria AH, Weidner N, Lorts A. Difficult decision: what should we do when a VAD­supported childexperiences a severe stroke?. Pediatr Transplant. 2015; 19:139­43.

19. Boczek NJ, Miller EM, Ye D, Nesterenko VV, Tester DJ, Antzelevitch C, Czosek RJ, Ackerman MJ, Ware SM. NovelTimothy syndrome mutation leading to increase in CACNA1C window current. Heart Rhythm. 2015; 12:211­9.

20. Brown DW, Cohen KE, O'Brien P, Gauvreau K, Klitzner TS, Beekman RH, 3rd, Kugler JD, Martin GR, Neish SR,

Rosenthal GL, Lannon C, Jenkins KJ. Impact of prenatal diagnosis in survivors of initial palliation of singleventricle heart disease: analysis of the national pediatric cardiology quality improvement collaborativedatabase. Pediatr Cardiol. 2015; 36:314­21.

21. Burns KM, Byrne BJ, Gelb BD, Kuhn B, Leinwand LA, Mital S, Pearson GD, Rodefeld M, Rossano JW, Stauffer BL,

Taylor MD, Towbin JA, Redington AN. New mechanistic and therapeutic targets for pediatric heart failure: reportfrom a National Heart, Lung, and Blood Institute working group. Circulation. 2014; 130:79­86.

22. Byrnes JW, Bhutta AT, Rettiganti MR, Gomez A, Garcia X, Dyamenahalli U, Johnson C, Jaquiss RD, Imamura M,

Prodhan P. Steroid therapy attenuates acute phase reactant response among children on ventricular assistdevice support. Ann Thorac Surg. 2015; 99:1392­8.

23. Caterini JE, Elzibak AH, Michel EJ, McCrindle BW, Redington AN, Thompson S, Noseworthy MD, Wells GD.

Characterizing blood oxygen level­dependent (BOLD) response following in­magnet quadriceps exercise.MAGMA. 2015; 28:271­8.

24. Ceco E, Bogdanovich S, Gardner B, Miller T, DeJesus A, Earley JU, Hadhazy M, Smith LR, Barton ER, Molkentin JD,

McNally EM. Targeting latent TGFbeta release in muscular dystrophy. Sci Transl Med. 2014; 6:259ra144.

25. Chamberlain AR, Sawyer JE, Cooper DS. Pharmacoeconomics. In: R Munoz, EM da Cruz, VL Vetter, DS Cooper,eds. Handbook of Pediatric Cardiovascular Drugs. New York, NY: Springer; 2014:59­72.

26. Conway J, St Louis J, Morales DL, Law S, Tjossem C, Humpl T. Delineating survival outcomes in children <10 kgbridged to transplant or recovery with the Berlin Heart EXCOR Ventricular Assist Device. JACC Heart Fail. 2015;3:70­7.

27. Cooper DS, Klugman D, Kinstler AJ, Nelson DP, Muething S. The Cardiac Intensive Care Unit and Operating RoomContinuum: Quality and Safety in the Cardiac Intensive Care Unit. In: PR Barach, JP Jacobs, SE Lipshultz, PCLaussen, eds. Pediatric and Congenital Cardiac Care: Quality Improvement and Patient Safety. London, UK:

Springer; 2014:91­104.

28. Cooper DS, Knilans TK. Arrhythmias. In: DS Wheeler, HR Wong, TP Shanley, eds. Pediatric Critical Care Medicine:

Respiratory, Cardiovascular and Central Nervous Systems. London, UK: Springer; 2014:451­466.

29. Cooper DS, Lorts A. Anticoagulation for Mechanical Circulatory Support. In: R Munoz, EM da Cruz, VL Vetter, DSCooper, eds. Handbook of Pediatric Cardiovascular Drugs. New York, NY: Springer; 2014:365­374.

30. Cooper DS, Morales DLS, del Corral M, Paden ML, Thiagarajan RR. Databases for Extracorporeal MembraneOxygenation and Ventricular Assist Devices. In: PR Barach, JP Jacobs, SE Lipshultz, PC Laussen, eds. Pediatricand Congenital Cardiac Care: Outcome Analysis. London, UK: Springer; 2014:211­218.

31. Covi SH, Whiteside W, Yu S, Zampi JD. Pulse fluoroscopy radiation reduction in a pediatric cardiaccatheterization laboratory. Congenit Heart Dis. 2015; 10:E43­7.

32. Czosek RJ, Cassedy AE, Wray J, Wernovsky G, Newburger JW, Mussatto KA, Mahony L, Tanel RE, Cohen MI,

Franklin RC, Brown KL, Rosenthal D, Drotar D, Marino BS. Quality of life in pediatric patients affected byelectrophysiologic disease. Heart Rhythm. 2015; 12:899­908.

33. Czosek RJ, Cnota JF, Knilans TK, Pratt J, Guerrier K, Anderson JB. Relationship between echocardiographic LVmass and ECG based left ventricular voltages in an adolescent population: related or random?. Pacing ClinElectrophysiol. 2014; 37:1133­40.

34. Czosek RJ, Spar DS, Khoury PR, Anderson JB, Wilmot I, Knilans TK, Jefferies JL. Outcomes, arrhythmic burdenand ambulatory monitoring of pediatric patients with left ventricular non­compaction and preserved leftventricular function. Am J Cardiol. 2015; 115:962­6.

35. Dandoy CE, Davies SM, Hirsch R, Chima RS, Paff Z, Cash M, Ryan TD, Lane A, El­Bietar J, Myers KC, Jodele S.

Abnormal echocardiography 7 days after stem cell transplantation may be an early indicator of thromboticmicroangiopathy. Biol Blood Marrow Transplant. 2015; 21:113­8.

36. D'Aniello E, Waxman JS. Input overload: Contributions of retinoic acid signaling feedback mechanisms to heartdevelopment and teratogenesis. Dev Dyn. 2015; 244:513­23.

37. Daniels CJ, Landzberg MJ, Beekman RH, 3rd. Structural heart disease: tetralogy, transposition, and truncus,too?. J Am Coll Cardiol. 2015; 65:2260­1.

38. Das A, Paul A, Taylor MD, Banerjee RK. Pulsatile arterial wall­blood flow interaction with wall pre­stresscomputed using an inverse algorithm. Biomed Eng Online. 2015; 14 Suppl 1:S18.

39. Decker JA, Knilans TK. Antiarrhythmics. In: R Munoz, EM da Cruz, VL Vetter, DS Cooper, eds. Handbook ofPediatric Cardiovascular Drugs. New York, NY: Springer; 2014:275­328.

40. DeSena HC, Nelson DP, Cooper DS. Cardiac intensive care for the neonate and child after cardiac surgery. CurrOpin Cardiol. 2015; 30:81­8.

41. Dragulescu A, Friedberg MK, Grosse­Wortmann L, Redington A, Mertens L. Effect of chronic right ventricularvolume overload on ventricular interaction in patients after tetralogy of Fallot repair. J Am Soc Echocardiogr.2014; 27:896­902.

42. Faircloth JM, Palumbo JS, Veldtman GR. Overcoming the challenges of anticoagulation in adults with congenitalheart disease. Heart. 2015; 101:418­20.

43. Fang M, Alfieri CM, Hulin A, Conway SJ, Yutzey KE. Loss of beta­catenin promotes chondrogenic differentiation ofaortic valve interstitial cells. Arterioscler Thromb Vasc Biol. 2014; 34:2601­8.

44. Frank DB, Crystal MA, Morales DL, Gerald K, Hanna BD, Mallory GB, Jr., Rossano JW. Trends in pediatricpulmonary hypertension­related hospitalizations in the United States from 2000­2009. Pulm Circ. 2015; 5:339­48.

45. Friess MR, Marino BS, Cassedy A, Wilmot I, Jefferies JL, Lorts A. Health­related quality of life assessment inchildren followed in a cardiomyopathy clinic. Pediatr Cardiol. 2015; 36:516­23.

46. Frommelt PC, Gerstenberger E, Cnota JF, Cohen MS, Gorentz J, Hill KD, John JB, Levine JC, Lu J, Mahle WT,

McCandless RT, Mertens L, Pearson GD, Spencer C, Thacker D, Williams IA, Wong PC, Newburger JW, Pediatric

Heart Network I. Impact of initial shunt type on cardiac size and function in children with single right ventricleanomalies before the Fontan procedure: the single ventricle reconstruction extension trial. J Am Coll Cardiol.2014; 64:2026­35.

47. Gaies M, Cooper DS, Tabbutt S, Schwartz SM, Ghanayem N, Chanani NK, Costello JM, Thiagarajan RR, Laussen

PC, Shekerdemian LS, Donohue JE, Willis GM, Gaynor JW, Jacobs JP, Ohye RG, Charpie JR, Pasquali SK, Scheurer

MA. Collaborative quality improvement in the cardiac intensive care unit: development of the Paediatric CardiacCritical Care Consortium (PC4). Cardiol Young. 2015; 25:951­7.

48. Ghelani SJ, Glatz AC, David S, Leahy R, Hirsch R, Armsby LB, Trucco SM, Holzer RJ, Bergersen L. Radiation dosebenchmarks during cardiac catheterization for congenital heart disease in the United States. JACC CardiovascInterv. 2014; 7:1060­9.

49. Gist KM, Schwartz SM, Krawczeski CD, Nelson DP, Wheeler DS. Single Ventricle Lesions. In: DS Wheeler, HR

Wong, TP Shanley, eds. Pediatric Critical Care Medicine: Respiratory, Cardiovascular and Central Nervous Systems.

London, UK: Springer; 2014:397­416.

50. Godby R, Munjal C, Opoka A, Smith J, Yutzey K, Narmoneva D, Hinton R. Cross Talk between NOTCH Signalingand Biomechanics in Human Aortic Valve Disease Pathogenesis. J Cardiovasc Dev Dis. 2014; 1:237­256.

51. Goldstein JA, Bogdanovich S, Beiriger A, Wren LM, Rossi AE, Gao QQ, Gardner BB, Earley JU, Molkentin JD,

McNally EM. Excess SMAD signaling contributes to heart and muscle dysfunction in muscular dystrophy. HumMol Genet. 2014; 23:6722­31.

52. Goldstein SL, Cooper DS. Drug Clearance on ECMO and Dialysis/CRRT. In: R Munoz, EM da Cruz, VL Vetter, DSCooper, eds. Handbook of Pediatric Cardiovascular Drugs. New York, NY: Springer; 2014:567­578.

53. Goonasekera SA, Davis J, Kwong JQ, Accornero F, Wei­LaPierre L, Sargent MA, Dirksen RT, Molkentin JD.

Enhanced Ca(2)(+) influx from STIM1­Orai1 induces muscle pathology in mouse models of muscular dystrophy.Hum Mol Genet. 2014; 23:3706­15.

54. Guerrier K, Anderson JB, Czosek RJ, Mays WA, Statile C, Knilans TK, Spar DS. Usefulness of ventricularpremature complexes in asymptomatic patients </=21 years as predictors of poor left ventricular function. Am JCardiol. 2015; 115:652­5.

55. Guerrier K, Anderson JB, Pratt J, King EC, Statile C, Wilmot I, Campbell M, Czosek RJ. Correlation of precordialvoltages to left ventricular mass on echocardiogram in adolescent patients with hypertrophic cardiomyopathycompared with that in adolescent athletes. Am J Cardiol. 2015; 115:956­61.

56. Gupta MK, Gulick J, Liu R, Wang X, Molkentin JD, Robbins J. Sumo E2 enzyme UBC9 is required for efficientprotein quality control in cardiomyocytes. Circ Res. 2014; 115:721­9.

57. Gutierrez­Aguilar M, Douglas DL, Gibson AK, Domeier TL, Molkentin JD, Baines CP. Genetic manipulation of thecardiac mitochondrial phosphate carrier does not affect permeability transition. J Mol Cell Cardiol. 2014; 72:316­25.

58. Hathout Y, Brody E, Clemens PR, Cripe L, DeLisle RK, Furlong P, Gordish­Dressman H, Hache L, Henricson E,

Hoffman EP, Kobayashi YM, Lorts A, Mah JK, McDonald C, Mehler B, Nelson S, Nikrad M, Singer B, Steele F, Sterling

D, Sweeney HL, Williams S, Gold L. Large­scale serum protein biomarker discovery in Duchenne musculardystrophy. Proc Natl Acad Sci U S A. 2015; 112:7153­8.

59. Hede SV, Morales DL, Parekh DR. At the heart of the old silk road. J Gen Intern Med. 2014; 29:1421­2.

60. Heiberg J, Ringgaard S, Schmidt MR, Redington A, Hjortdal VE. Structural and functional alterations of the rightventricle are common in adults operated for ventricular septal defect as toddlers. Eur Heart J CardiovascImaging. 2015; 16:483­9.

61. Heiberg J, Schmidt MR, Redington A, Hjortdal VE. Disrupted right ventricular force­frequency relationships inadults operated for ventricular septal defect as toddlers: abnormal peak force predicts peak oxygen uptakeduring exercise. Int J Cardiol. 2014; 177:918­24.

62. Heusch G, Botker HE, Przyklenk K, Redington A, Yellon D. Remote ischemic conditioning. J Am Coll Cardiol. 2015;65:177­95.

63. Heydarian HC, Madsen NL, Marino BS. Long­Term Outcomes in Congenital Heart Disease. In: DS Wheeler, HR

Wong, TP Shanley, eds. Pediatric Critical Care Medicine: Respiratory, Cardiovascular and Central Nervous Systems.

London, UK: Springer; 2014:417­440.

64. Hickey EJ, Nosikova Y, Pham­Hung E, Gritti M, Schwartz S, Caldarone CA, Redington A, Van Arsdell GS. NationalAeronautics and Space Administration "threat and error" model applied to pediatric cardiac surgery: errorcycles precede approximately 85% of patient deaths. J Thorac Cardiovasc Surg. 2015; 149:496­505; discussion505­7.

65. Hinton RB. Advances in the treatment of aortic valve disease: is it time for companion diagnostics?. Curr OpinPediatr. 2014; 26:546­52.

66. Hinton RB, Prakash A, Romp RL, Krueger DA, Knilans TK. Cardiovascular manifestations of tuberous sclerosiscomplex and summary of the revised diagnostic criteria and surveillance and management recommendationsfrom the international tuberous sclerosis consensus group. J Am Heart Assoc. 2014; 3:e001493.

67. Hong YH, Martin LA, Mulvaney JM, Burhans MS, Blaxall BC, Hinton RB. RNA extraction from healthy and failinghuman myocardium: a comparative evaluation. Biopreserv Biobank. 2015; 13:123­30.

68. Horsley M, Justice L, Moore R, Cooper DS. Parenteral Nutrition. In: R Munoz, EM da Cruz, VL Vetter, DS Cooper,eds. Handbook of Pediatric Cardiovascular Drugs. New York, NY: Springer; 2014:579­596.

69. Huang Y, Powers C, Madala SK, Greis KD, Haffey WD, Towbin JA, Purevjav E, Javadov S, Strauss AW, Khuchua Z.

Cardiac metabolic pathways affected in the mouse model of barth syndrome. PLoS One. 2015; 10:e0128561.

70. Huby AC, Mendsaikhan U, Takagi K, Martherus R, Wansapura J, Gong N, Osinska H, James JF, Kramer K, Saito K,

Robbins J, Khuchua Z, Towbin JA, Purevjav E. Disturbance in Z­disk mechanosensitive proteins induced by a

persistent mutant myopalladin causes familial restrictive cardiomyopathy. J Am Coll Cardiol. 2014; 64:2765­76.

71. Hussain T, Dragulescu A, Benson L, Yoo SJ, Meng H, Windram J, Wong D, Greiser A, Friedberg M, Mertens L, Seed

M, Redington A, Grosse­Wortmann L. Quantification and significance of diffuse myocardial fibrosis and diastolicdysfunction in childhood hypertrophic cardiomyopathy. Pediatr Cardiol. 2015; 36:970­8.

72. Hyldebrandt JA, Frederiksen CA, Heiberg J, Rothmann S, Redington AN, Schmidt MR, Ravn HB. Inotropic therapyfor right ventricular failure in newborn piglets: effect on contractility, hemodynamics, and interventricularinteraction. Pediatr Crit Care Med. 2014; 15:e327­33.

73. Inge TH, Siegel RM, Xanthakos SA. Weight loss maintenance: a hard nut to crack. JAMA Pediatr. 2014; 168:796­7.

74. Jolley M, Thiagarajan RR, Barrett CS, Salvin JW, Cooper DS, Rycus PT, Teele SA. Extracorporeal membraneoxygenation in patients undergoing superior cavopulmonary anastomosis. J Thorac Cardiovasc Surg. 2014;148:1512­8.

75. In: Jones MB, Klugman D, Fitzgerald RK, Kohr LM, Berger JT, Costello JM, Bronicki R, Cooper DS, eds.

(2015)Pediatric Cardiac Intensive Care Handbook. Washington D.C., Pediatirc Cardiac Intensive Care Books, LLC.

76. Kaddourah A, Uthup S, Madueme P, O'Rourke M, Hooper DK, Taylor MD, Colan SD, Jefferies JL, Rao MB, Goebel J.

Prevalence and predictors of aortic dilation as a novel cardiovascular complication in children with end­stagerenal disease. Clin Nephrol. 2015; 83:262­71.

77. Karani KB, Zafar F, Morales DL, Goldstein BH. Hybrid stage I palliation in a 1.1 kg, 28­week preterm neonate withposterior malalignment ventricular septal defect, left ventricular outflow tract obstruction, and coarctation ofthe aorta. World J Pediatr Congenit Heart Surg. 2014; 5:603­7.

78. Karch J, Kanisicak O, Brody MJ, Sargent MA, Michael DM, Molkentin JD. Necroptosis Interfaces with MOMP andthe MPTP in Mediating Cell Death. PLoS One. 2015; 10:e0130520.

79. Karch J, Molkentin JD. Regulated Necrotic Cell Death: The Passive Aggressive Side of Bax and Bak. Circ Res.2015; 116:1800­1809.

80. Karch J, Molkentin JD. Identifying the components of the elusive mitochondrial permeability transition pore. ProcNatl Acad Sci U S A. 2014; 111:10396­7.

81. Kassebaum NJ, Bertozzi­Villa A, Coggeshall MS, Shackelford KA, Steiner C, Heuton KR, Gonzalez­Medina D, Barber

R, Huynh C, Dicker D, Templin T, Wolock TM, Ozgoren AA, Abd­Allah F, Abera SF, Abubakar I, Achoki T, Adelekan A,

Ademi Z, Adou AK, Adsuar JC, Agardh EE, Akena D, Alasfoor D, Alemu ZA, Alfonso­Cristancho R, Alhabib S, Ali R, Al

Kahbouri MJ, Alla F, Allen PJ, AlMazroa MA, Alsharif U, Alvarez E, Alvis­Guzman N, Amankwaa AA, Amare AT, Amini

H, Ammar W, Antonio CA, Anwari P, Arnlov J, Arsenijevic VS, Artaman A, Asad MM, Asghar RJ, Assadi R, Atkins LS,

Badawi A, Balakrishnan K, Basu A, Basu S, Beardsley J, Bedi N, Bekele T, Bell ML, Bernabe E, Beyene TJ, Bhutta Z,

Bin Abdulhak A, Blore JD, Basara BB, Bose D, Breitborde N, Cardenas R, Castaneda­Orjuela CA, Castro RE, Catala­

Lopez F, Cavlin A, Chang JC, Che X, Christophi CA, Chugh SS, Cirillo M, Colquhoun SM, Cooper LT, Cooper C, da

Costa Leite I, Dandona L, Dandona R, Davis A, Dayama A, Degenhardt L, De Leo D, del Pozo­Cruz B, Deribe K,

Dessalegn M, deVeber GA, Dharmaratne SD, Dilmen U, Ding EL, Dorrington RE, Driscoll TR, Ermakov SP,

Esteghamati A, Faraon EJ, Farzadfar F, Felicio MM, Fereshtehnejad SM, de Lima GM, Forouzanfar MH, Franca EB,

Gaffikin L, Gambashidze K, Gankpe FG, Garcia AC, Geleijnse JM, Gibney KB, Giroud M, Glaser EL, Goginashvili K,

Gona P, Gonzalez­Castell D, Goto A, Gouda HN, Gugnani HC, Gupta R, Gupta R, Hafezi­Nejad N, Hamadeh RR,

Hammami M, Hankey GJ, Harb HL, Havmoeller R, Hay SI, Pi IB, Hoek HW, Hosgood HD, Hoy DG, Husseini A, Idrisov

BT, Innos K, Inoue M, Jacobsen KH, Jahangir E, Jee SH, Jensen PN, Jha V, Jiang G, Jonas JB, Juel K, Kabagambe

EK, Kan H, Karam NE, Karch A, Karema CK, Kaul A, Kawakami N, Kazanjan K, Kazi DS, Kemp AH, Kengne AP,

Kereselidze M, Khader YS, Khalifa SE, Khan EA, Khang YH, Knibbs L, Kokubo Y, Kosen S, Defo BK, Kulkarni C,

Kulkarni VS, Kumar GA, Kumar K, Kumar RB, Kwan G, Lai T, Lalloo R, Lam H, Lansingh VC, Larsson A, Lee JT,

Leigh J, Leinsalu M, Leung R, Li X, Li Y, Li Y, Liang J, Liang X, Lim SS, Lin HH, Lipshultz SE, Liu S, Liu Y, Lloyd BK,

London SJ, Lotufo PA, Ma J, Ma S, Machado VM, Mainoo NK, Majdan M, Mapoma CC, Marcenes W, Marzan MB,

Mason­Jones AJ, Mehndiratta MM, Mejia­Rodriguez F, Memish ZA, Mendoza W, Miller TR, Mills EJ, Mokdad AH,

Mola GL, Monasta L, de la Cruz Monis J, Hernandez JC, Moore AR, Moradi­Lakeh M, Mori R, Mueller UO,

Mukaigawara M, Naheed A, Naidoo KS, Nand D, Nangia V, Nash D, Nejjari C, Nelson RG, Neupane SP, Newton CR,

Ng M, Nieuwenhuijsen MJ, Nisar MI, Nolte S, Norheim OF, Nyakarahuka L, Oh IH, Ohkubo T, Olusanya BO, Omer SB,

Opio JN, Orisakwe OE, Pandian JD, Papachristou C, Park JH, Caicedo AJ, Patten SB, Paul VK, Pavlin BI, Pearce N,

Pereira DM, Pesudovs K, Petzold M, Poenaru D, Polanczyk GV, Polinder S, Pope D, Pourmalek F, Qato D, Quistberg

DA, Rafay A, Rahimi K, Rahimi­Movaghar V, ur Rahman S, Raju M, Rana SM, Refaat A, Ronfani L, Roy N, Pimienta

TG, Sahraian MA, Salomon JA, Sampson U, Santos IS, Sawhney M, Sayinzoga F, Schneider IJ, Schumacher A,

Schwebel DC, Seedat S, Sepanlou SG, Servan­Mori EE, Shakh­Nazarova M, Sheikhbahaei S, Shibuya K, Shin HH,

Shiue I, Sigfusdottir ID, Silberberg DH, Silva AP, Singh JA, Skirbekk V, Sliwa K, Soshnikov SS, Sposato LA,

Sreeramareddy CT, Stroumpoulis K, Sturua L, Sykes BL, Tabb KM, Talongwa RT, Tan F, Teixeira CM, Tenkorang EY,

Terkawi AS, Thorne­Lyman AL, Tirschwell DL, Towbin JA, Tran BX, Tsilimbaris M, Uchendu US, Ukwaja KN,

Undurraga EA, Uzun SB, Vallely AJ, van Gool CH, Vasankari TJ, Vavilala MS, Venketasubramanian N, Villalpando S,

Violante FS, Vlassov VV, Vos T, Waller S, Wang H, Wang L, Wang X, Wang Y, Weichenthal S, Weiderpass E,

Weintraub RG, Westerman R, Wilkinson JD, Woldeyohannes SM, Wong JQ, Wordofa MA, Xu G, Yang YC, Yano Y,

Yentur GK, Yip P, Yonemoto N, Yoon SJ, Younis MZ, Yu C, Jin KY, El Sayed Zaki M, Zhao Y, Zheng Y, Zhou M, Zhu J,

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170. Trasande L, Urbina EM, Khoder M, Alghamdi M, Shabaj I, Alam MS, Harrison RM, Shamy M. Polycyclic aromatichydrocarbons, brachial artery distensibility and blood pressure among children residing near an oil refinery.Environ Res. 2015; 136:133­40.

171. Urbina EM, Falkner B. Right Analysis­wrong conclusion: Obese youth with higher BP are at risk for target organdamage. Am J Hypertens. 2015; 28:570­1.

172. Urbina EM, Khoury PR, McCoy CE, Daniels SR, Dolan LM, Kimball TR. Comparison of mercurysphygmomanometry blood pressure readings with oscillometric and central blood pressure in predicting targetorgan damage in youth. Blood Press Monit. 2015; 20:150­6.

173. van Berlo JH, Molkentin JD. An emerging consensus on cardiac regeneration. Nat Med. 2014; 20:1386­93.

174. Varnell CD, Jr., Goldstein SL, Yee DL, Teruya J, Guyer KE, Siddiqui S, Jefferies JL. Age­related differences inurinary 11­dehydroxythromboxane B2 between infants, children, and adolescents: another example ofdevelopmental hemostasis?. Pediatr Blood Cancer. 2014; 61:2074­6.

175. Veldtman GR, Moore RA, Madueme PC, Webb GD. Tetralogy of Fallot. In: GN Levine, ed. Color Atlas ofCardiovascular Disease. New Delhi, India: Jaypee Brothers Medical Publishers, Ltd; 2015:745­762.

176. Veldtman GR, Webb GD. Improved survival in Fontan­associated protein­losing enteropathy. J Am Coll Cardiol.2014; 64:63­5.

177. Villa CR, Ryan TD, Collins JJ, Taylor MD, Lucky AW, Jefferies JL. Left ventricular non­compaction cardiomyopathyassociated with epidermolysis bullosa simplex with muscular dystrophy and PLEC1 mutation. NeuromusculDisord. 2015; 25:165­8.

178. Villa CR, Ryan TD, Taylor MD, Jefferies JL. Response to: PLEC1 mutation associated with left ventricularhypertrabeculation/noncompaction. Neuromuscul Disord. 2015; 25:448­9.

179. Villafane J, Baker GH, Austin EH, 3rd, Miller S, Peng L, Beekman R, 3rd. Melody pulmonary valve bacterialendocarditis: experience in four pediatric patients and a review of the literature. Catheter Cardiovasc Interv.2014; 84:212­8.

180. Villafane J, Lantin­Hermoso MR, Bhatt AB, Tweddell JS, Geva T, Nathan M, Elliott MJ, Vetter VL, Paridon SM,

Kochilas L, Jenkins KJ, Beekman RH, 3rd, Wernovsky G, Towbin JA, American College of Cardiology's Adult C,

Pediatric Cardiology C. D­transposition of the great arteries: the current era of the arterial switch operation. J

Am Coll Cardiol. 2014; 64:498­511.

181. Villamizar­Schiller IT, Pabon LA, Hufnagel SB, Serrano NC, Karl G, Jefferies JL, Hopkin RJ, Prada CE. Neurologicaland cardiac responses after treatment with miglustat and a ketogenic diet in a patient with Sandhoff disease.Eur J Med Genet. 2015; 58:180­3.

182. Votava­Smith JK, Habli M, Cnota JF, Divanovic A, Polzin W, Lim FY, Michelfelder EC. Diastolic dysfunction andcerebrovascular redistribution precede overt recipient twin cardiomyopathy in early­stage twin­twintransfusion syndrome. J Am Soc Echocardiogr. 2015; 28:533­40.

183. Wald RM, Altaha MA, Alvarez N, Caldarone CA, Cavalle­Garrido T, Dallaire F, Drolet C, Grewal J, Hancock Friesen

CL, Human DG, Hickey E, Kayedpour C, Khairy P, Kovacs AH, Lebovic G, McCrindle BW, Nadeem SN, Patton DJ,

Redington AN, Silversides CK, Tham EB, Therrien J, Warren AE, Wintersperger BJ, Vonder Muhll IF, Farkouh ME.

Rationale and design of the Canadian Outcomes Registry Late After Tetralogy of Fallot Repair: theCORRELATE study. Can J Cardiol. 2014; 30:1436­43.

184. Wallihan DB, Podberesky DJ, Marino BS, Sticka JS, Serai S. Relationship of MR elastography determined liverstiffness with cardiac function after Fontan palliation. J Magn Reson Imaging. 2014; 40:1328­35.

185. Ware SM, Shikany A, Landis BJ, James JF, Hinton RB. Twins with progressive thoracic aortic aneurysm,recurrent dissection and ACTA2 mutation. Pediatrics. 2014; 134:e1218­23.

186. Weaver KN, Wang D, Cnota J, Gardner N, Stabley D, Sol­Church K, Gripp KW, Witte DP, Bove KE, Hopkin RJ. Early­lethal Costello syndrome due to rare HRAS Tandem Base substitution (c.35_36GC>AA; p.G12E)­associatedpulmonary vascular disease. Pediatr Dev Pathol. 2014; 17:421­30.

187. Whiteside W, Christensen J, Zampi JD. Three­dimensional magnetic resonance imaging overlay to assist withpercutaneous transhepatic access at the time of cardiac catheterization. Ann Pediatr Cardiol. 2015; 8:150­2.

188. Wilmot I, Taylor B, Ryan TD. Oral Heart Failure Therapy. In: MB Jones, D Klugman, RK Fitzgeraldet al, eds. PediatricCardiac Intensive Care Handbook. Washington, DC: Pediatric Cardiac Intensive Care Books; 2015:73­76.

189. Wirrig EE, Gomez MV, Hinton RB, Yutzey KE. COX2 inhibition reduces aortic valve calcification in vivo.Arterioscler Thromb Vasc Biol. 2015; 35:938­47.

190. Wissing ER, Boyer JG, Kwong JQ, Sargent MA, Karch J, McNally EM, Otsu K, Molkentin JD. P38alpha MAPKunderlies muscular dystrophy and myofiber death through a Bax­dependent mechanism. Hum Mol Genet. 2014;

23:5452­63.

191. Yutzey KE. Regenerative biology: Neuregulin 1 makes heart muscle. Nature. 2015; 520:445­6.

192. Yutzey KE, Demer LL, Body SC, Huggins GS, Towler DA, Giachelli CM, Hofmann­Bowman MA, Mortlock DP, Rogers

MB, Sadeghi MM, Aikawa E. Calcific aortic valve disease: a consensus summary from the Alliance ofInvestigators on Calcific Aortic Valve Disease. Arterioscler Thromb Vasc Biol. 2014; 34:2387­93.

193. Zafar F, Castleberry C, Khan MS, Mehta V, Bryant R, 3rd, Lorts A, Wilmot I, Jefferies JL, Chin C, Morales DL.

Pediatric heart transplant waiting list mortality in the era of ventricular assist devices. J Heart Lung Transplant.2015; 34:82­8.

194. Zafar F, Jefferies JL, Tjossem CJ, Bryant R, 3rd, Jaquiss RD, Wearden PD, Rosenthal DN, Cabrera AG, Rossano JW,

Humpl T, Morales DL. Biventricular Berlin Heart EXCOR pediatric use across the united states. Ann Thorac Surg.2015; 99:1328­34.

2015 Research Annual Report

Click to view members

Heart InstituteRESEARCH AND TRAINING DETAILS

Faculty 67

Joint Appointment Faculty 2

Research Fellows 33

Research Students 24

Support Personnel 146

Direct Annual Grant Support $11,301,852

Direct Annual Industry Support $227,476

Peer Reviewed Publications 194

CLINICAL ACTIVITIES AND TRAINING

Clinical Staff 187

Staff Physicians 2

Clinical Fellows 21

Other Students 4

Inpatient Encounters 1,972

Outpatient Encounters 19,397

Division Publications

Division Publications1. Accornero F, Kanisicak O, Tjondrokoesoemo A, Attia AC, McNally EM, Molkentin JD. Myofiber­specific inhibition of

TGFbeta signaling protects skeletal muscle from injury and dystrophic disease in mice. Hum Mol Genet. 2014;

23:6903­15.

2. Accornero F, van Berlo JH, Correll RN, Elrod JW, Sargent MA, York A, Rabinowitz JE, Leask A, Molkentin JD. GeneticAnalysis of Connective Tissue Growth Factor as an Effector of Transforming Growth Factor beta Signaling andCardiac Remodeling. Mol Cell Biol. 2015; 35:2154­64.

3. Adachi I, Khan MS, Guzman­Pruneda FA, Fraser CD, 3rd, Mery CM, Denfield SW, Dreyer WJ, Morales DL, McKenzie

ED, Heinle JS, Fraser CD, Jr.. Evolution and impact of ventricular assist device program on children awaitingheart transplantation. Ann Thorac Surg. 2015; 99:635­40.

4. Adachi I, Morales DS. Implantation of total artificial heart in congenital heart disease. J Vis Exp. 2014; .

5. Aiyagari R, Rhodes JF, Shrader P, Radtke WA, Bandisode VM, Bergersen L, Gillespie MJ, Gray RG, Guey LT, Hill KD,

Hirsch R, Kim DW, Lee KJ, Pelech AN, Ringewald J, Takao C, Vincent JA, Ohye RG, Pediatric Heart Network I.

Impact of pre­stage II hemodynamics and pulmonary artery anatomy on 12­month outcomes in the PediatricHeart Network Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg. 2014; 148:1467­74.

6. Albrecht LV, Zhang L, Shabanowitz J, Purevjav E, Towbin JA, Hunt DF, Green KJ. GSK3­ and PRMT­1­dependentmodifications of desmoplakin control desmoplakin­cytoskeleton dynamics. J Cell Biol. 2015; 208:597­612.

7. Alman AC, Talton JW, Wadwa RP, Urbina EM, Dolan LM, Daniels SR, Hamman RF, D'Agostino RB, Marcovina SM,

Mayer­Davis EJ, Dabelea DM. Cardiovascular health in adolescents with type 1 diabetes: the SEARCH CVD study.Pediatr Diabetes. 2014; 15:502­10.

8. Alsaied T, Madueme PC. International medical graduates in cardiology fellowship: brain drain or brain gain?. JAm Coll Cardiol. 2015; 65:507­9; discussion 509­10.

9. Anderson JB, Beekman RH, 3rd. Addressing Nutrition and Growth in Children with Congenital Heart Disease. In:PR Barach, JP Jacobs, SE Lipshultz, PC Laussen, eds. Pediatric and Congenital Cardiac Care: Quality

Improvement and Patient Safety. London, UK: Springer; 2014:153­164.

10. Anderson JB, Beekman RH, 3rd, Kugler JD, Rosenthal GL, Jenkins KJ, Klitzner TS, Martin GR, Neish SR, Darbie L,

King E, Lannon C, National Pediatric Cardiology Quality Improvement C. Use of a learning network to improvevariation in interstage weight gain after the Norwood operation. Congenit Heart Dis. 2014; 9:512­20.

11. Anderson JB, Beekman RH, 3rd, Martin GR, Lannon C. Quality Improvement in Pediatric Cardiology: The NationalPediatric Cardiology Quality Improvement Collaborative. In: PR Barach, JP Jacobs, SE Lipshultz, PC Laussen,eds. Pediatric and Congenital Cardiac Care: Quality Improvement and Patient Safety. London, UK: Springer;

2014:51­68.

12. Anderson JB, Grenier M, Edwards NM, Madsen NL, Czosek RJ, Spar DS, Barnes A, Pratt J, King E, Knilans TK.

Usefulness of combined history, physical examination, electrocardiogram, and limited echocardiogram inscreening adolescent athletes for risk for sudden cardiac death. Am J Cardiol. 2014; 114:1763­7.

13. Apers S, Kovacs AH, Luyckx K, Alday L, Berghammer M, Budts W, Callus E, Caruana M, Chidambarathanu S, Cook

SC, Dellborg M, Enomoto J, Eriksen K, Fernandes SM, Jackson JL, Johansson B, Khairy P, Kutty S, Menahem S,

Rempel G, Sluman MA, Soufi A, Thomet C, Veldtman G, Wang JK, White K, Moons P, consortium A­I, International

Society for Adult Congenital Heart D. Assessment of Patterns of Patient­Reported Outcomes in Adults with

Congenital Heart disease ­ International Study (APPROACH­IS): rationale, design, and methods. Int J Cardiol.2015; 179:334­42.

14. Armsby L, Beekman RH, 3rd, Benson L, Fagan T, Hagler DJ, Hijazi ZM, Holzer R, Ing F, Kreutzer J, Lang P, Levi DS,

Latson L, Moore P, Mullins C, Ruiz C, Vincent R. SCAI expert consensus statement for advanced trainingprograms in pediatric and congenital interventional cardiac catheterization. Catheter Cardiovasc Interv. 2014;84:779­84.

15. Averin K, Lorts A, Connor C. Anaphylactic shock after amiodarone infusion resulting in haemodynamic collapserequiring a temporary ventricular assist device. Cardiol Young. 2015; 25:164­6.

16. Barac A, Murtagh G, Carver JR, Chen MH, Freeman AM, Herrmann J, Iliescu C, Ky B, Mayer EL, Okwuosa TM, Plana

JC, Ryan TD, Rzeszut AK, Douglas PS. Cardiovascular Health of Patients With Cancer and Cancer Survivors: ARoadmap to the Next Level. J Am Coll Cardiol. 2015; 65:2739­46.

17. Barbato E, Lara­Pezzi E, Stolen C, Taylor A, Barton PJ, Bartunek J, Iaizzo P, Judge DP, Kirshenbaum L, Blaxall BC,

Terzic A, Hall JL. Advances in induced pluripotent stem cells, genomics, biomarkers, and antiplatelet therapyhighlights of the year in JCTR 2013. J Cardiovasc Transl Res. 2014; 7:518­25.

18. Benoit S, Antommaria AH, Weidner N, Lorts A. Difficult decision: what should we do when a VAD­supported childexperiences a severe stroke?. Pediatr Transplant. 2015; 19:139­43.

19. Boczek NJ, Miller EM, Ye D, Nesterenko VV, Tester DJ, Antzelevitch C, Czosek RJ, Ackerman MJ, Ware SM. NovelTimothy syndrome mutation leading to increase in CACNA1C window current. Heart Rhythm . 2015; 12:211­9.

20. Brown DW, Cohen KE, O'Brien P, Gauvreau K, Klitzner TS, Beekman RH, 3rd, Kugler JD, Martin GR, Neish SR,

Rosenthal GL, Lannon C, Jenkins KJ. Impact of prenatal diagnosis in survivors of initial palliation of singleventricle heart disease: analysis of the national pediatric cardiology quality improvement collaborativedatabase. Pediatr Cardiol. 2015; 36:314­21.

21. Burns KM, Byrne BJ, Gelb BD, Kuhn B, Leinwand LA, Mital S, Pearson GD, Rodefeld M, Rossano JW, Stauffer BL,

Taylor MD, Towbin JA, Redington AN. New mechanistic and therapeutic targets for pediatric heart failure: reportfrom a National Heart, Lung, and Blood Institute working group. Circulation. 2014; 130:79­86.

22. Byrnes JW, Bhutta AT, Rettiganti MR, Gomez A, Garcia X, Dyamenahalli U, Johnson C, Jaquiss RD, Imamura M,

Prodhan P. Steroid therapy attenuates acute phase reactant response among children on ventricular assistdevice support. Ann Thorac Surg. 2015; 99:1392­8.

23. Caterini JE, Elzibak AH, Michel EJ, McCrindle BW, Redington AN, Thompson S, Noseworthy MD, Wells GD.

Characterizing blood oxygen level­dependent (BOLD) response following in­magnet quadriceps exercise.MAGMA. 2015; 28:271­8.

24. Ceco E, Bogdanovich S, Gardner B, Miller T, DeJesus A, Earley JU, Hadhazy M, Smith LR, Barton ER, Molkentin JD,

McNally EM. Targeting latent TGFbeta release in muscular dystrophy. Sci Transl Med. 2014; 6:259ra144.

25. Chamberlain AR, Sawyer JE, Cooper DS. Pharmacoeconomics. In: R Munoz, EM da Cruz, VL Vetter, DS Cooper,eds. Handbook of Pediatric Cardiovascular Drugs. New York, NY: Springer; 2014:59­72.

26. Conway J, St Louis J, Morales DL, Law S, Tjossem C, Humpl T. Delineating survival outcomes in children <10 kgbridged to transplant or recovery with the Berlin Heart EXCOR Ventricular Assist Device. JACC Heart Fail. 2015;3:70­7.

27. Cooper DS, Klugman D, Kinstler AJ, Nelson DP, Muething S. The Cardiac Intensive Care Unit and Operating RoomContinuum: Quality and Safety in the Cardiac Intensive Care Unit. In: PR Barach, JP Jacobs, SE Lipshultz, PCLaussen, eds. Pediatric and Congenital Cardiac Care: Quality Improvement and Patient Safety. London, UK:

Springer; 2014:91­104.

28. Cooper DS, Knilans TK. Arrhythmias. In: DS Wheeler, HR Wong, TP Shanley, eds. Pediatric Critical Care Medicine:

Respiratory, Cardiovascular and Central Nervous Systems. London, UK: Springer; 2014:451­466.

29. Cooper DS, Lorts A. Anticoagulation for Mechanical Circulatory Support. In: R Munoz, EM da Cruz, VL Vetter, DSCooper, eds. Handbook of Pediatric Cardiovascular Drugs. New York, NY: Springer; 2014:365­374.

30. Cooper DS, Morales DLS, del Corral M, Paden ML, Thiagarajan RR. Databases for Extracorporeal MembraneOxygenation and Ventricular Assist Devices. In: PR Barach, JP Jacobs, SE Lipshultz, PC Laussen, eds. Pediatricand Congenital Cardiac Care: Outcome Analysis. London, UK: Springer; 2014:211­218.

31. Covi SH, Whiteside W, Yu S, Zampi JD. Pulse fluoroscopy radiation reduction in a pediatric cardiaccatheterization laboratory. Congenit Heart Dis. 2015; 10:E43­7.

32. Czosek RJ, Cassedy AE, Wray J, Wernovsky G, Newburger JW, Mussatto KA, Mahony L, Tanel RE, Cohen MI,

Franklin RC, Brown KL, Rosenthal D, Drotar D, Marino BS. Quality of life in pediatric patients affected byelectrophysiologic disease. Heart Rhythm . 2015; 12:899­908.

33. Czosek RJ, Cnota JF, Knilans TK, Pratt J, Guerrier K, Anderson JB. Relationship between echocardiographic LVmass and ECG based left ventricular voltages in an adolescent population: related or random?. Pacing ClinElectrophysiol. 2014; 37:1133­40.

34. Czosek RJ, Spar DS, Khoury PR, Anderson JB, Wilmot I, Knilans TK, Jefferies JL. Outcomes, arrhythmic burdenand ambulatory monitoring of pediatric patients with left ventricular non­compaction and preserved leftventricular function. Am J Cardiol. 2015; 115:962­6.

35. Dandoy CE, Davies SM, Hirsch R, Chima RS, Paff Z, Cash M, Ryan TD, Lane A, El­Bietar J, Myers KC, Jodele S.

Abnormal echocardiography 7 days after stem cell transplantation may be an early indicator of thromboticmicroangiopathy. Biol Blood Marrow Transplant. 2015; 21:113­8.

36. D'Aniello E, Waxman JS. Input overload: Contributions of retinoic acid signaling feedback mechanisms to heartdevelopment and teratogenesis. Dev Dyn. 2015; 244:513­23.

37. Daniels CJ, Landzberg MJ, Beekman RH, 3rd. Structural heart disease: tetralogy, transposition, and truncus,too?. J Am Coll Cardiol. 2015; 65:2260­1.

38. Das A, Paul A, Taylor MD, Banerjee RK. Pulsatile arterial wall­blood flow interaction with wall pre­stresscomputed using an inverse algorithm. Biomed Eng Online. 2015; 14 Suppl 1:S18.

39. Decker JA, Knilans TK. Antiarrhythmics. In: R Munoz, EM da Cruz, VL Vetter, DS Cooper, eds. Handbook of PediatricCardiovascular Drugs. New York, NY: Springer; 2014:275­328.

40. DeSena HC, Nelson DP, Cooper DS. Cardiac intensive care for the neonate and child after cardiac surgery. CurrOpin Cardiol. 2015; 30:81­8.

41. Dragulescu A, Friedberg MK, Grosse­Wortmann L, Redington A, Mertens L. Effect of chronic right ventricularvolume overload on ventricular interaction in patients after tetralogy of Fallot repair. J Am Soc Echocardiogr.

2014; 27:896­902.

42. Faircloth JM, Palumbo JS, Veldtman GR. Overcoming the challenges of anticoagulation in adults with congenitalheart disease. Heart. 2015; 101:418­20.

43. Fang M, Alfieri CM, Hulin A, Conway SJ, Yutzey KE. Loss of beta­catenin promotes chondrogenic differentiation ofaortic valve interstitial cells. Arterioscler Thromb Vasc Biol. 2014; 34:2601­8.

44. Frank DB, Crystal MA, Morales DL, Gerald K, Hanna BD, Mallory GB, Jr., Rossano JW. Trends in pediatricpulmonary hypertension­related hospitalizations in the United States from 2000­2009. Pulm Circ. 2015; 5:339­48.

45. Friess MR, Marino BS, Cassedy A, Wilmot I, Jefferies JL, Lorts A. Health­related quality of life assessment inchildren followed in a cardiomyopathy clinic. Pediatr Cardiol. 2015; 36:516­23.

46. Frommelt PC, Gerstenberger E, Cnota JF, Cohen MS, Gorentz J, Hill KD, John JB, Levine JC, Lu J, Mahle WT,

McCandless RT, Mertens L, Pearson GD, Spencer C, Thacker D, Williams IA, Wong PC, Newburger JW, Pediatric

Heart Network I. Impact of initial shunt type on cardiac size and function in children with single right ventricleanomalies before the Fontan procedure: the single ventricle reconstruction extension trial. J Am Coll Cardiol.

2014; 64:2026­35.

47. Gaies M, Cooper DS, Tabbutt S, Schwartz SM, Ghanayem N, Chanani NK, Costello JM, Thiagarajan RR, Laussen

PC, Shekerdemian LS, Donohue JE, Willis GM, Gaynor JW, Jacobs JP, Ohye RG, Charpie JR, Pasquali SK,

Scheurer MA. Collaborative quality improvement in the cardiac intensive care unit: development of the PaediatricCardiac Critical Care Consortium (PC4). Cardiol Young. 2015; 25:951­7.

48. Ghelani SJ, Glatz AC, David S, Leahy R, Hirsch R, Armsby LB, Trucco SM, Holzer RJ, Bergersen L. Radiation dosebenchmarks during cardiac catheterization for congenital heart disease in the United States. JACC CardiovascInterv. 2014; 7:1060­9.

49. Gist KM, Schwartz SM, Krawczeski CD, Nelson DP, Wheeler DS. Single Ventricle Lesions. In: DS Wheeler, HR

Wong, TP Shanley, eds. Pediatric Critical Care Medicine: Respiratory, Cardiovascular and Central Nervous Systems.

London, UK: Springer; 2014:397­416.

50. Godby R, Munjal C, Opoka A, Smith J, Yutzey K, Narmoneva D, Hinton R. Cross Talk between NOTCH Signaling andBiomechanics in Human Aortic Valve Disease Pathogenesis. J Cardiovasc Dev Dis. 2014; 1:237­256.

51. Goldstein JA, Bogdanovich S, Beiriger A, Wren LM, Rossi AE, Gao QQ, Gardner BB, Earley JU, Molkentin JD, McNally

EM. Excess SMAD signaling contributes to heart and muscle dysfunction in muscular dystrophy. Hum Mol Genet.

2014; 23:6722­31.

52. Goldstein SL, Cooper DS. Drug Clearance on ECMO and Dialysis/CRRT. In: R Munoz, EM da Cruz, VL Vetter, DSCooper, eds. Handbook of Pediatric Cardiovascular Drugs. New York, NY: Springer; 2014:567­578.

53. Goonasekera SA, Davis J, Kwong JQ, Accornero F, Wei­LaPierre L, Sargent MA, Dirksen RT, Molkentin JD.

Enhanced Ca(2)(+) influx from STIM1­Orai1 induces muscle pathology in mouse models of muscular dystrophy.Hum Mol Genet. 2014; 23:3706­15.

54. Guerrier K, Anderson JB, Czosek RJ, Mays WA, Statile C, Knilans TK, Spar DS. Usefulness of ventricular prematurecomplexes in asymptomatic patients </=21 years as predictors of poor left ventricular function. Am J Cardiol.

2015; 115:652­5.

55. Guerrier K, Anderson JB, Pratt J, King EC, Statile C, Wilmot I, Campbell M, Czosek RJ. Correlation of precordialvoltages to left ventricular mass on echocardiogram in adolescent patients with hypertrophic cardiomyopathycompared with that in adolescent athletes. Am J Cardiol. 2015; 115:956­61.

56. Gupta MK, Gulick J, Liu R, Wang X, Molkentin JD, Robbins J. Sumo E2 enzyme UBC9 is required for efficientprotein quality control in cardiomyocytes. Circ Res. 2014; 115:721­9.

57. Gutierrez­Aguilar M, Douglas DL, Gibson AK, Domeier TL, Molkentin JD, Baines CP. Genetic manipulation of thecardiac mitochondrial phosphate carrier does not affect permeability transition. J Mol Cell Cardiol. 2014; 72:316­25.

58. Hathout Y, Brody E, Clemens PR, Cripe L, DeLisle RK, Furlong P, Gordish­Dressman H, Hache L, Henricson E,

Hoffman EP, Kobayashi YM, Lorts A, Mah JK, McDonald C, Mehler B, Nelson S, Nikrad M, Singer B, Steele F, Sterling

D, Sweeney HL, Williams S, Gold L. Large­scale serum protein biomarker discovery in Duchenne musculardystrophy. Proc Natl Acad Sci U S A. 2015; 112:7153­8.

59. Hede SV, Morales DL, Parekh DR. At the heart of the old silk road. J Gen Intern Med. 2014; 29:1421­2.

60. Heiberg J, Ringgaard S, Schmidt MR, Redington A, Hjortdal VE. Structural and functional alterations of the rightventricle are common in adults operated for ventricular septal defect as toddlers. Eur Heart J CardiovascImaging. 2015; 16:483­9.

61. Heiberg J, Schmidt MR, Redington A, Hjortdal VE. Disrupted right ventricular force­frequency relationships inadults operated for ventricular septal defect as toddlers: abnormal peak force predicts peak oxygen uptakeduring exercise. Int J Cardiol. 2014; 177:918­24.

62. Heusch G, Botker HE, Przyklenk K, Redington A, Yellon D. Remote ischemic conditioning. J Am Coll Cardiol. 2015;

65:177­95.

63. Heydarian HC, Madsen NL, Marino BS. Long­Term Outcomes in Congenital Heart Disease. In: DS Wheeler, HR

Wong, TP Shanley, eds. Pediatric Critical Care Medicine: Respiratory, Cardiovascular and Central Nervous Systems.

London, UK: Springer; 2014:417­440.

64. Hickey EJ, Nosikova Y, Pham­Hung E, Gritti M, Schwartz S, Caldarone CA, Redington A, Van Arsdell GS. NationalAeronautics and Space Administration "threat and error" model applied to pediatric cardiac surgery: errorcycles precede approximately 85% of patient deaths. J Thorac Cardiovasc Surg. 2015; 149:496­505; discussion505­7.

65. Hinton RB. Advances in the treatment of aortic valve disease: is it time for companion diagnostics?. Curr OpinPediatr. 2014; 26:546­52.

66. Hinton RB, Prakash A, Romp RL, Krueger DA, Knilans TK. Cardiovascular manifestations of tuberous sclerosiscomplex and summary of the revised diagnostic criteria and surveillance and management recommendationsfrom the international tuberous sclerosis consensus group. J Am Heart Assoc. 2014; 3:e001493.

67. Hong YH, Martin LA, Mulvaney JM, Burhans MS, Blaxall BC, Hinton RB. RNA extraction from healthy and failinghuman myocardium: a comparative evaluation. Biopreserv Biobank. 2015; 13:123­30.

68. Horsley M, Justice L, Moore R, Cooper DS. Parenteral Nutrition. In: R Munoz, EM da Cruz, VL Vetter, DS Cooper, eds.Handbook of Pediatric Cardiovascular Drugs. New York, NY: Springer; 2014:579­596.

69. Huang Y, Powers C, Madala SK, Greis KD, Haffey WD, Towbin JA, Purevjav E, Javadov S, Strauss AW, Khuchua Z.

Cardiac metabolic pathways affected in the mouse model of barth syndrome. PLoS One. 2015; 10:e0128561.

70. Huby AC, Mendsaikhan U, Takagi K, Martherus R, Wansapura J, Gong N, Osinska H, James JF, Kramer K, Saito K,

Robbins J, Khuchua Z, Towbin JA, Purevjav E. Disturbance in Z­disk mechanosensitive proteins induced by a

persistent mutant myopalladin causes familial restrictive cardiomyopathy. J Am Coll Cardiol. 2014; 64:2765­76.

71. Hussain T, Dragulescu A, Benson L, Yoo SJ, Meng H, Windram J, Wong D, Greiser A, Friedberg M, Mertens L, Seed

M, Redington A, Grosse­Wortmann L. Quantification and significance of diffuse myocardial fibrosis and diastolicdysfunction in childhood hypertrophic cardiomyopathy. Pediatr Cardiol. 2015; 36:970­8.

72. Hyldebrandt JA, Frederiksen CA, Heiberg J, Rothmann S, Redington AN, Schmidt MR, Ravn HB. Inotropic therapyfor right ventricular failure in newborn piglets: effect on contractility, hemodynamics, and interventricularinteraction. Pediatr Crit Care Med. 2014; 15:e327­33.

73. Inge TH, Siegel RM, Xanthakos SA. Weight loss maintenance: a hard nut to crack. JAMA Pediatr. 2014; 168:796­7.

74. Jolley M, Thiagarajan RR, Barrett CS, Salvin JW, Cooper DS, Rycus PT, Teele SA. Extracorporeal membraneoxygenation in patients undergoing superior cavopulmonary anastomosis. J Thorac Cardiovasc Surg. 2014;148:1512­8.

75. In: Jones MB, Klugman D, Fitzgerald RK, Kohr LM, Berger JT, Costello JM, Bronicki R, Cooper DS, eds.

(2015)Pediatric Cardiac Intensive Care Handbook. Washington D.C., Pediatirc Cardiac Intensive Care Books, LLC.

76. Kaddourah A, Uthup S, Madueme P, O'Rourke M, Hooper DK, Taylor MD, Colan SD, Jefferies JL, Rao MB, Goebel J.

Prevalence and predictors of aortic dilation as a novel cardiovascular complication in children with end­stagerenal disease. Clin Nephrol. 2015; 83:262­71.

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141. Prodhan P, Kalikivenkata G, Tang X, Thomas K, Byrnes J, Imamura M, Jaquiss RD, Garcia X, Frazier EA, Bhutta AT,

Dyamenahalli U. Risk factors for prolonged mechanical ventilation for children on ventricular assist devicesupport. Ann Thorac Surg. 2015; 99:1713­8.

142. Raman SV, Hor KN, Mazur W, Halnon NJ, Kissel JT, He X, Tran T, Smart S, McCarthy B, Taylor MD, Jefferies JL,

Rafael­Fortney JA, Lowe J, Roble SL, Cripe LH. Eplerenone for early cardiomyopathy in Duchenne musculardystrophy: a randomised, double­blind, placebo­controlled trial. Lancet Neurol. 2015; 14:153­61.

143. Ramby AL, Goodman DM, Wald EL, Weiss SL. Red Blood Cell Distribution Width as a Pragmatic Marker forOutcome in Pediatric Critical Illness. PLoS One. 2015; 10:e0129258.

144. Riesenkampff E, Messroghli DR, Redington AN, Grosse­Wortmann L. Myocardial T1 mapping in pediatric andcongenital heart disease. Circ Cardiovasc Imaging. 2015; 8:e002504.

145. Roche SL, Grosse­Wortmann L, Friedberg MK, Redington AN, Stephens D, Kantor PF. Exercise echocardiographydemonstrates biventricular systolic dysfunction and reveals decreased left ventricular contractile reserve inchildren after tetralogy of Fallot repair. J Am Soc Echocardiogr. 2015; 28:294­301.

146. Rohailla S, Clarizia N, Sourour M, Sourour W, Gelber N, Wei C, Li J, Redington AN. Acute, delayed and chronicremote ischemic conditioning is associated with downregulation of mTOR and enhanced autophagy signaling.PLoS One. 2014; 9:e111291.

147. Ryan TD, Jefferies JL, Wilmot I. Managing heart failure in adults with congenital heart disease. Curr Treat OptionsCardiovasc Med. 2015; 17:376.

148. Ryan TD, Jefferies JL, Zafar F, Lorts A, Morales DL. The evolving role of the total artificial heart in the managementof end­stage congenital heart disease and adolescents. ASAIO J. 2015; 61:8­14.

149. Sawyer JE, Chamberlain AR, Cooper DS. Pharmacogenomics. In: R Munoz, EM da Cruz, VL Vetter, DS Cooper, eds.Handbook of Pediatric Cardiovascular Drugs. New York, NY: Springer; 2014:49­58.

150. Schmidt MR, Redington A, Botker HE. Remote conditioning the heart overview: translatability and mechanism. Br

J Pharmacol. 2015; 172:1947­60.

151. Seckeler MD, Hirsch R, Beekman RH, 3rd, Goldstein BH. A new predictive equation for oxygen consumption inchildren and adults with congenital and acquired heart disease. Heart. 2015; 101:517­24.

152. Seckeler MD, Hirsch R, Beekman RH, 3rd, Goldstein BH. Validation of cardiac output using real­timemeasurement of oxygen consumption during cardiac catheterization in children under 3 years of age. CongenitHeart Dis. 2014; 9:307­15.

153. Seckeler MD, Villa C, Hirsch R. Percutaneous recanalization of occluded brachiocephalic vein­superior venacava connection after resection of mediastinal mass. JACC Cardiovasc Interv. 2014; 7:e69­70.

154. Sekar P, Heydarian HC, Cnota JF, Hornberger LK, Michelfelder EC. Diagnosis of congenital heart disease in an eraof universal prenatal ultrasound screening in southwest Ohio. Cardiol Young. 2015; 25:35­41.

155. Shah AS, Black S, Wadwa RP, Schmiege SJ, Fino NF, Talton JW, D'Agostino R, Jr., Hamman RF, Urbina EM, Dolan

LM, Daniels SR, Marcovina SM, Dabelea D. Insulin sensitivity and arterial stiffness in youth with type 1 diabetes:the SEARCH CVD study. J Diabetes Complications. 2015; 29:512­6.

156. Shah AS, Dabelea D, Talton JW, Urbina EM, D. Agostino RB J, Wadwa RP, Marcovina S, Hamman RF, Daniels SR,

Dolan LM. Smoking and arterial stiffness in youth with type 1 diabetes: the SEARCH Cardiovascular DiseaseStudy. J Pediatr. 2014; 165:110­6.

157. Siegel R, Eschenbacher W, Veldtman G. Fitness and cardiovascular health, not just a European issue. Heart.2015; 101:745­6.

158. Somers AE, Ware SM, Collins K, Jefferies JL, He H, Miller EM. Provision of cardiovascular genetic counselingservices: current practice and future directions. J Genet Couns. 2014; 23:976­83.

159. Sopariwala DH, Pant M, Shaikh SA, Goonasekera SA, Molkentin JD, Weisleder N, Ma J, Pan Z, Periasamy M.

Sarcolipin overexpression improves muscle energetics and reduces fatigue. J Appl Physiol (1985). 2015;118:1050­8.

160. Summer SS, Pratt JM, Koch EA, Anderson JB. Testing a novel method for measuring sleeping metabolic rate inneonates. Respir Care. 2014; 59:1095­100.

161. Tandon A, Jefferies JL, Villa CR, Hor KN, Wong BL, Ware SM, Gao Z, Towbin JA, Mazur W, Fleck RJ, Sticka JJ,

Benson DW, Taylor MD. Dystrophin genotype­cardiac phenotype correlations in Duchenne and Becker musculardystrophies using cardiac magnetic resonance imaging. Am J Cardiol. 2015; 115:967­71.

162. Tandon A, Taylor MD, Cripe LH. Co­occurring Duchenne muscular dystrophy and hypertrophic cardiomyopathy inan adult with atypical cardiac phenotype. Cardiol Young. 2015; 25:355­7.

163. Tandon A, Villa CR, Hor KN, Jefferies JL, Gao Z, Towbin JA, Wong BL, Mazur W, Fleck RJ, Sticka JJ, Benson DW,

Taylor MD. Myocardial fibrosis burden predicts left ventricular ejection fraction and is associated with age andsteroid treatment duration in duchenne muscular dystrophy. J Am Heart Assoc. 2015; 4.

164. Tanner BC, Wang Y, Robbins J, Palmer BM. Kinetics of cardiac myosin isoforms in mouse myocardium areaffected differently by presence of myosin binding protein­C. J Muscle Res Cell Motil. 2014; 35:267­78.

165. Thomas TO, Chandrakasan S, O'Brien M, Jefferies JL, Ryan TD, Wilmot I, Baker ML, Madueme PC, Morales D, Lorts

A. The use of a Berlin Heart EXCOR LVAD in a child receiving chemotherapy for Castleman's disease. PediatrTransplant. 2015; 19:E15­8.

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174. Varnell CD, Jr., Goldstein SL, Yee DL, Teruya J, Guyer KE, Siddiqui S, Jefferies JL. Age­related differences inurinary 11­dehydroxythromboxane B2 between infants, children, and adolescents: another example ofdevelopmental hemostasis?. Pediatr Blood Cancer. 2014; 61:2074­6.

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2014; 64:63­5.

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178. Villa CR, Ryan TD, Taylor MD, Jefferies JL. Response to: PLEC1 mutation associated with left ventricularhypertrabeculation/noncompaction. Neuromuscul Disord. 2015; 25:448­9.

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181. Villamizar­Schiller IT, Pabon LA, Hufnagel SB, Serrano NC, Karl G, Jefferies JL, Hopkin RJ, Prada CE. Neurologicaland cardiac responses after treatment with miglustat and a ketogenic diet in a patient with Sandhoff disease.

The Heart Institute includes the Divisions of Cardiology, Cardiothoracic Surgery, and Molecular Cardiovascular Biology

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Enkhsaikhan Purevjav, MD, PhD

PUBLISHED DEC. 30, 2014

Journal of the American College of Cardiology

Particularly in children, restrictive cardiomyopathy (RCM) has the poorest prognosis among heart muscle diseases.

The condition can lead to heart failure, arrhythmias and sudden cardiac death, with a 5-year mortality rate exceeding 70 percent.

To date, no medications have proven effective against RCM, which has left heart transplantation as the sole definitive treatment option. Now, fresh clues for finding new therapeutic targets are emerging thanks to a study led by Enkhsaikhan Purevjav, MD, PhD, a former researcher with the Heart Institute at Cincinnati Children’s who recently moved to the University of Tennessee Health Science Center.

Purevjav and former research fellow Anne-Cecile Huby, PhD, report that mutations in the myopalladin (MYPN) gene cause diverse cardiomyopathic phenotypes within a critical “final common pathway.” These mutations could explain why ACE inhibitors, beta-blockers and angiotensin receptor blockers are ineffective in patients with RCM. Their findings were published Dec. 30, 2014, in the Journal of the American College of Cardiology.

The study involved developing “knock-in” mice to carry mutations of the murine Mypn gene that would be homologous to the human MYPN-Q529X mutation. At six weeks, signs of restrictive physiology (RP) were detected in the mice carrying the mutation. At 12 weeks, the mice showed signs of impaired diastolic filling of the left ventricle, decreased T-wave duration, and other RCM symptoms.

“From these data, we hypothesize that the RCM phenotype results from persistence of dysfunctional truncated MypnQ526X protein and consequent multiple pathological ‘hits,’ ” Purevjav says.

MYPN is one of several genes that appear to be involved in RCM. These findings suggest that further studies on time-dependent expression changes in CARP, MLP, DES, and ERK1/2 proteins in patients with RCM may provide useful information for discovering diagnostic and therapeutic targets.

MYPN Mutations Affect Medication Effectiveness for Children with Restrictive Cardiomyopathy

Huby AC, Mendsaikhan U, Takagi K, Martherus R, Wansapura J, Gong N, Osinska H, James JF, Kramer K, Saito K, Robbins J, Khuchua Z, Towbin JA, Purevjav E. Disturbance in Z-disk mechanosensitive proteins induced by a persistent mutant myopalladin causes familial restrictive cardiomyopathy. J Am Coll Cardiol. 2014;64(25):2765-2776.

Heart Institute

70 CINCINNATI CHILDREN’S RESEARCH FOUNDATION

Electrocardiography in WT (A) and mutant (B-D) mice. MypnWT/Q526X mice display arrhythmias (arrows), including (B) premature atrial contractions, (C) premature ventricular contractions, and secondary atrioventricular block (D; 1:7 Wenkebach).

M-mode images of mitral valve (MV) movement (top) indicate an increase of early and late diastolic velocities (E/A) ratios in myopalladin (Mypn) WT/Q526X mice compared with wild-type (WT) mice or homozygotes, which became significant in 12-week-old heterozygote mutants compared with WT and homozygote mice. Cardiac magnetic resonance images (left) demonstrate enlarged left atria in MypnWT/Q526X mice (middle columns) compared with WT (left columns) and MypnQ526X (right columns) mice.

Heart Institute

MV

E/A

(AU)

3

2

1

0

WT MypnWTJQ526X

*

MypnQ526X

Myp

n W

T/Q5

26X

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CINCINNATICHILDRENS.ORG/RESEARCH 71

Shared Best Practices Can Reduce Mortality Risk in Complex Cardiac Surgery for Children With Heterotaxy Syndrome

PUBLISHED APRIL 23, 2015

The Annals of Thoracic Surgery

Even with the many advances in surgical repair of complex congenital heart malformations, total anomalous

pulmonary venous connection (TAPVC) repair in patients with heterotaxy syndrome carries a high mortality risk, particularly with functionally univentricular physiology.

Some of the most complex heart defects occur in children born with heterotaxy syndrome, in which abdominal organs form on the opposite side of the body. The cardiac lesions that result, which are almost always multiple, can vary widely in severity and potential outcome. An analysis led by David Morales, MD, and colleagues, published April 23, 2015, in the Annals of Thoracic Surgery is the first to provide national-level data on the mortality risks that certain patient populations face when receiving TAPVC repairs.

The study examined 261 operations for TAPVC repair in 258 patients from 65 medical centers. Overall, mortality was 38 percent. Heterotaxy patients undergoing TAPVC repair and requiring postcardiotomy ECMO had still higher operative mortality rates.

The findings suggest that early outcomes of TAPVC repair are significantly worse when patients with heterotaxy are in high-risk subgroups such as those who have functionally univentricular physiology.

“It is clear that TAPVC heterotaxy patients are very rare and present with extremely complicated anatomy and physiology that varies with patients, and therefore their care has to be individualized,” Morales says. “However, there are centers that have achieved higher levels of success treating these patients such as ours. The key next steps will be to understand and share best patient management practices, so that through education they can be spread throughout the country to improve outcomes for all of these children.”

Khan MS, Bryant R, 3rd, Kim SH, Hill KD, Jacobs JP, Jacobs ML, Pasquali SK, Morales DL. Contemporary Outcomes of Surgical Repair of Total Anomalous Pul-monary Venous Connection in Patients With Heterotaxy Syndrome. Ann Thorac Surg. 2015;99(6):2134-2140.

David Morales, MD

Heart Institute

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72 CINCINNATI CHILDREN’S RESEARCH FOUNDATION

Early outcomes of TAPVC repair are significantly worse when patients with heterotaxy are in high-risk subgroups, such as those who have functionally univentricular physiology. This table details outcomes from 261 operations for TAPVC repair involving 258 patients at 65 medical centers.

Heart Institute

OPERATIVE MORTALITY AMONG HIGH-RISK SUBGROUPS IN TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION HETEROTAXY PATIENTS

SubgroupsMortality With

Risk Factor, n (%)

Mortality Without Risk Factor, n (%)

P Value a

Single Ventricle

Mortality, n (%)

Non Single Ventricle

Mortality, n (%)P Value

b

Systemic-to-pulmonary artery shunt 38/84 (45) 62/177 (35) 0.134 33/71 (46) 5/13 (38) 0.764

Pulmonary atresia 26/64 (41) 74/197 (38) 0.660 24/51 (47) 2/13 (15) 0.057

Age at surgery of ≤48 hours 20/54 (37) 80/207 (39) 0.876 17/38 (45) 3/16 (19) 0.122

Weight at surgery <2.5 kg 13/31 (42) 87/230 (38) 0.696 10/21 (48) 3/10 (30) 0.452

Infracardiac TAPVC type 24/59 (41) 76/202 (38) 0.761 20/44 (45) 4/15 (27) 0.238

Postcardiotomy ECMO 13/20 (65) 87/241 (36) 0.015 10/13 (77) 3/7 (43) 0.174

Concomitant Norwood/DKS

procedure3/3 (100) 97/258 (38) 0.055 3/3 (100) ... ...

DKS = Damus-Kaye-Stansel; ECMO = extracorporeal membrane oxygenation; TAPVC = total anomalous pulmonary venous connection.

a — Comparison between patients with and without particular risk factor.

b — Comparison between single ventricle and non-single ventricle groups.

CINCINNATICHILDRENS.ORG/RESEARCH 73

PUBLISHED ONLINE JULY 13, 2014

Genes and Development

A new scientist at Cincinnati Children’s is beginning to unlock the genetic secrets of muscle cell fusion, an

advance that could have extensive implications for health.While working as a post-doctoral fellow at the University

of Texas Southwestern in Dallas, Doug Millay, PhD, and his mentor found the only muscle-specific protein known to be essential for fusion of embryonic and adult myoblasts. In a paper published in 2013 in Nature, they dubbed the gene “myomaker.” Then in a more recent paper in Genes and Development, the team showed that myomaker also is necessary for normal adult muscle cell regeneration.

Their work shows that myogenic basic helix-loop-helix (bHLH) transcription factors induce myomaker expression in satellite cells during acute and chronic muscle regeneration. Moreover, genetic deletion of myomaker in adult satellite cells completely abolishes muscle regeneration, resulting in severe muscle destruction after injury.

The ability of myomaker to promote fusion to adult muscle fibers and muscle regeneration suggests opportunities to enhance muscle repair through myomaker-directed cell-cell fusion. In mice, the myomaker gene can be expressed in non-muscle cells, which then allows these cells to fuse to skeletal muscle. This implies that myomaker might be useful as a delivery vehicle for future therapies to address muscle loss in Duchenne muscular dystrophy, cancer, AIDS, and COPD.

“In all of these conditions, restoring muscle cell growth may likely do even more than impact quality of life,” Millay says. “It could also slow the progression of the disease itself.”

Millay is one of two scientists at Cincinnati Children’s to be named Pew Scholars in 2015. He is continuing his work to more fully understand the machinery of muscle cell development.

Myomaker Protein Proves Essential for Muscle Regeneration

Millay DP, Sutherland LB, Bassel-Duby R, Olson EN. Myomaker is essential for muscle regeneration. Genes Dev. 2014;28(15):1641-1646.

Douglas Millay, PhD

Heart Institute

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Faculty 67Joint Appointment Faculty 2Research Fellows 33Research Students 24Support Personnel 146Direct Annual Grant Support $11.3MDirect Annual Industry Support $227,476Peer Reviewed Publications 194

74 CINCINNATI CHILDREN’S RESEARCH FOUNDATION

This confocal microscopic image shows that expression of the myomaker gene in fibroblasts (green) induces fusion with myoblasts (red) resulting in yellow/orange chimeric myotubes. Recent research shows that myomaker is necessary to promote fusion in skeletal muscle cells during prenatal development and later during the muscle repair process. In mice, myomaker also can be expressed in non-muscle cells, which implies that myomaker could serve as a delivery vehicle for future therapies to address muscle loss in conditions such as Duchenne muscular dystrophy and cancer.

Heart Institute

CINCINNATICHILDRENS.ORG/RESEARCH 75