1 CURRICULUM VITAE DAVID DUNBAR IVY, M.D. Current Position Professor of Pediatrics Chief of Pediatric Cardiology University of Colorado School of Medicine Professional Address Pediatric Cardiology Children's Hospital Colorado 13123 East 16th Avenue Box B100 Aurora, Colorado 80045 Phone: 720-777-3367 Fax: 720-777-7290 Education Davidson College, Davidson, NC BS in Biology, cum laude 1980-1984 Tulane University School of Medicine, New Orleans, LA MD 1984-1988 University of Colorado Health Sciences Center, Denver, CO 1988-1991 Pediatrics University of Colorado Health Sciences Center, Denver, CO 1991-1994 Pediatric Cardiology Fellowship Licensure Colorado State Board of Medical Examiners. License #31436 Board Certification FLEX Pediatrics Pediatric Cardiology June, 1988 #219149 #219149 Academic Appointments and Employment Instructor of Pediatrics, UCDHSC Assistant Professor of Pediatrics, UCDHSC Associate Professor of Pediatrics, UCDHSC Professor of Pediatrics, UCDHSC Award of Tenure, UCDHSC 1994-1996 1996-2001 2001-2007 2007 -present 2007-present 2 Honors /Awards Fellows Basic Research Award The Society For Pediatric Research Research paper 1st place Frontiers in Cardiology Children’s Choice Award Pulmonary Hypertension Program Selby’s Endowed Chair of Pediatric Cardiology Scientific Leadership Council Pulmonary Hypertension Association 5280 Magazine Top Docs Heart Who Cares Award- CHHI Patient Perception Award / Overall Top Performer PRC National Excellence in Healthcare Awards Board of Trustees Pulmonary Hypertension Association Board of Trustees Pediatric Congenital Heart Association 1993 1995 2001 2003 - present 2005-2013 2005-2008, 2010-2014 2008 2011-2012 2011-2014 2014 - present Society Memberships American College of Cardiology, Fellow American Academy of Pediatrics, Fellow American College of Chest Physicians, Fellow American Heart Association, Cardiovascular Disease in the Young Pediatric Cardiac Intensive Care Society Pulmonary Hypertension Association American Physiological Society Society For Pediatric Research Association for European Paediatric Cardiology Pulmonary Vascular Research Institute Institutional Committees Blood Utilization Review Committee, TCH 1999-2000 Search Committee, Cardiac ICU Director, TCH 1999-2002 Graduate Medical Education 2001 Internal review of Nuclear Medicine and Cardiac Electrophysiology Nominating Committee for The Children’s Hospital, 2000-2002 Imagine the Miracles, The Children’s Hospital Foundation 2003-2004 Search Committee, Chief Adult Cardiology, UCDHSC 2005-2006 Data Safety and Monitoring Board: “Effect of Statin Therapy 2010-2012 on Disease Progression in Children with ADPKD”, Melissa Cadnapaphornchai, M.D., PI 3 National Committees / Reviews NICHD Supplemental U01 Grants Society for Pediatric Research Young Investigator Award Committee American College of Cardiology, abstract reviewer Society of Pediatric Research, abstract reviewer NIH NHLBI Loan Repayment Program L30 Gilead Research Scholars Grant Review Board Guidelines for Pediatric Cardiology Training Programs Pulmonary Hypertension Academic Research Consortium NIH Workshop: Improving Outcomes in Pulmonary Hypertension Gilead Sciences Research Scholars Program Grant Review Committee American Heart Association, National Program Planning Committee Cardiovascular Medical Research and Education Fund Program Grant Review Committee Congressionally Directed Medical Research Program Department of Defense grant Reviewer Local Committees Executive Planning Committee, Denver American Heart Association Heart Ball Journal Review Boards Circulation Journal of the American College of Cardiology American Journal of Cardiology American Journal of Physiology British Journal of Pharmacology Biology of the Neonate Biology of Perinatal Medicine Critical Care Medicine Pediatric Pulmonology Catheterization and Cardiovascular Diagnosis Pediatric Research Annals of Thoracic Surgery American Journal of Respiratory and Critical Care Medicine Journal of Pediatrics Pediatrics European Respiratory Journal Journal of Applied Physiology Circulation Journal Japan Journal of Infection and Public Health Chest Journal of Critical Care Advances in Pulmonary Hypertension American Journal of Cardiovascular Drugs 2004 2005-2008 2006, 2011 2007 2009-2010 2009-2010 201020102011 2010-2012 2011-2013 2012 2013 2011-2013 4 Editorial Board American Thoracic Society Topic Editor, Pulmonary Circulation Journal Club Congenital Heart Disease: Clinical Studies from Fetus to Adulthood Advances in Pulmonary Hypertension Pulmonary Hypertension Association Frontiers in Cardiology 2002-2004 2005 to present 2011 to present 2011 to present Grant Support American Heart Association Fellowship Award 1993-1994 Title: Role of Endothelin in Perinatal Pulmonary Hypertension Source/Identifying No: American Heart Association of Colorado (CF-035-93) Role on Project: PI Effort: 75% Bugher Physician Scientist Training Program 1994-1996 Title: Endothelin in the Perinatal Pulmonary Circulation Source/Identifying No: Bugher Physician Scientist Training Program Role on Project: PI Effort: 75% Clinical Research Center, The Children’s Hospital of Denver 1995-1997 Title: Use of Intravascular Ultrasound in Pediatric Pulmonary Hypertension Source/Identifying No.: Clinical Research Center Role on Project: PI Effort: 75% Children’s Hospital Research Institute Career Development Award 1996-1998 Title: Endothelin in the Perinatal Pulmonary Circulation Source/Identifying No.: The Children’s Hospital Research Institute Role on Project: PI Effort: 75% March of Dimes Basil O’Connor Starter Scholar Award 1997-1999 Title: Role of Endothelin in Perinatal Pulmonary Hypertension Source/Identifying No.: March of Dimes Role on Project: PI Effort: 75% American Heart Association 1999-2001 Title: Role of the Endothelin B Receptor in a Model of Pulmonary Hypertension Source/Identifying No.: AHA, Desert / Mountain Affiliate (9951441Z) Role on Project: PI Effort: 75% 5 K08 Physician Scientist Award 1999-2003 Title: Role of Endothelin in Perinatal Pulmonary Vasoregulation Source/Identifying No.: NIH / NHLBI (K08 HL03823) Role on Project: PI Effort: 75% R01 National Institutes of Health 2001-2006 Title: Noninvasive Assessment of Pediatric Pulmonary Hypertension Source/Identifying No.: NIH / NHLBI (R01 HL067393) Role on Project: Collaborating Investigator PI on Grant: Robin Shandas, PhD Effort: 5% R38 US Department of Health and Human Services 2003-2006 Title: Infant Heart Transplantation from Non-Heart-Beating Donors: A Strategy to Reduce Waiting Mortality Source/Identifying No.: USDHHS (R38 OT0Role on Project: Collaborating Investigator PI on Grant: Mark Boucek, MD Effort: 10% U01 NIH/NHLBI Clinical Proteomics Programs Title: Clinical Proteomics Program in Lung Disease Source/Identifying No.: NIH / NHLBI (U01 HL081335) Role on Project: Collaborating Investigator PI on Grant: Frank Accurso, MD Effort: 3% 2005-2009 National Institutes of Health 2006-2009 Title: Sildenafil in Treatment of Sickle Cell Disease Associated Pulmonary Hypertension Source/Identifying No: NIH / NHLBI (HHSN268200617185C) Role on Project: Collaborating Investigator PI on Grant: David Badesch, MD Effort: 3% RC1 National Institutes of Health / NHLBI ARRA Challenge Grant 2009-2011 Title: Identification and validation of quantifiable biomarkers of vasodilator therapeutic response in children with IPAH Source/Identifying No: NIH / NHLBI (RC1HL099786) Role on Project: Collaborating Investigator PI on Grant: Allan Everett, MD Effort: 5% 6 P50 Specialized Centers of Clinically Oriented Research (SCCOR) 2006-2011 Title: Lung Vascular Disease in Infants and Children: Mechanisms and Treatment Source/Identifying No: NIH/NHLBI (P50 HL084923) Role on Project: CO-PI (Shandas) Module 3 PI on Grant: Kurt Stenmark, MD Effort: 10% R01 National Institutes of Health 2012-2017 Title: Functional and Biological Phenotyping of Pediatric Pulmonary Hypertension Source/Identifying No.: NIH / NHLBI (R01 HL114753) Role on Project: Collaborating Investigator PI on Grant: Robin Shandas, PhD Effort: 10% R24 National Institutes of Health 2012-2017 Title: National Biological Sample and Data Repository for PAH Source/Identifying No.: NIH / NHLBI (R24 HL105333) Role on Project: Collaborating Investigator PI on Grant: Bill Nichols, MD Effort: 5% U01 National Institutes of Health 2014-2018 Title: Data Fusion: A Sustainable, Scalable, Open Source Registry Advancing Pulmonary Vascular Disease Research Source/Identifying No.: NIH / NHLBI (U01HL121518) Role on Project: Collaborating Investigator PI on Grant: Steve Abman / Mandl MD Effort: 13% Major Scientific Interests Pulmonary Hypertension Pulmonary hypertension secondary to congenital heart disease Evaluation of pulmonary hypertension by echo Pulmonary Vascular Biology Endothelin Nitric Oxide Fetal Circulation Regulation of fetal pulmonary blood flow 7 Bibliography 1. Ivy DD, Abman SH. Gaining insights into pediatric pulmonary hypertensive disorders through patient registries. Am J Respir Crit Care Med. 2015 Jan 1;191(1):2-4. PMID: 25551339 2. Jone P, Ivy DD. Echocardiography in pediatric pulmonary hypertension. Front Pediatr. 2014 Nov 12;2:124. PMID: 25429362 3. Nies MK, Ivy DD, Everett AD. The untapped potential of proteomic analysis in pediatric pulmonary hypertension. Proteomics Clin Appl. 2014 Oct 28. PMID: 25348110 4. Nicolarsen J, Ivy D. Progress in the diagnosis and management of pulmonary hypertension in children. Curr Opin Pediatr. 2014 Oct;26(5):527-35. PMID: 25136947 5. Lopes AA, Barst RJ, Haworth SG, Rabinovitch M, Al Dabbagh M, Del Cerro MJ, Ivy D, Kashour T, Kumar K, Harikrishnan S, et al. Repair of congenital heart disease with associated pulmonary hypertension in children: what are the minimal investigative procedures? Consensus statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI). Pulm Circ. 2014 Jun;4(2):330-41. PubMed PMID: 25006452; PubMed Central PMCID: PMC4070778. 6. Di Maria MV, Younoszai AK, Mertens L, Landeck BF 2nd, Ivy DD, Hunter KS, Friedberg MK. RV stroke work in children with pulmonary arterial hypertension: estimation based on invasive haemodynamic assessment and correlation with outcomes. Heart. 2014 Sep;100(17):1342-7 PubMed PMID: 24780910. 7. Zijlstra WM, Douwes JM, Rosenzweig EB, Schokker S, Krishnan U, Roofthooft MT, Miller-Reed K, Hillege HL, Ivy DD, Berger RM. Survival differences in pediatric pulmonary arterial hypertension: clues to a better understanding of outcome and optimal treatment strategies. J Am Coll Cardiol. 2014;63(20):2159–69 PubMed PMID: 24681143. 8. Colvin KL, Dufva MJ, Delaney RP, Ivy DD, Stenmark KR, Yeager ME. Biomarkers for Pediatric Pulmonary Arterial Hypertension - A Call to Collaborate. Front Pediatr. 2014 Feb 3;2:7. PubMed PMID: 24551834 9. Barst RJ, Beghetti M, Pulido T, Layton G, Konourina I, Richardson H, Ivy DD, and the STARTS-2 Investigators. STARTS-2: Long-Term Survival With Oral Sildenafil Monotherapy in Treatment-Naïve Pediatric Pulmonary Arterial Hypertension. Circulation 2014;129(19):1914-1923 PubMed PMID: 24637559 10. Jone PN, Hinzman J, Wagner BD, Ivy DD, Younoszai A. Right Ventricular to Left Ventricular Diameter Ratio at End-Systole in Evaluating Outcomes in Children with Pulmonary Hypertension. J Am Soc Echocardiogr. 2014 Feb;27(2):172-8. PubMed PMID: 24325962 11. Barst RJ, Ivy DD, Foreman AJ, McGoon MD, Rosenzweig EB. Four- and Seven-Year Outcomes of Patients With Congenital Heart DiseaseAssociated Pulmonary Arterial Hypertension (from the REVEAL Registry). Am J Cardiol. 2014 Jan 1;113(1):147-55.PubMed PMID: 24176071 12. Vorhies EE, Ivy DD. Drug Treatment of Pulmonary Hypertension in Children. Paediatr Drugs. 2014 Feb;16(1):43-65 PubMed PMID: 8 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24114695. Ivy DD, Abman SH, Barst RJ, Berger RM, Bonnet D, Fleming TR, Haworth SG, Raj JU, Rosenzweig EB, Schulze Neick I, Steinhorn RH, Beghetti M. Pediatric Pulmonary Hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D117-26. PMID: 24355636 Rausch CM, Taylor AL, Ross H, Sillau S, Ivy DD. Ventilatory efficiency slope correlates with functional capacity, outcomes, and disease severity in pediatric patients with pulmonary hypertension. Int J Cardiol. 2013 Nov 30;169(6):445-8. PubMed PMID: 24144928. Wagner BD, Takatsuki S, Accurso FJ, Ivy DD. Evaluation of circulating proteins and hemodynamics towards predicting mortality in children with pulmonary arterial hypertension. PLoS One. 2013 Nov 20;8(11):e80235. PubMed PMID: 24278261. Colvin KL, Cripe PJ, Ivy DD, Stenmark KR, Yeager ME. Bronchusassociated Lymphoid Tissue in Pulmonary Hypertension Produces Pathologic Autoantibodies. Am J Respir Crit Care Med. 2013 Nov 1;188(9):1126-36. PubMed PMID: 24093638. Zhou Z, Malhotra SP, Yu X, Rutledge J, Rebeyka IM, Ross DB, Rausch C, Gu H, McCrindle B, Lacour-Gayet F, Ivy D, Li J. Moderate altitude is not associated with adverse postoperative outcomes for patients undergoing bidirectional cavopulmonary anastomosis and Fontan operation: a comparative study among Denver, Edmonton, and Toronto. J Thorac Cardiovasc Surg. 2013 Nov;146(5):1165-71. PubMed PMID: 23353110; PubMed Central PMCID: PMC3800264. Takatsuki S, Ivy DD. Current challenges in pediatric pulmonary hypertension. Semin Respir Crit Care Med. 2013 Oct;34(5):627-44. PubMed PMID: 24037630. Friesen RH, Nichols CS, Twite MD, Cardwell KA, Pan Z, Pietra B, Miyamoto SD, Auerbach SR, Darst JR, Ivy DD. The hemodynamic response to dexmedetomidine loading dose in children with and without pulmonary hypertension. Anesth Analg. 2013 Oct;117(4):953-9. PubMed PMID: 23960035; PubMed Central PMCID: PMC3830564. Maxey DM, Ivy DD, Ogawa MT, Feinstein JA. Food and Drug Administration (FDA) postmarket reported side effects and adverse events associated with pulmonary hypertension therapy in pediatric patients. Pediatr Cardiol. 2013 Oct;34(7):1628-36. PubMed PMID: 23532466; PubMed Central PMCID: PMC3783558. Truong U, Fonseca B, Dunning J, Burgett S, Lanning C, Ivy DD, Shandas R, Hunter K, Barker AJ. Wall shear stress measured by phase contrast cardiovascular magnetic resonance in children and adolescents with pulmonary arterial hypertension. J Cardiovasc Magn Reson. 2013 Sep 13;15(1):81. PubMed PMID: 24034144. Beghetti M, Berger RM, Schulze-Neick I, Day RW, Pulido T, Feinstein J, Barst RJ, Humpl T; TOPP Registry Investigators. Diagnostic evaluation of paediatric pulmonary hypertension in current clinical practice. Eur Respir J. 2013 Sep;42(3):689-700. PubMed PMID: 23563261. Wessel DL, Berger F, Li JS, Dahnert I, Rakhit A, Fontecave S, Newburger JW; CLARINET Investigators. Clopidogrel in infants with systemic-topulmonary-artery shunts. N Engl J Med. 2013 Jun 20;368(25):2377-84. 9 24. 25. 26. 27. 28. 29. 30. 31. 32. 33. 34. PubMed PMID: 23782178. Siehr SL, Ivy DD, Miller-Reed K, Ogawa M, Rosenthal DN, Feinstein JA. Children with pulmonary arterial hypertension and prostanoid therapy: long-term hemodynamics. J Heart Lung Transplant. 2013 May;32(5):54652. PubMed PMID: 23453572; PubMed Central PMCID: PMC3760159. Takatsuki S, Parker DK, Doran AK, Friesen RH, Ivy DD. Acute pulmonary vasodilator testing with inhaled treprostinil in children with pulmonary arterial hypertension. Pediatr Cardiol. 2013 Apr;34(4):1006-12. PubMed PMID: 23184020; PubMed Central PMCID: PMC3608847. Adatia I, Haworth SG, Wegner M, Barst RJ, Ivy D, Stenmark KR, Karkowsky A, Rosenzweig E, Aguilar C. Clinical trials in neonates and children: Report of the pulmonary hypertension academic research consortium pediatric advisory committee. Pulm Circ. 2013 Jan;3(1):25266. PubMed PMID: 23662203; PubMed Central PMCID: PMC3641736. Grieve AP, Chow SC, Curram J, Dawe S, Harnisch LO, Henig NR, Hung HM, Ivy DD, Kawut SM, Rahbar MH, Xiao S, Wilkins MR. Advancing clinical trial design in pulmonary hypertension. Pulm Circ. 2013 Jan;3(1):217-25. PubMed PMID: 23662200; PubMed Central PMCID: PMC3641733. Takatsuki S, Rosenzweig EB, Zuckerman W, Brady D, Calderbank M, Ivy DD. Clinical safety, pharmacokinetics, and efficacy of ambrisentan therapy in children with pulmonary arterial hypertension. Pediatr Pulmonol. 2013 Jan;48(1):27-34. PubMed PMID: 22511577; PubMed Central PMCID: PMC3412194. Krishnan U, Takatsuki S, Ivy DD, Kerstein J, Calderbank M, Coleman E, Rosenzweig EB. Effectiveness and safety of inhaled treprostinil for the treatment of pulmonary arterial hypertension in children. Am J Cardiol. 2012 Dec 1;110(11):1704-9. PubMed PMID: 22917554; PubMed Central PMCID: PMC3508003. Takatsuki S, Nakayama T, Jone PN, Wagner BD, Naoi K, Ivy DD, Saji T. Tissue Doppler imaging predicts adverse outcome in children with idiopathic pulmonary arterial hypertension. J Pediatr. 2012 Dec;161(6):1126-31. PubMed PMID: 22748515; PubMed Central PMCID: PMC3508005. Yeager ME, Colvin KL, Everett AD, Stenmark KR, Ivy DD. Plasma proteomics of differential outcome to long-term therapy in children with idiopathic pulmonary arterial hypertension. Proteomics Clin Appl. 2012 Jun;6(5-6):257-67. PubMed PMID: 22653875; PubMed Central PMCID: PMC3569520. Takatsuki S, Darst JR, Das BB, Fagan TE, Wolfe R, Ivy DD. Clinical manifestations and long-term follow-up in pediatric patients living at altitude with isolated pulmonary artery of ductal origin. Pediatr Cardiol. 2012 Jun;33(5):775-81. PubMed PMID: 22430374; PubMed Central PMCID: PMC3360790. Takatsuki S, Calderbank M, Ivy DD. Initial experience with tadalafil in pediatric pulmonary arterial hypertension. Pediatr Cardiol. 2012 Jun;33(5):683-8. PubMed PMID: 22402804; PubMed Central PMCID: PMC3366091. Stoica S, Carpenter E, Campbell D, Mitchell M, da Cruz E, Ivy D, Lacour- 10 35. 36. 37. 38. 39. 40. 41. 42. 43. 44. 45. Gayet F. Morbidity of the arterial switch operation. Ann Thorac Surg. 2012 Jun;93(6):1977-83. PubMed PMID: 22365263; PubMed Central PMCID: PMC3381339. Takatsuki S, Wagner BD, Ivy DD. B-type natriuretic peptide and aminoterminal pro-B-type natriuretic peptide in pediatric patients with pulmonary arterial hypertension. Congenit Heart Dis. 2012 May-Jun;7(3):259-67. PubMed PMID: 22325151; PubMed Central PMCID: PMC3355223. Yeager ME, Nguyen CM, Belchenko DD, Colvin KL, Takatsuki S, Ivy DD, Stenmark KR. Circulating myeloid-derived suppressor cells are increased and activated in pulmonary hypertension. Chest. 2012 Apr;141(4):944-52. PubMed PMID: 21940769; PubMed Central PMCID: PMC3318947. Yeager ME, Reddy MB, Nguyen CM, Colvin KL, Ivy DD, Stenmark KR. Activation of the unfolded protein response is associated with pulmonary hypertension. Pulm Circ. 2012 Apr-Jun;2(2):229-40. PubMed PMID: 22837864; PubMed Central PMCID: PMC3401877. Barst RJ, Channick R, Ivy D, Goldstein B. Clinical perspectives with longterm pulsed inhaled nitric oxide for the treatment of pulmonary arterial hypertension. Pulm Circ. 2012 Apr-Jun;2(2):139-47. PubMed PMID: 22837854; PubMed Central PMCID: PMC3401867. Takatsuki S, Ivy DD, Nuss R. Correlation of N-terminal fragment of B-type natriuretic peptide levels with clinical, laboratory, and echocardiographic abnormalities in children with sickle cell disease. J Pediatr. 2012 Mar;160(3):428-433.e1. PubMed PMID: 22048047; PubMed Central PMCID: PMC3274616. Ivy D. Advances in pediatric pulmonary arterial hypertension. Curr Opin Cardiol. 2012 Mar;27(2):70-81. PubMed PMID: 22274573; PubMed Central PMCID: PMC3319159. Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, Bonnet D, Schulze-Neick I, Barst RJ. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet. 2012 Feb 11;379(9815):537-46. PubMed PMID: 22240409; PubMed Central PMCID: PMC3426911. Barst RJ, Ivy DD, Gaitan G, Szatmari A, Rudzinski A, Garcia AE, Sastry BK, Pulido T, Layton GR, Serdarevic-Pehar M, Wessel DL. A randomized, double-blind, placebo-controlled, dose-ranging study of oral sildenafil citrate in treatment-naive children with pulmonary arterial hypertension. Circulation. 2012 Jan 17;125(2):324-34. PubMed PMID: 22128226. Barst RJ, McGoon MD, Elliott CG, Foreman AJ, Miller DP, Ivy DD. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation. 2012 Jan 3;125(1):113-22. PubMed PMID: 22086881. Feinstein JA, Benson DW, Dubin AM, Cohen MS, Maxey DM, Mahle WT, Pahl E, Villafañe J, Bhatt AB, Peng LF, Johnson BA, Marsden AL, Daniels CJ, Rudd NA, Caldarone CA, Mussatto KA, Morales DL, Ivy DD, Gaynor JW, Tweddell JS, Deal BJ, Furck AK, Rosenthal GL, Ohye RG, Ghanayem NS, Cheatham JP, Tworetzky W, Martin GR. Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol. 2012 Jan 3;59(1 Suppl):S1-42. PubMed PMID: 22192720. Yeager ME, Belchenko DD, Nguyen CM, Colvin KL, Ivy DD, Stenmark KR. 11 46. 47. 48. 49. 50. 51. 52. 53. 54. 55. 56. Endothelin-1, the unfolded protein response, and persistent inflammation: role of pulmonary artery smooth muscle cells. Am J Respir Cell Mol Biol. 2012 Jan;46(1):14-22. PubMed PMID: 21778413; PubMed Central PMCID: PMC3262656. Yeager ME, Nguyen CM, Belchenko DD, Colvin KL, Takatsuki S, Ivy DD, Stenmark KR. Circulating fibrocytes are increased in children and young adults with pulmonary hypertension. Eur Respir J. 2012 Jan;39(1):104-11. PubMed PMID: 21700605; PubMed Central PMCID: PMC3319160. Duncan M, Wagner BD, Murray K, Allen J, Colvin K, Accurso FJ, Ivy DD. Circulating cytokines and growth factors in pediatric pulmonary hypertension. Mediators Inflamm. 2012;2012:143428. PubMed PMID: 23316102; PubMed Central PMCID: PMC3536060. Law YM, Mack CL, Sokol RJ, Rice M, Parsley L, Ivy D. Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: pulmonary hypertension and pulmonary vascular dilatation. Pediatr Transplant. 2011 Dec;15(8):E162-8. PubMed PMID: 20565694; PubMed Central PMCID: PMC3203339. Lammers AE, Adatia I, Cerro MJ, Diaz G, Freudenthal AH, Freudenthal F, Harikrishnan S, Ivy D, Lopes AA, Raj JU, Sandoval J, Stenmark K, Haworth SG. Functional classification of pulmonary hypertension in children: Report from the PVRI pediatric taskforce, Panama 2011. Pulm Circ. 2011 Aug 2;1(2):280-285. PubMed PMID: 21874157; PubMed Central PMCID: PMC3161406. Takatsuki S, Soep JB, Calderbank M, Ivy DD. Connective tissue disease presenting with signs and symptoms of pulmonary hypertension in children. Pediatr Cardiol. 2011 Aug;32(6):828-33. PubMed PMID: 21448619; PubMed Central PMCID: PMC3142789. Abman SH, Ivy DD. Recent progress in understanding pediatric pulmonary hypertension. Curr Opin Pediatr. 2011 Jun;23(3):298-304. PubMed PMID: 21572384; PubMed Central PMCID: PMC3128451. Mallory GB Jr, Hanna BD, Ivy DD, Shardonofsky F, Farber HJ. Management and Controversies in Pediatric Pulmonary Hypertension. Pediatr Allergy Immunol Pulmonol. 2011 Mar 4;22(4):139-150. PubMed PMID: 21766047; PubMed Central PMCID: PMC3136886. Barst RJ, Ertel SI, Beghetti M, Ivy DD. Pulmonary arterial hypertension: a comparison between children and adults. Eur Respir J. 2011 Mar;37(3):665-77. PubMed PMID: 21357924; PubMed Central PMCID: PMC3128436. Olin J, Abman S, Grady R, Ivy D, Lovell M, Partrick D, Balasubramaniam V. A 7-year-old with pulmonary hypertension. BMJ Case Rep. 2011;2011:bcr0220113843. PubMed PMID: 21897452; PubMed Central PMCID: PMC3082056. Cerro MJ, Abman S, Diaz G, Freudenthal AH, Freudenthal F, Harikrishnan S, Haworth SG, Ivy D, Lopes AA, Raj JU, Sandoval J, Stenmark K, Adatia I. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ. 2011;1(2):286-298. PubMed PMID: 21874158; PubMed Central PMCID: PMC3161725. Phelps CM, Tissot C, Buckvold S, Gralla J, Ivy DD, Pietra BA, Miyamoto 12 57. 58. 59. 60. 61. 62. 63. 64. 65. 66. 67. SD. Outcome of acute graft rejection associated with hemodynamic compromise in pediatric heart transplant recipients. Pediatr Cardiol. 2011 Jan;32(1):1-7. PubMed PMID: 20963408; PubMed Central PMCID: PMC3120936. Hunter KS, Feinstein JA, Ivy DD, Shandas R. Computational Simulation of the Pulmonary Arteries and its Role in the Study of Pediatric Pulmonary Hypertension. Prog Pediatr Cardiol. 2010 Dec 1;30(1-2):63-69. PubMed PMID: 21499523; PubMed Central PMCID: PMC3076725. Hill KD, Lim DS, Everett AD, Ivy DD, Moore JD. Assessment of pulmonary hypertension in the pediatric catheterization laboratory: current insights from the Magic registry. Catheter Cardiovasc Interv. 2010 Nov 15;76(6):865-73. PubMed PMID: 20549685; PubMed Central PMCID: PMC3116922. Ivy DD, Rosenzweig EB, Lemarie JC, Brand M, Rosenberg D, Barst RJ. Long-term outcomes in children with pulmonary arterial hypertension treated with bosentan in real-world clinical settings. Am J Cardiol. 2010 Nov 1;106(9):1332-8. PubMed PMID: 21029834; PubMed Central PMCID: PMC2966874. Tissot C, Ivy DD, Beghetti M. Medical therapy for pediatric pulmonary arterial hypertension. J Pediatr. 2010 Oct;157(4):528-32. PubMed PMID: 20656296; PubMed Central PMCID: PMC3127257. Ivy D, Saji BT. A New Era in Medical Management of Severe Pediatric Pulmonary Arterial Hypertension. Nihon Shoni Junkanki Gakkai Zasshi. 2010 Jun 4;26(3):206-218. PubMed PMID: 23264720; PubMed Central PMCID: PMC3527842. Tian L, Hunter KS, Kirby KS, Ivy DD, Shandas R. Measurement uncertainty in pulmonary vascular input impedance and characteristic impedance estimated from pulsed-wave Doppler ultrasound and pressure: clinical studies on 57 pediatric patients. Physiol Meas. 2010 Jun;31(6):729-48. PubMed PMID: 20410558; PubMed Central PMCID: PMC3010409. Ivy DD. Prostacyclin in the intensive care setting. Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S41-5. PubMed PMID: 20216163; PubMed Central PMCID: PMC3116926. Ye M, Coldren C, Liang X, Mattina T, Goldmuntz E, Benson DW, Ivy D, Perryman MB, Garrett-Sinha LA, Grossfeld P. Deletion of ETS-1, a gene in the Jacobsen syndrome critical region, causes ventricular septal defects and abnormal ventricular morphology in mice. Hum Mol Genet. 2010 Feb 15;19(4):648-56. PMID: 19942620; PubMed Central PMCID: PMC2807373. Ivy DD, Feinstein JA, Humpl T, Rosenzweig EB. Non-congenital heart disease associated pediatric pulmonary arterial hypertension. Prog Pediatr Cardiol. 2009 Dec 1;27(1-2):13-23. PubMed PMID: 21852894; PubMed Central PMCID: PMC3156479. Rosenzweig EB, Feinstein JA, Humpl T, Ivy DD. Pulmonary arterial hypertension in children: Diagnostic work-up and challenges. Prog Pediatr Cardiol. 2009 Dec;27(1):4-11. PubMed PMID: 21691442; PubMed Central PMCID: PMC3117303. Beghetti M, Haworth SG, Bonnet D, Barst RJ, Acar P, Fraisse A, Ivy DD, 13 68. 69. 70. 71. 72. 73. 74. 75. 76. Jais X, Schulze-Neick I, Galie N, Morganti A, Dingemanse J, Kusic-Pajic A, Berger RM. Pharmacokinetic and clinical profile of a novel formulation of bosentan in children with pulmonary arterial hypertension: the FUTURE-1 study. Br J Clin Pharmacol. 2009 Dec;68(6):948-55. PubMed PMID: 20002090; PubMed Central PMCID: PMC2805863. Whitworth JR, Ivy DD, Gralla J, Narkewicz MR, Sokol RJ. Pulmonary vascular complications in asymptomatic children with portal hypertension. J Pediatr Gastroenterol Nutr. 2009 Nov;49(5):607-12. PubMed PMID: 19820411; PubMed Central PMCID: PMC2783348. Ivy DD, Calderbank M, Wagner BD, Dolan S, Nyquist AC, Wade M, Nickels WM, Doran AK. Closed-hub systems with protected connections and the reduction of risk of catheter-related bloodstream infection in pediatric patients receiving intravenous prostanoid therapy for pulmonary hypertension. Infect Control Hosp Epidemiol. 2009 Sep;30(9):823-9. PubMed PMID: 19637961; PubMed Central PMCID: PMC3117306. Tissot C, Buckvold S, Phelps CM, Ivy DD, Campbell DN, Mitchell MB, da Cruz SO, Pietra BA, Miyamoto SD. Outcome of extracorporeal membrane oxygenation for early primary graft failure after pediatric heart transplantation. J Am Coll Cardiol. 2009 Aug 18;54(8):730-7. PubMed PMID: 19679252; PubMed Central PMCID: PMC3117294. Miyamoto SD, Pietra BA, Chan KC, Ivy DD, Mashburn C, Campbell DN, Mitchell MB, Boucek MM. Long-term outcome of palliation with internal pulmonary artery bands after primary heart transplantation for hypoplastic left heart syndrome. Pediatr Cardiol. 2009 May;30(4):419-25. PubMed PMID: 19365660; PubMed Central PMCID: PMC3117302. Morchi GS, Ivy DD, Duster MC, Claussen L, Chan KC, Kay J. Sildenafil Increases Systemic Saturation and Reduces Pulmonary Artery Pressure in Patients with Failing Fontan Physiology. Congenit Heart Dis. 2009 Apr;4(2):107-111. PubMed PMID: 21866231; PubMed Central PMCID: PMC3159127. Goldberg SP, McCanta AC, Campbell DN, Carpenter EV, Clarke DR, da Cruz E, Ivy DD, Lacour-Gayet FG. Implications of incising the ventricular septum in double outlet right ventricle and in the Ross-Konno operation. Eur J Cardiothorac Surg. 2009 Apr;35(4):589-93. PubMed PMID: 19269838; PubMed Central PMCID: PMC3117298. Coldren CD, Lai Z, Shragg P, Rossi E, Glidewell SC, Zuffardi O, Mattina T, Ivy DD, Curfs LM, Mattson SN, Riley EP, Treier M, Grossfeld PD. Chromosomal microarray mapping suggests a role for BSX and Neurogranin in neurocognitive and behavioral defects in the 11q terminal deletion disorder (Jacobsen syndrome). Neurogenetics. 2009 Apr;10(2):89-95. PubMed PMID: 18855024; PubMed Central PMCID: PMC3050515. Mourani PM, Sontag MK, Ivy DD, Abman SH. Effects of long-term sildenafil treatment for pulmonary hypertension in infants with chronic lung disease. J Pediatr. 2009 Mar;154(3):379-84. PubMed PMID: 18950791; PubMed Central PMCID: PMC2783835. Bernus A, Wagner BD, Accurso F, Doran A, Kaess H, Ivy DD. Brain natriuretic peptide levels in managing pediatric patients with pulmonary arterial hypertension. Chest. 2009 Mar;135(3):745-51. PubMed PMID: 14 77. 78. 79. 80. 81. 82. 83. 84. 85. 86. 18849405; PubMed Central PMCID: PMC2715554. Phelps CM, Mengshol S, Ivy DD. A curious isolated cystic lesion of the membranous atrioventricular septum. Cardiol Young. 2008 Dec;18(6):6314. PubMed PMID: 18950547; PubMed Central PMCID: PMC3118089. Malhotra SP, Lacour-Gayet F, Campbell DN, Miyamoto S, Clarke DR, Dines ML, Ivy DD, Mitchell MB. Outcomes of reparative and transplantation strategies for multilevel left heart obstructions with mitral stenosis. Ann Thorac Surg. 2008 Oct;86(4):1305-9. PubMed PMID: 18805182; PubMed Central PMCID: PMC3128450. Malhotra SP, Ivy DD, Mitchell MB, Campbell DN, Dines ML, Miyamoto S, Kay J, Clarke DR, Lacour-Gayet F. Performance of cavopulmonary palliation at elevated altitude: midterm outcomes and risk factors for failure. Circulation. 2008 Sep 30;118(14 Suppl):S177-81. PubMed PMID: 18824752; PubMed Central PMCID: PMC3129266. Doran AK, Ivy DD, Barst RJ, Hill N, Murali S, Benza RL; Scientific Leadership Council of the Pulmonary Hypertension Association. Guidelines for the prevention of central venous catheter-related blood stream infections with prostanoid therapy for pulmonary arterial hypertension. Int J Clin Pract Suppl. 2008 Jul;(160):5-9. PubMed PMID: 18638170; PubMed Central PMCID: PMC3129264. Villavicencio K, Ivy D, Cole L, Nuss R. Symptomatic pulmonary hypertension in a child with sickle cell disease. J Pediatr. 2008 Jun;152(6):879-81. doi: 10.1016/j.jpeds.2008.01.042. PubMed PMID: 18492535; PubMed Central PMCID: PMC3128440. Hunter KS, Gross JK, Lanning CJ, Kirby KS, Dyer KL, Ivy DD, Shandas R. Noninvasive methods for determining pulmonary vascular function in children with pulmonary arterial hypertension: application of a mechanical oscillator model. Congenit Heart Dis. 2008 Mar-Apr;3(2):106-16. PubMed PMID: 18380759; PubMed Central PMCID: PMC3116924. Mourani PM, Sontag MK, Younoszai A, Ivy DD, Abman SH. Clinical utility of echocardiography for the diagnosis and management of pulmonary vascular disease in young children with chronic lung disease. Pediatrics. 2008 Feb;121(2):317-25. PubMed PMID: 18245423; PubMed Central PMCID: PMC3121163. Mourani PM, Ivy DD, Rosenberg AA, Fagan TE, Abman SH. Left ventricular diastolic dysfunction in bronchopulmonary dysplasia. J Pediatr. 2008 Feb;152(2):291-3. PubMed PMID: 18206706; PubMed Central PMCID: PMC2259289. Ivy DD, Doran AK, Smith KJ, Mallory GB Jr, Beghetti M, Barst RJ, Brady D, Law Y, Parker D, Claussen L, Abman SH. Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension. J Am Coll Cardiol. 2008 Jan 15;51(2):161-9. PubMed PMID: 18191742; PubMed Central PMCID: PMC3125969. Hunter KS, Lee PF, Lanning CJ, Ivy DD, Kirby KS, Claussen LR, Chan KC, Shandas R. Pulmonary vascular input impedance is a combined measure of pulmonary vascular resistance and stiffness and predicts clinical outcomes better than pulmonary vascular resistance alone in pediatric patients with pulmonary hypertension. Am Heart J. 2008 Jan;155(1):166-74. PubMed PMID: 18082509; PubMed Central PMCID: 15 87. 88. 89. 90. 91. 92. 93. 94. 95. 96. 97. PMC3139982. Yetman AT, Beroukhim RS, Ivy DD, Manchester D. Importance of the clinical recognition of Loeys-Dietz syndrome in the neonatal period. Pediatrics. 2007 May;119(5):e1199-202. PubMed PMID: 17470566; PubMed Central PMCID: PMC3131201. Zhang Y, Dunn ML, Hunter KS, Lanning C, Ivy DD, Claussen L, Chen SJ, Shandas R. Application of a microstructural constitutive model of the pulmonary artery to patient-specific studies: validation and effect of orthotropy. J Biomech Eng. 2007 Apr;129(2):193-201. PubMed PMID: 17408324; PubMed Central PMCID: PMC3114451. Ivy DD, Claussen L, Doran A. Transition of stable pediatric patients with pulmonary arterial hypertension from intravenous epoprostenol to intravenous treprostinil. Am J Cardiol. 2007 Mar 1;99(5):696-8. PubMed PMID: 17317374; PubMed Central PMCID: PMC1974790. Carmosino MJ, Friesen RH, Doran A, Ivy DD. Perioperative complications in children with pulmonary hypertension undergoing noncardiac surgery or cardiac catheterization. Anesth Analg. 2007 Mar;104(3):521-7. PubMed PMID: 17312201; PubMed Central PMCID: PMC1934984. Drexler ES, Quinn TP, Slifka AJ, McCowan CN, Bischoff JE, Wright JE, Ivy DD, Shandas R. Comparison of mechanical behavior among the extrapulmonary arteries from rats. J Biomech. 2007;40(4):812-9. PubMed PMID: 16682044. Artrip JH, Campbell DN, Ivy DD, Almodovar MC, Chan KC, Mitchell MB, Clarke DR, Lacour-Gayet F. Birth weight and complexity are significant factors for the management of hypoplastic left heart syndrome. Ann Thorac Surg. 2006 Oct;82(4):1252-7. PubMed PMID: 16996917. Hunter KS, Lanning CJ, Chen SY, Zhang Y, Garg R, Ivy DD, Shandas R. Simulations of congenital septal defect closure and reactivity testing in patient-specific models of the pediatric pulmonary vasculature: A 3D numerical study with fluid-structure interaction. J Biomech Eng. 2006 Aug;128(4):564-72. PubMed PMID: 16813447. Dyer K, Lanning C, Das B, Lee PF, Ivy DD, Valdes-Cruz L, Shandas R. Noninvasive Doppler tissue measurement of pulmonary artery compliance in children with pulmonary hypertension. J Am Soc Echocardiogr. 2006 Apr;19(4):403-12. PubMed PMID: 16581479; PubMed Central PMCID: PMC2003158. Resnik E, Herron J, Fu R, Ivy DD, Cornfield DN. Oxygen tension modulates the expression of pulmonary vascular BKCa channel alphaand beta-subunits. Am J Physiol Lung Cell Mol Physiol. 2006 Apr;290(4):L761-L768. PubMed PMID: 16284215. Dyer KL, Pauliks LB, Das B, Shandas R, Ivy D, Shaffer EM, Valdes-Cruz LM. Use of myocardial performance index in pediatric patients with idiopathic pulmonary arterial hypertension. J Am Soc Echocardiogr. 2006 Jan;19(1):21-7. PubMed PMID: 16423665; PubMed Central PMCID: PMC1934985. Ihida-Stansbury K, McKean DM, Gebb SA, Martin JF, Stevens T, Nemenoff R, Vaughn J, Lane K, Loyd J, Wheeler L, Morrell NW, Ivy D, Jones PL. Regulation and functions of the paired-related homeobox gene PRX1 in pulmonary vascular development and disease. Chest. 2005 16 Dec;128(6 Suppl):591S. PubMed PMID: 16373852. 98. Imamura M, Vitello AM, Limbird JN, Ivy DD, Fallon MB, Carter EP. Endothelin-B receptor overexpression prevents hypoxic pulmonary hypertension in cirrhotic rats. Chest. 2005 Dec;128(6 Suppl):580S-581S. PubMed PMID: 16373843. 99. Rosenzweig EB, Ivy DD, Widlitz A, Doran A, Claussen LR, Yung D, Abman SH, Morganti A, Nguyen N, Barst RJ. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol. 2005 Aug 16;46(4):697-704. PubMed PMID: 16098438. 100. Zhang Y, Dunn ML, Drexler ES, McCowan CN, Slifka AJ, Ivy DD, Shandas R. A microstructural hyperelastic model of pulmonary arteries under normo- and hypertensive conditions. Ann Biomed Eng. 2005 Aug;33(8):1042-52. PubMed PMID: 16133913. 101. Condino AA, Ivy DD, O'Connor JA, Narkewicz MR, Mengshol S, Whitworth JR, Claussen L, Doran A, Sokol RJ. Portopulmonary hypertension in pediatric patients. J Pediatr. 2005 Jul;147(1):20-6. PubMed PMID: 16027687; PubMed Central PMCID: PMC3326402. 102. Ivy DD, McMurtry IF, Colvin K, Imamura M, Oka M, Lee DS, Gebb S, Jones PL. Development of occlusive neointimal lesions in distal pulmonary arteries of endothelin B receptor-deficient rats: a new model of severe pulmonary arterial hypertension. Circulation. 2005 Jun 7;111(22):2988-96. PubMed PMID: 15927975; PubMed Central PMCID: PMC1934986. 103. Das BB, Narkewicz MR, Sokol RJ, Chen YT, Bali D, Li SC, Matthews MR, Mierau GW, Ivy DD. Amylopectinosis disease isolated to the heart with normal glycogen branching enzyme activity and gene sequence. Pediatr Transplant. 2005 Apr;9(2):261-5. PubMed PMID: 15787805. 104. Kinsella JP, Ivy DD, Abman SH. Pulmonary vasodilator therapy in congenital diaphragmatic hernia: acute, late, and chronic pulmonary hypertension. Semin Perinatol. 2005 Apr;29(2):123-8. PubMed PMID: 16052736. 105. Yetman AT, Taylor AL, Doran A, Ivy DD. Utility of cardiopulmonary stress testing in assessing disease severity in children with pulmonary arterial hypertension. Am J Cardiol. 2005 Mar 1;95(5):697-9. PubMed PMID: 15721127. 106. Imamura M, Luo B, Limbird J, Vitello A, Oka M, Ivy DD, McMurtry IF, Garat CV, Fallon MB, Carter EP. Hypoxic pulmonary hypertension is prevented in rats with common bile duct ligation. J Appl Physiol (1985). 2005 Feb;98(2):739-47. PubMed PMID: 15516365. 107. Rashid A, Ivy D. Severe paediatric pulmonary hypertension: new management strategies. Arch Dis Child. 2005 Jan;90(1):92-8. PubMed PMID: 15613526; PubMed Central PMCID: PMC1720073. 108. Das BB, Wolfe RR, Chan KC, Larsen GL, Reeves JT, Ivy D. High-altitude pulmonary edema in children with underlying cardiopulmonary disorders and pulmonary hypertension living at altitude. Arch Pediatr Adolesc Med. 2004 Dec;158(12):1170-6. PubMed PMID: 15583103. 109. Mitchell MB, Campbell DN, Ivy D, Boucek MM, Sondheimer HM, Pietra B, Das BB, Coll JR. Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure. J Thorac Cardiovasc Surg. 17 2004 Nov;128(5):693-702. PubMed PMID: 15514596. 110. Hartsfield CL, McMurtry IF, Ivy DD, Morris KG, Vidmar S, Rodman DM, Fagan KA. Cardioprotective and vasomotor effects of HO activity during acute and chronic hypoxia. Am J Physiol Heart Circ Physiol. 2004 Nov;287(5):H2009-15. PubMed PMID: 15217799. 111. Mourani PM, Ivy DD, Gao D, Abman SH. Pulmonary vascular effects of inhaled nitric oxide and oxygen tension in bronchopulmonary dysplasia. Am J Respir Crit Care Med. 2004 Nov 1;170(9):1006-13. PubMed PMID: 15184202. 112. Weinberg CE, Hertzberg JR, Ivy DD, Kirby KS, Chan KC, Valdes-Cruz L, Shandas R. Extraction of pulmonary vascular compliance, pulmonary vascular resistance, and right ventricular work from single-pressure and Doppler flow measurements in children with pulmonary hypertension: a new method for evaluating reactivity: in vitro and clinical studies. Circulation. 2004 Oct 26;110(17):2609-17. Epub 2004 Oct 18. PubMed PMID: 15492299.Cited in PMCRelated citations 113. Fagan KA, Oka M, Bauer NR, Gebb SA, Ivy DD, Morris KG, McMurtry IF. Attenuation of acute hypoxic pulmonary vasoconstriction and hypoxic pulmonary hypertension in mice by inhibition of Rho-kinase. Am J Physiol Lung Cell Mol Physiol. 2004 Oct;287(4):L656-64. PubMed PMID: 14977625. 114. Ivy DD, Doran A, Claussen L, Bingaman D, Yetman A. Weaning and discontinuation of epoprostenol in children with idiopathic pulmonary arterial hypertension receiving concomitant bosentan. Am J Cardiol. 2004 Apr 1;93(7):943-6. PubMed PMID: 15050507; PubMed Central PMCID: PMC3138203. 115. Ivy DD, Lee DS, Rairigh RL, Parker TA, Abman SH. Endothelin B receptor blockade attenuates pulmonary vasodilation in oxygen-ventilated fetal lambs. Biol Neonate. 2004;86(3):155-9. PubMed PMID: 15205573. 116. Wright JE, Drexler ES, Slifka AJ, McCowan CN, Ivy DD, Shandas R. Stress and strain in rat pulmonary artery material during a biaxial bubble test. Biomed Sci Instrum. 2004;40:303-8. PubMed PMID: 15133975. 106. Drexler ES, McCowan CN, Wright JE, Slifka AJ, Ivy DD, Shandas R. Comparison of strength properties of normotensive and hypertensive rat pulmonary arteries. Biomed Sci Instrum. 2004;40:297-302. PubMed PMID: 15133974. 107. Das BB, Sahoo S, Ivy DD. Neonatal aortic arch thrombosis masquerading as coarctation of the aorta. Pediatr Cardiol. 2004 Jan-Feb;25(1):80-3. PubMed PMID: 14583833. 108. Bourke KD, Sondheimer HM, Ivy DD, Pietra B, Gleason BK, Mashburn C, Boucek MM. Improved pretransplant management of infants with hypoplastic left heart syndrome enables discharge to home while waiting for transplantation. Pediatr Cardiol. 2003 Nov-Dec;24(6):538-43. PMID: 12947507. 109. Ivy DD, Parker D, Doran A, Parker D, Kinsella JP, Abman SH. Acute hemodynamic effects and home therapy using a novel pulsed nasal nitric oxide delivery system in children and young adults with pulmonary hypertension. Am J Cardiol. 2003 Oct 1;92(7):886-90. PubMed PMID: 14516902. 18 110. Carpenter T, Schomberg S, Steudel W, Ozimek J, Colvin K, Stenmark K, Ivy DD. Endothelin B receptor deficiency predisposes to pulmonary edema formation via increased lung vascular endothelial cell growth factor expression. Circ Res. 2003 Sep 5;93(5):456-63. PubMed PMID: 12919946. 111. Mitchell MB, Campbell DN, Boucek MM, Sondheimer HM, Chan KC, Ivy DD, Pietra B, Mackenzie T. Mechanical limitation of pulmonary blood flow facilitates heart transplantation in older infants with hypoplastic left heart syndrome. Eur J Cardiothorac Surg. 2003 May;23(5):735-42. PubMed PMID: 12754026. 112. Balasubramaniam V, Le Cras TD, Ivy DD, Grover TR, Kinsella JP, Abman SH. Role of platelet-derived growth factor in vascular remodeling during pulmonary hypertension in the ovine fetus. Am J Physiol Lung Cell Mol Physiol. 2003 May;284(5):L826-33. PubMed PMID: 12533438. 113. Kinsella JP, Parker TA, Ivy DD, Abman SH. Noninvasive delivery of inhaled nitric oxide therapy for late pulmonary hypertension in newborn infants with congenital diaphragmatic hernia. J Pediatr. 2003 Apr;142(4):397-401. PubMed PMID: 12712057. 114. Barst RJ, Ivy D, Dingemanse J, Widlitz A, Schmitt K, Doran A, Bingaman D, Nguyen N, Gaitonde M, van Giersbergen PL. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther. 2003 Apr;73(4):372-82. PubMed PMID: 12709727. 115. Balzer DT, Kort HW, Day RW, Corneli HM, Kovalchin JP, Cannon BC, Kaine SF, Ivy DD, Webber SA, Rothman A, Ross RD, Aggarwal S, Takahashi M, Waldman JD. Inhaled Nitric Oxide as a Preoperative Test (INOP Test I): the INOP Test Study Group. Circulation. 2002 Sep 24;106(12 Suppl 1):I76-81. PubMed PMID: 12354713. 116. Arce OX, Knudson OA, Ellison MC, Baselga P, Ivy DD, Degroff C, ValdesCruz L. Longitudinal motion of the atrioventricular annuli in children: reference values, growth related changes, and effects of right ventricular volume and pressure overload. J Am Soc Echocardiogr. 2002 Sep;15(9):906-16. PubMed PMID: 12221407. 117. Ivy DD, Yanagisawa M, Gariepy CE, Gebb SA, Colvin KL, McMurtry IF. Exaggerated hypoxic pulmonary hypertension in endothelin B receptordeficient rats. Am J Physiol Lung Cell Mol Physiol. 2002 Apr;282(4):L70312. PubMed PMID: 11880295. 118. Epting CL, Wolfe RR, Abman SH, Deutsch GH, Ivy D. Reversal of pulmonary hypertension associated with plexiform lesions in congenital heart disease: a case report. Pediatr Cardiol. 2002 Mar-Apr;23(2):182-5. Epub 2002 Feb 19. PubMed PMID: 11889532. 119. Ivy D, Tanzawa K, Abman SH. Ontogeny of endothelin-converting enzyme gene expression and protein content in the ovine fetal lung. Biol Neonate. 2002;81(2):139-44. PubMed PMID: 11844885. 120. Shandas R, Weinberg C, Ivy DD, Nicol E, DeGroff CG, Hertzberg J, Valdes-Cruz L. Development of a noninvasive ultrasound color M-mode means of estimating pulmonary vascular resistance in pediatric pulmonary hypertension: mathematical analysis, in vitro validation, and preliminary clinical studies. Circulation. 2001 Aug 21;104(8):908-13. PubMed PMID: 19 11514378. 121. Ivy D. Diagnosis and treatment of severe pediatric pulmonary hypertension. Cardiol Rev. 2001 Jul-Aug;9(4):227-37. PubMed PMID: 11405903. 122. Ivy D, McMurtry IF, Yanagisawa M, Gariepy CE, Le Cras TD, Gebb SA, Morris KG, Wiseman RC, Abman SH. Endothelin B receptor deficiency potentiates ET-1 and hypoxic pulmonary vasoconstriction. Am J Physiol Lung Cell Mol Physiol. 2001 May;280(5):L1040-8. PubMed PMID: 11290529. 123. Rairigh RL, Parker TA, Ivy DD, Kinsella JP, Fan ID, Abman SH. Role of inducible nitric oxide synthase in the pulmonary vascular response to birthrelated stimuli in the ovine fetus. Circ Res. 2001 Apr 13;88(7):721-6. PubMed PMID: 11304495. 124. Torielli F, Fashaw LM, Knudson O, Kinsella J, Ivy D, Valdes-Cruz L, Rosenberg A. Echocardiographic outcome of infants treated as newborns with inhaled nitric oxide for severe hypoxemic respiratory failure. J Pediatr. 2001 Mar;138(3):349-54. PubMed PMID: 11241041. 125. Ivy D, Eells P. Diagnosis and treatment of pulmonary hypertension in the adolescent. Adolesc Med. 2001 Feb;12(1):79-93. Review. 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Ziegler JW, Ivy DD, Wiggins JW, Kinsella JP, Clarke WR, Abman SH. Effects of dipyridamole and inhaled nitric oxide in pediatric patients with pulmonary hypertension. Am J Respir Crit Care Med. 1998 Nov;158(5 Pt 1):1388-95. PubMed PMID: 9817684. 131. Ivy DD, Griebel JL, Kinsella JP, Abman SH. Acute hemodynamic effects of pulsed delivery of low flow nasal nitric oxide in children with pulmonary hypertension. J Pediatr. 1998 Sep;133(3):453-6. PubMed PMID: 9738734. 132. Ivy DD, Parker TA, Kinsella JP, Abman SH. Endothelin A receptor blockade decreases pulmonary vascular resistance in premature lambs with hyaline membrane disease. Pediatr Res. 1998 Aug;44(2):175-80. PubMed PMID: 9702910. 133. Ivy DD, Le Cras TD, Horan MP, Abman SH. Chronic intrauterine pulmonary hypertension increases preproendothelin-1 and decreases endothelin B receptor mRNA expression in the ovine fetal lung. Chest. 20 1998 Jul;114(1 Suppl):65S. PubMed PMID: 9676634. 134. Ivy DD, Ziegler JW, Kinsella JP, Wiggins JW, Abman SH. Hemodynamic effects of dipyridamole and inhaled nitric oxide in pediatric patients with pulmonary hypertension. Chest. 1998 Jul;114(1 Suppl):17S. PubMed PMID: 9676605. 135. Ivy DD, Kinsella JP, Ziegler JW, Abman SH. Dipyridamole attenuates rebound pulmonary hypertension after inhaled nitric oxide withdrawal in postoperative congenital heart disease. J Thorac Cardiovasc Surg. 1998 Apr;115(4):875-82. PubMed PMID: 9576224. 136. Ivy DD, Le Cras TD, Horan MP, Abman SH. Increased lung preproET-1 and decreased ETB-receptor gene expression in fetal pulmonary hypertension. Am J Physiol. 1998 Apr;274(4 Pt 1):L535-41. PubMed PMID: 9575871. 137. Ziegler JW, Ivy DD, Fox JJ, Kinsella JP, Clarke WR, Abman SH. Dipyridamole potentiates pulmonary vasodilation induced by acetylcholine and nitric oxide in the ovine fetus. Am J Respir Crit Care Med. 1998 Apr;157(4 Pt 1):1104-10. PubMed PMID: 9563726. 138. Ivy DD, Neish SR, Knudson OA, Nihill MR, Schaffer MS, Tyson RW, Abman SH, Shaffer EM, Valdes-Cruz L. Intravascular ultrasonic characteristics and vasoreactivity of the pulmonary vasculature in children with pulmonary hypertension. Am J Cardiol. 1998 Mar 15;81(6):740-8. PubMed PMID: 9527085. 139. Rairigh RL, Le Cras TD, Ivy DD, Kinsella JP, Richter G, Horan MP, Fan ID, Abman SH. Role of inducible nitric oxide synthase in regulation of pulmonary vascular tone in the late gestation ovine fetus. J Clin Invest. 1998 Jan 1;101(1):15-21. PubMed PMID: 9421461; PubMed Central PMCID: PMC508535. 140. Ivy DD, Parker TA, Ziegler JW, Galan HL, Kinsella JP, Tuder RM, Abman SH. Prolonged endothelin A receptor blockade attenuates chronic pulmonary hypertension in the ovine fetus. J Clin Invest. 1997 Mar 15;99(6):1179-86. PubMed PMID: 9077525; PubMed Central PMCID: PMC507931. 141. Parker TA, Ivy DD, Kinsella JP, Torielli F, Ruyle SZ, Thilo EH, Abman SH. Combined therapy with inhaled nitric oxide and intravenous prostacyclin in an infant with alveolar-capillary dysplasia. Am J Respir Crit Care Med. 1997 Feb;155(2):743-6. PubMed PMID: 9032222. 142. Ivy DD, Kinsella JP, Abman SH. Endothelin blockade augments pulmonary vasodilation in the ovine fetus. J Appl Physiol (1985). 1996 Dec;81(6):2481-7. PubMed PMID: 9018495. 143. Fox JJ, Ziegler JW, Ivy DD, Halbower AC, Kinsella JP, Abman SH. Role of nitric oxide and cGMP system in regulation of ductus arteriosus tone in ovine fetus. Am J Physiol. 1996 Dec;271(6 Pt 2):H2638-45. PubMed PMID: 8997326. 144. Ivy DD, Ziegler JW, Dubus MF, Fox JJ, Kinsella JP, Abman SH. Chronic intrauterine pulmonary hypertension alters endothelin receptor activity in the ovine fetal lung. Pediatr Res. 1996 Mar;39(3):435-42. PubMed PMID: 8929863. 145. Abman SH, Ivy DD, Ziegler JW, Kinsella JP. Mechanisms of abnormal vasoreactivity in persistent pulmonary hypertension of the newborn infant. 21 J Perinatol. 1996 Mar-Apr;16(2 Pt 2 Su):S18-23. PubMed PMID: 8732544. 146. Ivy DD, Kinsella JP, Wolfe RR, Abman SH. Atrial natriuretic peptide and nitric oxide in children with pulmonary hypertension after surgical repair of congenital heart disease. Am J Cardiol. 1996 Jan 1;77(1):102-5. PubMed PMID: 8540445. 147. Kinsella JP, Torielli F, Ziegler JW, Ivy DD, Abman SH. Dipyridamole augmentation of response to nitric oxide. Lancet. 1995 Sep 2;346(8975):647-8. PubMed PMID: 7651043. 148. Ziegler JW, Ivy DD, Fox JJ, Kinsella JP, Clarke WR, Abman SH. Dipyridamole, a cGMP phosphodiesterase inhibitor, causes pulmonary vasodilation in the ovine fetus. Am J Physiol. 1995 Aug;269(2 Pt 2):H4739. PubMed PMID: 7653611. 149. Ziegler JW, Ivy DD, Kinsella JP, Abman SH. The role of nitric oxide, endothelin, and prostaglandins in the transition of the pulmonary circulation. Clin Perinatol. 1995 Jun;22(2):387-403. PubMed PMID: 7671544. 150. Ivy DD, Shaffer EM, Johnson AM, Kimberling WJ, Dobin A, Gabow PA. Cardiovascular abnormalities in children with autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1995 Jun;5(12):2032-6. PubMed PMID: 7579051. 151. McQueston JA, Kinsella JP, Ivy DD, McMurtry IF, Abman SH. Chronic pulmonary hypertension in utero impairs endothelium-dependent vasodilation. Am J Physiol. 1995 Jan;268(1 Pt 2):H288-94. PubMed PMID: 7840274. 152. Kinsella JP, Ivy DD, Abman SH. Ontogeny of NO activity and response to inhaled NO in the developing ovine pulmonary circulation. Am J Physiol. 1994 Nov;267(5 Pt 2):H1955-61. PubMed PMID: 7977827. 153. Kinsella JP, Ivy DD, Abman SH. Inhaled nitric oxide improves gas exchange and lowers pulmonary vascular resistance in severe experimental hyaline membrane disease. Pediatr Res. 1994 Sep;36(3):402-8. PubMed PMID: 7808839. 154. Ivy DD, Wiggins JW, Badesch DB, Kinsella JP, Kelminson LL, Abman SH. Nitric oxide and prostacyclin treatment of an infant with primary pulmonary hypertension. Am J Cardiol. 1994 Aug 15;74(4):414-6. PubMed PMID: 8059714. 155. Ivy DD, Kinsella JP, Abman SH. Physiologic characterization of endothelin A and B receptor activity in the ovine fetal pulmonary circulation. J Clin Invest. 1994 May;93(5):2141-8. PubMed PMID: 8182146; PubMed Central PMCID: PMC294348. 156. Ivy D, Kaye J, Flitter D, Wiggins J. Variant angina in an adolescent. Pediatr Cardiol. 1994 Jan-Feb;15(1):45-7. PubMed PMID: 8115273. 157. Ivy D, Loehr J, Schaffer M. Usefulness of prolonged prostaglandin infusion in neonates with Ebstein's anomaly. Am J Cardiol. 1993 Dec 1;72(17):1327-9. PubMed PMID: 8256717. 158. Kinsella JP, Neish SR, Ivy DD, Shaffer E, Abman SH. Clinical responses to prolonged treatment of persistent pulmonary hypertension of the newborn with low doses of inhaled nitric oxide. J Pediatr. 1993 Jul;123(1):103-8. PubMed PMID: 8320602. 22 Book Editor Beghetti M, Barst RJ, Berger RM, Humpl T, Ivy DD, Schulze-Neick I (editors): Pediatric Pulmonary Hypertension. Munich: Elsevier Urban & Fischer, 2011 Da Cruz E, Ivy DD, Jaggers JJ. Pediatric Cardiology, Cardiac Surgery, and Cardiac Intensive Care. London: Springer, 2013 Book Chapters and Scholarly Reviews 1. Ziegler JZ, Ivy DD, Kinsella JP, and Abman SH. The role of nitric oxide, endothelin and prostagaldins in the transition of the pulmonary circulation. Clinics in Perinatology 22 (2): 387-403, 1995 2. Ivy DD, Abman SH, Wolfe RR. Congenital Heart Disease. In: Peacock AJ, Ed. The Pulmonary Circulation: A Handbook For Clinicians. London, UK: Chapman and Hall, 1996:449-466 3. Abman SH, Ivy DD, Ziegler JW, and Kinsella JP. Mechanisms of abnormal vasoreactivity in persistent pulmonary hypertension of the newborn infant. J Perinatology 16(2 Pt 2 Su): S18-23, 1996 4. Ivy DD, Shaffer EM, Schaffer MS. Heart. In: Merenstein GB, Kaplan DW, Rosenberg AA, Eds. Handbook of Pediatrics. Stamford, Connecticut: Appleton & Lange, 1997:511-547 5. Ivy DD, Neish SH, Abman SH. Control of the Pulmonary Circulation. In: MacNamara D, Bricker T, Neish SR, Eds. The Science and Practice of Pediatric Cardiology. Malvern, PA: Lea and Febiger, 1998:329-347 6. Ivy DD. Echocardiographic Evaluation of Pulmonary Hypertension. In: ValdesCruz L, Cayre R, Eds. Echocardiographic Anatomy of Congenital Heart Disease, 1999:537-547 7. Ivy DD, Abman SH. The Role of Endothelin in Perinatal Pulmonary Vasoregulation. In: Weir EK, Archer SL, Reeves JT (Eds.). The Fetal and Neonatal Pulmonary Circulations, 1999: 279-302 8. Sondheimer HM, Boucek MM, Ivy DD, Lorts A, Schaffer MS, and Wolfe RR. Cardiovascular Diseases. In: Hay WW, Hayward AR, Levin MJ, and Sondheimer JM, Eds. Current Pediatric Diagnosis and Treatment, 2000 9. Ivy DD. Diagnosis and management of severe pediatric pulmonary hypertension. Cardiology in Review 9(4): 227-237, 2001 10. Ivy DD and Eells P. Diagnosis and treatment of pulmonary hypertension in the adolescent. Adolescent Medicine: State of the Art Reviews 12(1): 79-93, 2001 11. Rashid A and Ivy DD. Diagnosis and Management of Severe Pediatric Pulmonary Hypertension. Current Concepts in Pediatric Critical Care 43-58, 2004 12. Rashid A and Ivy DD. Severe Paediatric Pulmonary Hypertension: New Management Strategies. Archives of Disease in Childhood (90): 92-98, 2005 13. Rashid A and Ivy DD. New management strategies in severe paediatric pulmonary hypertension. Cardiology Today 9(2): 100-108, 2005 23 14. Kinsella JP, Ivy DD and Abman SH. Pulmonary vasodilator therapy in congenital diaphragmatic hernia: acute, late, and chronic pulmonary hypertension. Seminars in Perinatology 29: 123-128, 2005 15. Rashid A and Ivy DD. Pulmonary hypertension. In: Slonim AD and Pollack MM, Eds. Pediatric Critical Care Medicine, 2006:694-704 16. Rashid A and Ivy DD. Pulmonary hypertension in children. Current Paediatrics 16: 237-247, 2006 17. Ivy DD. Pulmonary arterial hypertension assessment in pediatric cardiology. In: Beghetti, Barst, Naeije, Rubin Eds. Pulmonary Arterial Hypertension Related to Congenital Heart Disease. Munich: Elsevier, 2006:93-111 18. Rashid A and Ivy DD. Pulmonary hypertension in children. In: Mandel and Taichman Eds. Pulmonary Vascular Disease. Philadelphia, PA: Saunders and Elsevier, 2006:274287 19. Ivy DD, Feinstein JA, Humpl T, Rosenzweig E. Non congenital heart disease associated pediatric pulmonary arterial hypertension. Progress in Pediatric Cardiology 9(4): 227-237, 2009 20. Rosenzweig EB, Feinstein J, Humpl T, and Ivy DD. Pulmonary Arterial Hypertension in Children: Diagnostic work-up and challenges. Progress in Pediatric Cardiology 9(4): 227-237, 2009 21. Mallory G, Ivy DD, Shardonovsky F, and Hanna B. Management and Controversies in Pediatric Pulmonary Hypertension. Pediatric Asthma, Allergy & Immunology 22(4): 139-150, 2009 22. Tissot C, Ivy DD, and Beghetti M. Update on pulmonary arterial hypertension focusing on new therapies. Journal of Pediatrics 157(4): 528-532, 2010 23. Ivy DD. Prostanoids in pediatric pulmonary hypertension. Pediatric Critical Care Medicine 9(4): 227-237, 2010 24. Kozik D, Ivy DD , Ibrahim J, Wise-Faberowski L, Goldberg S, Darst J, Kaufman J, Morell V, and da Cruz E. Patent Ductus Arteriosus. In: Ricardo Munoz, Victor Morell, Eduardo da Cruz, and Carol Vetterly Eds. Critical Care of Children with Heart Disease: Basic Medical and Surgical Concepts. London: Springer-Verlag, 2010:145-157 25. Kaufman J, Goldberg S, Ibrahim J, Ivy DD, Wise-Faberowski L, Buckvold S, Tissot C, Phelps C, Morell V, and da Cruz E. Complete Atrioventricular Septal Defect. In: Ricardo Munoz, Victor Morell, Eduardo da Cruz, and Carol Vetterly Eds. Critical Care of Children with Heart Disease: Basic Medical and Surgical Concepts. London: Springer-Verlag, 2010:177-189 26. da Cruz E, Goldberg S, Kaufman J, Artico A, Mackie S, Church J, Stimmler A, Rutz J, Kozik D, Ivy DD, Lacour-Gayet F, and Vouhe P. Single Ventricle, Basic Concepts. In: Ricardo Munoz, Victor Morell, Eduardo da Cruz, and Carol Vetterly Eds. Critical Care of Children with Heart Disease: Basic Medical and Surgical Concepts. London: SpringerVerlag, 2010:331-348 27. da Cruz E, Kaufman J, Morell V, Tissot C, Buckvold S, Goldberg S, Ivy DD, Ibrahim J, Wise-Faberowski L, and Kalangos A. Ebstein’s Anomaly. In: Ricardo Munoz, Victor Morell, Eduardo da Cruz, andCarol Vetterly Eds. Critical Care of Children with Heart Disease: Basic Medical and Surgical Concepts. London: Springer-Verlag, 2010:411-421 28. Ivy DD and Rashid A. Chronic Pulmonary Hypertension. In: Ricardo Munoz, Victor Morell, Eduardo da Cruz, andCarol Vetterly Eds. Critical Care of Children with Heart Disease: Basic Medical and Surgical Concepts. London: Springer-Verlag, 2010:483-505 29. Ivy DD, Saji B. A new era in medical management of severe pediatric pulmonary arterial hypertension. Pediatric Cardiology and Cardiac Surgery. 26(3):206-218, 2010 24 30. Hunter KS, Feinstein JA, Ivy DD, and Shandas RS. Computational simulation of the pulmonary arteries and its role in the study of pediatric pulmonary hypertension. Progress in Pediatric Cardiology 30(1-2):63-69, 2010 31. Tissot C, Ivy DD. Echocardiographic evaluation of pulmonary arterial hypertension in children. In: Beghetti M, Barst RJ, Berger RM, Humpl T, Ivy DD, Schulze-Neick I (editors): Pediatric Pulmonary Hypertension. Munich: Elsevier Urban & Fischer, 2011:131-154 32. Ivy DD, Saji B. Treatment of pulmonary arterial hypertension in children. In: Beghetti M, Barst RJ, Berger RM, Humpl T, Ivy DD, Schulze-Neick I (editors): Pediatric Pulmonary Hypertension. Munich: Elsevier Urban & Fischer, 2011:315-328 33. Ivy DD. Idiopathic pulmonary arterial hypertension in children. In: Peacock AJ, Ed. The Pulmonary Circulation: A Handbook For Clinicians. London, UK: Chapman and Hall, 2011:559-571 34. Ivy DD. Pulmonary Hypertension. In: McMillan, Barrett, Boney, and Jones eds. Clinical Decision Support: Pediatrics, 2012 35. Ivy DD. Diagnosis and Treatment of Pediatric Pulmonary Hypertension. In: Allen, Shaddy, Driscoll, Eds. Moss and Adams: Congenital Heart Disease. Philadelphia, Wolters Kluwer Health Lippincott Williams & Wilkins. 1433-1462, 2012 36. Takatsuki S, Beghetti M, Ivy DD. Pulmonary hypertension in Children. In: Hoeper, Humbert, Eds. European Respiratory Monograph. 57:94–107, 2012 37. Takatsuki S, Ivy DD. Current Challenges in Pediatric Pulmonary Hypertension. Seminars in Respiratory and Critical Care Medicine 34:627–644, 2013 38. Vorhies EE, Ivy DD. Drug Treatment of Pulmonary Hypertension in Children. Paediatr Drugs. 2014 Feb;16(1):43-65. 39. Takatsuki S, Eshelman J, Berg, A, Ivy DD. Pharmacological treatment of Pulmonary Hypertension. RMunoz, et al. (eds.), Handbook of Pediatric Cardiovascular Drugs, Springer-Verlag London 2014 40. Jone P, Ivy DD. Echocardiography in pediatric pulmonary hypertension. Front Pediatr. 2014;2:124. 41. Ivy DD. Idiopathic pulmonary arterial hypertension in children. In: Peacock AJ, Ed. The Pulmonary Circulation: A Handbook For Clinicians. London, UK: Chapman and Hall, 2015 42. Ivy DD. Diagnosis and Treatment of Pediatric Pulmonary Hypertension. In: Allen, Shaddy, Driscoll, Eds. Moss and Adams: Congenital Heart Disease. Philadelphia, Wolters Kluwer Health Lippincott Williams & Wilkins, 2015 Abstracts Accepted for Presentation 1. Ivy DD, Kinsella JP and Abman SH. BQ 123, an endothelin A receptor antagonist, causes ovine fetal pulmonary vasodilation. Children's National Medical Center ECMO Symposium, 1993 2. Ivy DD, Kinsella JP and Abman SH. Physiologic characterization of endothelin A and B receptor activity in the ovine fetal pulmonary circulation. Pediatr Res 33 (4): 247A, 1993 3. Kinsella JP, Ivy DD and Abman SH. Maturational changes in endotheliumderived nitric oxide activity in the developing pulmonary circulation. Pediatr Res 33 (4): 332A, 1993 4. McQueston JA, Kinsella JP, Ivy DD and Abman SH. Changes in endotheliumdependent and independent vasodilation during chronic intrauterine 25 pulmonary hypertension in fetal lambs. Pediatr Res 33 (4): 337A, 1993 5. Ivy DD, Schaffer MS and Loehr JP. Prolonged prostaglandin infusion in neonates with severe Ebstein's Anomaly. World Congress of Pediatric Cardiology. Paris, France, 1993 6. Ivy DD, Kinsella JP and Abman SH. Role of endothelin in autoregulation of blood flow in the ovine fetal lung. Pediatr Res 35 (4): 338A, 1994 7. Ivy DD, Kinsella JP, Wolfe RR, and Abman SH. Hemodynamic responses to atrial natriuretic peptide and inhaled nitric oxide in pediatric pulmonary hypertension. Pediatr Res 35 (4): 36A, 1994 8. Kinsella JP, Ivy DD and Abman SH. Inhaled nitric oxide improves gas exchange and lowers pulmonary vascular resistance in severe experimental hyaline membrane disease. Pediatr Res 35 (4): 340A, 1994 9. Ziegler JW, Ivy DD, Kinsella JP, Clarke WR and Abman SH. Dipyridamole, a cGMP phosphodiesterase inhibitor, augments inhaled nitric oxide -induced pulmonary vasodilation in the ovine transitional circulation. Pediatr Res 35 (4): 90A, 1994 10. Ivy DD, Ziegler JW, Fox JJ, Kinsella JP and Abman SH. Chronic intrauterine pulmonary hypertension alters endothelin receptor activity in the ovine fetus. Pediatr Res 37 (4): 27A, 1995 11. Ziegler JW, Ivy DD, Fox JJ, Kinsella JP and Abman SH. Hemodynamic effects of cGMP phosphodiesterase inhibitors during chronic pulmonary hypertension. Pediatr Res 37 (4): 38A, 1995 12. Ziegler JW, Ivy DD, Fox JJ, Kinsella JP, WR Clarke and Abman SH. Dipyridamole, a cGMP phosphodiesterase inhibitor, augments endotheliumindependent vasodilation in the ovine fetal lung. Pediatr Res 37 (4): 38A, 1995 13. Fox JJ, Ziegler JW, Kinsella JP, Ivy DD, Halbower AC and Abman SH. Role of the nitric oxide-cGMP system in regulation of tone of the ovine ductus arteriosus. Pediatr Res 37 (4): 76A, 1995 14. Ziegler JW, Fox JJ, Ivy DD, Kinsella JP and Abman SH. Potassium channel blockade does not inhibit nitric-oxide-induced vasodilation in the ovine perinatal lung. Pediatr Res 37 (4): 81A, 1995 15. Kennaugh JM, Moreland SM, Fashaw LM, Rosenberg AR, Kinsella JP, Abman SH, Ivy DD and Torielli FM. Neurodevelpomental and medical follow-up of infants with PPHN treated with inhaled nitric oxide. Pediatr Res 37 (4): 261A, 1995 16. Kinsella JP, Halbower AC, Ziegler JW, Fox JJ, Ivy DD, and Abman SH. Lowdose inhaled nitric oxide improves gas exchange without changing vascular permeability in experimental hyaline membrane disease. Pediatr Res 37 (4): 337A, 1995 17. Torielli F, Ivy DD, Abman SH, Kinsella JP, Moreland S, Rosenberg A, Knudson OA, Valdez-Cruz L. Long term echocardiographic outcome of infants with severe persistent pulmonary hypertension of the newborn after treatment with nitric oxide and/or high frequency oscillatory ventilation. American Academy of Pediatrics, 1995 18. Ivy DD, Neish SR, Knudson OA, Schaffer MS, Wiggins JW, Shaffer EM, Nihill MR, and Valdes-Cruz LM. Intravascular ultrasound of pulmonary arteries in children with pulmonary hypertension. J Am Coll Cardiol 27 (2): 324A, 1996 19. Ivy DD, Kinsella JP, Ziegler JW, and Abman SH. Dipyridamole, a cGMP phosphodiesterase inhibitor, attenuates rebound pulmonary hypertension after withdrawal of inhaled nitric oxide in postoperative congenital heart disease. Pediatr Res 39 (4): 30A, 1996 26 20. Ivy DD, Parker TA, Ziegler JW, Galan HL, Kinsella JP and Abman SH. Prolonged endothelin A receptor blockade attenuates chronic intrauterine pulmonary hypertension. Pediatr Res 39 (4): 335A, 1996 21. Ziegler JW, Ivy DD, Kinsella JP, Wiggins JW, and Abman SH. Hemodynamic effects of dipyridamole and inhaled nitric oxide in patients with severe pulmonary hypertension. Pediatr Res 39 (4): 40A, 1996 22. Torielli F, Moreland SG, Ivy DD, Abman SH, Kinsella JP, Rosenberg AA, Knudson OA, and Valdez-Cruz L. Long term echocardiographic follow-up of infants with severe persistent pulmonary hypertension of the newborn. Pediatr Res 39 (4): 282A, 1996 23. Kinsella JP, Parker TA, Galan HL, Ivy DD, and Abman SH. High frequency oscillatory ventilation augments the response to inhaled nitric oxide in severe experimental hyaline membrane disease. Pediatr Res 39 (4): 336A, 1996 24. Parker TA, Ivy DD, Galan HL, Kinsella JP and Abman SH. Effects of chronic estradiol administration in an experimental model of persistent pulmonary hypertension of the newborn. 25th Aspen Conference on Perinatal Research, Aspen, CO, 1996 25. Pietra BP, Ivy DD, Sondheimer HM, Luna M, Schneider K, Hall P, Campbell DC, Fullerton D, Kinsella JP, Abman SH, and Boucek MM. Use of post-operative pulmonary vasodilators for orthotopic heart transplantation in hypoplastic left heart syndrome: inhaled nitric oxide and milrinone. Third Loma Linda International Conference on Pediatric Heart and Lung Transplantation. January 1997 26. Sondheimer HM, Pietra BP, Luna M, Schneider K, Hall P, Ivy DD, Campbell DC, and Boucek MM. Pre- and post-transplantation management of infants with hypoplastic left heart syndrome. Third Loma Linda International Conference on Pediatric Heart and Lung Transplantation. January 1997 27. Boucek MM, Pietra BP, Shaffer EM, Sondheimer HM, Ivy DD, Luna M, Schneider K, Hall P, and Campbell DC. Improved overall (pre & post) cardiac transplant survival in infancy: strategies to regulate pulmonary resistance. J Am Coll Cardiol 29 (2): 105A, 1997 28. Ivy DD, Le Cras TD, Horan MP, and Abman SH. Increased lung preproendothelin-1 and decreased endothelin B receptor mRNA expression after chronic intrauterine pulmonary hypertension in the ovine fetus. Pediatr Res 41(4): 21A, 1997 29. Ivy DD, Kinsella JP, Parker TA, and Abman SH. Endothelin A receptor blockade decreases pulmonary vascular resistance in severe experimental hyaline membrane disease. Pediatr Res 41(4): 21A, 1997 30. Parker TA, Ivy DD, Galan HL, Kinsella JP and Abman SH. Intrauterine treatment with estradiol improves hemodynamics and vascular remodeling in an ovine model of pulmonary hypertension. Pediatr Res 41(4): 264A, 1997 31. Rairigh RL, Ivy DD, Kinsella JP, and Abman SH. Inducible nitric oxide synthase inhibitors increase pulmonary vascular resistance in the late-gestation fetus. Pediatr Res 41(4): 58A, 1997 32. Best TH, Ivy DD, Kinsella JP, Fox JJ, and Abman SH. Pulmonary vascular effects of selective cGMP-specific (type V) phosphodiesterase inhibitors in the ovine fetus. Pediatr Res 41(4): 55A, 1997 33. Ivy DD, Le Cras TD, Horan MP, and Abman SH. Increased lung preproendothelin-1 and decreased endothelin B receptor mRNA expression after chronic intrauterine pulmonary hypertension in the ovine fetus. Chest 114(1): 65S, 1998 34. Ivy DD, Ziegler JW, Kinsella JP, Wiggins JW, and Abman SH. Hemodynamic 27 effects of dipyridamole and inhaled nitric oxide in pediatric patients with pulmonary hypertension. Chest 114(1): 17S, 1998 35. Ivy DD, Le Cras TD, Horan MP, and Abman SH. Developmental regulation of endothelin gene expression in the ovine lung. Pediatr Res 43(4): 287A, 1998 36. Ivy DD, Griebel JL, Kinsella JP, and Abman SH. Acute hemodynamic effects of pulsed delivery of low flow nasal nitric oxide in children with pulmonary hypertension. Pediatr Res 43(4): 23A, 1998 37. Ivy DD, McMurtry IF, Yanagisawa M, Gariepy CE, Morris KG, Jakkula M, OliverPickett C, and Abman SH. Exaggerated hypoxic pulmonary vasoconstrictor response to acute hypoxia in the endothelin B receptor deficient rat. Pediatr Res 45(4): 24A, 1999 38. Ivy DD, Parker TA, Kinsella JP, and Abman SH. Prolonged endothelin B receptor blockade causes pulmonary hypertension in the ovine fetus. Pediatr Res 45(4): 24A, 1999 39. Parker TA, Kinsella JP, Ivy DD, and Abman SH. Pulmonary vascular effects of prolonged estrogen receptor blockade on the response to birth related stimuli in fetal lambs. Pediatr Res 45(4): 314A, 1999 40. Arce OX, Knudson, OA, Ivy DD, Ellison MC, and Valdes-Cruz L. Longitudinal motion of the atrioventricular annuli in children: a heart rate independent measure of ventricular dynamics. J Am Soc Echo 12(5): 802M, 1999 41. Shandas R, Nicol E, Weinberg C, DeGroff C, Hertzberg J, Ivy DD, and Valdes-Cruz L. Development of a noninvasive ultrasound color M-mode means of estimating pulmonary vascular resistance in pediatric pulmonary hypertension. J Am Coll Cardiol 35(2): 506A, 2000. 42. Boucek MM, Ivy DD, Pietra B, Shaffer EM, Sondheimer HM, Doremus TE, and Campbell DN. Induction immunotherapy in pediatric heart transplantation: influence of serum mycophenolate level monitoring. J Heart Lung Transplant 19(1): 84, 2000. 43. Ivy DD. Exaggerated pulmonary hypertension to prolonged hypoxia in the endothelin B receptor deficient rat. Pediatr Res 47(4): 44A, 2000 44. Boucek MM, Sondheimer HM, Ivy DD, Shaffer EM, Mashburn C, Ripe DW, Gilbert DJ, Kyle TE, Campbell DN, and Pietra B. Transplant coronary artery disease in pediatrics: favorable outcome with medical therapy. J Heart Lung Transplant 20(2): 261, 2001. 45. Pietra B, Ivy DD, Sondheimer HM, Shaffer EM, Mashburn C, Ripe DW, Gilbert DJ, Kyle TE, Mitchell MB, Campbell DN, Boucek MM. Comparison of pretransplant transfusion with or without cyclosporin inhibition on post transplant outcome. J Heart Lung Transplant 20(2): 222-223, 2001. 46. Resnik ER, Herron J, Fu R, Ivy DD, Cornfield DN. Hypoxia increases calciumsensitive K+ channel gene expression. Pediatr Res 49(4): 353A, 2001 47. McLaughlin VM, Sitbon O, Barst RJ, Schilz R, Robbins I, Ivy DD, Rich S. Long term hemodynamic and exercise effects of UT-15, a subcutaneous prostacyclin analogue. Circulation 104(17): II-249, 2001 48. The INOP I Study Group of Children’s Hospitals, (Salt Lake City, St Louis, Houston, Kansas City, Denver, Pittsburgh, UC San Diego, Detroit, Los Angeles). Inhaled nitric oxide as a pro-operative test: INOP Test I. Circulation 104(17): II-645, 2001 49. Barst R, Ivy DD, Widlitz AC, Moore K, Doran A, Nguyen N, Gaitonde M. Pharmacokinetics and safety of bosentan, an oral dual endothelin receptor antagonist, in children with pulmonary hypertension. European Heart Journal; (23):2560, 2002. 50. Ivy DD, Doran A, Kinsella JP, and Abman SH. Acute hemodynamic effects of a novel pulsed nasal nitric oxide delivery system. Pediatr Res 51(4): 469A, 2002 28 51. Kinsella JP, Parker TA, Ivy DD, and Abman SH. Role of non-invasive delivery of inhaled nitric oxide for late pulmonary hypertension in newborns with congenital diaphragmatic hernia. Pediatr Res 51(4): 391A, 2002 52. Barst R, Ivy DD, Widlitz AC, Moore K, Doran A, Nguyen N, Gaitonde M. Pharmacokinetics and safety of bosentan, an oral dual endothelin receptor antagonist, in children with pulmonary hypertension. Circulation 106(19): II-450, 2002 53. Mitchell MB, Campbell DN, Boucek MM, Sondheimer HM, Ivy DD, Pietra BP, and Das B. Favorable outcomes with heart transplantation for failed Fontan operation or cavopulmonary shunt. AATS, 2003 54. Das B, Wolfe RR, and Ivy DD. Baseline pulmonary hypertension: a previously undescribed risk factor for high altitude pulmonary edema in children. Pediatr Res 53(4): 30A, 2003 55. Giller RH, Quinones R, Ivy DD, Abman SH, Malcolm J, Coll J, Hild E, and Deterding R. Pulmonary hypertension complicating allogeneic hemapoietic stem cell transplantation in children and adolescents. Biology of Blood and Marrow Transplantation 9(2):74, 2003 56. Carpenter T, Schaumburg S, Colvin K, and Ivy DD. Exaggerated lung vascular leak in endothelin B receptor deficient rats is mediated by VEGF. Am J Resp Crit Care Med 167(7): A565, 2003 57. Deterding RR, Ivy DD, Abman SH, Malcolm J, Coll J, Quinones R, and Giller RH. Pulmonary hypertension predicts mortality in pediatric allogeneic bone marrow transplants. Am J Resp Crit Care Med 167(7): A388, 2003 58. Barst RJ, Langleben D, Frost A, Horn E, Oudiz R, Shapiro S, McLaughlin V, Hill N, Tapson V, Robbins I, Zwicke D, Duncan B, Frumkin L for the Stride Study Group. Sitaxsentan, a selective ETA antagonist, improves cardiopulmonary hemodynamics in pulmonary arterial hypertension. Am J Resp Crit Care Med 167(7): A273, 2003 59. Barst RJ, Langleben D, Frost A, Horn E, Oudiz R, Shapiro S, McLaughlin V, Hill N, Tapson V, Robbins I, Zwicke D, Duncan B, Frumkin L for the Stride Study Group. Sitaxsentan, a selective ETA antagonist, improves exercise capacity and NYHA functional class in pulmonary arterial hypertension. Am J Resp Crit Care Med 167(7): A440, 2003 60. Miyamoto SD, Pietra BA, Chan KC, Ivy DD, Mashburn C, Campbell DN, Mitchell MB, Boucek MM. Successful cardiac transplantation following interventional palliation in patients with hypoplastic left heart syndrome. Circulation 108(17): IV-495, 2003 61. Pietra BA, Mashburn C, Gilbert D, Legette F, Ivy DD, Sondheimer HM, Mitchell MB, Campbell DN, Boucek MM. Early management in transplantation for HLHS leads to infrequent rejection and improved survival. Circulation 108(17): IV-330, 2003 62. AA Condino, JA O’Connor, MR Narkewicz, L Claussen, A Doran, Ivy DD, RJ Sokol. Portopulmonary hypertension in pediatrics: a case series. J Pediatr Gastroenterol Nutr, 2004, 39, supplement 1, S11-12 63. Dyer KL, Pauliks L, Kirby SK, Claussen L, Ivy DD, Shandas R, Valdes-Cruz L. Myocardial performance index in pediatric patients with primary pulmonary hypertension: correlation with pulmonary artery pressure and response to vasodilators. J Am Coll Cardiol 43(5): 388A, 2004 64. Yetman AT, Taylor A, Ivy DD. Exercise responses in children with pulmonary hypertension. J Am Coll Cardiol 43(5): 392A, 2004 65. Ivy DD, Rosenzweig EB, Widlitz AC, Doran AK, Claussen LR, Yung D, Abman SH, Barst RJ. Effects of long-term bosentan in children with pulmonary arterial hypertension. Am J Resp Crit Care Med 169(7): A210, 2004 29 66. Mourani PM, Ivy DD, Gao D, and Abman SH. Pulmonary vascular reactivity in children and adolescents with bronchopulmonary dysplasia. Am J Resp Crit Care Med 169(7): A169, 2004 67. Das BB, Lanning C, Dyer KL, Ivy DD, Valdes-Cruz L, Shandas R. Evaluation of pulmonary artery pulsatility in pulmonary hypertension using color m-mode tissue Doppler imaging in infants and children. Pediatr Res 55(4): 51A, 2004 68. Dyer KL, Das BB, Lanning C, Ivy DD, Valdes-Cruz L, Shandas R. Noninvasive tissue Doppler measurement of pulmonary artery compliance in children with pulmonary hypertension. Pediatr Res 55(4): 39A, 2004 69. Yetman AT, Taylor AL, Wolfe RR and Ivy DD. Correlation between maximal and submaximal exercise capacity in young patients with pulmonary arterial hypertension. Presented at the North American Society for Pediatric Exercise Medicine 2004 Biennial Meeting. St Andrews, New Brunswick, Canada, 2004 70. Frost A, Ivy DD. Sitaxsentan for pulmonary arterial hypertension patients with clinical deterioration or significantly abnormal liver function test on Bosentan. Am J Resp Crit Care Med 2: A204, 2005 71. Kaufman J, Ivy DD, Jones PL, McMurtry IF, Gebb SA. The role of Rho GTPases in maintenance of fetal lung lipofibroblast phenotype. Am J Resp Crit Care Med 2: A464, 2005 72. Miyamoto SD, Pietra BP, Chan KC, Ivy DD, Mashburn C, Campbell DN, Mitchell MB, Boucek MM. Normal pulmonary arterial development following transplantation in patients with hypoplastic left heart syndrome who were palliated with internal pulmonary artery bands. Cardiology in the Young 15(2): O18-5, 21, 2005 73. Ivy DD, Frost A. Sitaxsentan for pulmonary arterial hypertension patients with clinical deterioration or significantly abnormal liver function test on Bosentan., World Congress of Pediatric Cardiology, Buenos Aires, Argentina, September 18-22, 2005 74. Ivy DD, Barst RJ for the Stride-2 Study Group. Sitaxsentan for the treatment of pulmonary arterial hypertension. World Congress of Pediatric Cardiology, Buenos Aires, Argentina, September 18-22, 2005 75. Pauliks LB, Dyer KD, Shaffer EM, Ivy DD, Valdes-Cruz LM. Myocardial acceleration during isovolumic contraction in children with pulmonary hypertension: a tissue Doppler study and correlation with catheterization data. Circulation, 112:17 (Supp II): II-764, 2005 76. Imamura M, Vitello AM, Limbird JN, Ivy DD, Fallon MB, Carter EP. Endothelin-B receptor overexpression prevents hypoxic pulmonary hypertension in cirrhotic rats. Chest 2005 Dec; 128(6 Suppl):580S-1S, 2005 77. Ihida-Stansbury K, McKean DM, Gebb SA, Martin JF, Stevens T, Nemenoff R, Vaughn J, Lane K, Loyd J, Wheeler L, Morrell NW, Ivy DD, Jones PL. Regulation and Functions of the Paired-Related Homeobox Gene PRX1 in Pulmonary Vascular Development and Disease. Chest Dec; 128(6 Suppl):591S, 2005 78. Beghetti M, Ivy DD, Barst RJ. Clinical experience with Bosentan in children with pulmonary arterial hypertension. Eur J Clinical Investigation: 35(Supplement 2):58, 2005 79. Dyer KL, Pauliks LB, Shaffer EM, Ivy DD, and Valdes-Cruz LM. Peak systolic strain and strain rate in children with pulmonary hypertension: a tissue Doppler study and correlation with cardiac catheterization. J Am Coll Cardiol 47(4): 306A, 2006 80. Darst JR, Ivy DD, and Chan KC. Pulmonary vein stenosis presenting as pulmonary hypertension: short and medium term outcome after stent implantation. Cardiology in the Young, 16 (2): 85, 2006 30 81. Bernus A, Chan KC, Doran A, Claussen L, Taylor A, Kay J, Ivy DD. Brain Natriuretic Peptide as a Predictive Marker of Right Ventricular Dysfunction and Clinical Worsening in Pediatric Patients with Pulmonary Arterial Hypertension. Cardiology in the Young, 16 (2): 93, 2006 82. Zhang Y, Hunter KS, Lanning C, Ivy DD, Claussen L, and Shandas R. A microstructural model applied to 3D patient-specific images of the pulmonary vasculature: prediction of structural remodeling in PAH. Proceedings of the American Thoracic Society 3: A60, 2006 83. Richman AP, Stenmark KR, Ivy DD, Hofmeister SE, Iglarz M, and Davie NJ. The endothelin receptor antagonist bosentan, attenuates hypoxia-induced angiogenesis in the pulmonary artery vaso vasorum microcirculation. Proceedings of the American Thoracic Society 3: A754, 2006 84. Vaughn JJ, Colvin KL, Crossno JT, Ivy DD, Stenmark K, and Davie NJ. Potential role of bone marrow-derived mesenchymal stem cells in remodeling / repair of the lung response to hypoxia. Proceedings of the American Thoracic Society 3: A755, 2006 85. Colvin KL, Duncan MW, Edwards MG, Hunsucker SW, Davie NJ, and Ivy DD. The plasma proteome of children with idiopathic pulmonary arterial hypertension: comparison between responders and non-responders to vasodilator therapy. Proceedings of the American Thoracic Society 3: A512, 2006 86. Rosenzweig EB, Ivy DD, Widlitz AC, Doran AK, Claussen LR, Yung D, Abman SH, and Barst RJ. Impact of bosentan in children with pulmonary arterial hypertension related to congenital heart disease. European Society of Cardiology, 2006 87. Ivy DD, Doran AK, Parker DK, Claussen LR, Smith KJ, Mallory GB, and Abman SH. Acute and chronic effects of inhaled iloprost therapy in children with pulmonary arterial hypertension. Chest: 130(4): 156S, 2006 88. Smith KJ, Mallory GB, Abman SH, and Ivy DD. Airway hyperreactivity after iloprost inhalation in pediatric patients with pulmonary arterial hypertension Chest: 130(4): 156S, 2006 89. Buckvold S, Tissot-Daguette C, Phelps CM, Mitchell MB, Campbell DN, Ivy DD, Pietra BA, Miyamoto SD. Primary heart transplantation for hypoplastic left heart syndrome: effect of waiting time on pulmonary artery pressure. Pediatric Critical Care Medicine: 8(3): A242, 2007 90. Tissot-Daguette C, Buckvold S, Phelps CM, Ivy DD, Campbell DN, Mitchell MB, Pietra BA, Miyamoto SD. Use of ECMO to reverse primary graft failure after heart transplantation in children. Pediatric Critical Care Medicine: 8(3): A279, 2007 91 Phelps CM, Tissot-Daguette C, Buckvold S, Ivy DD, Pietra BA, Miyamoto SD. Inotropic and mechanical support in acute graft rejection for pediatric heart transplant patients. Pediatric Critical Care medicine: 8(3): A282, 2007 92. Beghetti M, Haworth SG, Barst RJ, Jais X, Acar P, Fraisse A, Ivy DD, Schulze-Neick I, Bonnet D, Berger RMF. Pharmacokinetics and safety profile of a novel formulation of bosentan in children with pulmonary arterial hypertension (PAH): FUTURE-1 study. European Heart Journal : 28(Supplement 1): 150, 2007 93. Beghetti M, Doran AK, Mallory G, Barst RJ, Law Y, Abman SH, Ivy DD. Inhaled iloprost therapy in children with pulmonary arterial hypertension. Cardiology in the Young, 17 (1): 78, 2007 94. Tissot C, Pietra BA, Boucek MM, Gilbert DJ, Campbell DN, Mitchell MB, Ivy DD, Miyamoto SD. Coronary flow reserve can detect progressive transplant coronary artery disease in pediatric heart transplant recipients. Cardiology in the Young, 18 Suppl 1: 29, 2008 31 95. Tissot C, Pietra BA, Boucek MM, Gilbert DJ, Campbell DN, Mitchell MB, Ivy DD, Miyamoto SD. Early diagnosis of transplant coronary artery disease by intravascular ultrasound improves the outcome of pediatric heart transplant recipients. Cardiology in the Young, 18 Suppl 1: 88, 2008 96. Tissot C, Pietra BA, Boucek MM, Ripe DW, Mashburn C, Campbell DN, Mitchell MB, Ivy DD, Miyamoto SD. Long-term outcome of pediatric heart transplant recipients from donation after cardiac death. Cardiology in the Young, 18 Suppl 1: 52, 2008 97. Berger RMF, Beghetti M, Barst RJ, Jais X, Acar P, Fraisse A, Ivy DD, SchulzeNeick I, Bonnet D, Galie N, Haworth SG. Effect on quality of life and other subjective parameters of a novel formulation of bosentan in children with pulmonary arterial hypertension: FUTURE-1 study. Cardiology in the Young, 18 Suppl 1: 19, 2008 98. Ivy DD, Barst RJ, Doran AK, Lemarie JC, Brand M, Rosenberg D, Rosenzweig EB. Effects of Long-Term Bosentan in Children with Pulmonary Arterial Hypertension. Cardiology in the Young, 18 Suppl 1: 34, 2008 99. Barst RJ, Ivy DD, Badesch D, Benza R, Elliott G, Farber H, Frost A, Krichman A, Liou T, Raskob G, Giles S, Feldkircher K, Turner M, McGoon M. Reveal Registry: treatment at Baseline for patients with childhood-onset pulmonary arterial hypertension. American Academy of Pediatrics, 2008 100. Tissot C, Pietra BA, Boucek MM, Diamond BA, Buckvold S, Campbell DN, Mitchell MB, Ivy DD, Miyamoto SD. Coronary artery flow reserve is lower at the time of transplant coronary artery disease diagnosis in children transplanted in infancy. Scientific Basis of Heart Failure in Children, American Heart Association, Estes Park, CO, 2008 101. Mourani PM, Sontag M, Lui G, Ivy DD, and Abman SH. Sildenafil therapy for pulmonary hypertension in infants with chronic lung disease. Ped Res 169(7): A169, 2008 102. Barker AJ, Lanning C, Hunter K, Ivy DD and Shandas R. Artery dilatation in pediatric pulmonary hypertension patients decreases hemodynamic wall shear stress. Circulation, 118(18) Suppl 2: S879, 2008 103. Hunter K, Lanning C, Kirby KS, Ivy DD and Shandas R. In-vivo pulmonary vascular stiffness obtained from color m-mode tissue Doppler imaging and pressure measurements predicts clinical outcomes better than indexed pulmonary vascular resistance in pediatric patients with pulmonary hypertension. Circulation, 118(18) Suppl 2: S879, 2008 104. Stoica S, Clarke D, Kozik D, Goldberg S, Campbell D, Twite M, da Cruz E, Younoszai A, Fagan T, Ivy DD, Lacour-Gayet F. Morbidity factors in the arterial switch operation. Cardiology in the Young 19(Suppl. 2), 149, 2009 105. Tissot C, Pietra BA, Boucek MM, Gilbert DJ, Campbell DN, Mitchell MB, Ivy DD, Miyamoto SD. Coronary flow reserve can differentiate stable from progressive transplant coronary artery disease in pediatric heart transplant recipients. Cardiology in the Young 19(Suppl. 2), 156, 2009. 106. Tissot C, Pietra BA, Boucek MM, Gilbert DJ, Campbell DN, Mitchell MB, Ivy DD, Miyamoto SD. Early diagnosis of transplant coronary artery disease by intravascular ultrasound (IVUS) improves the outcome of pediatric heart transplant recipients. Cardiology in the Young 19(Suppl. 2), 158, 2009. 107. Barst RJ, Ivy DD, Badesch DB, Benza RL, Elliott CG, Farber HW, Frost AE, Krichman A, Liou TG, Raskob GE, Wason P, Feldkircher K, Foreman AJ, McGoon M. REVEAL Registry: Comparison of patients with childhood-onset and adult-onset idiopathic pulmonary arterial hypertension. The Journal of Heart and 32 Lung Transplantation, Volume 28, Issue 2, Supplement 1, S146:A228, 2009 108. Barst RJ, Ivy DD, Badesch DB, Benza RL, Elliott CG, Farber HW, Frost AE, Krichman A, Liou TG, Raskob GE, Wason P, Feldkircher K, Foreman AJ, McGoon M. REVEAL Registry: Pediatric IPAH versus pediatric pulmonary vascular disease associated with congenital heart disease. The Journal of Heart and Lung Transplantation, Volume 28, Issue 2, Supplement 1, S203:A397, 2009 109. Barst RJ, Ivy DD, Badesch DB, Benza RL, Elliott CG, Farber HW, Frost AE, Krichman A, Liou TG, Raskob GE, Wason P, Feldkircher K, Foreman AJ, McGoon M. REVEAL Registry: Comparison of patients with childhood-onset and adult-onset pulmonary vascular disease associated with congenital heart disease. The Journal of Heart and Lung Transplantation, Volume 28, Issue 2, Supplement 1, S3126:A708, 2009 110. Barst RJ, Foreman AJ, Ivy DD, McGoon MD. REVEAL Registry: One year outcome of patients with congenital heart disease-associated pulmonary arterial hypertension (CHD-APAH). Am. J. Respir. Crit. Care Med., 179:A2656, 2009 111. Howley LW, Fonseca B, Sigur C, Ivy DD, Shaffer E, Younoszai A. Abnormal systolic septal curvature and diastolic dysfunction predict moderate pulmonary arterial hypertension in children. Journal of the American Society of Echocardiography, 22(5); 551:P1-20, 2009 112. Howley LW, Fonseca B, Sigur C, Ivy DD, Younoszai A. Bi-ventricular diastolic dysfunction by Doppler tissue imaging correlates with invasive markers of pulmonary hypertension in children. Journal of the American Society of Echocardiography, 22(5); 552:P1-21, 2009 113. Barst RJ, Foreman AJ, Ivy DD, McGoon MD. REVEAL Registry: Two year outcome of patients with congenital heart disease-associated pulmonary arterial hypertension (CHD-APAH). European Heart Journal: 30(Supplement 1): 259:P1578, 2009 114. Beghetti M, Berger RMF, Barst RJ, Jais X, Acar P, Fraisse A, Ivy DD, Schulze-Neick I, Bonnet D, Haworth SG. BOS1987 Longer-term survival in paediatric patients with idiopathic or familial pulmonary arterial hypertension (IPAH/FPAH) treated with bosentan: data from the FUTURE-1 and FUTURE-2 studies European Heart Journal: 30(Supplement 1): 900:5176, 2009 115. Tissot C, Pietra BA, Boucek MM, Gilbert DJ, Campbell DN, Mitchell MB, Ivy DD, Miyamoto SD. Transplant coronary artery disease is diagnosed later and is more severe in pediatric heart recipients transplanted in infancy Swiss Pediatric Society Congress, 2009 116. Tissot C, Pietra BA, Ivy DD, Mitchell MB, Campbell DN, Gilbert DJ, Miyamoto SD. Pediatric Heart Transplant Recipients with Transplant Coronary Artery Disease show a steady increase in Filling Pressures over Time. The Journal of Heart and Lung Transplantation, Volume 29, Issue 2, Supplement 1, Page S170, 2010 117. Tissot C, Pietra BA, Ivy DD, Campbell DN, Mitchell MB, Diamond B, Miyamoto SD. Asymptomatic rejection diagnosed by surveillance endomyocardial biopsy in pediatric heart transplant recipients: Do we need to treat? The Journal of Heart and Lung Transplantation, Volume 29, Issue 2, Supplement 1, Pages S171-S172, 2010 118. Al-Naami GH, Landeck II B, Ivy DD, Younoszai A. The RV/LV end systolic and systolic/diastolic time interval ratios: quick and easy echocardiographic tools 33 for pulmonary hypertension assessment in a pediatric clinic.3rd International Conference on Neonatal and Childhood Pulmonary Hypertension. Banff, Canada, 2010 119. Al-Naami GH, Landeck II B, Ivy DD, Younoszai A. Altered Right Ventricular Longitudinal Deformation is seen in Children with Pulmonary Hypertension: A Study of Myocardial Strain and Strain Rate by Speckle Tracking. 3rd International Conference on Neonatal and Childhood Pulmonary Hypertension. Banff, Canada, 2010 120. Al-Naami GH, Landeck II B, Ivy DD, Younoszai A. Rotational left ventricular mechanics are affected in children with pulmonary hypertension. Journal of the American Society of Echocardiography, 2010 121. Ivy DD, Rosenzweig EB, Calderbank M, Coleman B, Miller-Reed K, and Geppner J. Pharmacokinetics And Safety Of Ambrisentan In Pediatric Patients With Pulmonary Arterial Hypertension . Am. J. Respir. Crit. Care Med. 181: A3330, 2010 122. Yeager ME, Nguyen CM, Colvin KC, Ivy DD, and Stenmark KR .Evidence Of Neo-Lymphangiogenesis In Severe Pulmonary Artery Hypertension Am J Respir Crit Care Med 181: A6291, 2010 123. Colvin KM, Nguyen CM, Ivy DD, Stenmark KR, and Yeager ME The Unfolded Protein Response Is Activated In Severe Pulmonary Artery Hypertension Am J Respir Crit Care Med 181: A5234, 2010 124. Belchenko DD, Colvin KM, Nguyen CM, Ivy DD, Stenmark KR, and Yeager ME. High Levels Of Endothelin-1 Induce The Unfolded Protein Response In Pulmonary Artery And Microvascular Endothelium. Am J Respir Crit Care Med 181: A5235, 2010 125. Vezmar M, Malhotra SP, Zhou Z, Yu XY, Abadilla A, Rausch C, Rutledge J, Rebeyka I, McCrindle B, Cai S, Lacour-Gayet F, Ivy DD, Li J. Altitude is not a risk factor for patients undergoing bidirectional cavopulmonary anastomoses and Fontan operation: A comparative study between Denver, Edmonton and Toronto. Circulation 122: A15611, 2010 126. Barst RJ, Ivy DD, Badesch D, Benza R, Elliott CG, Farber H, Frost A, Poms A, Liou T, Raskob GE, Watson P, Feldkircher K, Foreman AJ, and McGoon MD. REVEAL Registry: Comparison of Patients with Childhood-Onset and AdultOnset Pulmonary Vascular Disease Associated with Congenital Heart Disease. Pediatr Res 33 (4): E-PAS2011:2928.323 127. Barst RJ, Ivy DD, Badesch D, Benza R, Elliott CG, Farber H, Frost A, Poms A, Liou T, Raskob GE, Watson P, Feldkircher K, Foreman AJ, and McGoon MD. REVEAL Registry: Comparison of Patients with Childhood-Onset and AdultOnset Idiopathic Pulmonary Arterial Hypertension. Pediatr Res EPAS2011:2928.324 128. Barst RJ, Ivy DD, Elliott CG, Watson P, Foreman AJ, Miller DP, and McGoon MD. Childhood Pulmonary Arterial Hypertension: Insights from the REVEAL Registry. Pediatr Res E-PAS2011:2928.325 129. Barst RJ, Humpl T, Schulze-Neick I, Raskob GE, Ivy DD, Jing Z-C, Bonnet D, M Beghetti M and Berger RMF on Behalf of the TOPP Study Group. TOPP: Clinical Features of Pediatric Pulmonary Hypertension in an International Registry. Pediatr Res E-PAS2011:3645.1 130. Yeager ME, Colvin KL, Belchenko DD, Nega M, Barajas C, Cripe PJ, Ivy DD, 34 Stenmark K. Immunologically Active And Well-Vascularized Bronchus-Associated Lymphoid Tissue Is Evident In Pulmonary Hypertension. Am J Respir Crit Care Med 181: A1325, 2011 131. Burgett Graves SL, Lanning C, Kirby KS, Calderbank M, Geppner J, Nolan SJ, Ivy DD, Shandas R, Hunter K. In-Vivo Pulmonary Vascular Stiffness Obtained From Color M-Mode Tissue Doppler Imaging And Pressure Measurements Predicts Clinical Outcomes Better Than Indexed Pulmonary Vascular Resistance In Pediatric Patients With Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 181: A5748, 2011 132. Takatsuki S, Calderbank M, Ivy DD. Initial Experience with Tadalafil In Pediatric Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 181: A5897, 2011 133. Hale BW, Kirby KS, Calderbank M, Geppner J, Ivy DD, Shandas R, Hunter K. Comparison Of Pulmonary Vascular Input Impedance And Pulmonary Vascular Resistance To Model Clinical Outcomes In Pediatric Patients With Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 181: A5928, 2011 134. Rosenzweig EB, Krishnan U, Takatsuki S, Kerstein J, Calderbank M, D.D. Ivy DD. Inhaled Treprostinil In Pediatric Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 181: A6134, 2011 135. Maxey D, Ivy DD, Ogawa M, Feinstein J. Adverse And Significant Adverse Events Associated With Pulmonary Hypertension Therapy In Pediatric Patients: An FDA PostMarket Study Am J Respir Crit Care Med 181: A5891 , 2011 136. Siehr SL, Ivy DD, Miller-Reed K, Ogawa M, Rosenthal D, and Feinstein JA. Longitudinal hemodynamic changes in pediatric patients with severe pulmonary arterial hypertension on prostanoid therapy. The Journal of Heart and Lung Transplantation. 30: A197, 2011 137. Berger RMF, Humpl T, Schulze-Neick I, Raskob G, Ivy DD, Jing ZC, Bonnet D, Beghetti M, Barst RJ. Pediatric pulmonary hypertension: insights from the Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) global registry. Eur Heart Journal, 2011 138. Barst RJ, Konourina I, Watt S, Richardson H, Layton G, Ivy DD. Pediatric Pulmonary Arterial Hypertension: Sildenafil Monotherapy for One Year in Treatment-Naïve Children . Eur Resp Journal, 2011 139. Jone P, Howley LW, Hinzman J, Ivy DD, Younoszai A. Bi-ventricular diastolic function by tissue Doppler imaging correlates with severity of pulmonary hypertension in children . Cardiology in the Young 21(Suppl. 1), S117, 2011 140. Berger RMF, Humpl T, Schulze-Neick I, Raskob G, Ivy DD, Jing ZC, Bonnet D, Beghetti M, Barst RJ. Clinical characteristics of pediatric pulmonary hypertension: results from the global registry Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP). Cardiology in the Young 21(Suppl. 1), S124, 2011 141. Barst RJ, Russell SD, Oudiz RJ, Ivy DD, Layton G, Konourina I, Watt S Effects of Oral Sildenafil Treatment on Exercise Capacity in Pediatric Pulmonary Arterial Hypertension. Chest 140(4),396A,2011 142. Barst RJ, Ivy DD, Frantz B, Frost A, Rosenzweig EB, Foreman A, Shapiro S, Taichman D, McGoon M. Impact of Functional Class Change on Survival in Patients With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease: Insights From REVEAL. Chest 140(4),716A,2011 35 143. Taylor AL, Ivy DD, and Rausch C. VE/VCO2 Slope And Functional Class In Pediatric Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 185: A1903, 2012 144. De A, Coleman B, Ivy DD, Yaron M, Ward SD. A Rare And Interesting Case Of Syncope At Altitude. Am J Respir Crit Care Med 185: A3384, 2012 145. Barst RJ, Ivy DD, Rosenzweig EB, Foreman AJ, Turner M, Meltzer LA, and McGoon MD. Characteristics And Four-Year Outcomes Of Patients With Eisenmenger Syndrome In REVEAL. Am J Respir Crit Care Med 185: A4793, 2012 146. Burgett SL, Kirby KS, Calderbank M, Ivy DD, and Hunter KS. Diameter Of The Pulmonary Artery Measured With Color M-Mode Tissue Doppler Imaging Predicts Clinical Outcomes Better Than Indexed Pulmonary Vascular Resistance In Pediatric Patients With Pulmonary Arterial Hypertension Am. J. Respir. Crit. Care Med. 185: A6739, 2012 147. Barst RJ, Layton GR, Konourina I, Richardson H, Beghetti, Ivy DD. STARTS-2: long-term survival with oral sildenafil monotherapy in treatment-naïve pediatric pulmonary arterial hypertension. Eur Heart Journal 33(Suppl 1): P5340, 2012 148. Loloyan S, Barst RJ, Brown L, Ivy DD, Rosenzweig EB, Lai G, Elliott CG, Szmuszkovic J, Oudiz R. Time to Recognition of Pediatric Pulmonary Arterial Hypertension: Factors Identified From the REVEAL Registry. Chest 142(4):824A, 2012 149. Barst RJ, Humpl T, Schulze-Neick I, Jing ZC, Levy M, Ivy DD, Bonnet D, Kronmal D, Beghetti M, Berger RMF. Outcomes Of Pulmonary Hypertension (PH) In Childhood: Results From The Global TOPP Registry. Am J Respir Crit Care Med 187;A2089,2013 150. Beghetti M, Zhang M, Ivy DD, Barst RJ. Oral Sildenafil in Children With Down Syndrome and Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 187;A2091,2013 151. Oudiz RJ, Russell SD, Zhang M, Ivy DD, Barst RJ, Beghetti M. Effects of Oral Sildenafil Treatment on Exercise Capacity in Children With Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 187;A2092,2013 152. Takatsuki S, Naoi K, Ikehara S, Nakayama T, Matsuura H, Ivy DD, Saji T. Pulmonary arterial capacitance index can predict adverse outcome in children with idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med 187;A2537,2013 153. Hobson N, Ivy DD, Hunter K. Modified Single-Beat Method For The Estimation Of Ventricular-Arterial Coupling Ratio In The Right Ventricle For Use As A Prognostic In Pediatric Pulmonary Hypertension. Am J Respir Crit Care Med 187;A2543,2013 154. Nathan SD, Hansdottir S, Feldman J, Ishizawar D, Ivy DD, Schilz R, Shapiro S, White RJ. Safety And Feasibility Of Switching Between Oral And Parenteral Treprostinil. Am J Respir Crit Care Med 187;A3276,2013 155. Zijlstra WMH, Douwes JM, Rosenzweig EB, Schokker S, Krishnan U, Roofthooft MTR, Miller-Reed K, Hillege HL, Ivy DD, Berger RMF. Transatlantic survival differences in pediatric pulmonary arterial hypertension: clues to a better understanding of disease characteristics and treatment. Am J Respir Crit Care Med 187;A3285,2013 156. Berger RMF, Haworth SG, Bonnet D, Acar P, Fraisse A, Galié N, Ivy DD, Jais , Miera O, Rosenzweig EB, Efficace M, Kusic-Pajic A, Beghetti M. Results from an open-label, long-term safety and tolerability extension study using the pediatric FormUlation of bosenTan in pUlmonary arterial hypeRtEnsion (FUTURE-2). Association for European Pediatric Cardiology, 23;S1:PW1-7,2013 36 157. Hauck A, Ivy DD, Younoszai A. Tricuspid Annular Plane Systolic Excursion Correlates with Maximal Exercise Testing but Not Six-Minute Walk Distance in Pediatric Pulmonary Hypertension. Journal of the American Society of Echocardiography 26(6), B118, 2013 158. DiMaria M, Landeck B, Younoszai A, Mertens L, Ivy DD, Hunter K, Friedberg M. Echocardiographic estimation of right ventricular stroke work in children with p: comparison to invasive measurements. Journal of the American Society of Echocardiography 26(6), B5, 2013 159. Zijlstra WMH, Douwes JM, Rosenzweig EB, Schokker S, Krishnan U, Roofthooft MTR, Miller-Reed K, Hillege JL, Ivy DD, Berger RMF. Children with pulmonary arterial hypertension (PAH) associated with congenital heart disease are treated less intensively with PAH-targeted drugs compared to children with idiopathic/hereditary PAH. Eur Heart Journal 34(Suppl 1): P304, 2013 160. Douwes JM, Barst RJ, Humpl T, Beghettit M, Bonnet D, Schulze-Neick I, Ivy DD, Berger RMF. Acute vasodilator response in paediatric pulmonary arterial hypertension: current clinical practice from the TOPP-registry. Cardiology in the Young 24(Suppl. 1), S18, 2014 161. Takatsuki S, Naoi K, Ikehara S, Nakayama T, Matsuura H, Ivy DD, Saji T. Serial assessment of pulmonary arterial capacitance index during midterm follow-up predicts adverse outcomes in children with idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med 189;A3252,2014 162. Yeager ME, Colvin KL, Ivy DD, Bull TM, Stenmark KR. Reduced phagocytic capacity drives experimental pulmonary hypertension. Am J Respir Crit Care Med 189;A3355,2014 163. Yeager ME, Colvin KL, Ivy DD, Stenmark KR. Cardiopulmonary lymphatic vessel dynamics and development of right ventricular edema in experimental pulmonary hypertension. Am J Respir Crit Care Med 189;A5551,2014 164. Di Maria M, Fagan T, Wilson N, Mitchell M, Campbell D, Jaggers J, da Cruz EM, Ivy DD, Younoszai A. Pre-Superior cavopulmonary anastomosis cardiac catheterization at altitude. Circulation 130:A19807, 2014 165. Agyei-Ntim F, Hunter K, Truong U, Ivy DD, Shandas R. Statistical Shape Modeling of the Right Ventricle Differentiates Stages of RV Remodeling in Children With Pulmonary Hypertension: An Initial Analysis Circulation 130:A15746, 2014 166. Colvin KL, Lohani O, Williams SM, Ivy DD, Stenmark K, Yeager M. Right Ventricle Lymphatic Vessel Insufficiency Contributes to Interstitial Fluid Stasis, Inflammation, Fibrosis, and Failure in Pulmonary Hypertension Circulation 130:A11636, 2014 Administrative Positions Fellowship Director, Pediatric Cardiology Director, Pediatric Pulmonary Hypertension Program Chief, Pediatric Cardiology Interim Director, Cardiac ICU Teaching and Mentoring Physiology 5000 – Clinical Correlates First year medical students Pediatrics 7000 1996-2001 1997 to present 2003 to present 2006 to 2007 2001-2003 1994 to present 37 Third year medical students Core Lecture Series Pediatric residents Pediatric Academic Track Mentor pediatric residents Fellowship Director Mentorships Direct Pediatric resident teaching and supervision Direct Pediatric Cardiology fellow teaching and supervision Pediatric Resident Research Mentor Conrad Epting, MD Kirsten Bourke, MD Eric Austin, MD Pediatric Cardiology Fellow Research Mentor Dennis Chang, MD Bibhuti Das, MD Anna Bernus, MD Jane Nydam, MD Pediatric Faculty Pulmonary Hypertension Mentor Shinichi Takatsuki, MD Erika Harder, MD 1994 to present 1998 to present 1996-2001 1994 to present 1994 to present 1998-2000 1999-2002 2002-2004 2002-2004 2002-2003 2004-2007 2005-2008 2010-2012 2013 PhD Advisor Craig Weinberg, BS 2000-2003 Ph.D. Thesis “Ultrasound evaluation of the pulmonary vascular resistance, input impedance, and right ventricular power in children with pulmonary hypertension” Dong Seok Lee, MD, PhD 2003-2004 Post-doctoral fellow Endothelin in a novel model of pulmonary hypertension Alex Barker, BS 2007-present Wall stress as determined by cardiac MRI in patients with pulmonary arterial hypertension Masters Advisor Nick Hobson, MS 2011-2012 Utility of single beat measurement of ventricular vascular coupling in children with pulmonary arterial hypertension Clinical Trials 1. An international, multicenter, randomized, placebo controlled trial of chronic UT-15 (Uniprost) in patients with pulmonary hypertension, United Therapeutics, 1999-2000, Co-Investigator 2. An international, multicenter, uncontrolled, open evaluation of chronic UT-15 (Uniprost) plus conventional therapy in patients with pulmonary hypertension, United Therapeutics, 1999-2002, Co-Investigator 3. A double-blind, randomized, placebo-controlled study to assess the effects of 38 R0 47-0203 (bosentan) on exercise capacity in patients with pulmonary arterial hypertension. Actelion, 2000-2001, Co-Investigator 4. Prophylactic use of primacor in pediatric patients at high risk of developing low cardiac output syndrome following cardiac surgery, Sanofi-Synthelabo, 2000-2001, Co-Investigator 5. A double-blind, randomized, placebo-controlled safety and efficacy study of sitaxsentan sodium treatment in patients with pulmonary arterial hypertension, ICOS-Texas Biotechnology Corporation, 2001-2002, Co-Investigator 6. A multicenter study to evaluate efficacy, safety, and pharmacokinetics of Brevibloc (Esmolol) for the treatment of hypertension in infants and children after surgical repair of coarctation of the aorta, 2001-2002, Co-Investigator 7. A study to investigate the pharmacokinetics and tolerability of bosentan and its metabolites when bosentan is given as single and multiple oral doses in children with pulmonary arterial hypertension, Actelion, 2001-2002, Co-Investigator 8. Development of a hospital-based home program for the use of inhaled nitric oxide in the chronic management of severe cardiopulmonary diseases I-NOTherapeutics, 2005-present, Co-Investigator 9. A randomized, multicenter, double-blind, placebo-controlled, dose-ranging, parallel group study of intravenous sildenafil in the treatment of children, aged 0 to 17 with pulmonary hypertension after corrective cardiac surgery, 2003-2005, Co-Investigator 10. A randomized, multicenter, double-blind, placebo-controlled, dose-ranging, parallel group study of oral sildenafil in the treatment of children, aged 1 to 16 years, with pulmonary arterial hypertension, 2003-present, Co-Investigator 11. A phase III, randomized, double-blind, placebo-controlled safety and efficacy study of sitaxsentan sodium treatment with an open-label bosentan arm in patients with pulmonary arterial hypertension, Encysive, 2003-2006, Co-Investigator 12. A double-blind safety and efficacy study of sitaxsentan sodium treatment in patients with pulmonary arterial hypertension who have failed bosentan therapy, Encysive, 20032006, Co-Investigator 13. A long-term, open-label study to evaluate the safety of sitaxsentan sodium treatment in patients with pulmonary arterial hypertension, Encysive, 2003-present, Co-Investigator 14. STEP: A double-blind placebo controlled study to evaluate the efficacy and safety of iloprost in combination with bosentan in subjects with pulmonary arterial hypertension, CoTherix, 2004-2006, Co-Investigator 15. A randomized, double-blind, placebo controlled phase 3 study of the phosphodiesterase type 5 (PDE5) inhibitor tadalafil in the treatment of patients with pulmonary arterial hypertension, 2005-present, Co-Investigator 16. FUTURE 1: an open label, multicenter study to assess the pharmacokinetics, tolerability, and safety of a pediatric formulation of bosentan in children with idiopathic pulmonary arterial hypertension, 2005-present, Co-Investigator 17. A 16-week, international , multicenter, double-blind, randomized, placebo-controlled comparison of the efficacy and safety of oral UT-15C sustained release tablets in combination with an endothelin receptor antagonist and/or a phosphodiesterase-5 inhibitor in subjects with pulmonary arterial hypertension, 2006-present, Co-Investigator 18. Randomized, placebo-controlled, double-blind, multicenter, parallel group study to assess the efficacy, safety, and tolerability of Bosentan in patients with symptomatic pulmonary arterial hypertension (ASSET 1) or pulmonary hypertension associated with sickle cell disease (ASSET 2), 2006-2009, Co-Investigator 39 19. A 16-week, international, multicenter, double-blind, randomized, placebo-controlled comparison of the efficacy and safety of oral UT-15C sustained release tablets in combination with an endothelin receptor antagonist and/or a phosphodiesterase-5 inhibitor in subjects with pulmonary arterial hypertension, 2007-2010, Co-Investigator 20. An open label, long-term, safety and tolerability extension study using the pediatric formulation of bosentan in the treatment of children with idiopathic or familial pulmonary arterial hypertension who completed FUTURE 1: FUTURE 2, 2007-2012, Co-Investigator 21. TOPP Registry: tracking outcomes and practice in pediatric pulmonary hypertension, Co-Investigator, 2007-present 22. QUERI Registry: pulmonary arterial hypertension quality enhancement research initiative, Co-Investigator, 2006-2010 23. Congenital heart disease cardiac catheterization data consortium, Co-Investigator 24. REVEAL Registry: Registry to evaluate early and long-term PAH disease management, Co-Investigator , 2006-2013 25. PEACH: Prospective evaluation of adolescents and children with pulmonary arterial hypertension, PI, 2005-present 26. A Phase 2, Randomized, Double-blind, Placebo-controlled, Multicenter, Dose-ranging Study of Cicletanine in Subjects with Pulmonary Arterial Hypertension, Co-Investigator, 2010-2012 27. A randomized, open label study comparing safety and efficacy parameters for a high and a low dose of ambrisentan (adjusted for body weight) for the treatment of pulmonary arterial hypertension in paediatric patients aged 8 years up to 18 years. Glaxo Smith Kline, Co-Investigator, Steering Committee, 2010-present 28. An open label, prospective multicenter study to assess pharmacokinetics, tolerability and safety of the pediatric formulation of bosentan twice versus three times a day in children with pulmonary arterial hypertension, Future-3, Actelion, Co-Investigator, Steering Committee, 2010-2013 29. PROSPECT Registry: Registry to Prospectively Describe Use of Epoprostenol For Injection (“RTS Epoprostenol”) in Patients with Pulmonary Arterial Hypertension, CoInvestigator, 2010-2013 30. ASPIRE Study: “A Postmarketing Observational Study to Assess Respiratory Tract Adverse Events in Pulmonary Arterial Hypertension (PAH) Patients Treated with Tyvaso (treprostinil) Inhalation Solution” Co-Investigator, 2010-present 31. Protocol H6D-MC-LVIG: A Multiple Ascending Dose Study of Tadalafil to Assess the Pharmacokinetics and Safety in a Pediatric Population with Pulmonary Arterial Hypertension. Lilly, Data Safety Monitoring Board, 2011present 32. Protocol Protocol H6D-MC-LVHV. A Double-Blind Efficacy and Safety Study of the Phosphodiesterase Type 5 Inhibitor Tadalafil in Pediatric Patients with Pulmonary Arterial Hypertension. Steering Committee, 2011-present 33. A 24 week double-blind, placebo-controlled, randomized, multicenter clinical trial to determine efficacy, safety and tolerability of QTI571 (imatinib) in the treatment of children and adolescents, aged 9 months to < 18 years of age with pulmonary arterial hypertension (PAH) who remain symptomatic on maximal available PAH therapy. Novartis, Steering Committee, 2011-2013 34. A Phase 2, Placebo Controlled, Double-Blind, Randomized, Clinical Study to Determine Safety, Tolerability and Efficacy of Pulsed, Inhaled Nitric Oxide (iNO) Versus Placebo as Add-On Therapy in Symptomatic Subjects With Pulmonary Arterial Hypertension, 2012-present 40 35. A multicenter, open-label, 24-week, uncontrolled study to evaluate the safety, tolerability , and pharmacokinetics of oral treprostinil extended release tablets following transition from Remodulin or inhaled prostacyclin therapy or as add-on to current PAH therapy in prostacyclin-naïve pediatric patients aged 7 to 17 years with pulmonary arterial hypertension, United Therapeutics, Steering Committee, 2014-present Invited Regional / National Presentations / International Presentations 1. Diagnosis and Management of Aortic Stenosis Eliza Fernie Pediatric/Neonatal Critical Care Symposium, Denver, CO, 1993 2. Moderator: Pulmonary Hypertension and Right Heart Failure The Failing Heart. The Given Biomedical Institute, Aspen, CO, 1994 3. Endothelin in the Perinatal Period Rocky Mountain Center for the Biology of Development, Breckenridge, CO, 1994 4. The Role of the Endothelium in Pulmonary Hypertension Frontiers of Cardiology, Winter Park, CO, 1995 5. The Nitric Oxide-cGMP System in Pulmonary Hypertension Frontiers of Cardiology, Winter Park, CO, 1996 6. Intravascular ultrasound in pediatric pulmonary hypertension. Team Approach to Pediatric Cardiac Disease, Vail, CO, 1997 7. Nitric Oxide in Primary Pulmonary Hypertension Pulmonary Hypertension Association Annual Meeting, Dallas, Texas, 1998 8. Role of Endothelin in Perinatal Pulmonary Vasoregulation. American Heart Association. Scientific Conference on Control Mechanisms in the Fetal and Neonatal Pulmonary Circulations, Sedalia, Colorado, 1998 9. Molecular Aspects of Pulmonary Hypertension Frontiers in Cardiology, Kelsey-Seybold Foundation, Winter Park, CO, 1999 10. Hypoplastic Left Heart Syndrome Eliza Fernie Pediatric/Neonatal Critical Care Symposium, Denver, CO, 1999 11. Role of the Endothelin B Receptor in Regulation of Pulmonary Vascular Tone Department of Physiology, University of Wisconsin, Madison, WI, 1999 12. State of the Art: Management of severe pulmonary hypertension. American College of Cardiology. Anaheim, CA, 2000 13. Journeys in Pediatric Pulmonary Hypertension Pulmonary Hypertension Association Annual Meeting, Chicago, IL, 2000 14. Treatment of Severe Pulmonary Hypertension in Congenital Heart Disease Department of Pediatrics, University of Texas Southwestern, Dallas, TX, 2000 15. Childhood Pulmonary Hypertension: Novel Therapies United Kingdom Pulmonary Hypertension Association Derby, England, 2001 16. Advances in medical management of severe pulmonary hypertension in children Children’s Hospital of Philadelphia, Philadelphia, PA, 2002 17. Bosentan / Tracleer in Pulmonary Hypertension Fifth International Pulmonary Hypertension Conference, Irvine, CA, 2002 18. Pulmonary Hypertension and Congenital Heart Disease Fifth International Pulmonary Hypertension Conference, Irvine, CA, 2002 19. Inhaled Nitric Oxide in Pulmonary Hypertension Fifth International Pulmonary Hypertension Conference, Irvine, CA, 2002 20. Ask the Parents / Ask the Kids 41 Fifth International Pulmonary Hypertension Conference, Irvine, CA, 2002 21. Flolan Treatment of Children and Patients with Congenital Heart Disease Advisory Panel on Flolan, Chicago, IL, 2002 22. Advances in Treatment of Pediatric Pulmonary Hypertension Canadian Pediatric Cardiology Society, Edmonton, Alberta, Canada, 2002 23. Diagnosis and Novel Treatment in Pediatric Pulmonary Hypertension Jackson Memorial Hospital, Miami, Florida, 2003 24. Novel Treatments for Pediatric Pulmonary Hypertension Team Approach to Pediatric Cardiac Disease Sponsored by University of Oregon Health Sciences University, Vail, Colorado, 2003 25. A New Era in the Treatment of Pediatric Pulmonary Hypertension Texas Children’s Hospital, Houston, Texas, 2003 26. Novel roles of the endothelin B receptor. American Thoracic Society Special topics in pulmonary physiology: the next generation. Seattle, WA, 2003 27. Advances in treatment of pediatric pulmonary hypertension Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada, 2003 28. Diagnosis and Management of Severe Pediatric Pulmonary Hypertension Society of Critical Care Medicine, 33rd Critical Care Congress, Orlando, FL, 2004 29. Bosentan / Tracleer in Pulmonary Hypertension Sixth International Pulmonary Hypertension Conference, Miami, FL, 2004 30. Pulmonary Hypertension and Congenital Heart Disease Sixth International Pulmonary Hypertension Conference, Miami, FL, 2004 31. Inhaled Nitric Oxide in Pulmonary Hypertension Sixth International Pulmonary Hypertension Conference, Miami, FL, 2004 32. Ask the Parents / Ask the Kids Sixth International Pulmonary Hypertension Conference, Miami, FL, 2004 33. The Latest in PH Therapies for Children Sixth International Pulmonary Hypertension Conference, Miami, FL, 2004 34. Clinical Experience and Future Perspectives on the Use of Bosentan in Pulmonary Arterial Hypertension Related to Congenital Heart Disease Tracleer International Scientific Advisory Board Meeting, Lucerne, Switzerland, 2004 35. Genetics of Pulmonary Artery Hypertension American Academy of Pediatrics, San Francisco, CA, 2004 36. Current and Emerging Therapies for Primary and Secondary Pulmonary Hypertension in Children. American College of Chest Physicians, Seattle, WA, 2004 37. How to Evaluate and Manage Pediatric Patients with Pulmonary Hypertension American Heart Association, New Orleans, LA, 2004 38. Advances in medical management of pediatric pulmonary hypertension Southeastern Pediatric Cardiology Society, Nashville, TN, 2005 39. New pharmacology in the congenital armamentarium: Pulmonary Vascular Disease. American College of Cardiology, Atlanta, GA, 2006 40. Advances in medical management of pediatric pulmonary hypertension Western Society of Pediatric Cardiology, Newport Beach, CA, 2006 41. Pharmacotherapeutic options for pulmonary arterial hypertension in children. American College of Chest Physicians, Salt Lake City, UT, 2006 42. Advances in medical management of severe pediatric pulmonary hypertension University of Oklahoma, Oklahoma City, OK, 2007 43. Moderator, Can we fix fixed secondary pulmonary hypertension, American College of Cardiology, Chicago, IL, March 2008 42 44. The evaluation and management of the patient with secondary pulmonary hypertension, American College of Cardiology, Chicago, IL, March 2008 45. A new era in the medical management of the child with severe pulmonary hypertension, Children’s Healthcare of Atlanta, April 2008 46. Medical treatment and lung transplantation in children with pulmonary hypertension Eighth International Pulmonary Hypertension Conference, Houston, Texas, May 2008 47. Pulmonary hypertension: issues in adolescents and young women Eighth International Pulmonary Hypertension Conference, Houston, Texas, May 2008 48. Advances in treatment of pediatric pulmonary hypertension. Nationwide Columbus Children’s Hospital, October 2008 49. Proteomic evaluation of pediatric pulmonary hypertension. Nationwide Columbus Children’s Hospital, October 2008 50. Prostanoids in the management of acute pulmonary hypertension. Pediatric Cardiac Intensive care Society meeting, Miami, FL, December 2008 51. Preoperative testing: what do we make of vasodilator testing? 2 nd International Neonatal and Childhood Pulmonary Vascular Disease, San Francisco, CA, March 2009 52. Update on management of severe pediatric pulmonary hypertension, 12 International Vail conference on pediatric cardiac disease, Vail, CO, March 2009 53. Management of the acutely decompensated patient with chronic pulmonary hypertension, 1st European Conference on Pediatric and Neonatal Cardiac Intensive Care, Montreux, Switzerland, May 2009 54. A new era in the medical management of severe pediatric pulmonary arterial hypertension. The 45th Annual Meeting of Japanese Society of Pediatric Cardiology and Cardiac Surgery, Kobe, Japan, July 2009 55. Vasoreactivity testing in determining operability in congenital heart disease. The 45th Annual Meeting of Japanese Society of Pediatric Cardiology and Cardiac Surgery, Kobe, Japan, July 2009 56. A new era in the medical management of severe pediatric pulmonary arterial hypertension. Evolving Concepts in the Management of Complex Congenital Heart Disease II, San Diego, CA 2010 57. A new era in the medical management of severe pediatric pulmonary arterial hypertension. Korean Pulmonary Hypertension Association, Seoul, Korea, 2010 58. Inhaled prostanoids in pediatric pulmonary arterial hypertension. Korean Pulmonary Hypertension Association, Seoul, Korea, 2010 59. A new era in the medical management of severe pediatric pulmonary arterial hypertension. Seoul National University Hospital, Seoul, Korea, 2010 60. Improving delivery of care through collaboration between administrators and healthcare providers: Business meets cardiology: metrics and scorecards. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February 2010. 61. Basic science and translational research: Neointimal lesions in the pulmonary arteries of endothelin – B receptor deficient rats: a model for severe pulmonary hypertension. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February 2010. 62. Challenges and solutions in moving patients and caregivers: Moving to an entirely new hospital. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February 2010. 63. Developing evidence-based guidelines and protocols for the diagnosis and management of pulmonary hypertension in children: How I work up a patient with 43 suspected pulmonary hypertension. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February 2010. 64. Developing evidence-based guidelines and protocols for the diagnosis and management of pulmonary hypertension in children: How I minimize complications following catheterization or non-cardiac surgery in patients with pulmonary hypertension. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February 2010. 65. Cardiac catheterization is unnecessary in the evaluation of pulmonary hypertension: CON. 3rd International Conference on Neonatal and Childhood Pulmonary Hypertension. Banff, Canada, 2010 66. Cardiac catheterization: indications, hemodynamic evaluation and pitfalls. 3 rd International Conference on Neonatal and Childhood Pulmonary Hypertension. Banff, Canada, 2010 67. New medications in pediatric pulmonary hypertension Ninth International Pulmonary Hypertension Conference, Garden Grove, CA, 2010 68. Ask the Parents / Ask the Kids Ninth International Pulmonary Hypertension Conference, Garden Grove, CA, 2010 69. New medications in pediatric pulmonary hypertension Joe DiMaggio Children’s Hospital, Hollywood, FL, 2010 70. Cardiac catheterization is unnecessary in the evaluation of pulmonary hypertension: CON. Southeastern Pediatric Cardiology Meeting, Charlotte, NC, 2010 71. New medications in pediatric pulmonary hypertension Southeastern Pediatric Cardiology Meeting, Charlotte, NC, 2010 72. Pediatric baseline data in the REVEAL registry REVEAL registry yearly meeting, Boston, MA, 2010 73. Chronic Postoperative Pulmonary Hypertension: Pulmonary Arterial Hypertension in Congenital Heart Disease, Vienna, Austria, 2011 74. An update on medical management for severe pulmonary hypertension. 13 International Vail conference on pediatric cardiac disease, Vail, CO, March 2011 75. Optimizing the pulmonary vasculature: From RDS to ARDS. Society of Pediatric Research. State of the Art Plenary Session. Denver, CO. May 2011 76. High altitude pulmonary hypertension in children. High Altitude Summit. Keystone, Colorado, October, 2011 77. Discovery proteomics in pediatric pulmonary hypertension. Syzygy: Congenital Cardiology Group, California, February, 2012 78. Humanitarian Medicine in Nicaragua. Syzygy: Congenital Cardiology Group, California, February, 2012 79. Survival and insights from the U.S. Pediatric Pulmonary Hypertension Reveal Registry. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February, 2012. 80. The role of non-endothelial cells in pediatric pulmonary hypertension. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February, 2012. 81. How should nitric oxide be used in the neonatal CHD patient in 2012. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February, 2012. 82. Inhaled therapy for pediatric pulmonary hypertension. 4th International Conference on Neonatal and Childhood Pulmonary Hypertension. San Francisco, California, 2012 44 83. Future expectations in pediatric pulmonary hypertension, European Society of Cardiology, Pulmonary Hypertension: Future Expectations, Nice, France, October, 2012 84. The pediatric sildenafil trial and the FDA decisions, European Society of Cardiology, Pulmonary Hypertension: Future Expectations, Nice, France, October, 2012 85. Diagnosis and management of portopulmonary hypertension in children. Studies of Pediatric Liver Transplantation annual meeting, Denver, Colorado, October, 12012 86. Moderator. Hot Topics in Pediatric Pulmonary Hypertension. American Heart Association Scientific Sessions, Los Angeles, CA, November, 2012 87. Chairman, Pediatric Task Force, World Symposium of Pulmonary Hypertension, Nice, France, February 2013 88. Managing acute inhaled therapies in the critical care setting. 2013 PH Professional Network Symposium. Arlington, VA, September, 2013 89. Pharmacological management of pediatric PAH. 2013 PH Professional Network Symposium. Arlington, VA, September, 2013 90. Biomarkers in pediatric PAH. 1st European Conference on Neonatal and Pediatric Pulmonary Vascular Disease. Groningen, Netherlands, October, 2013 91. Update on the World Symposium of Pulmonary Hypertension. 1 st European Conference on Neonatal and Pediatric Pulmonary Vascular Disease. Groningen, Netherlands, October, 2013 92. Development of a pediatric pulmonary hypertension program. Pulmonary Vascular Research Institute, Giessen, Germany, January, 2014 93. The FDA Sildenafil Warning for Pediatric Pulmonary Hypertension, FDA, Washington, DC, January, 2014 94. The Registry to Evaluate Early and Long Term PAH Disease Management: Pediatric Cohort. 6th International Conference on Neonatal and Childhood Pulmonary Hypertension. San Francisco, California, March 2014 95. The FDA and the sildenafil decision. Joint Council on Congenital Heart Disease. Washington, DC, September 2014 96. Pulmonary Hypertension: Intravenous, Subcutaneous, Inhaled, or Oral – What to Choose and Why. 10th International Pediatric Cardiac Intensive Care Society, Miami, FL, December 2014 Grand Rounds 1. Diagnosis and Management of Primary Pulmonary Hypertension Department of Pediatrics Grand Rounds University of Wisconsin, Madison, WI, 1999 2. The Patrick Niland Lecture in Pediatric Cardiology Department of Pediatrics Grand Rounds University of Michigan, Ann Arbor, MI, 2000 3. Diagnosis and Novel Treatment in Pediatric Pulmonary Hypertension Miami Children’s Hospital Department of Pediatrics Grand Rounds Miami, Florida, 2003 4. Novel Treatments in Pediatric Pulmonary Hypertension Joe DiMaggio Children’s Hospital Department of Pediatrics Grand Rounds Miami, Florida , 2003 5. A New Era in the Treatment of Pediatric Pulmonary Hypertension Department of Pediatrics Grand Rounds 45 Children’s Hospital of Quebec Teleconference to Saint Justine Children’s Hospital / Montreal Children’s Hospital Quebec City, Quebec, Canada, 2003 6. The Casden Lecture in Pediatric Cardiology University of Oregon Health Sciences University Department of Pediatrics Grand Rounds Portland, OR, 2003 7. Pediatric Pulmonary Hypertension: An Update Department of Pediatrics Grand Rounds Wake Forest University School of Medicine Greensborough, NC, 2003 8. A New Era in the Treatment of Pediatric Pulmonary Hypertension Department of Pediatrics Grand Rounds University of Iowa Iowa City, IA, 2004 9. The Rae Lecture in Pediatric Cardiology Department of Pediatrics Grand Rounds Indiana University School of Medicine Indianapolis, IN, April 2006 10. University of Minnesota Department of Pediatrics Grand Rounds Novel therapy in Pediatric Pulmonary Hypertension Minneapolis, MN, August 2006 11. The Weinstein Lecture in Pediatric Cardiology Department of Pediatrics Grand Rounds Johns Hopkins University Baltimore, MD, October, 2006 12. The MacDonald Dick Lecture in Pediatric Cardiology Department of Pediatrics Grand Rounds Ann Arbor, MI, December 2006 13. Rainbow Children’s Hospital Department of Pediatrics Grand Rounds Cleveland, OH, April 19, 2007 14. Shands Children’s Hospital Department of Pediatrics Grand Rounds Gainesville, FL, September, 2007 15. Arkansas Children’s Hospital Department of Pediatrics Grand Rounds Little Rock, AR, October 2007 16. The Fashena Lecture in Pediatric Cardiology University of Texas Southwestern Department of Pediatrics Grand Rounds March 5, 2008 17. Wayne State University Department of Pediatrics Grand Rounds April 22, 2011 18. Miami Children’s Hospital Department of Pediatrics Grand Rounds April 20, 2012 46 19. Cincinnati Children’s Hospital Department of Pediatrics Grand Rounds May 29, 2012 20. Duke Children’s Hospital Department of Pediatrics Grand Rounds May 1, 2013 21. LeBonheur Children's Hospital Department of Pediatrics Grand Rounds James Hunt Lecture November 20, 2013 22. Lucille Packard Children's Hospital Department of Pediatrics Grand Rounds Dunlieve Lecture April 20, 2014 23. Omaha Children's Hospital Department of Pediatrics Grand Rounds October 17, 2014