Kathryn C - University of Colorado Denver



Curriculum VitaeDavid Dunbar Ivy, MDPERSONAL DATATitle: Professor and Selby’s Chair of Pediatrics and Chief of Pediatric Cardiology, University of Colorado School of Medicine, PediatricsCURRENT ADDRESSPediatric CardiologyUniversity of Colorado Denver School of MedicineP.O. Box B10013123 East 16th AvenueAurora, CO 80045 USATelephone+1 720-777-6820FAX+1 720-777-7290Emaildunbar.Ivy@EDUCATION:Institution and Location Degree Year Field of StudyDavidson College, Davidson, NC B.S. 1980-84 BiologyTulane University, New Orleans, LA M.D. 1984-88 MedicineChildren's Hospital of Colorado, Denver, CO Intern & Resident 1988-91 PediatricsChildren's Hospital of Colorado, Denver, CO Fellow 1991-94 Pediatric CardiologyAPPOINTMENTS HELD:1994-1996Instructor of Pediatrics University of Colorado School of Medicine, Aurora, CO 1996-2001Assistant Professor of PediatricsUniversity of Colorado School of Medicine2001-2007Associate Professor of PediatricsUniversity of Colorado School of Medicine2007-presentProfessor of Pediatrics with TenureUniversity of Colorado School of MedicineProfessional Positions:1994-PresentPediatric Cardiologist, Section of Cardiology Children’s Hospital Colorado, Aurora, Colorado1996-PresentDirector of Pediatric Pulmonary Hypertension Program Children’s Hospital Colorado, Aurora, Colorado2003-PresentChief, Section of Cardiology Children’s Hospital Colorado, Aurora, ColoradoUniversity of Colorado School of MedicineAWARDS AND HONORS:1993Fellows Basic Research, The Society for Pediatric Research1995Research paper 1st place, Frontiers in Cardiology Conference2001Children’s Choice Award, Pulmonary Hypertension Program2003Selby’s Endowed Chair of Pediatric Cardiology2007 Award of Tenure, University of Colorado2005-20085280 Magazine Top Docs2010-20195280 Magazine Top Docs2008Heart Who Cares Award, Children’s Hospital Heart Institute2011-2012Patient Perception Award / Overall Top PerformerPRC National Excellence in Healthcare Awards2016CHCO NRC Patient Satisfaction Award2017Jayden DeLuca Award for dedication in advances in pediatric heartdisease2017Pediatric Congenital Heart Association Heart Heroes AwardBoard Membership in Professional Organizations:2005-2013Scientific Leadership Council, Pulmonary Hypertension Association2011-2014Board of Trustees, Pulmonary Hypertension Association2011-2019Board, Pediatric Congenital Heart Association2014-presentBoard, Association of Pediatric Pulmonary HypertensionPROFESSIONAL SOCIETIES:American College of Cardiology, FellowAmerican Academy of Pediatrics, Fellow American College of Chest Physicians, FellowAmerican Heart Association, FellowPediatric Cardiac Intensive Care SocietyPulmonary Hypertension AssociationAmerican Physiological SocietySociety for Pediatric ResearchAssociation for European Paediatric CardiologyPulmonary Vascular Research InstituteAssociation for Pediatric Pulmonary HypertensionMajor Committee and Service Responsibilities:Local1999-2000Blood Utilization Review Committee, TCH1999-2002Search Committee, Cardiac ICU Director, TCH2001Graduate Medical EducationInternal review of Nuclear Medicine and Cardiac Electrophysiology2000-2002Nominating Committee for The Children’s Hospital, 2003-2004Imagine the Miracles, The Children’s Hospital Foundation 2005-2006Search Committee, Chief Adult Cardiology, UC SOM2010-2012Data Safety and Monitoring Board: “Effect of Statin Therapy on Disease Progression in Children with ADPKD”, Melissa Cadnapaphornchai, M.D., PI2011-2013Executive Planning Committee, Denver American Heart Association Ball2013-2014Search Committee Chair, Chief Pediatric Pulmonology, UC SOM2013-2015Inpatient Efficiency, Children’s Hospital ColoradoNational/International2004 NICHD Supplemental U01 Grant Review2005-2008Society for Pediatric Research Young Investigator Award Committee2006-11, -15American College of Cardiology, abstract reviewer2007Society of Pediatric Research, abstract reviewer2009-2010NIH NHLBI Loan Repayment Program L302009-2012Gilead Research Scholars Grant Review Board2010Guidelines for Pediatric Cardiology Training Programs2010Pulmonary Hypertension Academic Research Consortium 2011NIH Workshop: Improving Outcomes in Pulmonary Hypertension2011-2013American Heart Association, National Program Planning Committee2012Cardiovascular Medical Research and Education Fund, Program Grant Review Committee2013Congressionally Directed Medical Research ProgramDepartment of Defense Grant ReviewerLicensure and board certification1993-presentMedical license, Colorado 2010-presentMedical license, Wyoming 1991Diplomate, American Board of Pediatrics 1994Diplomate, Pediatric Cardiology, American Board of Pediatrics2015Diplomate, American Heart AssociationReview and referee work CirculationJournal of the American College of CardiologyAmerican Journal of CardiologyAmerican Journal of PhysiologyBritish Journal of PharmacologyBiology of the NeonateBiology of Perinatal MedicineCritical Care MedicinePediatric PulmonologyCatheterization and Cardiovascular DiagnosisPediatric ResearchAnnals of Thoracic SurgeryAmerican Journal of Respiratory and Critical Care MedicineJournal of PediatricsPediatricsEuropean Respiratory JournalJournal of Applied PhysiologyCirculation Journal JapanJournal of Infection and Public HealthChestJournal of Critical CareAdvances in Pulmonary HypertensionAmerican Journal of Cardiovascular DrugsFrontiers in Pediatric CardiologyCurrent Medical Research and OpinionInternational Journal of CardiologyActa PhysiologicaEditorial Board2002-2004Topic Editor, Pulmonary Circulation Journal Club American Thoracic Society2005-presentCongenital Heart Disease: Clinical Studies from Fetus to Adulthood 2011-presentAdvances in Pulmonary Hypertension, Pulmonary Hypertension Association2011-present Frontiers in Pediatric CardiologyAdministrative Positions1996-2001Fellowship Director, Pediatric Cardiology1997-presentDirector, Pediatric Pulmonary Hypertension Program2003-presentChief, Pediatric Cardiology2006-2007Interim Director, Cardiac ICUTeaching2001-2003Physiology 5000, Clinical Correlates / First year medical students1994-presentPediatrics 7000, Third year medical students1994-2002 Core Lecture Series, Pediatric residents1996-2001Fellowship Director1994-presentDirect pediatric resident teaching and supervision1994-presentDirect cardiology fellow teaching and supervisionMentoringMedical Student Research Mentor2016-2017 Karel BreemanCardiac MRI in determination of ventricular vascular coupling in children with pulmonary hypertension, University of Groningen, Netherlands2019-2020 Sandra BarzlaafCardiac MRI in determination of end-systolic and end-diastolic stress fraction in children with pulmonary hypertension, University of Munich, GermanPediatric Resident Research Mentor1998-2000Conrad Epting, MD1999-2002Kirsten Bourke, MD2002-2004Eric Austin, MD2018-2019Menno Douwes, MD, PhDPediatric Cardiology Fellow Research Mentor2002-2004Dennis Chang, MD 2002-2003Bibhuti Das, MD2004-2007Anna Bernus, MD2005-2008Jane Nydam, MD2014-2017Rick Friesen, MD2017-2019Benjamin Frank, MDPediatric Faculty Pulmonary Hypertension Mentor2003-2004Dong Seok Lee, MD, PhD2010-2012Shinichi Takatsuki, MD2013Erika Harder, MD2013-presentUyen Truong, MD2013-presentPei-Ni Jone, MD2019-presentBenjamin Frank, MDMaster’s Advisor2000-2003Craig Weinberg, MSUltrasound evaluation of the pulmonary vascular resistance, input impedance, and right ventricular power in children with pulmonary hypertension2007-2008Alex Barker, MSWall stress as determined by cardiac MRI in patients with pulmonary arterial hypertension2011-2012Nick Hobson, MSUtility of single beat measurement of ventricular vascular coupling in children with pulmonary arterial hypertension2016Michal Schafer, MS4D Flow in children with pulmonary hypertensionPhD Advisor2014-2015 Shawna Burgett, PhDMechanics of the proximal pulmonary artery in pulmonary hypertensionUniversity of Colorado, Department of Bioengineering2015-2017Willemijn Zijlstra, MD PhDPulmonary arterial hypertension: Towards optimal classification, treatment strategies and outcome University of Groningen, Netherlands2016-2017Frank Agyei-ntim, PhDStatistical shape modeling of the right ventricle in pediatric pulmonary hypertensionUniversity of Colorado, Department of Bioengineering2016-2018Michal Schafer, PhD4D Flow in children with pulmonary hypertensionUniversity of Colorado, Department of Bioengineering2018-2020Melanie Dufva, PhDLeft ventricular mechanics in relation to right ventricular contractile performance in pediatric pulmonary arterial hypertensionUniversity of Colorado, Department of BioengineeringRECENT RESEARCH GRANTS:75F40119F10090 (Ivy)9/01/19 - 8/31/212.0 calendar months$720,000DHHS/FDA/OAGS/DCSCStudy to Investigate the Combination of Physical Activity and Heart Rate with the Use of Actigraphy as a Novel, Well-Defined, Reliable, Sensitive, Easy to Use, and Non-Invasive Study Endpoint to Facilitate Pediatric Pulmonary Arterial Hypertension Trials and Drug Development for Children Ages 0-6 years. Goal: To evaluate actigraphy as an endpoint in pediatric pulmonary hypertension for children 0-6 yearsR01HL135114 (Everett)1/1/2017 – 12/31/200.3 calendar monthsNIH/NHLBI$413,137Clinical and Mechanistic Role of HDGF in Pulmonary HypertensionGoal: To validate hepatoma derived growth factor, a new circulating indicator of PAH severity and survival.Austin10/1/17 - 9/30/200.11 calendar month$6,818Cardiovascular Medical Research and Education Fund / Vanderbilt UniversityPPHNet BiOspecimen Laboratory Depot (PPHNet-BOLD)Goal: To establish a protein and DNA biobankCOMPLETED RESEARCH GRANTS:American Heart Association Fellowship AwardTitle: Role of Endothelin in Perinatal Pulmonary HypertensionFunding: AHA of Colorado (CF-035-93) Total Period 1993-1994PI:Dunbar Ivy, MDRole: PI% effort: 75%Bugher Physician Scientist Training ProgramTitle: Endothelin in the Fetal Pulmonary CirculationFunding: Bugher Physician Scientist ProgramTotal Period 1994-1996PI:Dunbar Ivy, MDRole: PI% effort: 75%Clinical Research Center, The Children’s Hospital of DenverTitle: Use of Intravascular Ultrasound in Pediatric Pulmonary HypertensionFunding: The Children’s Hospital of DenverTotal Period 1995-1997PI:DunbarIvy, MDRole: PI% effort: 25%Children’s Hospital Research Institute Career Development AwardTitle: Endothelin in the Perinatal Pulmonary CirculationFunding: The Children’s Hospital of DenverTotal Period 1996-1998PI:DunbarIvy, MDRole: PI% effort: 50%March of Dimes Basil O’Connor Starter Scholar AwardTitle: Role of Endothelin in Perinatal Pulmonary HypertensionFunding: March of DimesTotal Period 1997-1999PI:DunbarIvy, MDRole: PI% effort: 75%American Heart AssociationTitle: Role of the Endothelin B Receptor in a Model of Pulmonary HypertensionFunding: AHA, Desert/Mountain (9951441Z)Total Period 1999-2001PI:DunbarIvy, MDRole: PI% effort: 75%K-08 National Institutes of HealthTitle: Role of Endothelin in Perinatal Pulmonary VasoregulationFunding: NIH / NHLBI (K08 HL03823)Total Period 1999-2003PI:DunbarIvy, MDRole: PI % effort: 75%R01 National Institutes of HealthTitle: Noninvasive Assessment of Pediatric Pulmonary HypertensionFunding: NIH / NHLBI (R01 HL067393)Total Period 2001-2006PI:Robin Shandas, PhDRole: Co-Investigator% effort: 5%R38 US Department of Health and Human ServicesTitle: Infant Heart Transplantation from Non-Heart-Beating Donors: A Strategy to Reduce Waiting MortalityFunding: USDHHS (R38 OTO)Total Period 2003-2006PI:Mark Boucek, MDRole: Co-Investigator% effort: 10%U01 NIH/NHLBI Clinical Proteomics ProgramsTitle: Clinical Proteomics Program in Lung DiseaseFunding: NIH / NHLBI (U01 HL081335)Total Period 2005-2009PI:Frank Accurso, MDRole: Co-Investigator% effort: 3%National Institutes of HealthTitle: Sildenafil in Treatment of Sickle Cell Disease Associated Pulmonary HypertensionFunding: NIH / NHLBI (HHSN268200617185C)Total Period 2006-2009PI:David Badesch, MDRole: Co-Investigator% effort: 3%P50 Specialized Centers of Clinically Oriented Research (SCCOR)Title: Lung Vascular Disease in Infants and Children: Mechanisms and TreatmentFunding: NIH/NHLBI (P50 HL084923)Total Period 2006-2011PI:Kurt Stenmark, MDRole: Co-Investigator% effort: 10%R01 National Institutes of Health Title: Functional and Biological Phenotyping of Pediatric Pulmonary HypertensionFunding: NIH / NHLBI (R01 HL114753)Total Period 2012-2017PI:Robin Shandas, PhDRole: Co-Investigator% effort: 0.6 calendarR24 National Institutes of Health Title: National Biological Sample and Data Repository for PAHFunding: NIH / NHLBI (R24 HL105333)Total Period 2012-2018PI:Bill Nichols, MDRole: Co-Investigator% effort: 0.11 calendarU01 HL121518 (Abman/Mandl) 07/1/14 - 4/30/191.2 calendar monthsNIH/NHLBI$2,035,085Data Fusion: A Sustainable, Scalable, Open Source Registry Advancing Pulmonary Vascular Disease ResearchGoal: To compare informatics approaches to gain knowledge of pulmonary hypertension in children by i2b2 vs a dedicated registry.FDA HHSF223201610046C (Ivy)7/11/16 - 3/31/192.0 calendar months$234,699Office of Pediatric Therapeutics, Food and Drug AdministrationInvestigation of Actigraphy, an Exercise Measurement Device, as a Novel, Well-defined, Reliable, Feasible, Easy to Use, and Non-Invasive Study Endpoint to Facilitate Pediatric Pulmonary Arterial Hypertension Trials and Drug DevelopmentGoal: To evaluate actigraphy as an endpoint in pediatric pulmonary hypertension1R01HL135114 (Everett, PI) 11/18-10/19 0.6 calendar monthsAdmin. SupplementNIH/NHLBI$271,992Clinical and mechanistic role of HDGF in pulmonary hypertensionSerum from Down’s syndrome enrollees with and without pulmonary hypertension will be assayed for a panel of pulmonary hypertension biomarkers and compared to a large group of non-Downs patients for determination of potential additional benefit to patients with Down syndrome.PUBLICATIONS:Sehgal A, Steenhorst JJ, Mclennan DI, Merkus D,?Ivy D, McNamara PJ. The Left Heart, Systemic Circulation and Bronchopulmonary Dysplasia: Relevance to Pathophysiology and Therapeutics. J Peds 020 Jun 14;S0022-3476(20)30732-0. PMID:?32553872Ivy D. The Failing Right Ventricle: Where Do We Go From Here.Acta Physiol (Oxf). 2020 Jun 20:e13523. doi: 10.1111/apha.13523. PMID:?32562585Frank BS, Sch?fer M, Thomas TM, Haxel C, Ivy DD, Jone PN. Right Atrial Conduit Phase Emptying Predicts Risk of Adverse Events in Pediatric Pulmonary Hypertension. J Am Soc Echocardiography. 2020 Apr 23. PMID: 32336608Burkett DA, Patel SS, Mertens L, Friedberg MK,?Ivy DD. Relationship Between Left Ventricular Geometry and Invasive Hemodynamics in Pediatric Pulmonary Hypertension. Circ Cardiovasc Imaging.?2020May;13(5):e009825. PMID:?32408829Hopper RK, Ivy DD, Yung D, Mullen MP, Hanna BD, Kirkpatrick E, Hirsch R, Austin ED, Fineman J, Solum D, Deng CQ, Feinstein JA. Pharmacokinetics of Oral Treprostinil in Children with Pulmonary Arterial Hypertension. J Cardiovasc Pharmacol. 2020 Apr 16. PMID:32398473Kheyfets VO, Dufva MJ, Boehm M, Tian X, Qin X, Tabakh JE, Truong U,?Ivy D, Spiekerkoetter E. The left ventricle undergoes biomechanical and gene expression changes in response to increased right ventricular pressure overload. Physiol Rep. 2020 May;8(9):e14347. doi: 10.14814/phy2.14347. PMID: 32367677Sch?fer M, Frank BS, Humphries SM, Hunter KS, Carmody KL, Jacobsen R, Mitchell MB, Jaggers J, Stone ML, Morgan GJ, Barker AJ, Browne LP, Ivy DD, Younoszai A, DiMaria MV. Flow profile characteristics in Fontan circulation are associated with single ventricle dilation and function: principal component analysis study. Am J Physiol Heart Circ Physiol. 2020 May 1;318(5)H1032-H1040. PMID: 32167782Coleman RD, Chartan CA, Ivy DD. MicroRNA as a Biomarker in Pediatric Pulmonary Hypertension: A Step Closer to the Holy Grail? Pediatr Crit Care Med 2020 Apr;21(4):393-394. PMID: 32251186Simpson CE, Chen JY, Damico RL, Hassoun PM, Martin LJ, Yang J, Nies M, Griffiths M, Vaidya RD, Brandal S, Pauciulo MW, Lutz KA, Coleman AW, Austin ED, Ivy DD, Nichols WC, Everett AD. Cellular sources of IL-6 and associations with clinical phenotypes and outcomes in PAH. Eur Respir J. 2020 Feb 6. pii: 1901761. doi: 10.1183/13993003.01761-2019. PMID: 32029443Simpson CE, Damico RL, Hassoun PM, Martin LJ, Yang J, Nies MK, Vaidya RD, Brandal S, Pauciulo MW, Austin ED, Ivy DD, Nichols WC, Everett AD. Noninvasive prognostic biomarkers for left heart failure as predictors of survival in pulmonary arterial hypertension. Chest. 2020 Jan 24. pii: S0012-3692(20)30129-X. doi: 10.1016/j.chest.2019.12.037. PMID: 31987881Di Maria MV, Campbell KR, Burkett DA, Younoszai AK, Landeck BF 2nd, Mertens L, Ivy DD, Hunter KS, Friedberg MK. Parameters of Right Ventricular Function Reveal Ventricular-Vascular Mismatch as Determined by Right Ventricular Stroke Work versus Pulmonary Vascular Resistance in Children with Pulmonary Hypertension. J Am Soc Echocardiography. 2020 Feb;33(2):218-225. PMID: 31836268Frank B, Sch?fer M, Douwes JM, Ivy DD, Abman SH, Davidson JA, Burzlaff S, Mitchell MB, Morgan GJ, Browne L, Barker AJ, Truong U, Von Alvensleben JC. Novel Measures of Left Ventricular Electromechanical Discoordination Predict Clinical Outcomes in Children with Pulmonary Arterial Hypertension. Am J Physiol Heart Circ Physiol. 2020 Feb 1;318(2):H401-H412. PMID: 31858817.Sch?fer M, Barker AJ, Jaggers J, Morgan G, Stone ML, Truong U, Browne LP, Ivy DD, Mitchell MB. Abnormal aortic flow conduction is associated with increased viscous energy loss in patients with repaired tetralogy of Fallot. Eur J Cardiothoracic Surg. 2020 Mar 1;57(3);588-595. PMID: 31535124Sch?fer M, Barker AJ, Morgan GJ, Jaggers J, Stone ML, Browne LP, Ivy DD, Mitchell MB. Increased systolic vorticity in the left ventricular outflow tract is associated with abnormal aortic flow formations in Tetralogy of Fallot. Int J Cardiovasc Imaging. 2020 Apr;36(4):691-700. PMID: 31907684Avitabile CM, Vorhies EE, Ivy DD. Drug Treatment of Pulmonary Hypertension in Children.Paediatr Drugs. 2020 Apr;22(2):123-147. PMID: 31960361Geva A, Abman SH, Manzi SF, Ivy DD, Mullen MP, Griffin J, Lin C, Savova GK, Mandl KD. Adverse Drug Event Rates in Pediatric Pulmonary Hypertension: A Comparison of Real-World Data Sources. J Am Med Inform Assoc. 2020; 27: 294–300. PMID: 31769835.Sch?fer M, Browne LP, Jaggers J, Barker AJ, Morgan GJ, Ivy DD, Mitchell MB. Abnormal left ventricular flow organization following repair of tetralogy of Fallot. J Thorac Cardiovasc Surg. 2019 Nov 22. pii: S0022-5223(19)33122-8. PMID: 31924354Russell S, Beghetti M, Oudiz R, Balagtas C, Zhang M, Ivy DD. Effects of oral sildenafil on exercise capacity in children with pulmonary arterial hypertension: a randomized trial. Open Heart. 2019;6:e001149. PMID: 31908813.McLennan D,?Ivy D, Morgan GJ. Transvenous implantation of the Occlutech atrial flow regulator: preliminary results from swine models. Congenit Heart Dis. 2019 Sep;14(5):819-831. PubMed PMID: 31328339Beghetti M, Gorenflo M, Ivy DD, Moledina S, Bonnet D. Treatment of Pediatric Pulmonary Hypertension: A Focus on the NO-sGC-cGMP pathway. Pediatric Pulmonology. 2019 Oct;54(10):1516-1526. PubMed PMID: 31313530Bush D, Wolter-Warmerdam K, Wagner BD, Galambos C, Ivy DD, Abman S, McMorrow D, Hickey F. Angiogenic Profile Identifies Pulmonary Hypertension in Children with Down Syndrome. Pulm Circ. 2019 Jul 11:2045894019866549. doi: 10.1177/2045894019866549. PubMed PMID: 31293212Ivy DD, Feinstein J, Yung D, Mullen M, Kirkpatrick EC, Hirsch R, Austin E, Fineman J, Truong U, Solum D, Deng CQ, Hopper RK. Oral Treprostinil in Transition or as Add-on Therapy in Pediatric Pulmonary Arterial Hypertension. Pulm Circ.?2019 Jul-Sep;9(3):2045894019856471. doi: 10.1177/2045894019856471.?PubMed PMID: 31215336.Kanaan U, Varghese N, Coleman R, Huckaby J, Lawrence P, Jorgensen L, Miller-Reed K, Day R, Mallory G, Ivy DD. Oral treprostinil use in children - a multi-center, observational experience. Pulm Circ.?2019 Jul-Sep;9(3):2045894019862138. doi: 10.1177/2045894019862138. PubMed PMID: 31215346.Hall K, Ogawa M, Sakarovitch C, Hopper RK, Adamson GT, Hanna B, Ivy DD, Miller-Reed K, Yung D, McCarthy E, Siehr-Handler SL, Feinstein JA. Subcutaneous and Intravenous Treprostinil Pharmacokinetics in Children With Pulmonary Vascular Disease. J Cardiovasc Pharmacol. 2019 Jun;73(6):383-393. PubMed PMID: 31162247 Galambos C, Mullen MP, Shieh JT, Schwerk N, Kielt MJ, Ullmann N, Boldrini R, Stucin-Gantar I, Haass C, Bansal M, Agrawal PB, Johnson J, Peca D, Surace C, Cutrera R, Pauciulo MW, Nichols WC, Griese M,?Ivy D, Abman SH, Austin ED, Danhaive O. Phenotype characterisation of?TBX4?mutation and deletion carriers with neonatal and pediatric pulmonary hypertension. Eur Respir J. 2019 Aug 22;54(2). pii: 1801965. doi: 10.1183/13993003.01965-2018. PMID: 31151956 Caicedo L, Hopper RK, Garcia H, Ivy DD, Haag D, Fineman J, Humpi T, Al-Tamimi O, Feinstein J, Berger RM, Berman-Rosenzweig E, Kashour T, Diaz GF, Mendoza A, Bobhate P, Handler S, Lopes AA, Barwad P, Kumar Rahit M, Krishnan U, Adatia ITK, Moledina S, Abman S, Cerro Marin MJD. Acute Vasoreactivity Testing in Pediatric Idiopathic Pulmonary Arterial Hypertension: an international Survey on Current Practice. Pulm Circ. 2019 May 30:2045894019857533. doi: 10.1177/2045894019857533. PMID: 31144586Breeman KTN, Dufva M, Ploegstra MJ, Kheyfets V, Willems TP, Wigger J, Hunter KS, Ivy DD, Berger RMF, Truong U. Right ventricular-vascular coupling ratio in pediatric pulmonary arterial hypertension: A comparison between cardiac magnetic resonance and right heart catheterization measurements. Int J Cardiol. 2019 Oct 15;293:211-217. PMID: 31109778Ollivier C, Sun H, Amchin W, Beghetti M, Berger RMF, Breitenstein S, Garnett C, Gullberg N, Hassel P,?Ivy D, Kawut SM, Klein A, Lesage C, Migdal M, Nije B, Odermarsky M, Strait J, de Graeff PA, Stockbridge N. New Strategies for the Conduct of Clinical Trials in Pediatric Pulmonary Arterial Hypertension: Outcome of a Multistakeholder Meeting With Patients, Academia, Industry, and Regulators, Held at the European Medicines Agency on Monday, June 12, 2017. J Am Heart Assoc. 2019 May 21;8(10):e011306. doi: 10.1161/JAHA.118.011306. PubMed PMID:31088189Beghetti M, Brand M, Berger RMF, Humpl T, Wheeler JG, Ivy DD, Bonnet D; TOPP investigators. Meaningful and feasible composite clinical worsening definitions in paediatric pulmonary arterial hypertension: An analysis of the TOPP registry. Int J Cardiol. 2019 Aug 15;289:110-115. PubMed PMID:31072635Giesenhagen AM, Ivy DD, Brinton JT, Meier MR, Weinman JP, Liptzin DR.High Altitude Pulmonary Edema in Children: A Single Referral Center Evaluation. J Pediatr. 2019 Apr 17. pii: S0022-3476(19)30260-4. PubMed PMID:31005280McLennan D, Sch?fer M, Mitchell MB, Morgan GJ,?Ivy D, Barker AJ, Jacobsen R. Usefulness of 4D-Flow MRI in Mapping Flow Distribution Through Failing Fontan Circulation Prior to Cardiac Intervention. Pediatr Cardiol. 2019 Jun;40(5):1093-1096. PubMed PMID: 30982076.Sch?fer M, Browne LP, von Alvensleben JC, Mitchell MB, Morgan GJ, Ivy DD, Jaggers J. Ventricular interactions and electromechanical dyssynchrony after Ross and Ross-Konno operations. J Thorac Cardiovasc Surg 2019 Aug;158(2):509-517. PMID: 30928217Kiely D, Levin D, Hassoun P, Ivy DD, Jone PN, Bwika J, Kawut SM, Lordan J, Lungu A, Mazurek J, Moledina S, Olschewski H, Peacock A, Puri GD, Rahaghi F, Schafer M, Schiebler M, Screaton N, Tawhai M, Van Beek EJ, Vonk-Noordegraaf A, Vanderpool RR, Wort J, Zhao L, Wild J, Vogel-Claussen J, Swift A. Statement on imaging and pulmonary hypertension from the Pulmonary Vascular Research Institute (PVRI). Pulm Circ 2019; Mar 18:2045894019841990. doi: 10.1177/2045894019841990. PMID: 30880632Frank BS, Sch?fer M, Grenolds A, Ivy DD, Abman SH, Darst JR. Acute Vasoreactivity Testing during Cardiac Catheterization of Neonates with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension. J Pediatr. 2019 May;208:127-133. PubMed PMID: 30871795.Sch?fer M, Ivy DD, Abman SH, Stenmark KR, Browne L, Barker AJ, Mitchell MB, Morgan GJ, Wilson N, Shah A, Kollengode M, Naresh N, Fonseca B, DiMaria M, Buckner JK, Hunter KS, Kheyfets VO, Fenster BE, Truong U. Differences in pulmonary arterial flow hemodynamics between children and adults with pulmonary arterial hypertension as assessed by 4D-flow CMR studies. Am J Physiol Heart Circ Physiol.?2019?May 1;316(5):H1091-H1104. PubMed PMID: 30822118.Goreczny S, Ivy D, Anderson R. “The person who influenced me most was the person who disagreed most strongly with me": an interview with Professor Robert Anderson. Cardiol Young. 2019 Mar;29(3):259-262. PubMed PMID:30793679.Jone PN, Ivy DD.??Comprehensive Noninvasive Evaluation of Right Ventricle-Pulmonary Circulation Axis In Pediatric Patients with Pulmonary Hypertension. Curr Treat Options Cardiovasc Med. 2019 Feb 9;21(1):6. PubMed PMID: 30739224.Frank BS, Runciman M, Manning WA, Ivy DD, Abman SH, Howley L. Pulmonary Hypertension Secondary to Scurvy in a Developmentally Typical Child.?J Pediatr. 2019 May;208:291-291.e2. PubMed PMID: 30738657. Ivy D, Wilson N. Tale of 2 Endothelin Receptor Antagonists in Eisenmenger Syndrome. Circulation. 2019 Jan 2:139(1):64-66. PubMed PMID: 30715938Kheyfets V, Truong U, Ivy D, Shandas R. Structural and Biomechanical Adaptations of Right Ventricular Remodeling - in Pulmonary Arterial Hypertension - Reduces Left Ventricular Rotation During Contraction: A Computational Study. J Biomech Eng. 2019 2019 Feb 4. doi: 10.1115/1.4042682. PMID: 30714069.Jone PN, Sch?fer M, Pan Z, Ivy DD.?Right Ventricular-Arterial Coupling Ratio Derived From 3-Dimensional Echocardiography Predicts Outcomes in Pediatric Pulmonary Hypertension. Circ Cardiovasc Imaging. 2019 Dec;12(1):e008176. doi: 10.1161/CIRCIMAGING.118.008176. PMID: 30632388.Rosenzweig EB, Abman SH, Adatia I, Beghetti M, Bonnet D, Haworth S, Ivy DD, Berger RMF. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J. 2019 Jan 24;53(1). PubMed PMID: 30545978. Sch?fer M, Younoszai A, Truong U, Browne LP, Mitchell MB, Jaggers J, Campbell DN, Hunter KS, Ivy DD, Di Maria MV.?Influence of aortic stiffness on ventricular function in patients with Fontan circulation.?J Thorac Cardiovasc Surg.?2019 Feb;157(2):699-707. PubMed PMID: 30396734.Rhodes CJ, Batai K, Bleda M, Haimel M, Southgate L, Germain M, Pauciulo MW, Hadinnapola C, Aman J, Girerd B, Arora A, Knight J, Hanscombe KB, Karnes JH, Kaakinen M, Gall H, Ulrich A, Harbaum L, Cebola I, Ferrer J, Lutz K, Swietlik EM, Ahmad F, Amouyel P, Archer SL, Argula R, Austin ED, Badesch D, Bakshi S, Barnett C, Benza R, Bhatt N, Bogaard HJ, Burger CD, Chakinala M, Church C, Coghlan JG, Condliffe R, Corris PA, Danesino C, Debette S, Elliott CG, Elwing J, Eyries M, Fortin T, Franke A, Frantz RP, Frost A, Garcia JGN, Ghio S, Ghofrani HA, Gibbs JSR, Harley J, He H, Hill NS, Hirsch R, Houweling AC, Howard LS, Ivy D, Kiely DG, Klinger J, Kovacs G, Lahm T, Laudes M, Machado RD, Ross RVM, Marsolo K, Martin LJ, Moledina S, Montani D, Nathan SD, Newnham M, Olschewski A, Olschewski H, Oudiz RJ, Ouwehand WH, Peacock AJ, Pepke-Zaba J, Rehman Z, Robbins I, Roden DM, Rosenzweig EB, Saydain G, Scelsi L, Schilz R, Seeger W, Shaffer CM, Simms RW, Simon M, Sitbon O, Suntharalingam J, Tang H, Tchourbanov AY, Thenappan T, Torres F, Toshner MR, Treacy CM, Noordegraaf AV, Waisfisz Q, Walsworth AK, Walter RE, Wharton J, White RJ, Wilt J, Wort SJ, Yung D, Lawrie A, Humbert M, Soubrier F, Trégou?t DA, Prokopenko I, Kittles R, Gr?f S, Nichols WC, Trembath RC, Desai AA, Morrell NW, Wilkins MR; UK NIHR BioResource Rare Diseases Consortium.; UK PAH Cohort Study Consortium.; US PAH Biobank Consortium. Genetic Determinants of risk in pulmonary arterial hypertension: international genome-wide association studies and meta-analysis. Lancet Respir Med. 2019?Mar;7(3):227-238. PubMed PMID: 30527956.Sch?fer M, Frank BS, Ivy DD, Wilson N, Morgan GJ, Barker AJ, Browne LP, Mitchell MB, Truong U.?Close look at the Potts shunt flow hemodynamics in a patient with severe pulmonary hypertension: 4D-flow MRI evaluation.?J Magn Reson Imaging. 2019?Jun;49(6):1800-1802 PubMed PMID: 30451346.Sch?fer M, Younoszai A, Truong U, Browne LP, Mitchell MB, Jaggers J, Campbell DN, Hunter KS, Ivy DD, Di Maria MV.?Influence of aortic stiffness on ventricular function in patients with Fontan circulation. J Thorac Cardiovasc Surg. 2019?Feb;157(2):699-707. PubMed PMID: 30396734.Zhu N, Welch CL, Wang J, Allen PM, Gonzaga-Jauregui C, Ma L, King AK, Krishnan U, Rosenzweig EB, Ivy DD, Austin ED, Hamid R, Pauciulo MW, Lutz KA, Nichols WC, Reid JG, Overton JD, Baras A, Dewey FE, Shen Y, Chung WK. Rare variants in SOX17 are associated with pulmonary arterial hypertension with congenital heart disease. Genome Med. 2018 Jul 20;10(1):56. PubMed PMID: 30029678Sch?fer M, Browne LP, Morgan GJ, Barker AJ, Fonseca B, Ivy DD, Mitchell MB.?Reduced proximal aortic compliance and elevated wall shear stress after early repair of tetralogy of Fallot. J Thorac Cardiovasc Surg. 2018 Dec;156(6):2239-2249. PubMed PMID: 30449579.Bush D, Galambos C, Ivy DD, Abman SH, Wolter-Warmerdam, Hickey F. Clinical Characteristics and Risk Factors for Developing Pulmonary Hypertension in Children with Down Syndrome. J Pediatrics 2018 Nov; 202:212-219. PubMed PMID: 30025669Sch?fer M, Wilson N, Ivy DD, Ing R, Abman SH, Browne L, Morgan G, Ross M, McLennan D, Barker A, Fonseca B, Di Maria M, Hunter KS, Truong U. Non-invasive Wave Intensity Analysis Predicts Functional Worsening in Children with Pulmonary Arterial Hypertension. Am J Physiol Heart Circ Physiol. 2018 Oct 1;315(4):H968-H977. Pubmed PMID: 30004811Dufva MJ, Truong U, Tiwari P, Ivy DD, Shandas R, Kheyfets V. Left Ventricular Torsion Rate and the Relation to Right Ventricular Function in Pediatric Pulmonary Arterial Hypertension. Pulm Circ 2018; Jul-Sep;8(3):2045894018791352. Pubmed PMID: 30003835Friesen RM, Sch?fer M, Ivy DD, Abman SH, Stenmark K, Browne LP, Barker AJ, Hunter KS, Truong U. Proximal pulmonary vascular stiffness as a prognostic factor in children with pulmonary arterial hypertension. Eur Heart J Cardiovasc Imaging. 2019?Feb 1;20(2):209-217. PubMed PMID: 29788051Sch?fer M, Truong U, Ivy DD, Fonseca B, Malone L, DiMaria M, Barker AJ, Vargas D, Hunter KS, Jone PN, Browne LP. Children with Kawasaki disease present elevated stiffness of great arteries: Phase-contrast MRI study. J Magn Reson Imaging. 2018 Nov;48(5):1228-1236. PubMed PMID: 29707843.Sch?fer M, Browne LP, Truong U, Jaggers JJ, Mitchell MB, Malone L, Morgan G, Chatfield K, McLennan D, Turbendian H, Vargas D, Fonseca B, DiMaria M, Shah A, Ivy MP, Barker AJ, Hunter KS, Wilson N, Ivy DD, Campbell DN. Aortic stiffness in adolescent Turner and Marfan syndrome patients. Eur J Cardiothorac Surg. 2018 Nov 1;54(5):926-932. PubMed PMID: 29684119Sch?fer M, Morgan GJ, Mitchell MB, Ross M, Barker AJ, Hunter KS, Fonseca B, DiMaria M, Vargas D, Ivy DD, Wilson N, Browne LP. Impact of different coarctation therapies on aortic stiffness: phase-contrast MRI study. Int J Cardiovasc Imaging 34(9):1459-1469. PubMed PMID: 29667078Zhu N, Gonzaga-Jauregui C, Welch CL, Ma L, Qi H, King AK, Krishnan U, Rosenzweig EB, Ivy DD, Austin ED, Hamid R, Nichols WC, Pauciulo MW, Lutz KA, Sawle A, Reid JG, Overton JD, Baras A, Dewey F, Shen Y, Chung WK. Exome Sequencing in Children with Pulmonary Arterial Hypertension Demonstrates Differences Compared with Adults. Circ Genom Precis Med. 2018 Apr;11(4): e001887. PubMed PMID: 29631995Kinsella JP, Steinhorn RH, Mullen MP, Hopper RK, Keller RL, Ivy DD, Austin ED, Krishnan US, Rosenzweig EB, Fineman JR, Everett AD, Hanna BD, Humpl T, Raj JU, Abman SH; Pediatric Pulmonary Hypertension Network (PPHNet). The Left Ventricle in Congenital Diaphragmatic Hernia: Implications for the Management of Pulmonary Hypertension. J Pediatr. 2018 Jun; 197:17-22. PubMed PMID: 29628412Frank B, Ivy DD. Diagnosis, Evaluation and Treatment of Pulmonary Arterial Hypertension in Children. Children (Basel). 2018 Mar 23;5(4). PubMed PMID: 29570688Lee WS, Wong SY, Ivy DD, Sokol RJ. Hepatopulmonary Syndrome and Portopulmonary Hypertension in Children: Recent Advances in Diagnosis and Management. J Pediatr. 2018 May; 196:14-21. PubMed PMID: 29514741Sch?fer M, Collins KK, Browne LP, Ivy DD, Abman S, Friesen R, Frank B, Fonseca B, DiMaria M, Hunter KS, Truong U, von Alvensleben JC. Effect of electrical dyssynchrony on left and right ventricular mechanics in children with pulmonary arterial hypertension. J Heart Lung Transplant. 2018;37(7):870-878. PubMed PMID: 29496397Liptzin DR, Abman SH, Giesenhagen A, Ivy DD. An approach to pulmonary edema in children at high altitude. High Alt Med Biol. 2018 Mar;19(1):91-98PubMed PMID: 29470103.Torok RD, Li JS, Kannankeril PJ, Atz AM, Bishai R, Bolotin E, Breitenstein S, Chen C, Diacovo T, Feltes T, Furlong P, Hanna M, Graham EM, Hsu D, Ivy DD, Murphy D, Kammerman LA, Kearns G, Lawrence J, Lebeaut B, Li D, Male C, McCrindle B, Mugnier P, Newburger JW, Pearson GD, Peiris V, Percival L, Pina M, Portman R, Shaddy R, Stockbridge NL, Temple R, Hill KD.?Recommendations to Enhance Pediatric Cardiovascular Drug Development: Report of a Multi-Stakeholder Think Tank.?J Am Heart Assoc. 2018 Feb 10;7(4). PubMed PMID: 29440007.Di Maria MV, Mulvahill M, Jaggers J,Ivy DD, Younoszai AK. Predictive value of presuperior cavopulmonary anastomosis cardiac catheterization at increased altitude. Congenital Heart Disease. 2018; 13:311–318. Pubmed PMID: 29431283Sch?fer M, Truong U, Browne LP, Morgan GJ, Ross M, Ing R, Hunter KS, Kheyfets VO, Abman SH, Ivy DD, Wilson N. Measuring Flow Hemodynamic Indices and Oxygen Consumption in Children with Pulmonary Hypertension: A Comparison of Catheterization and Phase-Contrast MRI. Pediatr Cardiol. 2018; 39:268–274. PubMed PMID: 29043395Friesen RM, Sch?fer M, Burkett DA, Cassidy CJ, Ivy DD, Jone PN. Right Ventricular Tissue Doppler Myocardial Performance Index in Children with Pulmonary Hypertension: Relation to Invasive Hemodynamics. Pediatr Cardiol. 2018; 39:98-104. PubMed PMID: 28980052.Jone PN, Sch?fer M, Pan Z, Bremen C, Ivy DD. 3D echocardiographic evaluation of right ventricular function and strain: a prognostic study in paediatric pulmonary hypertension. Eur Heart J Cardiovasc Imaging. 2018;19(4):1026-1033. PubMed PMID: 28950335Johnson JN, Ivy DD. The 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery: Week in Review: heart failure, transplantation, and pulmonary hypertension. Cardiol Young. 2017 Dec;27(10):2018-2022. Pubmed PMID: 29286265Jone PN, Sch?fer M, Li L, Craft M, Ivy DD, Kutty S. Right Atrial Deformation in Predicting Outcomes in Pediatric Pulmonary Hypertension. Circ Cardiovasc Imaging. 2017 Dec;10(12). pii: e006250. PubMed PMID: 29233835Geva A, Gronsbell JL, Cai T, Cai T, Murphy SN, Lyons JC, Heinz MM, Natter MD, Patibandla N, Bickel J, Mullen MP, Mandl KD; Pediatric Pulmonary Hypertension Network and National Heart, Lung, and Blood Institute Pediatric Pulmonary Vascular Disease Outcomes Bioinformatics Clinical Coordinating Center Investigators. A Computable Phenotype Improves Cohort Ascertainment in a Pediatric Pulmonary Hypertension Registry. J Pediatr. 2017 Sep; 188:224-31. PubMed PMID:28625502Krishnan U, Feinstein JA, Adatia I, Austin ED, Mullen MP, Hopper RK, Hanna B, Romer L, Keller RL, Fineman J, Steinhorn R, Kinsella JP, Ivy DD, Rosenzweig EB, Raj U, Humpl T, Abman SH; Pediatric Pulmonary Hypertension Network (PPHNet). Evaluation and Management of Pulmonary Hypertension in Children with Bronchopulmonary Dysplasia. J Pediatr. 2017 Sep; 188:24-34. PubMed PMID: 28645441Kheyfets VO, Sucharov CC, Truong U, Dunning J, Hunter K,?Ivy D, Miyamoto S, Shandas R. Circulating miRNAs in Pediatric Pulmonary Hypertension Show. Promise as Biomarkers of Vascular Function. Oxidative Medicine and Cellular Longevity. 2017;2017:4957147. PubMed PMID: 28819545Parker DK, Shen S, Zheng J, Ivy DD, Crotwell DN, Hotz JC, DiBlasi RM. Inhaled Treprostinil Drug Delivery During Mechanical Ventilation and Spontaneous Breathing Using Two Different Nebulizers. Pediatr Crit Care Med. 2017; Jun;18(6): e253-e260. PubMed PMID: 28441181Sable CA, Ivy DD, Beekman RH 3rd, Clayton-Jeter HD, Jenkins KJ, MahleWT, Morrow WR, Murphy MD, Nelson RM, Rosenthal GL, Stockbridge N, Wessel DL. 2017 ACC/AAP/AHA health policy statement on opportunities and challenges in pediatric drug development: learning from sildenafil. J Am Coll Cardiol. Jul 25;70(4):495-503. PubMed PMID: 28669447Sable CA, Ivy DD, Beekman RH 3rd, Clayton-Jeter HD, Jenkins KJ, MahleWT, Morrow WR, Murphy MD, Nelson RM, Rosenthal GL, Stockbridge N, Wessel DL. 2017 ACC/AAP/AHA health policy statement on opportunities and challenges in pediatric drug development: learning from sildenafil. Circ Cardiovasc Qual Outcomes. 2017 Jul;10(7). pii: e000026. PubMed PMID: 28663437Berger RM, Gehin M, Beghetti M, Ivy D, Kusic-Pajic A, Cornelisse P, Grill S, Bonnet D; FUTURE-3 investigators. A bosentan pharmacokinetic study to investigate dosing regimens in paediatric patients with pulmonary arterial hypertension: FUTURE-3. Br J Clin Pharmacol. 2017 Aug;83(8):1734-1744. PubMed PMID: 28213957Sch?fer M, Ivy DD, Barker AJ, Kheyfets V, Shandas R, Abman SH, Hunter KS, Truong U. Characterization of CMR-derived haemodynamic data in children with pulmonary arterial hypertension. Eur Heart J Cardiovasc Imaging. 2017: Apr 1;18(4):424-431. PubMed PMID: 27444679Hauck A, Guo R, Ivy DD, Younoszai A.?Tricuspid annular plane systolic excursion is preserved in young patients with pulmonary hypertension except when associated with repaired congenital heart disease.?Eur Heart J Cardiovasc Imaging. 2017 Apr 1;18(4):459-466. PubMed PMID: 27099276.Sch?fer M, Ivy DD, Abman SH, Barker AJ, Browne LP, Fonseca B, Kheyfets V, Hunter KS, Truong U. Apparent Aortic Stiffness in Children with Pulmonary Arterial Hypertension: Existence of Vascular Interdependency? Circ Cardiovasc Imaging. 2017 Feb;10(2). pii: e005817. PubMed PMID: 28193613Zijlstra WM, Elmasry O, Pepplinkhuizen S, Ivy DD, Bonnet D, Luijendijk P, Lévy M, Gavilan JL, Torrent-Vernetta A, Mendoza A, Del Cerro MJ, Moledina S, Berger RM. Pulmonary arterial hypertension in children after neonatal arterial switch operation. Heart 2017; 103:1244–1249. PubMed PMID: 28115472Colvin KL, Ivy DD, Yeager ME. Altered Peripheral Blood Myeloid Cell Subpopulations in Children with Down Syndrome and Pulmonary Hypertension. J Pediatr Hematol Oncol. 2017;39(2):158-159. PubMed PMID: 28060127Humpl T, Berger RM, Austin ED, Fasnacht Boillat MS, Bonnet D, Ivy DD, Zuk M, Beghetti M, Schulze-Neick I. Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry. Cardiol Young. 2017 Aug;27(6):1123-1132. PubMed PMID: 27995825Patel SS, Fernie JC, Taylor AL, Ivy DD, Rausch CM. Evaluation of predictive models for six-minute walk test among children with pulmonary hypertension. Int J Cardiol. 2017 Jan 15; 227:393-398. PubMed PMID: 27838122Clopton RG, Friesen R, Partrick D, Wilson N, Jaggers J, Ivy DD, Schwartz LI, Ing RJ. The Anesthetic Challenges of Lung Biopsy-Associated Intrathoracic Hemorrhage in a Child with Suprasystemic Pulmonary Hypertension. Semin Cardiothorac Vasc Anesth. 2017 Jun;21(2):172-177. PubMed PMID: 27815350.Takatsuki S, Nakayama T, Ikehara S, Matsuura H, Ivy DD, Saji T.Pulmonary Arterial Capacitance Index Is a Strong Predictor for Adverse Outcome in Children with Idiopathic and Heritable Pulmonary Arterial Hypertension. J Pediatr. 2017; 180:75-79. PubMed PMID: 27810156Abman SH, Ivy DD, Archer SL, Wilson K; AHA/ATS Joint Guidelines for Pediatric Pulmonary Hypertension Committee. Executive Summary of the American Heart Association and American Thoracic Society Joint Guidelines for Pediatric Pulmonary Hypertension. Am J Respir Crit Care Med. 2016;194(7):898-906. PubMed PMID: 27689707. Kheyfets VO, Dunning J, Truong U, Ivy D, Hunter K, Shandas R. A Zero-Dimensional Model and Protocol for Simulating Patient Specific Pulmonary Hemodynamics from Limited Clinical Data. J Biomech Eng. 2016 Dec 1;138(12). PubMed PMID: 27684888.Burkett DA, Slorach C, Patel SS, Redington AN, Ivy DD, Mertens L, Younoszai AK, Friedberg MK. Impact of Pulmonary Hemodynamics and Ventricular Interdependence on Left Ventricular Diastolic Function in Children with Pulmonary Hypertension.?Circ Cardiovasc Imaging. 2016;9(9). PubMed PMID: 27581953Ivy D. Pulmonary Hypertension in Children. Cardiology Clinics. 2016;34(3):451-72. PubMed PMID: 27443141Yang J, Nies MK, Fu Z, Damico R, Korley FK, Hassoun PM, Ivy DD, Austin ED, Everett AD. Hepatoma Derived Growth Factor Predicts Disease Severity and Survival in Pulmonary Artery Hypertension. Am J Respir Crit Care Med. 2016 Nov 15;194(10):1264-1272. PubMed PMID: 27254543Zijlstra WM, Douwes JM, Ploegstra MJ, Krishnan U, Roofthooft MT, Hillege HL, Ivy DD, Rosenzweig EB, Berger RM. Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease. Pulmonary Circulation. 2016;6(3):302-312. PubMed PMID: 27683607Ploegstra MJ, Ivy DD, Wheeler JG, Brand M, Beghetti M, Rosenzweig EB, Humpl T, Iriart X, Rouzic EM, Bonnet D, Berger RM. Growth in children with pulmonary arterial hypertension: a longitudinal retrospective multiregistry study. Lancet Respir Med. 2016; 4(4):281-90. PubMed PMID: 26994897Douwes JM, Humpl T, Bonnet D, Beghetti M, Ivy DD, Berger RMF, on behalf of the TOPP Investigators. Acute Vasodilator Response in Pediatric Pulmonary Arterial Hypertension: Current Clinical Practice from the TOPP Registry. J Am Coll Cardiol. 2016; 67:1312–23. PubMed PMID: 26988953Beghetti M, Berger RM, Ivy DD, Bonnet D, Humpl T. To "Cath" or Not in Pediatric Pulmonary Hypertension? J Am Coll Cardiol. 2016 Mar 1;67(8):1010-1. PubMed PMID: 26916495Kim JS, McSweeney J, Lee J, Ivy D. Pediatric Cardiac Intensive Care Society 2014 Consensus Statement: Pharmacotherapies in Cardiac Critical Care Pulmonary Hypertension. Pediatr Crit Care Med. 2016 Mar;17(3 Suppl 1):S89-S100. PubMed PMID: 26945333Jone PN, Patel SS, Cassidy C, Ivy DD. Three-dimensional Echocardiography of Right Ventricular Function Correlates with Severity of Pediatric Pulmonary Hypertension. Congenit Heart Dis. 2016;11:562–569 PubMed PMID: 26899626.Hopper R, Abman SH, Ivy DD. Persistent Challenges in Pediatric Pulmonary Hypertension. Chest. 2016;150(1):226-236. PubMed PMID: 26836930del Cerro MJ, Moledina S, Haworth SG, Ivy DD, Al Dabbagh M, Banjar H, Diaz G, Heath-Freudenthal A, Nasser Galal A, Humpl T, Kulkarni S, Lopes A, Mocumbi AO, Puri GD, Rossouw B, Harikrishnan S, Saxena A, Udo P, Caicedo L, Tamimi O, Adatia I. Cardiac catheterization in children with pulmonary hypertensive vascular disease: consensus statement from the Pulmonary Vascular Research Institute, Pediatric and Congenital?Heart Disease Task Forces. Pulmonary Circulation. 2016;6(1): 118-125. PubMed PMID: 27076908Kinsella JP, Steinhorn RH, Krishnan US, Feinstein JA, Adatia I, Austin ED, Rosenzweig EB, Everett AD, Fineman JR, Hanna BD, Hopper RK, Humpl T, Ivy DD, Keller RL, Mullen MP, Raj JU, Wessel DL, Abman SH. Recommendations for the Use of Inhaled Nitric Oxide Therapy in Premature Newborns with Severe Pulmonary Hypertension. J Pediatr. 2016 Mar; 170:312-4. PubMed PMID: 26703869Beghetti M, Schulze-Neick I, Berger RM, Ivy DD, Bonnet D, Weintraub RG, Saji T, Yung D, Mallory GB, Geiger R, Berger JT, Barst RJ, Humpl T; TOPP investigators. Haemodynamic characterisation and heart catheterisation complications in children with pulmonary hypertension: Insights from the Global TOPP Registry (tracking outcomes and practice in paediatric pulmonary hypertension). Int J Cardiol. 2016; 203:325-330. PubMed PMID: 26583838.Berger RM, Haworth SG, Bonnet D, Dulac Y, Fraisse A, Galiè N, Ivy DD, Ja?s X, Miera O, Rosenzweig EB, Efficace M, Kusic-Pajic A, Beghetti M. FUTURE-2: Results from an open-label, long-term safety and tolerability extension study using the pediatric FormUlation of bosenTan in pUlmonary arterial hypeRtEnsion. Int J Cardiol. 2016; 202:52-8. PubMed PMID: 26386921.Friesen RF,?Twite MD,?Nichols CS,?Cardwell KA,?Pan Z,?Darst JD, Wilson N,?Fagan TE,?Miyamoto SD,?Ivy DD. Hemodynamic response to ketamine in children with pulmonary hypertension.?Paediatr Anaesth 2016; 26(1):102-8 PubMed PMID: 26564806Kheyfets VO, Dunning J, Truong U, Ivy DD, Hunter KA, Shandas R. Assessment of N-terminal prohormone B-type natriuretic peptide as a measure of vascular and ventricular function in pediatric pulmonary arterial hypertension. Pulmonary Circulation. 2015; 5(4):658-66. PubMed PMID: 26697173Abman SH,?Hansmann G,?Archer SL,?Ivy DD,?Adatia I,?Chung WK,?Hanna BD,?Rosenzweig EB, Raj JU,?Cornfield D,?Stenmark KR,?Robin Steinhorn R,?Thébaud B,?Fineman JR,?Kuehne T,?Feinstein JA,?Friedberg MK, Earing M,?Barst RJ, Keller RL,?Kinsella JP,?Mullen M,?Deterding R,?Kulik T, Mallory G,?Humpl T,?Wessel DL. Pediatric Pulmonary Hypertension: Guidelines from the American Heart Association and American Thoracic Society.?Circulation. 2015;132(21):2037-99. PubMed PMID: 26534956Di Maria MV,?Burkett DA,?Younoszai AK,?Landeck BF,?Mertens L,?Ivy DD,?Friedberg MK,?Hunter KS.?Echocardiographic Estimation of Right Ventricular Stroke Work in Children with Pulmonary Arterial Hypertension: Comparison with Invasive Measurements.?J Am Soc Echocardiogr. 2015;28(11):1350-7. PubMed PMID: 26299333Ivy DD,?Abman SH. Beyond the 6-Minute Walk Test for Assessing Pediatric Pulmonary Hypertension: Making Strides Through Combination End Points.?Chest. 2015;148(3):576-7. PubMed PMID: 26324131Truong U,?Patel SS,?Kheyfets V,?Dunning J,?Fonseca B,?Barker AJ,?Ivy DD, Shandas R,?Hunter K. Non-invasive determination by cardiovascular magnetic resonance of right ventricular-vascular coupling in children and adolescents with pulmonary hypertension.?J Cardiovasc Magn Reson. 2015;17(1):81. PubMed PMID: 26376972Burkett DA, Slorach C, Patel SS, Redington AN, Ivy DD, Mertens L, Younoszai AK, Friedberg MK. Left Ventricular Myocardial Function in Children with Pulmonary Hypertension: Relation to Right Ventricular Performance and Hemodynamics. Circ Cardiovasc Imaging. 2015 Aug;8(8). pii: e003260. PubMed PMID: 26259580Rosenkranz S, Ghofrani HA, Beghetti M, Ivy D, Frey R, Fritsch A, Weimann G, Saleh S, Apitz C. Riociguat for pulmonary arterial hypertension associated with congenital heart disease. Heart (British Cardiac Society). 2015;101(22):1792-9. PubMed PMID: 26135803Webber SA, Hsu DT, Ivy DD, Kulik TJ, Pahl E, Rosenthal DN, Morrow WR, Feinstein JA. Task Force 7: Pediatric Cardiology Fellowship Training in Pulmonary Hypertension, Advanced Heart Failure, and Transplantation. Circulation. 2015 Aug 11;132(6): e99-e106. PubMed PMID: 25769631Jone PN, Bremen C, DiMaria M, Campbell DN, Jaggers J, Younoszai A, Ivy DD. Three-dimensional echocardiography enhances diagnostic accuracy of supramitral ring. Echocardiography. 2015 Jun;32(6):1048-50. PubMed PMID: 25496227Abman SH, Ivy DD. Hospitalizations of Children with Pulmonary Hypertension: Implications for Improving Care. Pediatrics. 2015 Aug;136(2):392-3. PubMed PMID: 26148953Ross RD, Brook M, Feinstein JA, Koenig P, Lang P, Spicer R, Vincent JA, Lewis AB, Martin GR, Bartz PJ, Fischbach PS, Fulton DR, Matherne GP, Reinking B, Srivastava S, Printz B, Geva T, Shirali GS, Weinberg P, Wong PC, Armsby LB, Vincent RN, Foerster SR, Holzer RJ, Moore JW, Marshall AC, Latson L, Dubin AM, Walsh EP, Franklin W, Kanter RJ, Saul JP, Shah MJ, Van Hare GF, Feltes TF, Roth SJ, Almodovar MC, Andropoulos DB, Bohn DJ, Costello JM, Gajarski RJ, Mott AR, Stout K, Valente AM, Cook S, Gurvitz M, Saidi A, Webber SA, Hsu DT, Ivy DD, Kulik TJ, Pahl E, Rosenthal DN, Morrow R, Mahle WT, Murphy AM, Li JS, Law YM, Newburger JW, Daniels SR, Bernstein D, Marino BS.?SPCTPD/ACC/AAP/AHA Training Guidelines for Pediatric Cardiology Fellowship Programs (Revision of the 2005 Training Guidelines for Pediatric Cardiology Fellowship Programs).?Journal of the American College of Cardiology?2015;66(6):670-671. PubMed PMID: 25777637Steven A Webber, Daphne T Hsu, D Dunbar Ivy, Thomas J Kulik, Elfriede Pahl, David N Rosenthal, Robert Morrow, Jeffrey A Feinstein. Task Force 7: Pediatric Cardiology Fellowship Training in Pulmonary Hypertension, Advanced Heart Failure, and Transplantation.?Journal of the American College of Cardiology?2015;66(6):732-739.?PubMed PMID: 25777654Ivy 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. PubMed PMID: 25551339Jone P, Ivy DD. Echocardiography in pediatric pulmonary hypertension. Front Pediatr. 2014 Nov 12; 2:124. PubMed PMID: 25429362Nies MK, Ivy DD, Everett AD. The untapped potential of proteomic analysis in pediatric pulmonary hypertension. Proteomics Clin Appl. 2014 Oct 28. PubMed PMID: 25348110Nicolarsen J, Ivy DD. Progress in the diagnosis and management of pulmonary hypertension in children. Curr Opin Pediatr. 2014 Oct;26(5):527-35. PubMed PMID: 25136947Lopes 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.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.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.Ivy DD, Abman SH, Barst RJ, Berger RM, Bonnet D, et al. Pediatric Pulmonary Hypertension. Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir. 2014; 42 Suppl 1:153-64. PubMed PMID: 25697042Colvin 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: 24551834Barst 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-Na?ve Pediatric Pulmonary Arterial Hypertension. Circulation 2014;129(19):1914-1923 PubMed PMID: 24637559Ivy DD. Challenges in Endpoint Development for Pulmonary Hypertension Trials in Children. Advances in Pulmonary Hypertension. 2014;13(3):125-128.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: 24325962Barst RJ, Ivy DD, Foreman AJ, McGoon MD, Rosenzweig EB. Four- and Seven-Year Outcomes of Patients with Congenital Heart Disease-Associated Pulmonary Arterial Hypertension (from the REVEAL Registry). Am J Cardiol. 2014 Jan 1;113(1):147-55. PubMed PMID: 24176071Vorhies EE, Ivy DD. Drug Treatment of Pulmonary Hypertension in Children. Paediatr Drugs. 2014 Feb;16(1):43-65. PubMed PMID: 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: 24355636Rausch 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. Bronchus-associated 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: 23353110Takatsuki 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: 23532466Truong 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-to-pulmonary-artery shunts. N Engl J Med. 2013 Jun 20;368(25):2377-84. 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):546-52. PubMed PMID: 23453572Takatsuki 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: 23184020Adatia 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):252-66. PubMed PMID: 23662203Grieve 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: 23662200Takatsuki 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: 22511577Krishnan 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: 22917554Takatsuki 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: 22748515Yeager 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: 22653875Takatsuki 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: 22430374Takatsuki S, Calderbank M, Ivy DD. Initial experience with tadalafil in pediatric pulmonary arterial hypertension. Pediatr Cardiol. 2012 Jun;33(5):683-8. PubMed PMID: 22402804Stoica S, Carpenter E, Campbell D, Mitchell M, da Cruz E, Ivy D, Lacour-Gayet F. Morbidity of the arterial switch operation. Ann Thorac Surg. 2012 Jun;93(6):1977-83. PubMed PMID: 22365263Takatsuki S, Wagner BD, Ivy DD. B-type natriuretic peptide and amino-terminal pro-B-type natriuretic peptide in pediatric patients with pulmonary arterial hypertension. Congenit Heart Dis. 2012 May-Jun;7(3):259-67. PubMed PMID: 22325151Yeager 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: 21940769Yeager 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: 22837864Barst RJ, Channick R, Ivy D, Goldstein B. Clinical perspectives with long-term pulsed inhaled nitric oxide for the treatment of pulmonary arterial hypertension. Pulm Circ. 2012 Apr-Jun;2(2):139-47. PubMed PMID: 22837854Takatsuki 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: 22048047Ivy D. Advances in pediatric pulmonary arterial hypertension. Curr Opin Cardiol. 2012 Mar;27(2):70-81. PubMed PMID: 22274573Berger 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: 22240409Barst 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, Villafa?±e 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. 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: 21778413Yeager 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: 21700605Duncan 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: 23316102Law 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: 20565694Lammers 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: 21874157Takatsuki 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: 21448619Abman SH, Ivy DD. Recent progress in understanding pediatric pulmonary hypertension. Curr Opin Pediatr. 2011 Jun;23(3):298-304. PubMed PMID: 21572384 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: 21766047Barst 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: 21357924Olin J, Abman S, Grady R, Ivy D, Lovell M, Partrick D, Balasubramaniam V. A 7-year-old with pulmonary hypertension. BMJ Case Rep. 2011: bcr0220113843. PubMed PMID: 21897452Cerro 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: 21874158Phelps CM, Tissot C, Buckvold S, Gralla J, Ivy DD, Pietra BA, Miyamoto 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: 20963408Hunter 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: 21499523Hill 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: 20549685Ivy 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: 21029834Tissot C, Ivy DD, Beghetti M. Medical therapy for pediatric pulmonary arterial hypertension. J Pediatr. 2010 Oct;157(4):528-32. PubMed PMID: 20656296Ivy 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: 23264720Tian 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: 20410558Ivy DD. Prostacyclin in the intensive care setting. Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S41-5. PubMed PMID: 20216163Ye 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: 19942620Ivy 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: 21852894Rosenzweig 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: 21691442Beghetti M, Haworth SG, Bonnet D, Barst RJ, Acar P, Fraisse A, Ivy DD, 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: 20002090Whitworth 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: 19820411Ivy 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: 19637961Tissot 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: 19679252Miyamoto 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: 19365660Morchi 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: 21866231Goldberg 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: 19269838Coldren 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: 18855024Mourani 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: 18950791Bernus 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: 18849405Phelps CM, Mengshol S, Ivy DD. A curious isolated cystic lesion of the membranous atrioventricular septum. Cardiol Young. 2008 Dec;18(6):631-4. PubMed PMID: 18950547Malhotra 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: 18805182Malhotra 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 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 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 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 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: 18245423Mourani 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: 18206706Ivy 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 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: 18082509Yetman 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: 1747056Zhang 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 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 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 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: 16581479Resnik E, Herron J, Fu R, Ivy DD, Cornfield DN. Oxygen tension modulates the expression of pulmonary vascular BKCa channel alpha- and 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: 16423665Ihida-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 Dec;128(6 Suppl):591S. PubMed PMID: 16373852. 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.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.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.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 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 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. 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.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.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. Rashid A, Ivy D. Severe paediatric pulmonary hypertension: new management strategies. Arch Dis Child. 2005 Jan;90(1):92-8. PubMed PMID: 15613526 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. 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. 2004 Nov;128(5):693-702. PubMed PMID: 15514596. 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. 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.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. PubMed PMID: 15492299Fagan 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: 14977625Ivy 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: 15050507Ivy 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: 15205573Wright 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: 15133975Drexler 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: 15133974Das 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: 14583833Bourke 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: 12947507Ivy 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: 14516902Carpenter 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: 12919946Mitchell 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: 12754026Balasubramaniam 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: 12533438Kinsella 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: 12712057Barst 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: 12709727Balzer 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: 12354713Arce OX, Knudson OA, Ellison MC, Baselga P, Ivy DD, Degroff C, Valdes-Cruz 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: 12221407Ivy DD, Yanagisawa M, Gariepy CE, Gebb SA, Colvin KL, McMurtry IF. Exaggerated hypoxic pulmonary hypertension in endothelin B receptor-deficient rats. Am J Physiol Lung Cell Mol Physiol. 2002 Apr;282(4): L703-12. PubMed PMID: 11880295Epting 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. PubMed PMID: 11889532.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: 11844885Shandas 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: 11514378.Ivy D. Diagnosis and treatment of severe pediatric pulmonary hypertension. Cardiol Rev. 2001 Jul-Aug;9(4):227-37. PubMed PMID: 11405903.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: 11290529Rairigh RL, Parker TA, Ivy DD, Kinsella JP, Fan ID, Abman SH. Role of inducible nitric oxide synthase in the pulmonary vascular response to birth-related stimuli in the ovine fetus. Circ Res. 2001 Apr 13;88(7):721-6. PubMed PMID: 11304495Torielli 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: 11241041Ivy D, Eells P. Diagnosis and treatment of pulmonary hypertension in the adolescent. Adolesc Med. 2001 Feb;12(1):79-93. Review. PubMed PMID: 11224024Ivy DD, Parker TA, Abman SH. Prolonged endothelin B receptor blockade causes pulmonary hypertension in the ovine fetus. Am J Physiol Lung Cell Mol Physiol. 2000 Oct;279(4): L758-65. PubMed PMID: 11000137Ivy DD, le Cras TD, Parker TA, Zenge JP, Jakkula M, Markham NE, Kinsella JP, Abman SH. Developmental changes in endothelin expression and activity in the ovine fetal lung. Am J Physiol Lung Cell Mol Physiol. 2000 Apr;278(4): L785-93. PubMed PMID: 10749756Parker TA, Ivy DD, Galan HL, Grover TR, Kinsella JP, Abman SH. Estradiol improves pulmonary hemodynamics and vascular remodeling in perinatal pulmonary hypertension. Am J Physiol Lung Cell Mol Physiol. 2000 Feb;278(2): L374-81. PubMed PMID: 10666122Holcomb RG, Tyson RW, Ivy DD, Abman SH, Kinsella JP. Congenital pulmonary venous stenosis presenting as persistent pulmonary hypertension of the newborn. Pediatr Pulmonol. 1999 Oct;28(4):301-6. PubMed PMID: 10497380Ziegler 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: 9817684Ivy 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.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: 9702910Ivy 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. 1998 Jul;114(1 Suppl):65S. PubMed PMID: 9676634Ivy 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: 9676605Ivy 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. 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: 9575871Ziegler 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. 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: 9527085Rairigh 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: 9421461Ivy 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: 9077525Parker 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: 9032222Ivy 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: 9018495Fox 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: 8997326Ivy 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: 8929863Abman SH, Ivy DD, Ziegler JW, Kinsella JP. Mechanisms of abnormal vasoreactivity in persistent pulmonary hypertension of the newborn infant. J Perinatol. 1996 Mar-Apr;16(2 Pt 2 Su): S18-23. PubMed PMID: 8732544Ivy 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: 8540445Kinsella 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: 7651043Ziegler 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):H473-9. PubMed PMID: 7653611Ziegler 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: 7671544Ivy 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: 7579051McQueston 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: 7840274Kinsella 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: 7977827Kinsella 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: 7808839Ivy 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: 8059714Ivy 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: PMC294348Ivy D, Kaye J, Flitter D, Wiggins J. Variant angina in an adolescent. Pediatr Cardiol. 1994 Jan-Feb;15(1):45-7. PubMed PMID: 8115273Ivy 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: 8256717Kinsella 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: 8320602Book EditorBeghetti M, Barst RJ, Berger RM, Humpl T, Ivy DD, Schulze-Neick I (editors):? Pediatric Pulmonary Hypertension. Munich: Elsevier Urban & Fischer, 2011Da Cruz E, Ivy DD, Jaggers JJ. Pediatric Cardiology, Cardiac Surgery, and Cardiac Intensive Care. London: Springer, 2013Book chapters invited articles & reviews in non-peer reviewed journal 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, 1995Ivy DD, Abman SH, Wolfe RR. Congenital Heart Disease. In: Peacock AJ, Ed. The Pulmonary Circulation: A Handbook for Clinicians. London, UK: Chapman and Hall, 1996:449-466Abman 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, 1996Ivy DD, Shaffer EM, Schaffer MS. Heart. In: Merenstein GB, Kaplan DW,Rosenberg AA, Eds. Handbook of Pediatrics. Stamford, Connecticut: Appleton & Lange, 1997:511-547Ivy 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, 1998:329-347Ivy DD. Echocardiographic Evaluation of Pulmonary Hypertension. In: Valdes-Cruz L, Cayre R, Eds. Echocardiographic Anatomy of Congenital Heart Disease, 1999:537-547Ivy 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: 279-302Sondheimer 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, 2000Ivy DD. Diagnosis and management of severe pediatric pulmonary hypertension. Cardiology in Review 9(4): 227-237, 2001Ivy DD and Eells P. Diagnosis and treatment of pulmonary hypertension in the adolescent. Adolescent Medicine: State of the Art Reviews 12(1): 79-93, 2001Rashid A and Ivy DD. Diagnosis and Management of Severe Pediatric Pulmonary Hypertension. Current Concepts in Pediatric Critical Care 43-58, 2004Rashid A and Ivy DD. Severe Paediatric Pulmonary Hypertension: New Management Strategies. Archives of Disease in Childhood (90): 92-98, 2005Rashid A and Ivy DD. New management strategies in severe paediatric pulmonary hypertension. Cardiology Today 9(2): 100-108, 2005Kinsella 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, 2005Rashid A and Ivy DD. Pulmonary hypertension. In: Slonim AD and Pollack MM, Eds. Pediatric Critical Care Medicine, 2006:694-704Rashid A and Ivy DD. Pulmonary hypertension in children. Current Paediatrics 16: 237-247, 2006Ivy DD. Pulmonary arterial hypertension assessment in pediatric cardiology. In: Beghetti, Barst, Naeije, Rubin Eds. Pulmonary Arterial Hypertension Related to Congenital Heart Disease. Munich: Elsevier, 2006:93-111Rashid A and Ivy DD. Pulmonary hypertension in children. In: Mandel and Taichman Eds. Pulmonary Vascular Disease. Philadelphia, PA: Saunders and Elsevier, 2006:274-287Ivy DD, Feinstein JA, Humpl T, Rosenzweig E. Non-congenital heart disease associated pediatric pulmonary arterial hypertension. Progress in Pediatric Cardiology 9(4): 227-237, 2009Rosenzweig 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, 2009Mallory G, Ivy DD, Shardonovsky F, and Hanna B. Management and Controversies in Pediatric Pulmonary Hypertension. Pediatric Asthma, Allergy & Immunology 22(4): 139-150, 2009Tissot C, Ivy DD, and Beghetti M. Update on pulmonary arterial hypertension focusing on new therapies. Journal of Pediatrics 157(4): 528-532, 2010Ivy DD. Prostanoids in pediatric pulmonary hypertension. Pediatric Critical Care Medicine 9(4): 227-237, 2010Kozik 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 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 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: Springer-Verlag, 2010:331-348 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 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, 2010:483-505Ivy DD, Saji B. A new era in medical management of severe pediatric pulmonary arterial hypertension. Pediatric Cardiology and Cardiac Surgery. 26(3):206-218, 2010Hunter 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, 2010Tissot 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, 2011:131-154Ivy 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, 2011:315-328Ivy DD. Idiopathic pulmonary arterial hypertension in children. In: Peacock AJ, Ed. The Pulmonary Circulation: A Handbook for Clinicians. London, UK: Chapman and Hall, 2011:559-571Ivy DD. Pulmonary Hypertension. In: McMillan, Barrett, Boney, and Jones eds. Clinical Decision Support: Pediatrics, 2012Ivy 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, 2012Takatsuki S, Beghetti M, Ivy DD. Pulmonary hypertension in Children. In: Hoeper, Humbert, Eds. European Respiratory Monograph. 57:94–107, 2012 Takatsuki S, Ivy DD. Current Challenges in Pediatric Pulmonary Hypertension. Seminars in Respiratory and Critical Care Medicine 34:627–644, 2013Vorhies EE, Ivy DD. Drug Treatment of Pulmonary Hypertension in Children. Paediatr Drugs. 2014 Feb;16(1):43-65.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 2014Jone P, Ivy DD. Echocardiography in pediatric pulmonary hypertension. Front Pediatr. 2014;2:124.Ivy DD. Idiopathic pulmonary arterial hypertension in children. In: Peacock AJ, Ed. The Pulmonary Circulation: A Handbook for Clinicians. London, UK: Chapman and Hall, 2016Ivy 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, 2016D. Ivy, Right Heart Failure in Pediatric Pulmonary Hypertension, in: J.L. Jefferies, A.C. Chang, J.W. Rossano, R.E. Shaddy, J.A. Towbin (Eds.), Heart Failure in the Child and Young Adult: From Bench to Bedside, Academic Press, Elsevier, 2018, pp. 399–412.Scientific abstracts published or presented at scientific meetings 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, 19933. Kinsella JP, Ivy DD and Abman SH. Maturational changes in endothelium-derived nitric oxide activity in the developing pulmonary circulation. Pediatr Res 33 (4): 332A, 19934. McQueston JA, Kinsella JP, Ivy DD and Abman SH. Changes in endothelium-dependent and independent vasodilation during chronic intrauterine pulmonary hypertension in fetal lambs. Pediatr Res 33 (4): 337A, 19935. Ivy DD, Schaffer MS and Loehr JP. Prolonged prostaglandin infusion in neonates with severe Ebstein's Anomaly. World Congress of Pediatric Cardiology. Paris, France, 19936. 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, 19947. 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, 19948. 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, 19949. Ziegler JW, Ivy DD, Kinsella JP, Clarke WR and Abman SH. Dipyridamole, a cGMP phosphodiesterase inhibitor, augments inhaled nitric oxide -inducedpulmonary vasodilation in the ovine transitional circulation. Pediatr Res 35 (4): 90A, 199410. 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, 199511. 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, 199512. Ziegler JW, Ivy DD, Fox JJ, Kinsella JP, WR Clarke and Abman SH. Dipyridamole, a cGMP phosphodiesterase inhibitor, augments endothelium-independent vasodilation in the ovine fetal lung. Pediatr Res 37 (4): 38A, 199513. 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, 199514. 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, 199515. 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, 199516. Kinsella JP, Halbower AC, Ziegler JW, Fox JJ, Ivy DD, and Abman SH. Low-dose inhaled nitric oxide improves gas exchange without changing vascular permeability in experimental hyaline membrane disease. Pediatr Res 37 (4): 337A, 199517. 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, 199518. 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, 199619. 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, 199620. 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, 199621. 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, 199622. 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, 199623. 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, 199624. 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, 199625. 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 199726. 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 199727. 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, 199728. Ivy DD, Le Cras TD, Horan MP, and Abman SH. Increased lung prepro-endothelin-1 and decreased endothelin B receptor mRNA expression after chronic intrauterine pulmonary hypertension in the ovine fetus. Pediatr Res 41(4): 21A, 199729. 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, 199730. 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, 199731. 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, 199732. 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, 199733. Ivy DD, Le Cras TD, Horan MP, and Abman SH. Increased lung prepro-endothelin-1 and decreased endothelin B receptor mRNA expression after chronic intrauterine pulmonary hypertension in the ovine fetus. Chest 114(1): 65S, 199834. Ivy DD, Ziegler JW, Kinsella JP, Wiggins JW, and Abman SH. Hemodynamic effects of dipyridamole and inhaled nitric oxide in pediatric patients with pulmonary hypertension. Chest 114(1): 17S, 199835. 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, 199836. 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, 199837. Ivy DD, McMurtry IF, Yanagisawa M, Gariepy CE, Morris KG, Jakkula M, Oliver-Pickett C, and Abman SH. Exaggerated hypoxic pulmonary vasoconstrictor response to acute hypoxia in the endothelin B receptor deficient rat. Pediatr Res 45(4): 24A, 199938. 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, 199939. 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, 199940. 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, 199941. 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, 2000.41. 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, 200044. 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 calcium-sensitive K+ channel gene expression. Pediatr Res 49(4): 353A, 200147. 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, 200148. 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, 200251. 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, 200252. 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, 200253. 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, 200354. 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, 200355. 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, 200356. 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, 200357. 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, 200358. 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, 200359. 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, 200360. 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, 200361. 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, 200362. 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, S11-1263. 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, 200464. Yetman AT, Taylor A, Ivy DD. Exercise responses in children with pulmonary hypertension. J Am Coll Cardiol 43(5): 392A, 200465. 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, 200466. 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, 200467. 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, 200469. 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, 200470. 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, 200571. 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, 200572. 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, 200573. 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, 200574. 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, 200575. 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, 200576. 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, 200577. 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, 200578. Beghetti M, Ivy DD, Barst RJ. Clinical experience with Bosentan in children with pulmonary arterial hypertension. Eur J Clinical Investigation: 35(Supplement 2):58, 200579. 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, 200680. 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, 200681. 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, 200682. 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, 200683. 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, 200684. 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, 200685. 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, 200686. 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, 200687. 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, 200688. 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, 200689. 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, 200790. 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, 200791 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, 200792. 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, 200793. 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, 200794. 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, 200895. 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, 200896. 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, 200897. Berger RMF, Beghetti M, Barst RJ, Jais X, Acar P, Fraisse A, Ivy DD, Schulze-Neick 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, 200898. 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, 200899. 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, 2008101. 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, 2008102. 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, 2008103. 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, 2008104. 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, 2009105. 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 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, 2009109. 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, 2009110. 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, 2009113. 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, 2009114. 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, 2009115. 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, 2009116. 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, 2010117. 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, 2010118. 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 for pulmonary hypertension assessment in a pediatric clinic.3rd International Conference on Neonatal and Childhood Pulmonary Hypertension. Banff, Canada, 2010119. 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, 2010120. 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, 2010121. 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, 2010123. 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, 2010124. 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, 2010125. 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, 2010126. 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 Adult-Onset Pulmonary Vascular Disease Associated with Congenital Heart Disease. Pediatr Res 33 (4): E-PAS2011:2928.323127. 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 Adult-Onset Idiopathic Pulmonary Arterial Hypertension. Pediatr Res E-PAS2011:2928.324128. 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-PAS2011:2928.325129. 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-PAS2011:3645.1130. Yeager ME, Colvin KL, Belchenko DD, Nega M, Barajas C, Cripe PJ, Ivy DD, Stenmark K. Immunologically Active and Well-Vascularized Bronchus-Associated Lymphoid Tissue Is Evident in Pulmonary Hypertension. Am J Respir Crit Care Med 181: A1325, 2011131. 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, 2011132. Takatsuki S, Calderbank M, Ivy DD. Initial Experience with Tadalafil In Pediatric Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 181: A5897, 2011133. 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, 2011134. 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, 2011135. Maxey D, Ivy DD, Ogawa M, Feinstein J. Adverse and Significant Adverse Events Associated With Pulmonary Hypertension Therapy In Pediatric Patients: An FDA Post-Market Study Am J Respir Crit Care Med 181: A5891, 2011136. 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, 2011137. 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-Na?ve Children. Eur Resp Journal, 2011139. 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, 2011140. 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, 2011141. 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,2011142. 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 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, 2012144. 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, 2012145. 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, 2012146. 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, 2012147. Barst RJ, Layton GR, Konourina I, Richardson H, Beghetti, Ivy DD. STARTS-2: long-term survival with oral sildenafil monotherapy in treatment-na?ve 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, 2012149. 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; A2537,2013153. 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; A3285,2013156. 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:PW1-7,2013157. 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, 2013158. 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, 2013159. 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, 2013160. 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, 2014161. 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; A3252,2014162. Yeager ME, Colvin KL, Ivy DD, Bull TM, Stenmark KR. Reduced phagocytic capacity drives experimental pulmonary hypertension. Am J Respir Crit Care Med 189; A3355,2014163. 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; A5551,2014164. 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, 2014165. 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, 2014166. 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, 2014167. Ayei-Ntim F, Kheyfets V, Hunter K, Truong U, Ivy D, Shandas R. Statistical shape modeling of right ventricular deformation highlights key associations between biological and hemodynamic markers and RV morphology in children with pulmonary hypertension. Am J Respir Crit Care Med 191; A1044, 2015168. Zijstra W, Douwes JM, Ploegstra MJ, Krishnan U, Roofthooft M, Hillege H, Ivy D, Rosenzweig EB, Berger RMF. Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease. Am J Respir Crit Care Med 191; A4740, 2015169. Galambos C, Dishop M, Sims-Lucas S, Ivy D, Deterding R. Bronchopulmonary anastamotic pathways: possible etiology of isolated idiopathic hypoxemia in an 11-year-old girl. Am J Respir Crit Care Med 191; A4898, 2015170. Kheyfets V, Dunning J, Truong U, Ivy D, Hunter K, Shandas R. Pediatric patients with mild pulmonary hypertension might benefit from more aggressive therapy: a preliminary study. Am J Respir Crit Care Med 191; A5518, 2015171. Jone PN, Truong U, Cassidy C, Patel S, DiMaria M, Fonseca B, Younoszai A, Ivy D. Real time 3-dimenasional echocardiography correlates with cardiac magnetic resonance imaging in the assessment of right ventricular volumes and function in pediatric pulmonary hypertension. Am J Respir Crit Care Med 191; A5524, 2015172. Truong U, Dunning J, Patel S, Miller-Reed K, Kheyfets V, Fonseca B, Browne L, Shandas R, Ivy D, Hunter K. Ventricular vascular coupling by CMR in pediatric pulmonary hypertension predicts clinical outcomes. Am J Respir Crit Care Med 191; A5527, 2015173.Burkett DA, Slorach C, Patel SS, Redington AN, Ivy DD, Mertens L, Younoszai AK, Friedberg MK. Left Ventricular Diastolic Dysfunction Secondary to Adverse Ventricular Interdependence in Children with Pulmonary Hypertension. Journal of the American Society of Echocardiography 28(6), B6-7, 2015174.Cassidy C, Patel S, Ivy DD, Jone P. Evaluation of Right Ventricular Function in Pediatric Patients with Pulmonary Hypertension Using Myocardial Performance Index from Tissue Doppler Imaging. Journal of the American Society of Echocardiography 28(6), B106-107, 2015175.Kheyfets VO, Truong U, Dunning J, Hunter K, Sucharov CC, Ivy DD, Miyamoto S, and Shandas R. Circulating miRNAs as Biomarkers of Afterload, Acute Vascular Reactivity, and Ventricular-Vascular Coupling in Pediatric Idiopathic Pulmonary Arterial Hypertension: Initial Clinical Studies. Circulation. 2015;132: A14123176. Jone PN, SuHong T, Ivy DD. Comparison of Right Ventricular Functional Indices Using Two-dimensional and Three-dimensional Echocardiography to Predict Outcomes in Pediatric Pulmonary Hypertension. Circulation. 2015;132: A15413177. Coleman BA, Calderbank M, Ivy DD. Initial clinical experience with oral treprostinil (UT-15C) in pediatric pulmonary hypertension at a single center. Pulm Circ 2015;5(4):744.178. Schafer M, Hunter K, Truong U, Kheyfets V, Ivy DD, Shandas R. Aortic Wall Shear Stress and Left Ventricular Systolic Performance are Reduced in Pediatric Patients with Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 191; A4898, 2016179. Agyei-ntim F, Hunter K, Truong U, Kheyfets V, Ivy DD, Shandas R. Statistical Shape Modeling of the Interventricular Septum Reveals Key Associations Between Hemodynamic Markers and Septum Morphology in Children with Pulmonary Hypertension: Initial Analysis of 31 Patients. Am J Respir Crit Care Med 191; A4898, 2016180. Jone P, Bremen C, Mulvahill M, Ivy DD. Assessment of right ventricular function using three-dimensional echocardiography and right ventricular strain in pediatric pulmonary hypertension. Pulm Circ 2016181. Sch?fer M, Hunter KS, Barker AJ, Ivy DD, and Truong U. Changes in shear metrics correspond to vascular stiffening in pediatric pulmonary hypertension Pulm Circ 2016182. Ploegstra MJ, Ivy DD, Wheeler JG, Brand M, Beghetti M, Rosenzweig EB, Humpl T, Iriart X, Rouzic EM, Bonnet D, Berger RM. Growth in children with pulmonary arterial hypertension: a longitudinal multiregistry study. Cardiology in the Young 26 (Suppl. 1), S16, 2016183. Beghetti M, Brand M, Berger RMF, Humpl T, Wheeler J, Ivy DD, Bonnet D, on behalf of the TOPP investigators. Composite clinical worsening endpoint in Paediatric PAH - clinically meaningful and feasible for clinical research. Cardiology in the Young 26 (Suppl. 1), S17, 2016184. Zijlstra WMH, Elmasry O, Pepplinkhuizen S, Ivy DD, Bonnet D, Luijendijk P, Levy M, Gavilan JL, Torrent A, Mendoza A, del Cerro M, Moldeina S, Berger RMF. Pulmonary arterial hypertension in children with transposition of the great arteries after successful neonatal arterial switch operation. Cardiology in the Young 26 (Suppl. 1), S18, 2016185. Jone P, Sedlmeier A, Breeman C, Klas B, Cassidy C, Patel SS, Ivy DD. Vendor Independent Evaluation of Three-Dimensional Echocardiography of Right Ventricular Size and Function in Children. Cardiology in the Young 26 (Suppl. 1), S124, 2016186.Jone P-N, Bremen C, Mulvahill MJ, Ivy DD. Assessment of right ventricular function and severity of pulmonary hypertension using three-dimensional echocardiography and right ventricular strain in pediatric pulmonary hypertension. Journal of the Am Society of Echocardiography. 29(6), B18, 2016187. Jone P-N, Li L, Homer P, Mulvahill MJ, Younoaszai AK, Ivy DD, Kutty S. Right atrial and right ventricular deformation in predicting outcomes in pediatric pulmonary hypertension. Journal of the Am Society of Echocardiography. 29(6), B23, 2016188. DiMaria MV, Burkett DA, Younoszai AK, Landeck BF II, Mertens L, Ivy D, Friedberg MK, Hunter KS. Right ventricular stroke work characterizes ventricular-vascular interactions in children with pulmonary hypertension, Journal of the Am Society of Echocardiography. 29(6), B25, 2016189. Jone P-N, Li L, Mulvahill MJ, Craft M, Ivy DD, Kutty S. Right atrial and right ventricular deformation are reflections of right ventricular afterload in predicting outcomes in pediatric pulmonary hypertension. Circulation;132: A14123, 2016190. Nies MN, Yang J, Damico RL, Vaidya D, Ivy DD, Austin E, Hirsch R, Yung D, Rosenzweig E, Puuciulo M, Nichols W, Everett A. Hepatoma-Derived growth factor predicts disease severity in pediatric pulmonary arterial hypertension. Am J Respir Crit Care Med 195: A2652, 2017191. Jone PN, Bremen C, Mulvahill M, Ivy DD. Relation of Three-Dimensional Echocardiography Evaluation of Right Ventricular Volumes to Right Ventricular Strain in Pediatric Pulmonary Hypertension. Am J Respir Crit Care Med 195: A6180, 2017192. Giesenhagen A, Brinton J, Ivy DD, Meier MR, Weinman JP, Liptzin D. What the HAPE is going on: High altitude pulmonary edema at Children’s Hospital Colorado. Am J Respir Crit Care Med 195: A6877, 2017193. Jone PN, Sch?fer M, Bremen C, Ivy DD.??Relation of Right Ventricular Volumes and Strain using Three-Dimensional Echocardiography in Pediatric Pulmonary Hypertension. 11th Pulmonary Vascular Research Institute Annual World Congress on PVD in Miami, FL, January 2017.194. Friesen RM, Sch?fer M, Burkett DA, Ivy DD, Cassidy C,?Jone PN.??Right Ventricular Tissue Doppler Myocardial Performance Index Correlates with Severity of Pulmonary Hypertension in Children. American Society of Echocardiography 28th?Annual Scientific Session in Baltimore, MD. 2017;30(6): B27195. Jone PN, Sch?fer M, Bremen C, Ivy DD.??Right Atrial Area is Associated with Three-Dimensional Echocardiography Volumetric and Functional Indices in Children with Pulmonary Hypertension. American Society of Echocardiography 28th?Annual Scientific Session in Baltimore, MD. 2017;30(6): B32196. Jone PN, Sch?fer M, Bremen C, Ivy DD.??Three-Dimensional Echocardiography Right Ventricular Indices Assess Disease Severity in Pediatric Pulmonary Hypertension. 7th?World Congress of Pediatric Cardiology and Pediatric Cardiac Surgery in Barcelona, Spain, July 2017.197.Jone PN, Wilson N, Morgan G, Ivy DD, Ross M.??Three-Dimensional Echocardiographic Guidance of Right Heart Catheterization Decreases Radiation Exposure in Atrial Septal Defect Closures?–?poster presentation at 7th?World Congress of Pediatric Cardiology and Pediatric Cardiac Surgery in Barcelona, Spain, July 2017.198. Jone PN, Burkett D, Morgan G, Wilson N, Ivy DD, Ross M.??Radiation reduction in atrial septal defect closures using three-dimensional echocardiographic guidance of right heart catheterization in pediatric patients?– poster presentation at Euro Echo Imaging in Lisbon, Portugal December 2017199. Jone PN, Sch?fer M, Bremen C, Ivy DD. Right ventricular arterial coupling ratio correlates with right ventricular strain and predicts outcomes using three-dimensional echocardiography in pediatric pulmonary hypertension. Circulation. 136(Suppl_1): A13651, NOV 2017200. Kheyfets V, Dufva M, Ivy DD, Shandas R, Truong U. Decreasing LV torsion rate causes inter-ventricular mechanical de-coupling and thereby disrupts RV function in pediatric pulmonary hypertension. Circulation. 136(Suppl_1): A17447, NOV 2017201.Sch?fer M, Collins KK, Browne L, Ivy DD, Abman SH, Friesen R, Frank B, Fonseca B, DiMaria M, Hunter K, Truong U, von Alvensleben J. Children with pulmonary hypertension and LV dyssynchrony have worse left ventricular function. Circulation. 136(Suppl_1): A16973, NOV 2017202. Bush D, Galambos C, Ivy DD, Abman SH, Wolter-Warmerdam, Hickey F. Clinical Characteristics and Risk Factors for Developing Pulmonary Hypertension in Children with Down Syndrome. Pulmonary Circulation 2018; 8(3): 5203. Hopper R, Ivy D, Yung D, Mullen M, Kirkpatrick E, Hirsch R, Austin E, Fineman J, Hanna B, Doran A, Solum D, Deng CQ and Feinstein J. Pharmacokinetics of oral treprostinil in children with pulmonary arterial hypertension. Pulmonary Circulation 2018; 8(3): 7204. Griffiths M, Yang J, Ivy D, Nies M, Austin E, Pauciulo M, Nichols W and A. Everett A. IGFBP2 is associated with pediatric pulmonary hypertension and disease severity. Pulmonary Circulation 2018; 8(3): 8205. Frank B, Ivy D, Abman S and Howley L. A case of pulmonary arterial hypertension secondary to scurvy in a developmentally typical child. Pulmonary Circulation 2018; 8(3): 9.206. Shah A, Sch?fer M, Miller-Reed K, Parker D, Ivy D, Kheyfets V, S. Abman S and Truong U. Left ventricular vorticity by 4D flow MRI as a marker of left ventricular diastolic dysfunction in pediatric pulmonary arterial hypertension. Pulmonary Circulation 2018; 8(3): 10-11. 207. Ivy D, Feinstein J, Yung D, Mullen M, Kirkpatrick E, Hirsch R, Austin E, Fineman J Doran A, Solum D, Deng CQ, Hopper RK. Transition to Oral Treprostinil from Parenteral or Inhaled Treprostinil or as Add-On Therapy in Pediatric Subjects with Pulmonary Arterial Hypertension. Am J Respir Crit Care Med: D 25, 2018208. Sun H, Lawrence J, Stockbridge N, Bossart S, Taylor A, Ariagno R, Abman SH, Murphy D, Soreth J, Kleppinger C, Ivy D. Combined Physical Activity and Heart Rate Variability as Measured by Actigraphy as a Potential Assessment Endpoint in Children with Pulmonary Hypertension. Pediatric Res. May 2018. 1402.22209. Griffiths M, Yang J, Ivy D, Nies M, Austin E, Pauciulo M, Nichols W, Everett A. IGFBP2 is Associated with Pediatric Pulmonary Hypertension and Disease Severity.Pediatric Res. May 2018. 1690.7210.Frank B, Schafer M, Grenolds A, Abman SH, Darst J. Acute Vasoreactivity Testing during Cardiac Catheterization of Neonates with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension. Pediatric Res May 2018. 4460.8211.Danhaive O, Mullen M, Galambos C, Kielt M, Agrawal P, Shieh J, Bansal M, Stucin-Gantar, Haas C, Griese M, Ivy D, Abman S, Austin E. TBX4 Rare Variants Presenting with Neonatal Hypoxic Respiratory Failure and Persistent Pulmonary Hypertension of the Newborn. Pediatric Res. May 2018. 4107.79212. Shah A, Schaffer M, Browne L, Ivy DD, Kheyfets V, Truong. Visualization of 4D MRI vascular flow patterns in pediatric pulmonary arterial hypertension. Society of Cardiac MRI. Barcelona, Spain, 2018213. Cassidy C, Burkett D, Schafer M, Friedberg M, Ivy DD, Jone PN. Pulmonary artery Acceleration Time is a Poor Estimation of Severity of Pulmonary Hypertension in Pediatric Patients. Journal of the American Society of Echocardiography. 2018;31(6): B5214. Burkett D, Patel S, Mertens L, Friedberg M, Ivy DD. The Relationship Between Left Ventricular Geometry and Invasive Hemodynamics in Pediatric Pulmonary Hypertension. Journal of the American Society of Echocardiography. 2018;31(6): B13215. Naresh NK, Sch?fer M, Browne L, Ivy DD, Zimmerman D, Kheyfets V, Barker AJ, Truong U. Non-Invasive Evaluation of Inhaled Nitric Oxide on Pulmonary Blood Flow Dynamics using 4D Flow MRI in Pediatric Pulmonary Arterial Hypertension Patients. P352. Society of Cardiac MRI, Seattle, WA, 2019216. Zimmerman D, Douwes MJ, Sch?fer M, Ivy DD, Abman SH, Barker AJ, Naresh NK, Kheyfets V, Truong U. Non-Invasive Wave Intensity Analysis by Phase-Contrast MRI Correlates with Mean Pulmonary Artery Pressure Before and After Nitric Oxide Testing in Children with Pulmonary Arterial Hypertension. Society of Cardiac MRI, Seattle, WA, 2019217. Frank B, Sch?fer M, Ivy DD, Jone PN. Echocardiography-derived fractional right atrial area change outperforms catheterization-derived hemodynamics to predict risk of adverse events in pediatric pulmonary hypertension. Pulmonary Circulation 2019; 8(3): 9.218. Sch?fer M, Frank B, Ivy DD, Abman SH, Browne LP, Mitchell MB, Barker AJ, Truong U. Children with pulmonary arterial hypertension have abnormal left ventricular inflow dynamics with increased flow retention. Pulmonary Circulation 2019; 8(3): 9219.Jone PN, Duchateau N, Pan X, Ivy DD, Moceri P. Three-dimensional right ventricular strain in Pediatric Pulmonary Hypertension. American College of Cardiology, New Orleans, LA. 2019:1142-481220.Yang J, Nies M, Griffiths M, Damico R, Vaidya D, Brandl S, Pauciulio M, Austin E, Ivy DD, Nichols W, Everett A. Hepatoma-Derived Growth Factor is a Predictor of Disease Severity and Survival in Pulmonary Arterial Hypertension and Highly Associated with Portal Pulmonary Hypertension. Am J Respir Crit Care Med: A2521, 2019221.Simpson C, Damico R, Hassoun P, Yang J, Nies M, Griffiths M, Vaidya D, Brandl S, Pauciulio M, Austin E, Ivy DD, Nichols W, Everett A. Circulating serum ST2 as a biomarker of Disease Severity and Survival in Pulmonary Arterial. Am J Respir Crit Care Med: A4396, 2019222. Bush D, McMorrow D, Wagner B, Galambos C, Ivy DD, Abman S, Hickey F. Angiogenic Profile Identifies Pulmonary Hypertension in Down Syndrome. Am J Respir Crit Care Med: A4404, 2019223. Hong N, Ivy DD, Lai J, Frank B, Grenolds A, Stillwell P,Houin P. It’s Not the Heart. An Unusual Case of Medium Vessel Vasculitis and Pulmonary Hypertension. Am J Respir Crit Care Med: A4959, 2019224. Yang J, Griffiths M, Nies M, Damico R, Vaidya D, Pauciulio M, Nichols W, Ivy DD, Austin E, Everett A. Identification of Insulin-Like Growth Factor Binding Protein-2 as a Novel Biomarker of Pulmonary Arterial Hypertension Severity and Survival. Am J Respir Crit Care Med: A5048, 2019225.Jone PN, Duchateau N,?Pan Z, Ivy DD, Moceri P.?Three-Dimensional Right Ventricular Global and Regional Strain Predict Outcomes in Pediatric Pulmonary Hypertension. American Society of Echocardiography 30th?Annual Scientific Session in Portland, Oregon, JASE 2019;32(6): B24226.Cassidy C, Sch?fer M, Burkett D, Ivy DD,?Jone PN.??Comparison of Right Atrial and Ventricular Strain During Simultaneous Vasoreactivity Testing in Pediatric Pulmonary Hypertension. American Society of Echocardiography 30thAnnual Scientific Session in Portland, Oregon, JASE 2019;32(6): B28Frank B, Sch?fer M, Ivy DD,?Jone PN.??Decreased Reliance on Right Atrial Contraction for Atrial Emptying Predicts Decreased Risk of Adverse Events in Pediatric Pulmonary Hypertension. American Society of Echocardiography 30th?Annual Scientific Session in Portland, Oregon, JASE 2019;32(6): B8Frank BS, Lewis K, Khailova L, Silveira L, Mitchell MB, Morgan GJ, Twite M, Ivy DD, Di Maria MV, Klawitter J, Davidson JA. Endothelin-1 Activation Is Associated With Post-Operative Hypoxemia in Infants with Single Ventricle Heart Disease Undergoing Superior Cavo-Pulmonary Anastomosis. 4th European?Conference on Neonatal and?Paediatric Pulmonary?Vascular Disease, Groningen, NL, 2019Ivy DD, Rosenzweig EB, Elliott CG, Farber HW, Frantz RP, Gomberg-Maitland M,Mu Y, Zhao C, Kenny E, Selej M, Benza RL. Risk Assessment in Pediatric Patients with Pulmonary Arterial Hypertension (PAH): Application of the REVEAL 2.0 Risk Calculator. CHEST,?Vol. 156,?Issue 4,?A169–A171Berger RMF, Beghetti M, Bonnet D, Humpl T, Ivy DD. Pulmonary hypertension in childhood: long-term survival of newly diagnosed patients in the current treatment era. Results from the TOPP-1 registry. AEPC, 2020, abstract ID 27485Humpl T, Bonnet D, Ivy DD, Berger RMF, Beghetti M for the TOPP investigators. Classification of congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) within the TOPP registry. AEPC 2020, abstract ID 27161Apitz C, Ivy DD, Berger RMF, Humpl T, Bonnet D, Beghetti M, Schranz D, Latus H on behalf of the TOPP investigators. Impact of diastolic pulmonary arterial pressure on prognostic assessment in children with pulmonary arterial hypertension. AEPC 2020Jone PN, IvyDD. RV-PA coupling ratio using TAPSE/PAAT is not reliable measure of severity of pediatric pulmonary hypertension. PVRI, Lima Peru, 2020Safety and efficacy of combination therapy with ambrisentan and tadalafil for the treatment of pulmonary arterial hypertension in children: early experience. PVRI, Lima Peru, 2020Liptzin D, Ivy DD. Sex differences in pediatric HAPE. PVRI, Lima Peru, 2020Sun H, Ivy DD, Stockbridge N, Handler S, Bates A, Krishnan U, Yung D, Hopper R, Fineman J, Austin E, Mullen M. Utilizing Actigraphy to Measure Impact of Genetic Mutations on Heart Rate Variability in Pediatric Pulmonary Hypertension.American Thoracic Society, June, 2020Clinical Trials1. An international, multicenter, randomized, placebo-controlled trial of chronicsubcutaneous UT-15 (Uniprost) in patients with pulmonary hypertension, United Therapeutics, 1999-2000, Co-Investigator2. An international, multicenter, uncontrolled, open evaluation of chronic subcutaneous UT-15 (Uniprost) plus conventional therapy in patients with pulmonary hypertension, United Therapeutics, 1999-2002, Co-Investigator3. A double-blind, randomized, placebo-controlled study to assess the effects of R0 47-0203 (bosentan) on exercise capacity in patients with pulmonary arterial hypertension. Actelion, 2000-2001, Co-Investigator4. Prophylactic use of primacor in pediatric patients at high risk of developing low cardiac output syndrome following cardiac surgery, Sanofi-Synthelabo, 2000-2001, Co-Investigator5. A double-blind, randomized, placebo-controlled safety and efficacy studyof sitaxsentan sodium treatment in patients with pulmonary arterial hypertension, ICOS-Texas Biotechnology Corporation, 2001-2002, Co-Investigator6. 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 itsmetabolites when bosentan is given as single and multiple oral doses in children with pulmonary arterial hypertension, Actelion, 2001-2002, Co-Investigator8. Development of a hospital-based home program for the use of inhaled nitric oxide in the chronic management of severe cardiopulmonary diseases. I-NOTherapeutics, 2005-2010, Co-Investigator9. 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-Investigator10. 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, 2003-2008, Co-Investigator11. 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-Investigator12. A double-blind safety and efficacy study of sitaxsentan sodium treatment in patients with pulmonary arterial hypertension who have failed bosentan therapy, Encysive, 2003-2006, Co-Investigator13. A long-term, open-label study to evaluate the safety of sitaxsentan sodium treatment in patients with pulmonary arterial hypertension, Encysive, 2003-2006, Co-Investigator14. 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-Investigator15. 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-Investigator16. 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, 2005-2009, Co-Investigator17. 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, 2006-2009, Co-Investigator18. 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-Investigator19. 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-Investigator20. 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-Investigator21. TOPP Registry: tracking outcomes and practice in pediatric pulmonary hypertension, Co-Investigator, 2007-present22. QUERI Registry: pulmonary arterial hypertension quality enhancement research initiative, Co-Investigator, 2006-201023. Congenital heart disease cardiac catheterization data consortium, Co-Investigator24. 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-present26. A Phase 2, Randomized, Double-blind, Placebo-controlled, Multicenter, Dose-ranging Study of Cicletanine in Subjects with Pulmonary Arterial Hypertension, Co-Investigator, 2010-201227. 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, 2010-201528. 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, 2010-201329. PROSPECT Registry: Registry to Prospectively Describe Use of Epoprostenol For Injection (“RTS Epoprostenol”) in Patients with Pulmonary Arterial Hypertension, Co-Investigator, 2010-201330. 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, 2010-201331. 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, 2011-201632. 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, 2011-201633. 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, 2012-201435. 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-na?ve pediatric patients aged 7 to 17 years with pulmonary arterial hypertension, United Therapeutics, Steering Committee, 2014-201836. ???????AC-055-312. A multicenter, open-label, randomized, event-driven study to assess efficacy, safety and pharmacokinetics of macitentan versus standard of care in children with pulmonary arterial hypertension, Actelion, 2016-present37. ???????Investigation of Actigraphy, an Exercise Measurement Device, as a Novel, Well-defined, Reliable, Feasible, Easy to Use, and Non-Invasive Study Endpoint to Facilitate Pediatric Pulmonary Arterial Hypertension Trials and Drug Development, Food and Drug Administration, 2016-present38. ???????BAY 63-2521 / 15681. An open-label, individual dose titration study to evaluate safety, tolerability, and pharmacokinetics of riociguat in children from 6 to less than 18 years of age with pulmonary arterial hypertension, 2018-present39. ???????AC-065A203. A prospective, multicenter, open label, single arm, phase 2 study to investigate the safety, tolerability and pharmacokinetics of selexipag in children with pulmonary arterial hypertension, 2019-presentInvited Local / Regional Presentations1. Diagnoses and Management of Aortic StenosisEliza Fernie Pediatric/Neonatal Critical Care Symposium, Denver, CO, 19932. Moderator: Pulmonary Hypertension and Right Heart FailureThe Failing Heart. The Given Biomedical Institute, Aspen, CO, 19943. Endothelin in the Perinatal Period, Rocky Mountain Center for the Biology of Development, Breckenridge, CO, 19944. The Role of the Endothelium in Pulmonary Hypertension, Frontiers of Cardiology, Winter Park, CO, 19955. The Nitric Oxide-cGMP System in Pulmonary Hypertension, Frontiers of Cardiology, Winter Park, CO, 19966. Intravascular ultrasound in pediatric pulmonary hypertension. Team Approach to Pediatric Cardiac Disease, Vail, CO, 1997 7. 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 8. Molecular Aspects of Pulmonary HypertensionFrontiers in Cardiology, Kelsey-Seybold Foundation, Winter Park, CO, 19999. Hypoplastic Left Heart SyndromeEliza Fernie Pediatric/Neonatal Critical Care Symposium, Denver, CO, 199910. Novel Treatments for Pediatric Pulmonary HypertensionTeam Approach to Pediatric Cardiac DiseaseSponsored by University of Oregon Health Sciences University, Vail, Colorado, 200311. Update on management of severe pediatric pulmonary hypertension, 12 International Vail conference on pediatric cardiac disease, Vail, CO, March 200912. An update on medical management for severe pulmonary hypertension.13 International Vail conference on pediatric cardiac disease, Vail, CO, March 201113. High altitude pulmonary hypertension in children. High Altitude Summit. Keystone, Colorado, October 201114.A Primer on Pulmonary Hypertension. American Association of Critical Care Nurse Conference. Aurora, CO March 20, 2018Invited National Presentations1. Nitric Oxide in Primary Pulmonary Hypertension. Third Pulmonary Hypertension Association Meeting, Irving, Texas, 19982. Role of the Endothelin B Receptor in Regulation of Pulmonary Vascular ToneDepartment of Physiology, University of Wisconsin, Madison, WI, 19993. State of the Art: Management of severe pulmonary hypertension. American College of Cardiology. Anaheim, CA, 20004. Journeys in Pediatric Pulmonary Hypertension. Fourth Pulmonary Hypertension Association Meeting, Chicago, IL, 20005. Treatment of Severe Pulmonary Hypertension in Congenital Heart DiseaseDepartment of Pediatrics, University of Texas Southwestern, Dallas, TX, 20006. Advances in medical management of severe pulmonary hypertension in childrenChildren’s Hospital of Philadelphia, Philadelphia, PA, 20027. Bosentan / Tracleer in Pulmonary HypertensionFifth International Pulmonary Hypertension Association Conference, Irvine, CA, 20028. Pulmonary Hypertension and Congenital Heart DiseaseFifth International Pulmonary Hypertension Association Conference, Irvine, CA, 20029. Inhaled Nitric Oxide in Pulmonary HypertensionFifth International Pulmonary Hypertension Association Conference, Irvine, CA, 200210. Ask the Parents / Ask the Kids. Fifth International Pulmonary Hypertension Association Conference, Irvine, CA, 200211. Flolan Treatment of Children and Patients with Congenital Heart DiseaseAdvisory Panel on Flolan, Chicago, IL, 200212. Diagnosis and Novel Treatment in Pediatric Pulmonary HypertensionJackson Memorial Hospital, Miami, Florida, 200313. A New Era in the Treatment of Pediatric Pulmonary HypertensionTexas Children’s Hospital, Houston, Texas, 200314. Novel roles of the endothelin B receptor. American Thoracic SocietySpecial topics in pulmonary physiology: the next generation. Seattle, WA, 200315. Diagnosis and Management of Severe Pediatric Pulmonary HypertensionSociety of Critical Care Medicine, 33rd Critical Care Congress, Orlando, FL, 200416. Bosentan / Tracleer in Pulmonary Hypertension. Sixth International Pulmonary Hypertension Association Conference, Miami, FL, 200417. Pulmonary Hypertension and Congenital Heart Disease. Sixth International Pulmonary Hypertension Association Conference, Miami, FL, 200418. Inhaled Nitric Oxide in Pulmonary Hypertension. Sixth International Pulmonary Hypertension Association Conference, Miami, FL, 200419. Ask the Parents / Ask the Kids. Sixth International Pulmonary Hypertension Association Conference, Miami, FL, 200420. The Latest in PH Therapies for Children. Sixth International Pulmonary Hypertension Association Conference, Miami, FL, 200421. Genetics of Pulmonary Artery HypertensionAmerican Academy of Pediatrics, San Francisco, CA, 200422. Current and Emerging Therapies for Primary and Secondary Pulmonary Hypertension in Children. American College of Chest Physicians, Seattle, WA, 200423. How to Evaluate and Manage Pediatric Patients with Pulmonary HypertensionAmerican Heart Association, New Orleans, LA, 2004 24. Advances in medical management of pediatric pulmonary hypertensionSoutheastern Pediatric Cardiology Society, Nashville, TN, 200525. New pharmacology in the congenital armamentarium: Pulmonary Vascular Disease.American College of Cardiology, Atlanta, GA, 200626. Advances in medical management of pediatric pulmonary hypertensionWestern Society of Pediatric Cardiology, Newport Beach, CA, 200627. Pharmacotherapeutic options for pulmonary arterial hypertension in children. American College of Chest Physicians, Salt Lake City, UT, 200628. Advances in medical management of severe pediatric pulmonary hypertensionUniversity of Oklahoma, Oklahoma City, OK, 200729. Moderator, can we fix fixed secondary pulmonary hypertension, American College of Cardiology, Chicago, IL, March 200830. The evaluation and management of the patient with secondary pulmonary hypertension, American College of Cardiology, Chicago, IL, March 200831. A new era in the medical management of the child with severe pulmonary hypertension, Children’s Healthcare of Atlanta, April 200832. Medical treatment and lung transplantation in children with pulmonary hypertension Eighth International Pulmonary Hypertension Conference, Houston, Texas, May 200833. Pulmonary hypertension: issues in adolescents and young womenEighth International Pulmonary Hypertension Conference, Houston, Texas, May 200834. Advances in treatment of pediatric pulmonary hypertension. Nationwide Columbus Children’s Hospital, October 200835. Proteomic evaluation of pediatric pulmonary hypertension. Nationwide Columbus Children’s Hospital, October 200836. Prostanoids in the management of acute pulmonary hypertension. Pediatric Cardiac Intensive Care Society meeting, Miami, FL, December 200837. Preoperative testing: what do we make of vasodilator testing? 2nd International Neonatal and Childhood Pulmonary Vascular Disease, San Francisco, CA, March 200938. 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 201039. 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 2010.40. 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 2010.41. 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 2010.42. Developing evidence-based guidelines and protocols for the diagnosis and management of pulmonary hypertension in children: How I work up a patient with suspected pulmonary hypertension. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February 2010.43. 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 2010.44. New medications in pediatric pulmonary hypertensionNinth International Pulmonary Hypertension Conference, Garden Grove, CA, 201045. Ask the Parents / Ask the Kids Ninth International Pulmonary Hypertension Conference, Garden Grove, CA, 201046. New medications in pediatric pulmonary hypertensionJoe DiMaggio Children’s Hospital, Hollywood, FL, 201047. Cardiac catheterization is unnecessary in the evaluation of pulmonary hypertension: CON. Southeastern Pediatric Cardiology Meeting, Charlotte, NC, 201048. New medications in pediatric pulmonary hypertensionSoutheastern Pediatric Cardiology Meeting, Charlotte, NC, 201049. Pediatric baseline data in the REVEAL registryREVEAL registry yearly meeting, Boston, MA, 201050. Optimizing the pulmonary vasculature: From RDS to ARDS. Society of Pediatric Research. State of the Art Plenary Session. Denver, CO. May 201151. Discovery proteomics in pediatric pulmonary hypertension. Syzygy: Congenital Cardiology Group, California, February 201252. Humanitarian Medicine in Nicaragua. Syzygy: Congenital Cardiology Group, California, February 201253. 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 2012.54. The role of non-endothelial cells in pediatric pulmonary hypertension. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Orlando, Florida, February 2012.55. 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 2012.56. Inhaled therapy for pediatric pulmonary hypertension. 4th International Conference on Neonatal and Childhood Pulmonary Hypertension. San Francisco, California, March 201257. Diagnosis and management of portopulmonary hypertension in children. Studies of Pediatric Liver Transplantation annual meeting, Denver, Colorado, October 201258. Moderator. Hot Topics in Pediatric Pulmonary Hypertension. American Heart Association Scientific Sessions, Los Angeles, CA, November 201259. Managing acute inhaled therapies in the critical care setting. 2013 PH Professional Network Symposium. Arlington, VA, September 201360. Pharmacological management of pediatric PAH. 2013 PH Professional Network Symposium. Arlington, VA, September 201361. The FDA Sildenafil Warning for Pediatric Pulmonary Hypertension, FDA, Washington, DC, January 201462. The Registry to Evaluate Early and Long-Term PAH Disease Management: Pediatric Cohort. 7th International Conference on Neonatal and Childhood Pulmonary Hypertension. San Francisco, California, March 201463. The FDA and the sildenafil decision. Joint Council on Congenital Heart Disease. Washington, DC, September 201464. Pulmonary Hypertension: Intravenous, Subcutaneous, Inhaled, or Oral – What to Choose and Why. 10th International Pediatric Cardiac Intensive Care Society, Miami, FL, December 201465.Regulatory Challenges in Pediatric PAH: Trials and Tribulations. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Scottsdale, Arizona, February 201566.Treatment of Pediatric PAH. Update on Pediatric Cardiovascular Disease, Children’s Hospital of Philadelphia, Scottsdale, Arizona, February 201567. Combination Therapy in Pediatric PAH. 8th International Conference on Neonatal and Childhood Pulmonary Hypertension. San Francisco, California, March 201568. Treatment of pediatric pulmonary hypertension. American Thoracic Society Post Graduate Course. Denver, Colorado, May 201569. Update on Combination Therapy in Pediatric PAH. 9th International Conference on Neonatal and Childhood Pulmonary Hypertension. San Francisco, California, March 201670. Transitioning from Pediatric to Adult Care. Twelfth International Pulmonary Hypertension Conference, Dallas, Texas, 201671. Ask a Pediatrician (Infant- age 11). Twelfth International Pulmonary Hypertension Conference, Dallas, Texas, 201672. Challenges in Approval of Pulmonary Hypertension Medications in Children. Duke Clinical Research Institute- FDA Forum, Washington, DC, 201673. Recent Advances in the Management of Pediatric Pulmonary Hypertension. Fourth Pediatric Cardiac Forum. Kansas City, Missouri, 201674. The Basics of Pulmonary Hypertension. AAP Pediatric Cardiology Board Review Course, Chicago, Illinois, 201675. The Future of the Association for Pediatric Pulmonary Hypertension. Tracking Outcomes in Pediatric Pulmonary Hypertension Investigator Meeting, Chicago, Illinois, 201676. Research Review in Pediatric Pulmonary Hypertension, Actelion Pulmonary Hypertension Conference, New York, New York, 201677. Novel therapies in pediatric pulmonary hypertension. Pulmonary Vascular Research Institute International Meeting, Miami, FL, 201778. Moderator pediatric pulmonary hypertension case review. Pulmonary Vascular Research Institute International Meeting, Miami, FL, 201779. Echocardiography in Pediatric Pulmonary Hypertension. Kapiolani Medical Center for Women and Children. Honolulu, Hawai’i, August 201780.How to Read a Pediatric ECG. Kapiolani Medical Center for Women and Children. Honolulu, Hawai’i, August 201781. Innovative Medical Therapies in PH: When do we use adult therapies? When should we heed black box warning? American Heart Association. Anaheim, CA, Nov 14, 201782. Review of the World Symposium of Pulmonary Hypertension. UCSF Pediatric Pulmonary Vascular Disease Conference. April 21, 201883. Novel Pathway Targets in Pulmonary Hypertension. UCSF Pediatric Pulmonary Vascular Disease Conference. April 21, 201884. Pathobiology of Pediatric Pulmonary Hypertension. American Society of Echocardiography. Nashville, TN, June 26, 2018. 85. Pediatric PH Patients: New Guidelines and Differences from Adult Care. Pulmonary Hypertension Association, Orlando, FL, June 30, 201886. Pediatric PH Patients: New Guidelines and Differences from Adult Care. Pulmonary Hypertension Association, Moderator, Orlando, FL, June 30, 201887. PVRI 5th Annual Drug Discovery and Development Symposium for Pulmonary Hypertension, Moderator, Bethesda, MD, July 9-10, 201888. AAP Board Review: Pediatric Pulmonary Hypertension, Chicago, IL, August 17, 201889. Update on Pediatric Pulmonary Hypertension. Wayne State University. Detroit, MI. September 26, 201890. What have we learned from the Tracking Outcome in Pediatric Pulmonary Hypertension Registry? 12th International Neonatal and Childhood Pulmonary Vascular Disease, San Francisco, CA, March 201991. Co-Chair. Highlighted original research: Congenital Heart Disease and the Year in Review. 2019 American College of Cardiology, New Orleans, LA, March 201992. Echocardiographic evaluation of pulmonary hypertension in children. Arkansas Children’s Hospital, Little Rock, AR, September 201993. Pediatric Pulmonary Hypertension for the Pediatrician, PedsPlace, Arkansas Children’s Hospital, Little Rock, AR, September 2019Invited International Presentations1. Childhood Pulmonary Hypertension: Novel TherapiesUnited Kingdom Pulmonary Hypertension AssociationDerby, England, 20012. Advances in treatment of pediatric pulmonary hypertension. Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada, 20033. Advances in Treatment of Pediatric Pulmonary HypertensionCanadian Pediatric Cardiology Society, Edmonton, Alberta, Canada, 20024. 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, 20045. Management of the acutely decompensated patient with chronic pulmonary hypertension, 1st European Conference on Pediatric and Neonatal Cardiac Intensive Care, Montreux, Switzerland, May 20096. 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 20097. 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 20098. A new era in the medical management of severe pediatric pulmonary arterial hypertension. Korean Pulmonary Hypertension Association, Seoul, Korea, 20109. Inhaled prostanoids in pediatric pulmonary arterial hypertension. Korean Pulmonary Hypertension Association, Seoul, Korea, 201010. A new era in the medical management of severe pediatric pulmonary arterial hypertension. Seoul National University Hospital, Seoul, Korea, 201011. Cardiac catheterization is unnecessary in the evaluation of pulmonary hypertension: CON. 3rd International Conference on Neonatal and Childhood Pulmonary Hypertension. Banff, Canada, 201012. Cardiac catheterization: indications, hemodynamic evaluation and pitfalls. 3rd International Conference on Neonatal and Childhood Pulmonary Hypertension. Banff, Canada, 201013. Chronic Postoperative Pulmonary Hypertension: Pulmonary Arterial Hypertension in Congenital Heart Disease, Vienna, Austria, 201114. Future expectations in pediatric pulmonary hypertension, European Society of Cardiology, Pulmonary Hypertension: Future Expectations, Nice, France, October 201215. The pediatric sildenafil trial and the FDA decisions, European Society of Cardiology, Pulmonary Hypertension: Future Expectations, Nice, France, October 201216. Chairman, Pediatric Task Force, World Symposium of Pulmonary Hypertension, Nice, France, February 201317. Biomarkers in pediatric PAH. 1st European Conference on Neonatal and Pediatric Pulmonary Vascular Disease. Groningen, Netherlands, October 201318. Update on the World Symposium of Pulmonary Hypertension. 1st European Conference on Neonatal and Pediatric Pulmonary Vascular Disease. Groningen, Netherlands, October 201319. Development of a pediatric pulmonary hypertension program. Pulmonary Vascular Research Institute, Giessen, Germany, January 201420. The Pediatric Sildenafil Trial: Where Do We Go from Here? The Robyn Barst Memorial Lecture, Pulmonary Vascular Research Institute Annual Meeting, Guangzhou, China, January 201521. Evaluation and Treatment of Children with Severe Pulmonary Hypertension. Pediatric Cardiology Society of Columbia, Bogota, Columbia, October 201522. Echocardiography in Evaluation of Children with Pulmonary Hypertension. Pediatric Cardiology Society of Columbia, Bogota, Columbia, October 201523. Chair and Moderator, Pediatric Pulmonary Hypertension Session, World Congress of Pediatric Cardiology and Cardiac Surgery, Barcelona, Spain, July, 201724. Paediatric Pulmonary Hypertension in the Current Era. 3rd European Conference on Neonatal and?Paediatric Pulmonary?Vascular Disease. Groningen, Netherlands, October 201725. Member, Pediatric Pulmonary Hypertension Task Force, World Symposium of Pulmonary Hypertension, Nice, France, February 201826.Oral treprostinil in pediatric pulmonary hypertension. 13th Pulmonary Vascular Research Institute International Meeting, Barcelona, Spain, 201927. Actigraphy as a measure of function in pediatric pulmonary hypertension. 13th Pulmonary Vascular Research Institute International Meeting, Barcelona, Spain, 201928. Successes of the TOPP 1 registry. Association of Pediatric PH. Groningen, NL 201929. Epidemiology and Survivial of Pediatric PH in the Current Era. 4th European?Conference on Neonatal and?Paediatric Pulmonary?Vascular Disease, Groningen, NL, 201930. Diagnosis and Management of Pediatric Pulmonary Hypertension. Anzhen International Conference on Pulmonary Hypertension, Beijing, China, 2019Grand Rounds1. Diagnosis and Management of Primary Pulmonary HypertensionDepartment of Pediatrics Grand RoundsUniversity of Wisconsin, Madison, WI, 19992. The Patrick Niland Lecture in Pediatric CardiologyDepartment of Pediatrics Grand RoundsUniversity of Michigan, Ann Arbor, MI, 20003. Diagnosis and Novel Treatment in Pediatric Pulmonary HypertensionMiami Children’s HospitalDepartment of Pediatrics Grand RoundsMiami, Florida, 2003 4. Novel Treatments in Pediatric Pulmonary HypertensionJoe DiMaggio Children’s HospitalDepartment of Pediatrics Grand RoundsMiami, Florida, 20035. A New Era in the Treatment of Pediatric Pulmonary HypertensionDepartment of Pediatrics Grand RoundsChildren’s Hospital of QuebecTeleconference to Saint Justine Children’s Hospital / Montreal Children’s HospitalQuebec City, Quebec, Canada, 20036. The Casden Lecture in Pediatric CardiologyUniversity of Oregon Health Sciences UniversityDepartment of Pediatrics Grand RoundsPortland, OR, 20037. Pediatric Pulmonary Hypertension: An UpdateDepartment of Pediatrics Grand Rounds Wake Forest University School of MedicineGreensborough, NC, 20038. A New Era in the Treatment of Pediatric Pulmonary HypertensionDepartment of Pediatrics Grand RoundsUniversity of IowaIowa City, IA, 20049. The Rae Lecture in Pediatric CardiologyDepartment of Pediatrics Grand RoundsIndiana University School of Medicine Indianapolis, IN, April 200610. University of Minnesota Department of Pediatrics Grand RoundsNovel therapy in Pediatric Pulmonary HypertensionMinneapolis, MN, August 200611. The Weinstein Lecture in Pediatric CardiologyDepartment of Pediatrics Grand RoundsJohns Hopkins UniversityBaltimore, MD, October, 200612. The MacDonald Dick Lecture in Pediatric CardiologyDepartment of Pediatrics Grand RoundsAnn Arbor, MI, December 200613. Rainbow Children’s HospitalDepartment of Pediatrics Grand RoundsCleveland, OH, April 19, 200714. Shands Children’s HospitalDepartment of Pediatrics Grand RoundsGainesville, FL, September 200715. Arkansas Children’s HospitalDepartment of Pediatrics Grand RoundsLittle Rock, AR, October 200716. The Fashena Lecture in Pediatric Cardiology University of Texas SouthwesternDepartment of Pediatrics Grand RoundsMarch 5, 200817. Wayne State University Department of Pediatrics Grand RoundsApril 22, 201118. Miami Children’s HospitalDepartment of Pediatrics Grand RoundsApril 20, 201219. Cincinnati Children’s HospitalDepartment of Pediatrics Grand RoundsMay 29, 201220. Duke Children’s HospitalDepartment of Pediatrics Grand RoundsMay 1, 2013 21. LeBonheur Children's HospitalDepartment of Pediatrics Grand RoundsJames Hunt LectureNovember 20, 201322. Lucille Packard Children's HospitalDepartment of Pediatrics Grand RoundsDunlieve LectureApril 20, 201423. Omaha Children's HospitalDepartment of Pediatrics Grand RoundsOctober 17, 201424. Wayne State UniversityChildren’s Hospital of MichiganDepartment of Pediatrics Grand RoundsApril 10, 201525. Cohen Children’s HospitalLong Island Jewish Medical CenterDepartment of Pediatrics Grand RoundsRusoff Memorial LectureMay 1, 201526. Children’s Hospital of PittsburghDepartment of Pediatrics Grand RoundsPittsburgh, PAZuberbuhler LectureJune 15, 201727. Kapiolani Medical Center for Women and ChildrenDepartment of Pediatrics Grand RoundsHonolulu, HIAugust 31, 201728.Children’s Hospital of Illinois Department of Pediatrics Grand Rounds Peoria, ILKnepp LectureshipNovember 2, 201729.University of IowaDepartment of Pediatrics Grand Rounds Iowa City, IASeptember 10, 201830. University of Arkansas for Medical SciencesThe Robert Morrow Endowed LectureSouthesastern Pediatric Cardiology SocietyLittle Rock, ARSeptember 27, 2019 ................
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