About Pediatric Pulmonary Hypertension2023-03-17T16:12:51+00:00

About Childhood Pulmonary Hypertension

The following information about pediatric pulmonary hypertension is provided for patients and families by PPHNet caregivers.

PPHNet Publications2023-12-10T17:06:30+00:00
  1. The Inception of the Pediatric Trial to Determine Difference Between Mono or Dual Therapy for Pediatric Pulmonary Hypertension

Collaco JM, Abman SH, Austin ED, Avitabile CM, Bates A, Fineman JR, Freire GA, Handler SS, Ivy DD, Krishnan US, Mullen MP, Varghese NP, Yung D, Nies MK, Everett AD, Zimmerman KO, Simmons W, Chakraborty H, Yenokyan G, Newell-Sturdivant A, Christensen E, Eyzaguirre LM, Hanley DF, Rosenzweig EB, Romer LH. Kids Mod PAH trial: A multicenter trial comparing mono- versus duo-therapy for initial treatment of pediatric pulmonary hypertension. Pulm Circ. 2023 Oct 31;13(4):e12305.


  1. Determining the Success of Using Wearable Devices in Infants and Children with Pulmonary Hypertension

Avitabile CM, Yung D, Handler S, Hopper RK, Fineman J, Freire G, Varghese N, Mullen MP, Krishnan US, Austin E, Silveira L. Measurement of Physical Activity by Actigraphy in Infants and Young Children with Pulmonary Arterial Hypertension. The Journal of Pediatrics. 2023 Nov 1;262:113639.


  1. Pulmonary Hypertension in Children with Down Syndrome

Rachel K. Hopper, Steven H. Abman, Eleni G. Elia, Catherine M. Avitabile, Delphine Yung, Mary P. Mullen, Eric D. Austin, Angela Bates, Stephanie S. Handler, Jeffrey A. Feinstein, D. Dunbar Ivy, John P. Kinsella, Kenneth D. Mandl, J. Usha Raj, Lynn A. Sleeper. Pediatric Pulmonary Hypertension Network Investigators. Pulmonary Hypertension in Children with Down Syndrome: Results from the Pediatric Pulmonary Hypertension Network Registry. J Pediatr 2023;252:131-40



  1. Hemodynamics of Pediatric Pulmonary Hypertension Patients Enrolled in the PPHNet Registry

Rosenzweig EB, Bates A, Mullen MP, Abman SH, Austin ED, Everett A, Fineman J, Feinstein J, Hopper RK, Kinsella JP, Krishnan US, Lu M, Mandl KD, Raj JU, Varghese N, Yung D, Handler SS, Sleeper LA. Pediatric Pulmonary Hypertension Network. Cardiac Catheterization and Hemodynamics in a Multicenter Cohort of Children with Pulmonary Hypertension. Ann Am Thorac Soc. 2022 Jan 20. doi.org/10.1513/AnnalsATS.202108-998OC


  1. Building a Pediatric Pulmonary Hypertension Program

Handler, SS, Varghese, NP, Rosenzweig, EB, Yung, D, Krishnan, U, Whalen, E, Bates, A, Avitabile, CM, Jackson, EO, Hirsch, R, Fineman, J, Abman, SH, For The Pediatric Pulmonary Hypertension Network (PPHNet). Building a dedicated pediatric pulmonary hypertension program: a consensus statement from the Pediatric Pulmonary Hypertension Network. Pulm Circ. 2022; 12:e12031. doi.org/10.1002/pul2.12031


  1. Mental Health in Pediatric Pulmonary Hypertension

Parker C, Whalen E, McSweeney J, Brown A, Raj JU, Mullen M. Perspectives on Mental Health Evaluation in Pediatric Pulmonary Hypertension: A Call to Action. Advances in Pulmonary Hypertension. 2022; 21(1): 12-15.



  1. The Spectrum of Pulmonary Hypertension in the PPHNet Registry

Abman SH, Mullen MP, Sleeper LA, Austin ED, Rosenzweig EB, Kinsella JP, Ivy D, Hopper RK, Raj JU, Fineman J, Keller RL, Bates A, Krishnan US, Avitabile CM, Davidson A, Natter MD, Mandl KD. Pediatric Pulmonary Hypertension Network. Characterization of paediatric pulmonary hypertensive vascular disease from the PPHNet Registry. Eur Respir J. 2021 Dec 31;59(1):2003337. https://erj.ersjournals.com/content/59/1/2003337.short


  1. Adverse Drug Events in Pediatric Pulmonary Hypertension

Geva 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 Feb 1;27(2):294-300.



  1. Maintenance and Pain Control for Subcutaneous Treprostinil Sites in Pediatric Patients

Jackson EO, Brown A, McSweeney J, Parker C. Pediatric subcutaneous treprostinil site maintenance and pain control strategies from the Pediatric Pulmonary Hypertension Network. Pulm Circ. 2021 Mar 2;11(1):2045894021994450.



  1. Variations in Therapy for Pulmonary Hypertension Secondary to Left Heart Disease in Children

Nawaytou H, Fineman JR, Moledina S, Ivy D, Abman SH, Cerro MJD. Practice patterns of pulmonary hypertension secondary to left heart disease among pediatric pulmonary hypertension providers. Pulm Circ. 2021 Feb 9;11(1)



  1. Clinical Presentation of TBX4 Gene Mutations and Deletions in Children

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 paediatric pulmonary hypertension. Eur Respir J. 2019 Aug 22;54(2)



  1. Racial and Ethnic Diversity in Pediatric Pulmonary Hypertension

Ong MS, Abman S, Austin ED, Feinstein JA, Hopper RK, Krishnan US, Mullen MP, Natter MD, Raj JU, Rosenzweig EB, Mandl KD; Pediatric Pulmonary Hypertension Network and National Heart, Lung, and Blood Institute Pediatric Pulmonary Vascular Disease Outcomes Bioinformatics Clinical Coordinating Center Investigators. Racial and Ethnic Differences in Pediatric Pulmonary Hypertension: An Analysis of the Pediatric Pulmonary Hypertension Network Registry. J Pediatr. 2019 Aug;211:63-71.e6. doi.org/10.1016/j.jpeds.2019.04.046


  1. The Left Ventricle in Congenital Diaphragmatic Hernia and Implications for Pulmonary Hypertension Management


Kinsella JP, Steinhorn RH, Mullen MP, et al. The Left Ventricle in Congenital Diaphragmatic Hernia: Implications for the Management of Pulmonary Hypertension. J Pediatrics, Vol 197, June 2018 P17-22.



  1. Comparison Between Registry Data Collection and Electronic Health Record In Pediatric Pulmonary Hypertension


Geva 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;188:224-231.e5.


  1. Pulmonary Hypertension In Bronchopulmonary Dysplasia

Krishnan U, Feinstein JA, Adatia I, et al. Evaluation and Management of Pulmonary Hypertension in Children with Bronchoplumonary Dysplasia. J Pediatrics. Volume 188, 24-34.



  1. Use of Inhaled Nitric Oxide in Premature Newborns with Pulmonary Hypertension

Kinsella JP, Steinhorn RH, Krishnan US, et al. Recommendations for the Use of Inhaled Nitric Oxide Therapy in Premature Newborns with Severe Pulmonary Hypertension. J Pediatr 2016. 170:312-314.


  1. Pediatric Pulmonary Hypertension Guidelines

Abman SH, Hansmann G, Archer SL, et al. Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society. Circulation 2015; 132(21): 2037-2099.



  1. Implications of the Warning on the Use of Sildenafil in the Treatment of Pediatric Pulmonary Hypertension

Abman SH, Kinsella JP, Rosenzweig EB, et al. Implications of the U.S. Food and Drug Administration Warning against the Use of Sildenafil for the Treatment of Pediatric Pulmonary Hypertension. American Journal of Respiratory and Critical Care Medicine, Vol. 187, No. 6 (2013), pp. 572-575.



PPHNet Research and Grants2023-08-12T17:30:51+00:00

Actigraphy Jr. A Prospective Study 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,
FDA Project number 75F40199C0090
Site Principal Investigator (Study PI: D. Ivy)

1/2 Kids MoD PAH Trial: Mono- versus Duo-Therapy for Pediatric Pulmonary Arterial Hypertension
NIH NHLB1 1-UG3 HL 151458-01A1
Site Principal Investigators (PIs: L Romer, E Berman Rosenzweig, S Abman)

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.
Principal investigator: Dunbar Ivy  https://clinicaltrials.gov/ct2/show/NCT02909608

Pediatric Pulmonary Hypertension Network (PPHNet) Biorepository Program. Sponsor: Cardiovascular Medical Research Network
Principal investigators: Eric D. Austin, M.D., Allen Everett, M.D.

Data Fusion: A Sustainable, Scalable, Open Source Registry Advancing PVD Research. National Heart, Lung, and Blood Institute
(NHLBI) Project #1U01HL121518-01.
Principal Investigators: Abman SH, Mandl KD.

NHLBI T32 HD101388-01, “Multidisciplinary Research Training in Pediatric Pulmonary Vascular Disease.  Principal Investigators: Steven Abman, Eric Austin, Jeffrey Fineman

Relevant Research and Publications2023-03-14T00:31:11+00:00

Ivy D, Abman SH, Barst RJ, et al. “Pediatric Pulmonary Hypertension.” J Am Coll Cardiol. 2013;62(25_S):. doi:10.1016/j.jacc.2013.10.028.Full text online at http://content.onlinejacc.org/article.aspx?articleID=1790598 http://www.ncbi.nlm.nih.gov/pubmed

Progress in Pediatric Cardiology Volume 27, Issues 1–2, Pages 1-66 (December 2009) Pulmonary Hypertension. http://www.sciencedirect.com/science/journal/10589813/27/1

FDA Drug Safety Communication: Proglycem (diazoxide). Reports of Pulmonary Hypertension in Infants and Newborns

The U.S. Food and Drug Administration (FDA) warning on use of sildenafil in cases of pediatric pulmonary hypertension. http://www.fda.gov/Drugs/DrugSafety/ucm317123.htm

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