PPHNet Publications

Griffiths M, Yang J, Vaidya D, Nies M, Brandal S, Ivy DD, Hickey F, Wolter-Warmerdam K, Austin ED, Mullen M, Pauciulo MW, Lutz KA, Rosenzweig EB, Hirsch R, Yung D, Nichols WC, Everett AD. Biomarkers of Pulmonary Hypertension Are Altered in Children with Down Syndrome and Pulmonary Hypertension. J Pediatr. 2022 Feb;241:68-76.e3. doi: 10.1016/j.jpeds.2021.10.017. Epub 2021 Oct 21. PMID: 34687693.

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: 10.1513/AnnalsATS.202108-998OC. Epub ahead of print. PMID: 35049414. Full text online at  http://www.atsjournals.org/doi/10.1513/AnnalsATS.202108-998OC?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed

Handler, SSVarghese, NPRosenzweig, EBYung, DKrishnan, UWhalen, EBates, AAvitabile, CMJackson, EOHirsch, RFineman, JAbman, SHFor The Pediatric Pulmonary Hypertension Network (PPHNet)Building a dedicated pediatric pulmonary hypertension program: a consensus statement from the pediatric pulmonary hypertension networkPulm Circ202212:e12031. https://doi.org/10.1002/pul2.12031

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.  doi: https://doi.org/10.21693/1933-088X-21.1.12

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. Characterisation of paediatric pulmonary hypertensive vascular disease from the PPHNet Registry. Eur Respir J. 2021 Dec 31;59(1):2003337. doi: 10.1183/13993003.03337-2020. PMID: 34140292.

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. doi: 10.1093/jamia/ocz194. PMID: 31769835; PMCID: PMC7025334.

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. doi: 10.1177/2045894021994450. PMID: 33738093; PMCID: PMC7934043.

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):2045894021991446. doi: 10.1177/2045894021991446. PMID: 33623667; PMCID: PMC7879002.

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):1801965. doi: 10.1183/13993003.01965-2018. PMID: 31151956.

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: 10.1016/j.jpeds.2019.04.046. Epub 2019 Jun 5. PMID: 31176455; PMCID: PMC6776463.

Kinsella JP, Steinhorn RH, Mullen MP, et al. “The Left Ventricle in Congenital Diaphragmatic Hernia: Implications for the Management of Pulmonary Hypertension” J Pediatrics, April 2018. Full text online at http://www.jpeds.com/article/S0022-3476(18)30287-7/fulltext

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. doi: 10.1016/j.jpeds.2017.05.037. Epub 2017 Jun 16. PMID: 28625502; PMCID: PMC5572538.

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.  Full text online at http://www.jpeds.com/article/S0022-3476(17)30650-9/abstract​

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. http://www.atsjournals.org/doi/full/10.1164/rccm.201210-1928PP#.VS1ddBf7Kr8

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. http://www.ncbi.nlm.nih.gov/pubmed/26534956

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. http://www.ncbi.nlm.nih.gov/pubmed?term=%28%28Kinsella%20J[Author]%29%20AND%20recommendations%20for%20the%20use%20of%20inhaled[Title]%29%20AND%20Steinhorn%20RH[Author]

PPHNet Research

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 Publications

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