PPHNet Publications2025-09-25T07:20:37+00:00

Research and Publications

PPHNet Publications2026-10-29T07:54:48+00:00
  1. Sotatercept in Pediatric Pulmonary Hypertension: Experience from the Pediatric Pulmonary Hypertension Network

Paul J. Critser, Usha S. Krishnan, Catherine M. Avitabile, Andrea L. Jones, Benjamin S. Frank, Dunbar D. Ivy, Emily P. Williams, Rachel T. Sullivan, Eric D. Austin, Dephine Yung, Stephanie S. Handler, Edward Kirkpatrick, Mary P. Mullen, Claire Parker, Megan Griffiths, Patrick D. Evers, Meredith J. O’Neil, Neelam D. Bhatt, Rachel Capote, Erika B. Rosenzweig, Nidhy P. Varghese
Chest 2026; (October)

  1. Pulmonary Hemodynamics and Long-Term Outcomes in Children with Pulmonary Hypertension-Associated Bronchopulmonary Dysplasia

Eric D. Austin, Mary P. Mullen, Catherine M. Avitabile, Usha S. Krishnan, Erika B. Rosenzweig, Roberta L. Keller, John P. Kinsella, Delphine Yung, Lea Steffes, Angela Bates, Eleni G. Elia, Lewis H. Romer, Sharon McGrath-Morrow, Meghan L. Bernier, Kenneth D. Mandl, MD, J. Usha Raj, Lynn A. Sleeper, Steven H. Abman, For the Pediatric Pulmonary Hypertension Network (PPHNet) Investigators
J Pediatr. 2026 (February)

  1. Anxiety and Depression Screening of Youth in Pediatric Pulmonary Hypertension Clinic: A Multi-Center, Cross-Sectional Study

Claire Parker, Elise Whalen, Michael A. Smith, Jasmine Becerra, Leah Stevens, Catherine M. Avitabile, Anna Brown, Michelle Cash, Emma Olson Jackson, Julia McSweeney, Kathleen Miller-Reed, Janette T. Reyes, Cathy Sheppard, Mary P. Mullen, Pulmonary Hypertension Network (PPHNet)
Pulm Circ 2025; (April)

  1. SOX17 – Associated Pulmonary Hypertension in Children: A Distinct Developmental and Clinical Syndrome

Mary P. Mullen, D. Dunbar Ivy, Nidhy P. Varghese, Abbey J. Winant, Nahir Cortes-Santiago, Sara O. Vargas, Diego Porres, Nicola Maschietto, Paul J. Critser, Russel Hirsch, Catherine M. Avitabile, Rachel K. Hopper, Benkamin S. Frank, Ryan D. Coleman, Pankaj B. Agrawal, Jill A. Madden, Amy E. Roberts, Shane L. Collins, J. Usha Raj, Eric D. Austin, Wendy K. Chung, Steven H. Abman
J Pediat 2025; (March)

  1. Actigraphy methodology in the Kids Mod PAH trial: Physical activity as a functional endpoint in pediatric clinical trials

Catherine M. Avitabile, Usha S. Krishnan, Delphine Yung, Stephanie S. Handler, Nidhy Varghese, Angela Bates, Jeff Fineman, Rachel Sullivan, Grace Friere, Eric Austin, Mary P. Mullen, Carol Pereira, Eric J. Christensen, Gayane Yenokyan, Joseph M. Collaco, Steven H. Abman, Lew Romer, D. Dunbar Ivy, Erika B. Rosenzweig
Pulm Circ 2024; (March)

  1. Actigraphy Study Endpoints to Reduce Sample Size and Facilitate Drug Development for Pediatric Pulmonary Arterial Hypertension

Catherine M. Avitabile, Delphine Yung, Stephanie Handler, Rachel K. Hopper, Jeff Fineman, Grace Freire, Nidhy Varghese, Mary Mullen, Usha S. Krishnan, Eric Austin, Lori Silveira, D. Dunbar Ivy
J Pediatr 2023; (November)

  1. Kids Mod PAH trial: A multicenter trial comparing mono- versus duo-therapy for initial treatment of pediatric pulmonary hypertension

Joseph M. Collaco, Steven H. Abman, Eric D. Austin, Catherine M. Avitabile, Angela Bates, Jeffrey R. Fineman, Grace A. Freire, Stephanie S. Handler, Dunbar D. Ivy, Usha S. Krishnan, Mary P. Mullen, Nidhy P. Varghese, Delphine Yung, Melanie K. Nies, Allen D. Everett, Kanecia O. Zimmerman, William Simmons, Hrishikesh Chakraborty, Gayane Yenokyan, Allison Newell-Sturdivant, Eric Christensen, Lindsay M. Eyzaguirre, Daniel F. Hanley, Erika B. Rosenzweig, Lewis H. Romer
Pulm Circ 2023; (October)

  1. Pulmonary Hypertension in Children with Down Syndrome: Results from the Pediatric Pulmonary Hypertension Network Registry

Rachel K. Hopper, Steven H. Abman, Eleni G. Elia, Catherine M. Avitabile, Delphine Yung, Mary P. Mullen, Eric D. Austin, Angela Bates, Stephaie S. Handler, Jeffery A Feinstein, D. Dunbar Ivy, John P. Kinsella, Kenneth D. Mandel, J. Usha Raj, Lynn A. Sleeper. Pediatric Pulmonary Hypertension Network Investigators
J Pediatr 2023; (January)

  1. Cardiac Catheterization and Hemodynamics in a Multicenter Cohort of Children with Pulmonary Hypertension

Erika B. Rosenzweig, Angela Bates, Mary P. Mullen, Steven H. Abman, Eric D. Austin, Allen Everett, Jeffrey Fineman, Jeffrey Feinstein, Rachel K. Hopper, John P. Kinsella, Usha S. Krishnan, Minmin Lu, Kenneth D, Mandl, J. Usha Raj, Nidhy Varghese, Delphene Yung, Stephanie S. Handler, Lynn A. Sleeper,
Pediatric Pulmonary Hypertension Network
Ann Am Thorac Soc 2022; (June)

  1. Perspectives on Mental Health Evaluation in Pediatric Pulmonary Hypertension: A Call to Action

Claire Parker, Elise Whalen, Julia McSweeney, Anna Brown, J. Usha Raj, Mary P. Mullen
Adv Pulm Hypertens 2022; (March)

  1. Building a dedicated pediatric pulmonary hypertension program: A consensus statement from the pediatric pulmonary hypertension network

Stephanie S. Handler, Nidhy P. Varghese, Erika B. Rosenzweig, Delphine Yung, Usha Krishnan, Elise Whalen, Angela Bates, Catherine M. Avitabile, Emma O. Jackson, Russel Hirsch, Jeffrey Fineman, Steven H. Abman, The Pediatric Pulmonary Hypertension Network (PPHNet)
Pulm Circ 2022; (January)

  1. Characterisation of paediatric pulmonary hypertensive vascular disease from the PPHNet Registry

Steven H. Abman, Mary P. Mullen, Lynn A. Sleeper, Eric D. Austin, Erika B. Rosenzweig, John P. Kinsella, D. Dunbar Ivy, Rachel K. Hopper, J. Usha Raj, Jeffrey Fineman, Roberta L. Keller, Angela Bates, Usha S. Krishnan, Catherine M. Avitabile, Alexander Davidson, Marc D. Natter, Kenneth D. Mandl
Eur Respir J. 2021; (December)

  1. Pediatric subcutaneous treprostinil site maintenance and pain control strategies from the Pediatric Pulmonary Hypertension Network

Emma O. Jackson, Anna Brown, Julia McSweeney, Claire Parker
Pulm Circ 2021; (March)

  1. Practice patterns of pulmonary hypertension secondary to left heart disease among pediatric pulmonary hypertension providers

Hythem Nawaytou, Jeffrey R. Fineman, Shahin Moledina, Dunbar Ivy, Steven H. Abman, Maria J. Del Cerro
Pulm Circ 2021; (February)

  1. Adverse drug event rates in pediatric pulmonary hypertension: a comparison of real-world data sources

Alon Geva, Steven H. Abman, Shannon F. Manzi, Dunbar D. Ivy, Mary P. Mullen, John Griffin, Chen Lin, Guergana K. Savova, Kenneth D. Mandl
J Am Med Inform Assoc 2020; (February)

  1. Acute vasoreactivity testing in pediatric idiopathic pulmonary arterial hypertension: an international survey on current practice

Lina Caicedo, Rachel Hopper, Humberto Garcia Aguilar, Dunbar Ivy, Dora Haag, Jeff Fineman, Tillman Humpl, Omar Al-Tamimi, Jeff A. Feinstein, Rolf Berger, Erika Rosenzweig, Tarek Kashour, Gabriel Fernando Diaz, Alberto Mendoza, Usha Krishnan, Prashant Bobhate, Stephanie Handler, Antonio Augusto Lopes, Manoj Kumar Rahit, Parag Barward, Carlos Labrandero de Lera, Ian Adatia, Shahin Moledina, Steven Abman, Maria Jesus del Cerro; on behalf of PVRI Pediatric & CHD Taskforce, PPHNET, and REHIPED registry
Pulm Circ 2019; (November)

  1. Phenotype characterisation of TBX4 mutation and deletion carriers with neonatal and paediatric pulmonary hypertension

Csaba Galambos, Mary P. Mullen, Joseph T. Shieh, Nicolaus Schwerk, Matthew J. Kielt, Nicola Ullmann, Renata Boldrini, Irena Stucin-Gantar, Cristina Haass, Manish Bansal, Pankaj B. Agrawal, Joyce Johnson, Donatella Peca, Cecilia Surace, Renato Cutrera, Michael W. Pauciulo, William C. Nichols, Matthias Griese, Dunbar Ivy, Steven H. Abman, Eric D. Austin, Olivier Danhaive
Eur Respir J 2019; (August)

  1. Racial and Ethnic Differences in Pediatric Pulmonary Hypertension: An Analysis of the Pediatric Pulmonary Hypertension Network Registry

Mei-Sing Ong, Steve Abman, Eric D. Austin, Erika B. Rosenzweig, Kenneth D. Mandl, for the Pediatric Pulmonary Hypertension Network and National Heart, Lung, and Blood Institute Pediatric Pulmonary Vascular Disease Outcomes Bioinformatics Clinical Coordinating Center Investigators
J Pediatr 2019; (August)

  1. The Left Ventricle in Congenital Diaphragmatic Hernia: Implications for the Management of Pulmonary Hypertension

John P. Kinsella, Robin H. Steinhorn, Mary P. Mullen, Rachel K. Hopper, Roberta L. Keller, D. Dunbar Ivy, Eric D. Austin, Usha S. Krishnan, Erika b. Rosenzweig, Jeffrey R. Fineman, Allen D. Everett, Brian D. Hanna, Tilman Humpl, J. Usha Raj, Steven H. Abman, on behalf of the Pediatric Pulmonary Hypertension Network (PPHNet)
J Pediatr 2018; (June)

  1. A Computable Phenotype Improves Cohort Ascertainment in a Pediatric Pulmonary Hypertension Registry

Alon Geva, Jessica L. Gronsbell, Tianxi Cai, Mary P. Mullen, Kenneth D. Mandl, for the Pediatric Pulmonary Hypertension Network and National Heart, Lung, and Blood Institute Pediatric Pulmonary Vascular Disease Outcomes Bioinformatics Clinical Coordinating Center Investigators
J Pediatr 2017; (September)

  1. Evaluation and Management of Pulmonary Hypertension in Children with Bronchopulmonary Dysplasia

Usha Krishnan, Jeffrey A. Feinstein, Ian Adatia, Eric D. Austin, Mary P. Mullen, Rachel K. Hopper, Brian Hanna, Lew Romer, Roberta L. Keller, Jeffrey Fineman, Robin Steinhorn, John P. Kinsella, D. Dunbar Ivy, Erika B. Rosenzweig, Usha Raj, Tilman Humpl, Steven H. Abman, for the Pediatric Pulmonary Hypertension Network (PPHNet)
J Pediatr 2017; (September)

  1. Recommendations for the Use of Inhaled Nitric Oxide Therapy in Premature Newborns with Severe Pulmonary Hypertension

John P. Kinsella, Robin H. Steinhorn, Usha S. Krishnan, Jeffrey A. Feinstein, Ian Adatia, Eric D. Austin, Erika B. Rosenzweig, Allen D. Everett, Jeffrey R. Fineman, Brian D. Hanna, MD, Rachel K. Hopper, Tilman Humpl, D. Dunbar Ivy, Roberta L. Keller, Mary P. Mullen, J. Usha Raj, David L. Wessel, Steven H. Abman
J Pediatr 2016; (March)

  1. Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society

Steven H. Abman, Georg Hansmann, Stephen L. Archer, D. Dunbar Ivy, Ian Adatia, Wendy K. Chung, Brian D. Hanna, Erika B. Rosenzweig, J. Usha Raj, David Cornfield, Kurt R. Stenmark, Robin Steinhorn, Bernard Thébaud, Jeffrey R. Fineman, Titus Kuehne, Jeffrey A. Feinstein, Mark K. Friedberg, Michael Earing, Robyn J. Barst, Roberta L. Keller, John P. Kinsella, Mary Mullen, Robin Deterding, Thomas Kulik, George Mallory, Tilman Humpl, David L. Wessel
Circulation 2015; (November)

  1. Implications of the U.S. Food and Drug Administration Warning against the Use of Sildenafil for the Treatment of Pediatric Pulmonary Hypertension

Steven H. Abman, John P. Kinsella, Erika B. Rosenzweig, Usha Krishnan, Thomas Kulik, Marry Mullen, David L. Wessel, Robin Steinhorn, Ian Adatia, Brian Hanna, Jeffrey Feinstein, Jeffrey Fineman, Usha Raj, Tilman Humpl, on behalf of the Pediatric Pulmonary Hypertension Network (PPHNet)
Am J Respir Crit Care Med 2013; (March)

PPHNet Research and Grants2026-03-16T13:26:18+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.
https://clinicaltrials.gov/ct2/show/NCT02249923?term=pediatric+pulmonary+hypertension&rank=2

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

Children are Not Small Adults: Validation of the Pediatric Pulmonary Hypertension Risk Score
Janssen Pharmaceuticals (NOPRODPUH4017)
Principal Investigators: Lynn Sleeper, ScD and Megan Griffiths, MD

The PPHNet Registry2026-03-16T13:39:49+00:00

Who funded the study?

The National Institutes of Health funded this multi-center study, which is conducted through the PPHNet network of clinicians and researchers dedicated to the improvement of care for children with pulmonary vascular disease.

What is the purpose of the registry?

The goal of this study is to create a registry of patients with pediatric pulmonary hypertension. By gathering a large amount of information over an extended period of time we hope help to patients with childhood onset pulmonary hypertension.

What is a registry?

Patient registries are a way to collect and organize information about a group of people with a specific disease or condition for the purposes of improving understanding of the disease, and developing effective treatments.

Who can participate?

Any patient who has had onset of pulmonary hypertension between birth and 18 years of age may be enrolled. Patients do not have to exhibit signs or symptoms of the disease at the time of enrollment.

Where is the study being conducted?

The study is being conducted at Fifteen hospitals across the country and Canada. See the “Centers page” to find the center nearest you. We anticipate participation of approximately 1,000 patients across all sites.

What would I have to do?

The only information that we gather will already be regularly collected at office visits. No additional visits or tests will be ordered for this research. The information will be pulled directly from the electronic medical record and will immediately be de-identified.

What are the benefits?

This study will help researchers learn more about pediatric onset pulmonary hypertension and how to best care for this growing group of patients. What we learn from this registry may improve the care pediatric pulmonary hypertension patients receive in the future.

How can I get more information about clinical studies?

The National Institutes of Health’s web site, Clinical Trials, provides general background information on clinical trials.

The National Heart, Blood, and Lung Institute, in collaboration with New England Research Institutes, has published a resource for parents, Children and Clinical Studies, at http://www.nhlbi.nih.gov/childrenandclinicalstudies/index.php

How can I get more information about the study and participation?

A summary of the PPHNet study can be found at https://clinicaltrials.gov/ct2/show/NCT02249923

Please contact the PPHNet Center nearest to you for information about eligibility and participation.

Additional Pediatric PH Publications2025-03-10T00:32:45+00:00

PHresh Knowledge website (https://phreshknowledge.org/)

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