Class 3. Pulmonary Hypertension Associated with Lung Disease

Review of the Pathologic Characteristics in Myhre Syndrome: Gain-of-Function Pathogenic Variants in SMAD4 cause a Multisystem Fibroproliferative Response

Lois J. Starr, Mark E. Lindsay, Deborah Perry, Gregory Gheewalla, Paul A. VanderLaan, Adnan Majid, Charlie Strange, George-Claudiu Costea, Adrian Lungu, Angela E. LinUniversity of Nebraska Medical Center. Harvard Medical School and MassGeneral Hospital for Children. Massachusetts General Hospital. Methodist Hospital. Tufts University School of Medicine. Beth Israel Deaconess Medical Center. Medical University of South […]

Review of the Pathologic Characteristics in Myhre Syndrome: Gain-of-Function Pathogenic Variants in SMAD4 cause a Multisystem Fibroproliferative Response Read More »

MRI evaluation of right heart functions in children with mild cystic fibrosis

Hafize Otcu Temur, Alpay Alkan, Can Yilmaz Yozgat, Erkan Cakir, Hakan Yazan, Fatma Celik Yabul, Dilek Hacer Cesme, Yilmaz YozgatBezmialem Vakif University. Istanbul Medipol University.Turkey Cardiology in the YoungCardiol Youg 2022; 13: DOI: 10.1017/S1047951122003249 AbstractBackground: This study aimed to assess the ventricular anatomy, function of the right ventricle, and the haemodynamic findings of pulmonary artery in children

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Alveolar dead space fraction is not associated with early RV systolic dysfunction in pediatric ARDS

Daniel Chilcote, Laura Mercer-Rosa, Yan Wang, Steven M. Kawut, Robert A. Berg, Nadir Yehya, Adam S. HimebauchPerelman School of Medicine at the University of Pennsylvania and Children’s Hospital of Philadelphia. United States Pediatric PulmonologyPediatr Pulmonol 2023; 58: 559-565DOI: 10.1002/ppul.26237 AbstractPrimary hypothesis: We hypothesized that higher alveolar dead space fraction (AVDSf) at pediatric acute respiratory distress syndrome (PARDS)

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Outpatient prescription of pulmonary vasodilator therapy to preterm children with bronchopulmonary dysplasia

Ida Jeremiasen, Karin Tran-Lundmark, Mikaela Dolk, Estelle NaumburgLund University and Skåne University Hospital. Umeå University.Sweden Acta PaediatricaActa Paediatr 2023; 112: 409-416DOI: 10.1111/apa.16615 Abstract Aim: The use of pulmonary vasodilator therapy in children born preterm is largely unknown. Our aim was to map prescription patterns in children with bronchopulmonary dysplasia in Sweden.Methods: This was a descriptive national registry-based study

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Pharmacokinetics of L-Citrulline in Neonates at Risk of Developing Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension

Candice D. Fike, Charul Avachat, Angela K. Birnbaum, Judy L. Aschner, Catherine M. SherwinUniversity of Utah Health. University of Minnesota. Hackensack Meridian School of Medicine. Wright State University Boonshoft School of Medicine and Dayton Children’s Hospital.United States Paediatric DrugsPaediatr Drug 2023; 25: 87-96DOI: 10.1007/s40272-022-00542-x AbstractBackground: Options to treat pulmonary hypertension (PH) in neonates with bronchopulmonary dysplasia (BPD)

Pharmacokinetics of L-Citrulline in Neonates at Risk of Developing Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension Read More »

The biventricular contribution to chronic pulmonary hypertension of the extremely premature infant

Gabriela de Carvalho Nunes, Punnanee Wutthigate, Jessica Simoneau, Adrian Dancea, Marc Beltempo, Claudia Renaud, Gabriel AltitMcGill University Health Centre and Montreal Children’s Hospital. Siriraj Hospital and Mahidol University. Canada and Thailand Journal of PerinatologyJ Perinatol 2023; 43: 174-180DOI: 10.1038/s41372-022-01497-0 AbstractObjective: Evaluate factors associated with significant pulmonary hypertension [PH] (≥2/3 systemic) and its impact on ventricular function at

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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. MandlPediatric Pulmonary Hypertension NetworkUnited States and Canada European Respiratory JournalEur Respir J 2021; DOI: 10.1183/13993003.03337-2020 ExtractBackground: There are limited data about the range of diseases, natural history, age-appropriate end-points and optimal care for children with pulmonary hypertension (PH), including the need for developing

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Association of the dysfunctional placentation endotype of prematurity with bronchopulmonary dysplasia: a systematic review, meta-analysis and meta-regression

Maria Pierro, Eduardo Villamor-Martinez, Elke van Westering- Kroon, Maria Alvarez-Fuente, Steven H Abman, Eduardo VillamorMaastricht University Medical Centre. Maurizio Bufalini Hospital. Hospital Universitario Ramón y Cajal. University of Colorado – Anschutz Medical Campus. Netherlands, Italy, Spain and United States ThoraxThorax 2022; 77: 268-275DOI: 10.1136/thoraxjnl-2020-216485 AbstractBackground: Antenatal pathological conditions are key in the pathogenesis of bronchopulmonary dysplasia (BPD).

Association of the dysfunctional placentation endotype of prematurity with bronchopulmonary dysplasia: a systematic review, meta-analysis and meta-regression Read More »

Heterogenous Disease Course and Long-Term Outcome of Children’s Interstitial Lung Disease Related to Filamin A Gene Variants

Julia Carlens, K. Taneille Johnson, Andrew Bush, Diane Renz, Ute Hehr, Florian Laenger, Claire Hogg, Martin Wetzke, Nicolaus Schwerk, Jonathan H. RaymentImperial College London and Royal Brompton Hospital. University of Regensburg. Hannover Medical School. University of British Columbia and British Columbia Children’s Hospital Research Institute.United Kingdom, Germany and Canada Annals of the American Thoracic SocietyAnn

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DDAH1 SNP rs480414 that protects against the development of pulmonary hypertension in bronchopulmonary dysplasia results in lower nitric oxide production in neonatal cord blood-derived lymphoblastoid cell lines

Avante D. Milton, Hanadi Almazrouea, Yi Jina, Gloria Zenderb, Jennifer K. TrittmannaAbigail Wexner Research Institute at Nationwide Children’s Hospital. Ohio State University College of Medicine. United States Journal of Neonatal and Perinatal MedicineJ Neonatal Perinatal Med 2022; 15: 113-121DOI: 10.3233/NPM-210710 AbstractBackground: Bronchopulmonary dysplasia (BPD) is chronic lung disease of prematurity and pulmonary hypertension (PH) is a major

DDAH1 SNP rs480414 that protects against the development of pulmonary hypertension in bronchopulmonary dysplasia results in lower nitric oxide production in neonatal cord blood-derived lymphoblastoid cell lines Read More »

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