Class 1. Idiopathic Pulmonary Hypertension

Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension

Justin J. Kochanski, Jeffrey A. Feinstein, Michelle Ogawa, Victor Ritter, Rachel K. Hopper, Gregory T. AdamsonStanford University School of Medicine.United States Pulmonary CirculationPulm Circ 2024; DOI: 10.1002/pul2.12328 AbstractChildren with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown […]

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Altered Smooth Muscle Cell Histone Acetylome by the SPHK2/S1P Axis Promotes Pulmonary Hypertension

A. Dushani C. U. Ranasinghe, Maggie Holohan, Kalyn M. Borger, Deborah L. Donahue, Rafael D. Kuc, Martin Gerig, Andrew Kim, Victoria A. Ploplis, Francis J. Castellino, Margaret A. SchwarzHarper Cancer Research Institute and University of Notre Dame.United States Circulation ResearchCirc Res 2023; 133: 704-719DOI: 10.1161/CIRCRESAHA.123.322740 AbstractBackground: Epigenetic regulation of vascular remodeling in pulmonary hypertension (PH) is poorly

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Treprostinil effectiveness in higher-risk pediatric patients with Idiopathic and Heritable Pulmonary Arterial Hypertension

Yuan He, Qiangqiang Li, Chen Zhang, Bradley B. Keller, Hong GuBeijing Anzhen Hospital and Capital Medical University. Cincinnati Children’s Hospital Medical Center. China and United States Canadian Journal of CardiologyCan J Cardiol 2023; DOI: 10.1016/j.cjca.2023.11.004 AbstractIntroduction: Little is known about the effectiveness of treprostinil in higher-risk pediatric patients with various PAH genotypes. This study was designed to

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Single-Cell Imaging Maps Inflammatory Cell Subsets to Pulmonary Arterial Hypertension Vasculopathy

Selena Ferrian, Aiqin Cao, Erin McCaffrey, Toshie Saito, Noah F. Greenwald, Mark R. Nichols, Trevor Bruce, Roham T. Zamanian, Patricia Del Rosario, Marlene Rabinovitch, Michael AngeloStanford University School of Medicine. United States American Journal of Respiratory and Critical Care MedicineAm J Respir Crit Care Med 2023; DOI: 10.1164/rccm.202209-1761OC AbstractRationale: Elucidating the immune landscape within and surrounding pulmonary

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Rapidly Progressive Idiopathic Pulmonary Arterial Hypertension in a Paediatric Patient Treated with Lung Transplantation

Filip Baszkowski, Weronika Pelczar-Płachta, Nikola Pempera, Sylwia Sławek-Szmyt, Marta Kałuzna-Oleksy, Maciej Lesiak, Waldemar BobkowskiPoznan University of Medical Sciences.Poland DiagnosticsDiagnostics 2023; DOI: 10.3390/diagnostics13203185 AbstractPulmonary arterial hypertension (PAH) is a rare heterogeneous disorder in the paediatric population which is mostly associated with congenital heart disease. The management of paediatric idiopathic PAH (IPAH) is difficult due to insufficient comparative

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Case report: Beneficial long-term effect of the atrial-flow-regulator device in a pediatric patient with idiopathic pulmonary arterial hypertension and recurring syncope

Joseph Pattathu, Sebastian Michel, Anja Ingrid Tengler, Guido Mandilaras, Andre Jakob, Robert Dalla Pozza, Nikolaus A. HaasUniversity Hospital, Ludwigs-Maximilians University Munich.Germany Frontiers in Cardiovascular MedicineFront Cardiovasc Med 2023; 10DOI: 10.3389/fcvm.2023.1197985 AbstractWe report the long-term effect after successfully implanting an 8 mm Atrial-flow-regulator (AFR) device in a 7-year-old girl with idiopathic pulmonary hypertension with persistent syncope under

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Corrigendum: Case report: Selexipag in pediatric pulmonary hypertension: initiation, transition, and titration

Jenna M. Faircloth, Neelam D. Bhatt, Corey A. Chartan, Ryan D. Coleman, Natalie Villafranco, Fadel E. Ruiz, Raysa Morales-Demori, Elise Whalen, Erin Ely, Rozmeen Fombin, Nidhy P. VargheseTexas Children’s Hospital and Baylor College of Medicine.United States Frontiers in PediatricsFront Pediatr 2023; 11DOI: 10.3389/fped.2023.1275389 AbstractThis corrects the article DOI: 10.3389/fped.2023.1050508. CategoryClass I. Idiopathic Pulmonary HypertensionClass I. Heritable

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Cardiac Magnetic Resonance Derived Left Ventricular Eccentricity Index and Right Ventricular Mass Measurements Predict Outcome in Children with Pulmonary Arterial Hypertension

Meindina G. Haarman, Iris Coenraad, Quint A. J. Hagdorn, Hans L. Hillege, Tineke P. Willems, Rolf M. F. Berger, Johannes M. DouwesBeatrix Children’s Hospital and University Medical Center Groningen. Netherlands ChildrenChildren 2023; 10DOI: 10.3390/children10040756 AbstractPulmonary arterial hypertension (PAH) is associated with increased right ventricular (RV) afterload, affecting RV remodeling and RV performance, a major determinant of

Cardiac Magnetic Resonance Derived Left Ventricular Eccentricity Index and Right Ventricular Mass Measurements Predict Outcome in Children with Pulmonary Arterial Hypertension Read More »

Growing up with Idiopathic Pulmonary Arterial Hypertension: An Arduous Journey

Tanguy Dutilleux, Nesrine Farhat, Ruth Heying, Marie-Christine Seghaye, Maurice BeghettiUniversity Hospital Aachen. Centre Hospitalier Universitaire de Liège. University Hospital Leuven. Children’s University Hospital Geneva.Germany, Belgium and Switzerland Pediatric ReportsPediatr Rep 2023; 15: 301-310DOI: 10.3390/pediatric15020026 AbstractIdiopathic pulmonary arterial hypertension (IPAH) is an uncommon and severe disease. We report the case of a 7-year-old boy investigated for cardiac

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Metabolomic differences in connective tissue disease-associated versus idiopathic pulmonary arterial hypertension in the PVDOMICS cohort

Catherine E. Simpson, Anna R. Hemnes, Megan Griffiths, Gabriele Grunig, W. H. Wison Tang, Joe G. N. Garcia, John Barnard, Suzy A. Comhair, Rachel L. Damico, Stephen C. Mathai, Paul M. Hassoun, the PVDOMICS Study GroupJohns Hopkins University. Vanderbilt University. University of Texas Southwestern Medical Center. New York University Grossman School of Medicine. Cleveland Clinic.

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