Class 1. Idiopathic Pulmonary Hypertension

Hemodynamic and Clinical Profiles of Pulmonary Arterial Hypertension Patients with GDF2 and BMPR2 Variants

Mei-Tzu Wang, Ken-Pen Weng, Sheng-Kai Chang, Wei-Chun Huang, Lee-Wei ChenNational Yang Ming Chiao Tung University. Kaohsiung Veterans General Hospital. Excelsior Biopharma Inc. Fooyin University. National Sun Yat-Sen University.Taiwan International Journal of Molecular SciencesInt J Mol Sci 2024; 25:DOI: 10.3390/ijms25052734 AbstractAsians have a higher carrier rate of pulmonary arterial hypertension (PAH)-related genetic variants than Caucasians do. This […]

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Combination of high-flow nasal oxygen and ketamine/dexmedetomidine sedation for diagnostic catheterization in a child with pulmonary arterial hypertension: a case report

Kaoru Tsuboi, Misuzu Asai, Toshiki Nakamura, Jun Ninagawa, Hiroshi Ono, Shugo KasuyaNational Center for Child Health and Development. Yokohama City University Hospital.Japan Journal of Anesthesia Clinical ReportsJA Clin Rep 2024; 10:DOI: 10.1186/s40981-024-00699-z AbstractPulmonary hypertension is associated with significant risk of perioperative life-threatening events. We present a case of a 12-year-old child with severe pulmonary arterial hypertension

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Long-term safety and tolerability of ambrisentan treatment for pediatric patients with pulmonary arterial hypertension: An open-label extension study

Dunbar Ivy, Maurice Beghetti, Ernesto Juaneda‑Simian, Ramiya Ravindranath, Mary Ann Lukas, Sandra Machlitt‑Northen, Nicola Scott, Jun Narita, Rolf M. F. BergerChildren’s Hospital Colorado. University Children’s Hospital HUG and University of Geneva. Hospital de Niños de la Santísma Trinidad. Glaxo-Smith-Kline. Osaka University Graduate School of Medicine. Beatrix Children’s Hospital, University Medical Center Groningen and University of

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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

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

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

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

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

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

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

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

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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