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Defining the clinical validity of genes reported to cause pulmonary arterial hypertension

Carrie L. Welch, Micheala A. Aldred, Scrimmitha Balachandar, Dennis Dooijes, Christina A. Eichstaedt, Stefan Graf, Arjan C. Houweling, Rajiv D. Machado, Divya Pandya, Matina Prapa, Memoona Shaukat, Laura Southgate, Jair Tenorio-Castano, ClinGen PH VCEP, Wendy K. Chung, and Multiple Collaborators. International Consortium for Genetic Studies in Pulmonary Hypertension at the Pulmonary Vascular Research InstituteMultiple InstitutionsMultiple […]

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Retrospective on global pulmonary hypertension clinical trials: 1999-2021

Lu Zheng, Jun Tan, Yi Yan, Shang Wang, Ping Yuan, Cheng Wu, Yin-Tao Zhao, Hai-Bo Yang, Francesco Nappi, Adriano R. Tonelli, Lan Wang, Qing-Hua Hu, Rong JiangFirst Affiliated Hospital of Zhengzhou University. Yeda Hospital of Yantai. Shanghai Children’s Medical Center and National Children’s Medical Center. Shanghai Pulmonary Hospital and Tongji University. Naval Medical University. Centre

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Factors leading to supranormal cardiac index in pediatric pulmonary hypertension patients treated with parenteral prostanoid therapy

Kimberley G. Miles Paul J. Critser, Patrick D. Evers, Michelle Cash, Melissa Magness, Elizabeth Geers Meredith O’Neil, Zhiqian Gao, Nicholas J. Ollberding, Russel HirschCincinnati Children’s Hospital Medical Center and Cincinnati College of Medicine. Oregon Health and Sciences University. United States Pulmonary CirculationPulm Circ 2023;DOI: 10.1002/pul2.12264 AbstractParenteral prostanoid therapy (PPT) can result in supranormal cardiac index (SCI;

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Echocardiographic Assessment of Pulmonary Arterial Hypertension Following Inhaled Nitric Oxide in Infants with Severe Bronchopulmonary Dysplasia

Maria V. Fraga, Kevin C. Dysart, Jason Z. Stroller, Matthew Huber, Anysia Fedec, Laura Mercer-Rosa, Haresh KirpalaniThe Children’s Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine. Nemours Children’s Health and duPont Hospital for Children. McMaster University.United States and Canada NeonatologyNeonatology 2023; DOI: 10.1159/000531586 AbstractObjectives: Inhaled nitric oxide (iNO) is an effective pulmonary vasodilator. However,

Echocardiographic Assessment of Pulmonary Arterial Hypertension Following Inhaled Nitric Oxide in Infants with Severe Bronchopulmonary Dysplasia Read More »

Diastolic ventricular function in persistent pulmonary hypertension of the newborn

Kévin Le Duc, Thameur Rakza, Jean Benoit Baudelet, Mohamed Riadh Boukhris, Sébastien Mur, Ali Houeijeh, Laurent StormePôle Femme-Mère-Nouveau-Né, Hôpital Jeanne de Flandre, Centre Hospitalier Universitaire de Lille and University of Lille. France Frontiers in PediatricsFront Pediatr 2023;DOI: 10.3389/fped.2023.1175178 AbstractBackground: Persistent pulmonary hypertension of the newborn (PPHN) is usually considered a consequence of impaired pulmonary circulation. However, little

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Bilateral lung transplantation for pediatric pulmonary arterial hypertension: perioperative management and one-year follow-up

Thomas Jack, Julia Carlens, Franziska Diekmann, Hosan Hasan, Philippe Chouvarine, Nicolaus Schwerk, Carsten Müller, Ivonne Wieland, Igor Tudorache, Gregor Warnecke, Murat Avsar, Alexander Horke, Fabio Ius, Dmitry Bobylev, Georg HansmannHannover Medical School. European Pediatric Pulmonary Vascular Disease Network. University Hospital of Zürich. Ruprecht-Karls-University. Germany and Switzerland Frontiers in Cardiovascular MedicineFront Cardiovasc Med 2023; DOI: 10.3389/fcvm.2023.1193326 AbstractBackground: Bilateral

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Transcatheter Ductus Arteriosus Stenting for Acute Pediatric Pulmonary Arterial Hypertension is Associated with Improved Right Ventricular Echocardiography Strain

Jason S. Kerstein, Eleonore Valencia, Shane Collins, Alessandra M. Ferraro, David M. Harrild, Kimberlee Gauvreau, Ryan Callahan, Mary P. MullenBoston Children’s Hospital and Harvard Medical School. Children’s Hospital of Philadelphia.United States Pediatric CardiologyPediatr Cardiol 2023; DOI: 10.1007/s00246-023-03233-7 AbstractBackground: Interventional therapies for severe pulmonary arterial hypertension (PAH) can provide right ventricular (RV) decompression and preserve cardiac output. Transcatheter

Transcatheter Ductus Arteriosus Stenting for Acute Pediatric Pulmonary Arterial Hypertension is Associated with Improved Right Ventricular Echocardiography Strain Read More »

Clinical relevance of rapid FOXF1-targeted sequencing in patients suspected of alveolar capillary dysplasia with misalignment of pulmonary veins

Gabriëla G. Edel, Janna A. Hol, Evelien Slot, Jan H. von der Thüsen, Yolande van Bever, Rogier C.J. de Jonge, Marianne van Tienhoven, Hennie T. Bruggenwirth, Annelies de Klein, Robbert J. RottierErasmus MC Sophia Children’s Hospital. Netherlands Laboratory InvestigationLab Invest 2023; DOI: 10.1016/j.labinv.2023.100233 AbstractAlveolar capillary dysplasia with misalignment of pulmonary Veins (ACDMPV) is a lethal congenital

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Acid ceramidase gene therapy ameliorates pulmonary arterial hypertension with right heart dysfunction

Michael G. Katz, Yoav Hadas, Adam Vincek, Lina Freage‑Kahn, Nataly Shtraizent, Jeko M. Madjarov, Peter Pastuszko, Efrat Eliyahu Icahn School of Medicine at Mount Sinai. SeneX Therapeutics Inc. Atrium Health Sanger Heart and Vascular Institute. Wake Forest School of Medicine. United States Respiratory ResearchRespir Res 2023; 24DOI: 10.1186/s12931-023-02487-2 AbstractBackground: Up-regulation of ceramides in pulmonary hypertension (PH), contributing to

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Identification of ACKR4 as an immune checkpoint in pulmonary arterial hypertension

Chen-Yu Jiang, Li-Wei Wu, Yi-Wei Liu, Bei Feng, Lin-Cai Ye, Xu Huang, Yang-Yang He, Yi Shen, Yi-Fan Zhu, Xing-Liang Zhou, Dai-Ji Jiang, Hai-Kun Qi, Hao Zhang, Yi YanShanghai Children’s Medical Center. Henan University. Shanghaitech University. China Frontiers in ImmunologyFront Immunol 2023; DOI: 10.3389/fimmu.2023.1153573 AbstractObjective: Inflammation is recognized as a contributor in the development of pulmonary arterial hypertension

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