Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease Archives

Clinical characteristics and severity classification in Mixed Connective Tissue Disease: a nationwide retrospective study

Satoshi Kubo, Yurie Kanda, Hideto Kameda, Masataka Kuwana, Hidehiko Narazaki, Takao Fujii, Keishi Fujio, Yoshinao Muro, Konomi Akamatsu, Koichiro Ohmura, Takehisa Ogura, Yuichiro Shirai, Yujiro Tanabe, Ran Nakashima, Yumi Tsuchida, Shingo Nakayamada, Hisanori Hasegawa, Shintaro Hirata, Shusaku Fukaya, Takashi Kato, Hidekata Yasuoka, Yuta Yamashita, Michihito Kono, Tatsuya Atsumi, Yoshiya TanakaUniversity of Occupational and Environmental Health,

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Rare Coding Variants in FOXF1 are Associated with Pulmonary Arterial Hypertension

Jose Gomez-Arroyo, Arjan C. Houweling, Alex V. Postma, Tushar Ganjawala, Dennis Dooijes, Harm Jan Bogaard, Jurjan Aman, Rob Zwart, Joseph A. Kizmiller, Gabriëla G. Edel, Aleksey Porollo, Lilian J. Meijboom, Nidhy P. Varghese, Philip Hale, Jason Hong, Na Zhu, Carrie L. Welch, Yufeng Shen, Francis X. McCormack, Micheala A. Aldred, Matthew T. Weirauch, Robbert J.

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Portopulmonary hypertension: an integrated approach to diagnosis, risk stratification, and management

Jesús Donate, Susana del Prado, Andrés Tenes, María Jesus del Cerro, Agustín Albillos, Luis TéllezHospital Universitario Ramón y Cajal and Universidad de Alcalá. Spain Annals of HepatologyAnn Hepatol 2026; DOI: 10.1016/j.aohep.2026.102466 AbstractPortopulmonary hypertension (PoPH) is a complex and underrecognized form of pulmonary arterial hypertension arising in the setting of portal hypertension, in which outcomes are

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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,

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Echocardiographic assessment of pulmonary hypertension: a hierarchical interpretive framework for clinical decision-making and echo laboratory practice

Aditya Shrivastav, Suhas Tivaskar, Anurag Luharia, Shantanu Gomase, Ranjana LanjewarDatta Meghe Institute of Higher Education and Research. India Echo Research and PracticeEcho Res Pract 2026; 13: DOI: 10.1186/s44156-026-00138-y AbstractBackground: Echocardiography is central to the evaluation of pulmonary hypertension (PH), yet interpretation of echocardiographic findings remains challenging because non-invasive estimation of pulmonary pressures and right ventricular

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Extracellular Hemoglobin, Hypoxia, and Macrophage-Mediated Pulmonary Vascular Remodeling in Hemolytic Disease

Melissa J. Lucero, Eva Nozik, Kathryn Hassell, David C. Irwin, Paul W. Buehler, Scott K. FergusonUniversity of Colorado Anschutz Medical Campus. University of Maryland. Embry-Riddle Aeronautical University.United States International Journal of Molecular ScienceInt J Mol Sci 2026; 27: DOI: 10.3390/ijms27188170 AbstractPulmonary hypertension (PH) is a well-recognized complication of chronic hemolytic anemias such as sickle cell

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Long-Term Subcutaneous Treprostinil in Pediatric Pulmonary Arterial Hypertension at a Latin American Referral Center: A Retrospective Cohort Study

Angelo Valencia Salazar, Ernesto León Vallejo MondragónClínica Imbanaco.Columbia Pulmonary CirculationPulm Circ 2026; 16: DOI: 10.1002/pul2.70395 AbstractPediatric pulmonary arterial hypertension is a progressive vasculopathy with substantial early mortality. Subcutaneous treprostinil is an established prostacyclin-pathway therapy for higher-risk disease, but long-term pediatric data from Latin American referral centers remain limited. This retrospective cohort included all nine consecutive

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Energetic Decoding of VSMC Plasticity: Metabolic Reprogramming and Phenotypic Switching

Tingyu Yan, Qian Nie, Bo Xu, Xin Peng, Jiecan ZhuoFirst Affiliated Hospital, Hengyang Medical School and University of South China. China Cardiovascular Drugs and TherapyCardiovasc Drugs Ther 2026; DOI: 10.1007/s10557-026-07959-3 AbstractPhenotypic switching of vascular smooth muscle cells (VSMCs) constitutes a pivotal event in the initiation and progression of various vascular diseases, including vascular calcification, atherosclerosis,

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Right Ventricular Myocardial Work by Echocardiography: A Scoping Review of Methodology, Validation, and Clinical Applications

Ehsan hahverdi, Carsten Schneider, Mathias LangeKlinikum Osnabrück. Germany EchocardiographyEchocardiography 2026; 43: DOI: 10.1111/echo.70620 AbstractBackground: Echocardiographic assessment of right ventricular (RV) function remains challenging because conventional functional and deformation indices are strongly influenced by loading conditions. Right ventricular myocardial work (RVMW), derived by integrating myocardial deformation with ventricular pressure, has emerged as a potential framework for

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Beyond Heritable PAH: Pulmonary Hypertension in Genetic Syndromes

Julien Grynblat, Florence Coulet, Thomas Lacoste-Palasset, Xavier Jais, Eric Austin, Dunbar Ivy, Maria-Rosa Ghigna, Antoine Beauvais, Etienne-Marie Jutant, Athénaïs Boucly, Mathilde Meot, Sophie-Guiti Malekzadeh-Milani, Marilyn LevyUniversité Paris-Saclay and Hôpital Bicêtre. Hôpital Necker-Enfants malades and AP-HP, Université Paris Cité. Sorbonne Université, Assistance Publique-Hôpitaux de Paris and Hôpital Pitié-Salpêtrière. Vanderbilt University Medical Center and Monroe Carell Jr.

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