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Transpulmonary generation of cell-free hemoglobin contributes to vascular dysfunction in pulmonary arterial hypertension via dysregulated clearance mechanisms

Jamie E. Meegan, Vern Eric Kerchberger, Niki L. Fortune, Joel Brennan McNeil, Julie A. Bastarache, Eric D. Austin, Lorraine B. Ware, Anna R. Hemnes, Evan L. BrittainVanderbilt University Medical CenterUnited States Pulmonary CirculationPulm Circ 2023; 13: DOI: 10.1002/pul2.12185 AbstractCirculating cell-free hemoglobin (CFH) is elevated in pulmonary arterial hypertension (PAH) and associated with poor outcomes but the […]

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Innovations in Pediatric Therapeutics Development: Principles for the Use of Bridging Biomarkers in Pediatric Extrapolation

Thomas R. Fleming, Christine E. Garnett, Laurie S. Conklin, Solange Corriol‑Rohou, Sudharshan Hariharan, Daphne Hsu, Guenther Mueller‑Velten, Yeruk Mulugeta, Ronald Portman, Mark D. Rothmann, Norman L. Stockbridge, Simon Wandel, Jialu Zhang, Lynne YaoUniversity of Washington. U.S. Food and Drug Administration. Janssen Research and Development. AstraZeneca. Children’s Hospital at Montefiore and Albert Einstein College of Medicine. Novartis Pharma AG and Novartis Pharmaceuticals.United States Therapeutic Innovation and Regulatory ScienceTher Innov Regul Sci 2023; 57: 109-120DOI: 10.1007/s43441-022-00445-6 AbstractEven with recent substantive improvements in health care in pediatric populations, considerable need remains

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Hemodynamic and Clinical Response to Liver Transplantation in Children and Young Adults POPH Patients

Shiro Baba, Eri Ogawa, Kentaro Akagi, Koichi Matsuda, Takuya Hirata, Tatsuya Okamoto, Hideaki Okajima, Junko TakitaKyoto UniversityJapan Pediatric CardiologyPediatr Cardiol 2023; DOI: 10.1007/s00246-023-03121-0 AbstractPortopulmonary hypertension is an intractable form of pulmonary hypertension. Although liver transplantation is recommended for patients who respond poorly to treatments, the mechanisms by which liver transplantation improves pulmonary hypertension remain unclear. The

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Pulmonary Vein Atresia: Feasibility of Initial Recanalization Attempts with Subsequent Follow-up

Varun Aggarwal, Gary E. Stapleton, Lindsay F. Eilers, Srinath Gowda, Manish Bansal, Athar M. Qureshi, Melissa K. Webb, Asra Khan, Henri JustinoBaylor College of Medicine and Texas Children’s HospitalUnited States Current Problems in CardiologyCurr Probl Cardiol 2023; 48: DOI: 10.1016/j.cpcardiol.2022.101463 AbstractPulmonary vein atresia (PVA) may lead to pulmonary hypertension, cardiac failure, and death. Transcatheter or surgical treatments have rarely been offered to this population because of perceived poor outcomes. We describe single center

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Acute vasoreactivity testing during right heart catheterization in chronic thromboembolic pulmonary hypertension: Results from the pulmonary vascular disease phenomics study

Robert P. Frantz, Jane A. Leopold, Paul M. Hassoun, Anna R. Hemnes, Evelyn M. Horn, Stephen C. Mathai, Franz P. Rischard, A. Brett Larive, W.h. Wilson Tang, Margaret M. Park, Nicholas S. Hill, Erika B. RosenzweigMayo Clinic. Harvard Medical School. Johns Hopkins University. Vanderbilt University Medical Center. Weill Cornell Medicine. Cleveland Clinic. Tufts Medical Center.

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Aggrecan accumulates at sites of increased pulmonary arterial pressure in idiopathic pulmonary arterial hypertension

Oscar van der Have, Timothy J. Mead, Christian Westoo, Niccolo Peruzzi, Ayse C. Mutgan, Christian Norvik, Martin Bech, Andre Struglics, Konrad Hoetzenecker, Hans Brunnstrom, Gunilla Westergren-Thorsson, Grazyna Kwapiszewska, Suneel S. Apte, Karin Tran-Lundmark Lund University and Skåne University Hospital. Cleveland Clinic. Ludwig Boltzmann Institute for Lung Vascular Research. Medical University Graz. Medical University of Vienna.

Aggrecan accumulates at sites of increased pulmonary arterial pressure in idiopathic pulmonary arterial hypertension Read More »

Metallomics in pulmonary arterial hypertension patients

Karim El-Kersh, C. Danielle Hopkins, Xiaoyong Wu, Shesh N. Raj, Matthew C. Cave, M. Ryan Smith, Young-Mi Go, Dean P. Jones, Lu Cai, Jiapeng HuangUniversity of Nebraska Medical Center. University of Louisville School of Medicine. University of Cincinnati.  Emory University.United States Pulmonary CirculationPulm Circ 2023; DOI: 10.1002/pul2.12202 AbstractPulmonary arterial hypertension (PAH) prevalence is increasing worldwide, and

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NT-proBNP and Zlog-transformed NT-proBNP values predict extubation failure in critically ill neonates with pulmonary hypertension and ventricular dysfunction

Lukas Schroeder, Manuel Kuelshammer, Ramona Dolsheid-Pommerich, Stefan Holdenrieder, Andreas Mueller, Florian KipfmuellerUniversity Children’s Hospital Bonn. University Hospital Bonn. Technical University Munich.Germany Pediatric PulmonologyPediatr Pulmonol 2023; 58: 253-261DOI: 10.1002/ppul.26193 AbstractObjectives: Critically ill neonates with a history of pulmonary hypertension (PH) or ventricular dysfunction are at risk to experience an extubation failure (EF) after liberation from mechanical ventilation (MV).

NT-proBNP and Zlog-transformed NT-proBNP values predict extubation failure in critically ill neonates with pulmonary hypertension and ventricular dysfunction Read More »

Genetic counselling and testing in pulmonary arterial hypertension: a consensus statement on behalf of the International Consortium for Genetic Studies in PAH

Christina A. Eichstaedt, Catharina Belge, Wendy K. Chung, Stefan Gräf, Ekkehard Grünig, David Montani, Rozenn Quarck, Jair A. Tenorio-Castano, Florent Soubrier, Richard C. Trembath, Nicholas W. MorrellHeidelberg University Hospital. University of Leuven. Columbia University. University of Cambridge. Université Paris-Saclay. Hôpital Marie Lannelongue. Hospital Universitario La Paz. Centro de Investigación Biomédica en Red de Enfermedades Raras.

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Comparison of living-donor lobar lung transplantation and cadaveric lung transplantation for pulmonary hypertension

Hidenao Kayawake, Satona Tanaka, Yoshito Yamada, Shiro Baba, Hideyuki Kinoshita, Kazuhiro Yamazaki, Tadashi Ikeda, Kenji Minatoya, Yojiro Yutaka, Masatsugu Hamaji, Akihiro Ohsumi, Daisuke Nakajima, Hiroshi DateKyoto University. Kobe City Medical Center General Hospital.Japan European Journal of Cardiothoracic SurgeryEur J Cardiothorac Surg 2023; DOI: 10.1093/ejcts/ezad024 AbstractObjectives: Living-donor lobar lung transplantation (LDLLT) is a life-saving procedure for critically ill

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