Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing

Acute vasodilator testing: An opportunity to advance the precision care of pulmonary hypertension

Meghan M. Cirulis, Lynda O. Jorgensen, Ronald W. DayUniversity of Utah and Primary Children’s Hospital.United States Respiratory Medicine Case ReportsRespir Med Case Rep 2019; 28: DOI: 10.1016/j.rmcr.2019.100911 AbstractThe pulmonary arterial pressure of a child with severe pulmonary arterial hypertension immediately normalized while breathing nitric oxide during heart catheterization at 8 years of age. Her acute pulmonary […]

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Pulmonary artery acceleration time in young children is determined by heart rate and transpulmonary gradient but not by pulmonary blood flow: A simultaneous echocardiography-cardiac catheterization study

Christiana Tai, Anyir Hsieh, Anita J. Moon-Grady, Roberta L. Keller, David Teitel, Hythem M. NawaytouUniversity of California, San FranciscoUnited States EchocardiographyEchocardiography 2022; 39: 895-905DOI: 10.1111/echo.15397 AbstractIntroduction: Pulmonary artery acceleration time (PAAT) is considered useful for the non-invasive evaluation of pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR). PAAT is dependent on PAP, PVR, pulmonary artery compliance,

Pulmonary artery acceleration time in young children is determined by heart rate and transpulmonary gradient but not by pulmonary blood flow: A simultaneous echocardiography-cardiac catheterization study Read More »

Distinguishing exercise intolerance in early-stage pulmonary hypertension with invasive exercise hemodynamics: Rest VE /VCO2 and ETCO2 identify pulmonary vascular disease

Farhan Raza, Naga Dharmavaram MD, Timothy Hess, Ravi Dhingra, James Runo, Amy Chybowski, Callyn Kozitza, Supria Batra, Evelyn M. Horn, Naomi Chesler, Marlowe EldridgeUniversity of Wisconsin-Madison. Weill Cornell Medicine. University of California-Irvine.United States Clinical CardiologyClin Cardiol 2022; 45: 742-751DOI: 10.1002/clc.23831 AbstractBackground: Among subjects with exercise intolerance and suspected early-stage pulmonary hypertension (PH), early identification of pulmonary vascular

Distinguishing exercise intolerance in early-stage pulmonary hypertension with invasive exercise hemodynamics: Rest VE /VCO2 and ETCO2 identify pulmonary vascular disease Read More »

Non-Invasive Cardiac Output Determination Using Magnetic Resonance Imaging and Thermodilution in Pulmonary Hypertension

Lindsey A. Crowe, Léon Genecand, Anne-Lise Hachulla, Stéphane Noble, Maurice Beghetti, Jean-Paul Vallée, Frédéric LadorUniversity of Geneva and Geneva University Hospitals. Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique and University of Geneva and Lausanne.Switzerland Journal of Clinical MedicineJ Clin Med 2022; 11: DOI: 10.3390/jcm11102717 AbstractMagnetic resonance imaging (MRI) can be used to measure cardiac

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Parenteral Prostanoids in Pediatric Pulmonary Arterial Hypertension: Start Early, Dose High, Combine

Johannes M. Douwes, Willemijn M. H. Zijlstra, Erika B. Rosenzweig, Mark-Jan Ploegstra, Usha S. Krishnan, Meindina G. Haarman, Marcus T. R. Roofthooft, Douwe Postmus, Hans L. Hillege, D. Dunbar Ivy, Rolf M. F. BergerUniversity of Groningen, University Medical Center Groningen and Beatrix Children’s Hospital. Columbia University. University of Colorado, Children’s Hospital Colorado.Netherlands and United States

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Clinical differences between children and adults with idiopathic and heritable pulmonary arterial hypertension

Shinichi Takatsuki, Hiroto Shimokawahara, Yukira Shimizu, Reiko Kawai, Hiroyuki Matsuura, Hiromi MatsubaraToho University Omori Medical Center. National Hospital Organization Okayama Medical Center.Japan Cardiology in the YoungCardiol Young 2022; DOI: 10.1017/S1047951122003432 AbstractBackground: Although previous studies have demonstrated that paediatric pulmonary arterial hypertension remains distinct from that in adults, there are limited studies evaluating a direct comparison between children

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Vasoreactive phenotype in children with pulmonary arterial hypertension and syncope

Alexandra N. Linder, Jill Hsia, Sheila V. Krishnan, Erika B. Rosenzweig, Usha S. KrishnanColumbia University Irving Medical Center and New York Presbyterian Hospital. Children’s Hospital of Philadelphia, Philadelphia. Stonybrook University School of Medicine.United States European Respiratory Journal Open ResearchERJ Open Res 2022; 8: DOI: 10.1183/23120541.00223-2022 AbstractBackground: Syncope in Group 1 pulmonary arterial hypertension (PAH) is an independent

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Long-term prognostic value of cardiac catheterization and acute vasodilator testing with inhaled iloprost in pediatric idiopathic pulmonary arterial hypertension

Chen Zhang, Konstantinos Dimopoulos, Qiangqiang Li, Hong GuBeijing Anzhen Hospital, Capital Medical University. Royal Brompton Hospital.China and United Kingdom Pulmonary Circulation Pulm Circ 2022; 12: DOI: 10.1002/pul2.12169 AbstractTo assess the long-term prognostic value of cardiac catheterization and acute vasodilator testing (AVT) with inhaled iloprost in children with idiopathic pulmonary arterial hypertension (IPAH). Data on 81 consecutive

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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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