Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing

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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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.

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

Right ventriculo-pulmonary arterial uncoupling and poor outcomes in pulmonary arterial hypertension

Toshitaka Nakaya, Hiroshi Ohira, Takahiro Sato, Taku Watanabe, Masaharu Nishimura, Noriko Oyama-Manabe, Masaru Kato, Yoichi M. Ito and Ichizo TsujinoHokkaido University Hospital. The Institute of Statistical Mathematics.Japan Pulmonary CirculationPulm Circ 2020; 10: DOI: 10.1177/2045894020957223 AbstractRight ventricular function critically affects the prognosis of patients with pulmonary arterial hypertension. We aimed to analyze the prognostic value of right

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Prognostic value of longitudinal vasoreactivity in pediatric pulmonary hypertension

Patrick D. Evers, Patrick Quinn, Paul J. Critser, Benjamin S. Frank, Laurie B. ArmsbyOregon Health Sciences University. University of Cininnati. University of Colorado.United States Pulmonary CirculationPulm Circ 2022; 12: e12152DOI: 10.1002/pul2.12152 AbstractUpon diagnosis of pulmonary hypertension in pediatrics, standard practice often involves acute vasoreactivity testing (AVT) in the cardiac catheterization laboratory. However, the importance of repeated

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