Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing

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.

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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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Management of Complex Pulmonary Vein Stenosis at Altitude Combining Comprehensive Percutaneous Interventional Treatment with Sirolimus, Pulmonary Hypertension Medications and Intraluminal Imaging with Optical Coherence Tomography

Michael J. Shorofsky, Gareth J. Morgan, Ernesto Mejia, Salvador A. Rodriguez, Megan Greene, Paige Sheaks, Dunbar Ivy, Jenny E. ZablahUniversity of Colorado and Children’s Hospital Colorado.United States Pediatric CardiologyPediatr Cardiol 2023; DOI: 10.1007/s00246-023-03102-3 AbstractBackground: Pulmonary vein stenosis (PVS) is a growing problem for the pediatric congenital heart population. Sirolimus has previously been shown to improve survival and

Management of Complex Pulmonary Vein Stenosis at Altitude Combining Comprehensive Percutaneous Interventional Treatment with Sirolimus, Pulmonary Hypertension Medications and Intraluminal Imaging with Optical Coherence Tomography Read More »

Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management

Erika B. Rosenzweig, Steven H. Abman, Ian Adatia, Maurice Beghetti, Damien Bonnet, Sheila Haworth, D. Dunbar Ivy, Rolf M. F. BergerColumbia University and New York Presbyterian Hospital. University of Colorado and Children’s Hospital Colorado. Glenwood Children’s Heart Clinic. University Hospitals of Geneva and Lausanne. Hôpital Necker Enfants Malades, AP-HP, Université Paris Descartes. University College London.

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International practice heterogeneity in pre-transplant management of pulmonary hypertension related to pediatric left heart disease

Rachel K. Hopper, Oscar van der Have, Seth A. Hollander, Anne I. Dipchand, Valeria Perez de Sa, Jeffery A. Feinstein, Karin Tran-LundmarkStanford University and Lucille Packard Children’s Hospital. Lund University. Skane University Hospital. University of Toronto and the Hospital for Sick ChildrenUnited States, Sweden and Canada Pediatric TransplantationPediatr Transplant 2023; DOI: 10.1111/petr.14461 AbstractBackground: Elevated pulmonary vascular resistance

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