Pediatric Pulmonary Vascular Disease

Safety and Feasibility of Riociguat Therapy for the Treatment of Chronic Pulmonary Arterial Hypertension in Infancy

Regan E. Geisinger, Amy H. Stanford, Brady Thomas, Steven H. Abman, Patrick J. McNammaraUniversity of Iowa. University of Colorado.United States Journal of PediatricsJ Pediatr 2022; DOI: 10.1016/j.jpeds.2022.11.026 AbstractThe effects of riociguat, an oral-soluble guanylate-cyclase stimulator, were studied in 10 infants with chronic pulmonary arterial hypertension. Respiratory status (n = 8/10), right heart dilation (n = 7/10), […]

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Riociguat in children with pulmonary arterial hypertension: The PATENT-CHILD study

Humberto García Aguilar, Matthias Gorenflo, D. Dunbar Ivy, Shahin Moledina, Biagio Castaldi, Hidekazu Ishida, Paweł Cześniewicz, Jacek Kusa, Oliver Miera, Joseph Pattathu, Ken‐Pen Weng, Laszlo Ablonczy, Christian Apitz, Marta Katona, Kenichi Kurosaki, Tomas Pulido, Hiroyuki Yamagishi, Kazushi Yasuda, Galia Cisternas, Melanie Goth, Susanne Lippert, Anna Radomskyj, Soundos Saleh, Stefan Willmann, Gabriela Wirsching, Damien Bonnet, Maurice

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High-Altitude Pulmonary Edema in Colorado Children: A Cross-Sectional Survey and Retrospective Review

Timothy D. Kelly, Maxene Meier, Jason P. Weinman, Dunbar Ivy, John T. Brinton, Deborah R. LiptzinIndiana University. University of Colorado and Children’s Hospital Colorado. Colorado School of Public Health.United States High Altitude Medicine and BiologyHigh Alt Med Biol 2022; 23: 119-124DOI: 10.1089/ham.2021.0121 AbstractIntroduction: Few studies of high-altitude pulmonary edema (HAPE) are specific to the pediatric population. The

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Echocardiographic Assessment of Pulmonary Hypertension in Neonates with Congenital Diaphragmatic Hernia Using Pulmonary Artery Flow Characteristics

Florian Kipfmueller, Lukas Schroeder, Ulrich Gembruch, Suemeyra Akkas, Flaminia Pugnaloni, Bartolomeo Bo, Lotte Lemloh, Annegret Geipel, Christoph Berg, Andreas Heydweiller, Andreas MuellerUniversity of Bonn. Ospidale Bambinu Gesu. University Hospital Cologne.Germany and Italy Journal of Clinical MedicineJ Clin Med 2022; 11: DOI: 10.3390/jcm11113038 AbstractBackground: Assessment of pulmonary hypertension (PH) is essential in neonates with congenital diaphragmatic hernia

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Clinical Significance of Early Pulmonary Hypertension in Preterm Infants

Sanne Arjaans, Marian W. F. Fries, Mirthe H. Schoots, Carine F. M. Schilte, Marc T. R. Roofthooft, Elianne J. L. E. Vrijlandt, Arie F. Bos, Elisabeth M. W. Kooi, Rolf M. F. BergerUniversity of Groningen, Beatrix Children’s Hospital and University Medical Center Groningen.Netherlands Journal of PediatricsJ Pediatr 2022; 251: 74-81DOI: 10.1016/j.jpeds.2022.07.039 AbstractObjective: To characterize different phenotypes of

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A Morphomolecular Approach to Alveolar Capillary Dysplasia

Jan C. Kamp, Lavinia Neubert, Maxmilian Ackermann, Helge Stark, Edith Plucinski, Harshit R. Shah, Sabina Janciauskiene, Anke K. Bergmann, Gunnar Schmidt, Tobias Welte, Axel Haverich, Christopher Werlein, Peter Braubach, Florian Laenger, Nicolaus Schwerk, Karen M. Olsson, Jan Fuge, Da-Hee Park, Jonas C. Schupp, Marius M. Hoeper, Mark P. Kuehnel, Danny D. JonigkHannover Medical School. University

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Exercise and hypoxia unmask pulmonary vascular disease and right ventricular dysfunction in a 10- to 12-week-old swine model of neonatal oxidative injury

Jarno J. Steenhorst, Alexander Hirsch, Annemarie Verzijl, Piotr Wielopolski, Daphne de Wijs-Meijler, Dirk J. Duncker, Irwin K. M. Reiss, Daphne MerkusUniversity Medical Center Rotterdam. University Clinic Munich. Munich Heart Alliance.Netherlands and Germany Journal of PhysiologyJ Physiol 2022; DOI: 10.1113/JP282906 AbstractPrematurely born young adults who experienced neonatal oxidative injury (NOI) of the lungs have increased incidence of

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Biallelic variants of ATP13A3 cause dose-dependent childhood-onset pulmonary arterial hypertension characterised by extreme morbidity and mortality

Rajiv D. Machado, Carrie L. Welch, Matthias Haimel, Marta Bleda, Elizabeth Colglazier, John D. Coulson, Marusa Debeljak, Josef Ekstein, Jeffrey R. Fineman, William Christopher Golden, Emily L. Griffin, Charaka Hadinnapola, Michael A. Harris, Yoel Hirsch, Julie Elizabeth Hoover- Fong, Lawrence Nogee, Lewis H. Romer, Samo Vesel, NIHR Bioresource – Rare Diseases, Stefan Gräf, Nicholas W.

Biallelic variants of ATP13A3 cause dose-dependent childhood-onset pulmonary arterial hypertension characterised by extreme morbidity and mortality Read More »

Genetics dictating therapeutic decisions in pediatric pulmonary hypertension? A case report suggesting we are getting closer

Leah Stevens, Elizabeth Colglazier, Claire Parker, Elena K. Amin, Hythem Nawaytou, David Teitel, Vadiyala M. Reddy, Carrie L. Welch, Wendy K. Chung, Jeffrey R. FinemanUniversity of California San Francisco. Columbia University Irving Medical Center. United States Pulmonary CirculationPulm Circ 2022; 12: DOI: 10.1002/pul2.12033 AbstractDespite therapeutic advances over the past decades, pulmonary arterial hypertension (PAH) and related

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Possible association between a polymorphism of EPAS1 gene and persistent pulmonary hypertension of the newborn: a case-control study

Narongsak Nakwan, Surakameth Mahasirimongkol, Nusara Satproedprai, Tassamonwan Chaiyasung, Punna Kunhapan, Cheep Charoenlap, KumonnutSingkhamanan, Chariyawan CharalsawadiPrince of Songkla University. Nonthaburi Ministry of Public Heath. Hat Yai Hospital. Thailand Jornal de PediatriaJ Pediatr 2022; 98: 383-389DOI: 10.1016/j.jped.2021.09.003 AbstractObjective: To explore possible genes related to the development of persistent pulmonary hypertension of the newborn (PPHN).Methods: The authors identified 285 single nucleotide

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