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High-level gonosomal mosaicism for a pathogenic non-coding CNV deletion of the lung-specific FOXF1 enhancer in an unaffected mother of an infant with ACDMPV

Esra Yıldız Bölükbaşi, Tomasz Gambin, Justyna A. Karolak, Nicholas Willard, Przemyslaw Szafranski, Steven H. Abman, Csaba Galambos, John P. Kinsella, Paweł StankiewiczBaylor College of Medicine. Poznan University of Medical Sciences. Warsaw University of Technology. University of Colorado Anschutz Medical Campus.United States and Poland Molecular Genetics and Genomic MedicineMol Genet Genomic Med 2022; 10: DOI: 10.1002/mgg3.2062 AbstractBackground: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) results […]

High-level gonosomal mosaicism for a pathogenic non-coding CNV deletion of the lung-specific FOXF1 enhancer in an unaffected mother of an infant with ACDMPV Read More »

The biventricular contribution to chronic pulmonary hypertension of the extremely premature infant

Gabriela de Carvalho Nunes, Punnanee Wutthigate, Jessica Simoneau, Adrian Dancea, Marc Beltempo, Claudia Renaud, Gabriel AltitMcGill University Health Centre and Montreal Children’s Hospital. Siriraj Hospital and Mahidol University. Canada and Thailand Journal of PerinatologyJ Perinatol 2023; 43: 174-180DOI: 10.1038/s41372-022-01497-0 AbstractObjective: Evaluate factors associated with significant pulmonary hypertension [PH] (≥2/3 systemic) and its impact on ventricular function at

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Characterisation of paediatric pulmonary hypertensive vascular disease from the PPHNet Registry

Steven H. Abman, Mary P. Mullen, Lynn A. Sleeper, Eric D. Austin, Erika B. Rosenzweig, John P. Kinsella, D. Dunbar Ivy, Rachel K. Hopper, J. Usha Raj, Jeffrey Fineman, Roberta L. Keller, Angela Bates, Usha S. Krishnan, Catherine M. Avitabile, Alexander Davidson, Marc D. Natter, Kenneth D. MandlPediatric Pulmonary Hypertension NetworkUnited States and Canada European Respiratory JournalEur Respir J 2021; DOI: 10.1183/13993003.03337-2020 ExtractBackground: There are limited data about the range of diseases, natural history, age-appropriate end-points and optimal care for children with pulmonary hypertension (PH), including the need for developing

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Pregnancy outcomes of women with Eisenmenger syndrome: A single-center study

Yang Liu, Hong Gu, Yanna Li, Jun Zhang, Xiangming Fan, Yichen Zhao, Jiangang WangBeijing Anzhen Hospital affiliated to Capital Medical University. Hebei General Hospital. Zhejiang Children’s Hospital. China International Journal of CardiologyInt J Cardiol 2023; 374: 35-41DOI: 10.1016/j.ijcard.2022.12.014 AbstractBackground: To explore the outcomes of mothers with Eisenmenger syndrome (ES) and their offspring.Methods: Pregnant women with ES admitted to

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Novel variants of seryl-tRNA synthetase resulting in HUPRA syndrome featured in pulmonary hypertension

Fan Yang, Dan Wang, Xuehua Zhang, Haoqin Fan, Yu Zheng, Zhenghui Xiao, Zhi Chen, Yunbin Xiao, Qiming LiuSecond Xiangya Hospital and Central South University. Hunan Children’s Hospital. Fujian Children’s Hospital and Fujian Medical University.China Frontiers in Cardiovascular MedicineFront Cardiovasc Med 2023; DOI: 10.3389/fcvm.2022.1058569 AbstractHyperuricemia, pulmonary hypertension, and renal failure in infancy and alkalosis syndrome (HUPRA syndrome)

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Hemodynamic Characterization of Neonates With Congenital Diaphragmatic Hernia-Associated Pulmonary Hypertension by Cardiac Catheterization

Paula Dias Maia, Jason Gien, John P. Kinsella, Jenny Zablah, Gareth Morgan, D. Dunbar Ivy, Steven H. Abman, Benjamin S. FrankUniversity of Colorado School of Medicine.United States Journal of PediatricsJ Pediatr 2022; DOI: 10.1016/j.jpeds.2022.11.028 AbstractWe examined the results of cardiac catheterization in infants with congenital diaphragmatic hernia (CDH) from 2009 to 2020. Catheterization confirmed pulmonary arterial

Hemodynamic Characterization of Neonates With Congenital Diaphragmatic Hernia-Associated Pulmonary Hypertension by Cardiac Catheterization Read More »

The Oxygen Saturation Index as Early Predictor of Outcomes in Congenital Diaphragmatic Hernia

Emily J. J. Horn-Oudshoorn, Marijn J. Vermeulen, Ronny Knol, Arjan B. te Pas, Suzan C. M. Cochius-den Otter, J. Marco Schnater, Irwin K.M. Reiss, Philip L. J. DeKoninckErasmus MC University Medical Center. Leiden University Medical Center. Netherlands NeonatologyNeonatology 2023; 120: 63-70DOI: 10.1159/000527407 AbstractObjective: The aim of the study was to evaluate the oxygen saturation index (OSI) as

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Fetal endoscopic tracheal occlusion for moderate and severe congenital diaphragmatic hernia: a systematic review and meta-analysis of randomized controlled trials

Qiyu Li, Siyuan Liu, Xuemei Ma, Jiaping YuGeneral Hospital of Northern Theater Command. China Pediatric Surgery InternationalPediatr Surg 2022; 38: 1217-1226 DOI: 10.1007/s00383-022-05170-7 AbstractBackground: Fetoscopic endoluminal tracheal occlusion (FETO) is considered to increase survival among fetuses with congenital diaphragmatic hernia (CDH). Data from high-quality trials had been lacking until the largest randomized controlled trials (the TOTAL trials)

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A Measurement-Based Protocol Improves Interrater Agreement and Accuracy of Right Ventricular Systolic Pressure Assessment by Echocardiography in Children: A Call for Quality Improvement

Hythem Nawaytou, Christine Springston, Ann Lazar, Samuel Keller, Whitnee Hogan, Nicole Cresalia, Shabnam Peyvandi, Mark Cocalis, Anita Moon-Grady, Michael BrookUniversity of California, San Francisco.United States Journal of the American Society of EchocardiographyJ Am Soc Echocardiogr 2022; 35: 1091-1100DOI: 10.1016/j.echo.2022.06.014 AbstractBackground: Echocardiographic measurements carry the promise of improving interrater (IR) agreement over subjective assessment. The aim of this

A Measurement-Based Protocol Improves Interrater Agreement and Accuracy of Right Ventricular Systolic Pressure Assessment by Echocardiography in Children: A Call for Quality Improvement Read More »

Worsened short-term clinical outcomes in a cohort of patients with iNO-unresponsive PPHN: a case for improving iNO responsiveness

Julie Dillard, Leeann R. Pavlek, Saichidroopi Korada, Bernadette ChenSt. Louis University. Nationwide Children’s Hospital and Ohio State University.United States Journal of PerinatologyJ Perinatol 2022; 42: 37-44DOI: 10.1038/s41372-021-01228-x AbstractObjectives: To identify distinguishing characteristics of neonates with persistent pulmonary hypertension of the newborn (PPHN) unresponsive to inhaled nitric oxide (iNO) and evaluate the use of milrinone in this cohort.Study

Worsened short-term clinical outcomes in a cohort of patients with iNO-unresponsive PPHN: a case for improving iNO responsiveness Read More »

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