Pediatric Pulmonary Vascular Disease

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 […]

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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 »

Association of the dysfunctional placentation endotype of prematurity with bronchopulmonary dysplasia: a systematic review, meta-analysis and meta-regression

Maria Pierro, Eduardo Villamor-Martinez, Elke van Westering- Kroon, Maria Alvarez-Fuente, Steven H Abman, Eduardo VillamorMaastricht University Medical Centre. Maurizio Bufalini Hospital. Hospital Universitario Ramón y Cajal. University of Colorado – Anschutz Medical Campus. Netherlands, Italy, Spain and United States ThoraxThorax 2022; 77: 268-275DOI: 10.1136/thoraxjnl-2020-216485 AbstractBackground: Antenatal pathological conditions are key in the pathogenesis of bronchopulmonary dysplasia (BPD).

Association of the dysfunctional placentation endotype of prematurity with bronchopulmonary dysplasia: a systematic review, meta-analysis and meta-regression Read More »

Adenosine reverses life-threatening persistent pulmonary hypertension of the neonate refractory to triple vasodilator therapy

Lucie Genet, Daniele De Luca“A.Béclère” Medical Center and Paris Saclay University Hospitals.France Cardiology in the YoungCardiol Young 2022; 32: 996-997DOI: 10.1017/S1047951121004157 AbstractPersistent pulmonary hypertension of the neonate can cause acute and life-threatening hypoxia, but preterm neonates are not suitable candidate to extra-corporeal life support. We report the unique case of an extremely preterm neonates with life-threatening

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Pulmonary lymphangiectasia in myotubular myopathy: a novel unrecognized association?

Gabriela de Carvalho Nunes, Karl Grenier, Chelsea Maedler Kron, Thomas Kitzler, Janine El Helou, David S. Rosenblatt, Francois OlivierMcGill University Health Centre. Jewish General Hospital.Canada Neuromuscular DisordersNeuromuscul Disord 2022; 32: 512-515DOI: 10.1016/j.nmd.2022.04.010 AbstractChylothorax has been reported in rare cases of X-linked myotubular myopathy, but the pathophysiology of this association is not fully understood. We report a

Pulmonary lymphangiectasia in myotubular myopathy: a novel unrecognized association? Read More »

Case report: Pulmonary hypertensive crisis leading to cardiac arrest during endoscopic evaluation in a 6-year-old boy with autism, severe malnutrition, and undiagnosed scurvy

Laura A. Quinn, Stephanie P. Gilley, Allison D. Ta, Benjamin S. Frank, Carolyn B. Foley, Jaime M. MooreUniversity of Colorado School of Medicine.United States Frontiers in PediatricsFront Pediatr 2022; DOI: 10.3389/fped.2022.1008507 AbstractPediatric gastroenterologists are often responsible for the evaluation of malnutrition in the setting of selective eating. Endoscopic evaluation for conditions including eosinophilic esophagitis and celiac

Case report: Pulmonary hypertensive crisis leading to cardiac arrest during endoscopic evaluation in a 6-year-old boy with autism, severe malnutrition, and undiagnosed scurvy Read More »

Hidden cardiovascular morbidity in children and young adults born with congenital diaphragmatic hernia: A population-based study

Katarina Övermo Tydén, Felicia Nordenstam, Björn Frenckner, Carmen Mesas BurgosKarolinska University Hospital. Sweden Journal of Pediatric SurgeryJ Pediatr Surg 2022; 57: 510-515DOI: 10.1016/j.jpedsurg.2022.03.028 AbstractIntroduction: Congenital diaphragmatic (CDH) hernia is a rare congenital malformation with considerable mortality and morbidity in the neonatal period. The majority of the children today survive but little is known about long term cardiovascular

Hidden cardiovascular morbidity in children and young adults born with congenital diaphragmatic hernia: A population-based study Read More »

Indications and outcome after lung transplantation in children under 12 years of age: A 16-year single center experience

Paval Iablonskii, Julia Carlens, Carsten Mueller, Khalil Aburahma, Adelheid Niehaus, Dietmar Boethig, Maximilian Boethig, Katharina Floethmann, Wiebke Sommer, Joerg Optenhoefel, Igor Tudorache, Mark Greer, Harald Koeditz, Thomas Jack, Georg Hansmann, Christian Kuehn, Alexander Horke, Gesine Hansen, Axel Haverich, Gregor Warnecke, Murat Avsar, Jawad Salman, Dmitry Bobylev, Fabio Ius, Nicolaus SchwerkHannover Medical School. Saint Petersburg State

Indications and outcome after lung transplantation in children under 12 years of age: A 16-year single center experience Read More »

Fetal pulmonary hypertension: dysregulated microRNA-34c-Notch1 axis contributes to impaired angiogenesis in an ovine model

Devashis Mukherjee, Ujala Rana, Alison J. Kriegel, Pengyuan Liu, Teresa Michalkiewicz, Girija Ganesh KonduriRainbow Babies and Children’s Hospital and Case Western Reserve University School of Medicine. Medical College of Wisconsin and Children’s Wisconsin.United States Pediatric ResearchPediatr Res 2022; 93: 551-558DOI: 10.1038/s41390-022-02151-3 AbstractBackground: Persistent pulmonary hypertension of the newborn (PPHN) occurs when pulmonary vascular resistance (PVR) fails to

Fetal pulmonary hypertension: dysregulated microRNA-34c-Notch1 axis contributes to impaired angiogenesis in an ovine model Read More »

Persistence of persistent pulmonary hypertension of the newborn: A case of de novo TBX4 variant

Stephanie M. Tsoi, Kirk Jones, Elizabeth Colglazier, Claire Parker, Hythem Nawaytou, David Teitel, Jeffrey R. Fineman, Roberta L. KellerUniversity of California San Francisco.United States Pulmonary CirculationPul Circ 2022; 12: DOI: 10.1002/pul2.12108 AbstractWe present a case of a late preterm infant placed on extracorporeal life support in the first day of life for persistent pulmonary hypertension of

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