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JOURNAL ARTICLE
Geoffroy Hariri, Xavier Luxey, Stefanie Wenger, Pauline Dureau, Sarah Hariri, Ahmed Charfeddine, Guillaume Lebreton, Nima Djavidi, Aymeric Lancelot, Baptiste Duceau, Adrien Bouglé
BACKGROUND: Monitoring fluid therapy is challenging in patients assisted with Veno-arterial ECMO. The aim of our study was to evaluate the usefulness of capillary refill time to assess the response to fluid challenge in patients assisted with VA-ECMO. METHODS: Retrospective monocentric study in a cardiac surgery ICU. We assess fluid responsiveness after a fluid challenge in patients on VA-ECMO. We recorded capillary refill time before and after fluid challenge and the evolution of global hemodynamic parameters...
March 9, 2024: Journal of Critical Care
#2
JOURNAL ARTICLE
Robert A Byrne, Xavier Rossello, J J Coughlan, Emanuele Barbato, Colin Berry, Alaide Chieffo, Marc J Claeys, Gheorghe-Andrei Dan, Marc R Dweck, Mary Galbraith, Martine Gilard, Lynne Hinterbuchner, Ewa A Jankowska, Peter Jüni, Takeshi Kimura, Vijay Kunadian, Margret Leosdottir, Roberto Lorusso, Roberto F E Pedretti, Angelos G Rigopoulos, Maria Rubini Gimenez, Holger Thiele, Pascal Vranckx, Sven Wassmann, Nanette Kass Wenger, Borja Ibanez
No abstract text is available yet for this article.
September 22, 2023: European Heart Journal. Acute Cardiovascular Care
#3
JOURNAL ARTICLE
Arash Harzand, Alaaeddin Alrohaibani, Muhammed Y Idris, Hayden Spence, Cate G Parrish, Pratik K Rout, Rene Nazar, Michelle L Davis-Watts, Phyllis P Wright, Alexander A Vakili, Smah Abdelhamid, Harshvardhan Vathsangam, Adelanwa Adesanya, Linda G Park, Mary A Whooley, Nanette K Wenger, A Maziar Zafari, Amit J Shah
BACKGROUND: Cardiac rehabilitation (CR) improves outcomes in heart disease yet remains vastly underutilized. Remote CR enhanced with a digital health intervention (DHI) may offer higher access and improved patient-centered outcomes over non-technology approaches. We sought to pragmatically determine whether offering a DHI improves CR access, cardiac risk profile, and patient-reported outcome measures. METHODS: Adults referred to CR at a tertiary VA medical center between October 2017 and December 2021 were offered enrollment into a DHI alongside other CR modalities using shared decision-making...
September 12, 2023: BMC Cardiovascular Disorders
#4
JOURNAL ARTICLE
Robert A Byrne, Xavier Rossello, J J Coughlan, Emanuele Barbato, Colin Berry, Alaide Chieffo, Marc J Claeys, Gheorghe-Andrei Dan, Marc R Dweck, Mary Galbraith, Martine Gilard, Lynne Hinterbuchner, Ewa A Jankowska, Peter Jüni, Takeshi Kimura, Vijay Kunadian, Margret Leosdottir, Roberto Lorusso, Roberto F E Pedretti, Angelos G Rigopoulos, Maria Rubini Gimenez, Holger Thiele, Pascal Vranckx, Sven Wassmann, Nanette Kass Wenger, Borja Ibanez
No abstract text is available yet for this article.
August 25, 2023: European Heart Journal
#5
JOURNAL ARTICLE
Kelly Smallwood, Kristin E N Watt, Satoru Ide, Kristina Baltrunaite, Chad Brunswick, Katherine Inskeep, Corrine Capannari, Margaret P Adam, Amber Begtrup, Debora R Bertola, Laurie Demmer, Erin Demo, Orrin Devinsky, Emily R Gallagher, Maria J Guillen Sacoto, Robert Jech, Boris Keren, Jennifer Kussmann, Roger Ladda, Lisa A Lansdon, Sebastian Lunke, Anne Mardy, Kirsty McWalters, Richard Person, Laura Raiti, Noriko Saitoh, Carol J Saunders, Rhonda Schnur, Matej Skorvanek, Susan L Sell, Anne Slavotinek, Bonnie R Sullivan, Zornitza Stark, Joseph D Symonds, Tara Wenger, Sacha Weber, Sandra Whalen, Susan M White, Juliane Winkelmann, Michael Zech, Shimriet Zeidler, Kazuhiro Maeshima, Rolf W Stottmann, Paul A Trainor, K Nicole Weaver
Heterozygous pathogenic variants in POLR1A, which encodes the largest subunit of RNA Polymerase I, were previously identified as the cause of acrofacial dysostosis, Cincinnati-type. The predominant phenotypes observed in the cohort of 3 individuals were craniofacial anomalies reminiscent of Treacher Collins syndrome. We subsequently identified 17 additional individuals with 12 unique heterozygous variants in POLR1A and observed numerous additional phenotypes including neurodevelopmental abnormalities and structural cardiac defects, in combination with highly prevalent craniofacial anomalies and variable limb defects...
May 4, 2023: American Journal of Human Genetics
#6
JOURNAL ARTICLE
Evan A Blank, Mikhael F El-Chami, Nanette K Wenger
The field of cardiac pacing has been defined by constant development to provide efficacious, safe, and reliable therapy. Traditional pacing utilizes transvenous leads, which dwell in the venous system and place patients at risk for complications, including pneumothorax, bleeding, infection, vascular obstruction, and valvular compromise. Leadless pacemakers have been developed to overcome many of the challenges of transvenous pacing while providing safe and effective pacing therapy for an increasing population of patients...
March 31, 2023: Current Cardiology Reviews
#7
MULTICENTER STUDY
Ericka L Fink, Patrick M Kochanek, Ashok Panigrahy, Sue R Beers, Rachel P Berger, Hülya Bayir, Jose Pineda, Christopher Newth, Alexis A Topjian, Craig A Press, Aline B Maddux, Frederick Willyerd, Elizabeth A Hunt, Ashley Siems, Melissa G Chung, Lincoln Smith, Jesse Wenger, Lesley Doughty, J Wesley Diddle, Jason Patregnani, Juan Piantino, Karen Hallermeier Walson, Binod Balakrishnan, Michael T Meyer, Stuart Friess, David Maloney, Pamela Rubin, Tamara L Haller, Amery Treble-Barna, Chunyan Wang, Robert R S B Clark, Anthony Fabio
IMPORTANCE: Families and clinicians have limited validated tools available to assist in estimating long-term outcomes early after pediatric cardiac arrest. Blood-based brain-specific biomarkers may be helpful tools to aid in outcome assessment. OBJECTIVE: To analyze the association of blood-based brain injury biomarker concentrations with outcomes 1 year after pediatric cardiac arrest. DESIGN, SETTING, AND PARTICIPANTS: The Personalizing Outcomes After Child Cardiac Arrest multicenter prospective cohort study was conducted in pediatric intensive care units at 14 academic referral centers in the US between May 16, 2017, and August 19, 2020, with the primary investigators blinded to 1-year outcomes...
September 1, 2022: JAMA Network Open
#8
JOURNAL ARTICLE
Joseph S Leslie, Rim Hjeij, Asaf Vivante, Elizabeth A Bearce, Laura Dyer, Jiaolong Wang, Lettie Rawlins, Joanna Kennedy, Nishanka Ubeyratna, James Fasham, Zoe H Irons, Samuel B Craig, Julia Koenig, Sebastian George, Ben Pode-Shakked, Yoav Bolkier, Ortal Barel, Shrikant Mane, Kathrine K Frederiksen, Olivia Wenger, Ethan Scott, Harold E Cross, Esben Lorentzen, Dominic P Norris, Yair Anikster, Heymut Omran, Daniel T Grimes, Andrew H Crosby, Emma L Baple
PURPOSE: The clinical spectrum of motile ciliopathies includes laterality defects, hydrocephalus, and infertility as well as primary ciliary dyskinesia when impaired mucociliary clearance results in otosinopulmonary disease. Importantly, approximately 30% of patients with primary ciliary dyskinesia lack a genetic diagnosis. METHODS: Clinical, genomic, biochemical, and functional studies were performed alongside in vivo modeling of DAW1 variants. RESULTS: In this study, we identified biallelic DAW1 variants associated with laterality defects and respiratory symptoms compatible with motile cilia dysfunction...
September 7, 2022: Genetics in Medicine: Official Journal of the American College of Medical Genetics
#9
JOURNAL ARTICLE
Michael J Goldfarb, Christine Bechtel, Quinn Capers, Ann de Velasco, John A Dodson, Jamie L Jackson, Lisa Kitko, Ileana L Piña, Erin Rayner-Hartley, Nanette K Wenger, Martha Gulati
Family engagement empowers family members to become active partners in care delivery. Family members increasingly expect and wish to participate in care and be involved in the decision-making process. The goal of engaging families in care is to improve the care experience to achieve better outcomes for both patients and family members. There is emerging evidence that engaging family members in care improves person- and family-important outcomes. Engaging families in adult cardiovascular care involves a paradigm shift in the current organization and delivery of both acute and chronic cardiac care...
April 21, 2022: Journal of the American Heart Association
#10
JOURNAL ARTICLE
James Fasham, Siying Lin, Promita Ghosh, Francesca Clementina Radio, Emily G Farrow, Isabelle Thiffault, Jennifer Kussman, Dihong Zhou, Rick Hemming, Kenneth Zahka, Barry A Chioza, Lettie E Rawlins, Olivia K Wenger, Adam C Gunning, Simone Pizzi, Roberta Onesimo, Giuseppe Zampino, Emily Barker, Natasha Osawa, Megan Christine Rodriguez, Teresa M Neuhann, Elaine H Zackai, Beth Keena, Jenina Capasso, Alex V Levin, Elizabeth Bhoj, Dong Li, Hakon Hakonarson, Ingrid M Wentzensen, Adam Jackson, Kate E Chandler, Zeynep H Coban-Akdemir, Jennifer E Posey, Siddharth Banka, James R Lupski, Sarah E Sheppard, Marco Tartaglia, Barbara Triggs-Raine, Andrew H Crosby, Emma L Baple
PURPOSE: We previously defined biallelic HYAL2 variants causing a novel disorder in 2 families, involving orofacial clefting, facial dysmorphism, congenital heart disease, and ocular abnormalities, with Hyal2 knockout mice displaying similar phenotypes. In this study, we better define the phenotype and pathologic disease mechanism. METHODS: Clinical and genomic investigations were undertaken alongside molecular studies, including immunoblotting and immunofluorescence analyses of variant/wild-type human HYAL2 expressed in mouse fibroblasts, and in silico modeling of putative pathogenic variants...
November 30, 2021: Genetics in Medicine: Official Journal of the American College of Medical Genetics
#11
JOURNAL ARTICLE
Maya Dewan, Allison Parsons, Ken Tegtmeyer, Jesse Wenger, Dana Niles, Tia Raymond, Adam Cheng, Sophie Skellett, Joan Roberts, Priti Jani, Vinay Nadkarni, Heather Wolfe
INTRODUCTION: Pediatric quality improvement (QI) collaboratives are multisite clinical networks that support cooperative learning. Our goal is to identify the contextual facilitators and barriers to implementing QI resuscitation interventions within a multicenter resuscitation collaborative. METHODS: A mixed-methods evaluation of the contextual facilitators and barriers to implementation of a resuscitation QI bundle. We administered a quantitative questionnaire, the Model for Understanding Success in Quality (MUSIQ), to the Pediatric Resuscitation Quality (pediRES-Q) Collaborative...
September 2021: Pediatric Quality & Safety
#12
JOURNAL ARTICLE
Erin Walker MacKintosh, Maida Lynn Chen, Tara Wenger, Kristen Carlin, Luciana Young
The prevalence of obstructive sleep apnea (OSA) is increased in children and adults with Marfan syndrome (MFS) compared to the general population and has been shown to be associated with rapid aortic root dilation and dissection in adults. Early diagnosis and treatment of OSA may decrease long-term cardiac morbidity. We therefore studied the utility of noninvasive OSA screening tools in children with MFS. We hypothesized that youth with MFS would have higher OSA screening scores than the general pediatric population...
March 2021: Pediatric Cardiology
#13
REVIEW
Ashok Krishnaswami, Craig Beavers, Michael P Dorsch, John A Dodson, Ruth Masterson Creber, Spyros Kitsiou, Parag Goyal, Mathew S Maurer, Nanette K Wenger, Deborah S Croy, Karen P Alexander, John A Batsis, Mintu P Turakhia, Daniel E Forman, Gwen M Bernacki, James N Kirkpatrick, Nicole M Orr, Eric D Peterson, Michael W Rich, Andrew M Freeman, Sanjeev P Bhavnani
The growing population of older adults (age ≥65 years) is expected to lead to higher rates of cardiovascular disease. The expansion of digital health (encompassing telehealth, telemedicine, mobile health, and remote patient monitoring), Internet access, and cellular technologies provides an opportunity to enhance patient care and improve health outcomes-opportunities that are particularly relevant during the current coronavirus disease-2019 pandemic. Insufficient dexterity, visual impairment, and cognitive dysfunction, found commonly in older adults should be taken into consideration in the development and utilization of existing technologies...
December 1, 2020: Journal of the American College of Cardiology
#14
JOURNAL ARTICLE
Olivia Wenger, Miraides Brown, Brandon Smith, Devyani Chowdhury, Andrew H Crosby, Emma L Baple, Mark Yoder, William Laxen, Silvia Tortorelli, Kevin A Strauss
Propionic acidemia (PA) is caused by inherited deficiency of mitochondrial propionyl-CoA carboxylase (PCC) and results in significant neurodevelopmental and cardiac morbidity. However, relationships among therapeutic intervention, biochemical markers, and disease progression are poorly understood. Sixteen individuals homozygous for PCCB c.1606A > G (p.Asn536Asp) variant PA participated in a two-week suspension of therapy. Standard metabolic markers (plasma amino acids, blood spot methylcitrate, plasma/urine acylcarnitines, urine organic acids) were obtained before and after stopping treatment...
November 2020: Molecular Genetics and Metabolism
#15
JOURNAL ARTICLE
Inge J van den Hoogen, Umberto Gianni, Malissa J Wood, Viviany R Taqueti, Fay Y Lin, Sharonne N Hayes, Gudrun M Feuchtner, Alexander R van Rosendael, Nanette K Wenger, Janet Wei, C Noel Bairey Merz, Carl J Pepine, Leslee J Shaw
Women exhibit less burden of anatomic obstructive coronary atherosclerotic disease as compared with men of the same age, but contradictorily show similar or higher cardiovascular mortality rates. The higher prevalence of nonexertional cardiac symptoms and nonobstructive coronary atherosclerotic disease in women may lead to lack of recognition and appropriate management, resulting in undertesting and undertreatment. Leaders in women's health from the American College of Cardiology's Cardiovascular Disease in Women Committee present novel imaging cases that may provoke thought regarding the broad clinical spectrum of myocardial infarction and ischemia with nonobstructive coronary arteries in women...
May 2021: JACC. Cardiovascular Imaging
#16
JOURNAL ARTICLE
Ronak Shah, Danielle Pulton, Robert K Wenger, Bao Ha, Jared W Feinman, Saumil Patel, Christopher Lau, Lisa Q Rong, Stuart J Weiss, John G Augoustides, Danisa Daubenspeck, Mark A Chaney
No abstract text is available yet for this article.
January 2021: Journal of Cardiothoracic and Vascular Anesthesia
#17
JOURNAL ARTICLE
Heather A Wolfe, Jesse Wenger, Robert Sutton, Roopa Seshadri, Dana E Niles, Vinay Nadkarni, Jordan Duval-Arnould, Anita I Sen, Adam Cheng
INTRODUCTION: Clinical event debriefing functions to identify optimal and suboptimal performance to improve future performance. "Cold" debriefing (CD), or debriefing performed more than 1 day after an event, was reported to improve patient survival in a single institution. We sought to describe the frequency and content of CD across multiple pediatric centers. METHODS: Mixed-methods, a retrospective review of prospectively collected in-hospital cardiac arrest (IHCA) data, and a supplemental survey of 18 international institutions in the Pediatric Resuscitation Quality (pediRES-Q) collaborative...
July 2020: Pediatric Quality & Safety
#18
JOURNAL ARTICLE
Deborah K Steward, Nancy Ryan-Wenger, Tondi M Harrison, Karen F Pridham
BACKGROUND: Infants with complex congenital heart defects (CCHDs) experience alterations in growth that develop following surgical intervention and persist throughout early infancy, but the roles of nutritional intake and method of feeding require further exploration as their roles are not fully explained. OBJECTIVES: The purpose of this study was to characterize trends in growth and nutritional intake during the first 6 months of life in infants with CCHD. METHODS: We conducted a secondary analysis of growth and nutritional data from a pilot study designed to test the feasibility of nurse-guided participatory intervention with parents of infants with CCHD...
2020: Nursing Research
#19
RANDOMIZED CONTROLLED TRIAL
Carmen A Pfortmueller, Manuel Kindler, Noelle Schenk, Anna S Messmer, Benjamin Hess, Laura Jakob, Stefanie Wenger, Jan Waskowski, Patrick Zuercher, Frederik Stoehr, Stephan M Jakob, Lars Englberger, Joerg C Schefold
PURPOSE: Recent evidence questions a liberal approach to fluid resuscitation in intensive care unit (ICU) patients. Here, we assess whether use of hypertonic saline applied as single infusion at ICU admission after cardiac surgery can reduce cumulative perioperative fluid volume. METHODS: Prospective randomized double-blind single-center clinical trial investigates effects of a single infusion of hypertonic saline (HS) versus normal saline (comparator). Primary endpoint was the cumulative amount of fluid administered in patients in the hypertonic saline versus the 0...
September 2020: Intensive Care Medicine
#20
JOURNAL ARTICLE
Danny E Miller, Penny Chow, Emily R Gallagher, Jonathan A Perkins, Tara L Wenger
Catel-Manzke syndrome is characterized by hand anomalies, Robin sequence, cardiac defects, joint hyperextensibility, and characteristic facial features. Approximately 40 patients with Catel-Manzke have been reported, all with the pathognomonic bilateral or unilateral hyperphalangy caused by an accessory bone between the second metacarpal and proximal phalanx known as Manzke dysostosis. Here we present the first case of molecularly confirmed Catel-Manzke syndrome with Robin sequence but without Manzke dysostosis...
March 2020: American Journal of Medical Genetics. Part A
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