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Pediatric congenital heart disease

Rafael Arboleda Salazar, Jane Heggie, Piotr Wolski, Eric Horlick, Mark Osten, Massimiliano Meineri
BACKGROUND: Twenty percent of patients born with congenital heart disease present with right ventricular outflow tract abnormalities. These patients require multiple surgical procedures in their lifetime. Transcatheter pulmonary valve replacement (TPVR) has become a viable alternative to conventional pulmonary valve and right ventricular outflow tract surgery in pediatric and adult populations. In this retrospective review, we analyze the perioperative management of adult patients who underwent TPVR in our center...
March 14, 2018: Anesthesia and Analgesia
Albert C Hergenroeder, Douglas S Moodie, Daniel J Penny, Constance M Wiemann, Blanca Sanchez-Fournier, Lauren K Moore, Jane Head
OBJECTIVE: To describe changes in functional status between the last pediatric and first adult congenital heart disease (CHD) clinic visits in patients with moderate to severe CHD after implementing a healthcare transition (HCT) planning program. DESIGN: Quasi-experimental design. Patients were followed prospectively following the implementation of the intervention; Control patients transitioned from the Pediatric CHD Clinic into Adult CHD Clinic before the intervention...
March 15, 2018: Congenital Heart Disease
Erica Sood, Allison Karpyn, Abigail C Demianczyk, Jennie Ryan, Emily A Delaplane, Trent Neely, Aisha H Frazier, Anne E Kazak
OBJECTIVE: To inform pediatric critical care practice by examining how mothers and fathers experience the stress of caring for a young child with congenital heart disease and use hospital and community supports. DESIGN: Qualitative study of mothers and fathers of young children with congenital heart disease. SETTING: Tertiary care pediatric hospital in the Mid-Atlantic region of the United States. SUBJECTS: Thirty-four parents (20 mothers, 14 fathers) from diverse backgrounds whose child previously underwent cardiac surgery during infancy...
March 10, 2018: Pediatric Critical Care Medicine
Kristen M Brown, Shawna S Mudd, Elizabeth A Hunt, Julianne S Perretta, Nicole A Shilkofski, J Wesley Diddle, Gregory Yurasek, Melania Bembea, Jordan Duval-Arnould, Kristen Nelson McMillan
OBJECTIVES: Assess the effect of a simulation "boot camp" on the ability of pediatric nurse practitioners to identify and treat a low cardiac output state in postoperative patients with congenital heart disease. Additionally, assess the pediatric nurse practitioners' confidence and satisfaction with simulation training. DESIGN: Prospective pre/post interventional pilot study. SETTING: University simulation center. SUBJECTS: Thirty acute care pediatric nurse practitioners from 13 academic medical centers in North America...
March 10, 2018: Pediatric Critical Care Medicine
Karen C Uzark, John M Costello, Holly C DeSena, Ravi Thiagajaran, Melissa Smith-Parrish, Katja M Gist
OBJECTIVES: Pediatric cardiac intensive care continues to evolve, with rapid advances in knowledge and improvement in clinical outcomes. In the past, the Board of Directors of the Pediatric Cardiac Intensive Care Society created and subsequently updated a list of sentinel references focused on the care of critically ill children with congenital and acquired heart disease. The objective of this article is to provide clinicians with a compilation and brief summary of updated and useful references that have been published since 2012...
March 10, 2018: Pediatric Critical Care Medicine
Cheng Sun, Maria I Kontaridis
The heart is one of the first organs to form and function during embryonic development. It is comprised of multiple cell lineages, each integral for proper cardiac development, and include cardiomyocytes, endothelial cells, epicardial cells and neural crest cells. The molecular mechanisms regulating cardiac development and morphogenesis are dependent on signaling crosstalk between multiple lineages through paracrine interactions, cell-ECM interactions, and cell-cell interactions, which together, help facilitate survival, growth, proliferation, differentiation and migration of cardiac tissue...
February 2018: Current Opinion in Physiology
Rachel Levene, Elza Pollak-Christian, Ashish Garg, Michael Keenaghan
Coarctation of the aorta (CoA) is a congenital cardiac malformation that is well understood. Despite being well characterized, CoA is a commonly missed congenital heart disease (CHD) during the newborn period. We report a full-term nine-day-old male who presented to the pediatric emergency department (ED) with isolated tachypnea. After an initial sepsis workup, subsequent investigations revealed critical CoA. Because the primary workup focused on sepsis, there was a significant delay in prostaglandin E1 (PGE1 ) initiation...
2018: Case Reports in Pediatrics
Hong-Yu Kuang, Yu-Hao Wu, Qi-Jian Yi, Jie Tian, Chun Wu, We Nian Shou, Tie-Wei Lu
BACKGROUND: Oral bosentan has been widely applied in pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). A systemic review and meta-analysis was conducted for a therapeutic evaluation of oral bosentan in both adult and pediatric patients with PAH-CHD. The acute responses and a long-term effect were respectively assessed in a comparison with baseline characteristics, and the improvement of exercise tolerance was analyzed. METHODS: PubMed, Medline, Embase, and Cochrane Central Register of clinical controlled trails or observational studies have been searched for a recording of bosentan effects on the PAH-CHD participants...
March 2018: Medicine (Baltimore)
Paula Colvara de Souza, Vanessa Souza Gigoski, Camila Lúcia Etges, Lisiane da Rosa Barbosa
PURPOSE: Describe the findings of postoperative clinical evaluation of swallowing in infants with congenital heart disease (CHD) in a reference hospital in southern Brazil. METHODS: This is a cross-sectional study conducted postoperatively with infants with medical diagnosis of CHD aged 0-6 months in a Pediatric Intensive Care Unit. Exclusion criteria comprised infants with neurological and respiratory impairments, craniofacial malformation, structural alteration in the upper airways, and suspicion or diagnosis of genetic syndromes...
March 1, 2018: CoDAS
C-H Wang, J Luo, J Li, J-Z Zhang, S-Y Huang, W Shao, H-S Ma
OBJECTIVE: To investigate the efficacy of inhalational sevoflurane anesthesia induction on inhibiting the stress response to endotracheal intubation in pediatric patients with congenital heart disease (CHD). PATIENTS AND METHODS: Forty ASA physical status I/II pediatric patients scheduled for interventricular septal defect repair or interatrial septal defect repair, were randomly divided into two groups (20 each): intravenous induction group (Group C) and inhalational sevoflurane anesthesia induction group (Group D)...
February 2018: European Review for Medical and Pharmacological Sciences
Daniel Rosenfield, Jason W Fischer, Charisse W Kwan
The first presentation of congenital heart disease can be a diagnostic challenge in the emergency department. We report on 2 cases where point-of-care ultrasound identified gross cardiac abnormalities in 2 children and expedited disposition and downstream care.
March 2018: Pediatric Emergency Care
Nadine A Kasparian, Richard De Abreu Lourenco, David S Winlaw, Gary F Sholler, Rosalie Viney, Edwin P E Kirk
PurposeAs the molecular basis of congenital heart disease (CHD) comes into sharper focus, cardiac genetics services are likely to play an increasingly important role. This study aimed to identify parents' preferences for, and willingness to participate in, clinical genetics services for CHD.MethodsA discrete choice experiment was developed to assess parents' preferences for pediatric cardiogenetics services based on four attributes: appointment format, health professionals involved, waiting time, and information format...
March 1, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Danton S Char, Sandra Soo-Jin Lee, David Magnus, Mildred Cho
PurposeTo investigate the potential impacts of whole-genome sequencing (WGS) in the pediatric critical-care context, we examined how clinicians caring for critically ill children with congenital heart disease (CHD) anticipate and perceive the impact of WGS on their decision-making process and treatment recommendations.MethodsWe conducted semistructured in-person and telephone interviews of clinicians involved in the care of critically ill children with CHD at a high-volume pediatric heart center. We qualitatively analyzed the transcribed interviews...
March 1, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Andrés Alonso-García, Felipe Atienza, Pablo Ávila, Clara Ugueto, Miriam Centeno, Reyes Álvarez, Tomás Datino, Esteban González-Torrecilla, Evaristo Castellanos, Gerard Loughlin, Constancio Medrano, Ángel Arenal, Francisco Fernández-Avilés
INTRODUCTION AND OBJECTIVES: Catheter ablation has become the treatment of choice in an increasing number of arrhythmias in children and adolescents. There is still limited evidence of its use at a national level in Spain. The aim was to describe the characteristics and results of a modern monocentric series form a referral tertiary care centre. METHODS: Retrospective register of invasive procedures between 2004 and 2016 performed in patients under 17 years and recorded clinical characteristic, ablation methodology and acute and chronic results of the procedure...
February 23, 2018: Revista Española de Cardiología
Erin K Stenson, Rajesh Punn, Musaab Ramsi, Saraswati Kache
OBJECTIVES: The ability to plot the inferior vena cava (IVC) size on a normal curve for pediatric patients may prove beneficial. First, in patients with normal cardiac anatomy who present in shock, assessing IVC size may be valuable for evaluating the degree of dehydration. Second, in children with heart disease, understanding how a child's IVC size compares to normal could be particularly beneficial for patients with right heart disease. We sought to create normal curves for the IVC and aorta in children younger than 6 years...
February 26, 2018: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
Henry H Cheng, Satish K Rajagopal, Arnold J Sansevere, Erica McDavitt, Daniel Wigmore, Jessica Mecklosky, Kristofer Andren, Kathryn Williams, Amy Danehy, Janet S Soul
BACKGROUND: While therapeutic hypothermia (TH) is an effective neuroprotective therapy for neonatal hypoxic-ischemic encephalopathy, TH has not been demonstrated to improve outcome in other pediatric populations. Patients with acquired or congenital heart disease (CHD) are at high risk of both cardiac arrest and neurodevelopmental impairments, and therapies are needed to improve neurologic outcome. The primary goal of our study was to compare safety/efficacy outcomes in post-arrest CHD patients treated with TH versus controls not treated with TH...
February 21, 2018: Resuscitation
Melissa L Morello, Philip R Khoury, Timothy K Knilans, Gruschen Veldtman, David S Spar, Jeffery B Anderson, Richard J Czosek
OBJECTIVES: Evaluate the efficacy, outcomes and complications associated with DCCV in the treatment of arrhythmias in pediatric and adult CHD populations and identify patient and procedural characteristics associated with adverse events. BACKGROUND: Pediatric and adult patients with congenital heart disease (CHD) are at risk of atrial arrhythmias. Direct current cardioversion (DCCV) is effective but is associated with potential complications. METHODS: In this single-center retrospective series, patients undergoing DCCV Jan 2010-May 2015 were identified and categorized as pediatric (< 18 years) or adult (> 18 years)...
February 24, 2018: Pacing and Clinical Electrophysiology: PACE
Kalpana Thammineni, Jeffrey M Vinocur, Brian Harvey, Jeremiah S Menk, Michael Scott Kelleman, Anna-Maria Korakiti, Amanda S Thomas, James H Moller, James D St Louis, Lazaros K Kochilas
OBJECTIVE: Surgical coronary revascularisation in children with congenital heart disease (CHD) is a rare event for which limited information is available. In this study, we review the indications and outcomes of surgical coronary revascularisation from the Pediatric Cardiac Care Consortium, a large US-based multicentre registry of interventions for CHD. METHODS: This is a retrospective cohort study of children (<18 years old) with CHD who underwent surgical coronary revascularisation between 1982 and 2011...
February 22, 2018: Heart: Official Journal of the British Cardiac Society
Andrew T Trout, Rachel M Sheridan, Suraj D Serai, Stavra A Xanthakos, Weizhe Su, Bin Zhang, Daniel B Wallihan
Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease...
February 22, 2018: Radiology
Wadi Mawad, Luc L Mertens
Cardiac imaging is central to today's pediatric cardiology practice not only to diagnose structural congenital defects and delineate cardiac and extracardiac anatomy but also for determining the hemodynamic impact of the structural defects and acquired pediatric diseases. Not so long ago, clinicians had to heavily rely on angiography as the main cardiac imaging modality to visualize the heart. Particularly, the development of echocardiography in the 1970s and 1980s together with the development of magnetic resonance imaging (MRI) and computed tomography (CT) resulted in a non-invasive diagnostic revolution with diagnostic catheterization becoming obsolete apart for very specific indications...
February 21, 2018: Current Treatment Options in Cardiovascular Medicine
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