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Critical congenital heart disease

Ashley Gionfriddo, Mika L Nonoyama, Peter C Laussen, Peter N Cox, Megan Clarke, Alejandro A Floh
OBJECTIVES: To promote standardization, the Centers for Disease Control and Prevention introduced a new ventilator-associated pneumonia classification, which was modified for pediatrics (pediatric ventilator-associated pneumonia according to proposed criteria [PVAP]). We evaluated the frequency of PVAP in a cohort of children diagnosed with ventilator-associated pneumonia according to traditional criteria and compared their strength of association with clinically relevant outcomes. DESIGN: Retrospective cohort study...
March 15, 2018: Pediatric Critical Care Medicine
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
Hui Huang, Georg Kuenze, Jarrod A Smith, Keenan C Taylor, Amanda M Duran, Arina Hadziselimovic, Jens Meiler, Carlos G Vanoye, Alfred L George, Charles R Sanders
Mutations that induce loss of function (LOF) or dysfunction of the human KCNQ1 channel are responsible for susceptibility to a life-threatening heart rhythm disorder, the congenital long QT syndrome (LQTS). Hundreds of KCNQ1 mutations have been identified, but the molecular mechanisms responsible for impaired function are poorly understood. We investigated the impact of 51 KCNQ1 variants with mutations located within the voltage sensor domain (VSD), with an emphasis on elucidating effects on cell surface expression, protein folding, and structure...
March 2018: Science Advances
Silvia G V Alvarez, Angela McBrien
The ductus arteriosus (DA) is a crucial part of the fetal circulation, both in the normal fetus and in critical congenital heart disease (CHD). It allows shunting between the pulmonary and systemic circulations. In physiological prenatal conditions, the DA lets the majority of right ventricular output bypass the fluid-filled, high-resistance lungs. The DA can cause hemodynamic compromise in the fetus and neonate when constricted or absent (in isolation or in patients with CHD) and may lead to pre- or postnatal sequelae within other systems when forming part of a vascular ring...
March 7, 2018: Seminars in Fetal & Neonatal Medicine
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
Yuka Matsunaga, Masataka Ishimura, Hazumu Nagata, Kiyoshi Uike, Tadamune Kinjo, Masayuki Ochiai, Kenichiro Yamamura, Hidetoshi Takada, Yoshihisa Tanoue, Masaki Hayakawa, Masanori Matsumoto, Toshiro Hara, Shouichi Ohga
BACKGROUND: Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle...
February 7, 2018: Pediatrics and Neonatology
Erica Hasten, Donna M McDonald-McGinn, Terrence B Crowley, Elaine Zackai, Beverly S Emanuel, Bernice E Morrow, Silvia E Racedo
Non-allelic homologous recombination events on chromosome 22q11.2 during meiosis can result in either the deletion (22q11.2DS) or duplication (22q11.2DupS) syndrome. Although the spectrum and frequency of congenital heart disease (CHD) are known for 22q11.2DS, there is less known for 22q11.2DupS. We now evaluated cardiac phenotypes in 235 subjects with 22q11.2DupS including 102 subjects we collected and 133 subjects that were previously reported as a confirmation and found 25% have CHD, mostly affecting the cardiac outflow tract (OFT)...
March 2, 2018: Human Molecular Genetics
Wolf B Kratzert, Eva K Boyd, Johanna C Schwarzenberger
Survival of adults with congenital heart disease (CHD) has improved significantly over the last 2 decades, leading to an increase in hospital and intensive care unit (ICU) admissions of these patients. Whereas most of the ICU admissions in the past were related to perioperative management, the incidence of medical emergencies from long-term sequelae of palliative or corrective surgical treatment of these patients is rising. Intensivists now are confronted with patients who not only have complex anatomy after congenital cardiac surgery, but also complex pathophysiology due to decades of living with abnormal cardiac anatomy and diseases of advanced age...
November 16, 2017: Journal of Cardiothoracic and Vascular Anesthesia
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
Michael Narvey, Kenny K Wong, Anne Fournier
Pulse oximetry screening is safe, noninvasive, easy to perform and proven to enhance detection of critical congenital heart disease in newborns. However, this test has yet to be adopted as routine practice in Canada. The present practice point highlights essential details and recommendations for screening, which research has shown to be highly specific, with low false-positive rates. Optimal screening for critical congenital heart disease should include prenatal ultrasound, physical examination and pulse oximetry screening...
November 2017: Paediatrics & Child Health
Marnie Lightfoot, Philip Hough, Alan Hudak, Michelle Gordon, Sarah Barker, Robert Meeder, Melanie Colpitts, Gwendolyn Roberts, William Gary Smith
Objectives: To assess the efficacy of a new screening protocol for critical congenital heart disease (CCHD). Background: In March 2014, the Ontario Provincial Council for Maternal Child Health (PCMCH) recommended screening for CCHD, utilizing pulse oximetry to measure oxygen saturation as part of the newborn examination. However, this is yet to be implemented in all hospitals. Method: An audit of consecutive healthy normal newborn patients in a secondary level centre in Ontario with early adoption of the screening recommendation over a 1-year period was undertaken...
September 2017: Paediatrics & Child Health
Marine Flechet, Fabian Güiza, Dirk Vlasselaers, Lars Desmet, Stoffel Lamote, Heidi Delrue, Marc Beckers, Michaël P Casaer, Pieter Wouters, Greet van den Berghe, Geert Meyfroidt
OBJECTIVES: To assess whether near-infrared cerebral tissue oxygen saturation, measured with the FORESIGHT cerebral oximeter (CAS Medical Systems, Branford, CT) predicts PICU length of stay, duration of invasive mechanical ventilation, and mortality in critically ill children after pediatric cardiac surgery. DESIGN: Single-center prospective, observational study. SETTING: Twelve-bed PICU of a tertiary academic hospital. PATIENTS: Critically ill children and infants with congenital heart disease, younger than 12 years old, admitted to the PICU between October 2012 and November 2015...
February 19, 2018: Pediatric Critical Care Medicine
Rose Y Hardy, Michelle Gurvitz, Jamie L Jackson, Susan May, Paula Miller, Rachel Daskalov, Elyse Foster
No abstract text is available yet for this article.
February 16, 2018: Journal of American College Health: J of ACH
Emily M Bucholz, Lynn A Sleeper, Jane W Newburger
BACKGROUND: Children with single ventricle heart disease require frequent interventions and follow-up. Low socioeconomic status (SES) may limit access to high-quality care and place these children at risk for poor long-term outcomes. METHODS AND RESULTS: Data from the SVR (Pediatric Heart Network Single Ventricle Reconstruction Trial Public Use) data set were used to examine the relationship of US neighborhood SES with 30-day and 1-year mortality or cardiac transplantation and length of stay among neonates undergoing the Norwood procedure (n=525)...
February 2, 2018: Journal of the American Heart Association
Isabel E Mawson, Pratusha L Babu, John M Simpson, Grenville F Fox
A retrospective review of admission preductal oxygen saturations of neonates with antenatally diagnosed critical congenital heart disease (CCHD) was performed to investigate the differences in newborn pulse oximetry (Pulsox) by specific CCHD diagnosis. Saturations were recorded at median of < 1 h (range < 1-9 h) after delivery. Data was stratified by CCHD diagnosis and analysed according to the three different admission Pulsox thresholds, ≤ 90, ≤ 92 and ≤ 95%. Of the 276 neonates studied, 208 were clinically well at admission, with no co-morbidities, gestation > 34 weeks and birth weight > 1...
February 5, 2018: European Journal of Pediatrics
Bishnu Rath Giri, Ram Hari Chapagain, Samana Sharma, Sandeep Shrestha, Sunita Ghimire, P Ravi Shankar
BACKGROUND: Earthquakes impact child health in many ways. Diseases occurring immediately following an earthquake have been studied in field based hospitals but studies on the inpatient disease pattern among children without trauma in a permanent hospital setup is lacking. METHODS: We examined the diagnoses of all children without trauma, admitted to Kanti Children's Hospital, Kathmandu for fifteen-week duration (from 4th week to end of the 18th week) following the 7...
February 5, 2018: BMC Pediatrics
Michael S Paranka, Jeffrey M Brown, Robert D White, Matthew V Park, Amy S Kelleher, Reese H Clark
OBJECTIVES: The objectives were to determine the frequency with which pulse oximetry identifies critical congenital heart defects in asymptomatic full-term and late preterm newborns using the AAP expert panel algorithm in a variety of different hospital settings and to evaluate the impact of altitude on the rate of positive screens. METHODS: We conducted a prospective clinical study of implementation of a newborn pulse oximetry screening for congenital heart disease in 34 independent hospitals...
January 29, 2018: Journal of Perinatology: Official Journal of the California Perinatal Association
A Beningfield, A Jones
BACKGROUND: Chest physiotherapy (CPT) is implemented before and after congenital heart disease (CHD) surgery in paediatrics to prevent and treat postoperative pulmonary complications (PPC). Currently, there are no systematic reviews or meta-analyses on the efficacy of CPT in this population. OBJECTIVE: To conduct a systematic review and meta-analysis to determine whether peri-operative CPT is safe and effective for paediatric patients with CHD. DATA SOURCES: A literature search was conducted on PEDro, MEDLINE, CINAHL, Informit, The Cochrane Library and Scopus in March and April 2016...
December 5, 2017: Physiotherapy
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