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Critical Congenital heart

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https://www.readbyqxmd.com/read/27901294/performance-of-functional-fibrinogen-thromboelastography-in-children-undergoing-congenital-heart-surgery
#1
Nischal K Gautam, Chunyan Cai, Olga Pawelek, Muhammad B Rafique, Davide Cattano, Evan G Pivalizza
BACKGROUND: Functional Fibrinogen assay of the Thromboelastography (FFTEG), a whole blood viscoelastic hemostatic assay, has been used to estimate fibrinogen levels in adult patients undergoing major surgery but its performance in pediatric patients undergoing cardiac surgery requires evaluation. In this study, we evaluate the correlation between FFTEG parameters and standard laboratory tests for fibrinogen and platelet counts before and after cardiopulmonary bypass in children undergoing repair for congenital heart disease...
November 30, 2016: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/27899284/defective-lymphatic-valve-development-and-chylothorax-in-mice-with-a-lymphatic-specific-deletion-of-connexin43
#2
Stephanie J Munger, Michael J Davis, Alexander M Simon
Lymphatic valves (LVs) are cusped luminal structures that permit the movement of lymph in only one direction and are therefore critical for proper lymphatic vessel function. Congenital valve aplasia or agenesis can, in some cases, be a direct cause of lymphatic disease. Knowledge about the molecular mechanisms operating during the development and maintenance of LVs may thus aid in the establishment of novel therapeutic approaches to treat lymphatic disorders. In this study, we examined the role of Connexin43 (Cx43), a gap junction protein expressed in lymphatic endothelial cells (LECs), during valve development...
November 26, 2016: Developmental Biology
https://www.readbyqxmd.com/read/27849656/the-use-of-nesiritide-in-children-with-congenital-heart-disease
#3
Ronald A Bronicki, Michele Domico, Paul A Checchia, Curtis E Kennedy, Ayse Akcan-Arikan
OBJECTIVE: We evaluated the use of nesiritide in children with critical congenital heart disease, pulmonary congestion, and inadequate urine output despite conventional diuretic therapy. DESIGN: We conducted a retrospective analysis of 26 consecutive patients, comprising 37 infusions occurring during separate hospitalizations. Hemodynamic variables, urine output, and serum creatinine levels were monitored prior to and throughout the duration of therapy with nesiritide...
November 15, 2016: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/27834763/acquired-von-willebrand-syndrome-an-under-recognized-cause-of-major-bleeding-in-the-cardiac-intensive-care-unit
#4
Melissa B Jones, Karthik Ramakrishnan, Fahad A Alfares, Kendal M Endicott, Gary Oldenburg, John T Berger, Venkat Shankar, Dilip S Nath, Yaser A Diab
BACKGROUND: Acquired von Willebrand syndrome (AvWS) in the setting of congenital heart disease is an under-recognized cause of bleeding in the pediatric cardiac critical care unit. METHODS: Fourteen patients diagnosed with AvWS admitted to the cardiac intensive care unit at the Children's National Health System between December 2009 and September 2015 were identified with subsequent chart review and case analysis. RESULTS: Of the 14 patients included in this study, 4 patients were on ventricular-assist devices, 6 patients were on extracorporeal membrane oxygenation, and 4 were patients with congenital heart disease not receiving any mechanical circulatory support...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27831548/a-novel-more-efficient-staged-approach-for-critical-congenital-heart-disease-screening
#5
J Mouledoux, S Guerra, J Ballweg, Y Li, W Walsh
OBJECTIVE: Screening for critical congenital heart disease (CCHD) using pulse oximetry has been endorsed by the American Academy of Pediatrics and the American Heart Association. The recommended screening requires two saturation readings. We sought to determine the incidence of undetected CCHD in Tennessee for the 2 years following implementation of an algorithm that assigned an immediate pass to a single lower extremity saturation of 97% or higher. STUDY DESIGN: State Genetic Screening records and reports of missed cases from the Tennessee Initiative for Perinatal Quality Care were used to determine if CCHD cases were missed by the new screening algorithm...
November 10, 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/27808268/identification-of-candidate-genes-for-congenital-heart-defects-on-proximal-chromosome-8p
#6
Tingting Li, Chunjie Liu, Yuejuan Xu, Qianqian Guo, Sun Chen, Kun Sun, Rang Xu
With the application of advanced molecular cytogenetic techniques, the number of patients identified as having abnormal chromosome 8p has increased progressively. Individuals with terminal 8p deletion have been extensively described in previous studies. The manifestations usually include cardiac anomalies, developmental delay/mental retardation, craniofacial abnormalities, and multiple other minor anomalies. However, some patients with proximal deletion also presented with similar phenotypic features. Here we describe a female child with an 18...
November 3, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27807201/molecular-pathophysiology-of-congenital-long-qt-syndrome
#7
REVIEW
M S Bohnen, G Peng, S H Robey, C Terrenoire, V Iyer, K J Sampson, R S Kass
Ion channels represent the molecular entities that give rise to the cardiac action potential, the fundamental cellular electrical event in the heart. The concerted function of these channels leads to normal cyclical excitation and resultant contraction of cardiac muscle. Research into cardiac ion channel regulation and mutations that underlie disease pathogenesis has greatly enhanced our knowledge of the causes and clinical management of cardiac arrhythmia. Here we review the molecular determinants, pathogenesis, and pharmacology of congenital Long QT Syndrome...
January 2017: Physiological Reviews
https://www.readbyqxmd.com/read/27801690/five-decades-of-pediatric-heart-transplantation-challenges-overcome-challenges-remaining
#8
Steven Zangwill
PURPOSE OF REVIEW: Pediatric heart transplants continue to be the therapy of choice for children with end stage heart failure. The interplay of limited donor supply, improvement in ventricular assist device (VAD) technology and utilization, and a focus on optimizing long-term outcomes make it critically important for practitioners to be aware of an evolving diagnostic and therapeutic arsenal. RECENT FINDINGS: Data suitable to define best practices for pediatric heart transplantation consist of an amalgam of small single center series, registry reviews and judicious inference from adult studies...
January 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/27774972/balloon-angioplasty-of-severe-aortic-coarctation-in-a-newborn-using-axillary-artery-access-the-first-experience-in-turkey
#9
Kemal Nişli, Serra Karaca, Ümrah Aydoğan
Axillary artery access for demanding interventions in newborns is rarely described, in spite of the many clear advantages. This access route in newborns with critical congenital heart disease is a real alternative to the more commonly used femoral or carotid artery routes. In brief, axillary access is an attractive alternative approach in newborns with complex heart diseases. It is advisable to stop an elective procedure if axillary access is not successful. Presently described is the use of axillary artery access for balloon angioplasty in a newborn with critical aortic coarctation...
October 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/27756857/genetic-analysis-of-rare-coding-mutations-in-celsr1-3-in-chinese-congenital-heart-and-neural-tube-defects
#10
Xiaojing Qiao, Yahui Liu, Peiqiang Li, Zhongzhong Chen, Huili Li, Xueyan Yang, Richard H Finnell, Zhangmin Yang, Ting Zhang, Bin Qiao, Yufang Zheng, Hongyan Wang
The planar cell polarity (PCP) pathway is critical for proper embryonic development of the neural tube and heart. Mutations in these genes have previously been implicated in the pathogenesis of neural tube defects (NTDs), but not in congenital heart defects (CHDs) in humans. We systematically identified the mutation patterns of CELSR1-3 , one family of the core PCP genes, in human cohorts composed of 352 NTD cases, 412 CHD cases, and matched controls. A total of 72 disease-specific rare novel coding mutations were identified, of which 37 were identified in CHD cases, and 36 were identified in NTD patients...
October 18, 2016: Clinical Science (1979-)
https://www.readbyqxmd.com/read/27749513/the-utility-of-brain-natriuretic-peptide-in-pediatric-cardiology-a-review
#11
Ana L Neves, Tiago Henriques-Coelho, Adelino Leite-Moreira, José C Areias
OBJECTIVE: The aim of this article is to evaluate the clinical utility of brain natriuretic peptide in pediatric patients, examining the diagnostic value, management, and prognostic relevance, by critical assessment of the literature. DATA SOURCES: In December 2015, a literature search was performed (PubMed access to MEDLINE citations; http://www.ncbi.nlm.nih.gov/PubMed/) and included these Medical Subject Headings and text terms for the key words: "brain natriuretic peptide," "amino-terminal pro-brain natriuretic peptide," "children," "neonate/s," "newborn/s," "infant/s," and "echocardiography...
November 2016: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/27745924/best-practice-critical-cardiac-care-in-the-neonatal-unit
#12
Michael L Rigby
Major congenital or acquired heart disease in neonates presents with cyanosis, hypoxia, acute circulatory failure or cardiogenic shock. Antenatal diagnosis is made in up to 50% but heart disease is unanticipated in the remainder. The presence of significant heart disease in premature infants is also frequently not suspected at first; in general, whatever the underling cardiac anomaly, the clinical condition is worse, deteriorates more quickly and carries a poorer prognosis in premature and low birth weight infants...
October 13, 2016: Early Human Development
https://www.readbyqxmd.com/read/27739239/critical-congenital-heart-defects-and-abnormal-levels-of-routinely-collected-first-and-second-trimester-biomarkers
#13
Melissa Borelli, Rebecca J Baer, Christina D Chambers, Tyler C Smith, Laura L Jelliffe-Pawlowski
We examined the association between maternal characteristics, routinely collected first- and second-trimester biomarkers and the risk of having an infant with a critical congenital heart defect (CCHD). Included were women who participated in the California Prenatal Screening Program who had nuchal translucency (NT) measurement and first- and second-trimester serum screening. All pregnancies ended in a live birth of an infant without aneuploidy or a neural tube defect. Poisson regression analyses were used to estimate the relative risk and 95% confidence interval of a CCHD by maternal characteristics, first- and second-trimester serum biomarkers or NT measurements...
October 14, 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27720432/multidetector-computed-tomographic-angiography-imaging-of-congenital-pulmonary-venous-anomalies-a-pictorial-review
#14
Aysel Türkvatan, Alper Güzeltaş, Hasan Tahsin Tola, Yakup Ergül
Congenital pulmonary venous anomalies are not uncommon that can occur either in isolation or in association with different forms of congenital heart disease. Clinical presentation of these anomalies may vary from the relatively benign single anomalous partial pulmonary venous return to life-threatening critical obstructed total anomalous pulmonary venous return. Accurate delineation of these anomalies and accompanied cardiovascular anomalies are crucial to guide decision making in these patients. Low-dose high-pitch dual-source 256-detector multidetector computed tomographic angiography is a fast and reliable imaging modality allowing comprehensive noninvasive anatomic imaging in neonates and children with congenital pulmonary venous anomalies with lower radiation doses and should be preferred for these patients after transthoracic echocardiography...
October 6, 2016: Canadian Association of Radiologists Journal, Journal L'Association Canadienne des Radiologistes
https://www.readbyqxmd.com/read/27716711/critical-decision-of-operability-in-congenital-heart-disease-patient-with-severe-pulmonary-hypertension
#15
Raja Joshi, Rajat Kalra, Neeraj Kumar, Neeraj Aggarwal, Reena K Joshi, Mridul Aggarwal, Rakesh Pandey
Repair of congenital heart disease in the presence of high pulmonary pressure has always been a contentious issue. Pulmonary vascular resistance (PVR) is considered important for establishing operability in these patients. However, PVR estimation is not always accurate and cannot solely be relied upon to make critical decision of operability. Clinical examination, chest X-ray, and echocardiography are also important indicators of pulmonary vascular disease. Knowledge of pits and falls of each investigation is important for appropriate management in these patients...
October 2016: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/27716696/early-enteral-nutrition-therapy-in-congenital-cardiac-repair-postoperatively-a-randomized-controlled-pilot-study
#16
Manoj Kumar Sahu, Anuradha Singal, Ramesh Menon, Sarvesh Pal Singh, Alka Mohan, Mala Manral, Divya Singh, V Devagouru, Sachin Talwar, Shiv Kumar Choudhary
BACKGROUND AND OBJECTIVES: Adequate nutritional supplementation in infants with cardiac malformations after surgical repair is a challenge. Critically ill infants in the early postoperative period are in a catabolic stress. The mismatch between estimated energy requirement (EER) and the intake in the postoperative period is multifactorial, predisposing them to complications such as immune deficiency, more infection, and growth failure. This study aimed to assess the feasibility and efficacy of enriched breast milk feed on postoperative recovery and growth of infants after open heart surgery...
October 2016: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/27703660/dual-source-computed-tomography-evaluation-of-children-with-congenital-pulmonary-valve-stenosis
#17
Zhanguo Sun, Wenjian Xu, Shuran Huang, Yueqin Chen, Xiang Guo, Zhitao Shi
BACKGROUND: Despite dual-source computed tomography (DSCT) technology has been performed well on adults or infants with heart disease, specific knowledge about children with congenital pulmonary valve stenosis (PS) remained to be established. OBJECTIVES: This original research aimed to establish a professional approach of DSCT performing technology on children and to assess the image quality performed by DSCT to establish a diagnostic evaluation for children with PS...
April 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
https://www.readbyqxmd.com/read/27688672/predictors-of-cardio-pulmonary-resuscitation-outcome-in-postoperative-cardiac-children
#18
Bana Agha Nasser, Julinar Idris, Abdu Rahman Mesned, Tageldein Mohamad, Mohamed S Kabbani, Ali Alakfash
BACKGROUND: Outcomes of cardiopulmonary resuscitation (CPR) in children with congenital heart disease have improved and many children have survived after an in-hospital cardiac arrest. AIM: The purpose of this study is to determine predictors of poor outcome after CPR in critical children undergoing cardiac surgery. METHODS: We conducted a retrospective chart review and data analysis of all CPR records and charts of all postoperative cardiac children who had a cardiac arrest and required resuscitation from 2011 until 2015...
October 2016: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/27685638/human-exposure-to-environmental-contaminants-and-congenital-anomalies-a-critical-review
#19
Warren G Foster, Jane A Evans, Julian Little, Laura Arbour, Aideen Moore, Reg Sauve, Juan Andrés León, Wei Luo
Congenital anomalies are an important cause of infant mortality and disability. Developmental exposure to environmental contaminants is thought to increase the risk for congenital anomalies. Herein, we describe a critical review of the literature conducted between February and March 2014 yielding 3057 references from which 97 unique relevant articles published from 2003 through 2014 were evaluated. Common congenital anomalies including hypospadias, cryptorchidism, anogenital distance (AGD), congenital heart defects and oral clefts were well represented in the literature whereas other outcomes such as neural tube defects, limb deficiency defects and gastroschisis were rarely described...
August 11, 2016: Critical Reviews in Toxicology
https://www.readbyqxmd.com/read/27684424/photoplethysmographic-waveform-characteristics-of-newborns-with-coarctation-of-the-aorta
#20
L Palmeri, G Gradwohl, M Nitzan, E Hoffman, Y Adar, Y Shapir, R Koppel
OBJECTIVE: Fetal echocardiography, physical examination and pulse oximetry detect only half of coarctation of aorta (CoA) cases. We aimed to quantify delayed arrival and diminished amplitude of lower extremity photoplethysmographic (PPG) pulses relative to the right hand in affected patients. STUDY DESIGN: We studied 8 CoA infants and 32 healthy controls. The pulse arrival time difference between foot and hand (f-hTD) and pulse amplitude ratio (F/H) were measured on PPG signal waveforms by digitally-determining maxima and minima of systolic decrease of light transmission...
September 29, 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
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