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Critical congenital heart disease

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-)
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; 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...
September 30, 2016: Pediatric Critical Care Medicine
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
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
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
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
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
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
Kathryn Browning Carmo, Tracey Lutz, Andrew Berry, Martin Kluckow, Nick Evans
AIM: To determine the role of clinician performed ultrasound (CPU) during the retrieval and transport of critically ill term and near term newborns. METHODS: A neonatologist with portable ultrasound accompanied a sample of newborn retrievals to perform cardiac and cerebral ultrasound before and after transportation. RESULTS: A total of fifty-five babies were studied. Median birthweight: 3350 g (2220-5030 g). CPU led to a change in the planned receiving hospital in ten babies...
September 15, 2016: Acta Paediatrica
Rebecca C Ahrens-Nicklas, Shama Khan, Jennifer Garbarini, Stacy Woyciechowski, Lisa D'Alessandro, Elaine H Zackai, Matthew A Deardorff, Elizabeth Goldmuntz
Congenital heart defects (CHDs) are heterogeneous and present with a spectrum of severity, with roughly 25% of patients requiring intervention before age 1. The etiology of disease is unknown in many individuals; however, there is a rapidly expanding understanding of genetic risk factors that may contribute to pathogenesis. Through this work, we sought to evaluate the diagnostic yield of a clinical genetics evaluation and associated genetic testing among infants with critical CHDs. Furthermore, we aimed to both determine the utility of microarray and establish a strong baseline that can be used in future studies of the impact of exome sequencing in this population...
September 8, 2016: American Journal of Medical Genetics. Part A
Lisa A Hom, Gerard R Martin
Congenital heart disease (CCHD) is the most common birth defect. Screening for the most critical forms (CCHD) using pulse oximetry was added to the Recommended Uniform Screening Panel in the United States in 2011. Since then, CCHD screening has become nearly universal in the United States. Nurses are ideally situated to contribute to the development of best practices for implementation and provide education to families on CCHD screening. Much of the standardization, advocacy, and development of national recommendations occurred with key input from nurses...
September 2016: American Journal of Perinatology
Óscar Cano, Ana Andrés, Pau Alonso, Joaquín Osca, María-José Sancho-Tello, Joaquín Rueda, Ana Osa, Luis Martínez-Dolz
The prevalence of adults with congenital heart disease has dramatically increased during the last decades due to significant advances in the surgical correction of these conditions. As a result, patient's survival has been prolonged and arrhythmias have become one of the principal causes of morbidity and mortality for these patients. The surface 12-lead ECG may play a critical role in the identification of the underlying heart disease of the patient, the recognition of the arrhythmia mechanism and may also help in the planification of the ablation procedure in this setting...
August 21, 2016: Journal of Electrocardiology
K K Miller, K S Vig, E M Goetz, G Spicer, A J Yang, J S Hokanson
OBJECTIVE: This study evaluated pulse oximetry screening (POS) for critical congenital heart disease (CCHD) in planned out of hospital births with special attention to births in Plain communities (Amish, Mennonite and similar). STUDY DESIGN: Wisconsin out of hospital births in 2013 and 2014 were evaluated. Care providers were supplied with and trained in the use of pulse oximeters for CCHD screening. State records were reviewed to identify deaths and hospital admissions due to CCHD in this population...
September 1, 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
Rachael M Edwards, Gautham P Reddy, Gregory Kicska
Functional single ventricle (FSV) encompasses a spectrum of severe congenital heart disease. Patients with FSV are living longer than decades prior resulting in more frequent imaging both for surgical planning and functional evaluation. At each stage of surgical intervention, imaging plays a critical role in detecting postoperative complications and preprocedural planning. This article describes the unique imaging findings, including complications, that are most important to the referring physician or surgeon at each surgical stage of FSV management...
August 6, 2016: Clinical Imaging
Dave T Gerrard, Andrew A Berry, Rachel E Jennings, Karen Piper Hanley, Nicoletta Bobola, Neil A Hanley
Human organogenesis is when severe developmental abnormalities commonly originate. However, understanding this critical embryonic phase has relied upon inference from patient phenotypes and assumptions from in vitro stem cell models and non-human vertebrates. We report an integrated transcriptomic atlas of human organogenesis. By lineage-guided principal components analysis, we uncover novel relatedness of particular developmental genes across different organs and tissues and identified unique transcriptional codes which correctly predicted the cause of many congenital disorders...
2016: ELife
Jennifer C DeMichele, Nikhil Vajaria, Hongyue Wang, Dawn M Sweeney, Karen S Powers, Jill M Cholette
STUDY OBJECTIVE: To determine the incidence of postoperative airway complications in infants <5kg in weight undergoing cardiac surgery intubated with Microcuff (Kimberley-Clark, Roswell, GA) endotracheal tubes (ETTs). DESIGN: Retrospective review of infants weighing <5.0 kg with congenital heart disease (CHD) presenting for cardiac surgery. SETTING: Single-center, tertiary pediatric cardiac critical care unit at a university hospital...
September 2016: Journal of Clinical Anesthesia
J C Galán Gutiérrez, F E Fernández Suárez, P Miranda García, L A Sopena Zubiria
Eisenmenger syndrome (ES) is a complex combination of cardiovascular abnormalities defined as pulmonary hypertension with investment or bidirectional flow through an intracardiac or aortopulmonary communication, usually secondary to a congenital heart disease not resolved promptly. It carries a significant risk of perioperative mortality, with an incidence close to 30% for non-cardiac surgery. We report the anaesthetic management in a ES patient undergoing breast surgery, which was successfully performed under general anaesthesia combined with thoracic analgesic blocks...
August 20, 2016: Revista Española de Anestesiología y Reanimación
Xiao-Jing Hu, Qu-Ming Zhao, Xiao-Jing Ma, Wei-Li Yan, Xiao-Ling Ge, Bing Jia, Fang Liu, Lin Wu, Ming Ye, Guo-Ying Huang
AIM: Limited data have been available regarding critical congenital heart disease (CHD) screening in neonatal intensive care unit (NICUs). This study evaluated the feasibility of screening for CHD by adding pulse oximetry (POX) to clinical evaluation in a NICU in Shanghai, China. METHODS: We screened 4128 eligible consecutive NICU admissions using POX plus clinical evaluation. Infants with positive screening results were then evaluated with echocardiography. Those with negative screening results were put under observation, and they also underwent echocardiography if their oxygen saturation fell below 95% on room air during hospitalisation...
November 2016: Acta Paediatrica
Abdul Qader Tahir Ismail, Matt Cawsey, Andrew K Ewer
The concept of using pulse oximetry (PO) as a screening test to identify newborn babies with critical congenital heart defects (CCHD) before life-threatening collapse occurs has been debated for some time now. Several recent large studies have consistently shown that PO screening adds value to existing screening techniques with over 90% of CCHDs detected. It can also help identify newborn babies with low oxygen saturations due to infection, respiratory disease and non-critical CCHD. Many countries have now introduced PO screening as routine practice, and as screening gains more widespread acceptance in the UK, we have focused more on the practical aspects of screening in this article...
August 16, 2016: Archives of Disease in Childhood. Education and Practice Edition
David Faraoni, Daniel Vo, Viviane G Nasr, James A DiNardo
BACKGROUND: Children with major and severe congenital heart disease (CHD) undergoing noncardiac surgery are at increased risk of mortality. The objective of this study was to identify the predictors for in-hospital mortality, and to develop a risk stratification score that could be used to help decision making and the development of perioperative management guidelines. METHODS: We included all children with major (eg, tetralogy of Fallot with wide open pulmonary insufficiency, hypoplastic left heart syndrome including stage 1 repair) or severe CHD (eg, children with uncorrected CHD, children with documented pulmonary hypertension, children with ventricular dysfunction requiring medications, or children listed for heart transplant) recorded in the 2012 and 2013 American College of Surgeons National Surgical Quality Improvement Program Pediatric databases in a derivation cohort, and those recorded in the 2014 database in a validation cohort...
October 2016: Anesthesia and Analgesia
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