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Neonatal cardiology

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https://www.readbyqxmd.com/read/28629661/cornelia-de-lange-syndrome-congenital-heart-disease-in-149-patients
#1
Ariadna Ayerza Casas, Beatriz Puisac Uriol, María Esperanza Teresa Rodrigo, María Hernández Marcos, Feliciano J Ramos Fuentes, Juan Pie Juste
INTRODUCTION: Cornelia de Lange syndrome (CdLS) is produced by mutations in genes that encode regulatory or structural proteins of the cohesin complex. Congenital heart disease (CHD) is not a major criterion of the disease, but it affects many individuals. The objective of this study was to study the incidence and type of CHD in patients with CdLS. MATERIAL AND METHOD: Cardiological findings were evaluated in 149 patients with CdLS and their possible relationship with clinical and genetic variables...
June 16, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28621803/absent-pulmonary-valve-syndrome-diagnosis-associations-and-outcome-in-71-prenatally-diagnosed-cases
#2
Roland Axt-Fliedner, Andrii Kurkevych, Maciej Slodki, Maria Respondek-Liberska, Katarzyna Zych-Krekora, Rüdiger Stressig, Jochen Ritgen, Guiseppe Rizzo, Martin Krapp, Luc de Catte, Gunther Mielke, Stephan Bosselmann, Mathias Meyer-Wittkopf, Andrea Kawecki, Aline Wolter, Marios Mamalis, Christian Enzensberger
OBJECTIVE: To analyze the spectrum of prenatally diagnosed absent pulmonary valve syndrome (APVS) and the outcome from diagnosis onwards. Fetuses with APVS and tetralogy of Fallot (TOF/APVS) and with APVS and intact ventricular septum (APVS/IVS) were included. METHOD: Multicenter retrospective study of the International Prenatal Cardiology Collaboration Group (IPCCG). Clinical and echocardiographic databases of nine referral centers were reviewed from 2012-2016...
June 16, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28595675/is-the-medical-treatment-for-arterial-hypertension-after-primary-aortic-coarctation-repair-related-to-age-at-surgery-a-retrospective-cohort-study
#3
Peter J Lillitos, Mohamed S Nassar, Shane M Tibby, Joy Simmonds, Caner Salih, Conal Austin, David Anderson, Thomas Krasemann
BACKGROUND: Hypertension following primary coarctation repair affects up to a third of subjects. A number of studies suggest that future hypertension risk is reduced if primary repair is performed at a younger age. OBJECTIVES: The objective of this study was to evaluate the risk of future medical treatment for hypertension depending on age of primary coarctation repair. METHODS: This study was carried out at a tertiary paediatric cardiology referral centre...
June 9, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28579060/human-dihydrolipoamide-dehydrogenase-e3-deficiency-novel-insights-into-the-structural-basis-and-molecular-pathomechanism
#4
REVIEW
Attila Ambrus, Vera Adam-Vizi
This review summarizes our present view on the molecular pathogenesis of human (h) E3-deficiency caused by a variety of genetic alterations with a special emphasis on the moonlighting biochemical phenomena related to the affected (dihydro)lipoamide dehydrogenase (LADH, E3, gene: dld), in particular the generation of reactive oxygen species (ROS). E3-deficiency is a rare autosomal recessive genetic disorder frequently presenting with a neonatal onset and premature death; the highest carrier rate of a single pathogenic dld mutation (1:94-1:110) was found among Ashkenazi Jews...
June 2, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28536728/neonatologist-performed-point-of-care-functional-echocardiography-in-the-neonatal-intensive-care-unit
#5
Woei Bing Poon, Keng Yean Wong
Functional echocardiography (fECHO) refers to a bedside, limited assessment of the ductus arteriosus, myocardial performance and pulmonary or systemic haemodynamics that is brief in nature and addresses a specific clinical question or management dilemma. This point-of-care ultrasonography is increasingly used internationally and locally among neonatal units to assist with management of neonatal haemodynamic conditions. This article intends to explain the modality, its indications, interpretation and implications for management, and how it impacts long-term outcomes, particularly in chronic lung disease for premature infants born before 32 weeks of gestation...
May 2017: Singapore Medical Journal
https://www.readbyqxmd.com/read/28490416/development-and-assessment-of-an-e-learning-course-on-pediatric-cardiology-basics
#6
Ana Cristina Oliveira, Sandra Mattos, Miguel Coimbra
BACKGROUND: Early detection of congenital heart disease is a worldwide problem. This is more critical in developing countries, where shortage of professional specialists and structural health care problems are a constant. E-learning has the potential to improve capacity, by overcoming distance barriers and by its ability to adapt to the reduced time of health professionals. OBJECTIVE: The study aimed to develop an e-learning pediatric cardiology basics course and evaluate its pedagogical impact and user satisfaction...
May 10, 2017: JMIR Medical Education
https://www.readbyqxmd.com/read/28436859/in-vivo-intracardiac-vector-velocity-imaging-using-phased-array-transducers-for-pediatric-cardiology
#7
Solveig Fadnes, Morten Wigen, Siri Ann Nyrnes, Lasse Lovstakken
Two-dimensional blood speckle tracking has shown promise for measuring complex flow patterns in neonatal hearts using linear arrays and high-frame-rate plane wave imaging. For general pediatric applications, however, the need for phased array probes emerges due to the limited intercostal acoustic window available. In this work, a clinically approved real-time duplex imaging setup with phased array probes was used to investigate the potential of blood speckle tracking for 2-D vector velocity imaging of children with congenital heart disease...
April 24, 2017: IEEE Transactions on Ultrasonics, Ferroelectrics, and Frequency Control
https://www.readbyqxmd.com/read/28412852/transitional-hemodynamics-in-infants-of-diabetic-mothers-by-targeted-neonatal-echocardiography-electrocardiography-and-peripheral-flow-study
#8
Ingrid Anne Mandy Schierz, Giuseppa Pinello, Ettore Piro, Mario Giuffrè, Simona La Placa, Giovanni Corsello
OBJECTIVE: Metabolic alterations of intrauterine environment in diabetes mellitus (DM) lead to fetal cardiac dysfunctions that can persist after birth. The aim of the study was to assess the cardiovascular adaptation in infants born to diabetic mothers (IDM) with different degrees of glycometabolic control, in relation to revised guidelines for diagnosis of DM and quality improvements in neonatal care. MATERIALS AND METHODS: An observational case-control study was conducted on IDM with gestational, type 1 and type 2 DM...
May 5, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28289528/clinical-cardiac-regenerative-studies-in-children
#9
REVIEW
Imre J Pavo, Ina Michel-Behnke
Although the incidence of pediatric heart failure is low, the mortality is relatively high, with severe clinical symptoms requiring repeated hospitalization or intensive care treatment in the surviving patients. Cardiac biopsy specimens have revealed a higher number of resident human cardiac progenitor cells, with greater proliferation and differentiation capacity, in the neonatal period as compared with adults, demonstrating the regeneration potential of the young heart, with rising interest in cardiac regeneration therapy in critically ill pediatric patients...
February 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28271625/clinical-characteristics-of-patients-from-the-worldwide-registry-on-peripartum-cardiomyopathy-ppcm-eurobservational-research-programme-in-conjunction-with-the-heart-failure-association-of-the-european-society-of-cardiology-study-group-on-ppcm
#10
Karen Sliwa, Alexandre Mebazaa, Denise Hilfiker-Kleiner, Mark C Petrie, Aldo P Maggioni, Cecile Laroche, Vera Regitz-Zagrosek, Maria Schaufelberger, Luigi Tavazzi, Peter van der Meer, Jolien W Roos-Hesselink, Petar Seferovic, Karin van Spandonck-Zwarts, Amam Mbakwem, Michael Böhm, Frederic Mouquet, Burkert Pieske, Roger Hall, Piotre Ponikowski, Johann Bauersachs
AIMS: The purpose of this study is to describe disease presentation, co-morbidities, diagnosis and initial therapeutic management of patients with peripartum cardiomyopathy (PPCM) living in countries belonging to the European Society of Cardiology (ESC) vs. non-ESC countries. METHODS AND RESULTS: Out of 500 patients with PPCM entered by 31 March 2016, we report on data of the first 411 patients with completed case record forms (from 43 countries) entered into this ongoing registry...
March 8, 2017: European Journal of Heart Failure
https://www.readbyqxmd.com/read/28255743/the-role-and-limitations-of-cochrane-reviews-at-the-bedside-a%C3%A2-systematic-synopsis-of-five-pediatric-subspecialties
#11
Sascha Meyer, Martin Poryo, Sara Khosrawikatoli, Yvonne Goda, Michael Zemlin
BACKGROUND: Cochrane meta-analyses provide the physician at the bedside with the most relevant, up-to-date clinical information. However, implementation of evidence-based medicine (EBM) at the bedside may be difficult for a variety of reasons. The aim of this study was to assess relevant issues and obstacles related to implementing EBM in pediatrics in real life at the bedside/cotside. METHODS: We performed five systematic literature reviews of all published Cochrane reviews in neonatology (1996-2010), pediatric neurology (1996-2010), pediatric gastroenterology (1993-2012), pediatric cardiology (2001-2015), and complementary and alternative medicine (1996-2012; CAM) in children and neonates...
March 2, 2017: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28239752/potential-and-limitations-of-cochrane-reviews-in-pediatric-cardiology-a-systematic-analysis
#12
Martin Poryo, Sara Khosrawikatoli, Hashim Abdul-Khaliq, Sascha Meyer
Evidence-based medicine has contributed substantially to the quality of medical care in pediatric and adult cardiology. However, our impression from the bedside is that a substantial number of Cochrane reviews generate inconclusive data that are of limited clinical benefit. We performed a systematic synopsis of Cochrane reviews published between 2001 and 2015 in the field of pediatric cardiology. Main outcome parameters were the number and percentage of conclusive, partly conclusive, and inconclusive reviews as well as their recommendations and their development over three a priori defined intervals...
April 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28192391/cmos-nanoelectrode-array-for-all-electrical-intracellular-electrophysiological-imaging
#13
Jeffrey Abbott, Tianyang Ye, Ling Qin, Marsela Jorgolli, Rona S Gertner, Donhee Ham, Hongkun Park
Developing a new tool capable of high-precision electrophysiological recording of a large network of electrogenic cells has long been an outstanding challenge in neurobiology and cardiology. Here, we combine nanoscale intracellular electrodes with complementary metal-oxide-semiconductor (CMOS) integrated circuits to realize a high-fidelity all-electrical electrophysiological imager for parallel intracellular recording at the network level. Our CMOS nanoelectrode array has 1,024 recording/stimulation 'pixels' equipped with vertical nanoelectrodes, and can simultaneously record intracellular membrane potentials from hundreds of connected in vitro neonatal rat ventricular cardiomyocytes...
May 2017: Nature Nanotechnology
https://www.readbyqxmd.com/read/28153478/perfusion-index-and-pulse-oximetry-screening-for-congenital-heart-defects
#14
MULTICENTER STUDY
Federico Schena, Irene Picciolli, Massimo Agosti, Antonio Alberto Zuppa, Gianvincenzo Zuccotti, Luciana Parola, Giulia Pomero, Giorgio Stival, Markus Markart, Silvia Graziani, Luigi Gagliardi, Cristina Bellan, Simona La Placa, Giuseppe Limoli, Gabriella Calzetti, Andrea Guala, Enza Bonello, Fabio Mosca
OBJECTIVE: To evaluate the efficacy of combined pulse oximetry (POX) and perfusion index (PI) neonatal screening for severe congenital heart defects (sCHD) and assess different impacts of screening in tertiary and nontertiary hospitals. STUDY DESIGN: A multicenter, prospective study in 10 tertiary and 6 nontertiary maternity hospitals. A total of 42 169 asymptomatic newborns from among 50 244 neonates were screened; exclusion criteria were antenatal sCHD diagnosis, postnatal clinically suspected sCHD, and neonatal intensive care unit admission...
April 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/27989374/functional-quality-of-life-and-neurodevelopmental-outcomes-after-congenital-cardiac-surgery
#15
REVIEW
Megan L Ringle, Gil Wernovsky
Throughout the past few decades, advances in cardiology, neonatal intensive care, and surgical techniques have resulted in a growing cohort of thriving school-aged children with previously lethal complex congenital heart diseases. While survival has increased, there remains significant morbidity following repair including neurodevelopmental sequelae. Compared to children with a structurally normal heart, these infants and children have a higher frequency of abnormalities in tone, feeding, and delayed developmental milestones, as well as challenges with speech and learning disabilities, while a higher proportion of adolescents suffer from problems with processing speed, executive function, and a unique set of medical hardships related to exercise intolerance and obesity, medication burden, and mental health comorbidities...
December 2016: Seminars in Perinatology
https://www.readbyqxmd.com/read/27917566/perioperative-hypothermia-in-neonatal-intensive-care-unit-patients-effectiveness-of-a-thermoregulation-intervention-and-associated-risk-factors
#16
Branden M Engorn, Stephanie L Kahntroff, Karen M Frank, Sarabdeep Singh, Helen A Harvey, Charles T Barkulis, Annika M Barnett, Olamide O Olambiwonnu, Eugenie S Heitmiller, Robert S Greenberg
BACKGROUND: Hypothermia in neonatal intensive care unit patients is associated with morbidity. Perioperative normothermia is the standard of care. AIMS: We hypothesized that a quality improvement intervention (transport protocol, transport education, ongoing monitoring) would decrease the incidence of perioperative hypothermia. Secondarily, we hypothesized that patients undergoing surgery at a postmenstrual age of <37 weeks or at a weight of <1.5 kg would be at higher risk for perioperative hypothermia...
February 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/27869423/-progressive-moderate-mitral-regurgitation-in-a-children-with-axenfeld-rieger-syndrome-the-importance-of-cardiologic-follow-up
#17
Francisco Sánchez Ferrer, María D Grima Murcia
Axenfeld-Rieger syndrome is a congenital disease with an estimated prevalence of one in 200,000 individuals. This is an ophthalmic disorder related to anterior segment dysgenesis, which may be present from the neonatal period. It is associated with extraocular affectations such as cranial dimorphism, maxillofacial or dental anomalies. Cardiological or pituitary manifestations are less common. The congenital heart disease in Axenfeld-Rieger syndrome has been described in very few cases in the literature. We report a 7-year-old patient with Axenfeld-Rieger syndrome and mild mitral insufficiency since the age of 3 years, which is progressing to moderate mitral regurgitation at the present time...
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27821886/microcephaly-in-north-east-brazil-a-retrospective-study-on-neonates-born-between-2012-and-2015
#18
Juliana Sousa Soares de Araújo, Cláudio Teixeira Regis, Renata Grigório Silva Gomes, Thiago Ribeiro Tavares, Cícera Rocha Dos Santos, Patrícia Melo Assunção, Renata Valéria Nóbrega, Diana de Fátima Alves Pinto, Bruno Vinícius Dantas Bezerra, Sandra da Silva Mattos
OBJECTIVE: To assess the number of children born with microcephaly in the State of Paraíba, north-east Brazil. METHODS: We contacted 21 maternity centres belonging to a paediatric cardiology network, with access to information regarding more than 100 000 neonates born between 1 January 2012 and 31 December 2015. For 10% of these neonates, nurses were requested to retrieve head circumference measurements data from delivery-room books. We used three separate criteria to classify whether a neonate had microcephaly: (i) the Brazilian Ministry of Health proposed criterion: term neonates (gestational age ≥ 37 weeks) with a head circumference of less than 32 cm; (ii) Fenton curves: neonates with a head circumference of less than -3 standard deviation for age and gender; or (iii) the proportionality criterion: neonates with a head circumference of less than ((height/2))+10) ± 2...
November 1, 2016: Bulletin of the World Health Organization
https://www.readbyqxmd.com/read/27788933/risk-of-aortic-dissection-in-pregnant-patients-with-the-marfan-syndrome
#19
Rafael Kuperstein, Tal Cahan, Rakefet Yoeli-Ullman, Sagit Ben Zekry, Amihay Shinfeld, Michal J Simchen
Patients with Marfan syndrome (MS) face a high risk of aortic dissection during pregnancy. A dilated aortic root (>40 to 45 mm) is considered a relative contraindication for pregnancy. We investigated the risk for aortic dissection and pregnancy outcome in patients with MS. Women with MS who attended our cardiology high-risk pregnancy clinic from 2006 to 2015 were followed clinically and with serial echocardiograms by a multidisciplinary team. Beta blockers were offered and titrated by blood pressure and heart rate...
January 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/27749513/the-utility-of-brain-natriuretic-peptide-in-pediatric-cardiology-a-review
#20
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
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