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Congenital Heart AND Infant* AND Mortal*

Iman Manavitehrani, Pegah Ebrahimi, Irene Yang, Sean Daly, Aaron Schindeler, Akshat Saxena, David G Little, David F Fletcher, Fariba Dehghani, David S Winlaw
Despite advances in modern surgery, congenital heart disease remains a medical challenge and major cause of infant mortality. Valved conduits are routinely used to surgically correct blood flow in hearts with congenital malformations by connecting the right ventricle to the pulmonary artery (RV-PA). This review explores the current range of RV-PA conduits and describes their strengths and disadvantages. Homografts and xenografts are currently the primary treatment modalities, however both graft types have limited biocompatibility and durability, and present a disease transmission risk...
February 14, 2019: Cardiovascular Engineering and Technology
Kerry M Dorr, Frank L Conlon
Congenital malformations, or structural birth defects, are now the leading cause of infant mortality in the United States and Europe (Dolk et al., 2010; Heron et al., 2009). Of the congenital malformations, congenital heart disease (CHD) is the most common (Dolk et al., 2010; Heron et al., 2009). Thus, a molecular understanding of heart development is an essential goal for improving clinical approaches to CHD. However, CHDs are commonly a result of genetic defects that manifest themselves in a spatial and temporal manner during the early stages of embryogenesis, leaving them mostly intractable to mass spectrometry-based analysis...
January 31, 2019: Current Opinion in Chemical Biology
Indranil Sarkar, Adriana Zardini Buzatto, Ravendra Garg, Liang Li, Sylvia van Drunen Littel-van den Hurk
Respiratory syncytial virus (RSV) is a significant cause of mortality and morbidity in infants, elderly, immunocompromised individuals and patients with congenital heart diseases. Despite extensive efforts, a vaccine against RSV is still not available. We have previously reported the development of a subunit vaccine (ΔF/TriAdj) composed by a truncated version of the fusion protein (ΔF) and a polymer-based combination adjuvant (TriAdj). In the present study, we compared inflammatory responses of ΔF/TriAdj-vaccinated and unvaccinated mice following intranasal challenge with RSV...
February 1, 2019: Journal of Proteome Research
Meagan E Kaulfus, Helena Gardiner, S Shahrukh Hashmi, Hector Mendez-Figueroa, Victoria J Miller, Blair Stevens, Rebecca Carter
Trisomy 18 is an autosomal trisomy condition characterized by minor to major birth defects, severe disabilities, and high rates of pre- and postnatal mortality. Interventions for these infants have traditionally been withheld with focus instead on palliative support. The issues and attitudes surrounding corrective surgery of congenital heart defects, which is a birth defect that occurs in approximately 90% of infants with trisomy 18, is of our study's interest as recent literature has indicated that cardiac surgery is being performed and may lead to improved survival compared to palliative care...
January 28, 2019: Journal of Genetic Counseling
Xing Chen, Wu Zhou, Wanjun Luo, Xia Wang, Xiaolu Deng, Xuliang Chen, Qinghua Hu, Yingji Chen, Lingjin Huang
To observe the results of different treatment of congenital heart disease (CHD) complicated with severe pneumonia in infants.
 Methods: A total of 27 infants with CHD and severe pneumonia were admitted to the Department of Pediatrics, Xiangya Hospital from January 2014 to December 2014, of whom 18 were male and 9 were female. The average age was 2.0-19.0(5.9±4.3) months, with an average body weight of 3.3-10.0 (5.8±1.8) kg. Infants were treated with a strategy of complete control of the lung infection before surgery (internal medicine group)...
November 28, 2018: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
Danielle S Burstein, Pirouz Shamszad, Dingwei Dai, Christopher S Almond, Jack F Price, Kimberly Y Lin, Matthew J O'Connor, Robert E Shaddy, Christopher E Mascio, Joseph W Rossano
BACKGROUND: Children with congenital heart disease (CHD) are at risk for advanced heart failure (AHF). We sought to define the mortality and resource utilization in CHD-related AHF in children and young adults. METHODS: All hospitalizations in the Pediatric Health Information System database involving patients ≤21 years old with a CHD diagnosis and heart failure requiring at least 7 days of continuous inotropic support between 2004 and 2015 were included. Hospitalizations including CHD surgery were excluded...
December 5, 2018: American Heart Journal
J M Bubberman, A van Zoonen, J L M Bruggink, M van der Heide, R M F Berger, A F Bos, E M W Kooi, J B F Hulscher
BACKGROUND: Necrotizing enterocolitis (NEC) predominantly occurs in preterm infants (PT-NEC). In term neonates, NEC occurs more frequently when a congenital heart disease is present (CHDNEC). Our aim was to evaluate differences and similarities in disease characteristics of PT-NEC versus CHD-NEC. METHODS: In this retrospective case-control study we identified all CHD infants who developed NEC Bell's stage ≥2 in our center from 2004 to 2014. We randomly selected (1:2 ratio) PT-NEC infants from the same period...
December 13, 2018: Journal of Pediatric Surgery
Mana Taweevisit, Paul Thorner
INTRODUCTION: Congenital heart defects (CHDs) carry significant morbidity and mortality in pediatric patients. This study determined the spectrum of CHDs based on fetal and pediatric autopsies. METHODS: Autopsy reports over a 15-year period were reviewed. Postmortem findings were correlated with echocardiography records. RESULTS: From 608 autopsies, 119 cases with CHDs were identified (11% of fetal, 53% of neonatal, 18% of infant, and 4.5% of childhood autopsies)...
January 11, 2019: Fetal and Pediatric Pathology
Asimina Tsintoni, Gabriel Dimitriou, Ageliki A Karatza
INTRODUCTION: Congenital heart disease is one of the most of the groups of congenital anomalies with an incidence of about 1 per 100 live births. Almost one-third of these infants require some type of intervention, usually in the first year of life and increasingly often in the neonatal period. Innovative reparative and palliative surgical procedures and advanced medical support in the Neonatal Intensive Care Unit have significantly reduced the mortality related to congenital heart disease...
January 4, 2019: Journal of Maternal-fetal & Neonatal Medicine
Peter Murin, Wolfgang Boettcher, Shinichi Ozaki, Alexa Wloch, Mi-Young Cho, Mathias Redlin, Oliver Miera, Nicodème Sinzobahamvya, Joachim Photiadis
BACKGROUND:  We routinely start cardiopulmonary bypass (CPB) for pediatric congenital heart surgery without homologous blood, due to circuit miniaturization, and blood-saving measures. Blood transfusion is applied if hemoglobin concentration falls under 8 g/dL, or it is postponed to after coming off bypass or after operation. How this strategy impacts on postoperative mortality and morbidity, in infants weighing ≤ 7 kg? METHODS:  Six-hundred fifteen open-heart procedures performed from January 2014 to June 2018 were selected...
January 2, 2019: Thoracic and Cardiovascular Surgeon
Jamie N Watson, Nikki J Seagraves
Cardiac malformations (CVMs) are a leading cause of infant morbidity and mortality. CVMs are particularly prevalent when the developing fetus is exposed to high levels of phenylalanine in-utero in mothers with Phenylketonuria. Yet, elucidating the underlying molecular mechanism leading to CVMs has proven difficult. In this study we used RNA-Seq to investigate an avian model of MPKU and establish differential gene expression (DEG) characteristics of the early developmental stages HH10, 12, and 14. In total, we identified 633 significantly differentially expressed genes across stages HH10, 12, and 14...
September 6, 2018: Molecular Genetics and Metabolism
Anastasios C Polimenakos, Lauren Mathis, Brent Shafer, M Vinayak Kamath
Use of temporary epicardial pacing (TEP) leads remains a routine perioperative strategy in congenital heart surgery. Selective use of TEP in neonates and infants undergoing cardiac intervention within the first 6 months of life has, yet, to be assessed. Outcome analysis was undertaken. From August 2014 to December 2016, 112 consecutive neonates and infants underwent cardiac intervention within the first 6 months of life. Patients with STS/EACTS Congenital Heart Surgery Mortality (STAT categories) 1-5 were prospectively followed from the index cardiac operation until hospital discharge and included in the study...
December 18, 2018: Pediatric Cardiology
Ulf Kessler, Eva-Maria Hau, Marcin Kordasz, Stephanie Haefeli, Catherine Tsai, Peter Klimek, Dietmar Cholewa, Mathias Nelle, Mladen Pavlovic, Steffen Berger
Background: Studies on the influence of congenital heart disease (CHD) on neonates with necrotizing enterocolitis (NEC) have produced varied results. We therefore examined the influence of CHD on NEC outcomes. Methods: We carried out a retrospective single-center study including infants with confirmed NEC, treated between 2004 and 2017. We excluded patients with isolated patent ductus arteriosus or pulmonary hypertension ( n = 45) and compared outcomes of patients with hemodynamically relevant CHD ( n = 38) and those without CHD ( n = 91)...
2018: Frontiers in Pediatrics
Antonia Mantakaki, Adegbenro Omotuyi John Fakoya, Fatemeh Sharifpanah
Congenital heart disease (CHD) affects a considerable number of children and adults worldwide. This implicates not only developmental disorders, high mortality, and reduced quality of life but also, high costs for the healthcare systems. CHD refers to a variety of heart and vascular malformations which could be very challenging to reconstruct the malformed region surgically, especially when the patient is an infant or a child. Advanced technology and research have offered a better mechanistic insight on the impact of CHD in the heart and vascular system of infants, children, and adults and identified potential therapeutic solutions...
2018: PeerJ
Shabnam Peyvandi, Rebecca J Baer, Anita J Moon-Grady, Scott P Oltman, Christina D Chambers, Mary E Norton, Satish Rajagopal, Kelli K Ryckman, Laura L Jelliffe-Pawlowski, Martina A Steurer
Background Racial/ethnic and socioeconomic disparities exist in outcomes for children with congenital heart disease. We sought to determine the influence of race/ethnicity and mediating socioeconomic factors on 1-year outcomes for live-born infants with hypoplastic left heart syndrome and dextro-Transposition of the great arteries. Methods and Results The authors performed a population-based cohort study using the California Office of Statewide Health Planning and Development database. Live-born infants without chromosomal anomalies were included...
October 16, 2018: Journal of the American Heart Association
Martina A Steurer, Rebecca J Baer, Edmund Burke, Shabnam Peyvandi, Scott Oltman, Christina D Chambers, Mary E Norton, Larry Rand, Satish Rajagopal, Kelli K Ryckman, Sky K Feuer, Liang Liang, Randi A Paynter, Molly McCarthy, Anita J Moon-Grady, Roberta L Keller, Laura L Jelliffe-Pawlowski
Background Infants with critical congenital heart disease ( CCHD ) are more likely to be small for gestational age (GA). It is unclear how this affects mortality. The authors investigated the effect of birth weight Z score on 1-year mortality separately in preterm (GA <37 weeks), early-term (GA 37-38 weeks), and full-term (GA 39-42 weeks) infants with CCHD . Methods and Results Live-born infants with CCHD and GA 22 to 42 weeks born in California 2007-2012 were included in the analysis. The primary predictor was Z score for birth weight and the primary outcome was 1-year mortality...
September 4, 2018: Journal of the American Heart Association
Frederic R Lyimo, Pedro Pallangyo, Naizihijwa Majani, Theophylly L Mushi, Sulende Kubhoja
BACKGROUND: Congenital cardiac defects are not rare among neonates. Prompt assessment for life-threatening anomalies is essential for rapid management decisions and positive outcomes. Extracardiac anomalies can occur in congenital heart defects, and their presence increases morbidity and mortality in these neonates. CASE PRESENTATION: We report a case of a 31- month-old infant black girl in Tanzania who presented with an on-and-off history of difficulty in breathing, easy fatigability, facial and lower-limb swelling, recurrent respiratory tract infections, and failure to thrive...
October 24, 2018: Journal of Medical Case Reports
Ilona C Narayen, Arjan B Te Pas, Nico A Blom, M Elske van den Akker-van Marle
Pulse oximetry (PO) screening is used to screen newborns for critical congenital heart defects (CCHD). Analyses performed in hospital settings suggest that PO screening is cost-effective. We assessed the costs and cost-effectiveness of PO screening in the Dutch perinatal care setting, with home births and early postnatal discharge, compared to a situation without PO screening. Data from a prospective accuracy study with 23,959 infants in the Netherlands were combined with a time and motion study and supplemented data...
January 2019: European Journal of Pediatrics
Shahzad Alam, Akunuri Shalini, Rajesh G Hegde, Rufaida Mazahir, Akanksha Jain
Objective: The objective of the current study was to evaluate the timing of first extubation and compare the outcome of patient extubated early with others; we also evaluated the predictors of early extubation in our cohort. Materials and Methods: This prospective cohort study included children <1 year of age undergoing surgery for congenital heart disease. Timing of first extubation was noted, and patients were dichotomized in the group taking 6 h after completion of surgery as cutoff for early extubation...
October 2018: Annals of Cardiac Anaesthesia
Samantha C Butler, Anjali Sadhwani, Christian Stopp, Jayne Singer, David Wypij, Carolyn Dunbar-Masterson, Janice Ware, Jane W Newburger
OBJECTIVE: Mortality rates for children with congenital heart disease (CHD) have significantly declined, resulting in a growing population with associated neurodevelopmental disabilities. American Heart Association guidelines recommend systematic developmental screening for children with CHD. The present study describes results of inpatient newborn neurodevelopmental assessment of infants after open heart surgery. OUTCOME MEASURES: We evaluated the neurodevelopment of a convenience sample of high-risk infants following cardiac surgery but before hospital discharge using an adaptation of the Newborn Behavioral Observation...
October 15, 2018: Congenital Heart Disease
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