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Neonatal palliative

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https://www.readbyqxmd.com/read/28981194/elevated-preoperative-von-willebrand-factor-is-associated-with-perioperative-thrombosis-in-infants-and-neonates-with-congenital-heart-disease
#1
Ryan Hunt, Corey M Hoffman, Sirisha Emani, Cameron C Trenor, Sitaram M Emani, David Faraoni, Chava Kimchi-Sarfaty, Juan C Ibla
BACKGROUND: The surgical repair of congenital heart lesions is frequently complicated by perioperative thrombosis of unclear etiology. An imbalance between von Willebrand Factor (VWF) and ADAMTS-13 is an emerging variable in thrombosis. OBJECTIVES: To describe perioperative changes to VWF, ADAMTS-13 and NETosis and evaluate clinical and biochemical associations with postoperative thrombosis. METHODS: Neonates and infants undergoing palliation or definitive repair of congenital heart disease (CHD) were recruited (n =133)...
October 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28977325/analysis-of-death-and-palliative-care-in-a-neonatal-intensive-care-unit
#2
Ligia Marçola, Silvia Maria Macedo de Barbosa, Ivete Zoboli, Rita Tiziana Verardo Polastrini, Maria Esther Jurfest Ceccon
OBJECTIVE: To characterize cases of children admitted to the Neonatal Intensive Care Unit of a tertiary university hospital who died in the period ranging from January 01, 2012 to July 31, 2014, and who required palliative care and/or were subjected to it. METHODS: A retrospective descriptive study was carried out by reviewing the medical records of these patients to collect data and to perform descriptive statistical analysis. RESULTS: During the study period, 49 children died after at least 48 hours from the time of admission...
April 2017: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
https://www.readbyqxmd.com/read/28954835/retrospective-uk-multicentre-study-of-the-pregnancy-outcomes-of-women-with-a-fontan-repair
#3
Matthew Cauldwell, Philip J Steer, Samantha Bonner, Omar Asghar, Lorna Swan, Kenneth Hodson, Catherine E G Head, Adam Daniel Jakes, Nicola Walker, Margaret Simpson, Aidan P Bolger, Farah Siddiqui, Katherine M English, Lucy Maudlin, Dilip Abraham, Andrew J Sands, Aarthi R Mohan, Stephanie L Curtis, Louise Coats, Mark R Johnson
BACKGROUND: The population of women of childbearing age palliated with a Fontan repair is increasing. The aim of this study was to describe the progress of pregnancy and its outcome in a cohort of patients with a Fontan circulation in the UK. METHODS: A retrospective study of women with a Fontan circulation delivering between January 2005 and November 2016 in 10 specialist adult congenital heart disease centres in the UK. RESULTS: 50 women had 124 pregnancies, resulting in 68 (54...
September 27, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28916237/ethics-and-palliative-care-in-the-perinatal-world
#4
REVIEW
Colleen M Marty, Brian S Carter
The perinatal world is unique in its dutiful consideration of two patients along the lines of decision-making and clinical management - the fetus and the pregnant woman. The potentiality of the fetus-newborn is intertwined with the absolute considerations for the woman as autonomous patient. From prenatal diagnostics, which may be quite extensive, to potential interventions prenatally, postnatal resuscitation, and neonatal management, the fetus and newborn may be anticipated to survive with or without special needs and technology, to have a questionable or guarded prognosis, or to live only minutes to hours...
September 13, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28915527/palliative-therapies-for-congenital-heart-disease-with-ductus-dependent-pulmonary-circulation
#5
Shi-Min Yuan
In neonates, the management of ductus dependent pulmonary circulation is challenging. There have been three palliative therapies for this lesion: the modified Blalock-Taussig shunt, ductal stenting and prostaglandin E infusions, for maintaining the ductal patency before definite operations. Debates remain with regard to the indications and disadvantages of three palliative therapies. The aim of this article is to give a brief review of these three palliative therapies.
September 15, 2017: Zeitschrift Für Geburtshilfe und Neonatologie
https://www.readbyqxmd.com/read/28901225/promoting-pulmonary-arterial-growth-via-right-ventricle-to-pulmonary-artery-connection-in-children-with-pulmonary-atresia-ventricular-septal-defect-and-hypoplastic-pulmonary-arteries
#6
Edon J Rabinowitz, Shilpi Epstein, Nina Kohn, David B Meyer
BACKGROUND: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation...
September 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28889833/acute-kidney-injury-following-first-stage-palliation-in-hypoplastic-left-heart-syndrome-hybrid-versus-norwood-palliation
#7
Richard U Garcia, Girija Natarajan, Henry L Walters, Ralph E Delius, Sanjeev Aggarwal
OBJECTIVE: The aim of this study was to evaluate the prevalence of acute kidney injury after first-stage surgical palliation in patients with a single ventricle and to explore associated risk factors and outcomes. Design and patients This single-centre retrospective study included neonates who underwent either Norwood or Hybrid procedure from 2008 to 2015 for a single ventricle. Postoperative acute kidney injury was defined using the paediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), criteria within 72 hours of the procedure...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28889828/atrial-septal-defect-morphology-and-stenting-in-hypoplastic-left-heart-syndrome-after-hybrid-palliation
#8
Lilia Oreto, Giuseppe Mandraffino, Lucia Manuri, Michele B Saitta, Salvatore Agati, Concetta Zito, Fiore S Iorio, Scipione Carerj, Paolo Guccione
Aims The aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect. Methods and results From 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28889816/first-stage-palliation-strategy-for-univentricular-heart-disease-may-impact-risk-for-acute-kidney-injury
#9
Bryan H Goldstein, Stuart L Goldstein, Prasad Devarajan, Farhan Zafar, David M Kwiatkowski, Bradley S Marino, David L S Morales, Catherine D Krawczeski, David S Cooper
OBJECTIVE: Norwood palliation for patients with single ventricle heart disease is associated with a significant risk for acute kidney injury, which portends a worse prognosis. We sought to investigate the impact of hybrid stage I palliation (Hybrid) on acute kidney injury risk. DESIGN: This study is a single-centre prospective case-control study of seven consecutive neonates with single ventricle undergoing Hybrid palliation. Levels of serum creatinine and four novel urinary biomarkers, namely neutrophil gelatinase-associated lipocalin, interleukin-18, liver fatty acid-binding protein, and kidney injury molecule-1, were obtained before and after palliation...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28889811/cardiovascular-ct-for-evaluation-of-single-ventricle-heart-disease-risks-and-accuracy-compared-with-interventional-findings
#10
B Kelly Han, Marnie Huntley, David Overman, Dawn Witt, David Dassenko, Ross F Garberich, John R Lesser
OBJECTIVE: We sought to evaluate the risk and image quality from cardiovascular CT in patients across all stages of single-ventricle palliation, and to define accuracy by comparing findings with intervention and surgery. METHODS: Consecutive CT scans performed in patients with single-ventricle heart disease were retrospectively reviewed at a single institution. Diagnosis, sedation needs, estimated radiation dose, and adverse events were recorded. Anatomical findings, image quality (1-4, 1=optimal), and discrepancy compared with interventional findings were determined...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28874025/contemporary-results-of-aortic-valve-repair-for-congenital-disease-lessons-for-management-and-staged-strategy
#11
Mathieu Vergnat, Boulos Asfour, Claudia Arenz, Philipp Suchowerskyj, Benjamin Bierbach, Ehrenfried Schindler, Martin Schneider, Victor Hraska
OBJECTIVES: Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease. METHODS: From 2003 to 2015, 193 consecutive patients were included...
September 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28838527/aortic-dissection-after-the-hybrid-stage-1-procedure-diagnosis-and-management-strategy
#12
Scott Hittinger, Varsha Bandisode, Shahryar Chowdhury, Minoo N Kavarana
Hybrid Norwood palliation is gaining acceptance as an alternative to open Norwood palliation in high-risk neonates with hypoplastic left heart syndrome. Aortic dissection after hybrid Norwood palliation is a rare but ominous complication that has not been reported.
September 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28807643/-use-of-subcutaneous-route-for-comfort-care%C3%A2-in-neonatal-palliative-population-systematic-review-and-survey-of-practices-in-france
#13
N Pouvreau, J Tandonnet, O Tandonnet, L Renesme
INTRODUCTION: Subcutaneous hydration (hypodermoclysis) and drug administration is a widely used method of analgesic therapy in adult palliative care medicine. Very little is known about its use in neonatal medicine. Evidence-based guidelines do not exist due to a lack of data. In this study, the advantages of subcutaneous analgesic therapy in terms of comfort in neonatal palliative care situations were investigated. METHODS: This report details the results of a systematic review associated with a survey in neonatal intensive care units (NICUs) and pediatric palliative care departments (PPCTs) in France...
September 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28802248/utility-of-rapid-whole-exome-sequencing-in-the-diagnosis-of-neonatal-niemann-pick-disease-type-c-presenting-with-fetal-hydrops-and-liver-failure
#14
Mersedeh Rohanizadegan, Sarah El-Almery, Anne O'Donnell-Luria, Ivana Mihalek, Peggy Chen, Marilyn Sanders, Kristen Leeman, Megan Cho, Christina Hung, Olaf Bodamer
Rapid whole exome sequencing (rWES) is increasingly used in critically ill newborn infants to inform about diagnosis, clinical management and prognosis. Here we report a male newborn infant with hydrops, pancytopenia and acute liver failure who was listed for liver transplantation. Given the acuity of the presentation, the procedure related morbidity and mortality and lack of diagnosis we employed rWES in the proband and both parents with a turn-around time of 10 business days. rWES returned one maternally inherited, likely pathogenic and one paternally inherited, likely pathogenic variant in NPC1 suggestive of a diagnosis of Niemann Pick disease type C (NPC)...
August 11, 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/28790278/-perioperative-care-for-patients-with-hypoplastic-left-heart-syndrome
#15
Yujiro Ide, Kisaburo Sakamoto
Hypoplastic Left Heart Syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. In normal risk cohort, its surgical outcome has been improved dramatically since Dr. William Norwood reported the 1st successful case more than 30 years ago. The introduction of routine bilateral pulmonary artery banding with subsequent Norwood operation during early infantile( we call it as "rapid 2 stage Norwood operation") has brought an earlier hemodynamic stabilization after Norwood operation...
July 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28783102/the-pulmonary-circulation-in-the-single-ventricle-patient
#16
REVIEW
Amanda Hauck, Nicolas Porta, Steven Lestrud, Stuart Berger
In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in 'single ventricle' physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many cases into adulthood, despite having a single functional ventricular pumping chamber supplying both the pulmonary and systemic circulation. Our growing understanding of the functionally univentricular heart has resulted in freedom from Fontan failure of >50% at 25 years post-Fontan...
August 7, 2017: Children
https://www.readbyqxmd.com/read/28777689/the-role-of-pediatric-palliative-care-in-complex-congenital-heart-disease-three-illustrative-cases
#17
Rachna May, Jess Thompson
BACKGROUND: Although the role of pediatric palliative care (PPC) is well described in oncology, neonatal, and pediatric intensive care patients, the involvement of PPC in patients with congenital heart disease (CHD) is not well explored. CHD is a leading cause of neonatal morbidity and can cause ongoing morbidity throughout the course of a child's life. PPC, with its focus on quality of life and longitudinal care through the course of an illness, could be of benefit to this population...
August 4, 2017: Journal of Palliative Medicine
https://www.readbyqxmd.com/read/28758538/-you-can-only-give-warmth-to-your-baby-when-it-s-too-late-parents-bonding-with-their-extremely-preterm-and-dying-child
#18
Andrea Abraham, Manya J Hendriks
This study on end-of-life decisions in extremely preterm babies shows that the parents under study experience a multitude of stressors due to the immediate separation after birth, the alienating setting of the neonatal intensive care unit (NICU), the physical distance to the child, medical uncertainties, and upcoming decisions. Even though they are considered to be parents (assigned parenthood), they cannot act as primary caregivers. Instead, they depend on professional instructions for access and care. Embodied parenthood can be experienced only at the end-of-life, that is, during the dying trajectory and after the child's death...
July 1, 2017: Qualitative Health Research
https://www.readbyqxmd.com/read/28736841/outcomes-of-blalock-taussig-shunts-in-current-era-a-single-center-experience
#19
Navaneetha Sasikumar, Antony Hermuzi, Chun-Po Steve Fan, Kyong-Jin Lee, Rajiv Chaturvedi, Edward Hickey, Osami Honjo, Glen S Van Arsdell, Christopher A Caldarone, Arnav Agarwal, Lee Benson
OBJECTIVES: Mortality associated with the modified Blalock-Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity. DESIGN: A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox's proportional hazard regression...
July 24, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28711967/fate-of-duct-dependent-discontinuous-pulmonary-arteries-after-arterial-duct-stenting
#20
Giuseppe Santoro, Giovanbattista Capozzi, Mario Giordano, Gianpiero Gaio, Maria Teresa Palladino, Carola Iacono, Heba Talat Mahmoud, Maria Giovanna Russo
Significant and balanced PA growth following arterial duct (AD) stenting has already been consistently reported in literature. However, to date, no data are available about the role of this approach as palliation of congenital heart disease with a duct-dependent discontinuous pulmonary artery (dPA). The aim of this study was to evaluate the fate of a dPA of ductal origin following trans-catheter AD stabilization. Angiographic PA evaluation was performed in seven patients submitted to neonatal AD stenting as palliative recruitment of dPA...
July 15, 2017: Pediatric Cardiology
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