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"Hypoplastic left heart"

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https://www.readbyqxmd.com/read/28072476/aberrant-right-subclavian-artery-correlation-between-fetal-and-neonatal-abnormalities-and-abnormal-genetic-screening-or-testing
#1
Angela C Ranzini, Francine Hyman, Emily Jamaer, Tim van Mieghem
OBJECTIVES: To determine whether fetuses with an isolated aberrant course of the right subclavian artery (ARSA) have increased risk for chromosomal abnormalities, including trisomy 21 or 22q11 deletion. METHODS: We performed a retrospective chart review of all fetuses with antenatally diagnosed ARSA. Data were collected from fetal anatomic surveys, fetal echocardiograms, noninvasive trisomy 21 screening programs, invasive genetic studies, and neonatal records. RESULTS: Seventy-nine fetuses with ARSA were identified at 20...
January 10, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28043677/pressure-volume-loop-derived-cardiac-indices-during-dobutamine-stress-a-step-towards-understanding-limitations-in-cardiac-output-in-children-with-hypoplastic-left-heart-syndrome
#2
James Wong, Kuberan Pushparajah, Adelaide de Vecchi, Bram Ruijsink, Gerald F Greil, Tarique Hussain, Reza Razavi
BACKGROUND: Children with a single systemic right ventricle, such as in hypoplastic left heart syndrome (HLHS), frequently experience reduced exercise capacity. Elucidating the causes could help with optimising treatment strategies. METHODS: Prospective data from 10 consecutive symptomatic patients with HLHS undergoing clinical cardiac magnetic resonance with catheterisation (XMR) were analysed. Mean age 8.6years (range 3.5-11.6years), mean time since Fontan completion 5...
December 22, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28033074/preoperative-feeding-neonates-with-cardiac-disease
#3
Carly J Scahill, Eric M Graham, Andrew M Atz, Scott M Bradley, Minoo N Kavarana, Sinai C Zyblewski
BACKGROUND: The potential for necrotizing enterocolitis (NEC) in neonates requiring cardiac surgery has contributed largely to wide feeding practice variations and a hesitation to initiate enteral feeding during the preoperative period, specifically those patients with hypoplastic left heart syndrome. METHODS: A retrospective chart review of neonates undergoing cardiac surgery at a single institution between July 2011 and July 2013 was performed. The primary objective of this study was to determine if preoperative feeding was associated with NEC in neonates requiring cardiac surgery...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28024650/recoarctation-after-norwood-i-procedure-for-hypoplastic-left-heart-syndrome-impact-of-patch-material
#4
Keti Vitanova, Julie Cleuziou, Jelena Pabst von Ohain, Melchior Burri, Andreas Eicken, Rüdiger Lange
BACKGROUND: The development of recoarctation after the Norwood I procedure is a known complication in patients with hypoplastic left heart syndrome (HLHS). METHODS: We reviewed data on 194 consecutive patients with HLHS who underwent the Norwood I procedure between 2000 and 2015. All patients who survived until the second stage of the procedure were included. Recoarctation was defined by the need for intervention, either catheter based or surgical. The aim of our study was to determine if the patch material used for the initial arch reconstruction had an influence on the development of recoarctation...
December 23, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28018895/regional-differences-in-end-diastolic-volumes-between-3d-echo-and-cmr-in-hlhs-patients
#5
Alberto Gomez, Ozan Oktay, Daniel Rueckert, Graeme P Penney, Julia A Schnabel, John M Simpson, Kuberan Pushparajah
Ultrasound is commonly thought to underestimate ventricular volumes compared to magnetic resonance imaging (MRI), although the reason for this and the spatial distribution of the volume difference is not well understood. In this paper, we use landmark-based image registration to spatially align MRI and ultrasound images from patients with hypoplastic left heart syndrome and carry out a qualitative and quantitative spatial comparison of manual segmentations of the ventricular volume obtained from the respective modalities...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28013288/structural-cerebral-abnormalities-and-neurodevelopmental-status-in-single-ventricle-congenital-heart-disease-before-fontan-procedure
#6
Walter Knirsch, Kristina Nadine Mayer, Ianina Scheer, Ruth Tuura, Dietmar Schranz, Andreas Hahn, Kristina Wetterling, Ingrid Beck, Beatrice Latal, Bettina Reich
OBJECTIVES: Neonates with single ventricle congenital heart disease are at risk for structural cerebral abnormalities. Little is known about the further evolution of cerebral abnormalities until Fontan procedure. METHODS: Between August 2012 and July 2015, we conducted a prospective cross-sectional two centre study using cerebral magnetic resonance imaging (MRI) and neuro-developmental outcome assessed by the Bayley-III. Forty-seven children (31 male) were evaluated at a mean age of 25...
December 23, 2016: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28012642/postoperative-cerebral-and-somatic-near-infrared-spectroscopy-saturations-and-outcome-in-hypoplastic-left-heart-syndrome
#7
George M Hoffman, Nancy S Ghanayem, John P Scott, James S Tweddell, Michael E Mitchell, Kathleen A Mussato
BACKGROUND: Circulatory vulnerability reflected by low systemic venous oxygen saturation after surgical palliation of hypoplastic left heart syndrome predicts adverse neurologic outcome and reduced survival, and targeting venous saturation may improve outcome. We herein test the hypothesis that near-infrared spectroscopy (NIRS)-derived cerebral and somatic/renal regional saturations can predict survival. METHODS: Patient data, from a prospective Institutional Review Board-approved registry of hemodynamic measures after initial palliation of hypoplastic left heart syndrome, were analyzed with logistic and multivariable mixed regression methods to determine relationships between standard hemodynamic measures, direct and NIRS measures of saturation, and outcome...
December 21, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28007262/off-pump-atrial-septectomy-for-infants-with-restrictive-atrial-septal-defect
#8
Tai Fuchigami, Masahiko Nishioka, Toru Akashige, Shotaro Higa, Nobuhiro Nagata
The management of some subsets of patients with an unsuccessful balloon atrial septostomy (BAS) and poor general condition is typically challenging. Our novel technique involves off-pump atrial septectomy using laparoscopic forceps guided by intraoperative direct pericardial echocardiography. The technique was first used in a case of tricuspid atresia in a patient in preshock and was then used in 2 more patients with hypoplastic left heart syndrome (HLHS) (age, 10-116 days; body weight, 2.4-3.7 kg). There were no operative complications of embolism or bleeding in our series...
January 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28000391/psychosocial-impact-on-families-with-an-infant-with-a-hypoplastic-left-heart-syndrome-during-and-after-the-interstage-monitoring-period-a-prospective-mixed-method-study
#9
Gaby Stoffel, Rebecca Spirig, Brian Stiasny, Vera Bernet, Hitendu Dave, Walter Knirsch
AIMS AND OBJECTIVES: To investigate parents' experiences, coping ability and quality of life while monitoring their sick child with hypoplastic left heart syndrome at home. BACKGROUND: Interstage home monitoring for children with hypoplastic left heart syndrome reduces interstage mortality between Norwood stages I and II. Little is known about the psychosocial impact of interstage home monitoring. DESIGN: Prospective mixed-method study. METHOD: This study assessed the psychosocial impact on parents during IHM...
December 21, 2016: Journal of Clinical Nursing
https://www.readbyqxmd.com/read/27998845/initial-experience-with-live-three-dimensional-image-overlay-for-ductal-stenting-in-hypoplastic-left-heart-syndrome
#10
Sebastian Goreczny, Gareth J Morgan, Pawel Dryzek, Jadwiga A Moll, Tomasz Moszura
AIMS: Precise visualisation of ductal morphology and adjacent vessels is crucial for accurate stent choice and placement during hybrid palliation of hypoplastic left heart syndrome (HLHS). We aimed to describe our initial experience with live three-dimensional reconstruction overlay derived from rotational angiography (RA) for ductal stenting in HLHS. METHODS AND RESULTS: We carried out a retrospective review of ductal stenting in 18 newborns with HLHS, including six patients with 3D reconstruction overlay used to guide the intervention...
December 20, 2016: EuroIntervention
https://www.readbyqxmd.com/read/27992621/survivorship-in-children-and-young-adults-with-congenital-heart-disease-in-sweden
#11
Zacharias Mandalenakis, Annika Rosengren, Kristofer Skoglund, Georgios Lappas, Peter Eriksson, Mikael Dellborg
Importance: Mortality in patients with congenital heart disease (CHD) has markedly decreased during recent decades because of advancement in pediatric care. However, there are limited data on survival trends in children and young adults with CHD compared with the general population. Objective: To determine survivorship in children and young adults with CHD compared with matched controls. Design, Setting, and Participants: A registry-based, prospective, matched-cohort study was conducted in Sweden...
December 19, 2016: JAMA Internal Medicine
https://www.readbyqxmd.com/read/27981915/variability-of-antithrombotics-use-in-patients-with-hypoplastic-left-heart-syndrome-and-its-variants-following-first-and-second-stage-palliation-surgery-a-national-report-using-the-national-pediatric-cardiology-quality-improvement-collaborative-registry
#12
Preeti Ramachandran, Eileen King, Ashley Nebbia, Robert H Beekman, Jeffrey B Anderson
: Purpose Patients with hypoplastic left heart syndrome and its variants following palliation surgery are at risk for thrombosis. This study examines variability of antithrombotic practice, the incidence of interstage shunt thrombosis, and other adverse events following Stage I and Stage II palliation within the National Pediatric Cardiology Quality Improvement Collaborative registry. METHODS: We carried out a multicentre, retrospective review using the National Pediatric Cardiology Quality Improvement Collaborative registry including patients from 2008 to 2013 across 52 surgical sites...
August 30, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27958617/results-of-the-fontan-operation-with-no-early-mortality-in-248-consecutive-patients
#13
Edward Malec, Christoph Schmidt, Anja Lehner, Katarzyna Januszewska
BACKGROUND: Fontan operation has undergone several modifications and today is the primary way to treat a broad spectrum of congenital heart defects AIM: The purpose of this study is to present the results of treatment of children with a single ventricle operated by the same surgical team and managed according to a uniform strategy. METHODS: In the years 2007-2015, in 248 children aged 3.7 ± 2.6 years and weigh of 14.6 ± 6.1 kg with a single ventricle the Fontan surgery was performed...
December 13, 2016: Kardiologia Polska
https://www.readbyqxmd.com/read/27940784/parental-refusal-of-surgery-in-an-infant-with-tricuspid-atresia
#14
Alexander A Kon, Angira Patel, Steven Leuthner, John D Lantos
We present a case of a fetal diagnosis of tricuspid atresia (TA). The pregnant woman and her husband requested that the baby be treated with only palliative care. The cardiologist did not think it would be appropriate to withhold life-prolonging surgery once the infant was born. The neonatologist argued that outcomes for TA are similar to those for hypoplastic left heart syndrome, and the standard practice at the institution was to allow parents to choose surgery or end-of-life care for those infants. The team requested an ethics consultation to assist in determining whether forgoing life-prolonging interventions in this case would be ethically supportable...
November 2016: Pediatrics
https://www.readbyqxmd.com/read/27908945/norwood-procedure-for-palliation-of-hypoplastic-left-heart-syndrome-right-ventricle-to-pulmonary-artery-conduit-vs-modified-blalock-taussig-shunt
#15
Dorothy M Beke
Patients with hypoplastic left heart syndrome undergo a series of operations to separate the pulmonary and systemic circulations. The first of at least 3 operations occurs in the newborn period, with a stage I palliation. The goal of stage I palliation is to provide pulmonary blood flow and create an unobstructed systemic outflow tract. Advances in surgical techniques and intraoperative and postoperative care have helped decrease morbidity and mortality for patients with hypoplastic left heart syndrome who have the stage I Norwood operation, but the patients continue to be at increased risk for hemodynamic collapse and adverse outcomes...
December 2016: Critical Care Nurse
https://www.readbyqxmd.com/read/27896819/extracorporeal-membrane-oxygenation-outcomes-after-the-comprehensive-stage-ii-procedure-in-patients-with-single-ventricles
#16
Daniel Gomez, Vicky Duffy, Diane Hersey, Carl Backes, Peter Rycus, Patrick McConnell, Jordan Voss, Mark Galantowicz, Clifford L Cua
Outcomes for extracorporeal membrane oxygenation (ECMO) have been described for patients with single ventricle physiology (SVP) undergoing cavopulmonary connection (Glenn procedure). An alternative surgical pathway for patients with SVP consists of an initial hybrid procedure followed by a comprehensive Stage II procedure. No data exist describing the outcomes of patients requiring ECMO after the comprehensive Stage II procedure. The goal of this study is to describe the outcomes for patients who required ECMO after the comprehensive Stage II procedure...
November 29, 2016: Artificial Organs
https://www.readbyqxmd.com/read/27894567/proportion-of-selected-congenital-heart-defects-attributable-to-recognized-risk-factors
#17
Regina M Simeone, Sarah C Tinker, Suzanne M Gilboa, A J Agopian, Matthew E Oster, Owen J Devine, Margaret A Honein
PURPOSE: To assess the contribution of multiple risk factors for two congenital heart defects-hypoplastic left heart syndrome (HLHS) and tetralogy of Fallot (TOF). METHODS: We used data from the National Birth Defects Prevention Study (1997-2011) to estimate average adjusted population attributable fractions for several recognized risk factors, including maternal prepregnancy overweight-obesity, pregestational diabetes, age, and infant sex. RESULTS: There were 594 cases of isolated simple HLHS, 971 cases of isolated simple TOF, and 11,829 controls in the analysis...
December 2016: Annals of Epidemiology
https://www.readbyqxmd.com/read/27885446/post-cardiotomy-rescue-extracorporeal-cardiopulmonary-resuscitation-in-neonates-with-single-ventricle-after-intractable-cardiac-arrest-attrition-after-hospital-discharge-and-predictors-of-outcome
#18
Anastasios C Polimenakos, Vincent Rizzo, Chawki F El-Zein, Michel N Ilbawi
Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We reviewed outcomes of neonates with functional single ventricle (FSV) surviving post-cardiotomy ECPR after hospital discharge. Fifty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2011) were identified. Forty-one were neonates...
November 24, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27879470/incidence-and-management-of-thrombotic-and-thromboembolic-complications-following-the-norwood-procedure-a-systematic-review
#19
Arnav Agarwal, Mohammed Firdouse, Nishaan Brar, Andy Yang, Panos Lambiris, Anthony K Chan, Tapas K Mondal
BACKGROUND: The stage 1 Norwood procedure and its variants represent the first step of palliation for hypoplastic left heart syndrome. Although appropriate postoperative thromboprophylaxis is integral, significant variance remains across institutional practices. The purpose of this systematic review is to estimate the incidence of thrombosis and thromboembolism following the Norwood or modified Blalock-Taussig shunt procedure and examine current thromboprophylaxis regimens. METHODS: Ovid MEDLINE and Embase were searched from January 2000 to June 2016 for primary studies explicitly reporting incidence of thrombosis, thromboembolism (strokes and pulmonary embolisms), or shunt occlusion in neonates, infants, and children undergoing the Norwood procedure or any variant...
November 21, 2016: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/27865477/tracheostomy-among-infants-with-hypoplastic-left-heart-syndrome-undergoing-cardiac-operations-a-multicenter-analysis
#20
Parthak Prodhan, Amit Agarwal, Nahed O ElHassan, Elijah H Bolin, Brandon Beam, Xiomara Garcia, Michael Gaies, Xinyu Tang
BACKGROUND: Less than 2.7% of infants undergoing congenital heart disease operations have difficulty weaning from invasive mechanical ventilation. In such instances, clinicians may choose to perform tracheostomy. Limited literature has examined tracheostomy placement specifically in infants with hypoplastic left heart syndrome (HLHS). This study evaluated the risk factors for tracheostomy placement in infants with HLHS and examined the outcomes of these infants before their first hospital discharge...
November 16, 2016: Annals of Thoracic Surgery
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