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"Hypoplastic left heart"

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https://www.readbyqxmd.com/read/28809063/color-and-power-doppler-combined-with-fetal-intelligent-navigation-echocardiography-fine-to-evaluate-the-fetal-heart
#1
L Yeo, R Romero
OBJECTIVE: To evaluate the performance of color and bidirectional power Doppler ultrasound combined with Fetal Intelligent Navigation Echocardiography (FINE) in examining the fetal heart. METHODS: A prospective cohort study was conducted of fetuses in the second and third trimesters with a normal heart or with congenital heart disease (CHD). One or more spatiotemporal image correlation (STIC) volume datasets, combined with color or bidirectional power Doppler (S-flow) imaging, were acquired in the apical four-chamber view...
August 14, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28793912/re-evaluation-of-hypoplastic-left-heart-syndrome-from-a-developmental-and-morphological-perspective
#2
A Crucean, A Alqahtani, D J Barron, W J Brawn, R V Richardson, J O'Sullivan, R H Anderson, D J Henderson, B Chaudhry
BACKGROUND: Hypoplastic left heart syndrome (HLHS) covers a spectrum of rare congenital anomalies characterised by a non-apex forming left ventricle and stenosis/atresia of the mitral and aortic valves. Despite many studies, the causes of HLHS remain unclear and there are conflicting views regarding the role of flow, valvar or myocardial abnormalities in its pathogenesis, all of which were proposed prior to the description of the second heart field. Our aim was to re-evaluate the patterns of malformation in HLHS in relation to recognised cardiac progenitor populations, with a view to providing aetiologically useful sub-groupings for genomic studies...
August 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28790278/-perioperative-care-for-patients-with-hypoplastic-left-heart-syndrome
#3
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/28761995/intermediate-term-thrombotic-risk-in-contemporary-total-cavo-pulmonary-connection-for-single-ventricle-circulations
#4
Jenna M Faircloth, Olivia Roe, Tarek Alsaied, Joseph S Palumbo, Alexander Vinks, Gruschen R Veldtman
Despite the common occurrence of thrombosis in Fontan circulations, the mid-term thrombotic risk beyond the first two postoperative years is poorly defined especially in total cavo pulmonary Fontan. This study examines the thrombotic incidence and risk beyond the first 2 years after contemporary Fontan surgery. Using a retrospective cohort study design, 89 Fontan patients, 50 male, were included and evaluated with a median of 8.3 years (IQR 6.8-11.4) follow-up. Hospital records were reviewed for known risk factors of thrombosis, thrombotic events, antiplatelet and anticoagulation management, and basic characteristics...
July 31, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28754809/ventricular-function-and-vascular-dimensions-after-norwood-and-hybrid-palliation-of-hypoplastic-left-heart-syndrome
#5
Heiner Latus, Mohamed S Nassar, James Wong, Pauline Hachmann, Hannah Bellsham-Revell, Tarique Hussain, Christian Apitz, Caner Salih, Conal Austin, David Anderson, Can Yerebakan, Hakan Akintuerk, Juergen Bauer, Reza Razavi, Dietmar Schranz, Gerald Greil
OBJECTIVE: Norwood and hybrid procedure are two options available for initial palliation of patients with hypoplastic left heart syndrome (HLHS). Our study aimed to assess potential differences in right ventricular (RV) function and pulmonary artery dimensions using cardiac magnetic resonance (CMR) in survivors with HLHS. METHODS: 42 Norwood (mean age 2.4±0.8) and 44 hybrid (mean age 2.0±1.0 years) patients were evaluated by CMR after stage II palliation prior to planned Fontan completion...
July 28, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28744764/the-miracle-baby-grows-up-hypoplastic-left-heart-syndrome-in-the-adult
#6
REVIEW
Matthew Lewis, Marlon Rosenbaum
PURPOSE OF REVIEW: Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the mitral valve, left ventricle, and aorta and is ultimately palliated with a single-ventricle repair. Universally fatal in infancy prior to the advent of modern surgical techniques, the majority of HLHS patients will now reach adulthood. However, despite improvements in early survival, the HLHS population continues to face significant morbidity and early mortality. This review delineates common sources of patient morbidity and highlights areas in need of additional research for this growing segment of the adult congenital heart disease population...
August 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28736841/outcomes-of-blalock-taussig-shunts-in-current-era-a-single-center-experience
#7
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/28736836/identification-of-adults-with-congenital-heart-disease-of-moderate-or-great-complexity-from-administrative-data
#8
Jill M Steiner, James N Kirkpatrick, Susan R Heckbert, Asma Habib, James Sibley, William Lober, J Randall Curtis
INTRODUCTION: There is relatively sparse literature on the use of administrative datasets for research in patients with adult congenital heart disease (ACHD). The goal of this analysis is to examine the accuracy of administrative data for identifying patients with ACHD who died. METHODS: A list of the International Classification of Diseases codes representing ACHD of moderate- or great-complexity was created. A search for these codes in the electronic health record of adults who received care in 2010-2016 was performed, and used state death records to identify patients who died during this period...
July 24, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28734628/adrenergic-receptor-genotypes-influence-postoperative-outcomes-in-infants-in-the-single-ventricle-reconstruction-trial
#9
Ronand Ramroop, George Manase, Danny Lu, Dorin Manase, Shan Chen, Richard Kim, Teresa Lee, William T Mahle, Kimberly McHugh, Mike Mitchell, Martin Tristani-Firouzi, Stephanie B Wechsler, Nicole S Wilder, Victor Zak, Myriam Lafreniere-Roula, Jane W Newburger, J William Gaynor, Mark W Russell, Seema Mital
OBJECTIVES: Adrenergic receptor (ADR) genotypes have been associated with adverse outcomes in heart failure. Our objective was to evaluate the association of ADR genotypes with post-Norwood outcomes in infants with hypoplastic left heart syndrome (HLHS). METHODS: Infants with HLHS participating in the Pediatric Heart Network Single-Ventricle Reconstruction Trial underwent genotyping for 4 single-nucleotide polymorphisms in 3 ADR genes: ADRB1_231A/G, ADRB1_1165G/C, ADRB2_5318C/G, and ADRA2A_2790C/T...
June 24, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28711428/-congenital-heart-disease-mortality-in-spain-during-a-10-year-period-2003-2012
#10
Javier Pérez-Lescure Picarzo, Margarita Mosquera González, Pello Latasa Zamalloa, David Crespo Marcos
INTRODUCTION AND OBJECTIVES: Congenital heart disease is a major cause of infant mortality in developed countries. In Spain, there are no publications at national level on mortality due to congenital heart disease. The aim of this study is to analyse mortality in infants with congenital heart disease, lethality of different types of congenital heart disease, and their variation over a ten-year period. METHODS: A retrospective observational study was performed to evaluate mortality rate of children under one year old with congenital heart disease, using the minimum basic data set, from 2003 to 2012...
July 12, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28698113/patient-specific-biomechanical-model-of-hypoplastic-left-heart-to-predict-post-operative-cardio-circulatory-behaviour
#11
Elena Cutrì, Alessio Meoli, Gabriele Dubini, Francesco Migliavacca, Tain-Yen Hsia, Giancarlo Pennati
Hypoplastic left heart syndrome is a complex congenital heart disease characterised by the underdevelopment of the left ventricle normally treated with a three-stage surgical repair. In this study, a multiscale closed-loop cardio-circulatory model is created to reproduce the pre-operative condition of a patient suffering from such pathology and virtual surgery is performed. Firstly, cardio-circulatory parameters are estimated using a fully closed-loop cardio-circulatory lumped parameter model. Secondly, a 3D standalone FEA model is build up to obtain active and passive ventricular characteristics and unloaded reference state...
July 8, 2017: Medical Engineering & Physics
https://www.readbyqxmd.com/read/28693354/perinatal-outcomes-of-congenital-heart-disease-after-emergent-neonatal-cardiac-procedures
#12
Aline Wolter, Helene Holtmann, Andreea Kawecki, Jan Degenhardt, Christian Enzensberger, Oliver Graupner, Hakan Akintürk, Can Yerebakan, Markus Khalil, Dietmar Schranz, Roland Axt-Fliedner
PURPOSE: We compared outcome of neonates with prenatal and postnatal diagnosis of congenital heart disease presenting in our paediatric heart centre between 2005-05/2015 who underwent an emergent intervention within 48 hours postpartum. MATERIALS AND METHODS: In 52/111 (46.8%) with emergent intervention, congenital heart disease was diagnosed prenatally, in 59/111 (53.2%) with no specialized fetal echocardiography, diagnosis was made postnatally. In 98/111 (88...
July 10, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28689327/in-utero-evidence-of-impaired-somatic-growth-in-hypoplastic-left-heart-syndrome
#13
Jourdan E Triebwasser, Marjorie C Treadwell
We hypothesized that fetuses with hypoplastic left heart syndrome (HLHS) have impaired growth compared to expected growth for gestational age. This is a retrospective cohort study including singleton fetuses with isolated HLHS identified from a single, referral center's ultrasound database. To account for variable timing of ultrasounds, z-scores for gestational age were assigned for each biometric parameter. We identified 169 fetuses, of which 96 had more than one ultrasound. The median number of ultrasound evaluations per fetus was 2 (range 1-5)...
July 8, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28687711/the-optimal-timing-of-stage-2-palliation-for-hypoplastic-left-heart-syndrome-an-analysis-of-the-pediatric-heart-network-single-ventricle-reconstruction-trial-public-dataset
#14
James M Meza, Edward J Hickey, Eugene H Blackstone, Robert D B Jaquiss, Brett R Anderson, William G Williams, Sally Cai, Glen S Van Arsdell, Tara Karamlou, Brian W McCrindle
Background -In infants requiring three-stage single ventricle palliation for hypoplastic left heart syndrome, attrition after the Norwood procedure remains significant. The effect of the timing of stage-2-palliation (S2P), a physician-modifiable factor, on long term survival is not well understood. We hypothesized that an optimal interval between the Norwood and S2P that both minimizes pre-S2P attrition and maximizes post-S2P survival exists and is associated with individual patient characteristics. Methods -The NIH/NHLBI Pediatric Heart Network Single Ventricle Reconstruction Trial public dataset was used...
July 7, 2017: Circulation
https://www.readbyqxmd.com/read/28684000/management-of-tricuspid-regurgitation-in-patients-with-hypoplastic-left-heart-syndrome
#15
Frank A Pigula, Bret Mettler
Tricuspid valve (TV) performance is critical for palliation of hypoplastic left heart syndrome. We will review current TV repair techniques, outcomes, and novel approaches.
April 2017: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28683514/endocardial-fibroelastosis-of-the-left-ventricle-affects-right-ventricular-performance-in-fetuses-with-hypoplastic-left-heart-syndrome-a-prospective-study-using-m-mode-pw-and-tissue-doppler-techniques
#16
Oliver Graupner, Christian Enzensberger, Larissa Wieg, Jan Degenhardt, Aline Wolter, Markus Khalil, Dietmar Schranz, Can Yerebakan, Astrid Doelle, Johannes Herrmann, Roland Axt-Fliedner
Purpose Myocardial function (MF) of the systemic right ventricle (RV) influences the postnatal course of neonates with hypoplastic left heart syndrome (HLHS). Our study examines whether the presence of endocardial fibroelastosis of the left ventricle (LV EFE) influences MF of the RV in HLHS fetuses. Materials and Methods A prospective study was conducted including 10 controls (group 1), 10 HLHS fetuses with (group 2) and 10 without LV EFE (group 3) - all matched for gestational age. M-mode was used to assess tricuspid plane systolic excursion (TAPSE) and the shortening fraction (SF)...
July 6, 2017: Ultraschall in der Medizin
https://www.readbyqxmd.com/read/28677480/preoperative-venovenous-ecmo-in-an-infant-with-late-diagnosis-of-hypoplastic-left-heart-syndrome
#17
Lena K Eschenbach, Jelena Kasnar-Samprec, Kilian Ackermann, Christian Schreiber, Rudiger Lange, Julie Cleuziou
Late diagnosis and late referral for the Norwood stage 1 procedure in patients with hypoplastic left heart syndrome is rare and associated with a higher mortality. We present a case of a cyanotic almost five-week-old infant with hypoplastic left heart syndrome, highly restrictive foramen ovale, and patent ductus arteriosus, who was bridged with venovenous extracorporeal membrane oxygenation to the Norwood stage 1 procedure.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28667405/utilizing-hybrid-techniques-to-maximize-clinical-outcomes-in-congenital-heart-disease
#18
REVIEW
David W Bearl, Gregory A Fleming
PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital cardiology. Recent collaborations between interventional cardiologists and cardiac surgeons have sparked novel innovative hybrid procedures that provide potentially safer, faster, and less invasive alternatives to the management of many diseases. This paper will review the most recent advances in hybrid interventions in the field of pediatric and adult congenital cardiology...
August 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28661741/hypoplastic-left-heart-syndrome-the-case-against-palliative-surgery
#19
Gwen E Erkonen, Robert A Hanfland
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28661739/growing-discomfort-with-comfort-care-for-hypoplastic-left-heart-syndrome-why-we-should-still-defer-to-parental-wishes
#20
Erin A Paul, Elizabeth M Kohlberg, Kristina Orfali
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
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