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https://www.readbyqxmd.com/read/28804953/basic-helix-loop-helix-transcription-factors-in-evolution-roles-in-development-of-mesoderm-and-neural-tissues
#1
REVIEW
Fuki Gyoja
Basic helix-loop-helix (bHLH) transcription factors have attracted the attention of developmental and evolutionary biologists for decades because of their conserved functions in mesodermal and neural tissue formation in both vertebrates and fruit flies. Their evolutionary history is of special interest because it will likely provide insights into developmental processes and refinement of metazoan-specific traits. This review briefly considers advances in developmental biological studies on bHLHs/HLHs. I also discuss recent genome-wide surveys and molecular phylogenetic analyses of these factors in a wide range of metazoans...
August 14, 2017: Genesis: the Journal of Genetics and Development
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/28754809/ventricular-function-and-vascular-dimensions-after-norwood-and-hybrid-palliation-of-hypoplastic-left-heart-syndrome
#4
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/28744980/building-a-comprehensive-team-for-the-longitudinal-care-of-single-ventricle-heart-defects-building-blocks-and-initial-results
#5
Karen Texter, Jo Ann M Davis, Christina Phelps, Sharon Cheatham, John Cheatham, Mark Galantowicz, Timothy F Feltes
INTRODUCTION: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring. METHODS: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues. RESULTS: Comprehensive Single Ventricle Team in 2014 was begun, to standardize care for children with single ventricle heart defects from diagnosis to adulthood within our institution...
July 26, 2017: Congenital Heart Disease
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/28734628/adrenergic-receptor-genotypes-influence-postoperative-outcomes-in-infants-in-the-single-ventricle-reconstruction-trial
#7
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/28689327/in-utero-evidence-of-impaired-somatic-growth-in-hypoplastic-left-heart-syndrome
#8
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/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
#9
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/28661733/the-ethics-of-treatment-for-hypoplastic-left-heart-syndrome-hlhs
#10
Jeffrey P Spike
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28643402/clinical-findings-in-right-ventricular-noncompaction-in-hypoplastic-left-heart-syndrome
#11
Monique M Gardner, Meryl S Cohen
BACKGROUND: Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution. METHODS: We performed a retrospective review of patients diagnosed with RVNC over a 12-month period at our institution and reviewed their imaging and clinical course...
June 23, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28627006/parents-perceptions-during-the-transition-to-home-for-their-child-with-a-congenital-heart-defect-how-can-we-support-families-of-children-with-hypoplastic-left-heart-syndrome
#12
Sarita March
PURPOSE: The aim of the study was to explore the literature related to transitions in healthcare between the hospital and home that caregivers experience with a child who has a congenital heart defect (CHD), specifically related to hypoplastic left heart syndrome (HLHS). DESIGN AND METHODS: A systematic literature review was conducted searching OVID Medline, CINAHL, and PubMed to discover the caregivers' perceptions on their transitions between hospital care and home care of their child with a CHD...
June 18, 2017: Journal for Specialists in Pediatric Nursing: JSPN
https://www.readbyqxmd.com/read/28608148/hypoplastic-left-heart-syndrome-sequencing-reveals-a-novel-notch1-mutation-in-a-family-with-single-ventricle-defects
#13
Matthew D Durbin, Adrian G Cadar, Charles H Williams, Yan Guo, David P Bichell, Yan Ru Su, Charles C Hong
Hypoplastic left heart syndrome (HLHS) has been associated with germline mutations in 12 candidate genes and a recurrent somatic mutation in HAND1 gene. Using targeted and whole exome sequencing (WES) of heart tissue samples from HLHS patients, we sought to estimate the prevalence of somatic and germline mutations associated with HLHS. We performed Sanger sequencing of the HAND1 gene on 14 ventricular (9 LV and 5 RV) samples obtained from HLHS patients, and WES of 4 LV, 2 aortic, and 4 matched PBMC samples, analyzing for sequence discrepancy...
August 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28568976/percutaneous-obliteration-of-left-ventricular-cavity-to-eliminate-aortic-regurgitation-and-presumed-coronary-steal-in-an-infant-with-hypoplastic-left-heart-syndrome
#14
Howaida G El-Said, Charissa R Pockett, John W Moore
The phenomenon of coronary steal is well known in the setting of HLHS (Hypoplastic Left Heart Syndrome) early after the Classical Norwood Operation. We report a rare case of an infant with HLHS [Severe Aortic Stenosis (AS), Mitral Stenosis (MS) and small Left Ventricle (LV)], who developed aortic regurgitation and presumed coronary steal late after the Sano Modification of the Norwood Procedure. Coronary steal developed secondary to progressive aortic and mitral regurgitation and resulted in poor right ventricular function and severe tricuspid regurgitation...
June 1, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28543953/improved-technical-success-postnatal-outcomes-and-refined-predictors-of-outcome-for-fetal-aortic-valvuloplasty
#15
Kevin G Friedman, Lynn A Sleeper, Lindsay R Freud, Audrey C Marshall, Max E Godfrey, Monika Drogosz, Terra Lafranchi, Carol B Benson, Louise E Wilkins-Haug, Wayne Tworetzky
BACKGROUND: Fetal aortic valvuloplasty (FAV) may prevent progression of mid-gestation aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). METHODS: We evaluate outcomes in 123 fetuses that underwent FAV between 2000 and 2015. We analyze whether technical success and biventricular (Biv) outcome changed from the early era (2000-08) to a more recent era (2009-15) and identify pre-FAV predictors of Biv outcome. We developed CART models to predict the likelihood of Biv outcome...
May 22, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28543854/endocardial-fibroelastosis-is-secondary-to-hemodynamic-alterations-in-the-chick-embryonic-model-of-hypoplastic-left-heart-syndrome
#16
Zivorad Pesevski, Alena Kvasilova, Tereza Stopkova, Ondrej Nanka, Eliska Drobna Krejci, Christine Buffinton, Radka Kockova, Adam Eckhardt, David Sedmera
BACKGROUND: Endocardial fibroelastosis (EFE) is a diffuse thickening of the ventricular endocardium, causing myocardial dysfunction and presenting as unexplained heart failure in infants and children. One of the postulated causes is persistent and increased wall tension in the ventricles. RESULTS: To examine whether reduced ventricular pressure in a chick model of hypoplastic left heart syndrome (HLHS) induced by left atrial ligation (LAL) at embryonic day (ED) 4 is associated with EFE at later stages, myocardial fibrosis was evaluated by histology and immunoconfocal microscopy and mass spectrometry (MS) at ED12...
May 20, 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/28530678/the-complex-genetics-of-hypoplastic-left-heart-syndrome
#17
Xiaoqin Liu, Hisato Yagi, Shazina Saeed, Abha S Bais, George C Gabriel, Zhaohan Chen, Kevin A Peterson, You Li, Molly C Schwartz, William T Reynolds, Manush Saydmohammed, Brian Gibbs, Yijen Wu, William Devine, Bishwanath Chatterjee, Nikolai T Klena, Dennis Kostka, Karen L de Mesy Bentley, Madhavi K Ganapathiraju, Phillip Dexheimer, Linda Leatherbury, Omar Khalifa, Anchit Bhagat, Maliha Zahid, William Pu, Simon Watkins, Paul Grossfeld, Stephen A Murray, George A Porter, Michael Tsang, Lisa J Martin, D Woodrow Benson, Bruce J Aronow, Cecilia W Lo
Congenital heart disease (CHD) affects up to 1% of live births. Although a genetic etiology is indicated by an increased recurrence risk, sporadic occurrence suggests that CHD genetics is complex. Here, we show that hypoplastic left heart syndrome (HLHS), a severe CHD, is multigenic and genetically heterogeneous. Using mouse forward genetics, we report what is, to our knowledge, the first isolation of HLHS mutant mice and identification of genes causing HLHS. Mutations from seven HLHS mouse lines showed multigenic enrichment in ten human chromosome regions linked to HLHS...
July 2017: Nature Genetics
https://www.readbyqxmd.com/read/28521042/induced-pluripotent-stem-cell-modelling-of-hlhs-underlines-the-contribution-of-dysfunctional-notch-signalling-to-impaired-cardiogenesis
#18
Chunbo Yang, Yaobo Xu, Min Yu, David Lee, Sameer Alharti, Nicola Hellen, Noor Ahmad Shaik, Babajan Banaganapalli, Hussein Ali Mohamoud Sheikh, Elango Ramu, Stefan Przyborski, Gennadiy Tenin, Simon Williams, John O'Sullivan, Osman O Al-Radi, Jameel Atta, Sian E Harding, Bernard Keavney, Majlinda Lako, Lyle Armstrong
Hypoplastic left heart syndrome (HLHS) is among the most severe forms of congenital heart disease. Although the consensus view is that reduced flow through the left heart during development is a key factor in the development of the condition, the molecular mechanisms leading to hypoplasia of left heart structures are unknown. We have generated induced pluripotent stem cells (iPSC) from five HLHS patients and two unaffected controls, differentiated these to cardiomyocytes and identified reproducible in vitro cellular and functional correlates of the HLHS phenotype...
May 17, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28512719/hypoplastic-left-heart-syndrome-with-congenital-complete-heart-block
#19
Maytham Al-Kubaisi, Safwat A Aly, Bassel Mohammad Nijres, Sawsan Awad
Congenital complete atrioventricular block (CCAVB) is a rare condition with an incidence of 1 of 20,000 live births. Hypoplastic left heart syndrome (HLHS) occurs more frequently than CCAVB and occurs in 1 of 5000 live births. HLHS in association with CCAVB is exceedingly rare. In this report, we describe a rare case of HLHS and CCAVB diagnosed in utero. Postnatal diagnosis, management and outcome are presented as well as review of the medical literature.
August 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28446969/micrornas-pleiotropic-players-in-congenital-heart-disease-and-regeneration
#20
REVIEW
Sarah C Hoelscher, Stefanie A Doppler, Martina Dreßen, Harald Lahm, Rüdiger Lange, Markus Krane
Congenital heart disease (CHD) is the leading cause of infant death, affecting approximately 4-14 live births per 1,000. Although surgical techniques and interventions have improved significantly, a large number of infants still face poor clinical outcomes. MicroRNAs (miRs) are known to coordinately regulate cardiac development and stimulate pathological processes in the heart, including fibrosis or hypertrophy and impair angiogenesis. Dysregulation of these regulators could therefore contribute (I) to the initial development of CHD and (II) at least partially to the observed clinical outcomes of many CHD patients by stimulating the aforementioned pathways...
March 2017: Journal of Thoracic Disease
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