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https://www.readbyqxmd.com/read/28938963/study-design-and-rationale-for-elpis-a-phase-i-iib-randomized-pilot-study-of-allogeneic-human-mesenchymal-stem-cell-injection-in-patients-with-hypoplastic-left-heart-syndrome
#1
Sunjay Kaushal, Brody Wehman, Nicholas Pietris, Casey Naughton, Soren M Bentzen, Grace Bigham, Rachana Mishra, Sudhish Sharma, Luca Vricella, Allen D Everett, Kristopher B Deatrick, Sihong Huang, Helina Mehta, William A Ravekes, Naru Hibino, Darcy L Difede, Aisha Khan, Joshua M Hare
Despite advances in surgical technique and postoperative care, long-term survival of children born with hypoplastic left heart syndrome (HLHS) remains limited, with cardiac transplantation as the only alternative for patients with failing single ventricle circulations. Maintenance of systemic right ventricular function is crucial for long-term survival, and interventions that improve ventricular function and avoid or defer transplantation in patients with HLHS are urgently needed. We hypothesize that the young myocardium of the HLHS patient is responsive to the biological cues delivered by bone marrow-derived mesenchymal stem cells (MSCs) to improve and preserve right ventricle function...
October 2017: American Heart Journal
https://www.readbyqxmd.com/read/28936753/long-term-results-of-bilateral-pulmonary-artery-banding-versus-primary-norwood-procedure
#2
Yasutaka Hirata, Hiroaki Miyata, Norimichi Hirahara, Arata Murakami, Hideaki Kado, Kisaburo Sakamoto, Shunji Sano, Shinichi Takamoto
Bilateral pulmonary artery banding (PAB) has emerged to be an attractive option as an initial procedure for the treatment of hypoplastic left heart syndrome (HLHS), and some centers report excellent survival. However, its usage is variable among institutions and its true efficacy is unknown due to reporting biases. We aimed to describe the results of bilateral PAB use, preoperative risk factors, and long-term outcomes compared with primary Norwood procedure, using a national database. Infants who underwent bilateral PAB or Norwood procedure as an initial palliation for HLHS between January 2008 and December 2012 listed in the Japan Congenital Cardiovascular Surgery Database (JCCVSD) were included...
September 21, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28921168/chromosomal-abnormalities-affect-the-surgical-outcome-in-infants-with-hypoplastic-left-heart-syndrome-a-large-cohort-analysis
#3
Dala Zakaria, Xinyu Tang, Rupal Bhakta, Nahed O ElHassan, Parthak Prodhan
Patients with hypoplastic left heart syndrome (HLHS) can have associated genetic abnormalities. This study evaluated the incidence of genetic abnormalities among infants with HLHS and the short-term outcomes of this population during the first hospitalization. This is a retrospective analysis of the multi-center Pediatric Heath Information System database of infants with HLHS who underwent Stage I Norwood, Hybrid, or heart transplant during their first hospitalization from 2004 through 2013. We compared clinical data between infants with and without genetic abnormality, among the three most common chromosomal abnormalities, and between survivors and non-survivors...
September 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28918207/the-association-between-cardiac-physiology-acquired-brain-injury-and-postnatal-brain-growth-in-critical-congenital-heart-disease
#4
Shabnam Peyvandi, Hosung Kim, Joanne Lau, A James Barkovich, Andrew Campbell, Steven Miller, Duan Xu, Patrick McQuillen
OBJECTIVE: To assess the trajectory of perioperative brain growth in relationship to cardiac diagnosis and acquired brain injuries. METHODS: This was a cohort study of term neonates with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). Subjects underwent magnetic resonance imaging of the brain pre- and postoperatively to determine the severity of brain injury and total and regional brain volumes by the use of automated morphometry...
August 24, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28904928/prenatal-sonographic-diagnosis-of-hypoplastic-left-heart-syndrome
#5
Raghu Teja Sadineni, B Santh Kumar, N B Chander, Durga Mahita Boppana
Hypoplastic left heart syndrome (HLHS) represents a variety of cardiac malformations that may result from errors in the early stages of cardiac development. HLHS includes a wide spectrum of cardiac malformations including hypoplasia of the left ventricle, ascending aorta, hypoplasia, or atresia of the aortic and mitral valves. Over the recent years, the improved resolution of advanced equipment with awareness and increased performance of second-trimester ultrasound examinations for the assessment of fetal anomalies have helped in understanding the spectrum and have expanded our knowledge of HLHS...
July 2017: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/28867155/the-boundaries-of-fetal-cardiac-intervention-expand-or-tighten
#6
REVIEW
Laura Gellis, Wayne Tworetzky
Fetal cardiac intervention (FCI) is a relatively new and continually evolving field, and, for select cardiac defects, offers the potential to alter the progression of the disease and improve outcomes. It is a procedure that requires a collaborative effort between maternal-fetal medicine, interventional cardiology and fetal echo/ultrasound specialists, as well as fetal and maternal anesthesiologists, nursing specialists, and social workers. This article reviews the most recently reported data and advances in FCI...
August 31, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28864444/hypoplastic-left-heart-syndrome-with-intact-or-restrictive-atrial-septum-a-report-from-the-international-fetal-cardiac-intervention-registry
#7
David W Jantzen, Anita J Moon-Grady, Shaine A Morris, Aimee K Armstrong, Christoph Berg, Joanna H Dangel, Carlen G Fifer, Michele Frommelt, Ulrich Gembruch, Ulrike Herberg, Edgar T Jaeggi, Eftichia V Kontopoulos, Audrey C Marshall, Owen Miller, Renate Oberhoffer, Dick Oepkes, Carlos A Pedra, Simone R Pedra, Fabio Peralta, Ruben A Quintero, Greg Ryan, Sarah K Gelehrter
Infants with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum (HLHS-IAS) represent a high risk subset. Fetal cardiac intervention (FCI) for fetuses with HLHS-IAS has been reported in single-institution series as a therapy that may improve outcomes. This study utilizes the International Fetal Cardiac Intervention Registry (IFCIR) to examine fetal and maternal characteristics and pregnancy and neonatal outcome data for FCI in this population.
September 1, 2017: Circulation
https://www.readbyqxmd.com/read/28863042/the-bioengineered-cardiac-left-ventricle
#8
Nikita M Patel, Ravi K Birla
Left ventricle and aortic valve underdevelopment are presentations in the congenital cardiac condition hypoplastic left heart syndrome (HLHS); current clinical treatments involve right ventricle refunctionalization. Cardiac organoid models provide simplified open chambers engineered into a flow loop, to ameliorate ventricle-type function. Complete bioengineered ventricle development presents a significant advancement in cardiac organoids. This study provides the foundation for bioengineered complete ventricle (BECV) fabrication...
August 31, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28834879/effect-of-acid-base-balance-on-postoperative-course-in-children-with-hypoplastic-left-heart-syndrome-after-the-modified-norwood-procedure
#9
Marcin Gładki, Tomasz Składzień, Rafał Żurek, Elżbieta Broniatowska, Elżbieta Wójcik, Janusz H Skalski
Hypoplastic left heart syndrome (HLHS) is a congenital heart defect that requires 3-stage cardiac surgical treatment and multidirectional specialist care. The condition of newborns in the first postoperative days following the modified Norwood procedure is characterized by considerable hemodynamic instability that may result in a sudden cardiac arrest. It is believed that the most important cause of hemodynamic instability is the fluctuations in redistribution between pulmonary and systemic blood flow.The paper analyzes the postoperative course in 40 neonates with HLHS following the modified Norwood procedure performed under deep hypothermic cardiopulmonary bypass hospitalized in Cardiac Surgical Intensive Care Unit (CSICU) in the years 2014-2015...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28832800/modified-hybrid-procedure-in-hypoplastic-left-heart-syndrome-initial-experience-of-a-center-in-northeastern-brazil
#10
Renato Max Faria, Juliana Torres Pacheco, Itamar Ribeiro de Oliveira, José Madson Vidal, Anilton Bezerra Rodrigues, Ana Luiza Lafeta Costa, Vinicius José da Silva Nina, Marcelo Matos Cascudo
Introduction: Although it only corresponds to 2.5% of congenital heart defects, hypoplastic left heart syndrome (HLHS) is responsible for more than 25% of cardiac deaths in the first week of life. Palliative surgery performed after the second week of life is considered an important risk factor in the treatment of HLHS. Objective: The aim of this study is to describe the initial experience of a medical center in Northeastern Brazil with a modified off-pump hybrid approach for palliation of HLHS...
May 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28831530/evolution-of-ventricular-energetics-in-the-different-stages-of-palliation-of-hypoplastic-left-heart-syndrome-a-retrospective-clinical-study
#11
A Di Molfetta, R Iacobelli, P Guccione, L Di Chiara, M Rocchi, F Cobianchi Belisari, M Campanale, M G Gagliardi, S Filippelli, G Ferrari, A Amodeo
Hyperplastic left heart syndrome (HLHS) patients are palliated by creating a Fontan-type circulation passing from different surgical stages. The aim of this work is to describe the evolution of ventricular energetics parameters in HLHS patients during the different stages of palliation including the hybrid, the Norwood, the bidirectional Glenn (BDG), and the Fontan procedures. We conducted a retrospective clinical study enrolling all HLHS patients surgically treated with hybrid procedure and/or Norwood and/or BDG and/or Fontan operation from 2011 to 2016 collecting echocardiographic and hemodynamic data...
August 22, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28804953/basic-helix-loop-helix-transcription-factors-in-evolution-roles-in-development-of-mesoderm-and-neural-tissues
#12
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
#13
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
#14
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
#15
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
#16
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 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28744764/the-miracle-baby-grows-up-hypoplastic-left-heart-syndrome-in-the-adult
#17
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
#18
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
#19
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
#20
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
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