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https://www.readbyqxmd.com/read/29350395/early-results-of-aortic-arch-reconstruction-and-bilateral-pulmonary-arteries-banding-modification-of-the-norwood-operation-for-treatment-of-hypoplastic-left-heart-syndrome
#1
Jacek W Pająk, Michał Buczyński, Piotr Stanek, Grzegorz Zalewski, Marek Wites, Lesław Szydłowski, Lidia Tomkiewicz-Pająk
BACKGROUND: In the period from 2003 to the end of 2015, 96 Norwood I procedures were performed in Pediatric Heart Surgery Department in Katowice, Poland, in children with hypoplastic left heart syndrome (HLHS). AIM: This paper presents a retrospective analysis of early surgical results. METHODS: The patients consisted of two groups: Group I included 59 children operated on in the years 2003-2012 in whom the stage I Norwood procedure with the Sano modification was performed with the aortic arch reconstructed by use of a homogenous pulmonary artery patch or a bovine pericardial patch...
January 19, 2018: Kardiologia Polska
https://www.readbyqxmd.com/read/29350247/prolonged-pr-interval-at-birth-predicting-the-high-occurrence-of-fatal-atrioventricular-block-in-hypoplastic-left-heart-syndrome
#2
Eiji Morihana, Kenichiro Yamamura, Yuichiro Sugitani, Hideaki Kado, Yasushi Takahata, Toshihide Nakano, Yasutaka Nakashima, Naoki Fusazaki, Shouichi Ohga
Infants with hypoplastic left heart syndrome (HLHS) are at high mortality especially when they are associated with bradyarrhythmias. However, the risk factor of developing high-grade atrioventricular block (HAVB) is still unclear. Seventy-three patients with HLHS in our institutions from 2002 to 2011 were enrolled. The survival rate was assessed by the anatomical types, treatments, occurrence of HAVB, severe tricuspid regurgitation (TR), and restrictive atrial septal defect (ASD) along with electrocardiogram findings at birth...
January 19, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29332707/cutaneous-hemophagocytosis-clinicopathologic-features-of-21-cases
#3
Francesca Boggio, Viviana Lora, Carlo Cota, Amanda Pereira, Robert Müllegger, Lucia Prieto-Torres, Lorenzo Cerroni
BACKGROUND: Hemophagocytosis is well known in cytotoxic cutaneous T-cell lymphomas (CTCLs), in which it may represent a sign of hemophagocytic lymphohistiocytosis syndrome (HLHS), and is also typical of cutaneous Rosai-Dorfman disease (cRDD) (without prognostic relevance). Only rarely, has cutaneous hemophagocytosis (CH) been described in other skin conditions. OBJECTIVE: To characterize the clinicopathologic features of CH in skin biopsy specimens from patients with conditions other than CTCL or cRDD...
February 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29317056/a-potentially-curative-fetal-intervention-for-hypoplastic-left-heart-syndrome
#4
Andrew T Hattam
Hypoplastic left heart syndrome (HLHS) encapsulates a spectrum of complex congenital cardiovascular malformations involving varying degrees of underdevelopment of the left-sided heart structures. However, despite improved survival rates since the introduction of staged surgical reconstruction, treatment options for HLHS remain palliative rather than curative. A major limiting factor in the development of definitive curative therapy for HLHS is an incomplete understanding of its pathogenesis. Currently, the aetiology HLHS is best conceptualised by the 'flow theory' of cardiogenesis, which states that normal cardiac development is reliant on the interrelationship of normal flow patterns of blood through the developing heart, and appropriate growth of the cardiac valves and myocardium...
January 2018: Medical Hypotheses
https://www.readbyqxmd.com/read/29299618/heart-transplantation-in-children-with-turner-syndrome-analysis-of-a-linked-dataset
#5
Joshua D Chew, Jonathan H Soslow, Cary Thurm, Matt Hall, Debra A Dodd, Brian Feingold, Jill Simmons, Justin Godown
Turner syndrome (TS) patients with hypoplastic left heart syndrome (HLHS) have poor single ventricle palliation outcomes; therefore, consideration of other potential management strategies is important. Little is known about heart transplantation (HTx) in this group, as standard HTx databases do not allow for identification of TS. This study describes experiences and outcomes of HTx in TS using a unique linkage between the Scientific Registry of Transplant Recipients and the Pediatric Health Information System databases...
January 3, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29290485/tricuspid-valve-adaptation-during-the-first-interstage-period-in-hypoplastic-left-heart-syndrome
#6
Timothy Colen, Shelby Kutty, Richard B Thompson, Edythe Tham, Andrew S Mackie, Ling Li, Dongngan T Truong, Michiko Maruyama, Jeffrey F Smallhorn, Nee Scze Khoo
BACKGROUND: Tricuspid regurgitation (TR) is an important risk factor for morbidity and mortality in hypoplastic left heart syndrome (HLHS), yet the evolution of tricuspid valve (TV) dysfunction in HLHS is poorly understood. This study sought to examine changes in TV function in HLHS between the first two stages of surgical palliation and to determine the mechanism of TR at the time of stage two surgery-bidirectional cavopulmonary anastomosis (BCPA). METHODS: We prospectively investigated 44 infants at two time points-prior to Norwood-Sano (T1 - median age 5...
December 29, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29288017/improved-lung-perfusion-after-left-pulmonary-artery-patch-enlargement-during-the-norwood-operation
#7
Mona Salehi Ravesh, Jens Scheewe, Tim Attmann, Abdullah Al Bulushi, Marka-Jill Jussli-Melchers, Michael Jerosch-Herold, Dominik D Gabbert, Philip Wegner, Hans-Heiner Kramer, Carsten Rickers
BACKGROUND: Optimal pulmonary perfusion is crucial for a well-functioning Fontan circulation in patients with hypoplastic left heart syndrome (HLHS). To obtain an adequate size of the left pulmonary artery (LPA), patch enlargement is a routine part of Hemi-Fontan procedure in our center. However, LPA patch enlargement at the time of the modified Norwood procedure may have surgical advantages. Therefore, the aim of this study was to evaluate if anatomical and functional effects of the new approach are superior...
December 26, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29287136/cerebrovascular-hemodynamics-in-fetuses-with-congenital-heart-disease
#8
Tingting Man, Yihua He, Ying Zhao, Lin Sun, Xiaowei Liu, Shuping Ge
BACKGROUND AND OBJECTIVE: It is hypothesized that diminished cerebral vascular resistance or the "brain sparing effect" is associated with fetuses with complex congenital heart defects (CHD) and may affect their neurodevelopmental outcome. An alternative explanation is that it is related to the location, cardiac output, pressure, and resistance in left heart obstructive CHDs. We sought to determine the effects of various left and right heart obstructive defects on the cerebral and placental hemodynamics and to evaluate the utility of these variables for the assessment and prognosis of CHDs...
December 2017: Echocardiography
https://www.readbyqxmd.com/read/29281616/influence-of-type-of-congenital-heart-defects-on-epithelial-lining-fluid-composition-in-infants-undergoing-cardiac-surgery-with-cardiopulmonary-bypass
#9
Manuela Simonato, Aldo Baritussio, Virgilio P Carnielli, Luca Vedovelli, Gianclaudio Falasco, Michele Salvagno, Massimo Padalino, Paola Cogo
BACKGROUND: In children with congenital heart disease (CHD), altered pulmonary circulation compromises gas exchange. Moreover, pulmonary dysfunction is a complication of cardiac surgery with cardiopulmonary bypass (CPB). No data are available on the effect of different CHDs on lung injury. The aim of this study was to analyze epithelial lining fluid (ELF) surfactant composition in children with CHD. METHODS: Tracheal aspirates (TAs) from 72 CHD children [age 2.9 (0...
December 27, 2017: Pediatric Research
https://www.readbyqxmd.com/read/29280178/relationship-between-cavum-septi-pellucidi-measurements-and-fetal-hypoplastic-left-heart-syndrome-or-dextro-transposition-of-the-great-arteries
#10
Michael Saadeh, Yili Zhao, Hadiza Galadima, Rabih Chaoui, Elena Sinkovskaya, Alfred Abuhamad
OBJECTIVES: The aim of this study was to compare the size and position of the cavum septi pellucidi (CSP) in fetuses with hypoplastic left heart syndrome (HLHS) or dextro-transposition of the great arteries (d-TGA) with healthy fetuses. METHODS: The CSP length, CSP width, and frontal lobe length were measured in 185 healthy fetuses (404 scans), 11 fetuses with HLHS (16 scans), and 11 fetuses with d-TGA (12 scans) between January 2005 and April 2016. Each measurement was compared between healthy fetuses and those with HLHS or d-TGA, controlling for the biparietal diameter...
December 27, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/29118890/a-novel-technique-for-the-administration-of-sub-ambient-oxygen-in-the-operating-room
#11
Asad A Khawaja, Marco Corridore, Joseph D Tobias
Hypoplastic left heart syndrome (HLHS) is a common form of congenital heart disease with abnormal development of left-sided structures of the heart. As excessive pulmonary blood flow is common prior to palliative surgical procedures, therapeutic maneuvers may be required to decrease pulmonary blood flow and augment systemic cardiac output. Respiratory manipulations to optimize the ratio of pulmonary to systemic blood flow may include techniques to induce hypercarbia or the delivery of sub-ambient concentrations of oxygen (FiO2 less than 0...
October 2017: Cardiology Research
https://www.readbyqxmd.com/read/29104017/neurodevelopmental-outcome-and-health-related-quality-of-life-in-children-with-single-ventricle-heart-disease-before-fontan-procedure
#12
Bettina Reich, Kristina Heye, Ruth Tuura, Ingrid Beck, Kristina Wetterling, Andreas Hahn, Karoline Hofmann, Dietmar Schranz, Hakan Akintürk, Beatrice Latal, Walter Knirsch
Neurodevelopmental impairment and impaired quality of life constitute a major source of morbidity among children with complex congenital heart disease, in particular for single-ventricle (SV) morphologies. Risk factors and quality of life determining clinical and neurodevelopmental outcome at 2 years of age are examined. In a 2-center cohort study, 48 patients with SV morphology (26 hypoplastic left heart syndrome and 22 other types of univentricular heart defect) have been examined before Fontan procedure between 2010 and 2015...
December 5, 2017: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29096870/hypoplastic-left-heart-syndrome-is-not-a-predictor-of-worse-intermediate-mortality-post-fontan
#13
Billie-Jean Martin, Kandice Mah, Luke Eckersley, Joyce Harder, Charissa Pockett, Daryl Schantz, John Dyck, Mohammed Al Aklabi, Ivan M Rebeyka, David B Ross
BACKGROUND: An increasing proportion of those living with single ventricle physiology have hypoplastic left heart syndrome (HLHS). Our objective was to assess the association between HLHS and outcomes post Fontan operation. METHODS: All pediatric patients who underwent a Fontan procedure at the University of Alberta between 1996 and 2016 were included. Follow-up clinical data collected included early and late surgical or catheter reintervention, echocardiography, and long-term transplant-free survival...
December 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29057564/the-effect-of-fetal-hemodynamics-on-fetal-growth-in-single-ventricle-and-transposition-of-the-great-arteries
#14
Tarek Alsaied, Stephanie Tseng, Eileen King, Eunice Hahn, Allison Divanovic, Mounira Habli, James Cnota
INTRODUCTION: As birth weight is a critical predictor of neonatal congenital heart defect (CHD) outcomes, the common problem of poor fetal growth in this population is clinically important but not well understood. The impact of fetal hemodynamics on fetal growth and birth weight in CHD has not been assessed. Combined cardiac output (CCO) has been used to evaluate patients with structurally normal heart but rarely in CHD. Middle cerebral artery and umbilical artery pulsatility indices (PIs) have been used to evaluate the relative distribution of circulation in patients with CHD...
October 23, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29016838/the-hand1-frameshift-a126fs-mutation-does-not-cause-hypoplastic-left-heart-syndrome-in-mice
#15
Beth A Firulli, Kevin P Toolan, Jade Harkin, Hannah Millar, Santiago Pineda, Anthony B Firulli
Aims: To test if a human Hand1 frame shift mutation identified in human samples is causative of hypoplastic left heart syndrome (HLHS). Methods and results: HLHS is a poorly understood single ventricle congenital heart defect that affects two to three infants in every 10 000 live births. The aetiologies of HLHS are largely unknown. The basic helix-loop-helix transcription factor HAND1 is required for normal heart development. Interrogation of HAND1 sequence from fixed HLHS tissues identified a somatic frame-shift mutation at Alanine 126 (NP_004812...
August 31, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28976608/cerebrovascular-response-to-maternal-hyperoxygenation-mh-in-fetuses-with-hypoplastic-left-heart-syndrome-hlhs-depends-upon-gestational-age-ga-and-baseline-cerebrovascular-resistance
#16
Anita Szwast, Mary Putt, J William Gaynor, Daniel Licht, Jack Rychik
OBJECTIVES: Compared to normal fetuses, HLHS fetuses have smaller brain volumes and more brain injury, possibly due to diminished cerebral blood flow and oxygen content. By increasing cerebral oxygen delivery, maternal hyperoxygenation (MH) might improve brain development and reduce brain injury in these fetuses. This study investigates whether gestational age (GA) and baseline cerebrovascular resistance impact response to MH in HLHS fetuses. METHODS: The middle cerebral artery (MCA) pulsatility index (PI), a surrogate measure of cerebrovascular resistance, was assessed in 43 HLHS fetuses...
October 4, 2017: Ultrasound in Obstetrics & Gynecology
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
#17
RANDOMIZED CONTROLLED TRIAL
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
#18
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
#19
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
#20
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
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