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"Hypoplastic left heart"

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https://www.readbyqxmd.com/read/29448010/improved-outcomes-in-management-of-hypoplastic-left-heart-syndrome-associated-with-congenital-diaphragmatic-hernia-an-algorithmic-approach
#1
Kaitlin Balduf, T K Susheel Kumar, Umar Boston, Shyam Sathanandam, Marc V Lee, Tim Jancelewicz, Christopher J Knott-Craig
BACKGROUND: Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival of neonates with CDH and HLHS is only 1-5%. We review our experience with CDH and HLHS and compare our outcomes to published literature. METHODS: Retrospective review of all neonates with CDH and HLHS at our institution over a 10 year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies and outcomes were reviewed and an algorithmic approach is proposed...
February 12, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29447332/the-impact-of-the-maternal-foetal-environment-on-outcomes-of-surgery-for-congenital-heart-disease-in-neonates
#2
James William Gaynor, Samuel Parry, Julie S Moldenhauer, Rebecca A Simmons, Jack Rychik, Richard F Ittenbach, William W Russell, Erin Zullo, John Laurenson Ward, Susan C Nicolson, Thomas L Spray, Mark P Johnson
OBJECTIVES: Pregnancies with congenital heart disease in the foetus have an increased prevalence of pre-eclampsia, small for gestational age and preterm birth, which are evidence of an impaired maternal-foetal environment (MFE). METHODS: The impact of an impaired MFE, defined as pre-eclampsia, small for gestational age or preterm birth, on outcomes after cardiac surgery was evaluated in neonates (n = 135) enrolled in a study evaluating exposure to environmental toxicants and neuro-developmental outcomes...
February 13, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29440831/prognostic-value-of-troponin-in-infants-with-hypoplastic-left-heart-syndrome-between-stage-i-and-ii-of-palliation
#3
Martin Christmann, Emanuela R Valsangiacomo Büchel, Hitendu Dave, Dietrich Klauwer, Anna Cavigelli-Brunner
Background: The period between stage I and II procedure for treatment of hypoplastic left heart syndrome (HLHS) bears high mortality and morbidity. Methods: We sought to analyze the prognostic value of Troponin T/I (Trop), a well-recognized marker for myocardial damage and heart failure, for predicting outcome in a retrospective analysis of 70 infants with HLHS at our institution between March 2001 and October 2014. Results: Stage I procedure consisted of Norwood I operation in 35 (50%) and Hybrid-approach in 22 (31%) patients...
January 2018: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/29436717/early-hemodynamic-changes-after-fetal-aortic-stenosis-valvuloplasty-predict-biventricular-circulation-at-birth
#4
Aaron R Prosnitz, Monika Drogosz, Audrey C Marshall, Louise E Wilkins-Haug, Carol B Benson, Lynn A Sleeper, Wayne Tworetzky, Kevin G Friedman
OBJECTIVE: To describe the early hemodynamic changes after fetal aortic valvuloplasty (FAV) for evolving hypoplastic left heart syndrome due to mid-gestational aortic stenosis and to assess whether these early changes predict biventricular (BiV) circulation at neonatal discharge. METHOD: We retrospectively reviewed all technically successful FAV cases resulting in live birth between 2000 and 2015 (n=93, 45% BiV circulation at neonatal discharge). Paired testing methods were used to compare pre- and post-intervention measures of left ventricular hemodynamics...
February 13, 2018: Prenatal Diagnosis
https://www.readbyqxmd.com/read/29432364/per-ventricular-insertion-of-melody-valve-in-valve-in-the-neoaortic-position-in-a-single-ventricle-patient
#5
Nicholas S Clarke, Howaida El-Said, John J Lamberti, Raghav A Murthy
Percutaneous therapies for congenital heart disease have been evolving rapidly despite limited investment from industry. The Melody transcatheter pulmonary valve (Medtronic, Inc, Minneapolis, MN USA) replacement therapy represents an important advancement in this arena. It has been approved in the United States for use in the pulmonary position, on a Humanitarian Device Exemption status. Off-label use of the Melody transcatheter pulmonary valve has extended to the mitral, pulmonary, and aortic valves, especially in previously implanted valves with prosthetic valve degeneration...
February 9, 2018: Innovations: Technology and Techniques in Cardiothoracic and Vascular Surgery
https://www.readbyqxmd.com/read/29425520/giessen-procedure-as-comprehensive-stage-ii-palliation-with-aortic-arch-reconstruction-after-hybrid-bilateral-pulmonary-artery-banding-and-ductal-stenting-for-hypoplastic-left-heart-syndrome
#6
REVIEW
Uygar Yörüker, Hakan Akintürk
This article reviews our experience using hybrid stage I palliation in the neonatal period and subsequently with comprehensive stage II palliation for hypoplastic left heart syndrome. Between June 1998 and April 2017, 154 patients with the diagnosis of hypoplastic left heart syndrome and variants underwent a hybrid stage I palliation (bilateral pulmonary artery banding and ductal stenting). One hundred thirty-nine patients could be further univentricularly palliated. One hundred twenty-one patients underwent a comprehensive stage II operation with an operative mortality of 6...
March 2018: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://www.readbyqxmd.com/read/29407078/long-term-survival-of-hypoplastic-left-heart-syndrome-infants-meta-analysis-comparing-outcomes-from-the-modified-blalock-taussig-shunt-and-the-right-ventricle-to-pulmonary-artery-shunt
#7
Jacob Y Cao, Kevin Phan, Julian Ayer, David S Celermajer, David S Winlaw
BACKGROUND: Stage 1 palliation of hypoplastic left heart syndrome (HLHS) involves the Norwood procedure combined with a modified Blalock-Taussig shunt (mBTS) or right ventricle to pulmonary artery shunt (RVPAS). Short-term survival has been described previously, whereas longer-term outcomes remain a subject of debate. This meta-analysis aimed to describe the short and long-term survival outcomes of these two shunts, and explore factors that might influence survival. METHODS: Medline, Cochrane Libraries and EMBASE were systematically searched, and 32 studies were included for statistical synthesis, comprising 1348 mBTS and 1258 RVPAS patients...
March 1, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29406374/cerebral-oxygen-metabolism-before-and-after-rbc-transfusion-in-infants-following-major-surgical-procedures
#8
Felix Neunhoeffer, Michael Hofbeck, Martin Ulrich Schuhmann, Jörg Fuchs, Christian Schlensak, Martin Esslinger, Ines Gerbig, Vanya Icheva, Ellen Heimberg, Matthias Kumpf, Jörg Michel
OBJECTIVE: Although infants following major surgery frequently require RBC transfusions, there is still controversy concerning the best definition for requirement of transfusion in the individual patient. The aim of this study was to determine the impact of RBC transfusion on cerebral oxygen metabolism in noncardiac and cardiac postsurgical infants. DESIGN: Prospective observational cohort study. SETTING: Pediatric critical care unit of a tertiary referral center...
February 5, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29402692/life-after-surviving-fontan-surgery-a-meta-analysis-of-the-incidence-and-predictors-of-late-death
#9
REVIEW
C L Poh, Y d'Udekem
AIM: We now know that 20-40% of patients with a single ventricle will develop heart failure after the second decade post-Fontan surgery. However, we remain unable to risk-stratify the cohort to identify patients at highest risk of late failure and death. We conducted a systematic review of all reported late outcomes for patients with a Fontan circulation to identify predictors of late death. METHODS: We searched MEDLINE, Embase and PubMed with subject terms ("single ventricle", "Hypoplastic left heart syndrome", "congenital heart defects" or "Fontan procedure") AND ("heart failure", "post-operative complications", "death", "cause of death", "transplantation" or "follow-up studies") for relevant studies between January 1990 and December 2015...
December 20, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29392349/necrotizing-enterocolitis-in-infants-with-hypoplastic-left-heart-syndrome-following-stage-1-palliation-or-heart-transplant
#10
Nahed O ElHassan, Xinyu Tang, Jeffrey Gossett, Dala Zakaria, Ashley Ross, Sashi K Kona, Parthak Prodhan
Previous studies of necrotizing enterocolitis (NEC) among infants with hypoplastic left heart syndrome (HLHS) were conducted in single centers or had small sample sizes. This study aimed to determine the mortality rate and the risk factors for NEC among infants with HLHS who were discharged over a 10-year period (2004-2013) from 41 Pediatric Health Information System affiliated children's hospitals. Either stage 1 palliation and/or heart transplant were completed prior to patient's death or hospital discharge...
February 1, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29379235/levoatrial-cardinal-vein-with-normal-left-ventricle-a-forgotten-cause-of-pulmonary-arterial-hypertension
#11
Onkar B Auti, Varun Shetty, Vinay Belaval, Vimal Raj
Levoatrial cardinal vein (LACV) is anomalous connection between left atrium or pulmonary veins and systemic veins such as innominate vein or superior vena cava. This persistence of splanchnic circulation occurs when there is left-sided obstructive cardiac lesions such as hypoplastic left heart or mitral atresia. In this report we present three cases of LACV with well-developed left heart, without any obstructive lesions. All our cases presented with pulmonary arterial hypertension (PAH) and had associated intracardiac shunt such as ventricular/atrial septal defect and supracardiac partial anomalous pulmonary venous connection...
October 2017: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/29378757/systemic-right-ventricle-in-adults-with-congenital-heart-disease-anatomic-and-phenotypic-spectrum-and-current-approach-to-management
#12
REVIEW
Margarita Brida, Gerhard-Paul Diller, Michael A Gatzoulis
The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol...
January 30, 2018: Circulation
https://www.readbyqxmd.com/read/29377646/stillborn-infants-associated-malformations
#13
Lewis B Holmes, Hanah Nasri, Rebecca Beroukhim, Anne-Therese Hunt, Drucilla J Roberts, M Hassan Toufaily, Marie-Noel Westgate
BACKGROUND: Stillbirth, defined as death of a fetus in utero after 20 weeks of gestation, occurs in 1 to 2% of pregnancies in the United States. Many of these stillborn infants have associated malformations, including chromosome abnormalities, neural tube defects, and malformation syndromes. Other causes are abnormalities of the placenta and maternal conditions, such as pre-eclampsia and obesity. A consecutive sample of malformed stillborn infants can establish the relative frequency and severity of the associated malformations...
January 2018: Birth Defects Research
https://www.readbyqxmd.com/read/29373649/application-of-hybrid-stage-i-palliation-for-patients-with-two-ventricular-cavities-and-hypoplastic-left-heart-structures
#14
Akihiko Higashida, Takaya Hoashi, Masataka Kitano, Masatoshi Shimada, Tomohiro Nakata, Hideto Ozawa, Kenichi Kurosaki, Hajime Ichikawa
OBJECTIVES: To assess the feasibility of hybrid Stage I palliation consisting of bilateral pulmonary artery bandings and ductal stenting for patients with 2 ventricular cavities and hypoplastic left heart structures. METHODS: Eleven consecutive patients who underwent hybrid Stage I palliation between 2010 and 2017 were enrolled. The diagnoses were interrupted aortic arch/coarctation of the aorta, ventricular septal defect and significant left ventricular (LV) outflow tract obstruction in 5 patients, critical aortic stenosis and reduced LV contraction in 3 patients and hypoplastic left heart complex in 3 patients...
January 24, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29371373/interventional-treatments-and-risk-factors-in-patients-born-with-hypoplastic-left-heart-syndrome-in-england-and-wales-from-2000-to-2015
#15
Libby Rogers, Christina Pagel, Ian D Sullivan, Muhammed Mustafa, Victor Tsang, Martin Utley, Catherine Bull, Rodney C Franklin, Kate L Brown
OBJECTIVE: To describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales. METHODS: The UK's national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code...
January 25, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29370913/hypoplastic-left-heart-syndrome-and-the-allure-of-a-valved-conduit
#16
EDITORIAL
Harold M Burkhart, Arshid Mir, Jess L Thompson
No abstract text is available yet for this article.
December 26, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29351346/the-congenital-heart-disease-genetic-network-study-cohort-description
#17
Thanh T Hoang, Elizabeth Goldmuntz, Amy E Roberts, Wendy K Chung, Jennie K Kline, John E Deanfield, Alessandro Giardini, Adolfo Aleman, Bruce D Gelb, Meghan Mac Neal, George A Porter, Richard Kim, Martina Brueckner, Richard P Lifton, Sharon Edman, Stacy Woyciechowski, Laura E Mitchell, A J Agopian
The Pediatric Cardiac Genomics Consortium (PCGC) designed the Congenital Heart Disease Genetic Network Study to provide phenotype and genotype data for a large congenital heart defects (CHDs) cohort. This article describes the PCGC cohort, overall and by major types of CHDs (e.g., conotruncal defects) and subtypes of conotrucal heart defects (e.g., tetralogy of Fallot) and left ventricular outflow tract obstructions (e.g., hypoplastic left heart syndrome). Cases with CHDs were recruited through ten sites, 2010-2014...
2018: PloS One
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
#18
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
#19
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/29338622/the-grief-and-coping-of-parents-whose-child-has-a-constant-life-threatening-disability-hypoplastic-left-heart-syndrome-with-reference-to-the-dual-process-model
#20
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