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https://www.readbyqxmd.com/read/28689327/in-utero-evidence-of-impaired-somatic-growth-in-hypoplastic-left-heart-syndrome
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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...
June 12, 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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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.
May 17, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28446969/micrornas-pleiotropic-players-in-congenital-heart-disease-and-regeneration
#13
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
https://www.readbyqxmd.com/read/28444210/initial-shunt-type-at-the-norwood-operation-impacts-myocardial-function-in-hypoplastic-left-heart-syndrome%C3%A2
#14
Hanna K Ruotsalainen, Jaana Pihkala, Jukka Salminen, Lisa K Hornberger, Heikki Sairanen, Tiina Ojala
OBJECTIVES: We investigated the impact of initial shunt type, a Blalock-Taussig (BT) shunt versus a right ventricle to pulmonary artery conduit (RV-PA) on myocardial function at different stages of surgical palliation in patients with hypoplastic left heart syndrome (HLHS). METHODS: A population-based cohort of 63 Finnish children with HLHS (BT n  = 23, RV-PA n  = 40) born between 2003 and 2010 were studied retrospectively by echocardiography prior to Stages 1, 2 and 3 palliation and 0...
April 24, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28421915/use-of-a-modified-classic-bt-shunt-in-a-patient-with-multiple-conduit-obstructions-with-positive-lupus-anticoagulant
#15
Pallav Bhattarai, Ayesha Zia, Joseph M Forbess, Kristine J Guleserian, Susan R Hupp, Timothy Pirolli
The use of a right ventricle-to-pulmonary artery conduit has re-emerged as a popular alternative to a systemic artery-to-pulmonary artery shunt in the Norwood procedure. Both proximal obstruction secondary to dynamic compression and distal obstruction at the anastomosis site with the pulmonary arteries are well described. In an effort to prevent complications, a technique where in the conduit is placed through the entire full thickness, or dunked, through the RV free wall has been described. We report a case of a patient with HLHS and positive lupus anticoagulant who developed recurrent conduit obstructions...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28393581/the-effect-of-transport-on-the-physiologic-stability-of-neonates-withductal-dependent-single-ventricle-lesions
#16
Silvestre R Duran, Sanjeev Aggarwal, Girija Natarajan
OBJECTIVE: To compare the status of infants with hypoplastic left heart syndrome (HLHS) or pulmonary atresia-hypoplastic right heart (PA-HRH) before and following transport using the validated Transport Risk Index of Physiologic Stability (TRIPS) score. METHODS: In this retrospective review of infants with HLHS or PA-HRH transported to a Children's Hospital by a pediatric transport team, an increase in TRIPS score (temperature, blood pressure, respiratory status, and response to stimuli) following transport was defined as deterioration...
April 10, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28359493/twin-to-twin-heart-transplantation-a-unique-event-with-a-25-year-follow-up
#17
David Blitzer, Grace Yedlicka, Joshua Manghelli, John Dentel, Randall Caldwell, John W Brown
Solid organ transplantation in pediatric patients has been a reality since 1954, when the first kidney transplantation was successfully performed between identical twins. We report the long-term outcomes, with more than 25 years of follow-up, in a patient born with hypoplastic left heart syndrome (HLHS) who received a heart transplant from a dizygotic twin. While we would not wish for this situation to reoccur, we hope that in reporting it, we can add to the discussion surrounding pediatric heart transplantation and the management of HLHS...
April 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28345115/counseling-practices-for-fetal-hypoplastic-left-heart-syndrome
#18
Michael J Walsh, George R Verghese, M Eric Ferguson, Nora F Fino, David J Goldberg, Sonal T Owens, Nelangi Pinto, Sinai C Zyblewski, Michael D Quartermain
While counseling parents of a fetus diagnosed with hypoplastic left heart syndrome (HLHS), pediatric cardiologists play a critical role in shaping a family's expectations for the months and years to come. However, techniques for the most effective counseling practices have not been studied, and significant variation among physicians is likely present. Web-based survey of pediatric cardiologists that perform fetal echocardiography using snowball sampling. 201 physicians responded (61% male, 81% from academic centers, and 95% from the U...
March 27, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28341901/hypoplastic-left-heart-syndrome-is-not-associated-with-worse-clinical-or-neurodevelopmental-outcomes-than-other-cardiac-pathologies-after-the-norwood-sano-operation
#19
Billie-Jean Martin, I De Villiers Jonker, Ari R Joffe, Gwen Y Bond, Bryan V Acton, David B Ross, Charlene M T Robertson, Ivan M Rebeyka, Joseph Atallah
There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood-Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database...
March 24, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28323386/the-association-of-maternal-lymphatic-markers-and-critical-congenital-heart-defects-in-the-fetus-a-population-based-case-control-study
#20
Martina A Steurer, Mary E Norton, Rebecca J Baer, Gary M Shaw, Sheila Keating, Anita J Moon-Grady, Christina D Chambers, Laura L Jelliffe-Pawlowski
The objective ot this study was to investigate whether lymphatic markers measured in women during the second trimester are associated with critical congenital heart defects (CCHDs) in offspring. This is a retrospective cohort study of pregnant women who participated in the California Prenatal Screening Program. CCHD data in the offspring was captured by linking birth certificate data with hospital patient discharge records. Second trimester samples were assayed for vascular endothelial growth factor (VEGF), platelet derived growth factor (PDGF) AA/BB, and PDGF AB...
May 2017: American Journal of Medical Genetics. Part A
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