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Hypoplastic heart

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https://www.readbyqxmd.com/read/28732025/hand-factor-ablation-causes-defective-left-ventricular-chamber-development-and-compromised-adult-cardiac-function
#1
Joshua W Vincentz, Kevin P Toolan, Wenjun Zhang, Anthony B Firulli
Coordinated cardiomyocyte growth, differentiation, and morphogenesis are essential for heart formation. We demonstrate that the bHLH transcription factors Hand1 and Hand2 play critical regulatory roles for left ventricle (LV) cardiomyocyte proliferation and morphogenesis. Using an LV-specific Cre allele (Hand1LV-Cre), we ablate Hand1-lineage cardiomyocytes, revealing that DTA-mediated cardiomyocyte death results in a hypoplastic LV by E10.5. Once Hand1-linage cells are removed from the LV, and Hand1 expression is switched off, embryonic hearts recover by E16...
July 21, 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28711428/-congenital-heart-disease-mortality-in-spain-during-a-10-year-period-2003-2012
#2
Javier Pérez-Lescure Picarzo, Margarita Mosquera González, Pello Latasa Zamalloa, David Crespo Marcos
INTRODUCTION AND OBJECTIVES: Congenital heart disease is a major cause of infant mortality in developed countries. In Spain, there are no publications at national level on mortality due to congenital heart disease. The aim of this study is to analyse mortality in infants with congenital heart disease, lethality of different types of congenital heart disease, and their variation over a ten-year period. METHODS: A retrospective observational study was performed to evaluate mortality rate of children under one year old with congenital heart disease, using the minimum basic data set, from 2003 to 2012...
July 12, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28706241/abnormal-paraventricular-nucleus-of-hypothalamus-and-growth-retardation-associated-with-loss-of-nuclear-receptor-gene-coup-tfii
#3
Su Feng, Can Xing, Tingyu Shen, Yunbo Qiao, Ran Wang, Jun Chen, Jiaoyang Liao, Zhuo Lu, Xiong Yang, Saber Mohamed Abd-Allah, Jinsong Li, Naihe Jing, Ke Tang
The paraventricular nucleus of hypothalamus plays important roles in the regulation of energy balance and fetal growth. However, the molecular mechanisms underlying its formation and function have not been clearly elucidated. Various mutations in the human COUP-TFII gene, which encodes a nuclear receptor, result in growth retardation, congenital diaphragmatic hernia and congenital heart defects. Here, we show that COUP-TFII gene is expressed in the developing hypothalamus in mouse. The ventral forebrain-specific RXCre/+; COUP-TFII (F/F) mutant mice display growth retardation...
July 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28698113/patient-specific-biomechanical-model-of-hypoplastic-left-heart-to-predict-post-operative-cardio-circulatory-behaviour
#4
Elena Cutrì, Alessio Meoli, Gabriele Dubini, Francesco Migliavacca, Tain-Yen Hsia, Giancarlo Pennati
Hypoplastic left heart syndrome is a complex congenital heart disease characterised by the underdevelopment of the left ventricle normally treated with a three-stage surgical repair. In this study, a multiscale closed-loop cardio-circulatory model is created to reproduce the pre-operative condition of a patient suffering from such pathology and virtual surgery is performed. Firstly, cardio-circulatory parameters are estimated using a fully closed-loop cardio-circulatory lumped parameter model. Secondly, a 3D standalone FEA model is build up to obtain active and passive ventricular characteristics and unloaded reference state...
July 8, 2017: Medical Engineering & Physics
https://www.readbyqxmd.com/read/28693354/perinatal-outcomes-of-congenital-heart-disease-after-emergent-neonatal-cardiac-procedures
#5
Aline Wolter, Helene Holtmann, Andreea Kawecki, Jan Degenhardt, Christian Enzensberger, Oliver Graupner, Hakan Akintürk, Can Yerebakan, Markus Khalil, Dietmar Schranz, Roland Axt-Fliedner
PURPOSE: We compared outcome of neonates with prenatal and postnatal diagnosis of congenital heart disease presenting in our paediatric heart centre between 2005-05/2015 who underwent an emergent intervention within 48 hours postpartum. MATERIALS AND METHODS: In 52/111 (46.8%) with emergent intervention, congenital heart disease was diagnosed prenatally, in 59/111 (53.2%) with no specialized fetal echocardiography, diagnosis was made postnatally. In 98/111 (88...
July 10, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28689327/in-utero-evidence-of-impaired-somatic-growth-in-hypoplastic-left-heart-syndrome
#6
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/28687711/the-optimal-timing-of-stage-2-palliation-for-hypoplastic-left-heart-syndrome-an-analysis-of-the-pediatric-heart-network-single-ventricle-reconstruction-trial-public-dataset
#7
James M Meza, Edward J Hickey, Eugene H Blackstone, Robert D B Jaquiss, Brett R Anderson, William G Williams, Sally Cai, Glen S Van Arsdell, Tara Karamlou, Brian W McCrindle
Background -In infants requiring three-stage single ventricle palliation for hypoplastic left heart syndrome, attrition after the Norwood procedure remains significant. The effect of the timing of stage-2-palliation (S2P), a physician-modifiable factor, on long term survival is not well understood. We hypothesized that an optimal interval between the Norwood and S2P that both minimizes pre-S2P attrition and maximizes post-S2P survival exists and is associated with individual patient characteristics. Methods -The NIH/NHLBI Pediatric Heart Network Single Ventricle Reconstruction Trial public dataset was used...
July 7, 2017: Circulation
https://www.readbyqxmd.com/read/28684000/management-of-tricuspid-regurgitation-in-patients-with-hypoplastic-left-heart-syndrome
#8
Frank A Pigula, Bret Mettler
Tricuspid valve (TV) performance is critical for palliation of hypoplastic left heart syndrome. We will review current TV repair techniques, outcomes, and novel approaches.
April 2017: Seminars in Thoracic and Cardiovascular Surgery
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/28677480/preoperative-venovenous-ecmo-in-an-infant-with-late-diagnosis-of-hypoplastic-left-heart-syndrome
#10
Lena K Eschenbach, Jelena Kasnar-Samprec, Kilian Ackermann, Christian Schreiber, Rudiger Lange, Julie Cleuziou
Late diagnosis and late referral for the Norwood stage 1 procedure in patients with hypoplastic left heart syndrome is rare and associated with a higher mortality. We present a case of a cyanotic almost five-week-old infant with hypoplastic left heart syndrome, highly restrictive foramen ovale, and patent ductus arteriosus, who was bridged with venovenous extracorporeal membrane oxygenation to the Norwood stage 1 procedure.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28674724/infantile-scimitar-syndrome-with-unusual-associations
#11
Abullah Al-Shamrani, Reem S AlSadi, Motea E Elhoury, Adel S AlHarbi
Scimitar syndrome is a variant of partial anomalous pulmonary venous connection (PAPVC), in which all or part of the right lung is drained by right pulmonary veins that anomalously connect to the inferior vena cava (IVC). The affected lung and its associated airways are often hypoplastic. In addition, aortopulmonary collateral vessels may be involved on the affected side, causing sequestration of that side; such involvement is commonly associated with cardiac defects. We report a case of infantile scimitar syndrome that involved a typical association with the right lung, but with extremely unusual associations with congenital hydrocephalus and heart blockage...
July 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28667405/utilizing-hybrid-techniques-to-maximize-clinical-outcomes-in-congenital-heart-disease
#12
REVIEW
David W Bearl, Gregory A Fleming
PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital cardiology. Recent collaborations between interventional cardiologists and cardiac surgeons have sparked novel innovative hybrid procedures that provide potentially safer, faster, and less invasive alternatives to the management of many diseases. This paper will review the most recent advances in hybrid interventions in the field of pediatric and adult congenital cardiology...
August 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28661741/hypoplastic-left-heart-syndrome-the-case-against-palliative-surgery
#13
Gwen E Erkonen, Robert A Hanfland
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28661739/growing-discomfort-with-comfort-care-for-hypoplastic-left-heart-syndrome-why-we-should-still-defer-to-parental-wishes
#14
Erin A Paul, Elizabeth M Kohlberg, Kristina Orfali
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28661733/the-ethics-of-treatment-for-hypoplastic-left-heart-syndrome-hlhs
#15
Jeffrey P Spike
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28661728/may-we-take-our-baby-with-hypoplastic-left-heart-syndrome-home
#16
Mary Devereaux, Alexander A Kon
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28658842/a-case-series-of-young-patients-with-completely-reversed-severe-pulmonary-hypertension
#17
Aniketh Vijay Balegadde, Vikrant Vijan, Rajesh Thachathodiyl
Pulmonary Arterial Hypertension (PAH) is a progressive symptomatic disorder, which may ultimately lead to death if left untreated. Although majority of PAH cases are idiopathic, pulmonary hypertension resulting due to certain underlying conditions are also observed frequently. In such cases, it becomes essential to identify any potentially treatable or reversible causes for PAH. There have been significant advances in the medical management of PAH and various medicines have been approved by US Food and Drug Administration (FDA) for various stages of PAH...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28643402/clinical-findings-in-right-ventricular-noncompaction-in-hypoplastic-left-heart-syndrome
#18
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/28631156/diagnosis-of-secondary-pulmonary-lymphangiectasia-in-congenital-heart-disease-a-novel-role-for-chest-ultrasound-and-prognostic-implications
#19
Christopher Z Lam, Tanmay Anant Bhamare, Tamadhir Gazzaz, David Manson, Tilman Humpl, Mike Seed
BACKGROUND: Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction. OBJECTIVES: We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia. MATERIALS AND METHODS: Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study...
June 19, 2017: Pediatric Radiology
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
#20
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
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