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https://www.readbyqxmd.com/read/29154821/heterotaxy-syndrome-and-associated-arrhythmias-in-pediatric-patients
#1
Mary C Niu, Heather A Dickerson, Judson A Moore, Caridad de la Uz, Santiago O Valdés, Jeffrey J Kim, David E Bard, Shaine A Morris, Christina Y Miyake
BACKGROUND: Heterotaxy Syndrome (HS) is a rare disorder with complex anatomy involving misarrangements of the cardiac conduction system. Arrhythmias may be related to anatomic variations and contribute to morbidity. OBJECTIVE: This study investigated the associations between arrhythmias, anatomy, and outcomes in a large HS cohort. METHODS: A single center retrospective review of patients diagnosed with HS ≤ 21 years of age was performed...
November 14, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29148256/toward-standardization-of-care-the-feeding-readiness-assessment-after-congenital-cardiac-surgery
#2
Daniel E Ehrmann, Matthew Mulvahill, Shaunda Harendt, Jessica Church, Amy Stimmler, Piyagarnt Vichayavilas, Sanja Batz, Jennifer Rodgers, Michael DiMaria, James Jaggers, Cindy Barrett, Jon Kaufman
BACKGROUND: Feeding practices after neonatal and congenital heart surgery are complicated and variable, which may be associated with prolonged hospitalization length of stay (LOS). Systematic assessment of feeding skills after cardiac surgery may earlier identify those likely to have protracted feeding difficulties, which may promote standardization of care. METHODS: Neonates and infants ≤3 months old admitted for their first cardiac surgery were retrospectively identified during a 1-year period at a single center...
November 17, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/29148248/recurrent-coarctation-of-aorta-after-norwood-operation-in-single-ventricle-patients-the-case-of-the-missing-ideal-stent
#3
EDITORIAL
Dhaval R Parekh, Zvonimir Krajcer
Recurrent coarctation postoperative Norwood is poorly tolerated in single ventricle patients. Although transcatheter balloon angioplasty is considered first line, it often leads to inadequate or non-durable relief of obstruction. Transcatheter stent implantation provides excellent relief of obstruction but necessitates staged redilation to account for somatic growth in children. Stent technology, delivery systems, and procedural techniques will need to evolve to obviate reported adverse events mainly due to patient size...
November 15, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/29147786/risk-factors-for-development-of-ectopic-atrial-tachycardia-in-post-operative-congenital-heart-disease
#4
Bradley C Clark, John T Berger, Charles I Berul, Richard A Jonas, Jonathan R Kaltman, Julianne Lapsa, Dilip S Nath, Elizabeth D Sherwin, Pranava Sinha, David Zurakowski, Jeffrey P Moak
Ectopic atrial tachycardia (EAT) is common in surgically repaired congenital heart disease (CHD) and carries the potential for significant hemodynamic compromise. Our objective was to determine the incidence, and risk factors of EAT after CHD surgery. Prospective study of patients that underwent surgery for CHD from February to October 2016 was performed. Demographic, perioperative and electrophysiologic data were collected. Sustained EAT (> 30 s) was documented by telemetry or electrocardiogram and confirmed by a pediatric electrophysiologist...
November 17, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29124022/outcomes-of-extracorporeal-membrane-oxygenation-in-children-an-11-year-single-center-experience-in-korea
#5
Hongsun Kim, Ji-Hyuk Yang, Yang Hyun Cho, Tae-Gook Jun, Kiick Sung, Woosik Han
Background: Extracorporeal membrane oxygenation (ECMO) has become an important treatment modality in pediatric patients with cardiopulmonary failure, but few studies have been conducted in Korea. Methods: We conducted a retrospective review of pediatric patients younger than 18 years who were placed on ECMO between January 2004 and December 2014 at Samsung Medical Center. Results: We identified 116 children on ECMO support. The overall rate of successful weaning was 51...
October 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29116689/evaluation-of-hemodynamic-performance-of-total-cavopulmonary-connection-tcpc-with-porous-inserts
#6
K Dhayananth, Arunn Narasimhan
Infants born with univentricular heart disease undergo Fontan surgery in order to establish separate systemic and pulmonary circulations. This surgery results in better blood circulation across a single ventricle that supplies oxygenated blood to the body and passively returns venous blood to the lungs through the total cavopulmonary connection (TCPC). Reducing the pressure drop across the TCPC during Fontan circulation helps in reducing the work load of univentricular heart and various designs have been proposed for this purpose...
November 8, 2017: International Journal for Numerical Methods in Biomedical Engineering
https://www.readbyqxmd.com/read/29111297/mid-term-outcomes-of-repair-of-coarctation-of-aorta-with-hypoplastic-arch-extended-end-to-side-anastomosis-technique
#7
Eung Re Kim, Woong-Han Kim, Jinhae Nam, Kwangho Choi, Woo Sung Jang, Jae Gun Kwak
OBJECTIVE: The optimal surgical repair technique for coarctation associated with aortic arch hypoplasia (CoA/AAH) in neonates and infants is controversial. This study evaluates our current strategy utilizing extended end-to-side anastomosis (EESA) under selective cerebral and myocardial perfusion (SCMP) in treating this group of patients. METHODS: Through a retrospective review, we analyzed the outcome of 87 infants who underwent surgical repair of CoA/AAH from January 2004 to December 2015...
October 27, 2017: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29105891/aortopulmonary-collateral-flow-quantification-by-mr-at-rest-and-during-continuous-submaximal-exercise-in-patients-with-total-cavopulmonary-connection
#8
Naira Mkrtchyan, Yvonne Frank, Eva Steinlechner, Lenika Calavrezos, Christian Meierhofer, Alfred Hager, Stefan Martinoff, Peter Ewert, Heiko Stern
BACKGROUND: Aortopulmonary collateral flow is considered to have significant impact on the outcome of patients with single ventricle circulation and total cavopulmonary connection (TCPC). There is little information on collateral flow during exercise. PURPOSE: To quantify aortopulmonary collateral flow at rest and during continuous submaximal exercise in clinical patients doing well with TCPC. STUDY TYPE: Prospective, case controlled. POPULATION: Thirteen patients with TCPC (17 (11-37) years) and 13 age and sex-matched healthy controls (18 (11-38) years)...
November 6, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29104017/neurodevelopmental-outcome-and-health-related-quality-of-life-in-children-with-single-ventricle-heart-disease-before-fontan-procedure
#9
Bettina Reich, Kristina Heye, Ruth Tuura, Ingrid Beck, Kristina Wetterling, Andreas Hahn, Karoline Hofmann, Dietmar Schranz, Hakan Akintürk, Beatrice Latal, Walter Knirsch
OBJECTIVE: Neurodevelopmental impairment and impaired quality of life constitute a major source of morbidity among children with complex congenital heart disease (CHD), in particular for single ventricle morphologies. Risk factors and quality of life determining clinical and neurodevelopmental outcome at two years of age are examined. METHODS: In a two-center cohort study, 48 patients with SV-morphology (26 HLHS, 22 other types of univentricular heart defect) have been examined prior Fontan procedure between 2010 and 2015...
November 2, 2017: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29103817/simulation-of-single-ventricle-physiology-%C3%A2-a-start
#10
EDITORIAL
M'hamed Hamy Temkit, Tara Karamlou
No abstract text is available yet for this article.
October 20, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29096870/hypoplastic-left-heart-syndrome-is-not-a-predictor-of-worse-intermediate-mortality-post-fontan
#11
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...
October 30, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29071790/stress-and-quality-of-life-among-parents-of-children-with-congenital-heart-disease-referred-for-psychological-services
#12
Astrida Kaugars, Clarissa Shields, Cheryl Brosig
OBJECTIVE: The study examined parent stress and health-related quality of life (HRQOL) among families of children with congenital heart disease (CHD) referred for psychological services. METHODS: Parents of 54 children (85% boys) aged 3 to 13 (Mage  = 7.48, SD = 2.38) completed measures to assess parenting stress (Parenting Stress Index - Short Form; Pediatric Inventory for Parents) and the PedsQL Family Impact Module. Medical information was retrieved from medical record review...
October 25, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/29071373/longitudinal-myocardial-deformation-does-not-predict-single-ventricle-ejection-fraction-assessed-by-cardiac-magnetic-resonance-imaging-in-children-with-a-total-cavopulmonary-connection
#13
L P Koopman, L M Geerdink, S S M Bossers, N Duppen, I M Kuipers, A D Ten Harkel, G van Iperen, G Weijers, C de Korte, W A Helbing, L Kapusta
Survival of children with single ventricle heart defects after the total cavopulmonary connection (TCPC) has improved, but impaired cardiac function remains a major cause of morbidity and mortality. Cardiac magnetic resonance imaging (cMRI) is the gold standard in assessing single ventricle volume and function, but high costs and limited availability hamper its routine use. A cheaper and more available alternative is echocardiography. Myocardial function can be studied in more detail using speckle tracking echocardiography (STE)...
October 25, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29061637/unique-developmental-trajectories-and-genetic-regulation-of-ventricular-and-outflow-tract-progenitors-in-the-zebrafish-second-heart-field
#14
Noelle Paffett-Lugassy, Natasha Novikov, Spencer Jeffrey, Maryline Abrial, Burcu Guner-Ataman, Srinivasan Sakthivel, Caroline E Burns, C Geoffrey Burns
During mammalian embryogenesis, cardiac progenitor cells constituting the second heart field (SHF) give rise to the right ventricle and primitive outflow tract (OFT), the latter of which is particularly vulnerable to developmental malformations that underlie common congenital heart defects. In zebrafish, SHF progenitors produce half of the single ventricle's myocardium and contribute significantly to the OFT. Previous lineage tracing and mutant analyses suggested that SHF ventricular and OFT progenitors co-migrate to the arterial pole of the zebrafish heart tube soon after their specification in the nkx2...
October 23, 2017: Development
https://www.readbyqxmd.com/read/29061467/an-interactive-simulation-tool-for-patient-specific-clinical-decision-support-in-single-ventricle-physiology
#15
Timothy Conover, Anthony M Hlavacek, Francesco Migliavacca, Ethan Kung, Adam Dorfman, Richard S Figliola, Tain-Yen Hsia
OBJECTIVE: Modeling of single-ventricle circulations has yielded important insights into their unique flow dynamics and physiology. Here we translated a state-of-the-art mathematical model into a patient-specific clinical decision support interactive Web-based simulation tool and show validation for all 3 stages of single-ventricular palliation. METHODS: Via the adoption a validated lumped parameter method, complete cardiovascular-pulmonary circulatory models of all 3 stages of single-ventricle physiology were created within a simulation tool...
September 20, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29058030/prevalence-of-coronary-artery-disease-risk-factors-and-metabolic-syndrome-in-children-with-heart-disease
#16
Adam L Ware, Paul C Young, Cindy Weng, Angela P Presson, L LuAnn Minich, Shaji C Menon
Children with acquired and congenital heart disease (CHD) are increasingly surviving to adulthood. Our aim was to determine the prevalence of coronary artery disease (CAD) risk factors in children at known risk for early CAD or with severe CHD. We recruited children (8-19 years) at risk for early CAD-Kawasaki disease (KD, N = 36) and coarctation (69) or severe CHD: > 1 cardiopulmonary bypass surgery (60), single ventricle (15), prosthetic valves (13). Anthropometric measurements, blood pressure, and fasting lipid data were compared with summaries from National Health and Nutrition Examination Survey (NHANES) publications (1999-2012)...
October 23, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29057564/the-effect-of-fetal-hemodynamics-on-fetal-growth-in-single-ventricle-and-transposition-of-the-great-arteries
#17
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/29057376/p2y12-receptor-function-and-response-to-cangrelor-in-neonates-with-cyanotic-congenital-heart-disease
#18
Elisabeth A Kaza, Matthew C Egalka, Hairu Zhou, Jianchun Chen, Denise Evans, Jayne Prats, Ruizhi Li, Scott L Diamond, Julie A Vincent, Emile A Bacha, Thomas G Diacovo
Shunt thrombosis remains a major cause of morbidity and mortality, especially during the initial palliation for single-ventricle physiology. The authors present evidence that the P2Y12 inhibitor cangrelor may fill a therapeutic void in thromboprophylaxis. They base this theory on results showing that platelets from neonatal patients with cyanotic congenital heart disease have a robust response to adenosine diphosphate and are amenable to P2Y12 inhibition with cangrelor. Unique to this study was their ability to establish drug efficacy in an avatar mouse model that permits the in vivo evaluation of human platelet-mediated thrombus formation illustrating that this P2Y12 inhibitor yields the intended biological response...
August 2017: JACC. Basic to Translational Science
https://www.readbyqxmd.com/read/29054305/scimitar-syndrome-in-children-and-adults-natural-history-outcomes-and-risk-analysis
#19
Hanjay Wang, David Kalfa, Marlon S Rosenbaum, Jonathan N Ginns, Matthew J Lewis, Julie S Glickstein, Emile A Bacha, Paul J Chai
BACKGROUND: Scimitar syndrome involves congenital anomalous pulmonary venous return to the inferior vena cava. Optimal management remains controversial. We describe the natural history of disease, nonsurgical and surgical outcomes, and risk factors for poor outcomes at our institution. METHODS: Patients with anomalous pulmonary venous return to the inferior vena cava documented on echocardiography at our institution between January 1994 and January 2015 were reviewed retrospectively...
October 18, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29051776/fontan-circulation-and-implications-for-future-reproduction
#20
REVIEW
Niharika Mehta, Srilakshmi Mitta
This article summarizes and critiques four recent publications looking at preconception counseling, pregnancy outcomes, and cardiac complications in women with history of Fontan circulation. The Fontan procedure is a palliative strategy for single-ventricle type congenital heart disease and involves passive flow of venous return into the pulmonary circulation, bypassing the ventricles. Pregnancy in these patients is not without risk and preconception counseling and contraception practices vary widely. High rates of miscarriage, prematurity, and small-for-gestational-age babies are reported...
September 2017: Obstetric Medicine
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