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https://www.readbyqxmd.com/read/28329460/model-based-comparison-of-the-normal-and-fontan-circulatory-systems-part-iii
#1
Raymond L Watrous, Alvin J Chin
BACKGROUND: For patients with the Fontan circulatory arrangement, angiotensin-converting enzyme inhibition, guanylate cyclase activation, phosphodiesterase 5 inhibition, and endothelin receptor antagonism have so far resulted in little or no improvement in [Formula: see text] or peak cardiac index (CI), suggesting that our understanding of the factors that most impact the exercise hemodynamics is incomplete. METHODS: To facilitate comparisons with clinical reports of the exercise performance of preadolescent Fontan patients, we rescaled our previously reported computational models of a two-year-old normal child and similarly aged Fontan patient, extended our Fontan model to capture the nonlinear relationship between flow and resistance quantified from previous computational fluid dynamic analyses of the total cavopulmonary connection (TCPC), and added respiration as well as skeletal muscle contraction...
March 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28329109/impact-of-early-fontan-completion-on-postoperative-outcomes-in-patients-with-a-functional-single-ventricle%C3%A2
#2
Masamichi Ono, Melchior Burri, Julie Cleuziou, Jelena Pabst von Ohain, Elisabeth Beran, Martina Strbad, Alfred Hager, Jürgen Hörer, Christian Schreiber, Rüdiger Lange
OBJECTIVES: Our aim was to evaluate whether early timing of total cavopulmonary connection (TCPC) affects postoperative outcomes. METHODS: Of 460 consecutive TCPC patients, 51 (11.1%) underwent TCPC ≤ 18 months of age (group A), and 409 patients >18 months of age (group B). We compared the clinical outcomes and exercise capacity between groups. RESULTS: Median age at TCPC was 1.4 (interquartile ranges: 1.3-1.5) years in group A and 2...
February 23, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28329058/improved-long-term-outcomes-in-double-inlet-left-ventricle-and-tricuspid-atresia-with-transposed-great-arteries-systemic-outflow-tract-obstruction-present-at-birth-defines-long-term-outcome
#3
Lotte C Franken, Manouk Admiraal, Charlotte E Verrall, Diana Zannino, Julian G Ayer, Ajay J Iyengar, Andrew D Cole, Gary F Sholler, Yves D'Udekem, David S Winlaw
OBJECTIVES: In 2 subtypes of functional single ventricle, double inlet left ventricle (DILV) and tricuspid atresia with transposed great arteries (TA-TGA), systemic output passes through an outflow chamber before entering the aorta. Intracardiac obstruction to this pathway causing systemic outflow tract obstruction (SOTO) may be present at birth or develop over time. Long-term survival after Fontan has not been defined. We defined outcomes utilizing records from 2 centres that were cross-checked with data from a bi-national Fontan Registry for completeness and accuracy...
March 4, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28320993/intractable-back-pain-after-coil-embolization-of-giant-veno-venous-collaterals-in-a-patient-with-fontan-circulation
#4
Seigo Okada, Masahiro Kamada, Naomi Nakagawa, Yukiko Ishiguchi, Yuji Moritoh, Mayuko Shohi, Kengo Okamoto, Shunji Hasegawa, Shouichi Ohga
Veno-venous collaterals are sometimes seen in patients after the Fontan procedure. A 28-year-old female with tricuspid atresia who underwent the Fontan procedure had oxygen desaturation due to a giant veno-venous collateral. Coil embolization was performed for the collateral. After the procedure, she complained of severe back pain. Anti-inflammatory analgesics and steroids were not effective, although carbamazepine promptly relieved the intractable pain. Treatment-related pain after coil embolization for veno-venous collaterals in patients with Fontan physiology is quite rare, although cardiologists must recognize a critical condition to be differentiated from vascular occlusion...
March 21, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28319522/a-numerical-simulation-comparing-a-cavopulmonary-assist-device-and-va-ecmo-for-failing-fontan-support
#5
Po-Lin Hsu, Dongfang Wang, Cherry Ballard-Croft, Dawei Xiao, Joseph B Zwischenberger
A cavopulmonary assist device (CPAD) has been developed for failing Fontan support. This CPAD pumps blood from superior/inferior vena cavae to pulmonary artery. In this study, we compared failing Fontan support with CPAD versus VA ECMO in silico. A numerical lumped-parameter circulatory system model was used to simulate Fontan circulation. Failing Fontan was simulated by increased pulmonary resistance. Central venous pressure (CVP), mean pulmonary arterial pressure (mPAP), left atrial pressure (LAP), and univentricular outflow (CO) were simulated and compared to published clinical data...
March 17, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28314526/what-is-the-best-material-for-extracardiac-fontan-operation
#6
EDITORIAL
Toshiharu Shinoka
No abstract text is available yet for this article.
February 21, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28314184/how-does-the-body-representation-system-develop-in-the-human-brain
#7
Aurelie Fontan, Fabien Cignetti, Bruno Nazarian, Jean-Luc Anton, Marianne Vaugoyeau, Christine Assaiante
Exploration of the body representation system (BRS) from kinaesthetic illusions in fMRI has revealed a complex network composed of sensorimotor and frontoparietal components. Here, we evaluated the degree of maturity of this network in children aged 7-11 years, and the extent to which structural factors account for network differences with adults. Brain activation following tendon vibration at 100Hz ('illusion') and 30Hz ('no illusion') were analysed using the two-stage random effects model, with or without white and grey matter covariates...
February 28, 2017: Developmental Cognitive Neuroscience
https://www.readbyqxmd.com/read/28297630/genomics-of-multiple-myeloma
#8
Sebastien Robiou du Pont, Alice Cleynen, Charlotte Fontan, Michel Attal, Nikhil Munshi, Jill Corre, Hervé Avet-Loiseau
Multiple myeloma (MM) is characterized by wide variability in the chromosomal/genetic changes present in tumor plasma cells. Genetically, MM can be divided into two groups according to ploidy and hyperdiploidy versus nonhyperdiploidy. Several studies in gene expression profiling attempted to identify subentities in MM without convincing results. These studies mostly confirmed the cytogenetic data and subclassified patients according to 14q32 translocations and ploidy. More-recent data that are based on whole-exome sequencing have confirmed this heterogeneity and show many gene mutations but without a unifying mutation...
March 20, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28292590/fontan-failure-associated-with-a-restrictive-systemic-ventricle
#9
Joeri Van Puyvelde, Erik Verbeken, Marc Gewillig, Bart Meyns
No abstract text is available yet for this article.
February 15, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28291606/geometry-and-growth-of-the-reconstructed-aorta-in-patients-with-hypoplastic-left-heart-syndrome-and-variants
#10
Christoph Haller, Devin Chetan, Arezou Saedi, Rachel Parker, Glen S Van Arsdell, Osami Honjo
OBJECTIVE: The interdigitating technique in aortic arch reconstruction in hypoplastic left heart syndrome and variants (HLHS) reduces the recoarctation rate. Little is known on aortic arch growth characteristics and resulting clinical impact. METHODS: A total of 139 patients with HLHS underwent staged palliation between 2007 and 2014; 73 patients underwent arch reconstruction. Dimensions of ascending aorta, transverse arch, interdigitating anastomosis, and descending aorta in pre-stage II and pre-Fontan angiograms were measured...
February 14, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28284501/cardiopulmonary-exercise-test-in-adults-with-prior-fontan-operation-the-prognostic-value-of-serial-testing
#11
Alexander C Egbe, David J Driscoll, Arooj R Khan, Sameh S Said, Emmanuel Akintoye, Fernando M Berganza, Heidi M Connolly
BACKGROUND: The purpose of the study was to determine the role of cardiopulmonary exercise test (CPET) indices in predicting cardiovascular adverse events (CAEs) in patients with Fontan palliation. CAE was defined as death or cardiac surgery. METHODS: Retrospective review of adult Fontan patients who had treadmill CPET at Mayo Clinic, 1994-2013. Patients with loss of follow-up defined as ≥2years without clinical follow-up were excluded. The results of serial CPETs were reviewed, and patients with CPETs meeting the following criteria were selected for analysis: maximum effort on serial CPETs, minimum of 3-year interval between CPETs, and absence of CAE between CPETs...
February 28, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28283234/endothelin-inhibitors-lower-pulmonary-vascular-resistance-and-improve-functional-capacity-in-patients-with-fontan-circulation
#12
Gabriella Agnoletti, Simona Gala, Francesca Ferroni, Roberto Bordese, Lorenzo Appendini, Carlo Pace Napoleone, Laura Bergamasco
OBJECTIVES: To evaluate the effects of endothelin inhibitors (ERAs) on hemodynamic and functional parameters in patients post-Fontan procedure with high pulmonary vascular resistance (PVR). METHODS: Among our cohort of patients with Fontan circulation, 8 children, 8 adolescents, and 8 adults had PVR ≥2 WU*m(2). These patients were treated with ERAs (minors with bosentan, adults with macitentan) and reevaluated after 6 months. Pre- and posttreatment hemodynamic variables were assessed by cardiac catheterization...
February 10, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28281411/abnormalities-in-serum-biomarkers-correlate-with-lower-cardiac-index-in-the-fontan-population
#13
MULTICENTER STUDY
Bradley S Marino, David J Goldberg, Adam L Dorfman, Eileen King, Heidi Kalkwarf, Babette S Zemel, Margaret Smith, Jesse Pratt, Mark A Fogel, Amanda J Shillingford, Barbara J Deal, Anitha S John, Caren S Goldberg, Timothy M Hoffman, Marshall L Jacobs, Asher Lisec, Susan Finan, Lazaros K Kochilas, Thomas W Pawlowski, Kathleen Campbell, Clinton Joiner, Stuart L Goldstein, Paul Stephens, Alvin J Chin
BACKGROUND: Fontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients. Methods and results This study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance...
January 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28280718/response-to-comment-on-assessment-of-liver-stiffness-in-pediatric-fontan-patients-using-transient-elastography
#14
COMMENT
Becky Chen, Richard A Schreiber, Derek G Human, James E Potts, Orlee R Guttman
No abstract text is available yet for this article.
2017: Canadian Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28277152/visuospatial-processing-in-adolescents-with-critical-congenital-heart-disease-organization-integration-and-implications-for-academic-achievement
#15
Jessica L Bean Jaworski, Matthew T White, David R DeMaso, Jane W Newburger, David C Bellinger, Adam R Cassidy
Among the most significant factors affecting quality of life in individuals with critical congenital heart disease (CCHD) are neurodevelopmental challenges, including deficits in visuospatial processing and academic achievement. Few studies have compared outcomes across CCHD subgroups, despite their significant differences in anatomy/physiology and medical/surgical courses. This study compared visuospatial processing abilities using the Developmental Scoring System for the Rey-Osterrieth Complex Figure (DSS-ROCF) across groups of adolescents with CCHD (d-transposition of the great arteries [TGA, n = 139], Tetralogy of Fallot [TOF, n = 68], single-ventricle cardiac anatomy requiring the Fontan operation [SVF, n = 145]) and a group of healthy controls (CTR, n = 111), and examined the validity of visuospatial processing in predicting concurrent academic outcomes...
February 16, 2017: Child Neuropsychology: a Journal on Normal and Abnormal Development in Childhood and Adolescence
https://www.readbyqxmd.com/read/28275561/computed-tomography-in-congenital-heart-disease-how-generic-principles-can-be-applied-to-create-bespoke-protocols-in-the-fontan-circuit
#16
REVIEW
Will W Loughborough, Michael Yeong, Mark Hamilton, Nathan Manghat
Cardiac computed tomography (CCT) has become an invaluable cross-sectional imaging modality in congenital heart disease (CHD) patients. However, altered anatomical connections and cardiovascular physiology makes CHD arguably the most challenging area in CCT imaging, which remains a complimentary modality to cardiac magnetic resonance and echocardiography. A bespoke CT protocol is often required to achieve a diagnostic examination; this can be achieved through careful consideration of the basic principles of image acquisition and contrast administration...
February 2017: Quantitative Imaging in Medicine and Surgery
https://www.readbyqxmd.com/read/28271152/arrhythmias-after-fontan-operation-with-intra-atrial-lateral-tunnel-versus-extra-cardiac-conduit-a-systematic-review-and-meta-analysis
#17
Dongxu Li, Qiang Fan, Yasutaka Hirata, Minoru Ono, Qi An
Current studies on the incidence of arrhythmias after the intra-atrial lateral tunnel (ILT) Fontan operation and the extra-cardiac conduit (ECC) Fontan operation are limited, with controversial results. This systematic review aimed to compare the prevalence of arrhythmias in patients who underwent ECC or ILT Fontan. Relevant studies comparing the incidence of arrhythmias and pacemaker implantation in ILT with ECC were identified through a literature search using MEDLINE, EMBASE, and the cochrane central register of controlled trials...
March 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28265820/how-a-bottom-up-multi-stakeholder-initiative-helped-transform-the-renal-replacement-therapy-landscape-in-spain
#18
Rafael Selgas, Laura Rodriguez, Juan Carlos Julian, Cesar Remon, Mario Prieto-Velasco, Javier Perez-Contreras, Miguel Perez Fontan
Healthcare reforms aim to change certain parts of the health system to improve quality of care, access, or financial sustainability. Traditionally, healthcare reform is understood as an action undertaken by a government at a national or local level. However, bottom-up changes can also lead to improvements in the health system. This paper describes the efforts of a coordinated multi-stakeholder advocacy group in Spain to promote a more cost-effective and patient-centred treatment for people receiving renal replacement therapy and assesses the outcomes of their advocacy for health system financing and patient satisfaction...
March 6, 2017: Applied Health Economics and Health Policy
https://www.readbyqxmd.com/read/28262297/femoral-vein-homograft-as-right-ventricle-to-pulmonary-artery-conduit-in-stage-1-norwood-operation
#19
T K Susheel Kumar, Mario Briceno-Medina, Shyam Sathanandam, Vijaya M Joshi, Christopher J Knott-Craig
BACKGROUND: The polytetrafluoroethylene tube used as right ventricle to pulmonary artery conduit in the stage 1 Norwood operation is associated with risks of suboptimal branch pulmonary artery growth, thrombosis, free insufficiency, and long-term right ventricular dysfunction. Our experience with use of valved femoral vein homograft as right ventricle to pulmonary artery conduit is described. METHODS: Between June 2012 and December 2015, 15 neonates with hypoplastic left heart syndrome or complex single ventricle underwent stage 1 Norwood operation with valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit...
March 2, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28262200/outcomes-of-direct-current-cardioversion-in-adults-with-congenital-heart-disease
#20
Alexander C Egbe, Samuel J Asirvatham, Heidi M Connolly, Suraj Kapa, Christopher V Desimone, Vaibhav R Vaidya, Abhishek J Deshmukh, Arooj R Khan, Christopher J McLeod, Rowlens M Melduni, Naser M Ammash
Few data exist on direct current cardioversion (DCCV) in adult patients with congenital heart disease (CHD). This is a retrospective case-control study of 279 adults with CHD and 279 adults without CHD (control group) who had elective DCCV for atrial arrhythmias at Mayo Clinic, 2001 to 2013. Control patients were matched by gender and arrhythmia type. The objective was to compare DCCV procedural failure (failure to terminate the presenting arrhythmia) and arrhythmia recurrence (AR). In the CHD group (mean age 55 ± 20 years; men 166 [59%]), the most common diagnosis was Fontan palliation (61; 22%)...
February 9, 2017: American Journal of Cardiology
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