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https://www.readbyqxmd.com/read/27903666/managing-adult-fontan-patients-where-do-we-stand
#1
REVIEW
Paul Clift, David Celermajer
The Fontan operation is performed as a palliative procedure to improve survival in infants born with a functionally univentricular circulation. The success of the operation is demonstrated by a growing adult Fontan population that exists with this unique physiology. Late follow-up has demonstrated expected and unexpected sequelae, and has shown multisystem effects of this circulation. This review discusses the challenges of managing the late complications in terms of understanding this unique physiology and the innovative therapeutic interventions that are being investigated...
December 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27898601/fontan-fenestration-closure-prior-to-posterior-spinal-fusion-in-patients-with-single-ventricle-heart-disease
#2
Matthew C Schwartz, David Nykanen, Craig Fleishman, Hamish M Munro, Jonathan Phillips, Raymond D Knapp, Donald Felix
STUDY DESIGN: Case series. OBJECTIVE: To describe transcatheter closure of the Fontan fenestration prior to posterior spinal fusion in two children to prevent paradoxical venous air embolism during the operation. SUMMARY OF BACKGROUND DATA: Scoliosis is common among patients with single-ventricle congenital heart disease who have undergone Fontan operation and spinal surgery can offer physiologic benefits. Venous air embolism is a rare, but important reported complication during spinal surgery performed in the prone position...
December 1, 2016: Spine
https://www.readbyqxmd.com/read/27893192/serial-cardiac-mris-in-adult-fontan-patients-detect-progressive-hepatic-enlargement-and-congestion
#3
Matthew J Lewis, Elizabeth Hecht, Jonathan Ginns, Joshua Benton, Martin Prince, Marlon S Rosenbaum
BACKGROUND: The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified. METHODS AND RESULTS: A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed...
November 28, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27888756/isolated-heart-transplant-and-combined-heart-liver-transplant-in-adult-congenital-heart-disease-patients-insights-from-the-united-network-of-organ-sharing
#4
Elisa A Bradley, Krong-On Pinyoluksana, Melissa Moore-Clingenpeel, Yongjie Miao, Curt Daniels
BACKGROUND/METHODS: The aging patient with severe congenital heart disease (CHD) faces many challenges: heart failure, arrhythmia, and in the Fontan patient, liver disease. Our goal was to define combined heart liver transplant (CHLT) and isolated orthotopic heart transplant (OHT) outcomes in U.S. adult CHD patients. The U.S. United Network for Organ Sharing (UNOS) thoracic and liver databases were queried for cardiac and CHD diagnoses, from inception-2014. RESULTS: In CHLT, CHD made up 22% of waitlist patients (non-CHD n=262 vs...
November 10, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27886757/different-sample-treatments-for-the-determination-of-icm-xr-in-fish-samples-followed-by-lc-hrms
#5
Mireia Núñez, Francesc Borrull, Núria Fontanals, Eva Pocurull
Iodinated X-ray contrast media (ICM-XR) are a group of pharmaceuticals widely used in medicine. Due to their low biodegradation rate, which makes their removal at wastewater treatment plants difficult, and the high doses at which they are administered, they have been detected in aquatic environments. In the present paper, a method for the quantitative determination of a group of ICM-XR in different fish species was developed and validated for the first time. Two extraction techniques were compared: pressurised liquid extraction (PLE) and QuEChERS (Quick, Easy, Cheap, Effective, Rugged and Safe), with PLE being selected, followed by liquid chromatography-high resolution mass spectrometry...
January 15, 2017: Talanta
https://www.readbyqxmd.com/read/27886635/multiplex-pcr-reveals-that-viruses-are-more-frequent-than-bacteria-in-children-with-cystic-fibrosis
#6
Sílvia Miró-Cañís, Sílvia Capilla-Rubio, Laura Marzo-Checa, Dionisia Fontanals-Aymerich, Isabel Sanfeliu-Sala, Mateu Espasa-Soley, Oscar Asensio-de-la-Cruz
BACKGROUND: Cystic fibrosis is a degenerative disease characterized by progressive epithelial secretory gland dysfunction associated with repeated respiratory infections. Bacterial infections are very frequent in children with cystic fibrosis, but because rapid METHODS: for screening for the wide variety of potentially involved viruses were unavailable until recently, the frequency of viral presence is unknown. Multiplex PCR enables screening for many viruses involved in respiratory infections...
November 13, 2016: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/27882688/changing-expectations-for-fontan-patient-survival-after-heart-transplantation
#7
EDITORIAL
Kathleen E Simpson
No abstract text is available yet for this article.
December 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27878634/relationship-between-habitual-exercise-and-performance-on-cardiopulmonary-exercise-testing-differs-between-children-with-single-and-biventricular-circulations
#8
Michael L O'Byrne, Sanyukta Desai, Megan Lane, Michael McBride, Stephen Paridon, Elizabeth Goldmuntz
Increasing habitual exercise has been associated with improved cardiopulmonary exercise testing (CPET) performance, specifically maximal oxygen consumption in children with operatively corrected congenital heart disease. This has not been studied in children following Fontan palliation, a population in whom CPET performance is dramatically diminished. A single-center cross-sectional study with prospective and retrospective data collection was performed that assessed habitual exercise preceding a clinically indicated CPET in children and adolescents with Fontan palliation, transposition of the great arteries following arterial switch operation (TGA), and normal cardiac anatomy without prior operation...
November 23, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27878633/cardiopulmonary-exercise-testing-in-fontan-patients-with-and-without-isomerism-heterotaxy-as-compared-to-patients-with-primary-ciliary-dyskinesia-and-subjects-with-structurally-normal-hearts
#9
Rohit S Loomba, Michael Danduran, Kim G Nielsen, Astrid M Ring, Joshua Kovach, Robert H Anderson
Isomerism, also known as heterotaxy, is a clinical entity that impacts multiple organ systems both anatomically and functionally. The airways and lungs are involved in a great number of these patients, leading to increased sinopulmonary symptoms, increased need for oxygenation, and increased postoperative ventilatory support. Additionally, these patients often have congenital heart disease requiring Fontan palliation. What has not been previously described, and is the focus of this study, is the results of cardiopulmonary exercise testing in those who have undergone Fontan palliation with and without isomerism...
November 23, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27875570/towards-a-tissue-engineered-contractile-fontan-conduit-the-fate-of-cardiac-myocytes-in-the-subpulmonary-circulation
#10
Daniel Biermann, Alexandra Eder, Florian Arndt, Hatim Seoudy, Hermann Reichenspurner, Thomas Mir, Arlindo Riso, Rainer Kozlik-Feldmann, Kersten Peldschus, Michael G Kaul, Tillman Schuler, Susanne Krasemann, Arne Hansen, Thomas Eschenhagen, Jörg S Sachweh
The long-term outcome of patients with single ventricles improved over time, but remains poor compared to other congenital heart lesions with biventricular circulation. Main cause for this unfavourable outcome is the unphysiological hemodynamic of the Fontan circulation, such as subnormal systemic cardiac output and increased systemic-venous pressure. To overcome this limitation, we are developing the concept of a contractile extracardiac Fontan-tunnel. In this study, we evaluated the survival and structural development of a tissue-engineered conduit under in vivo conditions...
2016: PloS One
https://www.readbyqxmd.com/read/27871592/stability-of-suxamethonium-in-pharmaceutical-solution-for-injection-by-validated-stability-indicating-chromatographic-method
#11
William Beck, Sofiane Kabiche, Issa-Bella Balde, Sandra Carret, Jean-Eudes Fontan, Salvatore Cisternino, Joël Schlatter
STUDY OBJECTIVE: To assess the stability of pharmaceutical suxamethonium (succinylcholine) solution for injection by validated stability-indicating chromatographic method in vials stored at room temperature. METHODS: The chromatographic assay was achieved by using a detector wavelength set at 218 nm, a C18 column, and an isocratic mobile phase (100% of water) at a flow rate of 0.6 mL/min for 5 minutes. The method was validated according to the International Conference on Harmonization guidelines with respect to the stability-indicating capacity of the method including linearity, limits of detection and quantitation, precision, accuracy, system suitability, robustness, and forced degradations...
December 2016: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/27866787/cavopulmonary-assist-bridge-to-fontan-repair-of-single-ventricle-circulation
#12
EDITORIAL
Mark Rodefeld
No abstract text is available yet for this article.
October 24, 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27863728/fontan-patient-survival-after-pediatric-heart-transplantation-has-improved-in-the-current-era
#13
Kathleen E Simpson, Elizabeth Pruitt, James K Kirklin, David C Naftel, Rakesh K Singh, R Erik Edens, Aliessa P Barnes, Charles E Canter
BACKGROUND: Historically, patients with a prior Fontan procedure for complex congenital heart disease (CHD) have been considered at higher risk for death after heart transplant (HT) compared with other HT transplant candidates. With the overall trend of improved survival of pediatric HT recipients, it is unclear of Fontan patient post-HT survival has also improved in the current era. METHODS: Data from the Pediatric Heart Transplant Study database for Fontan patients who underwent HT was compared between the early era (1993 to 2006, n = 150) and late era (2007 to 2014, n = 252)...
November 15, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27862239/quantitative-echocardiographic-measures-in-the-assessment-of-single-ventricle-function-post-fontan-incorporation-into-routine-clinical-practice
#14
Rodrigo Rios, Salil Ginde, David Saudek, Rohit S Loomba, Jessica Stelter, Peter Frommelt
BACKGROUND: Quantitative echocardiographic measurements of single ventricular (SV) function have not been incorporated into routine clinical practice. METHODS: A clinical protocol, which included quantitative measurements of SV deformation (global circumferential and longitudinal strain and strain rate), standard deviation of time to peak systolic strain, myocardial performance index (MPI), dP/dT from an atrioventricular valve regurgitant jet, and superior mesenteric artery resistance index, was instituted for all patients with a history of Fontan procedure undergoing echocardiography...
November 16, 2016: Echocardiography
https://www.readbyqxmd.com/read/27855985/can-time-averaged-flow-boundary-conditions-be-used-to-meet-the-clinical-timeline-for-fontan-surgical-planning
#15
Zhenglun Alan Wei, Phillip M Trusty, Mike Tree, Christopher M Haggerty, Elaine Tang, Mark Fogel, Ajit P Yoganathan
Cardiovascular simulations have great potential as a clinical tool for planning and evaluating patient-specific treatment strategies for those suffering from congenital heart diseases, specifically Fontan patients. However, several bottlenecks have delayed wider deployment of the simulations for clinical use; the main obstacle is simulation cost. Currently, time-averaged clinical flow measurements are utilized as numerical boundary conditions (BCs) in order to reduce the computational power and time needed to offer surgical planning within a clinical time frame...
November 10, 2016: Journal of Biomechanics
https://www.readbyqxmd.com/read/27853067/surgical-resection-for-hepatocellular-carcinoma-with-cardiac-cirrhosis-after-the-fontan-procedure
#16
Yoshitaka Takuma, Yuji Fukada, Shota Iwadou, Hirokazu Miyatake, Shuji Uematsu, Ryoichi Okamoto, Daisuke Sato, Hiroyoshi Matsukawa, Shigehiro Shiozaki, Masahiro Kamada, Toshiaki Morito, Yasuyuki Araki
A 29-year-old woman who underwent the Fontan procedure at 10 years of age had an incidental finding of liver masses on abdominal ultrasonography. Subsequent gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid magnetic resonance imaging showed a 15 mm hypervascular mass with washout in the hepatobiliary phase in liver segment 4 (S4), and an 18 mm hypervascular mass without washout in the hepatobiliary phase in liver segment 2 (S2). The S2 liver mass was pathologically diagnosed to be a regenerative nodule by an ultrasound-guided needle biopsy, and the S4 liver mass was pathologically diagnosed as a poorly differentiated hepatocellular carcinoma after partial hepatectomy...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27850078/438-gestalt-clinical-severity-score
#17
Joseph Shiber, Adeolu Akinleye, Jignesh Patel, Emily Fontane, William Chiu, Thomas Scalea
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27842994/ferroportin-mrna-is-down-regulated-in-granulosa-and-cervical-cells-from-infertile-women
#18
José Maria Moreno-Navarrete, Eva López-Navarro, Luz Candenas, Francisco Pinto, Francisco J Ortega, Mònica Sabater-Masdeu, Manuel Fernández-Sánchez, Victor Blasco, Antonio Romero-Ruiz, Marina Fontán, Wifredo Ricart, Manuel Tena-Sempere, José M Fernández-Real
OBJECTIVE: To explore the relationship between iron and infertility by investigating iron-related gene expression in granulosa and uterine cervical cells. DESIGN: Case-control study. SETTING: Two tertiary hospitals. PATIENT(S): Two independent cohorts of fertile (n = 18 and n = 17) and infertile (n = 31 and n = 35) women. INTERVENTION(S): In vitro fertilization. MAIN OUTCOME MEASURE(S): Gene expression levels of ferritin light chain (FTL), ferritin heavy chain (FTH), transferrin receptor (TFRC), and ferroportin (SLC40A1) mRNA were analyzed in granulosa and cervical cells...
November 11, 2016: Fertility and Sterility
https://www.readbyqxmd.com/read/27834768/design-and-implementation-of-a-prospective-adult-congenital-heart-disease-biobank
#19
Alexander R Opotowsky, Brittani Loukas, Christina Ellervik, Lilamarie E Moko, Michael N Singh, Elizabeth I Landzberg, Eric B Rimm, Michael J Landzberg
BACKGROUND: Adults with congenital heart disease (ACHD) comprise a growing, increasingly complex population. The Boston Adult Congenital Heart Disease Biobank is a program for the collection and storage of biospecimens to provide a sustainable resource for scientific biomarker investigation in ACHD. METHODS: We describe a protocol to collect, process, and store biospecimens for ACHD or associated diagnoses developed based on existing literature and consultation with cardiovascular biomarker epidemiologists...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27834759/current-expectations-for-cardiac-transplantation-in-patients-with-congenital-heart-disease
#20
James K Kirklin, Waldemar F Carlo, F Bennett Pearce
Congenital heart disease accounts for 40% of pediatric heart transplants and presents unique challenges to the transplant team. Suitability for transplantation is defined in part by degree of sensitization, pulmonary vascular resistance, and hepatic reserves. The incremental transplant risk for patients with congenital heart disease occurs within the first 3 months, after which survival is equivalent to transplantation for cardiomyopathy. Single ventricle with prior palliation, and especially the failing Fontan, carry the highest risk for transplantation and are least amenable to bridging with mechanical circulatory support...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
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