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https://www.readbyqxmd.com/read/28480728/hepatocellular-carcinoma-in-a-non-failing-fontan-circulation
#1
Efrén Martínez-Quintana, Alberto Monescillo, Fayna Rodríguez-González
Patients after a Fontan operation exhibit some degree of fibrosis which are primarily located in a sinusoidal or centrilobular pattern. Cirrhosis may develop years later and the incidence of cancer is up to 5%. That is why some authors recommend that patients who are >10 years out from their Fontan operation should undergo cardiac assessment as well as the determination of alpha-fetoprotein level, the realization of liver imaging tests or even liver biopsy to stay ahead of neoplastic transformation.
May 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28439697/heart-transplantation-for-adults-with-congenital-heart-disease-current-status-and-future-prospects
#2
REVIEW
Hikaru Matsuda, Hajime Ichikawa, Takayoshi Ueno, Yoshiki Sawa
Increased survival rates after corrective or palliative surgery for complex congenital heart disease (CHD) in infancy and childhood are now being coupled with increased numbers of patients who survive to adulthood with various residual lesions or sequelae. These patients are likely to deteriorate in cardiac function or end-organ function, eventually requiring lifesaving treatment including heart transplantation. Although early and late outcomes of heart transplantation have been improving for adult survivors of CHD, outcomes and pretransplant management could still be improved...
April 24, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28341356/mortality-risk-stratification-in-fontan-patients-who-underwent-heart-transplantation
#3
Christopher J Berg, Brenton S Bauer, Abbie Hageman, Jamil A Aboulhosn, Leigh C Reardon
The number of patients who require orthotopic heart transplantation (OHT) for failing Fontan physiology continues to grow; however, the methods and tools to evaluate risk of OHT are limited. This study aimed to identify a set of preoperative variables and characteristics that were associated with a greater risk of postoperative mortality in patients who received OHT for failing Fontan physiology. Thirty-six Fontan patients were identified as having undergone OHT at University of California-Los Angeles Medical Center from 1991 to 2014...
May 15, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28319522/a-numerical-simulation-comparing-a-cavopulmonary-assist-device-and-va-ecmo-for-failing-fontan-support
#4
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/28242080/can-fontan-conversion-for-patients-without-late-fontan-complications-be-justified
#5
Akihiko Higashida, Takaya Hoashi, Koji Kagisaki, Masatoshi Shimada, Hideo Ohuchi, Isao Shiraishi, Hajime Ichikawa
BACKGROUND: Fontan conversion from a classic Fontan operation such as atriopulmonary connection to total cavopulmonary connection with antiarrhythmia surgery is currently not indicated for patients without any late Fontan complications. METHODS: Thirty-two consecutive patients who underwent Fontan conversion between 1991 and 2012 were divided into 2 groups by the presence (group 1: n = 25, atrial tachyarrhythmia [AT] in 24 and protein-losing enteropathy in 4) or absence (group 2: n = 7) of late Fontan complications, and the surgical outcomes were retrospectively compared...
June 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28210768/the-lymphatic-circulation-in-adaptations-to-the-fontan-circulation
#6
REVIEW
Sabarinath Menon, Murthy Chennapragada, Shinya Ugaki, Gary F Sholler, Julian Ayer, David S Winlaw
Failing Fontan continues to be major problem for patients on the univentricular pathway. Failing Fontan is often complicated by chylothorax, plastic bronchitis and protein loosing enteropathy. The role of lymphatic circulation in Fontan circulation is still being researched. Newer imaging modalities give insight into the role of abnormal dilatation and retrograde flow in lymphatic channels post Fontan. Interventional strategies targeting abnormal lymphatic channels, provides an alternative management strategy for patients with failing Fontan...
February 16, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28118264/in-vitro-examination-of-the-heartware-circulite-ventricular-assist-device-in-the-fontan-connection
#7
Michael Tree, Phillip M Trusty, Timothy C Slesnick, Ajit Yoganathan, Shriprasad Deshpande, Kevin Maher
The failing Fontan physiology may benefit from ventricular assist device (VAD) mechanical circulatory support, though a sub-pulmonary VAD placed at the Fontan connection has never successfully supported the Fontan circulation long term.The HeartWare CircuLite continuous flow VAD was examined for Fontan circulatory support in an in vitro mock circulation. The VAD was tested in three different scenarios: VAD in parallel, baffle restricted VAD in parallel, and VAD in series. Successful support was defined as simultaneous decrease in inferior vena cava (IVC) pressure of 5 mmHg or more and an increase in cardiac output (CO) to 4...
January 20, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28070902/a-modified-lvad-technique-to-augment-caval-and-pulmonary-arterial-blood-flow-in-the-failing-fontan-circulation
#8
Michael F Swartz, Louis DiVincenti, Karen Smith, Robin Westcott, Kevin Belmont, William Harris, Francisco Gensini, George M Alfieris
BACKGROUND: There are minimal circulatory support options for patients with a failing Fontan. The Heartmate II (HMII) left ventricular assist device (Thoratec, Bedford, MA) in its packaged state cannot augment caval/pulmonary arterial blood flow. AIM: We hypothesized that a modified HMII pump could augment caval and pulmonary arterial blood flow. METHODS: A bifurcated ringed Gore-Tex graft (W. L. Gore & Associates, Flagstaff, AZ) was sewn to the HMII inflow, and the outflow graft transected and tapered from 16 mm to 8 mm in diameter...
February 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28066626/update-in-the-extraction-of-airway-foreign-bodies-in-adults
#9
REVIEW
Montserrat Blanco Ramos, Maribel Botana-Rial, Eva García-Fontán, Alberto Fernández-Villar, Mercedes Gallas Torreira
Foreign body (FB) aspiration into the airway is lees common in adults than children. Nevertheless its incidence does not decrease through time. We present clinical relevant aspects of airway FBs on the basis of a selective review of pertinent literature retrieved by a search in the PubMed database. The most common aspirated FBs by adults are organics, especially fragments of bones and seeds. Symptoms usually are cough, chocking and dyspnea. Right localization, especially bronchus intermedius and right lower lobe, is more frequent...
November 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28007062/rescuing-the-late-failing-fontan-focus-on-surgical-treatment-of-dysrhythmias
#10
REVIEW
Carl L Backer
Patients with a failing Fontan frequently have dysrhythmias. This review will focus on the treatment of those dysrhythmias based on the 140 patients who have had a Fontan conversion with arrhythmia surgery at Ann & Robert H. Lurie Children's Hospital of Chicago (Chicago, IL). Important technical points to be emphasized are the following:• Atrial fibrillation is extremely common in patients with a failing Fontan and strong consideration should be given to a Cox-maze III during any Fontan conversion• Sinus node dysfunction is common post-conversion and all patients should receive a dual-chamber epicardial pacemaker• Careful preoperative imaging with either computed tomographic or magnetic resonance imaging is needed to predict re-entry problems at the time of repeat sternotomy• If the patient has protein-losing enteropathy, ascites, or a right or indeterminate ventricular morphology, strong consideration should be given to heart transplantation rather than a Fontan conversion and arrhythmia surgery...
January 2017: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://www.readbyqxmd.com/read/27988989/single-center-outcomes-of-combined-heart-and-liver-transplantation-in-the-failing-fontan
#11
Benjamin A D'Souza, Stephanie Fuller, Lacey P Gleason, Nicole Hornsby, Joyce Wald, Karen Krok, Abraham Shaked, Lee R Goldberg, Alberto Pochettino, Kim M Olthoff, Yuli Y Kim
Long-term outcomes of the Fontan operation include Fontan failure and liver disease. Combined heart-liver transplantation (CHLT) is an option for select patients although limited data exist on this strategy. A retrospective review of Fontan patients 18 years or older referred for cardiac transplant evaluation between 2000 and 2013 at the Hospital of the University of Pennsylvania was performed. All patients were considered for potential CHLT. Clinical variables such as demographics, perioperative factors, and short-term outcomes were reviewed...
March 2017: Clinical 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
#12
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...
March 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/27834759/current-expectations-for-cardiac-transplantation-in-patients-with-congenital-heart-disease
#13
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
https://www.readbyqxmd.com/read/27810293/usefulness-of-stroke-volume-monitoring-during-upright-ramp-incremental-cycle-exercise-in-young-patients-with-fontan-circulation
#14
Antoine Legendre, Aurore Guillot, Magalie Ladouceur, Damien Bonnet
BACKGROUND: Aerobic capacity (VO2 max) of patients with Fontan circulation (FC) is lowest within patients with congenital heart disease. The reasons have not been completely elucidated. METHODS: Twenty five young patients with non-failing FC underwent a cardiopulmonary test during an upright ramp cycling. By using a signal morphology impedance cardiography device (physioflow®), stroke volume (SV) was evaluated along with effort. The results were compared with paired healthy controls...
January 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27646132/a-rare-cause-of-persisting-anaemia-in-a-patient-with-a-failing-fontan-circulation
#15
Loes Lambrecht, Bjorn Cools, Peter Witters
This case report describes intestinal angiodysplasias as a cause of persisting anaemia in a 16-year-old girl with a failing Fontan circulation. Although this is a very rare cause of anaemia, we highlight the importance of video capsule endoscopy in such patients to initiate a life-saving treatment.
January 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/27625527/unusual-method-of-creation-of-a-transcatheter-fenestration-in-an-extracardiac-conduit-fontan-circulation
#16
Anil K Singhi, Sivakumar Kothandum
Failing Fontan physiology in univentricular hearts manifest with protein-losing enteropathy, plastic bronchitis, low cardiac output, and recurrent effusions. Transcatheter creation of fenestration in a failing Fontan may be useful in alleviating the symptoms by improving the cardiac output. It is traditionally achieved by puncturing through the conduit from femoral or jugular venous access. In the absence of good venous path, transhepatic access provides a direct route for needle puncture of the conduit. If marked intimal ingrowth into the conduit results in increasing rigidity and makes the conduit nonyielding, alternative approaches may be needed...
September 2016: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/27616284/diverse-multi-organ-histopathologic-changes-in-a-failed-fontan-patient
#17
Masanori Mizuno, Hideo Ohuchi, Taka-Aki Matsuyama, Aya Miyazaki, Hatsue Ishibashi-Ueda, Osamu Yamada
We report multi-organ histopathological changes in a patient with protein-losing enteropathy (PLE) over 12 years after Fontan operation. A 14-year-old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre-renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension...
October 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27556319/major-intrahepatic-veno-venous-fistula-after-fontan-operation-treated-by-transcatheter-implantation-of-amplatzer-septal-occluder-through-internal-jugular-vein
#18
Alper Guzeltas, Ibrahim Cansaran Tanidir, Murat Saygi
Six months after undergoing a Fontan operation, a 7-year-old boy with right atrial isomerism and a single functional ventricle was admitted to our emergency department with cyanosis. Emergency cardiac catheterization revealed a large veno-venous fistula that began in a left hepatic vein, connected to the left accessory hepatic veins, and drained into the common atrium, resulting in desaturation. The fistula was occluded proximally with an Amplatzer septal occluder, with satisfying results; the patient's systemic arterial saturation decreased during his hospital stay...
April 2016: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/27552237/management-of-1-lung-ventilation-in-a-patient-with-failing-fontan-circulation
#19
Stephen H Gregory, Madhav Swaminathan, Yasmin Maisonave, Kelly A Machovec
The number of patients reaching adulthood after undergoing Fontan palliation for the repair of a congenital heart defect continues to increase. In this case report, we present the anesthetic management of a patient with a history of tricuspid atresia treated with palliative Fontan repair who had developed clinical evidence of Fontan failure. He presented with septic shock secondary to streptococcal toxic shock syndrome complicated by a loculated pleural effusion. He underwent open thoracic decortication under 1-lung ventilation...
October 15, 2016: A & A Case Reports
https://www.readbyqxmd.com/read/27402424/perioperative-management-of-pediatric-en-bloc-combined-heart-liver-transplants-a-case-series-review
#20
Manchula Navaratnam, Ann Ng, Glyn D Williams, Katsuhide Maeda, Julianne M Mendoza, Waldo Concepcion, Seth A Hollander, Chandra Ramamoorthy
BACKGROUND: Combined heart and liver transplantation (CHLT) in the pediatric population involves a complex group of patients, many of whom have palliated congenital heart disease (CHD) involving single ventricle physiology. OBJECTIVE: The purpose of this study was to describe the perioperative management of pediatric patients undergoing CHLT at a single institution and to identify management strategies that may be used to optimize perioperative care. METHODS: We did a retrospective database review of all patients receiving CHLT at a children's hospital between 2006 and 2014...
October 2016: Paediatric Anaesthesia
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