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https://www.readbyqxmd.com/read/29105236/liver-abnormalities-and-post-transplant-survival-in-pediatric-fontan-patients
#1
Sophie C Hofferberth, Tajinder P Singh, Heather Bastardi, Elizabeth D Blume, Francis Fynn-Thompson
The impact of liver parenchymal abnormalities on survival post-heart transplant remains unknown in pediatric Fontan patients. We assessed pediatric Fontan patients who underwent heart transplant and had documented pretransplant hepatic ultrasound (U/S) studies. Liver U/S findings were classified as normal (Group 1), mildly abnormal (Group 2, hepatomegaly/vascular congestion), or severely abnormal (Group 3, heterogeneous echotexture/nodularity). Among 30 study patients, 8 were classified as Group 1, 14 as Group 2, while 8 met Group 3 criteria...
November 3, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29103357/noninvasive-liver-assessment-in-adult-patients-with-fontan-circulation-using-acoustic-radiation-force-impulse-elastography-and-hepatic-magnetic-resonance-imaging
#2
F Buendía-Fuentes, J L Melero-Ferrer, D Plaza-López, J Rueda-Soriano, A Osa-Saez, J Aguero, P Calvillo-Batllés, C Fonfria-Esparcia, A Ballesta-Cuñat, L Martí-Bonmatí, L Martínez-Dolz
BACKGROUND: Patients who have undergone the Fontan procedure are at risk of developing hepatic dysfunction. However, broad recommendations regarding liver monitoring are limited. The purpose of this study was to characterize the frequency of liver disease in adult Fontan patients using multimodality imaging (hepatic magnetic resonance imaging [MRI], acoustic radiation force impulse [ARFI] elastography, or hepatic ultrasound). METHODS: In a prospective cross-sectional analysis of adult patients palliated with a Fontan procedure, hepatic MRI, ARFI, and hepatic ultrasound were used to assess for liver disease...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29084189/-multiorgan-malfunction-after-fontan-operation-in-adult-patients
#3
Monika Smaś-Suska, Paweł Iwaszczuk, Beata Róg, Piotr Weryński, Maria Olszowska, Piotr Podolec, Lidia Tomkiewicz-Pająk
Although 85% of patients with univentricular heart after Fontan procedure survive twenty years after operation, the procedure alone seems to be an inadequate treatment as a permanent clinical solution. Patients with a "Fontan physiology" additionally have to face a various extra-cardiac complications, including thyroid, liver and kidney dysfunction, which are not only potentially life-threatening, but also can potentiate the circulatory insufficiency. AIM: The aim of the study was to assess a multiorgan dysfunction in adult patients after Fontan operation in long term follow-up and compare to healthy controls...
October 23, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28954837/clinical-laboratory-and-molecular-findings-and-long-term-follow-up-data-in-96-french-patients-with-pmm2-cdg-phosphomannomutase-2-congenital-disorder-of-glycosylation-and-review-of-the-literature
#4
Manuel Schiff, Céline Roda, Marie-Lorraine Monin, Alina Arion, Magali Barth, Nathalie Bednarek, Maud Bidet, Catherine Bloch, Nathalie Boddaert, Delphine Borgel, Anaïs Brassier, Alexis Brice, Arnaud Bruneel, Roger Buissonnière, Brigitte Chabrol, Marie-Chantal Chevalier, Valérie Cormier-Daire, Claire De Barace, Emmanuel De Maistre, Anne De Saint-Martin, Nathalie Dorison, Valérie Drouin-Garraud, Thierry Dupré, Bernard Echenne, Patrick Edery, François Feillet, Isabelle Fontan, Christine Francannet, François Labarthe, Cyril Gitiaux, Delphine Héron, Marie Hully, Sylvie Lamoureux, Dominique Martin-Coignard, Cyril Mignot, Gilles Morin, Tiffany Pascreau, Olivier Pincemaille, Michel Polak, Agathe Roubertie, Christel Thauvin-Robinet, Annick Toutain, Géraldine Viot, Sandrine Vuillaumier-Barrot, Nathalie Seta, Pascale De Lonlay
BACKGROUND: Phosphomannomutase 2-congenital disorder of glycosylation (PMM2-CDG) is a multisystem inborn error of metabolism. OBJECTIVES: To better characterise the natural history of PMM2-CDG. METHODS: Medical charts of 96 patients with PMM2-CDG (86 families, 41 males, 55 females) were retrospectively reviewed. Data on clinical, laboratory and molecular parameters at diagnosis were analysed. Follow-up data at last examination were reported for 25 patients...
September 27, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28950354/outcomes-after-extracardiac-fontan-procedure-with-a-16-mm-polytetrafluoroethylene-conduit
#5
Sungkyu Cho, Woong-Han Kim, Eun Seok Choi, Jae Gun Kwak, Hyoung Woo Chang, Kwanyong Hyun, Chang-Ha Lee
OBJECTIVES: We evaluated the outcomes of patients who underwent extracardiac Fontan circulation procedures (ECFP) and received 16-mm polytetrafluoroethylene conduits. METHODS: From June 1997 to May 2015, among the 408 patients who underwent ECFP, 66 patients (Group S) also received 16-mm polytetrafluoroethylene conduits. To compare this patient cohort with similarly sized patients who received larger conduits, a matched cohort (66 patients, Group L) was selected according to age and body weight...
August 10, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28941165/combined-heart-liver-transplantation-for-failing-fontan-circulation-in-a-late-survivor-with-single-ventricle-physiology
#6
Phuoc Duong, Louise Coats, John O'Sullivan, David Crossland, Beate Haugk, Sonya V Babu-Narayan, Jennifer Keegan, Mark Hudson, Gareth Parry, Derek Manas, Asif Hasan
Management of adults with failing Fontan physiology poses many challenges, especially as transplantation offers the only realistic alternative to palliative care. We present the first combined heart and liver transplant performed in Europe, for a late survivor of single ventricle palliation with the Fontan circulation. In addition to the conventional medical and surgical challenges posed, we highlight the management of the associated multi-organ failure with focus on the liver and novel strategies for assessment and optimization...
September 21, 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28881280/a-computational-study-of-the-fontan-circulation-with-fenestration-or-hepatic-vein-exclusion
#7
Charles Puelz, Sebastián Acosta, Béatrice Rivière, Daniel J Penny, Ken M Brady, Craig G Rusin
Fontan patients may undergo additional surgical modifications to mitigate complications like protein-losing enteropathy, liver cirrhosis, and other issues in their splanchnic circulation. Recent case reports show promise for several types of modifications, but the subtle effects of these surgeries on the circulation are not well understood. In this paper, we employ mathematical modeling of blood flow to systematically quantify the impact of these surgical changes on extracardiac Fontan hemodynamics. We investigate two modifications: (1) the fenestrated Fontan and (2) the Fontan with hepatic vein exclusion...
October 1, 2017: Computers in Biology and Medicine
https://www.readbyqxmd.com/read/28879464/prevalence-of-subclinical-enteric-alpha-1-antitrypsin-loss-in-children-with-univentricular-circulation-following-total-cavopulmonary-connection
#8
Colm R Breatnach, Aoife Cleary, Terence Prendiville, Kathleen Crumlish, Helene Murchan, Colin J McMahon
Protein-Losing Enteropathy post Fontan palliation is associated with significant morbidity and mortality. To date, very little research has been carried out to improve early identification of enteric protein loss in these patients. We hypothesise that subclinical enteric protein loss may occur in patients post Fontan surgery. A cross-sectional study was performed on 43 patients post Fontan surgery. We collected specimens of stool and blood from patients with no symptoms of protein-losing enteropathy post Fontan...
September 6, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28695974/what-determine-the-quality-of-life-of-adult-patients-after-fontan-procedure
#9
Monika Smaś-Suska, Natalia Dłużniewska, Piotr Weryński, Jacek Pająk, Wojciech Płazak, Maria Olszowska, Piotr Podolec, Lidia Tomkiewicz-Pająk
BACKGROUND: Despite the low early mortality of Fontan procedures, Fontan patients are prone to various cardiac and extra-cardiac complications in the long term. This may influence patient perception of their health and outcome. The aim of the study was to assess the relationship of multi-organ complications and physical efficiency with self-reported health-related quality of life (QOL) in adult Fontan patients. METHODS: Quality of life was assessed with the Short Form-36 questionnaire...
July 11, 2017: Cardiology Journal
https://www.readbyqxmd.com/read/28602501/abdominal-imaging-surveillance-in-adult-patients-after-fontan-procedure-risk-of-chronic-liver-disease-and-hepatocellular-carcinoma
#10
Sadhna B Nandwana, Babatunde Olaiya, Kelly Cox, Anurag Sahu, Pardeep Mittal
PURPOSE: To assess the prevalence of chronic liver disease (CLD) and hepatocellular carcinoma (HCC) in adult patients who had surveillance imaging after Fontan procedure. METHODS: Institutional review board-approved, Health Insurance Portability and Accountability Act-compliant retrospective study evaluated electronic medical records including radiology reports and clinical notes for adult patients after Fontan procedure between January 1993 and January 2016. Abdominal ultrasound, computed tomography, and magnetic resonance imaging reports were reviewed for changes of CLD and HCC...
April 11, 2017: Current Problems in Diagnostic Radiology
https://www.readbyqxmd.com/read/28480728/hepatocellular-carcinoma-in-a-non-failing-fontan-circulation
#11
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/28465331/renal-resistive-index-reflects-fontan-pathophysiology-and-predicts-mortality
#12
Hideo Ohuchi, Jun Negishi, Yohsuke Hayama, Aya Miyazaki, Isao Shiraishi, Hajime Ichikawa
OBJECTIVES: The renal resistive index (RRI) reflects non-renal pathophysiology, such as great artery stiffness, haemodynamics and even end-organ damage in patients with hypertension. This study was conducted to clarify the clinical significance of the RRI in Fontan pathophysiology. METHODS: We measured the RRI in 280 consecutive Fontan patients and 36 healthy controls. RESULTS: The patients exhibited a higher RRI than the controls (0.71±0...
October 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28446492/hepatic-fibrosis-is-universal-following-fontan-operation-and-severity-is-associated-with-time-from-surgery-a-liver-biopsy-and-hemodynamic-study
#13
David J Goldberg, Lea F Surrey, Andrew C Glatz, Kathryn Dodds, Michael L O'Byrne, Henry C Lin, Mark Fogel, Jonathan J Rome, Elizabeth B Rand, Pierre Russo, Jack Rychik
BACKGROUND: Congestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking. METHODS AND RESULTS: Liver biopsies and cardiac catherizations were performed as part of an evaluation offered to all patients ≥10 years after Fontan. Quantitative determination of hepatic fibrosis was performed using Sirius red staining with automated calculation of collagen deposition per slide (%CD)...
April 26, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28432659/current-role-of-blood-and-urine-biomarkers-in-the-clinical-care-of-adults-with-congenital-heart-disease
#14
REVIEW
Saurabh Rajpal, Laith Alshawabkeh, Alexander R Opotowsky
PURPOSE OF REVIEW: There is an increasing number of adult patients with congenital heart disease (CHD). While several biomarkers have been validated and integrated into general cardiology clinical practice, these tests are often applied to adults with CHD in the absence of disease-specific validation. Although these patients are often grouped into a single population, there is heterogeneous pathophysiology, variable disease chronicity, extensive multisystem involvement, and a low event rate relative to acquired heart disease...
June 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28396861/non-invasive-investigations-for-the-diagnosis-of-fontan-associated-liver-disease-in-pediatric-and-adult-fontan-patients
#15
Amyna Fidai, Frederic Dallaire, Nanette Alvarez, Yvonne Balon, Robin Clegg, Michael Connelly, Frank Dicke, Deborah Fruitman, Joyce Harder, Kimberley Myers, David J Patton, Tim Prieur, Erika Vorhies, Robert P Myers, Steven R Martin, Steven C Greenway
Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8...
2017: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28341356/mortality-risk-stratification-in-fontan-patients-who-underwent-heart-transplantation
#16
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/28280718/response-to-comment-on-assessment-of-liver-stiffness-in-pediatric-fontan-patients-using-transient-elastography
#17
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/28258242/successful-cardiac-transplantation-outcomes-in-patients-with-adult-congenital-heart-disease
#18
Jonathan N Menachem, Jessica R Golbus, Maria Molina, Jeremy A Mazurek, Nicole Hornsby, Pavan Atluri, Stephanie Fuller, Edo Y Birati, Yuli Y Kim, Lee R Goldberg, Joyce W Wald
OBJECTIVES: The purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT). BACKGROUND: Heart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT...
September 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28257439/diminished-liver-microperfusion-in-fontan-patients-a-biexponential-dwi-study
#19
Hildebrand Dijkstra, Djoeke Wolff, Joost P van Melle, Beatrijs Bartelds, Tineke P Willems, Matthijs Oudkerk, Hans Hillege, Aad P van den Berg, Tjark Ebels, Rolf M F Berger, Paul E Sijens
It has been demonstrated that hepatic apparent diffusion coefficients (ADC) are decreasing in patients with a Fontan circulation. It remains however unclear whether this is a true decrease of molecular diffusion, or rather reflects decreased microperfusion due to decreased portal blood flow. The purpose of this study was therefore to differentiate diffusion and microperfusion using intravoxel incoherent motion (IVIM) modeled diffusion-weighted imaging (DWI) for different liver segments in patients with a Fontan circulation, compare to a control group, and relate with liver function, chronic hepatic congestion and hepatic disease...
2017: PloS One
https://www.readbyqxmd.com/read/28140526/surveillance-for-liver-complications-after-the-fontan-procedure
#20
REVIEW
Moira B Hilscher, Jonathan N Johnson, Frank Cetta, David J Driscoll, John J Poterucha, William Sanchez, Heidi M Connolly, Patrick S Kamath
The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population...
March 2017: Congenital Heart Disease
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