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https://www.readbyqxmd.com/read/28602501/abdominal-imaging-surveillance-in-adult-patients-after-fontan-procedure-risk-of-chronic-liver-disease-and-hepatocellular-carcinoma
#1
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
#2
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
#3
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...
May 2, 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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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...
March 3, 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
#10
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
#11
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
https://www.readbyqxmd.com/read/28064364/significance-of-right-atrial-tension-for-the-development-of-complications-in-patients-after-atriopulmonary-connection-fontan-procedure-potential-indicator-for-fontan-conversion
#12
Gaku Izumi, Hideaki Senzaki, Atsuhito Takeda, Hirokuni Yamazawa, Kohta Takei, Takuo Furukawa, Kei Inai, Tokuko Shinohara, Toshio Nakanishi
Elevated right atrial (RA) pressure and progressive RA dilation are thought to play pivotal roles in the development of late complications after atriopulmonary connection (APC) Fontan surgery. However, no clear cut-off value for RA pressure or RA volume has been determined for stratifying the risk of developing Fontan complications. We hypothesized that RA tension, which incorporates information about both RA pressure and volume, might help predict the risk of developing complications. We retrospectively studied 51 consecutive APC Fontan patients (median postoperative period 14 years)...
January 7, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28025635/comment-on-assessment-of-liver-stiffness-in-pediatric-fontan-patients-using-transient-elastography
#13
COMMENT
Xiulan Xue, Shaohang Cai
No abstract text is available yet for this article.
2016: Canadian Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/27998609/what-is-the-status-of-liver-health-in-adults-living-with-the-fontan-circulation-now-that-is-a-good-question
#14
EDITORIAL
Osman O Al-Radi
No abstract text is available yet for this article.
November 18, 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27988989/single-center-outcomes-of-combined-heart-and-liver-transplantation-in-the-failing-fontan
#15
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/27955914/liver-health-in-adults-with-fontan-circulation-a-multicenter-cross-sectional-study
#16
Fred M Wu, Brian Kogon, Michael G Earing, Jamil A Aboulhosn, Craig S Broberg, Anitha S John, Amy Harmon, Nisha I Sainani, Andrew J Hill, Robert D Odze, Melanie E Johncilla, Chinweike Ukomadu, Kimberlee Gauvreau, Anne Marie Valente, Michael J Landzberg
OBJECTIVES: Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. METHODS: Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained...
March 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27893192/serial-cardiac-mris-in-adult-fontan-patients-detect-progressive-hepatic-enlargement-and-congestion
#17
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
#18
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...
February 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27853067/surgical-resection-for-hepatocellular-carcinoma-with-cardiac-cirrhosis-after-the-fontan-procedure
#19
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/27787594/the-long-term-management-of-children-and-adults-with-a-fontan-circulation-a-systematic-review-and-survey-of-current-practice-in-australia-and-new-zealand
#20
Ganesh K Gnanappa, David S Celermajer, Gary F Sholler, Tom Gentles, David Winlaw, Yves d'Udekem, Julian Ayer
Although long-term survival is now the norm, Fontan patients face significant morbidity and premature mortality. Wide variation exists in long-term Fontan management. With an aim of improving their long-term management, we conducted a systematic review to identify best available evidence and gaps in knowledge for future research focus. We also surveyed cardiologists in Australia and New Zealand managing Fontan patients, to determine the alignment of current local practice with best available evidence. A systematic review was conducted using strict search criteria (PRISMA guidelines), pertaining to long-term Fontan management...
January 2017: Pediatric Cardiology
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