Read by QxMD icon Read

fontan liver

Isabel Kazour, Suraj D Serai, Stavra A Xanthakos, Robert J Fleck
The goal of this study was to assess the ability of quantitative T1 cardiovascular magnetic resonance (CMR) imaging to calculate liver extracellular volume (ECV) in patients with varying degrees of congestive hepatopathy (CH). T1 measurements and ECV calculations were performed retrospectively in three cohorts of patients: normal cardiac function, tetralogy of fallot (TOF) repair and Fontan palliation. All CMR studies included modified look-locker inversion recovery (MOLLI) T1 mapping scans performed pre- and post-injection of a gadolinium-based contrast agent (GBCA)...
March 3, 2018: Abdominal Radiology
Michael V Di Maria, Lindsey Silverman, Adel K Younoszai, Mariana L Meyers, Kelley E Capocelli, Michael R Narkewicz
BACKGROUND: The current management paradigm for children with single ventricle heart disease involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results in a high central venous pressure, and in the setting of single ventricle heart disease, results in hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse (ARFI), has been utilized in this cohort...
February 23, 2018: Congenital Heart Disease
Andrew T Trout, Rachel M Sheridan, Suraj D Serai, Stavra A Xanthakos, Weizhe Su, Bin Zhang, Daniel B Wallihan
Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease...
February 22, 2018: Radiology
Luis Téllez, Enrique Rodríguez-Santiago, Agustín Albillos
Fontan-associated liver disease is a hepatic disorder arising from hemodynamic changes and systemic venous congestion following Fontan surgery. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease. While the natural history of this form of liver disease is not well established, over time many Fontan patients develop portal hypertension-related complications such as ascites, variceal hemorrhage or encephalopathy. Fontan survivors also show an increased risk of hepatocellular carcinoma...
March 1, 2018: Annals of Hepatology
Yohei Yamada, Ken Hoshino, Takayuki Oyanagi, Ryohei Gatayama, Jun Maeda, Nobuyuki Katori, Yasushi Fuchimoto, Taizo Hibi, Masahiro Shinoda, Kentaro Matsubara, Hideaki Obara, Ryo Aeba, Yuko Kitagawa, Hiroyuki Yamagishi, Tatsuo Kuroda
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology...
February 19, 2018: Pediatric Transplantation
Masanari Kuwabara, Koichiro Niwa, Tomohiko Toyoda, Takeaki Shirai, Shigeru Tateno, Hideo Ohuchi, Yasuhiko Tanaka, Fukiko Ichida, Tomoo Fujisawa, Teiji Akagi, Yoshiki Mori
BACKGROUND: Fontan-associated liver disease (FALD) is an important late complication involving liver dysfunction, such as liver cirrhosis (LC) and hepatocellular carcinoma (HCC), in patients undergoing the Fontan procedure. However, the prevalence, clinical manifestation, and methods of diagnosis of FALD are still not well established.Methods and Results:This study comprised 2 nationwide surveys in Japan. First, the prevalence of LC and/or HCC in patients undergoing the Fontan procedure was determined...
February 14, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
Alexander Egbe, William R Miranda, Heidi M Connolly, Arooj R Khan, Mohamad Al-Otaibi, Sudhakar K Venkatesh, Douglas Simonetto, Patrick Kamath, Carole Warnes
BACKGROUND: The relationship between temporal progression of magnetic resonance elastography derived liver stiffness (MRE-LS) and progression of Fontan associated liver disease (FALD) is unknown. To assess this relationship, we hypothesized that progression of MRE-LS correlated with progression FALD severity and clinical outcomes. METHODS: Retrospective review of Fontan patients who had >1 liver MRE, 2010-2016. Annual change in MRE-LS was the quotient of the difference between baseline and subsequent MRE-LS, and the interval between scans...
February 9, 2018: International Journal of Cardiology
Luis Téllez, Enrique Rodríguez de Santiago, Agustín Albillos
Fontan-associated liver disease refers to the disturbance in the liver secondary to hemodynamic changes and systemic venous congestion following Fontan surgery. Although the natural history of this disease has not yet been established, patients with more advanced liver injury develop the complications of portal hypertension, such as ascites, variceal haemorrhage, or encephalopathy. Moreover, patients with Fontan surgery may have an increased risk of hepatocellular carcinoma. Periodic liver monitoring is essential to prevent this disease and provide early treatment of liver complications...
February 9, 2018: Revista Española de Cardiología
Anushree Agarwal, Colin Cunnington, Aarthi Sabanayagam, Lucas Zier, Charles E McCulloch, Ian S Harris, Elyse Foster, Dougal Atkinson, Angela Bryan, Petra Jenkins, Jaspal Dua, Michael J Parker, Devinda Karunaratne, John A Moore, Jeffrey Meadows, Bernard Clarke, J Andreas Hoschtitzky, Vaikom S Mahadevan
BACKGROUND: Liver disease (LD) is a long-term complication in patients with a single ventricle who have had the Fontan operation. A decline in cardiopulmonary exercise testing (CPET) variables is associated with increased risk of hospitalization, but its association with LD is unknown. AIM: To determine the association between CPET variables and LD in adults who have had the Fontan operation. METHODS: We retrospectively reviewed the medical records from two tertiary institutions...
December 1, 2017: Archives of Cardiovascular Diseases
William N Evans, Ruben J Acherman, Michael L Ciccolo, Sergio A Carrillo, Alvaro Galindo, Abraham Rothman, Gary A Mayman, Elizabeth A Adams, Leigh C Reardon, Brody J Winn, Noel S Yumiaco, Lesley Shimuizu, Yoko Inanaga, Rowena J Deleon, Humberto Restrepo
OBJECTIVE: We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements, either alone or in combination with other noninvasive variables, might correlate with liver-biopsy fibrosis scores in patients post-Fontan. METHODS: Between March 2012 and February 2017, we identified patients post-Fontan that underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography...
January 2018: Congenital Heart Disease
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
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
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
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
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
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
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
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
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
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
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"