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fontan liver

Frank W DiPaola, Kurt R Schumacher, Caren S Goldberg, Joshua Friedland-Little, Aishwarya Parameswaran, Jonathan R Dillman
OBJECTIVES: Assess liver stiffness using ultrasound point shear wave elastography (US P-SWE) in children before and after the Fontan operation. METHODS: Eighteen children undergoing the Fontan operation were prospectively enrolled. Eight US P-SWE measurements were obtained from the right hepatic lobe before surgery, and at multiple postoperative time points. Temporally related inferior vena cava pressure (IVC) data was collected from medical records, when available...
October 17, 2016: European Radiology
Chaeyoun Oh, Joong Kee Youn, Ji-Won Han, Gi Beom Kim, Hyun-Young Kim, Sung-Eun Jung
INTRODUCTION: The Fontan procedure (FP) has become the standard operation for patients with single ventricle physiology. However, a long period of elevated systemic venous pressure and low cardiac output after the procedure result in chronic inflammation and liver cirrhosis, which may eventually lead to the occurrence of hepatocellular carcinoma (HCC). CLINICAL FINDINGS: We described the case of a 16-year-old female who developed HCC after the FP. At 21 months, the patient received a lateral tunnel FP, and 14 years later, she began complaining of abdominal distension, telangiectasia, and fatigue...
October 2016: Medicine (Baltimore)
Michael R Conroy, Tabitha G Moe
In this study, we describe the case of a 36-year-old woman who was diagnosed with hepatocellular carcinoma on a background of Fontan procedure for tricuspid atresia. She had worsening heart failure in the months before presentation, and early investigations noted derangement in liver enzymes and hepatomegaly. Liver biopsy confirmed a hepatocellular carcinoma. Hepatocellular carcinoma is a rare but recognised consequence of cardiac cirrhosis in Fontan patients.
September 19, 2016: Cardiology in the Young
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...
September 11, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Camden Hebson, Wendy Book, Robert W Elder, Ryan Ford, Maan Jokhadar, Kirk Kanter, Brian Kogon, Adrienne H Kovacs, Rebecca D Levit, Michael Lloyd, Kevin Maher, Preeti Reshamwala, Fred Rodriguez, Rene Romero, Thor Tejada, Anne Marie Valente, Gruschen Veldtman, Michael McConnell
"Frontiers in Fontan Failure" was the title of a 2015 conference sponsored by Children's Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions...
September 6, 2016: Congenital Heart Disease
Fred M Wu, Michael G Earing, Jamil A Aboulhosn, Melanie E Johncilla, Michael N Singh, Robert D Odze, Chinweike Ukomadu, Kimberlee Gauvreau, Michael J Landzberg, Anne Marie Valente
BACKGROUND: Hepatic fibrosis is highly prevalent in individuals with Fontan circulation. FibroSure (LabCorp, Burlington, NC) and hyaluronic acid (HA) have been validated for assessment of hepatic fibrosis in several forms of liver disease. We sought to determine whether these tests could identify Fontan patients with advanced hepatic fibrosis or cirrhosis. METHODS: Subjects who had liver biopsy and FibroSure or HA testing within 6 months of biopsy were identified from the Alliance for Adult Research in Congenital Cardiology Fontan Liver Health study...
July 27, 2016: Journal of Heart and Lung Transplantation
Lea F Surrey, Pierre Russo, Jack Rychik, David J Goldberg, Kathryn Dodds, Michael L O'Byrne, Andrew C Glatz, Elizabeth B Rand, Henry C Lin
The Fontan operation is a widely used palliative procedure in patients with single ventricle anatomy that results in liver injury. As timely identification of liver fibrosis may result in management changes to Fontan patients, the aim of our study was to identify clinically meaningful semi-quantitative/quantitative pathologic parameters for biopsy assessment. We performed a retrospective review of 74 liver needle biopsies from Fontan patients. Fibrosis was assessed using quantitative % collagen deposition (%CD) by Sirius red image analysis, METAVIR, congestive hepatic fibrosis score (CHFS), sinusoidal fibrosis (SF) score, and sinusoidal dilation score...
July 27, 2016: Human Pathology
Xiao-Ke Shang, Rong Lu, Xi Zhang, Chang-Dong Zhang, Shu-Na Xiao, Mei Liu, Bin Wang, Nian-Guo Dong
Fontan surgery is a widely used palliative procedure that significantly improves the survival period of patients with complex congenital heart disease (CHD). However, it does not decrease postoperative complication rate. Previous studies suggested that elevated mean pulmonary artery pressure (mPAP) and vascular resistance lead to decreased exercise tolerance and myocardial dysfunction. Therapy with endothelial receptor antagonists (Bosentan) has been demonstrated to improve the patients' prognosis. A double-blind, randomized controlled trial was performed to explore the efficacy of Bosentan in treating patients who underwent the Fontan procedure...
August 2016: Journal of Huazhong University of Science and Technology. Medical Sciences
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
Bari Murtuza, Antony Hermuzi, David S Crossland, Gareth Parry, Stephen Lord, Mark Hudson, Milind P Chaudhari, Simon Haynes, John J O'Sullivan, Asif Hasan
OBJECTIVES: Adults with failing Fontan circulation (FFC) represent a heterogeneous, high-risk group for cardiac transplantation with poor reported outcomes. We studied the impact of mode of Fontan failure (preserved versus impaired systolic ventricular function) and end-organ dysfunction on early survival in adults undergoing cardiac transplantation for FFC. METHODS: A single-centre, retrospective study of 26 adults (≥16 years) with FFC undergoing cardiac transplantation between 1990 and 2015...
July 11, 2016: European Journal of Cardio-thoracic Surgery
Kavitha N Pundi, Krishna Pundi, David J Driscoll, Joseph A Dearani, Zhuo Li, Sonja H Dahl, Bassem N Mora, Patrick W O'Leary, Richard C Daly, Frank Cetta, Jonathan N Johnson
We performed a retrospective review of outcomes after heart transplantation during long-term follow-up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow-up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty-four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23...
July 10, 2016: Pediatric Transplantation
Masaya Sugimoto, Hideharu Oka, Aya Kajihama, Kouichi Nakau, Seiko Kuwata, Clara Kurishima, Hiroshi Azuma
BACKGROUND: The incidence of late liver complications such as fibrosis or cirrhosis has increased among patients who have undergone the Fontan procedure. Magnetic resonance elastography (MRE) recently emerged as a technique to clinically evaluate liver fibrosis. However, few reports have described its use in evaluating liver fibrosis in children with congenital heart disease (CHD). METHODS AND RESULTS: Fifty-seven children were examined and divided into four groups: 27 with CHD who underwent intracardiac repair (ICR); 16 with CHD who underwent the Fontan procedure (Fontan); 14 in a control group (control); and two with cirrhosis (cirrhosis)...
September 2016: Journal of Cardiology
Luca Deorsola, Enrico Aidala, Maria Teresa Cascarano, Andrea Valori, Gabriella Agnoletti, Carlo Pace Napoleone
OBJECTIVES: Hepatic damage in Fontan circulation setting is well known. Mainly represented by fibrosis, it is still poorly understood. Transient hepatic elastography (Fibro-Scan) is a useful tool to test liver stiffness and is commonly used in the evaluation of liver fibrosis. Unfortunately, the increase of inferior vena cava pressure consequent to total cavopulmonary connection can probably interfere with Fibro-Scan evaluation altering the result evaluation with the conventional scale...
October 2016: Interactive Cardiovascular and Thoracic Surgery
William N Evans, Ruben J Acherman, Leigh C Reardon, Alvaro Galindo, Abraham Rothman, Michael L Ciccolo, Sergio A Carrillo, Brody J Winn, Noel S Yumiaco, Humberto Restrepo
This brief report describes an observation from liver biopsy results in nonfailing Fontan patients, currently in their second postoperative decade. In three patients, with either atriopulmonary or atrioventricular connections and functional left ventricles, we found no portal fibrosis. In contrast, we found portal fibrosis in three clinically similar, nonfailing Fontan patients with lateral tunnel connections and functional left ventricles. We recognize the results may be secondary to chance; nevertheless, we speculate about possible relevancy...
August 2016: Pediatric Cardiology
Ari M Cedars, Joshua Saef, Linda R Peterson, Andrew R Coggan, Eric L Novak, Debra Kemp, Philip A Ludbrook
The Fontan operation is a common end point for children born with a single functional ventricle. Fontan patients typically experience physiological deterioration leading to transplant or death in their third or fourth decades of life. This deterioration is partially attributable to progressive increases in pulmonary vascular resistance (PVR) and as such endothelin receptor antagonists, which are known to decrease pulmonary vascular resistance, have been proposed as potentially beneficial in this population...
May 1, 2016: American Journal of Cardiology
Robert D B Jaquiss, Hamza Aziz
For the child born with a functionally univentricular heart, multi-stage surgical palliation culminating in the Fontan operation is now well established as the standard of care. Though this is an effective approach, there are several inherent disadvantages to the Fontan circulation: subnormal resting cardiac output, impaired exercise capacity, increased risk of thromboembolism, and increased risk of arrhythmia. Perhaps most importantly, the long-term deleterious effects of substantial systemic venous hypertension, which is necessary to "drive" blood through the pulmonary vascular bed, include progressive dysfunction of other organ systems, particularly renal, lymphatic, gastrointestinal, and hepatic systems...
2016: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Jack Rychik
The Fontan operation is the anticipated palliative strategy for children born with single-ventricle type of congenital heart disease. As a result of important circulatory limitations, a series of end-organ complications are now increasingly recognized. Elevated central venous pressure and impaired cardiac output are the hallmarks of cavo-pulmonary flow, which result in a cascade of pathophysiological consequences. The Fontan circulation likely impacts all organ systems in an indolent and relentless manner, with progressive decline in functionality likely to occur in many...
2016: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Devaraj Navaratnam, Samantha Fitzsimmons, Michael Grocott, Harry B Rossiter, Yaso Emmanuel, Gerard-Paul Diller, Timothy Gordon-Walker, Sandy Jack, Nick Sheron, John Pappachan, Jayant Nick Pratap, Joseph J Vettukattil, Gruschen Veldtman
Increasingly end-organ injury is being demonstrated late after institution of the Fontan circulation, particularly liver fibrosis and cirrhosis. The exact mechanisms for these late phenomena remain largely elusive. Hypothesizing that exercise induces precipitous systemic venous hypertension and insufficient cardiac output for the exercise demand, that is, a possible mechanism for end-organ injury, we sought to demonstrate the dynamic exercise responses in systemic venous perfusion (SVP) and concurrent end-organ perfusion...
May 15, 2016: American Journal of Cardiology
I D Ba, A Ba, P M Faye, F N Diouf, A Sagna, A Thiongane, M D M Diop, A Sow, I Fall, M Ba
UNLABELLED: Liver abscess is a serious infection that can cause life-threatening complications. OBJECTIVE: To describe the epidemiology, diagnosis, and progression of liver abscess at the Dakar National Albert-Royer Children's Hospital. METHOD: A retrospective study was conducted from over a period of 5 years (1st January 2010 to 31st December 2014). All children aged 0-15 years hospitalized for liver abscess with ultrasound confirmation were included...
May 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Masamichi Ono, Jelena Kasnar-Samprec, Alfred Hager, Julie Cleuziou, Melchior Burri, Constantin Langenbach, Alessia Callegari, Martina Strbad, Manfred Vogt, Jürgen Hörer, Christian Schreiber, Rüdiger Lange
OBJECTIVES: This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome. METHODS: Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed. RESULTS: Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92...
March 23, 2016: European Journal of Cardio-thoracic Surgery
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