scholarly journals Radiologic follow-up in Fontan-associated liver disease in Europe: European Society of Paediatric Radiology survey demonstrates the need for a consensus protocol

2021 ◽  
Author(s):  
Giulia Perucca ◽  
Charlotte de Lange ◽  
Stéphanie Franchi-Abella ◽  
Marcello Napolitano ◽  
Michael Riccabona ◽  
...  
Keyword(s):  
Liver Disease ◽  
European Society ◽  
Current Practice ◽  
Paediatric Radiology ◽  
Consensus Protocol ◽  
Online Questionnaire ◽  
Life Saving ◽  
Fontan Surgery ◽  
Fontan Patients

AbstractFontan surgery is a life-saving procedure for newborns with complex cardiac malformations, but it originates complications in different organs. The liver is also affected, with development of fibrosis and sometimes cirrhosis and hepatocellular carcinoma. There is no general agreement on how to follow-up these children for the development of liver disease. To understand the current practice on liver follow-up, we invited members of the European Society of Paediatric Radiology (ESPR) to fill out an online questionnaire. The survey comprised seven questions about when and how liver follow-up is performed on Fontan patients. While we found some agreement on the use of US as screening tool, and of MRI for nodule characterization, the discrepancies on timing and the lack of a shared protocol make it currently impossible to compare data among centers.

scholarly journals Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of Paediatric Radiology Abdominal Task Force

2021 ◽  
Author(s):  
Giulia Perucca ◽  
Charlotte de Lange ◽  
Stéphanie Franchi-Abella ◽  
Marcello Napolitano ◽  
Michael Riccabona ◽  
...  
Keyword(s):  
Liver Disease ◽  
European Society ◽  
Task Force ◽  
Fontan Procedure ◽  
Neurologic Complications ◽  
Paediatric Radiology ◽  
Surgical Palliation ◽  
Current Standards ◽  
Heart Malformations

AbstractSince Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.

Impact of the Fontan Operation on Organ Systems

2019 ◽  
Vol 19 (3) ◽  
pp. 205-214
Author(s):  
Miriam Michel ◽  
Manuela Zlamy ◽  
Andreas Entenmann ◽  
Karin Pichler ◽  
Sabine Scholl-Bürgi ◽  
...  
Keyword(s):  
Systematic Study ◽  
Fontan Operation ◽  
Gastrointestinal System ◽  
Adjunct Treatment ◽  
Organ Systems ◽  
Fontan Surgery ◽  
Fontan Patients

: In patients having undergone the Fontan operation, besides the well discussed changes in the cardiac, pulmonary and gastrointestinal system, alterations of further organ systems including the hematologic, immunologic, endocrinological and metabolic are reported. As a medical adjunct to Fontan surgery, the systematic study of the central role of the liver as a metabolizing and synthesizing organ should allow for a better understanding of the pathomechanism underlying the typical problems in Fontan patients, and in this context, the profiling of endocrinological and metabolic patterns might offer a tool for the optimization of Fontan follow-up, targeted monitoring and specific adjunct treatment.

(AVK) Anti Vitamin K Treatment In patients with Fontan Surgery

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4397-4397
Author(s):  
Maria Stefania Infante ◽  
Cristina Pascual Izquierdo ◽  
Fernando Carretero Lopez ◽  
Jose Manuel Sanchez Ramirez ◽  
Ana Maria Rodriguez Huerta ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Fontan Operation ◽  
Thrombotic Event ◽  
Bleeding Complications ◽  
Target Range ◽  
Group A ◽  
Group B ◽  
Fontan Surgery ◽  
Fontan Patients

Abstract Abstract 4397 OBJECTIVES Thrombotic events have been reported as a major cause of morbidity after the Fontan procedure. There is no consensus about the type and duration of postoperative anticoagulation prophylaxis, due to the high risk of bleeding complications, the difficulties in monitoring and the questionable therapeutic compliance in children. In spite of the lack of long term prospective studies in this situation, the ACCP has recommended in their guidelines OAT following Fontan or Glenn operation for at least 6 months. This has also been our practice in our institution during the period of study. AIM OF THE STUDY To analyze the efficacy and complications of OAT in our pediatric patients after undergoing the Fontan operation. METHODS Retrospective chart review of all the children treated with OAT in our institution between 1995 and 2009. All patients were treated initially with acenocumarol 0,2 mg/kg, except the Fontan patients, who received 0,1 mg/kg. Target INR was 2–3 for all patients. The Mann-Whitney test was used to compare the rate of complications, and the percentage of visits out of target INR between the Fontan patients and the rest of the cohort. RESULTS There were 61 children (26 female/35 males) aged between 1 month and 17 years, who received OAT with a range of follow up between 4 months and 14 years: 27 after Fontan operation (Group A), an 34 for other reasons (Group B: n=13 non prothetic valve cardiopathy, n=21 treatment of thromboembolic disease). The average follow-up was similar in both groups (median of 6.5 months in group A vs.7.5 months in group B). There were few complications: 1 mild epistaxis and 1 thrombotic event in group B, and none in group A. There were no differences in the proportion of controls in normal range between both groups; there was a moderate proportion of controls outside the target range of INR, with higher distribution below the range than above the range. The median dose used to achieve the target INR was 0.3 mg/kg/d in Group A and 0.4 mg/kg/d in Group B. CONCLUSIONS Oral Anticoagulant therapy is safe and effective in pediatric patients,with very low rates of thrombotic or hemorragic complications including those undergoing the Fontan surgery. There were not any differences between both groups in any of the analizyed parameters. Disclosures: No relevant conflicts of interest to declare.

scholarly journals P.096 Practice patterns in the management of residual/recurrent non-functioning pituitary adenomas: results from a Canada-wide survey

2019 ◽  
Vol 46 (s1) ◽  
pp. S39
Author(s):  
N Samuel ◽  
G Kasper ◽  
OH Khan
Keyword(s):  
Pituitary Adenomas ◽  
Current Practice ◽  
Practice Patterns ◽  
Recurrent Disease ◽  
Treatment Options ◽  
Subsequent Treatment ◽  
Online Questionnaire ◽  
Radiation Oncologists ◽  
Non Functioning Pituitary Adenomas

Background: Postoperative follow-up of non-functioning pituitary adenomas (NFAs) occasionally detects residual or recurrent disease. Subsequent treatment options range from continued follow-up, to re-resection or radiotherapy. To better understand current practice patterns on this topic, we surveyed neurosurgeons and radiation oncologists in Canada. Methods: Skull-base neurosurgeons and radiation oncologists across Canada were invited to complete a 25-item online questionnaire. Summary statistics were computed and 2-tailed t-tests were performed to assess significance. Results: 33 participants returned completed questionnaires: neurosurgeons (n=20, 61%) and radiation oncologists (ROs; n=13, 39%). When treating giant (>3cm) tumours, 92% of neurosurgeons in practice for less than 15 years use an endoscopic approach, as compared to only 57% of neurosurgeons in practice for 15 years or more. Additionally, younger neurosurgeons have a greater tendency to advocate for stereotactic radiosurgery (SRS) or re-resection (54% and 38%, respectively), as compared to older surgeons who show a higher propensity (29%) to advocate for observation. The presence of cavernous sinus extension appears to encourage neurosurgeons (40%) to offer radiotherapy sooner, as compared to 62% of ROs. Conclusions: Our results identify both variations and commonalities in practice amongst Canadian neurosurgeons. Approaches deviate in the setting of residual tumors based on years of practice.

scholarly journals Assessment of Liver Stiffness in Pediatric Fontan Patients Using Transient Elastography

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Becky Chen ◽  
Richard A. Schreiber ◽  
Derek G. Human ◽  
James E. Potts ◽  
Orlee R. Guttman
Keyword(s):  
Transient Elastography ◽  
Liver Stiffness ◽  
Noninvasive Method ◽  
Demographic Information ◽  
Fontan Surgery ◽  
Fontan Patients ◽  
Age And Sex ◽  
Patients Acceptance

Background. Hepatic fibrosis is a potential complication following Fontan surgery and heralds long-term risk for cirrhosis. Transient elastography (TE) is a rapid, noninvasive method to assess liver fibrosis by measuring liver stiffness.Objectives. To compare liver stiffness and liver biochemistries in pediatric Fontan patients with age- and sex-matched controls and to determine patients’ acceptance of TE.Methods. Patients were recruited from British Columbia Children’s Hospital. Twenty-two Fontan patients (15 males) were identified. Demographic information and cardiac data were collected. TE was measured using size-appropriate probes.Results. The median age of the Fontan cohort was 13.7 (5.9–16.8) years. Time from Fontan surgery to TE was 9.6 (1.0–12.9) years. The median Fontan circuit pressure was 13 (11–14) mmHg. TE values were higher in Fontan patients versus controls (18.6 versus 4.7 kPa,p<0.001). There was no association between TE values and patient age (r=0.41,p=0.058), time since Fontan surgery (r=0.40,p=0.062), or median Fontan circuit pressure (CVP) (r=0.35,p=0.111). Patients found TE to be nonpainful, convenient, and safe.Conclusions. TE is feasible to assess liver stiffness in children following Fontan surgery. Pediatric Fontan patients have markedly elevated liver stiffness values. TE may have important utility in liver care follow-up of pediatric Fontan patients.

scholarly journals Management of Latent Tuberculosis Infections in Australia and New Zealand: A Review of Current Practice

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Justin T. Denholm ◽  
Emma S. McBryde
Keyword(s):  
New Zealand ◽  
Current Practice ◽  
Latent Tuberculosis ◽  
Large Degree ◽  
Physician Education ◽  
Online Questionnaire ◽  
Liver Function Testing ◽  
Clinical Scenarios ◽  
Function Testing

Aim: To survey practices in the diagnosis and management of latent tuberculosis infection (LTBI) in Australia and New Zealand.Methods: Infectious diseases and respiratory physicians and trainees were invited to complete an online questionnaire concerning various aspects of LTBI management.Results: The questionnaire was completed by 126 clinicians self-reporting regular management of LTBI. Respondents were experienced physicians, with 95/126 (75.4%) having managed LTBI for more than 5 years. Forty-seven (37.3%) reported seeing more than 5 patients per month for assessment of LTBI. Substantial variation among clinicians was reported in relation to a number of common clinical scenarios. For instance, while 52/126 (43.7%) informed patients that the incidence of severe hepatotoxicity related to isoniazid monotherapy was 0.1–0.5%, 21/126 (15.7%) thought it was>5%. 36/126 (28.6%) clinicians would proceed with TNF-atherapy following an indeterminate screening: interferon-?assay, while 78/126 (61.9%) would perform further investigations and 12/126 (9.5%) would initiate isoniazid therapy. Follow-up intervals during therapy varied from 1–3 monthly, with liver function testing performed routinely by 89/126 (70.6%).Conclusion: This study demonstrated a large degree of variation in clinical practice of LTBI management in Australia and New Zealand. Strategies for increasing uniformity of practice are required, including improved guidelines and physician education.

scholarly journals European guideline for imaging in paediatric and adolescent rhabdomyosarcoma — joint statement by the European Paediatric Soft Tissue Sarcoma Study Group, the Cooperative Weichteilsarkom Studiengruppe and the Oncology Task Force of the European Society of Paediatric Radiology

2021 ◽  
Author(s):  
Roelof van Ewijk ◽  
Reineke A. Schoot ◽  
Monika Sparber-Sauer ◽  
Simone A. J. ter Horst ◽  
Nina Jehanno ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Children And Adolescents ◽  
European Society ◽  
Task Force ◽  
Study Group ◽  
Paediatric Radiology ◽  
European Paediatric ◽  
Imaging Guideline

AbstractAppropriate imaging is essential in the treatment of children and adolescents with rhabdomyosarcoma. For adequate stratification and optimal individualised local treatment utilising surgery and radiotherapy, high-quality imaging is crucial. The paediatric radiologist, therefore, is an essential member of the multi-disciplinary team providing clinical care and research. This manuscript presents the European rhabdomyosarcoma imaging guideline, based on the recently developed guideline of the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) Imaging Committee. This guideline was developed in collaboration between the EpSSG Imaging Committee, the Cooperative Weichteilsarkom Studiengruppe (CWS) Imaging Group, and the Oncology Task Force of the European Society of Paediatric Radiology (ESPR). MRI is recommended, at diagnosis and follow-up, for the evaluation of the primary tumour and its relationship to surrounding tissues, including assessment of neurovascular structures and loco-regional lymphadenopathy. Chest CT along with [F-18]2-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET)/CT or PET/MRI are recommended for the detection and evaluation of loco-regional and distant metastatic disease. Guidance on the estimation of treatment response, optimal long-term follow-up, technical imaging settings and standardised reporting are described. This European imaging guideline outlines the recommendations for imaging in children and adolescents with rhabdomyosarcoma, with the aim to harmonise imaging and to advance patient care.

Abstract 16359: Incidence of Fontan Associated Liver Disease and Its Impact on Mortality in Patients With Single Ventricle Physiology

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Carlos Guerrero ◽  
judith Therrien ◽  
Aihua Liu ◽  
Liming Guo ◽  
Masaki Kodaira ◽  
...  
Keyword(s):  
Liver Disease ◽  
Fontan Operation ◽  
Population Based ◽  
Cumulative Probability ◽  
P Value ◽  
Logrank Test ◽  
Long Term Follow Up ◽  
The Impact ◽  
Fontan Patients

Introduction: Data on the burden of clinically significant Fontan associated liver disease (FALD) and its relationship to mortality is scant. We performed a retrospective cohort study to assess the incidence of FALD and its association with mortality. Methods: Data source was the Quebec Congenital Heart Disease (CHD) Database, a population-based cohort of over 100,000 CHD patients followed from 1983-2017. Fontan patients surviving longer than 30 days post-Fontan were identified, each were matched to 20 VSD patients on age and sex. The Fontan-VSD cohort were used to assess the association between Fontan and the risk of developing FALD. The VSD cohort served as “Non-exposed to Fontan” group. FALD was defined as at least one hospitalization due to liver disease. Kaplan-Meier curves were used to estimate and compare the cumulative probability of 1) developing FALD between Fontan and VSD patients; and 2) mortality between Fontan patients w/o FALD. Results: A total of 512 Fontan patients and 10,232 VSD patients were included. The cumulative probability of developing FALD at 10 and 25 years of follow-up was higher in Fontan patients (13.0% and 37.1%, respectively), compared to 0.7% and 2.0% for VSD patients respectively (p-value <0.0001-Logrank test). In Fontan patients with FALD, the cumulative probability of mortality by 5 years after the diagnosis of FALD was 12.6%, 11 times higher than the risk in Fontan Patients without FALD (Figure) . Calendar year of Fontan operation was found to be an important predictor of developing FALD. Conclusions: This is the first study documenting the impact of FALD on mortality, using a large cohort with long-term follow up. The findings support the use of well-defined surveillance protocols to identify potential precipitants of FALD before liver disease becomes irreversible.

Abstract 2972: Permanent Atrial Pacing Lead Implantation Route After Fontan Operation

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Kazuhiro Takahashi ◽  
Frank Cecchin ◽  
Elizabeth B Fortescue ◽  
Charles I Berul ◽  
Mark E Alexander ◽  
...  
Keyword(s):  
Thromboembolic Event ◽  
Sinus Node ◽  
Fontan Operation ◽  
Atrial Pacing ◽  
Retained Foreign Body ◽  
Pacing Lead ◽  
Fontan Surgery ◽  
Skin Erosion ◽  
Fontan Patients

Background: Atrial pacing is commonly used for sinus node dysfunction (SND) after Fontan surgery. The preferred route of lead implant has been debated. We compare procedural and clinical outcomes of transvenous (TV) and epicardial (Epi) atrial lead implants in this population. Methods: All Fontan patients having an atrial lead implant without other associated surgery between 1992 and 2007 were studied. Demographics, pacing lead performance data and procedural outcome were retrospectively analyzed. Results: 78 patients (22 TV and 56 Epi) had 90 leads implanted (25 TV/ 65 Epi). Mean follow-up was 3.3±4.1 yrs (TV) and 4.4±3.6 yrs (Epi). TV leads were implanted in older patients (mean age: 23.9 vs. 13.8 yrs, p<0.001), at longer interval after Fontan surgery (mean: 15.1 vs. 5.7 yrs, p<0.001). Indication for TV was more likely to be SND, while Epi pacing was indicated for AV block. Pacing modes were exclusively AAI/AAI-T in TV and mostly DDD in Epi leads. Acute complication occurred in 2/25 (8%) of TV (pneumothorax n=1, skin erosion n=1) and 12/65 (19%, p=0.23) of Epi implants (effusions n=5, heart failure n=1, retained foreign body n=1, pneumothorax n=1, hematoma n=1, disconnection n=1, sepsis n=1, blood loss n=1). Median hospital stay was shorter in TV (2.0 vs 4.5 days, p=0.03). All TV patients and 43 Epi (77%) were anticoagulated. At follow-up, pocket infection occurred in one Epi patient. No clinical thromboembolic event was observed in either group. 3 TV leads failed in 2 patients (9%), while 12 Epi leads failed in 10 patients (18%); however, mean duration of freedom from lead failure was not significantly different (TV 9.9 vs. Epi 8.0 yrs, P=ns). The mean energy threshold was lower at implant for TV leads (0.9 vs 2.2 μJ, P=0.049), but similar for both leads on follow-up (1.2 vs 2.6 μJ, P=0.35). Atrial sensing was unchanged over time for TV (2.2 to 2.0 mV, P=ns), but significantly decreased in Epi (3.4 to 2.4 mV, p=0.006). Conclusions: Transvenous atrial pacing leads may be placed in Fontan patients with lower procedural morbidity than epicardial leads, and equivalent expectation of lead performance and longevity. Although the thromboembolic event rate appears low in anticoagulated patients with both lead types, the present study design cannot fully address this important issue.

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