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.

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.

scholarly journals Managing adult Fontan patients: where do we stand?

2016 ◽  
Vol 25 (142) ◽  
pp. 438-450 ◽  
Author(s):  
Paul Clift ◽  
David Celermajer
Keyword(s):  
Quality Of Life ◽  
Life Expectancy ◽  
Fontan Operation ◽  
Adult Survivors ◽  
Late Complications ◽  
Therapeutic Interventions ◽  
Palliative Procedure ◽  
Fontan Patients

The Fontan operation is performed as a palliative procedure to improve survival in infants born with a functionally univentricular circulation. The success of the operation is demonstrated by a growing adult Fontan population that exists with this unique physiology. Late follow-up has demonstrated expected and unexpected sequelae, and has shown multisystem effects of this circulation. This review discusses the challenges of managing the late complications in terms of understanding this unique physiology and the innovative therapeutic interventions that are being investigated. The challenge remains to maintain quality of life for adult survivors, as well as extending life expectancy.

scholarly journals Neutrophil-to-Lymphocyte and Platelet-to-Lymphocyte Ratio in Univentricular Patients From Birth to Follow-Up After Fontan—Predicting Lymphatic Abnormalities

2021 ◽  
Vol 9 ◽  
Author(s):  
Julia Moosmann ◽  
Christian Schroeder ◽  
Robert Cesnjevar ◽  
Kathrin Rottermann ◽  
Annika Weigelt ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Lymphatic Malformations ◽  
Protein Losing Enteropathy ◽  
Lymphocyte Ratio ◽  
Lymphatic Abnormalities ◽  
Grade Classification ◽  
Magnetic Resonance Imaging Mri ◽  
Fontan Surgery ◽  
Lymphatic Imaging

Background: Reliable laboratory parameters identifying complications after Fontan surgery including the lymphatic abnormalities and the development of protein-losing enteropathy (PLE) are rare. Neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocte ratio (PLR) are inflammatory markers and have been studied to predict outcome and prognosis in various diseases. The aim of this study was to investigate NLR and PLR from birth to follow-up after Fontan and evaluate their use as prognostic parameters for single ventricle patients regarding the development of lymphatic malformations during follow-up.Materials and Methods: Sixty-six univentricular patients who underwent Fontan surgery and had 6-month follow-up magnetic resonance imaging (MRI) with T2 weighted lymphatic imaging after total cavopulmonary connection (TCPC) surgery were included in the study. NLR and PLR were determined at specific time points, from neonatal age to follow-up after Fontan operation and correlated to data from the MRI 6 months after Fontan.Results: NLR and PLR increase significantly over time from the first surgery during infancy to the follow-up after Fontan (both p &lt; 0.0001), with a significant increase after the Glenn surgery for both ratios (each p &lt; 0.0001). Higher NLR (p = 0.002) and higher PLR (p = 0.004) correlated with higher-grade classification of lymphatic abnormalities in T2-weighted imaging 6 months after Fontan surgery and higher NLR correlated with higher transpulmonary gradient prior to Fontan surgery (p = 0.035) Both ratios showed a significant correlation to total protein at follow-up (NLR p = 0.0038; PLR&lt;0.0001).Conclusion: Increased NLR and PLR correlate with higher degree lymphatic malformations after TCPC and therefore might contribute as valuable additional biomarker during follow-up after TCPC. NLR and PLR are simple, inexpensive and easily available parameters to complement diagnostics after TCPC.

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.

Abstract 17119: Sufficient Pulmonary Vascular Bed Before Fontan Surgery Secures Favorable Fontan Hemodynamics

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Seiko Kuwata ◽  
Hirofumi Saiki ◽  
Manabu Takanashi ◽  
Kenji Sugamoto ◽  
Hideaki Senzaki
Keyword(s):  
Organ Dysfunction ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Fontan Circulation ◽  
Cardiovascular Remodeling ◽  
Vascular Bed ◽  
Pulmonary Arterial ◽  
Fontan Surgery ◽  
Fontan Patients ◽  
Pulmonary Vascular Bed

Introduction: While improvement of perioperative care markedly expanded candidacy of Fontan surgery, increasing number of Fontan patients is currently subjected to advanced heart failure therapies. Low pulmonary flow before Fontan procedure has been considered to be acceptable as it keeps pulmonary resistance low, however, compromised growth of pulmonary vascular bed after Fontan completion may deteriorate potential to preserve better Fontan circulation later. We tested our hypothesis that sufficient growth of pulmonary vascular bed before Fontan surgery chronically preserves favorable Fontan hemodynamics and prevents patients from cardiovascular remodeling. Methods and Results: Consecutive 33 patients who had undergone Fontan operation (median year after the surgery: 5.3years) were enrolled in this study. During cardiac catheterization, pulmonary arterial index (PAI) as the representative of vascular bed both before and after Fontan procedure was measured and hemodynamics as well as blood samples as a marker for end-organ dysfunction were analyzed. Before Fontan procedure, PAI ranged 74-426 and resistance of pulmonary artery (PAR) ranged 0.4-3.1. After median of 5.3 years of Fontan procedure, CVP exhibited modest decline with preserving cardiac index (CI). Interestingly, central venous pressure (CVP) with chronic Fontan circulation was negatively correlated with PAI of pre-Fontan procedure (P=0.02) whereas it was independent of PAR. Consistent with this, higher PAI before Fontan procedure was also significantly associated with higher CI (P<0.01) and lower levels of plasma brain natriuretic peptides (P=0.03). Importantly, patients with low PAI showed markedly elevated activation of renin-angiotensin-aldosterone (RAAS) activation and elevation of serum gamma-gltamyl transferase (P<0.01), a surrogate marker of liver congestion. Multivariate analysis revealed that high PAI was determinant of low CVP in the chronic Fontan patients, independent of body size, postoperative interval and pulmonary arterial resistance. Conclusion: Sufficient pulmonary vascular bed before Fontan procedure independently lowers CVP, may prevent cardiovascular remodeling by RAAS suppression as well as end organ dysfunction in chronic Fontan patients. Our result suggested importance of strategies to accelerate pulmonary vascular growth before Fontan procedure.

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.

Role of amiodarone in the management of atrial arrhythmias in adult Fontan patients

Heart ◽  
2020 ◽  
pp. heartjnl-2020-317378
Author(s):  
Satoshi Kawada ◽  
Praloy Chakraborty ◽  
Lucy Roche ◽  
Erwin N Oechslin ◽  
Candice Silversides ◽  
...  
Keyword(s):  
Side Effects ◽  
Thyroid Dysfunction ◽  
Common Side Effect ◽  
Cardiac Side Effects ◽  
Increased Risk ◽  
Term Administration ◽  
Fontan Patients

BackgroundPatients with Fontan circulation are known to be at high risk for developing atrial tachyarrhythmias (AAs). Our objective was to examine the efficacy and safety of amiodarone in the management of ATs in adult Fontan patients.MethodsPrimary outcomes of this single-centre, retrospective study included freedom from AAs and incidence of adverse effects of amiodarone on Fontan patients. Heart failure (HF) events and composite outcomes of death from any cause, Fontan revision and heart transplantation were evaluated as secondary outcomes. Predictors of HF and discontinuing amiodarone were also evaluated.ResultsA total of 61 patients (mean age 31.6±11.3 years, 40.9% female), who were treated with amiodarone in between 1995 and 2018, were included. AAs free survival at 1, 3 and 5 years were 76.2%, 56.9% and 30.6%, respectively. During a median follow-up of 50.5 months, 34 (55.7%) patients developed side effects, and 20 (32.8%) patients discontinued amiodarone due to side effects. Thyroid dysfunction was the most common side effect (n=26, 76.5%), amiodarone-induced thyrotoxicosis (AIT) (n=16, 27.1%) being most common thyroid dysfunction. Young age (age <28.5 years) was associated with discontinuing amiodarone (HR 5.50, 95% CI 1.19 to 25.4, p=0.029). AIT significantly increased risk of HF (HR 4.82, 95% CI 1.71 to 13.6, p=0.003).ConclusionsShort-term efficacy of amiodarone in Fontan physiology is acceptable. However, long-term administration is associated with a reduction of efficacy and a significant prevalence of non-cardiac side effects. AIT is associated with exacerbation of HF. The judicious use of amiodarone administration should be considered in this population.

Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy

2015 ◽  
Vol 25 (8) ◽  
pp. 1489-1492 ◽  
Author(s):  
Brian S. Snarr ◽  
Stephen M. Paridon ◽  
Jack Rychik ◽  
David J. Goldberg
Keyword(s):  
Single Ventricle ◽  
Fontan Operation ◽  
Vasodilator Therapy ◽  
Pulmonary Vasodilators ◽  
Pulmonary Vasodilator ◽  
Fontan Physiology ◽  
Fontan Surgery ◽  
Failing Fontan ◽  
Fontan Patients

AbstractThe Fontan operation is the final step of palliation for patients with a functionally single ventricle. Since its introduction in the 1970s, the Fontan surgery has become part of a successful surgical strategy that has improved single ventricle mortality. In recent years, we have become more aware of the limitations and long-term consequences of the Fontan physiology. Pulmonary vascular resistance plays an important role in total cavopulmonary circulation, and has been identified as a potential therapeutic target to mitigate Fontan sequelae. In this review, we will discuss the results of different pulmonary vasodilator trials and the use of pulmonary vasodilators as a treatment strategy for Fontan patients.

Abstract 19355: Lower Heart Rates are Associated with Better Mid-term Outcomes in Fontan Patients

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Clara Kurishima ◽  
Hideaki Senzaki ◽  
Tokuko Shinohara ◽  
Toshio Nakanishi
Keyword(s):  
Heart Failure ◽  
Therapeutic Target ◽  
Fontan Operation ◽  
Chronic Phase ◽  
Walk Distance ◽  
Heart Rates ◽  
Group 2 ◽  
Fontan Surgery ◽  
First Time ◽  
Fontan Patients

Background: In adult heart failure, higher heart rates (HR) are known to be associated with increased risks of myocardial infarction and sudden cardiac death. Thus, HR modulation has been increasingly recognized as a potentially effective therapy for heart failure. HR may also be a useful therapeutic target in patients after the Fontan surgery, in which effective treatment to improve prognosis remains to be established. We hypothesized that lower HR is associated with better mid-term outcomes in Fontan patients. Methods: We retrospectively analyzed 24-hour Holter ECG in 56 consecutive patients in the chronic phase (at least 1 year) after the Fontan operation and in the sinus rhythm (mean age; 19 years, range; 9-49 years). Data for minimum, maximum, and average HR were extracted. We then examined the relationships between HR values and mid-term hemodynamic and clinical (6-min walk-distance) outcomes. Mid-term outcomes were assessed both at 1-3 years (mean 1.5 years) after the initial Holter recording (Group 1) and at more than 3 years (mean 4.9 years) after the initial Holter recording (Group 2). Results: Lower values of mean and minimum HR were significantly correlated with lower CVP in both groups (P<0.05, for each). Mean and minimum HR were also significantly and negatively correlated with the 6-min walk-distance in Group 2 (P <0.05 for each). The results were similar after controlling for age by multivariate analysis. Importantly, lower HR was not associated with decreased cardiac output. Conclusions: The present study demonstrated for the first time that lower HR can lead to better mid-term outcomes of hemodynamics and exercise capacity in Fontan patients. HR can be an important therapeutic target to improve the prognosis after the Fontan operation.

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