scholarly journals A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome

2019 ◽  
Vol 29 (3) ◽  
pp. 461-468 ◽  
Author(s):  
Friso M Rijnberg ◽  
Nico A Blom ◽  
Vladimir Sojak ◽  
Eline F Bruggemans ◽  
Irene M Kuipers ◽  
...  
Keyword(s):  
Adverse Outcome ◽  
Sinus Node ◽  
Fontan Procedure ◽  
Study Cohort ◽  
Protein Losing Enteropathy ◽  
Long Term Outcome ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Fontan Failure ◽  
Important Sign

Abstract OBJECTIVES This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity. METHODS Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016. RESULTS The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1–Q3 7.6–17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18–44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63–96%) at 20 years for lateral tunnel and 99% (95% CI 96–100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III–IV) at 15 years was 65% (95% CI 52–76%) for atriopulmonary connection/Björk, 90% (95% CI 73–97%) for lateral tunnel and 90% (95% CI 82–94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2–5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4–9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4–9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0–11.1; P < 0.001). CONCLUSIONS With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.

Late outcomes after the Fontan procedure in patients with single ventricle: a meta-analysis

Heart ◽  
2018 ◽  
Vol 104 (18) ◽  
pp. 1508-1514 ◽  
Author(s):  
Ilana Schwartz ◽  
Courtney E McCracken ◽  
Christopher J Petit ◽  
Ritu Sachdeva
Keyword(s):  
Single Ventricle ◽  
Meta Analysis ◽  
Fontan Procedure ◽  
Time Intervals ◽  
Protein Losing Enteropathy ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Fontan Physiology ◽  
Pacemaker Insertion

ObjectiveMore patients with Fontan physiology are reaching adulthood. The purpose of this meta-analysis was to evaluate the late outcomes of patients palliated with Fontan procedure and to assess the risk factors for mortality.MethodsPubMed, Embase and Web of Science were queried to retrieve observational studies of survival in patients following the Fontan procedure with ≥5 years of follow-up. A random-effects model was used to determine pooled survival estimates at 5, 10 and 15 years. Meta-regression was used to assess potential moderators for death.ResultsNineteen articles with a total of 5859 patients were included. The weighted mean follow-up time was 8.94±2.64 years with overall 8.3% deaths and 1.5% transplants. Pooled survival estimates at 5, 10 and 15 years were 90.7%, 87.2% and 87.5%, respectively; and 88.4%, 85.7% and 84.1%, respectively, for studies that included all three time intervals (n=4). Earliest surgical year included in the study, proportion of atriopulmonary connections versus extracardiac conduit or lateral tunnel, and older age at Fontan were associated with higher rates of death, but ventricular morphology was not. Protein-losing enteropathy, reoperation and pacemaker insertion were reported in 2.1%, 5.6% and 6.8% patients, respectively.ConclusionsSurvival following the Fontan procedure has improved with time and is influenced by Fontan type and age at the time of Fontan. At a mean follow-up of 8.9 years, there was no significant association between survival and ventricular morphology, not taking into account the mortality prior to Fontan.

Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle

Heart ◽  
2021 ◽  
pp. heartjnl-2021-319760
Author(s):  
Christopher Anigwe ◽  
Vidhushei Yogeswaran ◽  
Anita Moon-Grady ◽  
Sophie McAllister ◽  
Anika Aggarwal ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Adverse Outcome ◽  
Survival Rates ◽  
Adult Survivors ◽  
Adverse Outcomes ◽  
Protein Losing Enteropathy ◽  
Tertiary Centre ◽  
Lateral Tunnel ◽  
Extracardiac Fontan ◽  
Fontan Palliation

ObjectiveThis tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle.MethodsThis retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death.Results160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8–21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration.ConclusionsIn this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients.

Long-term outcome after atrioventricular valve surgery following modified Fontan operation

2010 ◽  
Vol 21 (1) ◽  
pp. 83-88 ◽  
Author(s):  
Shaji C. Menon ◽  
Joseph A. Dearani ◽  
Frank Cetta
Keyword(s):  
Ventricular Function ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Valve Surgery ◽  
Atrioventricular Valve ◽  
Term Outcome ◽  
Protein Losing Enteropathy ◽  
Long Term Outcome

AbstractObjectiveThe objective of this study was to evaluate the early and late results of atrioventricular valve surgery after Fontan operation.BackgroundAtrioventricular valve regurgitation is a known perioperative risk factor for Fontan operation. There are limited data on the outcomes of late atrioventricular valve surgery following Fontan operation.MethodsPatients who underwent atrioventricular valve surgery following Fontan procedure were identified from the Mayo Clinic Fontan database. Medical records were reviewed for pre-operative, operative, and post-operative clinical and haemodynamic data. All patients not known to be deceased were sent health status questionnaires.ResultsA total of 61 patients (28 females) underwent atrioventricular valve surgery following Fontan procedure. The median age at atrioventricular valve surgery was 14 years. The median duration between Fontan and atrioventricular valve surgery was 4.7 years. Median follow-up was 9 years. There were a total of 32 (52%) deaths with 8 (13%) within 30 days of surgery. The 5-, 10-, and 15-year survival rates were 67%, 57%, and 45%, respectively. On follow-up, 44 of 61 (72%) had arrhythmias, 21 of 29 (72%) were symptomatic, and 12 of 61 (20%) developed protein-losing enteropathy. On multivariate analysis, reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.ConclusionAtrioventricular valve surgery after Fontan procedure is associated with substantial late morbidity and mortality. Atrioventricular valve surgery in this cohort of patients portends poor long-term outcome and is associated with a high incidence of protein-losing enteropathy. Reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.

Computational Model for the Study of Fontan Circulation

2018 ◽  
pp. 587-606
Author(s):  
Alejandro Talaminos-Barroso ◽  
Laura María Roa-Romero ◽  
Javier Reina-Tosina
Keyword(s):  
Computational Model ◽  
Cardiovascular System ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Univentricular Heart ◽  
Physiological Basis ◽  
Hemodynamic Variables ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Key Factor

In this chapter, the design and development of a computational model of the cardiovascular system is presented for patients who have undergone the Fontan operation. The model has been built from a physiological basis, considering some of the mechanisms associated to the cardiovascular system of patients with univentricular heart disease. Thus, the model allows the prediction of some hemodynamic variables considering different physiopathological conditions. The original conditions of the model are changed in the Fontan procedure and these new dynamics force the hemodynamic behaviours of the different considered variables. The model has been proved considering the classic Fontan procedure and the techniques from the lateral tunnel and the extracardiac conduit. The results compiled knowledge of several cardiovascular surgeons with many years of experience in such interventions, and have been validated by using other authors' data. In this sense, the participation of a multidisciplinary team has been considered as a key factor for the development of this work.

scholarly journals Comparison of lateral tunnel and extracardiac conduit Fontan procedure

2007 ◽  
Vol 6 (3) ◽  
pp. 328-330 ◽  
Author(s):  
J. R. Lee ◽  
J. Kwak ◽  
K. C. Kim ◽  
S. K. Min ◽  
W.-H. Kim ◽  
...  
Keyword(s):  
Fontan Procedure ◽  
Extracardiac Conduit ◽  
Lateral Tunnel

Comparison of Clinical and Simulation Results for the Stanford Y-Graft Fontan Pilot Trial

2012 ◽  
Author(s):  
Weiguang Yang ◽  
Jeffrey A. Feinstein ◽  
V. Mohan Reddy ◽  
Frandics P. Chan ◽  
Alison L. Marsden
Keyword(s):  
Inferior Vena ◽  
Single Ventricle ◽  
Heart Defects ◽  
Fontan Procedure ◽  
Pilot Trial ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Third Stage

Without surgical palliation, single ventricle heart defects are uniformly fatal. A three-staged surgical repair is typically performed on these patients, who are otherwise severely cyanotic. In the third stage, the Fontan procedure, the inferior vena cava (IVC) is connected to the pulmonary arteries (PAs) via a lateral tunnel or extracardiac conduit. Following Fontan completion, deoxygenated blood from the upper and lower body is redirected to the PAs, bypassing the heart.

Long-term outcome and anaesthetic management for non-cardiac surgery after Fontan palliation: a single-centre retrospective analysis

2014 ◽  
Vol 25 (6) ◽  
pp. 1148-1154 ◽  
Author(s):  
Thomas Palumbo ◽  
Thierry Sluysmans ◽  
Jean E. Rubay ◽  
Alain J. Poncelet ◽  
Mona Momeni
Keyword(s):  
Cardiac Surgery ◽  
Surgical Techniques ◽  
Bleeding Complications ◽  
Term Outcome ◽  
Term Survival ◽  
Long Term Outcome ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Significant Difference

AbstractObjectives: The improved management of Fontan patients has resulted in good outcome. As such, these patients may necessitate care for non-cardiac surgery. We sought to determine the long-term outcome of our Fontan series palliated with the most recent surgical techniques. Our second objective was to report the incidence and the perioperative course after non-cardiac procedures. We reviewed the records of all patients with either a lateral tunnel or an extracardiac conduit Fontan between 1996 and 2008. Follow-up was recorded until June, 2013, including records regarding non-cardiac interventions. Results: Overall, 58 patients were included. Of them, one patient underwent a takedown of his Fontan, and five patients died in the immediate postoperative course. The cumulative survival of the remaining 52 patients was 81%. There was no significant difference in survival between right and left ventricle morphologies (p=0.56), nor between both types of Fontan (p=0.9). Chronic arrhythmias (25%), fatigue/dyspnoea (40%), thrombotic complications (19%), and embolic events (10%) were among the most recurrent comorbidities. In total, 45 non-cardiac interventions were performed on 26 patients, with three bleeding complications and one death. Conclusions: This study shows excellent long-term survival after both lateral tunnel and extracardiac conduit Fontan. The incidence of cardiovascular morbidity remains high, however. We also report a high number of non-cardiac interventions. Thorough understanding of the Fontan physiology is mandatory when non-cardiac anaesthesiologists are in charge of these patients.

Sign in / Sign up

Export Citation Format

Share Document