Comparison of extracardiac conduit and lateral tunnel for functional single-ventricle patients: A meta-analysis

2017 ◽  
Vol 12 (6) ◽  
pp. 711-720 ◽  
Author(s):  
Zhiyong Lin ◽  
Hanwei Ge ◽  
Jiyang Xue ◽  
Guowei Wu ◽  
Jie Du ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Meta Analysis ◽  
Extracardiac Conduit ◽  
Lateral Tunnel

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.

Improved early results with cavopulmonary connections

2001 ◽  
Vol 11 (1) ◽  
pp. 3-11 ◽  
Author(s):  
Antonio R. Mott ◽  
Thomas L. Spray ◽  
J. William Gaynor ◽  
Rodolfo I. Godinez ◽  
Susan C. Nicolson ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Demographic Data ◽  
Diuretic Therapy ◽  
Double Outlet Right Ventricle ◽  
Long Term Results ◽  
Atrial Pacing ◽  
Perioperative Morbidity ◽  
Early Results ◽  
Extracardiac Conduit ◽  
Lateral Tunnel

AbstractBackground: We describe the recent results in a large cohort of patients with functionally single ventricle who underwent various modifications of cavopulmonary connections.Methods: Using the database at our institution, we identified all children who underwent cavopulmonary connection operations between June 1995 and June 1997. Demographic data, surgical history, and perioperative course were reviewed.Results: We performed 130 consecutive operations in 113 patients. The procedures included superior cavopulmonary connections in the form of the HemiFontan procedure in 45 instances, and bidirectional Glenn procedures in 11, and bilateral superior cavopulmonary connections in 7. The median age of these patients was 7.0 months. We completed Fontan operations using a fenestrated lateral tunnel on 47 occasions, and using an extracardiac conduit 9 times, 5 of which were fenestrated. A lateral tunnel without fenestration was constructed inone patient. The median age for these procedures was 19.5 months. In the remaining 10 instances, we revised Fontan procedures at a median age of 8 years. Diagnoses included hypoplastic left heart syndrome in 43 patients, double outlet right ventricle in 22, heterotaxy in 13, tricuspid atresia in 13, and a miscellaneous group accounting for the other 22. One death (0.7%) occurred within 30 days of surgery. Clinical seizures occurred in 7 children (5.3%), 6 had no residual neurologic deficits. Atrial pacing was needed in 14 children (10.7%) because of transient junctional rhythm, and 2 received treatment for supraventricular tachycardia. Pleural effusions were diagnosed radiographically after 31 of 130 (24%) procedures. Diuretic therapy resolved the effusion in 21 of these, with only 6 children requiring thoracostomy catheter drainage, and 4 undergoing thoracentesis alone. The median length of stay on the intensive care unit was 2 days, with a range from 1 to 30 days, and median stay in hospital was 6 days, with a range from 3 to 58 days.Conclusion: Mortality and perioperative morbidity after cavopulmonary connections have decreased dramatically in the current era. The long-term results of staged reconstruction for functionally single ventricle, nonetheless, await ongoing study.

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.

Fontan with lateral tunnel is associated with improved survival compared with extracardiac conduit

2020 ◽  
Vol 159 (4) ◽  
pp. 1480-1491.e2 ◽  
Author(s):  
Viktoria H.M. Weixler ◽  
David Zurakowski ◽  
John Kheir ◽  
Alvise Guariento ◽  
A.K. Kaza ◽  
...  
Keyword(s):  
Extracardiac Conduit ◽  
Lateral Tunnel

Abstract 16822: Growth Curves Describing a Single Ventricle Population After Fontan Procedure

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Bethany Runkel ◽  
Vincent Staggs ◽  
Chelsea Hosey Cojocari ◽  
Jonathan B Wagner
Keyword(s):  
Single Ventricle ◽  
Multiple Stressors ◽  
Pediatric Population ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Growth Patterns ◽  
Growth Charts ◽  
Genetic Syndrome ◽  
Adiposity Rebound ◽  
Extracardiac Conduit

Introduction: Complex single ventricle congenital heart disease (CHD) patients are subject to multiple stressors early in life that affect somatic growth. Pre-Fontan growth patterns are well-described, but post-Fontan growth has not been extensively studied in the current era. We sought to describe post-Fontan growth patterns by creating sex-specific body mass index (BMI)-for-age curves. Hypothesis: There is no difference in the growth of post-Fontan pediatric patients when compared to published normative data. Methods: A single-center, preexisting database was retrospectively queried for all patients who underwent Fontan procedure between 2006 and 2018. Patients with a genetic syndrome, a primary endocrine disorder, or significant prematurity were excluded. BMI-for-age curves were created for both male and female patients using anthropometric data extracted from the electronic medical record. Curves were then visually compared with Centers for Disease Control and Prevention (CDC) growth charts. Results: Of 227 patients who underwent primary Fontan operation, 37 were excluded. Of the remaining 190 patients, 59% were male. All had an extracardiac conduit, half had right-ventricular dominant CHD, and 15% had a Fontan fenestration. Median age and weight at surgery were 3.9 years and 15 kilograms, respectively. BMI curves were similar in appearance to CDC BMI-for-age growth charts, with adiposity rebound at age 6. BMI increased more rapidly in teen boys compared with girls, and a total of five patients (2.6%) had BMI values greater than 25 kg/m 2 at 16 years of age. Conclusion: Post-Fontan patients at our institution demonstrate BMI-for-age patterns similar to those of the general population during childhood and early adolescence, with adiposity rebound occurring at a typical age. Though excess weight gain does not completely spare patients with single ventricle CHD, overweight status appears to be less common than in the general pediatric population.

scholarly journals LONG TERM OUTCOMES AFTER THE FONTAN PROCEDURE IN PATIENTS WITH SINGLE VENTRICLE: A META-ANALYSIS

2017 ◽  
Vol 69 (11) ◽  
pp. 622
Author(s):  
Ilana Schwartz ◽  
Courtney McCracken ◽  
Christopher Petit ◽  
Ritu Sachdeva
Keyword(s):  
Single Ventricle ◽  
Meta Analysis ◽  
Fontan Procedure ◽  
Long Term Outcomes

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.

Modes of late mortality in patients with a Fontan circulation

Heart ◽  
2020 ◽  
Vol 106 (18) ◽  
pp. 1427-1431
Author(s):  
Chin Poh ◽  
Tim Hornung ◽  
David S Celermajer ◽  
Dorothy J Radford ◽  
Robert N Justo ◽  
...  
Keyword(s):  
Neurological Complication ◽  
Significant Proportion ◽  
National Registry ◽  
Fontan Circulation ◽  
Circulatory Failure ◽  
Special Focus ◽  
Late Death ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Patients At Risk

ObjectivesThe mechanisms of attrition of the Fontan population have been poorly characterised and it is unclear whether some of the deaths are potentially preventable. We analysed the circumstances of late death in patients with a Fontan circulation, with a special focus on identifying lesions amenable to intervention that may have contributed to the decline of their circulation.MethodsBetween 1975 and 2018, a total of 105 patients from a Bi-National Registry died beyond 1 year after Fontan completion, at a median age of 18.6 (IQR 13.8–26.0) years old, 12.7 (IQR 6.0–19.3) years after Fontan completion.ResultsA total of 105 patients died—63 patients (60%) with an atriopulmonary (AP) Fontan, 21 patients (20%) with a lateral tunnel (LT) and 21 patients (20%) with an extracardiac conduit (ECC). 72 patients (69%) were reviewed within 2 years preceding death, with 32% (23/72) deemed to be clinically well. Fontan circulatory failure was the most common cause of death in 42 patients (45%). Other causes of death included sudden death/arrhythmia (19%), perioperative death (12%), neurological complication (7%) and thromboembolism (7%). All patients with an LT or ECC who died from Fontan failure had at least one surgical defect that was amenable to intervention at time of death.ConclusionsConventional clinical surveillance has been insensitive in detecting a significant proportion of patients at risk of late death. Fontan circulatory failure contributes to half of the late deaths. Patients with an LT or ECC Fontan who died with a clinical picture of circulation failure may have potentially correctable lesions.

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