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.

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.

scholarly journals Atrioventricular valve closure in Fontan palliation

2019 ◽  
Vol 57 (5) ◽  
pp. 945-950 ◽  
Author(s):  
Gregory King ◽  
David S Winlaw ◽  
Nelson Alphonso ◽  
David Andrews ◽  
Kirsten Finucance ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Population Based ◽  
Valve Closure ◽  
Atrioventricular Valve ◽  
Atrioventricular Valve Regurgitation ◽  
Patch Technique ◽  
Pacemaker Insertion ◽  
Population Based Registry

Abstract OBJECTIVES Atrioventricular valve regurgitation is known to adversely impact outcomes of single-ventricle palliation, and valve repair rarely provides long-lasting results. Closure of a atrioventricular valve can sometimes be performed, but the long-term outcomes of this manoeuvre are unknown. METHODS This retrospective study was conducted using patient data extracted from an existing bi-national, population-based registry of survivors of the Fontan procedure. RESULTS Between January 1975 and June 2018, 1574 patients survived to hospital discharge with an intact Fontan circulation. Of these patients, 128 with a common atrioventricular valve were excluded. Thirty-eight patients underwent closure of an atrioventricular valve, and complete follow-up data were available for 36 patients. Twenty-nine patients underwent closure of the tricuspid valve and 7 patients underwent closure of the mitral valve. Seventeen patients underwent valve closure prior to Fontan, 13 patients underwent valve closure concomitant with Fontan and 6 patients underwent valve closure post-Fontan. Valve closure was performed using a patch technique in 29 cases and with direct suture in 7 cases. At the most recent echocardiography, 33 patients had no regurgitation, 2 patients had recurrent mild regurgitation and 1 patient had no echocardiographic follow-up. Six patients required reintervention post-valve closure and 7 patients required permanent pacemaker insertion post-valve closure. Freedom from reintervention at 1, 5 and 18 years post-valve closure was 86% [95% confidence interval (CI) 76–98%], 83% (95% CI 72–96%) and 83% (95% CI 72–96%), respectively. CONCLUSION Atrioventricular valve closure is an effective surgical technique in selected patients with a single ventricle providing long-lasting competency in the majority of cases.

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

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.

scholarly journals Mid Term Results of Fenestrated Intra-Extracardiac Fontan Procedure: Insights, Experiences and Expectations

2021 ◽  
Author(s):  
Arda Ozyuksel ◽  
Baran Simsek ◽  
Sener Demiroluk ◽  
Murat Saygi ◽  
Mehmet Bilal
Keyword(s):  
Fontan Procedure ◽  
Arterial Oxygen Saturation ◽  
Atrioventricular Septal Defect ◽  
Double Outlet Right Ventricle ◽  
Arterial Oxygen ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Cavopulmonary Anastomosis ◽  
Mid Term Results

Background: Intraextracardiac Fontan procedure aimed to combine the advantages of lateral tunnel and extracardiac conduit modifications of the original technique. Herein, we present our experience in our patients with intraextracardiac fenestrated Fontan Procedure. Methods: A retrospective analysis was performed in order to evaluate intraextracardiac fenestrated Fontan patients between 2014 and 2021. Seventeen patients were operated on with a mean age and body weight of 9.1 ± 5.5 years and 28.6 ± 14.6 kg. Results: Sixteen patients (94%) were palliated as univentricular physiology with hypoplasia of one of the ventricles. One patient (6%) with well-developed two ventricles with double outlet right ventricle and complete atrioventricular septal defect had straddling of the chordae prohibiting a biventricular repair. All of the patients had cavopulmonary anastomosis prior to Fontan completion, except one case. Fenestration was performed in all cases. Postoperative mean pulmonary artery pressures and arterial oxygen saturation levels at follow up were 10 ± 2.4 mmHg and 91.3 ± 2.7 %, respectively. Mean duration of pleural drainage was 5.4 ± 2.3 days. All of the fenestrations are patent at a mean follow up period of 4.8 ± 7.7 years, except one case. Any morbidity and mortality were not encountered. Conclusions: The mid-term results of intraextracardiac fenestrated Fontan procedure are encouraging. This procedure may improve the results in a patient population who should be palliated as univentricular physiology, especially in cases with complex cardiac anatomy.

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

scholarly journals Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation

2018 ◽  
Vol 38 (6) ◽  
pp. e5-e12 ◽  
Author(s):  
Christine Peyton
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Complex Disease ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Venous Hypertension ◽  
Plastic Bronchitis ◽  
Protein Losing Enteropathy ◽  
Fontan Physiology

Protein-losing enteropathy and plastic bronchitis remain challenging to treat despite recent treatment advances. Protein-losing enteropathy and plastic bronchitis have been diagnosed in patients with cardiomyopathy, constrictive pericarditis, and congestive heart failure. This article focuses on patients with protein-losing enteropathy or plastic bronchitis following the Fontan procedure. Patients with single-ventricle physiology who have undergone the Fontan procedure are at risk for these conditions. Fontan physiology predisposes patients to chronically low cardiac output, increased central venous pressure, and congestive heart failure. These altered hemodynamics lead to increased mesenteric vascular resistance, resulting in venous hypertension and congestion in protein-losing enteropathy. Plastic bronchitis is a complex disease in which chronic high lymphatic pressures from Fontan physiology cause acellular bronchial casts to develop. These entities may also occur in patients with normal Fontan hemodynamics. This article also covers medical and surgical interventions for protein-losing enteropathy and plastic bronchitis. (Critical Care Nurse. 2018;38[6]:e5–e12)

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