Abstract 14773: Biventricular Conversion After Fontan Completion: A Preliminary Experience

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Ilias P Doulamis ◽  
Supreet P Marathe ◽  
Breanna L Piekarski ◽  
Rebecca S Beroukhim ◽  
Gerald R Marx ◽  
...  
Keyword(s):  
Fontan Circulation ◽  
Exercise Intolerance ◽  
Optimal Timing ◽  
Atrioventricular Canal ◽  
Protein Losing Enteropathy ◽  
Systolic Volume ◽  
Hepatic Congestion ◽  
Atrioventricular Canal Defect ◽  
Fontan Physiology ◽  
Failing Fontan

Hypothesis: Biventricular conversion (BiVC) following takedown of Fontan circulation is feasible and results in improved hemodynamics. Methods: Retrospective analysis of patients who had takedown of Fontan circulation and conversion to BiV circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure >15 mmHg. Results: There were 23 patients (median age: 10.0 (7.5-13.0) years); 15 (65%) had failing Fontan physiology and 8 (35%) underwent elective takedown of their Fontan circulation. Of the 15 patients with failing Fontan physiology, 4 had exercise intolerance or cyanosis, 3 had hepatic congestion or cirrhosis, 3 had end-organ damage and 1 patient had protein losing enteropathy; the rest 4 patients had no other sign of SVP complications. A subset of patients (n=6) underwent recruitment of the non-dominant ventricle prior to conversion. HLHS (p<0.01) and sub-/aortic stenosis (p<0.01) were more common in these patients. BiVC with or without staged ventricular recruitment led to a significant increase in indexed end-diastolic volume (p<0.01), indexed end-systolic volume (p<0.01) and ventricular mass (p<0.01) of the non-dominant ventricle (14 RV, 9 LV). There were 1 (4%) early and 4 (17%) late deaths. All who underwent elective BiVC survived, while 2-year survival rate for patients with a failing Fontan circulation was 72.7% (95% CI: 37-90%) (Figure 1). The overall, 1-year reintervention free survival was 44.1% (95% CI: 21-65%). Left dominant atrioventricular canal defect (p<0.01) and early year of BiVC (p=0.02) were significant predictors for mortality. Conclusions: BiVC is feasible in patients with failing Fontans, and has promising outcomes after elective takedown of Fontan circulation. A staged approach for ventricular recruitment does not seem inferior to primary BiVC. The optimal timing for BiVC in Fontan patients needs further evaluation.

Rebuttal

2004 ◽  
Vol 14 (S1) ◽  
pp. 114-114 ◽  
Author(s):  
Leonard L. Bailey
Keyword(s):  
Coronary Disease ◽  
Reconstructive Surgery ◽  
Fontan Circulation ◽  
Actuarial Survival ◽  
Term Survival ◽  
Long Term Survival ◽  
Fontan Physiology ◽  
Failing Fontan

The portrayal of a beautiful youngster performing uninhibited acrobatics based on Fontan physiology, as presented by Marshall Jacobs, is a brilliant and beautiful thing for us to see. It is, perhaps, all about will over physiology. But it is, nevertheless, happening for that child. Marshall mentioned the need for re-transplantation, whether the beginning strategy was transplantation or reconstructive surgery. Indeed, a relatively small percentage of children transplanted will require re-transplantation because of severe graft coronary disease. Remarkably, in the Loma Linda experience, 10-year actuarial survival for 26 patients following elective re-transplantation is over 85%, exceeding overall actuarial survival at 10 years for children following primary transplantation. Many of the transplanted infants, however, seem to be realistically hopeful that one heart will last their entire lifetime. Of course, the hope is that their's will be a long and healthy lifetime. The requirement for late transplantation following Fontan procedures, however, seems almost inevitable. We'll simply have to keep these children with Fontan circulation under surveillance to see when, in the course of their lives, transplantation will become necessary. Unfortunately, operative and long-term survival among children who are transplanted for failing Fontan physiology have, as yet, been somewhat suboptimal.

Current treatment options for the Failing Fontan Circulation

2022 ◽  
Vol 18 ◽  
Author(s):  
Bart. W. Driesen ◽  
Michiel Voskuil ◽  
Heynric B. Grotenhuis
Keyword(s):  
Myocardial Dysfunction ◽  
Fontan Procedure ◽  
Treatment Options ◽  
Fontan Operation ◽  
Current Treatment ◽  
Fontan Circulation ◽  
Protein Losing Enteropathy ◽  
Resistance Protein ◽  
Failing Fontan

Abstract: The Fontan operation was introduced in 1968. For congenital malformations where biventricular repair is not suitable, the Fontan procedure has provided a long-term palliation strategy with improved outcome compared to the initially developed procedures. Despite these improvements, several complications merely as a result of a failing Fontan circulation (including myocardial dysfunction, arrhythmias, increased pulmonary vascular resistance, protein losing enteropathy, hepatic dysfunction, plastic bronchitis and thrombo-embolism) will limit life-expectancy in this patient cohort. This review provides an overview of the most common complications of the Fontan circulation and the currently available treatment options.

scholarly journals Nutritional status correction options in children with congenital heart disease

2015 ◽  
Vol 96 (4) ◽  
pp. 654-659
Author(s):  
L A Gandaeva ◽  
E N Basargina ◽  
T E Borovik ◽  
N G Zvonkova
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Nutritional Status ◽  
Total Protein ◽  
Congenital Heart ◽  
Interventricular Septum ◽  
Atrioventricular Canal ◽  
Protein Losing Enteropathy ◽  
Atrioventricular Canal Defect ◽  
Status Assessment

Aim. To justify the need of nutritional status assessment and correction in children with congenital heart disease. Methods. Clinical examination, somatometric methods (height, weight, head and chest circumference measuring) were used. Anthropometric data were evaluated using the WHO Anthro, WHO AnthroPlus (2009) programm. The following parameters were analyzed: weight/age, weight/height, height/age, body mass index/age. Results. The review summarizes the existing data on the etiology and pathogenesis of protein-losing enteropathy and methods of its correction. Own observation is described. A girl, born in 1999 from the fifth pregnancy, occurring on the background of toxicosis and threat of abortion, premature second delivery. Birth weight was 1280 g, length 34 cm. Congenital heart disease (double discharge of the right ventricle main vessels, common open atrioventricular canal, defect of interventricular septum, common atrium, the «riding» mitral valve, left ventricle hypoplasia) is diagnosed in maternity hospital. One year after the Fontan surgery was conducted (in August, 2011) hypoproteinemia (total protein content of 44 g/l) was noticed for the first time in the biochemical blood analysis, and swelling appeared on her feet. Intravenous administration of 20% albumin solution was performed numerously, however without positive changes, plasma protein level was low (37 g/l). According to the guidelines, the child was assigned to a specialized diet with «Nutrien standard» 200 ml/dayas an additional source of protein. The child was discharged from the hospital with improvement and recommendations to continue treatment as an outpatient. At follow-up examination in 6 months at our center (November, 2012) the condition was stable, we managed to stop manifestations of exudative enteropathy and to normalize the total protein levels (November, 2012 - 73 g/l), albumin (46 g/l), and blood electrolytes; the girl gained body weight by 2 kg and height increased by 9 cm. Conclusion. Complex examination and treatment of children with congenital heart disease should include nutritional status assessment and, if necessary, its correction.

scholarly journals The Effect of Surgical Technique, Age and Trisomy 21 on Early Outcome of Surgical Management of Complete Atrioventricular Canal Defect

2021 ◽  
Author(s):  
Sherief Azzab ◽  
Ahmed Samy ◽  
Hamdy Singab ◽  
Mohamed Zeinah ◽  
Gledisa Musollari ◽  
...  
Keyword(s):  
Early Intervention ◽  
Surgical Technique ◽  
Trisomy 21 ◽  
Surgical Repair ◽  
Early Period ◽  
Optimal Timing ◽  
P Value ◽  
Atrioventricular Canal ◽  
Atrioventricular Canal Defect ◽  
Significant Difference

Abstract Background: The optimal timing, surgical technique and the influence of Trisomy 21 on outcome of surgical repair of Complete Atrio-Ventricular Canal Defect (CAVC) remain uncertain. We reviewed our experience in repair of CAVC to identify the influence of these factors on operative outcome. Methods: A prospective study included 70 patients who underwent repair of CAVC at our institute between July 2016 to October 2019. Primary endpoint was mortality and secondary endpoint was degree of left atrio‐ventricular valve (LAVV) regurgitation.Results: No significant difference noted between patients operated on, at the first 6 months of age versus later, regarding mortality or LAVV regurgitation. Surgical repair by modified single patch technique showed significant reduction in bypass time (71.13 ± 13.507 min vs 99.19 ± 27.092 min, P value =0.001). Comparison to closure of cleft only, posterior annuloplasty used for repair of LAVV resulted in significant reduction in the occurrence of post-operative valve regurgitation during the early period (LAVV 2+ 43 % vs 7 %, P value=0.03) and at 6 months of follow up (LAVV 2+ 35.4 % vs 0 %, P value=0.01) respectively.Conclusion: Early intervention, in the first 6 months in patients with CAVC by surgical repair gives comparable acceptable results to later repair; Trisomy 21 was not found to be a risk factor for early intervention. Repair of common AV valve by cleft closure with posterior LAVV annuloplasty showed better results with significant decrease in postoperative LAVV regurgitation and early mortality in comparison to closure of cleft only.

scholarly journals Thoracic duct decompression and jugular vein banding—an effective treatment option for protein-losing enteropathy and plastic bronchitis in severe failing Fontan circulation: a case report

2020 ◽  
Vol 4 (5) ◽  
pp. 1-4
Author(s):  
Christoph Bauer ◽  
Roland Mair ◽  
Rudolf Mair ◽  
Gerald Tulzer
Keyword(s):  
Effective Treatment ◽  
Internal Jugular Vein ◽  
Treatment Option ◽  
Thoracic Duct ◽  
Jugular Vein ◽  
Fontan Circulation ◽  
Plastic Bronchitis ◽  
Protein Losing Enteropathy ◽  
System Integrity ◽  
Failing Fontan

Abstract Background Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are devastating complications after Fontan palliation that lead to uncontrolled loss of protein-rich lymphatic fluid into extra lymphatic compartments. Decompression of the thoracic duct is a new treatment option that effectively restores lymphatic system integrity by redirecting lymphatic flow into the low-pressure levels of the common atrium. Case summary We report a patient with severe failing Fontan circulation where surgical thoracic duct decompression leads to resolution of PLE and PB symptoms but worsening hypoxaemia that could be managed with banding of internal jugular vein. Discussion Thoracic duct decompression in patients with failing Fontan circulation can be a simple and effective treatment for PLE and PB. Hypoxaemia may occur but can be managed with banding of internal jugular vein.

scholarly journals Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation

2016 ◽  
Vol 101 (6) ◽  
pp. 2370-2373 ◽  
Author(s):  
Marta António ◽  
Andreia Gordo ◽  
Conceição Pereira ◽  
Fátima Pinto ◽  
Isabel Fragata ◽  
...  
Keyword(s):  
Thoracic Duct ◽  
Fontan Circulation ◽  
Protein Losing Enteropathy ◽  
Failing Fontan

Complete Atrioventricular Canal Defect: Influence of Timing of Repair on Intermediate Outcomes

2017 ◽  
Vol 8 (3) ◽  
pp. 361-366
Author(s):  
Khushboo N. Parikh ◽  
Nishant C. Shah ◽  
John L. Myers ◽  
Allen R. Kunselman ◽  
Joseph B. Clark
Keyword(s):  
Regression Models ◽  
Pacemaker Implantation ◽  
Patient Characteristics ◽  
Optimal Timing ◽  
Atrioventricular Canal ◽  
Atrioventricular Valve Regurgitation ◽  
Intermediate Outcomes ◽  
Age At Surgery ◽  
Atrioventricular Canal Defect ◽  
Complete Atrioventricular

Background: The optimal timing of repair of complete atrioventricular canal defect (CAVC) remains uncertain. When early repair is indicated due to clinical conditions, patients may be potentially exposed to excess morbidity or mortality. We reviewed our experience with repair of CAVC to determine the influence of age on outcomes. Methods: The study included 48 patients who underwent repair of CAVC at our institution from 2004 to 2014. To assess the association of age at surgery with outcomes, logistic regression models were fit to binary outcomes and linear regression models were fit for continuous outcomes. Age at surgery was dichotomized into early (≤90 days; n = 18) and late repair (>90 days; n = 30). Chi-square and two-sample t tests were used to compare early to late repair with respect to patient characteristics and outcomes. Results: Patient characteristics were similar except for mean weight at surgery (3.9 vs 4.6 kg) and presence of greater than equal to moderate left atrioventricular valve regurgitation (LAVVR; 1 vs 11). When assessed by continuous scale or dichotomized at 90 days, there was no association of age at repair with outcomes including median bypass and clamp times, need for pacemaker implantation, pulmonary hypertension requiring oxygen and/or medication, median length of stay, incidence of greater than equal to moderate LAVVR at discharge, and rate of reoperation for LAVVR. Freedom from greater than equal to moderate LAVVR was similar between the groups at latest follow-up. Conclusion: Contemporary repair of CAVC is associated with low mortality and favorable outcomes. In the presence of clear signs of congestive heart failure, primary repair can be safely accomplished in patients under three months of age.

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