THE FATE OF THE FAILING FONTAN CIRCULATION – NO TWO ARE ALIKE

Author(s):  
Kriti Puri ◽  
Martin Schweiger ◽  
Joseph W Rossano
2011 ◽  
Vol 6 (2) ◽  
pp. 175-178 ◽  
Author(s):  
Madhavi Velpula ◽  
Nick Sheron ◽  
Neill Guha ◽  
Tony Salmon ◽  
Nigel Hacking ◽  
...  

2004 ◽  
Vol 14 (S1) ◽  
pp. 114-114 ◽  
Author(s):  
Leonard L. Bailey

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.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Ilias P Doulamis ◽  
Supreet P Marathe ◽  
Breanna L Piekarski ◽  
Rebecca S Beroukhim ◽  
Gerald R Marx ◽  
...  

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.


2018 ◽  
Vol 10 (3) ◽  
pp. 357-359 ◽  
Author(s):  
Saurabh Rajpal ◽  
Alexander R. Opotowsky ◽  
Anna Rutherford ◽  
Fred Wu

Recurrent ascites is a common manifestation of failing Fontan circulation. We present the hemodynamic response to large-volume paracentesis in a patient with Fontan circulation and Fontan-associated liver disease, documenting an immediate and substantial decline in systemic venous pressure and increase in cardiac output. These preliminary observations may have implications for the management of ascites in adults with Fontan circulation.


2008 ◽  
Vol 3 (1) ◽  
pp. 60-62 ◽  
Author(s):  
Adrian M. Moran ◽  
Elizabeth D. Blume ◽  
Reed Quinn

Heart ◽  
2012 ◽  
Vol 98 (14) ◽  
pp. 1098-1104 ◽  
Author(s):  
Barbara J Deal ◽  
Marshall L Jacobs

2022 ◽  
Vol 18 ◽  
Author(s):  
Bart. W. Driesen ◽  
Michiel Voskuil ◽  
Heynric B. Grotenhuis

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.


2019 ◽  
Vol 40 (5) ◽  
pp. 1093-1096 ◽  
Author(s):  
Daniel McLennan ◽  
Michal Schäfer ◽  
Max B. Mitchell ◽  
Gareth J. Morgan ◽  
Dunbar Ivy ◽  
...  

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