Investigating Fontan Failure Using Mathematical Modeling

Babies born with a single functioning heart ventricle instead of two require a series of surgeries during the first few years of life to redirect their blood flow, resulting in a Fontan circulation. Patients with Fontan circulations have excellent early survival; however, over time, their circulations begin to fail, ultimately resulting in their death. Currently, the only treatment for failing Fontan circulation is a heart transplant; however, many Fontan patients do not survive long enough to receive a donor heart. One of the reasons for this is a lack of understanding of the Fontan failure cascade. Often patients are identified as failing when they do not have enough time left to receive a heart transplant. The objective of this problem is to develop mathematical models of healthy and failing Fontan circulations to i) improve our understanding of Fontan failure from a hemodynamic perspective, and ii) identify physiologically-relevant ranges of parameters.

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Fate of the liver in the survivors of adult heart transplant for a failing Fontan circulation

The Journal of Heart and Lung Transplantation ◽

10.1016/j.healun.2021.10.020 ◽

2021 ◽

Author(s):

Christopher R Broda ◽

Rafael Alonso-Gonzalez ◽

Anand Ghanekar ◽

Aliya Gulamhusein ◽

Michael McDonald ◽

...

Keyword(s):

Heart Transplant ◽

Fontan Circulation ◽

Adult Heart ◽

Failing Fontan

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Mechanical Support of the Failing Fontan Circulation

Seminars in Thoracic and Cardiovascular Surgery ◽

10.1053/j.semtcvs.2020.09.005 ◽

2020 ◽

Author(s):

Christopher E. Mascio

Keyword(s):

Fontan Circulation ◽

Mechanical Support ◽

Failing Fontan

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Direct Measurement of Porto-systemic Gradient in a Failing Fontan Circulation

Congenital Heart Disease ◽

10.1111/j.1747-0803.2010.00451.x ◽

2011 ◽

Vol 6 (2) ◽

pp. 175-178 ◽

Cited By ~ 13

Author(s):

Madhavi Velpula ◽

Nick Sheron ◽

Neill Guha ◽

Tony Salmon ◽

Nigel Hacking ◽

...

Keyword(s):

Direct Measurement ◽

Fontan Circulation ◽

Failing Fontan

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Perioperative Prognostic Factors for Fontan Patients Undergoing Orthotopic Heart Transplant

The Journal of Heart and Lung Transplantation ◽

10.1016/j.healun.2015.01.803 ◽

2015 ◽

Vol 34 (4) ◽

pp. S287

Author(s):

C.J. Berg ◽

B. Bauer ◽

A. Hageman ◽

L.C. Reardon

Keyword(s):

Prognostic Factors ◽

Heart Transplant ◽

Orthotopic Heart Transplant ◽

Fontan Patients

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Rebuttal

Cardiology in the Young ◽

10.1017/s1047951104006432 ◽

2004 ◽

Vol 14 (S1) ◽

pp. 114-114 ◽

Cited By ~ 1

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.

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Abstract 14203: Secondary Sarcopenia Assessed by Computed Tomography Can Predict Hospitalization for Heart Failure in Adults With Fontan Circulation

Circulation ◽

10.1161/circ.142.suppl_3.14203 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Yumi Shiina ◽

Michinobu Nagao ◽

Kei Inai

Keyword(s):

Heart Failure ◽

Computed Tomography ◽

Skeletal Muscle ◽

Negative Impact ◽

Heart Association ◽

Functional Class ◽

Fontan Circulation ◽

Protein Losing Enteropathy ◽

Psoas Major Muscle ◽

Fontan Patients

Introduction: Sarcopenia is one of the important predictors of heart failure (HF) in patients with cardiac problems. Hypothesis: Skeletal muscle pump is very important to maintain Fontan circulation. We aimed to quantify the volume of the psoas major muscle (PMM) and investigate whether it is relevant to HF hospitalization in adults with Fontan circulation. Methods: A total of 154 adults with Fontan circulation, and 30 age-matched adults were studied retrospectively. We identified secondary sarcopenia by measuring the PMM volume, using non-contrast abdominal computed tomography (CT). Results: PMM indexed volume in Fontan patients was lower than controls (206.4+/-18.0 cc/m2 vs 288.0+/-61.1 cc/m2, P<0.0001). In Fontan men, PMM indexed volume correlated with New York Heart Association functional class (NYHA FC). PMM indexed volume in Fontan women correlated with age, NYHA FC, and serum creatinine. Using a multivariate Cox hazards analysis, NYHA FC ≧3 was an important prediction of hospitalization due to HF in Fontan men. In Fontan women, NYHA FC ≧3, brain natriuretic peptide (BNP), PMM indexed volume, and protein losing enteropathy (PLE) were important predictions of hospitalization due to HF. Among all Fontan patients, those with low PMM volumes had a poorer prognosis in HF (Log rank P=0.012 [men] and 0.0009 [women]). Conclusions: PMM volume reduction has an adjunctive prediction of HF hospitalization in adults with Fontan circulation, particularly in Fontan females. Secondary sarcopenia may have a negative impact on the prognosis of HF in this population. The assessment of skeletal muscle mass also may be a comprehensive screening tool for multi-organ dysfunction in Fontan circulation.

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

Circulation ◽

10.1161/circ.142.suppl_3.14773 ◽

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.

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Hemodynamic Effects of Paracentesis in a Patient With a Fontan Circulation

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135118779351 ◽

2018 ◽

Vol 10 (3) ◽

pp. 357-359 ◽

Cited By ~ 1

Author(s):

Saurabh Rajpal ◽

Alexander R. Opotowsky ◽

Anna Rutherford ◽

Fred Wu

Keyword(s):

Cardiac Output ◽

Liver Disease ◽

Large Volume ◽

Venous Pressure ◽

Fontan Circulation ◽

Hemodynamic Effects ◽

Large Volume Paracentesis ◽

Substantial Decline ◽

Failing Fontan ◽

Common Manifestation

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.

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