Relationship of Ventricular Morphology and Atrioventricular Valve Function to Long-Term Outcomes Following Fontan Procedures

Moon, Jiyong; Shen, Li; Likosky, Donald S.; Sood, Vikram; Hobbs, Reilly D.; Sassalos, Peter; Romano, Jennifer C.; Bove, Edward L.; Si, Ming‐Sing

doi:10.1016/j.jacc.2020.05.059

Cited by 51 publications

(38 citation statements)

References 35 publications

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“…A study of 683 adult Fontan patients published in 2018 from the Australian and New Zealand Fontan Registry found that, by age 40, mortality reached 20%, only 53% of patients were free of heart failure symptoms, and only 41% were free of serious adverse events [1]. Similar data has been widely reported [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. Approximately half of the instances of mortality and significant morbidity can be attributed to the failure of the unique Fontan circulatory system.…”

Section: Introductionmentioning

confidence: 78%

“…This approach revealed the model sensitivity to these parameters as well as a trend in IVC pressure reduction. When comparing ∆ 𝐼𝐽𝑆−𝑇𝐶𝑃𝐶 for constant 𝐷 𝑖𝑗𝑠 (cases [3,6,9], [4,7,10] or [5,8,11]), it can be seen that as ∆ 𝐼𝐽𝑆−𝑇𝐶𝑃𝐶 decreases (7cm to 3cm) IVC pressure increases. Conversely, comparison for varying 𝐷 𝑖𝑗𝑠 for constant ∆ 𝐼𝐽𝑆−𝑇𝐶𝑃𝐶 (cases [3,4,5], [6,7,8], or [9,10,11]) shows that for decreasing 𝐷 𝑖𝑗𝑠 (5mm to 3mm) IVC pressure decreases.…”

Section: Design Space Explorationmentioning

confidence: 99%

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Parametric Investigation of an Injection-Jet Self-Powered Fontan Circulation

Prather

Das²,

Farias

et al. 2021

Preprint

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Rationale: Approximately 1/2500 babies are born with only one functioning ventricle and the Fontan is the third and, ideally final staged palliative operation for these patients. This altered circulation is prone to failure with survival rates below 50% into adulthood in some series. Chronically elevated inferior vena cava (IVC) pressure is implicated as one cause of the mortality and morbidity in this population.Objective: An injection jet shunt (IJS) drawing blood-flow directly from the aortic arch to significantly lower IVC pressure is proposed.Methods and Results: A computer-generated 3D model of a 2–4 year old patient with a fenestrated Fontan and a cardiac output of 2.3L/min was generated. The detailed 3D pulsatile hemodynamics are resolved in a zero-dimensional lumped parameter network tightly-coupled to a 3D computational fluid dynamics model accounting for non-Newtonian blood rheology and resolving turbulence using large eddy simulation. IVC pressure and systemic oxygen saturation were tracked for various IJS-assisted Fontan configurations, altering design parameters such as shunt and fenestration diameters and locations.A baseline “failing” Fontan with 4mm fenestration was tuned to elevated IVC pressure (\(+17.8mmHg\)). Enlargement of the fenestration to 8mm resulted in a 3mmHg IVC pressure drop but an unacceptable reduction in systemic oxygen saturation below 80%. Addition of an IJS with a 2mm nozzle and minor volume load to the ventricle improved the IVC pressure drop to 3.2mmHg while increasing systemic oxygen saturation above 80%.Conclusions: The salutary effects of the IJS to effectively lower IVC pressure while retaining acceptable levels of oxygen saturation are successfully demonstrated.

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Section: Introductionmentioning

confidence: 78%

Section: Design Space Explorationmentioning

confidence: 99%

Parametric Investigation of an Injection-Jet Self-Powered Fontan Circulation

Prather

Das²,

Farias

et al. 2021

Preprint

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“…1,2 Such effectors may include, but are not limited to, Fontan connection compliance, Fontan fenestrations and venovenous collaterals, disturbances at Fontan connection junctions, compliance of the large pulmonary arteries and vascular obstructions, pulmonary arterial microvascular cross-sectional area, pulmonary venous vascular compliance and obstructions, and functional univentricular diastolic performance. [13][14][15][16][26][27][28][29][30][31][32][33][34][35][36] In particular, a neonatal history of obstructed pulmonary blood, chiefly when accompanied by a functional univentricle of right-right ventricular type, appears to especially negatively affect the outcome of FALD. 14 In conclusion, in a relatively young, stable extracardiac Fontan patient cohort, average composite anatomical risk scores strongly correlated with average hepatic total fibrosis scores by anatomical group.…”

Section: Discussionmentioning

confidence: 99%

“…12 Patients were assigned to anatomical groups (with a minimum n ≥ 3) by functional univentricle type, presence or absence of venovenous collaterals and pulmonary artery stents, and neonatal history of obstructed or unobstructed pulmonary blood flow. Based on previous investigations, [13][14][15][16][26][27][28][29][30][31][32][33][34][35][36] we developed a composite anatomical risk score, which resulted in the best correlation with total fibrosis scores. We assigned 0 points for functional univentricles of either right and left ventricular type without venovenous collaterals or pulmonary artery stents.…”

Section: Methodsmentioning

confidence: 99%

Fontan‐associated liver disease and total cavopulmonary anatomical flow effectors

Evans

Acherman

Galindo

et al. 2021

Journal of Cardiac Surgery

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Objective: We investigated a relationship between a composite index comprised of Fontan-circuit anatomical features and hepatic fibrosis scores from biopsy.Methods: We identified living extracardiac Fontan patients, ≥7 years old and ≥5 but <20 years postoperative, that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2020. We divided patients into anatomical groups and applied a risk score to each patient. We compared average anatomical risk scores with average hepatic total fibrosis scores by group. Results:We identified 111 patients that met inclusion criteria. After excluding four patients, we assigned 107 to one of 12 anatomical variant groups (n ≥ 3). For the 107, the average age at liver biopsy was 14 ± 6 years old. Of the 107, 105 (98%)were New York Heart Association Class 1. We found average anatomical risk scores by group correlated with average total fibrosis scores by group (R = 0.8; p = .005). An average Fontan duration to biopsy of 10 ± 1 years was similar for all 12 anatomical groups. We found no other clinical variables, laboratory, or hemodynamic values that trended with anatomical risk scores or hepatic total fibrosis scores.Conclusions: In a cohort of relatively young, stable extracardiac Fontan patients, average composite anatomical risk scores strongly correlated with average hepatic total fibrosis scores by anatomical group. These findings suggest that some anatomical variants in extracardiac Fontan patients are associated with higher Fontanassociated liver disease (FALD)-related hepatic total fibrosis scores than others, despite similar Fontan durations.

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“…3 Single-ventricle palliation is performed for a broad range of congenital anomalies, and the rate of AVV failure is, predictably, related to the morphology of the AVV and, thus, to the extent to which the fibrous skeleton of the heart is developed. 3,10 Consequently, patients with tricuspid atresia have the lowest rate of AVV failure, with 8% having valve failure at 25 years, because they have a mitral valve as their AVV. The rate of AVV failure is higher in those with mitral atresia reaching 46% at 25 years.…”

Section: Incidence Of Atrioventricular Valve Dysfunction In Patients With Single-ventricle Physiologymentioning

confidence: 99%

Atrioventricular valve surgery: Restoration of the fibrous skeleton of the heart

Buratto

Konstantinov

2021

The Journal of Thoracic and Cardiovascular Surgery

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Millions of years of relentless evolution have shaped the human heart into a durable and incredibly efficient pump. This high durability and efficiency are impossible without the normal fibrous skeleton of the heart. To achieve optimal results in our surgical work, we must emulate the work of nature as closely as possible. A perfect example of this would be surgery for the atrioventricular valve (AVV). Although the results of AVV repair have improved over time, AVV regurgitation remains a formidable challenge, particularly, in the univentricular circulation. [1][2][3][4][5][6][7] Although more than 20% of patients with univentricular circulation develop moderate or greater AVV regurgitation of mitral, tricuspid, or common AVV by 20 years, the rate of failure of common AVV in the univentricular circulation is greater than 50% by 20 years. 3 Furthermore, it is unequivocal that AVV regurgitation is associated with poorer survival in patients with univentricular circulation. 5 Achieving a durable repair of AVV, including that in common AVV, is often difficult in univentricular circulation, especially in patients with right ventricular dominance. 3 In this focused review, we summarize the recent literature on the outcomes of AVV surgery in patients with univentricular circulation with emphasis on the importance of restoring the fibrous skeleton of the heart.

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Relationship of Ventricular Morphology and Atrioventricular Valve Function to Long-Term Outcomes Following Fontan Procedures

Cited by 51 publications

References 35 publications

Parametric Investigation of an Injection-Jet Self-Powered Fontan Circulation

Parametric Investigation of an Injection-Jet Self-Powered Fontan Circulation

Fontan‐associated liver disease and total cavopulmonary anatomical flow effectors

Atrioventricular valve surgery: Restoration of the fibrous skeleton of the heart

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