Exercise Capacity and Predictors of Performance After Fontan: Results from the Pediatric Heart Network Fontan 3 Study

Goldberg, David J.; Zak, Victor; McCrindle, Brian W.; Ni, Hua; Gongwer, Russell; Rhodes, Jonathan; Garofano, Robert P.; Kaltman, Jonathan R.; Lambert, Linda M.; Mahony, Lynn; Margossian, Renée; Spector, Zebulon Z.; Williams, Richard V.; Atz, Andrew M.; Paridon, Stephen M.

doi:10.1007/s00246-020-02465-1

Cited by 37 publications

(36 citation statements)

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“…None of the people with the "Super-Fontan" phenotype were obese based on BMI compared to 22% in the comparator group with impaired exercise performance. The latest Pediatric Heart Network results also support this finding (34). This may be related to the impact of excess adiposity on respiratory muscle pump function and increased mechanical loading, which may impair exercise performance.…”

Section: Factors Associated With Superior Physical Performancementioning

confidence: 69%

“…However, although not statistically significant, there was a higher percentage of females in the “Super-Fontan” group compared to those with impaired exercise performance. Previous studies have also shown that a higher proportion of females are able to achieve better exercise performance ( 6 , 7 , 34 ), and have superior long term-outcomes ( 47 , 48 ). The mechanisms underlying these sex differences are unclear and warrant further investigation.…”

Section: Discussionmentioning

confidence: 97%

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The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance

Tran

Celermajer

Ayer

et al. 2021

Front. Cardiovasc. Med.

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Background: People with a Fontan circulation usually have moderately impaired exercise performance, although a subset have high physical performance (“Super-Fontan”), which may represent a low-risk phenotype.Methods: People with a “Super-Fontan” phenotype were defined as achieving normal exercise performance [≥80% predicted peak oxygen uptake (VO2) and work rate] during cardiopulmonary exercise testing (CPET) and were identified from the Australian and New Zealand Fontan Registry. A Fontan control group that included people with impaired exercise performance (<80% predicted VO2 or work rate) was also identified based on a 1:3 allocation ratio. A subset of participants were prospectively recruited and completed a series of physical activity, exercise self-efficacy, and health-related quality of life questionnaires.Results: Sixty CPETs (“Super-Fontan”, n = 15; control, n = 45) were included. A subset (“Super-Fontan”, n = 10; control, n = 13) completed a series of questionnaires. Average age was 29 ± 8 years; 48% were males. Exercise capacity reflected by percent predicted VO2 was 67 ± 17% in the entire cohort. Compared to the “Super-Fontan” phenotype, age at Fontan completion was higher in controls (4.0 ± 2.9 vs. 7.2 ± 5.3 years, p = 0.002). Only one (7%) person in the “Super-Fontan” group had a dominant right ventricle compared to 15 (33%) controls (p = 0.043). None of those in the “Super-Fontan” group were obese, while almost a quarter (22%) of controls were obese based on body mass index (p = 0.046). Lung function abnormalities were less prevalent in the “Super-Fontan” group (20 vs. 70%, p = 0.006). Exercise self-efficacy was greater in the “Super-Fontan” group (34.2 ± 3.6 vs. 27.9 ± 7.2, p = 0.02). Self-reported sports participation and physical activity levels during childhood and early adulthood were higher in the “Super-Fontan” group (p < 0.05). The total average time spent participating in structured sports and physical activity was 4.3 ± 2.6 h/wk in the “Super-Fontan” group compared to 2.0 ± 3.0 h/wk in controls, p = 0.003. There were no differences in self-reported current total physical activity score or health-related quality of life between groups (p ≥ 0.05).Conclusions: The “Super-Fontan” phenotype is associated with a healthy weight, lower age at Fontan completion, better exercise self-efficacy, and higher overall levels of sport and physical activity participation during physical development.

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Section: Factors Associated With Superior Physical Performancementioning

confidence: 69%

Section: Discussionmentioning

confidence: 97%

The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance

Tran

Celermajer

Ayer

et al. 2021

Front. Cardiovasc. Med.

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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%

“…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. Given the values in Table 2, it can be determined that IVC pressure variation due to 𝐷 𝑖𝑗𝑠 reduction ranges between 3.1-3.8% whereas for a decrease in ∆ 𝐼𝐽𝑆−𝑇𝐶𝑃𝐶 , IVC pressure changes 0.24-0.97%.…”

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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Exercise capacity after total cavopulmonary anastomosis: a longitudinal paediatric and adult study

et al. 2021

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Aims Fontan palliation is a surgical strategy for patients with complex congenital heart disease, in whom biventricular circulation cannot be achieved. Long‐term survival is negatively affected by the absence of sub‐pulmonary ventricle and increased systemic venous pressure. Exercise capacity is a known predictor of overall survival and quality of life in congenital heart defects. We aim to track individual trends of peak oxygen uptake (V̇O2peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease‐specific reference V̇O2peak dataset. Methods and results A retrospective study of serial cardiopulmonary exercise testing (CPET) data, gathered from all patients who underwent TCPC in the Czech Republic between 1992 and 2016. Of 354 consecutive patients with TCPC, 288 (81.4%) patients underwent one or more CPETs yielding 786 unique V̇O2peak values used as a reference dataset. Longitudinal data were available in 206 (58.2%) patients, who underwent a median (inter‐quartile range) of 3.0 (2.0–5.0) CPETs over a mean (standard deviation) of 8.9 (5.5) years. The decline of exercise capacity with age was linear and not faster than in healthy peers (P = 0.47), but relative values of V̇O2peak in TCPC patients were 12.6 mL/min/kg lower. Single ventricular morphology and pulmonary artery size had no significant influence on the exercise capacity dynamics. V̇O2peak decline correlated negatively with the trend of body mass index z‐score (P = 0.006) and was faster in women than men (P = 0.008). Conclusions Total cavopulmonary connection patients have significantly reduced exercise capacity. The age‐related decline paralleled the healthy population and correlated negatively with the body mass index trend. The presented V̇O2peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients.

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Exercise Capacity and Predictors of Performance After Fontan: Results from the Pediatric Heart Network Fontan 3 Study

Abstract: Impaired exercise following Fontan is a surrogate of morbidity. Single-center longitudinal data exist, but there is a lack of contemporary multi-center data. Ramp cycle ergometry was re-

Cited by 37 publications

References 38 publications

The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance

The “Super-Fontan” Phenotype: Characterizing Factors Associated With High Physical Performance

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

Exercise capacity after total cavopulmonary anastomosis: a longitudinal paediatric and adult study

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