Providing a framework of principles for conceptualising the Fontan circulation

Gewillig, Marc; Brown, Stephen C.; Bruaene, Alexander Van De; Rychik, Jack

doi:10.1111/apa.15098

Cited by 47 publications

(62 citation statements)

References 22 publications

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“…Conversely, one study found a positive correlation between age at Fontan completion, and percent predicted peak VO 2 ( 30 ). Although later age at Fontan completion is accompanied by an extended period of volume overload and cyanosis, it may allow for pulmonary artery catch-up growth and a larger conduit to optimize flow ( 31 , 32 ); however, this theory requires verification.…”

Section: Discussionmentioning

confidence: 99%

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: Discussionmentioning

confidence: 99%

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

Tran

Celermajer

Ayer

et al. 2021

Front. Cardiovasc. Med.

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“…This creates an unphysiological circulation with many potential problems. [19][20][21][22] The single pumping ventricle has to provide enough blood flow energy to perfuse the systemic as well as pulmonary vascular beds. This entails increased afterload for the ventricle and significantly increased systemic venous pressure and venous congestion in most organs.…”

Section: The Fontan Circul Ati Onmentioning

confidence: 99%

“…After the Fontan operation, the ventricle is volume deprived with limited ability to increase stroke volume. 21,39 The abnormal shape and function of the ventricle can lead to myocardial inflammation and fibrosis, further reducing output. Heart failure with low output and venous congestion becomes symptomatic, but the common underlying mechanism may be a slowly progressing pulmonary vascular resistance.…”

Section: F I G U R E 1 Illustrations Of Surgical Palliation Of Univentricular Heart Defects Bidirectional Glenn (A) With Anastomosis Of Tmentioning

confidence: 99%

Fontan circulation has improved life expectancy for infants born with complex heart disease over the last 50 years but has also resulted in significant morbidity

Hedlund

Lundell

2021

Acta Paediatrica

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The first patient, who survived a cavopulmonary and atriopulmonary connection as a treatment for tricuspid atresia, was described 50 years ago by Fontan et al. 1 Before that, most infants and children with univentricular heart malformations died young from cyanosis and heart failure. Few were palliated with pulmonary artery banding or aortopulmonary shunts. Severe complications were common from shunt obstruction, cyanosis, thromboembolism, bleeding, heart failure and often associated with a very poor quality of life. Only a few reached adult age. With the introduction of the Fontan operation and the resulting Fontan circulation, the prognosis improved dramatically. It is estimated that almost 25,000 patients are alive today in Europe with a univentricular heart malformation palliated with some type of Fontan operation. The large and increasing number of young and adult subjects with a Fontan circulation is all under regular follow-up by specialised cardiologists. However, they may also occasionally be seen by paediatricians and family doctors who should have a brief understanding of the Fontan circulation and the most frequent complications. The Fontan operation was initially used in subjects with tricuspid atresia. With modifications of the operation, most types of univentricular heart malformations could be successfully converted to a Fontan circulation. The original incorporation of the right atrium

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“…However, the risks of mortality and morbidity remain high in these patients. This review by Gewillig et al 7 proposes a framework of principles to conceptualise the functionality and limitations of a Fontan circulation.…”

Section: A Framework Of Principles For Conceptualising the Fontal Circulationmentioning

confidence: 99%

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Käll¹,

Lagercrantz²

2020

Acta Paediatrica

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Providing a framework of principles for conceptualising the Fontan circulation

Cited by 47 publications

References 22 publications

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

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

Fontan circulation has improved life expectancy for infants born with complex heart disease over the last 50 years but has also resulted in significant morbidity

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