Does functional health status predict health-related quality of life in children after Fontan operation?

Dulfer, Karolijn; Bossers, Sjoerd S. M.; Utens, Elisabeth M. W. J.; Duppen, Nienke; Kuipers, Irene M.; Kapusta, Livia; Iperen, G. van; Schokking, Michiel; Harkel, Arend D J Ten; Takken, Tim; Helbing, Willem A.

doi:10.1017/s1047951115000426

Cited by 23 publications

(28 citation statements)

References 42 publications

(84 reference statements)

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“…In fact, any physical limitation could invalidate both physical and psychological aspects. 40 In our study population, the mean score of every category of the SF-36 showed no relevant difference in comparison to the expected scores of a healthy comparison group. As explanation to such high scores, it has been suggested that the patients cope with their pathology and express their feelings more realistic than their peers, a fact that is already known from childhood oncology patients.…”

Section: Discussionmentioning

confidence: 45%

A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

Callegari

Neidenbach

Milanesi

et al. 2018

Congenital Heart Disease

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Aim:The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and quality of life is unknown and objective of this study.Methods: This multicenter retrospective/cross-sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connection). Resting spirometry, cardiopulmonary exercise tests, and quality-of-life assessment (SF-36 questionnaire) were performed between 2003 and 2015.Results: Overall, mean forced expiratory volume in one second (FEV 1 ) was 74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV 1 was <80%pred., and all of these patients had FEV 1 /forced vital capacity (FVC) > 80%, suggestive of a restrictive ventilatory pattern. Reduced FEV 1 was associated with a reduced peakVO 2 of 67.0 ± 17.6%pred. (r = 0.43, P < .0001), even if analyzed together with possible confounding factors (sex, BMI, age, years after palliation, number of interventions, scoliosis, diaphragmatic paralysis). Synergistically to exercise capacity, FEV 1 was associated to quality of life in terms of physical component summary (r = 0.30, P = .002), physical functioning (r = 0.25, P = .008), bodily pain (r = 0.22, P = .02), and general health (r = 0.16, P = .024). Lower FEV 1 was associated with diaphragmatic paralysis (P = .001), scoliosis (P = .001), higher number of interventions (P = .002), and lower BMI (P = .01). No correlation was found to ventricular morphology, type of surgeries, or other perioperative/long-term complications.Conclusions: This study shows that the common restrictive ventilatory pattern in Fontan patients is associated with lower exercise capacity and quality of life. Risk factors are diaphragmatic paralysis, scoliosis, a high total number of interventions and low BMI. K E Y W O R D SCPET, Fontan, quality of life, spirometry, ventilatory function

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Section: Discussionmentioning

confidence: 45%

A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

Callegari

Neidenbach

Milanesi

et al. 2018

Congenital Heart Disease

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“…Patients with Fontan circulation might experience an affected quality of life (37). Studies have reported a negatively affected quality of life (37)(38)(39)(40)(41)(42)(43) and reduced exercise performance even in patients without severe complications during surgical procedures and medical follow-ups (23,25,26,30). Also, it has been shown that patients with Fontan circulation have a lower quality of life compared with patients with less severe heart defects (38).…”

Section: Long-term Outcomementioning

confidence: 99%

“…In a recent study (24), it was shown that patients with Fontan circulation had decreased physical performance, but similar quality of life compared with healthy subjects. Atz et al 44 health-related quality of life (37). A clinical problem, raised by patients and their parents, is disturbed sleep and daily fatigue.…”

Section: Long-term Outcomementioning

confidence: 99%

Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training

et al. 2018

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Fontan patients have a restrictive lung pattern, reduced pulmonary diffusing capacity, and reduced maximal oxygen uptake compared with healthy controls. Endurance training may improve vital capacity in Fontan patients. The normal increase in pulmonary diffusing capacity with age and growth was reduced in Fontan patients, which is concerning. Apart from general health effects, exercise may improve lung function in young Fontan patients and should be encouraged.

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“…of pumping ventricle, number of surgical procedures and complications, age at completed Fontan circulation and arrhythmia have also been reported to influence healthrelated quality of life (11). To our knowledge, there are no available reports on the daily participation in physical exercise and sports among young Fontan patients and how exercise is perceived.…”

Section: Key Notesmentioning

confidence: 99%

“…The quality of life for children with severe cardiovascular disease has been shown to be lower than that of healthy children . In Fontan patients, factors such as the type of pumping ventricle, number of surgical procedures and complications, age at completed Fontan circulation and arrhythmia have also been reported to influence health‐related quality of life . To our knowledge, there are no available reports on the daily participation in physical exercise and sports among young Fontan patients and how exercise is perceived.…”

Section: Introductionmentioning

confidence: 99%

Reduced physical exercise and health‐related quality of life after Fontan palliation

et al. 2016

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Does functional health status predict health-related quality of life in children after Fontan operation?

Cited by 23 publications

References 42 publications

A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training

Reduced physical exercise and health‐related quality of life after Fontan palliation

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