Abstract

There are no previous studies on arterial stiffness and its associations with cardiorespiratory fitness in young Fontan patients. Therefore, we examined the arterial stiffness and its relationship to cardiorespiratory fitness in children and young adults with a Fontan circulation. Altogether, 17 Fontan patients and 26 healthy controls (16 females and 27 males aged 8–40 years) participated in this cross-sectional study. The cardiorespiratory fitness was assessed by cardiopulmonary exercise testing on a cycle ergometer and was defined as the standard deviation scores (SDS) of peak oxygen uptake per body mass (VO2peak/kg) based on the national reference values and assessed with cardiopulmonary exercise testing on a cycle ergometer. Aortic pulse wave velocity (PWVao) as a measure of arterial stiffness and aortic Augmentation Index (AIX) as a measure of peripheral arterial tone, were assessed by non-invasive oscillometric device from upper arm. Body adiposity was determined by body mass index SDS and the sport participation by interview. Data were analyzed using linear regression analyses and Pearson’s correlations, adjusted for age and sex. Fontan patients had a lower VO2peak/kg-SDS (− 2.69 vs 0.078), higher PWVao-SDS (1.13 vs − 0.24) and higher AIX (19.26% vs 8.49%) in comparison with healthy controls. PWVao and AIX were negatively associated with VO2peak/kg (standard regression coefficient (β) − 0.525, 95% confidence interval (CI) − 0.722 to − 0.227, p < 0.01 and β − 0.371, 95% CI − 0.672 to − 0.080, p = 0.014). Young Fontan patients have the arterial stiffness of healthy people who are twice as old. Thereby, children and young adults with a Fontan circulation have a lower cardiorespiratory fitness and less sport participation. Arterial stiffness is inversely associated with cardiorespiratory fitness and exercise training might be an intervention to improve vascular health in this population.

Highlights

  • 6–8 per 1000 children are born with a congenital heart disease (CHD) [1]

  • Amount of sport participation was accessed by interview and the children and adolescents were asked to determinate the amount of time a week they participate in sport

  • The gender distribution and average age between the Fontan patients and control were comparable, the body mass index (BMI) was lower for the Fontan patients (Table 1)

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Summary

Introduction

6–8 per 1000 children are born with a congenital heart disease (CHD) [1]. Fontan procedure is performed when there is congenitally one viable ventricle and a biventricular repair is not possible. Since the introduction of the Fontan procedure, most patients are able to survive into adulthood [2]. After this procedure, the blood from the superior and inferior vena cava is diverted to the pulmonary arteries, to partly repair the pulmonary blood flow [3]. Even when the Fontan procedure confers an improvement of ca. Exercise is safe for stable Fontan patients and can be beneficial to increase exercise capacity [5].

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