Home-Based Long-Term Physical Endurance and Inspiratory Muscle Training for Children and Adults With Fontan Circulation-Initial Results From a Prospective Study.

Stefan Dirks,Thomas Thouet,Stanislav Ovroutski,Hannes Sallmon,Bernd Wolfarth,Anastasia Schleiger,Peter Kramer,Hans-Martin Speck,Felix Berger

doi:10.3389/fcvm.2021.784648

Abstract

BackgroundPatients with congenital heart disease (CHD)—including those after Fontan operation—are encouraged to be physically active.AimTo prospectively determine the effects of an individually adapted, home-based cycle ergometer endurance training in combination with inspiratory muscle training (IMT) in pediatric and adult Fontan patients. We, herein, report the results of the initial 10-months follow-up (phase 1).Methods18 patients (median age 16.5 years; range 10-43 years) completed baseline check-ups, and 4 and 10 months follow-up visits, which each included cardiopulmonary exercise testing (CPET), bodyplethysmography (including measurement of respiratory muscle strength), and a quality of life questionnaire (PedsQL™). The training program consisted of a home-based cycle ergometer endurance training on a “Magbike® AM-5i/3i” (DKN Technology®, Clermont-Ferrand, France) and IMT with a handheld “POWERbreathe® Medic plus” device. Patients performed 90 min of endurance training per week in addition to IMT (30 breaths per day, 6-7 times per week). After the first 4 months, patients underwent additional interval training.ResultsAfter 10 months of training, we observed significant increases in maximum relative workload (W/kg, p = 0.003) and in maximum inspiratory (MIP, p = 0.002) and expiratory (MEP, p = 0.008) pressures. Peak VO2 values did not increase significantly as compared to baseline (p = 0.12) in the entire cohort (n = 18), but reached statistical significance in a subgroup analysis of teenage/adult patients (n = 14; p = 0.03). Patients' subjective quality of life did not show any significant changes after 10 months of training.DiscussionIn Fontan patients, an individually adapted home-based training is safe and associated with improvements in some CPET variables. However, these improvements did not translate into an improved QoL after 10 months. With an unclear, but most likely negative, impact of the COVID-19 pandemic, improvements in QoL may become evident during further follow-up (phase 2 of the study).

Highlights

The development of the Fontan operation and its subsequent modifications represent a mainstay of surgical palliation in children with single-ventricle malformations [1]
A decreased heart rate reserve, ventricular fibrosis and/or hypertrophy, and an impaired systolic and/or diastolic function all result in a reduced capacity to increase stroke volume and cardiac output during physical activity [2, 5]
We observed significant increases in maximum power/weight ratio, as well as in maximum inspiratory (MIP) and expiratory (MEP) pressures after 10 months of combined endurance and inspiratory muscle training

Summary

Introduction

The development of the Fontan operation and its subsequent modifications represent a mainstay of surgical palliation in children with single-ventricle malformations [1]. A decreased heart rate reserve, ventricular fibrosis and/or hypertrophy, and an impaired systolic and/or diastolic function all result in a reduced capacity to increase stroke volume and cardiac output during physical activity [2, 5]. Fontan patients exhibit a restrictive lung function pattern, an impaired pulmonary diffusing capacity, and a reduced maximal oxygen uptake [7], which, in turn, are associated with decreases in health-related quality of life [8,9,10,11] and exercise tolerance [12,13,14]. Patients with congenital heart disease (CHD)—including those after Fontan operation—are encouraged to be physically active

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