Abstract

Patients with Fontan circulation have no subpulmonary ventricle and a passive pulmonary perfusion. Considerable percentage of the pulmonary blood flow is driven by pressure shift due to respiration. Impairments in respiratory musculature strength are associated with a reduced exercise capacity. This study investigated the effect of a daily six months inspiratory muscle training (IMT) on exercise and lung capacity in adult Fontan patients. After a lung function and cardiopulmonary exercise test (CPET), 42 Fontan patients (50% female; 30.5±8.1years) were randomized into either an intervention group (IG), or a control group (CG). The IG performed a telephone-supervised, daily IMT of three sets with 10-30 repetitions for six months. After six months of IMT, the IG did not improve in any exercise and lung capacity parameter compared to CG. VO2peak (ΔVO2peak: IG: 0.05 [-1.53; 1.33] ml/kg/min vs. CG: -0.50 [-1.20; 0.78] ml/kg/min; p=.784) and FVC (ΔFVC: IG: 0.07 [-0.16; 0.22] l vs. CG:-0.05 [-0.24; 0.18] l; p=.377) remained unchanged, while FEV1 trended to improve (ΔFEV1: IG: 0.05 [-0.07; 0.13] l vs. CG: -0.10 [-0.19; 0.03] l; p=.082). Only oxygen saturation at rest improved significantly (ΔSpO2: IG: 1.50 [-0.25; 3.00] % vs. CG: -0.50 [-1.75; 0.75] %; p=.017). A daily six months IMT did not improve exercise and lung capacity and lung volumes in Fontan patients.

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