BACKGROUNDS: 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. METHODS: After a lung function and cardiopulmonary exercise test (CPET), 42 Fontan patients (50% female; 30.5 +/- 8.1 years) 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 to 30 repetitions for six months. RESULTS: After six months of IMT, the IG did not improve in any exercise and lung capacity parameter compared to CG. VO2peak (delta VO2peak IG 0.05 (-1.53 to 1.33) ml/kg/min versus CG -0.50 (-1.20 to 0.78) ml/kg/min; p = 0.784) and FVC (delta FVC IG 0.07 (-0.16 to 0.22) l versus CG -0.05 (-0.24 to 0.18) l; p = 0.377) remained unchanged, while FEV1 trended to improve (delta FEV1 IG 0.05 (-0.07 to 0.13) l versus CG -0.10 (-0.19 to 0.03) l; p = 0.082). Only oxygen saturation at rest improved significantly (delta SpO2 IG 1.50% (-0.25 to 3.00) versus CG -0.50% (-1.75 to 0.75); p = 0.017). CONCLUSIONS: A daily six months IMT did not improve exercise and lung capacity and lung volumes in Fontan patients.

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