Clinical aspects and targeted inspiratory muscle training in children and adolescents with Fontan circulation: a randomized controlled trial

BACKGROUND: Since 1968, thousands of patients with a morphologically or functionally univentricular heart have been treated with a total cavopulmonary connection/Fontan operation. Because of the resulting passive pulmonary perfusion, blood flow is assisted by the pressure shift during respiration. Respiratory training is known to improve exercise capacity and cardiopulmonary function. However, there are limited data on whether respiratory training can also improve physical performance after Fontan surgery. The aim of the present study was to clarify the effects of six months of daily home-based inspiratory muscle training (IMT) aimed at increasing physical performance by strengthening respiratory muscles, improving lung function and peripheral oxygenation. METHODS: In this non-blinded randomized controlled trial, the effects of IMT on lung capacity and exercise capacity were measured in a large cohort of 40 Fontan patients (25% female; 12.3±2.2 years) who were under regular follow-up by the outpatient clinic of the Department of Congenital Heart Defects and Pediatric Cardiology of the German Heart Center Munich. After a lung function test and a cardiopulmonary exercise test, patients were randomly assigned in a parallel arm design to either an intervention group (IG) or a control group (CG) via stratified and computer-generated letter randomization from May 2014 to May 2015. The IG completed a daily, telephone-monitored IMT of three sets of 30 repetitions for six months with an inspiratory resistive training device (POWERbreathe medic(®)), the CG continued their usual daily activities without an IMT until the second examination within the timeframe of November 2014 until November 2015. RESULTS: After six months of IMT, lung capacity values in the IG (n=18) did not increase significantly compared to the CG [n=19; ΔFVC: IG: 0.21±0.16 l vs. CG 0.22±0.31 l; P=0.946 (CI: −0.16, 0.17); ΔFEV1: CG: 0.14±0.30 vs. IG: 0.17±0.20 P=0.707 (CI: −0.20, 0.14)]. Exercise capacity did not improve significantly, yet the maximum workload achieved trended to improve with an increase of 14% in the IG vs. 6.5% in the CG [P=0.113 (CI: −15.8, 1.76)]. There was a significant increase of oxygen saturation at rest in the IG compared to the CG [IG: 3.31%±4.09% vs. CG: 0.17%±2.92%, P=0.014 (CI: −5.60, −0.68)]. Compared to the CG, the mean oxygen saturation at peak exercise no longer dropped below 90% in the IG. This observation is thus not statistically significant, yet of clinical relevance. CONCLUSIONS: The results of this study show benefits of an IMT in young Fontan patients. Even if some data are not statistically significant, they may still be clinically relevant and may contribute to a multidisciplinary approach in patient care. IMT should therefore be an additional target and integrated into the training program to improve the prognosis of Fontan patients. TRIAL REGISTRATION: German Clinical Trials Register; DRKS.de; registration ID: DRKS00030340