Resistance training improves cardiac output, exercise capacity and tolerance to positive airway pressure in Fontan physiology.

Subjects with Fontan-type circulation have no sub-pulmonary ventricle and thus depend exquisitely on the respiratory bellows and peripheral muscle pump for cardiac filling. We hypothesised that resistance training to augment the peripheral muscle pump might improve cardiac filling, reduce inspiratory-dependence of IVC return to the heart and thus improve exercise capacity and cardiac output on constant positive airway pressure (CPAP). Eleven Fontan subjects (32+/-2 years, mean+/-SEM) had cardiac magnetic resonance imaging (MRI) and exercise testing (CPET); six underwent 20 weeks of high-intensity resistance training; others were non-exercising controls. After training, CPET was repeated. Four trainers had MRI with real-time flow measurement at rest, exercise and on CPAP in the trained state and following a 12-month detrain. In the trained state, muscle strength increased by 43% (p=0.002), as did total muscle mass (by 1.94 kg, p=0.003) and peak VO2 (by 183 ml/min, p=0.02). After detraining, calf muscle mass and peak workload had fallen significantly (p<0.03 for both) as did peak VO2 (2.72 vs. 2.18 l/min, p<0.001) and oxygen pulse, a surrogate for SV (16% lower, p=0.005). Furthermore after detraining, SV on MRI decreased at rest (by 11 ml, p=0.01) and during moderate-intensity exercise (by 16 ml, p=0.04); inspiratory-dependent IVC blood return during exercise was 40% higher (p=0.02). On CPAP, cardiac output was lower in the detrained state (101 vs. 77 ml/s, p=0.03). Resistance muscle training improves muscle mass, strength and is associated with improved cardiac filling, stroke volume, exercise capacity and cardiac output on CPAP, in adults with Fontan-type circulation.

Cordina RL ，O'Meagher S ，Karmali A ，Rae CL ，Liess C ，Kemp GJ ，Puranik R ，Singh N ，Celermajer DS ... -  《-》

Inspiratory Muscle Training Is Associated With Improved Inspiratory Muscle Strength, Resting Cardiac Output, and the Ventilatory Efficiency of Exercise in Patients With a Fontan Circulation.

Patients with a Fontan circulation have reduced exercise capacity and respiratory muscle strength. Inspiratory muscle training (IMT) improves exercise capacity and quality of life in adults with heart failure. We assessed whether 6 weeks of a home-based program of IMT improves inspiratory muscle strength and the ventilatory efficiency of exercise in adolescent patients with a Fontan circulation. Twenty-three adolescent participants (aged 16±2 years) with a Fontan circulation underwent 6 weeks of IMT for 30 minutes daily. Respiratory muscle strength (maximal inspiratory pressure and expiratory pressure), lung function, and exercise capacity (cardiopulmonary exercise testing) were assessed. Fourteen of 23 participants also underwent exercise cardiac magnetic resonance imaging to examine the effects of IMT on cardiac output and systemic and pulmonary blood flow. Six weeks of IMT improved maximal inspiratory pressure by 36±24 cm H2O (61±46%) with no change in maximal expiratory pressure. Ventilatory efficiency of exercise improved after 6 weeks of IMT (from 34.2±7.8 to 32.2±5.6, P=0.04). In those who underwent exercise cardiac magnetic resonance imaging, IMT increased resting cardiac output (from 4.2±1.2 to 4.5±1.0 L/min, P=0.03) and ejection fraction (from 50.1±4.3 to 52.8±6.1%, P=0.03). Six weeks of IMT is associated with improved inspiratory muscle strength, ventilatory efficiency of exercise, and resting cardiac output in young Fontan patients. IMT may be a simple beneficial addition to the current management of Fontan patients, potentially reducing exercise intolerance and long-term morbidity and mortality.

Laohachai K ，Winlaw D ，Selvadurai H ，Gnanappa GK ，d'Udekem Y ，Celermajer D ，Ayer J ... -  《Journal of the American Heart Association》

Relation of systemic venous return, pulmonary vascular resistance, and diastolic dysfunction to exercise capacity in patients with single ventricle receiving fontan palliation.

Fontan patients have a reduced exercise capacity, primarily owing to limitations in the ability to augment pulmonary blood flow and stroke volume. To date, the mechanism of peak exercise pulmonary blood flow restriction has not been elucidated. We performed a single-center, prospective, crossover trial of supine and upright exercise in Fontan patients and healthy controls to determine the mechanisms of exercise limitation in the Fontan-palliated patient. A total of 29 Fontan patients and 16 control subjects completed the protocol. The duration of exercise, percentage of predicted peak oxygen consumption (VO(2)) and peak work were reduced in the Fontan group, regardless of posture (p < or = 0.03). The percentage of predicted oxygen pulse, a surrogate for pulmonary stroke volume, was not increased with supine posture in the Fontan cohort (upright, 82.3 + or - 18.8% vs supine, 82.4 + or - 19.7%; p = 0.6). In both groups, the percentage of predicted peak VO(2) was lower with supine exercise than with upright exercise (p < or =0.002). Diastolic dysfunction was present in 57% of the Fontan patients and was associated with a reduced percentage of predicted peak VO(2) (p = 0.04) and supine peak work (p = 0.008). Six Fontan patients who underwent supine exercise with indwelling catheters failed to demonstrate the expected decrease in pulmonary vascular resistance characteristically seen with peak exercise (at rest, 2.8 + or - 0.7 mm Hg/L/min/m(2) vs at peak, 2.8 + or - 0.9 mm Hg/L/min/m(2); p = 0.9). In conclusion, supine exercise in Fontan patients does not result in an increased VO(2) or oxygen pulse, suggesting that inadequate venous return might not be the primary limitation of exercise capacity in this population. Diastolic dysfunction and relatively excessive peak exercise pulmonary vascular resistance might be more important factors in Fontan exercise limitation.

Goldstein BH ，Connor CE ，Gooding L ，Rocchini AP ... -  《-》

Sildenafil improves exercise hemodynamics in Fontan patients.

Van De Bruaene A ，La Gerche A ，Claessen G ，De Meester P ，Devroe S ，Gillijns H ，Bogaert J ，Claus P ，Heidbuchel H ，Gewillig M ，Budts W ... -  《-》

Skeletal muscle abnormalities and exercise capacity in adults with a Fontan circulation.

Cordina R ，O'Meagher S ，Gould H ，Rae C ，Kemp G ，Pasco JA ，Celermajer DS ，Singh N ... -  《-》