Hedlund Eva R, Ljungberg Henrik, Söderström Liselott, Lundell Bo, Sjöberg Gunnar
Department of Women's and Children's Health,Karolinska Institutet,Stockholm,Sweden.
Cardiol Young. 2018 Sep;28(9):1115-1122. doi: 10.1017/S1047951118000902. Epub 2018 Jul 4.
The objective of this research was to study lung function, physical capacity, and effect of endurance training in children and adolescents after Fontan palliation compared with healthy matched controls.
Fontan patients (n=30) and healthy matched control patients (n=25) performed dynamic and static spirometry, and pulmonary diffusing capacity and maximal oxygen uptake tests, before and after a 12-week endurance training programme and at follow-up after 1 year.
Fontan patients had a restrictive lung pattern, reduced pulmonary diffusing capacity (4.27±1.16 versus 6.61±1.88 mmol/kPa/minute, p<0.001), and a reduced maximal oxygen uptake (35.0±5.1 versus 43.7±8.4 ml/minute/kg, p<0.001) compared with controls. Patients had air trapping with a higher portion of residual volume of total lung capacity compared with controls (26±6 versus 22±5%, p<0.05). Vital capacity increased for patients, from 2.80±0.97 to 2.91±0.95 L, p<0.05, but not for controls after endurance training. The difference in diffusing capacity between patients and controls appeared to be greater with increasing age.
Fontan patients have a restrictive lung pattern, reduced pulmonary diffusing capacity, and reduced maximal oxygen uptake compared with healthy controls. Endurance training may improve vital capacity in Fontan patients. The normal increase in pulmonary diffusing capacity with age and growth was reduced in Fontan patients, which is concerning. Apart from general health effects, exercise may improve lung function in young Fontan patients and should be encouraged.
本研究的目的是比较接受Fontan姑息手术的儿童和青少年与健康对照者的肺功能、身体能力以及耐力训练的效果。
Fontan患者（n = 30）和健康对照患者（n = 25）在为期12周的耐力训练计划前后以及1年后的随访时进行了动态和静态肺量测定、肺弥散功能和最大摄氧量测试。
与对照组相比，Fontan患者呈现限制性肺型，肺弥散功能降低（4.27±1.16对比6.61±1.88 mmol/kPa/分钟，p<0.001），最大摄氧量降低（35.0±5.1对比43.7±8.4 ml/分钟/千克，p<0.001）。与对照组相比，患者存在气体潴留，残气量占肺总量的比例更高（26±6对比22±5%，p<0.05）。耐力训练后，患者的肺活量从2.80±0.97升增加到2.91±0.95升，p<0.05，但对照组未增加。随着年龄增长，患者与对照组之间的弥散功能差异似乎更大。
与健康对照组相比，Fontan患者呈现限制性肺型，肺弥散功能降低，最大摄氧量降低。耐力训练可能改善Fontan患者的肺活量。Fontan患者中随着年龄和生长肺弥散功能的正常增加减少，这令人担忧。除了对总体健康的影响外，运动可能改善年轻Fontan患者的肺功能，应予以鼓励。
