Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney School of Health Sciences, University of Sydney, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Sydney, NSW, Australia; Heart Research Institute, Sydney, NSW, Australia.
Murdoch Children's Research Institute, Melbourne, Vic, Australia.
Heart Lung Circ. 2021 Sep;30(9):1356-1363. doi: 10.1016/j.hlc.2021.01.004. Epub 2021 Mar 18.
In people with a Fontan circulation, serial cardiopulmonary exercise testing (CPET) to evaluate change in peak exercise capacity has been increasingly recognised as a useful prognostic tool; a decline is associated with adverse clinical outcomes. The aim of this study is to describe the "natural" history of exercise capacity in the Australian and New Zealand (ANZ) Fontan cohort and to identify factors associated with a decline.
The ANZ Fontan registry was retrospectively reviewed for adolescent and adult patients (≥16 years) with serial CPET results performed on a cycle ergometer ≥6 months apart. Patients were excluded if they underwent a surgical procedure or fenestration closure in-between tests or if the tests were considered as submaximal effort. Exercise capacity trajectory was defined as the change in percentage of predicted peak oxygen uptake (% pred VO) points per year.
Thirty-seven (37) patients (59.5% male, mean age 24±7 years) were eligible. Average duration between CPET was 5.3±3.9 years. At baseline, % pred VO was 61.3±14.5%. Thirteen (13) (35%) had a systemic right ventricle, and 14 (38%) had an atriopulmonary type Fontan circulation. Average change in % pred VO overall was +1.3±6.4 percentage points per year. Sixteen (16) had a negative exercise capacity trajectory, and the average decline in that group was -2.7±3.4 percentage points per year. There was no association between exercise capacity trajectory and clinical characteristics. Of the 18 patients with physical activity levels recorded, 12 (67%) were physically active and % pred VO in that group increased by 2.7±4.0 percentage points per year compared with the physically inactive group who fell by 0.5±0.8 percentage points per year.
In this ANZ series of Fontan patients, over half of our cohort had stable, or an increase, in peak exercise capacity. Regular participation in physical activity was common in patients with a positive exercise capacity trajectory. Clinical characteristics were not associated with exercise capacity trajectory.
在 Fontan 循环患者中，连续心肺运动测试（CPET）评估峰值运动能力的变化已逐渐被认为是一种有用的预后工具；运动能力下降与不良临床结局相关。本研究旨在描述澳大利亚和新西兰（ANZ）Fontan 队列中运动能力的“自然”变化过程，并确定与运动能力下降相关的因素。
回顾性分析 ANZ Fontan 注册中心接受≥6 个月间隔的连续 CPET 检查的青少年和成年患者（≥16 岁）的资料。如果患者在两次测试之间接受手术或开窗闭合，或者测试被认为是亚最大努力，则将其排除在外。运动能力轨迹定义为每年预测峰值摄氧量（% pred VO）的变化点。
37 例（59.5%为男性，平均年龄 24±7 岁）患者符合条件。CPET 之间的平均间隔时间为 5.3±3.9 年。基线时，% pred VO 为 61.3±14.5%。13 例（35%）患者存在系统性右心室，14 例（38%）患者存在三尖瓣肺静脉 Fontan 循环。总体而言，% pred VO 的平均变化为每年增加 1.3±6.4 个百分点。16 例（16%）患者的运动能力轨迹呈负向，该组的平均下降速度为每年-2.7±3.4 个百分点。运动能力轨迹与临床特征之间无相关性。在记录体力活动水平的 18 例患者中，12 例（67%）患者有体力活动，该组的% pred VO 每年增加 2.7±4.0 个百分点，而无体力活动组的% pred VO 每年下降 0.5±0.8 个百分点。
在本 ANZ Fontan 患者系列中，超过一半的患者峰值运动能力稳定或增加。在运动能力呈正性变化的患者中，经常进行体力活动很常见。临床特征与运动能力轨迹无相关性。
