Laohachai Karina, Webb Annabel, Cordina Rachael, d'Udekem Yves, Weintraub Robert, Rice Kathryn, Ayer Julian
The University of Sydney, Sydney, New South Wales 2006, Australia.
Women's and Children's Hospital, Adelaide, South Australia 5006, Australia.
Int J Cardiol Congenit Heart Dis. 2024 Apr 29;17:100514. doi: 10.1016/j.ijcchd.2024.100514. eCollection 2024 Sep.
Exercise capacity is reduced in patients with a Fontan circulation compared to their healthy peers secondary to altered haemodynamics of the Fontan circulation. Cardiopulmonary exercise testing is routinely used in Fontan patients to assess their cardiopulmonary response to exercise and help guide management decisions, however their results are routinely compared to normative data derived from healthy individuals.
Using data available from the Pediatric Heart Network, we performed a retrospective study to derive Fontan-specific normative values in a subgroup of well-functioning adolescent patients. We utilised results from the Congenital Heart Adolescent and Teenager (CHAT) Questionnaire to classify patients into well-functioning and lower-functioning groups.
We generated Fontan-specific regression equations in adolescent male and females for normative values of peak oxygen consumption (VO), maximal workload and VO at anaerobic threshold.
We propose that Fontan-specific normative values are a more useful comparison than healthy normative data due to the known circulatory limitations of the Fontan circulation and will assist in management decision and prognostication.
与健康同龄人相比，接受Fontan循环手术的患者运动能力下降，这是由于Fontan循环的血流动力学改变所致。心肺运动试验常用于Fontan患者，以评估他们对运动的心肺反应并帮助指导管理决策，然而其结果通常与来自健康个体的标准数据进行比较。
利用从儿科心脏网络获得的数据，我们进行了一项回顾性研究，以得出一组功能良好的青少年患者的Fontan特异性标准值。我们利用先天性心脏病青少年和青少年（CHAT）问卷的结果将患者分为功能良好组和功能较低组。
我们针对青少年男性和女性，生成了Fontan特异性回归方程，用于峰值耗氧量（VO）、最大工作量和无氧阈值时的VO的标准值。
我们提出，由于已知Fontan循环的循环限制，Fontan特异性标准值比健康标准数据更具可比性，将有助于管理决策和预后判断。
