Alsaied Tarek, Li Runjia, Christopher Adam B, Fogel Mark A, Slesnick Timothy C, Krishnamurthy Rajesh, Muthurangu Vivek, Dorfman Adam L, Lam Christopher Z, Weigand Justin D, Robinson Joshua D, Cordina Rachael, Olivieri Laura J, Rathod Rahul H
The Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pennsylvania, USA.
Department of Biostatistics, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
JACC Adv. 2024 Sep 9;3(10):101254. doi: 10.1016/j.jacadv.2024.101254. eCollection 2024 Oct.
Fontan patients exhibit decreased exercise capacity. However, there is a subset of high-performing Fontan (HPF) patients with excellent exercise capacity.
This study aims to: 1) create a Fontan-specific percent predicted peak VO tool using exercise data; 2) examine clinical factors associated with HPF patients; and 3) examine late outcomes in HPF patients.
Patients in the multi-institutional Fontan Outcomes Registry Using CMR Examination above the age of 8 years who had a maximal exercise test were included. An HPF patient was defined as a patient in the upper Fontan-specific percent predicted peak VO quartile. Multivariable logistic regression was employed to investigate factors associated with the HPF and Cox regression was used to examine the association between HPF patients and late outcomes (composite of death or listing for cardiac transplant).
The study included 813 patients (mean age: 20.2 ± 8.7 years). An HPF patient was associated with left ventricular morphology (OR: 1.50, = 0.04), mixed morphology (OR: 2.23, < 0.001), and a higher ejection fraction (OR: 1.31 for 10% increase, = 0.01). Patients with at least moderate atrioventricular valve regurgitation, protein-losing enteropathy, or who were using psychiatric medications, were less likely to be an HPF patient. After a mean follow-up of 3.7 years, 46 (5.7%) patients developed a composite endpoint. HPF had a lower risk of death or listing for cardiac transplant (HR: 0.06 [95% CI: 0.01-0.25]).
Patients with HPF have more favorable outcomes when compared to patients with lower exercise capacity. This large registry data highlights the role of exercise testing in providing personalized care and surveillance post-Fontan.
Fontan手术患者的运动能力下降。然而，有一部分高功能Fontan（HPF）患者具有出色的运动能力。
本研究旨在：1）利用运动数据创建一个Fontan特异性预测峰值VO₂百分比工具；2）研究与HPF患者相关的临床因素；3）研究HPF患者的晚期结局。
纳入多机构使用CMR检查且年龄超过8岁并进行了最大运动试验的Fontan结局登记患者。HPF患者定义为处于Fontan特异性预测峰值VO₂四分位数上限的患者。采用多变量逻辑回归研究与HPF相关的因素，并使用Cox回归检查HPF患者与晚期结局（死亡或列入心脏移植名单的综合情况）之间的关联。
该研究纳入了813例患者（平均年龄：20.2±8.7岁）。HPF患者与左心室形态（OR：1.50，P = 0.04）、混合形态（OR：2.23，P < 0.001）以及较高的射血分数（每增加10%，OR为1.31，P = 0.01）相关。至少有中度房室瓣反流、蛋白丢失性肠病或正在使用精神科药物的患者不太可能是HPF患者。平均随访3.7年后，46例（5.7%）患者出现了复合终点。HPF患者死亡或列入心脏移植名单的风险较低（HR：0.06 [95% CI：0.01 - 0.25]）。
与运动能力较低的患者相比，HPF患者的结局更有利。这项大型登记数据突出了运动试验在Fontan术后提供个性化护理和监测中的作用。
