Gallegos Flora Nuñez, Bernstein Daniel, Punn Rajesh, Long Jin, Stauffer Katie Jo, Thorson Kelly, Chen Sharon, Lui Mavis C, Olson Inger, Myers Jonathan, Palaniappan Latha, Tierney Seda
Stanford University School of Medicine, Stanford, CA 291 Campus Drive, Stanford, CA.
Stanford University School of Medicine, Stanford, CA 291 Campus Drive, Stanford, CA.
Am Heart J. 2025 May 31;290:58-68. doi: 10.1016/j.ahj.2025.05.015.
By age 40, roughly half of the individuals with Fontan circulation will have died or undergone heart transplantation. Poor exercise capacity and endothelial dysfunction accelerate disease progression.
This study aims to assess the systolic function of the single ventricle (SV) in pediatric Fontan patients entering an exercise intervention (RE-ENERGIZE FONTAN) and how it is associated with exercise capacity and endothelial function.
This cohort comes from an ongoing randomized trial in Fontan patients, using live video conferencing for supervised exercise. Participants (ages 8-19) cleared for exercise underwent 2D/3D echocardiograms, cardiopulmonary testing, and endothelial function (RHI). 2D longitudinal strain (LS, right ventricle), 2D global longitudinal strain (GLS, left ventricle), and circumferential strain were measured with TomTec, and 3D ejection fraction (3D-EF) and 3D-GLS were calculated.
We have enrolled 114 Fontan patients. Median age was 12.7 years (IQR 10.2, 15.6). The median time from Fontan operation was 8.8 years (IQR 6.2, 12.0). Fifty-seven patients (50%) had a single right ventricle. SV systolic performance measures were: 2D-LS/GLS -15.6% ± 4.19%, circumferential strain was -18.7% ± 6.83%, 3D-EF 49.9% ± 7.26%, and 3D-GLS -16.8% ± 4.37%. Peak VO was 1,290 ± 502 mL/min, and percent predicted peak VO was 67.8% ± 15.6%. RHI was 1.44 ± 0.576. 2D-LS/GLS and 3D EF correlated with percent predicted peak VO (R = -0.28, P = .007 and R = 0.24, P = .019). 3D-EF correlated positively with RHI (R = 0.29, P = .0071).
In this cohort of pediatric Fontan patients, SV systolic function was diminished at baseline, and there was a direct correlation between 2D strain and 3D-EF with percent predicted peak VO and additionally, 3D-EF with endothelial function.
NCT04195451.
到40岁时，大约一半接受Fontan循环的患者会死亡或接受心脏移植。运动能力差和内皮功能障碍会加速疾病进展。
本研究旨在评估参加运动干预（RE-ENERGIZE FONTAN）的小儿Fontan患者单心室（SV）的收缩功能，以及它与运动能力和内皮功能的关系。
该队列来自一项正在进行的Fontan患者随机试验，使用实时视频会议进行监督运动。符合运动条件的参与者（8至19岁）接受了二维/三维超声心动图、心肺测试和内皮功能（RHI）检查。使用TomTec测量二维纵向应变（LS，右心室）、二维整体纵向应变（GLS，左心室）和圆周应变，并计算三维射血分数（3D-EF）和三维GLS。
我们招募了114名Fontan患者。中位年龄为12.7岁（四分位间距10.2，15.6）。距Fontan手术的中位时间为8.8年（四分位间距6.2，12.0）。57名患者（50%）有单一右心室。SV收缩功能指标为：二维LS/GLS -15.6%±4.19%，圆周应变-18.7%±6.83%，3D-EF 49.9%±7.26%，三维GLS -16.8%±4.37%。峰值VO为1290±502 mL/分钟，预测峰值VO百分比为67.8%±15.6%。RHI为1.44±0.576。二维LS/GLS和3D-EF与预测峰值VO百分比相关（R = -0.28，P = 0.007；R = 0.24，P = 0.019）。3D-EF与RHI呈正相关（R = 0.29，P = 0.0071）。
在这组小儿Fontan患者中，SV收缩功能在基线时降低，并存在二维应变和3D-EF与预测峰值VO百分比之间的直接相关性，此外，3D-EF与内皮功能也存在相关性。
NCT04195451。
