Diab Simone Goa, Rösner Assami, Døhlen Gaute, Brun Henrik, Grindheim Guro, Vithessonthi Kanyalak, Friedberg Mark K, Holmstrøm Henrik, Möller Thomas
Department of Paediatric Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
Department of Cardiology, University Hospital of North Norway, Tromsø, Norway; Department of Clinical Medicine, UiT, The Arctic University of Norway, Tromsø, Norway.
J Am Soc Echocardiogr. 2025 Apr;38(4):342-352. doi: 10.1016/j.echo.2024.11.005. Epub 2024 Nov 15.
Fontan circulatory failure with impaired systolic function is well documented; however, its mechanisms are not fully understood. The aim of this study was to explore myocardial functional reserve in adolescent patients with Fontan circulation in response to exercise or acute preload increase.
The study included 32 patients (median age, 16.7 years; range, 15.4-17.9 years; 12 female patients) with Fontan circulation. Echocardiographic imaging was performed during exercise using a recumbent cycle ergometer and during heart catheterization with a rapid infusion of 0.9% saline infusion at 5 mL/kg body weight. Myocardial peak longitudinal strain (LS) was measured in a four-chamber view during specific time intervals before, during, and after exercise (LS) and volume load (LS). During catheterization, central venous pressure and ventricular end-diastolic pressure were simultaneously recorded. A control group of 16 healthy individuals participated in the exercise test.
Mean LS was less negative for patients than for control subjects (P ≤ .001 at all stages); however, it significantly improved from -18.4 ± 5.5% at baseline to -22.0 ± 6.5% (P = .004) at maximal loading. LS at maximal loading did not correlate with changes in heart rate. During catheterization, mean LS was -19.6 ± 6.0% at baseline and did not improve significantly at 1.00 to 2.00 minutes and at 4.00 to 6.00 minutes after saline infusion. In more than half of the patients, LS worsened or improved by less than -2% after saline infusion. Worsening of LS correlated with central venous pressure and ventricular end-diastolic pressure in all conditions (P ≤ .017). There was no difference in LS or LS between the morphologic right ventricle and the morphologic left ventricle.
Patients with Fontan circulation demonstrate systolic myocardial functional reserve that can be recruited with exercise stress but not with an acute increase in preload.
收缩功能受损的Fontan循环衰竭已有充分记录；然而，其机制尚未完全明确。本研究的目的是探讨Fontan循环青少年患者在运动或急性前负荷增加时的心肌功能储备。
本研究纳入32例Fontan循环患者（中位年龄16.7岁；范围15.4 - 17.9岁；12例女性患者）。使用卧式自行车测力计在运动期间以及在心脏导管插入术期间以5 mL/kg体重快速输注0.9%生理盐水时进行超声心动图成像。在运动（纵向应变[LS]）和容量负荷（LS）之前、期间和之后的特定时间间隔内，在四腔视图中测量心肌峰值纵向应变。在导管插入术期间，同时记录中心静脉压和心室舒张末期压力。16名健康个体组成的对照组参与运动测试。
患者的平均LS较对照组的负性更小（所有阶段P≤0.001）；然而，其从基线时的-18.4±5.5%显著改善至最大负荷时的-22.0±6.5%（P = 0.004）。最大负荷时的LS与心率变化无关。在导管插入术期间，基线时平均LS为-19.6±6.0%，在输注生理盐水后1.00至2.00分钟以及4.00至6.00分钟时未显著改善。超过一半的患者在输注生理盐水后LS恶化或改善小于-2%。在所有情况下，LS的恶化与中心静脉压和心室舒张末期压力相关（P≤0.017）。形态学右心室和形态学左心室之间的LS或LS无差异。
Fontan循环患者表现出收缩期心肌功能储备，可通过运动应激激发，但不能通过急性前负荷增加激发。
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