Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
University of Cincinnati College of Medicine, Department of Pediatrics, Cincinnati, OH, USA.
Cardiol Young. 2021 Aug;31(8):1283-1289. doi: 10.1017/S1047951121000159. Epub 2021 Jan 28.
Systemic venous hypertension and low cardiac output are believed to be important mediators of liver injury after the Fontan procedure. Pulmonary vasodilators have the potential to improve such haemodynamics. The aim of this study was to assess the acute effects of exercise on liver stiffness and venous pressures and to assess the impact of inhaled Treprostinil on this response.
In this prospective, double-blind, placebo-controlled, crossover trial, 14 patients with a Fontan circulation were randomised to inhalation of placebo and Treprostinil. Incremental and constant work rate exercise tests were performed to assess the effect of Treprostinil on exercise tolerance. Venous pressures were measured throughout and liver stiffness at rest and immediately after peak exercise.
Mean age was 27.8 ± 7.9 years and 66% were females. Exercise acutely increased liver stiffness by 30% (mean shear wave speed: 2.38 ± 0.71 versus 2.89 ± 0.51 ms, p = 0.02). Peripheral venous pressures increased acutely during both incremental (12.1 ± 2.4 versus 22.6 ± 8.0 mmHg, p < 0.001) and constant work rate exercise (12.5 ± 2.5 versus 23.4 ± 5.2 mmHg, p < 0.001). Overall, Treprostinil failed to attenuate exercise-induced increases in liver stiffness. Compared with placebo, Treprostinil did not significantly impact venous pressure responses, VO2peak, nor exercise endurance times.
Peripheral venous pressure increased acutely during exercise by an average of 88% above baseline and was not altered by administration of inhaled Treprostinil. Liver stiffness measured immediately post-exercise increased acutely by an average of 30%, with no attenuation following Treprostinil inhalation.
人们认为体循环静脉高压和心输出量低是法洛四联症根治术后肝损伤的重要介质。肺血管扩张剂有可能改善这种血液动力学。本研究的目的是评估运动对肝硬度和静脉压的急性影响，并评估吸入曲前列尼尔对这种反应的影响。
在这项前瞻性、双盲、安慰剂对照、交叉试验中，14 例法洛四联症患者被随机分为吸入安慰剂和曲前列尼尔组。进行递增和恒功运动试验，以评估曲前列尼尔对运动耐量的影响。在整个过程中测量静脉压，在休息时和峰值运动后立即测量肝硬度。
平均年龄为 27.8 ± 7.9 岁，66%为女性。运动使肝硬度在 30 分钟内急性增加 30%（平均剪切波速度：2.38 ± 0.71 与 2.89 ± 0.51 ms，p = 0.02）。在递增（12.1 ± 2.4 与 22.6 ± 8.0 mmHg，p < 0.001）和恒功运动（12.5 ± 2.5 与 23.4 ± 5.2 mmHg，p < 0.001）期间，外周静脉压均急性增加。与安慰剂相比，曲前列尼尔并不能显著减弱运动引起的肝硬度增加。与安慰剂相比，曲前列尼尔对静脉压反应、VO2peak 或运动耐力时间均无显著影响。
运动时外周静脉压平均增加 88%，高于基线，吸入曲前列尼尔并未改变。运动后立即测量的肝硬度平均增加 30%，吸入曲前列尼尔后无明显减弱。
