Miranda William R, Kamath Patrick S, Jain C Charles, Connolly Heidi C, Egbe Alexander C
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA.
Can J Cardiol. 2023 Oct;39(10):1349-1357. doi: 10.1016/j.cjca.2023.04.024. Epub 2023 May 5.
Alterations in liver perfusion and venous hypertension have been implicated in the pathophysiology of Fontan-associated liver disease (FALD). However, the correlation between exercise hemodynamics and markers of FALD have not been studied.
We performed a retrospective review of 32 consecutive adults undergoing exercise catheterisation at the Mayo Clinic, Minnesota. Invasive hemodynamics were correlated with aspartate transaminase to platelet ratio index (APRI) and the Fibrosis-4 (Fib-4) score, well validated surrogates of liver fibrosis.
The mean age was 30.9 ± 7 years. The mean APRI was 0.5 ± 0.2 and the mean Fib-4 score 1.3 ± 0.8. Fib-4 scores correlated with spleen size on abdominal imaging (r = 0.40; P = 0.03). Resting Fontan pressure was 13.9 ± 3.9 mm Hg and pulmonary artery wedge pressure (PAWP) 10.0 ± 3.5 mm Hg. At peak exercise (69.4 ± 23.2 W), Fontan pressures increased to 26.5 ± 6.2 mm Hg and PAWP to 22.4 ± 7.1 mm Hg. APRI and Fib-4 score were directly related to Fontan pressure and PAWP at rest and during exercise, and inversely related to exercise arterial O saturation. Fib-4 inversely correlated with O delivery indices. Similarly, when categorising patients according to high APRI (> 0.5 vs ≤ 0.5) or Fib-4 score (≥ 1.45 vs < 1.45) according to previously proposed cutoffs for diagnosis of liver fibrosis, those with elevated scores had higher resting and exercise Fontan and PAWP pressure with lower O arterial saturation.
APRI and Fib-4 score correlated with resting and exercise Fontan pressure and PAWP. In addition, Fib-4 scores were inversely related to O delivery indices. These findings support a role played by hepatic venous hypertension and reduced O supply in patients with FALD.
肝脏灌注改变和静脉高压与Fontan相关肝病（FALD）的病理生理学有关。然而，运动血流动力学与FALD标志物之间的相关性尚未得到研究。
我们对明尼苏达州梅奥诊所连续32例接受运动导管检查的成年人进行了回顾性研究。有创血流动力学与天冬氨酸转氨酶与血小板比值指数（APRI）和Fibrosis-4（Fib-4）评分相关，这两者是经过充分验证的肝纤维化替代指标。
平均年龄为30.9±7岁。平均APRI为0.5±0.2，平均Fib-4评分为1.3±0.8。Fib-4评分与腹部影像学检查中的脾脏大小相关（r = 0.40；P = 0.03）。静息Fontan压力为13.9±3.9 mmHg，肺动脉楔压（PAWP）为10.0±3.5 mmHg。在运动峰值（69.4±23.2 W）时，Fontan压力升至26.5±6.2 mmHg，PAWP升至22.4±7.1 mmHg。APRI和Fib-4评分在静息和运动时与Fontan压力和PAWP直接相关，与运动时动脉血氧饱和度呈负相关。Fib-4与氧输送指数呈负相关。同样，根据先前提出的肝纤维化诊断临界值，将患者按高APRI（>0.5 vs≤0.5）或Fib-4评分（≥1.45 vs<1.45）进行分类时，评分升高的患者静息和运动时的Fontan和PAWP压力更高，动脉血氧饱和度更低。
APRI和Fib-4评分与静息和运动时的Fontan压力及PAWP相关。此外，Fib-4评分与氧输送指数呈负相关。这些发现支持肝静脉高压和氧供应减少在FALD患者中所起的作用。
