Patel Jyoti K, Loomes Kathleen M, Goldberg David J, Mercer-Rosa Laura, Dodds Kathryn, Rychik Jack
Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Ann Thorac Surg. 2016 Mar;101(3):1025-30. doi: 10.1016/j.athoracsur.2015.09.036. Epub 2015 Dec 1.
Protein-losing enteropathy (PLE) is a challenging complication after a Fontan operation. Subclinical enteric protein loss may precede development of overt PLE. We evaluated the acute effects of Fontan circulation on enteric protein loss and mesenteric vascular resistance.
A prospective cohort study was performed evaluating enteric protein loss in children undergoing Fontan operations. Stool alpha-1 antitrypsin (A1AT) concentration was measured in the preoperative, early postoperative, and intermediate postoperative (3-9 months) periods. The intestinal circulation was characterized by Doppler-derived resistance indices of the superior mesenteric artery (SMA), and serum albumin and protein levels were obtained.
We enrolled 33 participants at a median age at operation of 3.0 years (interquartile range [IQR], 2.5-3.3 years). No clinical PLE was observed. Six of the 93 stool samples obtained had elevated A1AT levels (>54 mg/dL), with 2 abnormal samples at each of the 3 time points. Two of the 5 participants with elevated stool A1AT values had significant hemodynamic disturbances requiring intervention (junctional bradycardia or tricuspid stenosis). There was no difference in SMA resistance in the preoperative versus early postoperative periods (p = 0.9). Serum albumin levels were lower in the early postoperative period compared with the preoperative period (3.2 mg/dL [{IQR}, 2.9-3.5] versus 4.1 mg/dL; IQR, 3.4-4.5; p = 0.01) but did not correlate with abnormal stool A1AT concentration or SMA resistance indices.
The Fontan operation does not commonly result in acute development of increased enteric protein loss. However, increased enteric protein loss may occur in children before or after a Fontan operation, particularly when hemodynamic disturbances are present.
蛋白质丢失性肠病（PLE）是Fontan手术后具有挑战性的并发症。亚临床肠道蛋白质丢失可能先于显性PLE的发生。我们评估了Fontan循环对肠道蛋白质丢失和肠系膜血管阻力的急性影响。
进行了一项前瞻性队列研究，评估接受Fontan手术的儿童的肠道蛋白质丢失情况。在术前、术后早期和术后中期（3 - 9个月）测量粪便α-1抗胰蛋白酶（A1AT）浓度。通过多普勒得出的肠系膜上动脉（SMA）阻力指数来表征肠道循环，并获取血清白蛋白和蛋白质水平。
我们纳入了33名参与者，手术时的中位年龄为3.0岁（四分位间距[IQR]，2.5 - 3.3岁）。未观察到临床PLE。在获取的93份粪便样本中，有6份A1AT水平升高（>54 mg/dL），在3个时间点各有2份异常样本。5名粪便A1AT值升高的参与者中有2名出现需要干预的显著血流动力学紊乱（交界性心动过缓或三尖瓣狭窄）。术前与术后早期的SMA阻力无差异（p = 0.9）。术后早期的血清白蛋白水平低于术前（3.2 mg/dL [IQR，2.9 - 3.5] 对 4.1 mg/dL；IQR，3.4 - 4.5；p = 0.01），但与异常粪便A1AT浓度或SMA阻力指数无关。
Fontan手术通常不会导致肠道蛋白质丢失急性增加。然而，在Fontan手术前后，儿童可能会出现肠道蛋白质丢失增加，特别是在存在血流动力学紊乱时。
