Thorne S A, Hooper J, Kemp M, Somerville J
Grown up Congenital Heart Disease Unit, Royal Brompton Hospital, London, U.K.
Eur Heart J. 1998 Mar;19(3):514-20. doi: 10.1053/euhj.1997.0777.
Protein losing enteropathy is a serious complication of Fontan surgery. The aim of this study was to investigate gastro-intestinal protein loss in adults with congenital heart disease, both with and without Fontan surgery, and to correlate findings with systemic venous pressure.
Forty eight patients were studied. The first group included adult survivors of Fontan surgery. The second and third groups were control patients with congenital heart disease who had not had Fontan surgery and had either normal or chronically elevated systemic venous pressure. Gastro-intestinal protein loss was assessed by measurement of faecal alpha-1-antitrypsin. Faecal alpha-1-antitrypsin levels were significantly higher in the Fontan group (0.55 +/- 0.15 mg. g-1 faeces, P = 0.002) and the control group with chronically elevated venous pressure (0.60 +/- 0.30 mg. g-1 faeces, P < 0.001) compared to the controls with normal venous pressure (0.29 = 0.12 mg. g-1 faeces). Of the 15 subjects who were found to have increased gastro-intestinal protein loss, only four had clinical protein-losing enteropathy. The degree of gastro-intestinal protein loss correlated significantly with venous pressure (P = 0.01) and with serum aspartate transaminase (P = 0.04).
Increased gastro-intestinal protein loss is common in this select group of late survivors of Fontan surgery and in other subjects with congenital heart disease and chronic elevation of systemic venous pressure, and was present in patients who did not have protein-losing enteropathy. Increased faecal alpha-1-antitrypsin is an important finding in these patients as intervention at this stage, before the onset of florid protein-losing enteropathy, might prevent the development of further complications.
蛋白丢失性肠病是Fontan手术的一种严重并发症。本研究的目的是调查先天性心脏病成人患者（无论是否接受Fontan手术）的胃肠道蛋白丢失情况，并将研究结果与体静脉压进行关联分析。
对48例患者进行了研究。第一组包括Fontan手术的成年幸存者。第二组和第三组为未接受Fontan手术的先天性心脏病对照患者，其体静脉压正常或长期升高。通过测量粪便α-1抗胰蛋白酶来评估胃肠道蛋白丢失情况。与体静脉压正常的对照组（0.29±0.12mg·g⁻¹粪便）相比，Fontan组（0.55±0.15mg·g⁻¹粪便，P = 0.002）和体静脉压长期升高的对照组（0.60±0.30mg·g⁻¹粪便，P < 0.001）的粪便α-1抗胰蛋白酶水平显著更高。在15例被发现胃肠道蛋白丢失增加的受试者中，只有4例患有临床蛋白丢失性肠病。胃肠道蛋白丢失程度与静脉压（P = 0.01）和血清天冬氨酸转氨酶（P = 0.04）显著相关。
在这组经过筛选的Fontan手术晚期幸存者以及其他患有先天性心脏病且体静脉压长期升高的受试者中，胃肠道蛋白丢失增加很常见，且在未患蛋白丢失性肠病的患者中也存在。粪便α-1抗胰蛋白酶增加是这些患者的一个重要发现，因为在明显的蛋白丢失性肠病发作之前的这个阶段进行干预，可能会预防进一步并发症的发生。
