Protein-losing enteropathy (primary intestinal lymphangiectasia) after the Fontan operation: clinical analysis of nine cases.

Chang Gung Med J. 2006 Sep-Oct;29(5):505-12.

Lin WS,
Hwang MS,
Chung HT,
Chu JJ,
Lai MW,
Yang JS,
Huang SC,
Huang JL,
Su WJ.
Department of Pediatrics, Chang Gung Children's Hospital. 5, Fushing Street, Gueishan Shiang, Taoyuan, Taiwan 333, ROC.

BACKGROUND: Protein-losing enteropathy (PLE) is a serious complication of a Fontan operation and has a very high mortality rate. The purpose of this study was to investigate the incidence, clinical manifestations, diagnostic approaches, laboratory findings, therapeutic modalities and outcome of patients with PLE at our institution.

METHODS: The diagnosis of PLE was based on clinical manifestations and laboratory studies. We reviewed medical records of patients who received a Fontan operation at our hospital form July 1985 to October 2005.

RESULTS: A total 101 patients underwent various modifications of the Fontan procedure during this period. Nine of the 75 patients (12%) who survived 30 days after surgery developed PLE, including 4 boys and 5 girls. The median time interval between the Fontan operation and onset of PLE was 3.7 years (range 1.2 to 9.7 years). Laboratory examination showed low serum albumin levels and increased fecal alpha-1-antitrypsin excretion. Lymphangiectasia was found on intestinal biopsy. Six patients had cardiac catheterization after development of PLE which demonstrated an elevated mean right atrium pressure (22.5 +/- 6.4 mmHg, range 16 to 33 mmHg) and mean pulmonary artery pressure (22.3 +/- 6.4 mmHg, range 16 to 33 mmHg).

Treatment included diet modification, albumin infusion, diuretics, inotropes, corticosteroids, heparin, and surgery. Four patients received medical treatment only. Two of these patients died due to sepsis and heart failure and 2 survived with partial relief of PLE. The remaining five received surgery for PLE after medical treatment failure. Three of them died after the operation and the two survivors were free of PLE, but one died of ventricular tachycardia 8 years later. The overall mortality rate was 67% (6/9).

CONCLUSIONS: The current treatment for PLE is associated with a very high mortality rate. Further investigation is needed to determine the exact mechanism of the disease and to develop new therapeutic approaches.

PMID: 17214396 [PubMed - in process]