engleski

LONG TERM HEPARIN TREATMENT OF PROTEIN LOSING ENTEROPATHY IN A CHILD WITH HETEROTAXY AFTER FONTAN PROCEDURE

Protein-losing enteropathy is a serious complication after Fontan procedure with high mortality rate and, by now, unknown pathogenesis. Several successfully treated cases of PLE with high molecular weight heparin have been reffered to in literature. We present the case of a 10-year old girl who developed PLE and has been treated with HMWH for a period of 50 months. We present a girl with the initial diagnosis of heterotaxia syndrome(dextrocardia, single inlet ventricle of undetermined type, single AV valve, pulmonary atresia, hemyazygos vein continuity). The first palliative operation of Blalock-Taussig shunt on the right side was performed in early infant age, followed by bidirectional Glenn anastomosis at the age of 1. At teh age of 3 Fontan procedure with fenestration was performed. Four months later teh child developed PLE with tipical clinical and laboratory signs: chronic pleural effusions, hepatomegaly, edema, hypoalbuminemia, hypocalcemia, lymphopenia and enteral losing of proteins (elevated levels of a1-antitripsin in stool). Clinical condition was improved after reoperation on cardiopulmonary bypass due to stenosis of pulmonary arteries, but after 3 months the PLE developed again. In June 1997 the therapy with HMWH was started with the initial dose of 8000i.j./m2 with gradual increasement of the dose until clinical achievement and normalization of laboratory findings were achieved after 5 months with a dose of 15 000 i.j./m2. Due to HMWH therapy and PLE, the child developed sacrodynia with the possibility of lumbal vertebrae fracture. Bone densitomerty showed a significant reduction of bone density, and Rocaltrol and elementary calcium were introduced in therapy. Every attempt to lower HMWH dose ended in worsening of clinical condition so, the therapy of 15 000 i.j. /m2 has been continued. Total protein serum level is 45-50 g/L, with albumin fraction of 20-25 g/L . Serum calcium level is kept insid normal values. Conclusion: Lomg-term PLE treatment after Fontan (TCPC) procedure is possible with high doses of HMWH, with prevention of bone demineralization with vitamine D and calcium. Besides adequate high level protein, low fat diet is required.

heparin; protein-losing enteropathy; Fontan procedure

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