This retrospective study was designed to evaluate the effectiveness of subcutaneous
heparin therapy for the treatment of
protein-losing enteropathy (PLE) associated with single-ventricle palliation and to evaluate the side effects of long-term
heparin use. PLE affects 4% to 13% of Fontan operative survivors. Five-year survival after onset of PLE is only 46% to 59%. We studied a cohort of patients with single-ventricle palliation who developed PLE and were treated with subcutaneous
heparin. Seventeen patients were included in the study. Symptoms of PLE appeared on average 43 months after surgical palliation. At diagnosis of PLE, mean
albumin level was 2.0 +/- 0.4 g/dl. At cardiac catheterization, mean systemic venous pressure was 11.6 mm Hg. Subjective symptomatic improvement on
heparin therapy occurred in 13 patients (76%). Three patients (18%) went into clinical remission. Compared with the period before initiation of
heparin, there was no significant difference in the number of hospital admissions (p = 0.99) or
albumin infusions (p = 0.88) during the first year of
heparin therapy. Five patients had x-rays of their thoracolumbar spine, and 9 patients had bone
mineral analyses; all scans were grossly abnormal. In conclusion, subcutaneous
heparin therapy leads to subjective improvement of PLE symptoms in most patients; however, it does not change the need for frequent
albumin infusions and does not increase the rate of remission above that for standard medical
therapy.