Abstract
Protein-losing enteropathy (PLE) is a serious complication of congenital heart disease (CHD) and is associated with pronounced mortality. Medical management of PLE has only been partially successful. This patient improved temporarily using heparin therapy after a Rastelli operation for complete atrioventricular septal defect, double outlet right ventricle, and pulmonary atresia. However, the patient suffered a recurrence of PLE after three months of being weaned off heparin. The patient was switched to steroid therapy, then a combination of heparin and a small amount of steroid therapy, and finally only heparin therapy. Unfortunately, the patient died due to respiratory failure. The treatment of PLE with heparin therapy requires frequent subcutaneous injections, which are unpleasant for the patient. Although heparin therapy was temporarily effective, it did not lead to complete remission.
