ABSTRACT
BACKGROUND: A 40-year-old male with pentalogy of Fallot (a congenital heart defect with five anatomical components) presented with recurrent gastrointestinal bleeding. He had recently recovered from a heart operation, which was performed to reconstruct the right ventricular outflow tract. INVESTIGATIONS: Laboratory tests and absorption tests, esophagogastroduodenoscopy, capsule endoscopy, human serum albumin scintigraphy, lymphoscintigraphy, CT and abdominal lymph-node histology. DIAGNOSIS: Intestinal lymphangiectasia with concurrent protein-losing gastroenteropathy and recurrent gastrointestinal bleeding. MANAGEMENT Despite a low-fat diet and surgical suturing of multiple small-bowel ulcerations the gastrointestinal bleeding continued. Serum albumin levels remained very low and severe lymphedema occurred. Unfortunately, the patient developed severe sepsis and died of multiple organ failure.
