Legionella pneumonia and bacteraemia in association with protein-losing enteropathy after Fontan operation

A 20-year-old young man suffered from severe protein-losing enteropathy 4 years after Fontan operation. His postoperative haemodynamics were unfavourable in terms of poor myocardial contractility and moderate atrioventricular prosthetic paravalvar leakage. A 2-week trial of steroid therapy for protein-losing enteropathy was given without success. The clinical course was complicated by Legionella pneumophila pneumonia and bacteraemia, which respond to the combination therapy with intravenous erythromycin and ciprofloxacin. The vulnerability of the patient to infection was related to an immuno-deficiency state secondary to protein-losing enteropathy and steroid therapy. Steroids should be used with caution in the management of protein-losing enteropathy after Fontan operation.