Immune deficiency secondary to protein-losing enteropathy after Fontan procedure for tricuspid atresia

Author(s): R Chee, M J Thomas & R J Powell

Description

A 26 year old lady was referred to the Immunology department in January 2001 with persistent genital and perianal warts despite medical and surgical therapies. She was born at full term with tricuspid atresia, and hence underwent a Glen procedure aged 1 year, a Blalock operation aged 10 years, followed by a Fontan procedure aged 12 years. The Fontan procedure involves bypassing the right heart via an intra- or extra-cardiac tunnel to connect the inferior vena cava vein to the pulmonary artery thereby combining the pulmonary and systemic circulations. At the age 16 years, this lady experienced pulmonary emboli, which were treated with long-term warfarin. Aged 18 years the patient had an episode of cardiac failure, and at 20 years she developed secondary amenorrhoea with osteoporosis which was treated with hormone replacement therapy.