Autoimmune vasculitis in a child following tetralogy of Fallot repair in Uganda

Abstract

Tetralogy of Fallot is the most common cyanotic congenital heart disease worldwide [1]. Among other conotruncal abnormalities, 13-16% of Tetralogy of Fallot individuals are reported to have 22q11.2 deletion syndrome which is characterized by thymic hypoplasia, endocrine abnormalities and hypocalcaemia [2]. Individuals with micro deletion 22q11.2 deletion syndrome are prone to auto immune disorders. Defects in thymic development predispose them to impaired immune function especially T cell deficiency [3]. However autoimmune vasculitis has not been well documented in children with Tetralogy of Fallot. We report on an eleven-year-old child post Tetralogy of Fallot repair who presented with persistent fevers and an over whelming infection.