Truncus arteriosus (TA) is a rare cardiac anomaly constituting less than 1% of all congenital heart defects. The association of complete atrioventricular septal defect (AVSD) and truncus arteriosus is uncommon. The management of this uncommon combination of lesions has not been well characterized and there is limited surgical options for treatment of these patients. We present our case report of a late presentation of Down syndrome with unbalanced complete AVSD and type 1 truncus arteriosus in a tertiary hospital in South Africa.

Introduction: Acute rheumatic fever (ARF) is a post infectious, non-suppurative sequela of pharyngeal infection with streptococcal pyogens, or group a beta haemolytic streptococcus. Rheumatic heart disease (RHD) is a condition of global health importance. It is estimated that 15.6-19.6 million people are living with RHD, with almost 80% of those residing in low and middle income countries.