Heterotaxy — Heterotaxy, derived from Greek (hetero, meaning “different,” and taxy, meaning “arrangement”), is also referred to as visceral heterotaxy or heterotaxy syndrome. It is defined as an abnormal arrangement of the internal thoracic-abdominal organs across the left-right axis of the body. Patients with heterotaxy have been historically stratified into either the subsets of asplenia syndrome or polysplenia syndrome [1-3]. However, this classification is not useful in describing the cardiac malformations associated with heterotaxy.