ccTGA with situs inversus, levocardia and pulmonary atresia

Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac anomaly which is characterized by atrioventricular (AV) and ventriculoarterial discordance (transposition of the great arteries), representing less than one percent of all congenital cardiac diseases (1–3). Associated cardiac anomalies are common with CCTGA and seen in eighty percent of all cases (4). The most relevant common associations are ventricular septal defect (VSD), pulmonary stenosis (PS), left AV valve (morphological tricuspid valve) regurgitation and complete heart block (2–4). CCTGA may present as situs solitus or situs inversus (5). In situs solitus, the morphologic right ventricle (mRV) and right atrium (RA) are located on the left and right side, respectively. On the other hand, situs inversus is characterized by the mirror-image location of the thoracic and abdominal organs. Levocardia usually exists in which the cardiac apex is directed to the left. The majority of these patients suffer from situs solitus, and only around thirty four percent of cases have situs inversus (5). Indication for surgery is also determined by the nature and severity of associated cardiac lesions (3). Herein, we present a rare case of an adult with CCTGA and situs inversus with levocardia which, associated with PS and VSD, is a very rare combination.