Discordant Atrioventricular Connections in Situs Inversus, Dextrocardia with Double Outlet Right Ventricle, Pulmonary Stenosis, and Anomalous Coronary Artery: A Rare Case Report.

ABSTRACT

We report a case of a 2-year-old female presenting with cyanosis since birth and having rare cardiac anatomy of visceral situs inversus, dextrocardia, discordant atrioventricular connections with double outlet right ventricle, atrial communication, subaortic interventricular communication, anteriorly leftward aorta with right aortic arch, single coronary artery with a branch crossing right ventricle outflow tract, pulmonary stenosis, and bilateral superior vena cava. Corrective repair with atrial switch (Senning technique), intracardiac baffle, and right ventricle outflow reconstruction was successfully performed with challenges from altered anatomical location and orientation of the heart and abnormal coronary artery.