Complete surgical correction of tetralogy of Fallot with Ebstein's anomaly.

Abstract

Association of Ebstein's anomaly of tricuspid valve (TV) with tetralogy of Fallot (TOF) is extremely rare. Their coexistence is unique as they modify each other's physiology. We report a case of a girl child having this rare combination along with Diamond-Blackfan syndrome awaiting a bone marrow transplant. She underwent intracardiac repair for TOF along with cone reconstruction of the TV, as tricuspid regurgitation of Ebstein's anomaly would worsen with TOF correction alone. Through this surgery, we aimed to achieve a competent pulmonary valve using a transannular patch with a monocusp and adequate relief of the right ventricular tract obstruction, thus preserving the right ventricular function, as both can affect the repaired TV function in the long term. The child had an uneventful postoperative course and is asymptomatic on follow-up.