Repair of common atrioventricular canal associated with transposition of the great arteries and left ventricular outflow obstruction

A 9-year-old girl was successfully operated upon for transposition of the great arteries (TGA), complete atrioventricular (AV) canal, severe left ventricular outflow obstruction, absence of the coronary sinus, and a large left superior vena cava draining into the left upper corner of a common atrium. The interventricular communication was closed and the common AV orifice was partitioned, a Mustard operation was performed, and a valved conduit was inserted between the left ventricle and the pulmonary artery. This operation, which leads to a four-chamber heart, may be preferable for this condition to the previously reported Fontan type of repair. The same approach may be also used to correct those cases of double-outlet right ventricle with complete AV canal in which the interventricular communication does not extend into the perimembranous area.