Surgical Management of straddling tricuspid valve and associated defects is a complex problem. Between August 1992 and August 1995, 5 patients with major straddling of the tricuspid valve underwent a complete or partial biventricular repair. All patients had either an inlet ventricular septal defect (n=4) or a ventricular septal defect with an inlet component (n=1), Co-existing cardiac lesions included hypoplasia of the right ventricle (n=3), discordant ventriculoarterial connections (n=1), tetralogy of Fallot (n=1), and multiple muscular vetricular septal defects (n=2). At the time of presentation to our institution, two of these patients had previously been palliated in preparation for a Fontan procedure, having undergone construction of a bidirectional superior cavopulmonary shunt. One patient was referred specifically for a Fontan procedure. The tricuspid valve was repaired by transecting all of the straddling cords and reattaching them in the right ventricle or onto the right side of the patch used to close the ventricular septal defect. Associated procedures included closure of the septum in all patients, an arterial switch procedure in one, repair of tetralogy of Fallot in one, and construction of a bidirectional superior cavopulmonary shunt in one. There has been no early or late mortality. Complete heart block requiring insertion of a pacemaker occurred after surgery in three patients. At a median follow-up of 32 months, functional integrity of the tricuspid valve is well maintained, with only one patient having moderate tricuspid regurgitation. None of the patients are receiving any cardiac medication.