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Straddling atrioventricular valve (SAVV) is a rare anomaly. Only recently have the premortem diagnostic features been elucidated. Repair of the associated ventricular septal defect poses a considerable technical problem. Corrective operations, infrequently reported, have usually involved replacement of the SAVV. This report describes the successful management of a patient with a straddling left atrioventricular valve in whom the valve was preserved by a technique previously used for septation of the univentricular heart. This technique offers a useful alternative to valve replacement in the management of patients with SAVV.
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