INTRODUCTION: This study was conducted to address whether staged surgical intervention or primary repair would be preferred for treating patients with tetralogy of Fallot and small left ventricle. METHODS: We retrospectively assessed 28 patients with tetralogy of Fallot and small left ventricle at the Chinese PLA 208th Hospital, Changchun, between January 2005 and December 2008. Of this cohort, 11 patients had undergone a systemic-to-pulmonary arterial shunt with a polytetrafluoroethylene interposition graft (off pump), followed by ancillary repair procedures as a surgically staged group; the remaining 17 patients underwent primary intracardiac repair via cardiopulmonary bypass. The oxygen level as measured by pulse oximetry (SpO2), the McGoon ratio, and the left ventricular end-diastolic volume index (LVEDVI) were measured before shunt procedures and at 6 to 18 months afterwards. All data were analyzed statistically with the paired Student t test. The 2 groups were compared with respect to LVEDVI values and McGoon indices at baseline by the independent t test. RESULTS: None of the patients died as a direct result of the surgery, but 1 fatality due to pneumonia occurred 4 months after shunting. Mean postshunt values for SpO2, the McGoon ratio, and the LVEDVI increased significantly from 76.8% ± 9.0% to 87.5% ± 2.4% (P < .01), from 1.42 ± 0.27 to 1.62 ± 0.32 (P < .05), and from 20.1 ± 7.0 mL/m2 to 34.3 ± 7.4 mL/m2 (P < .01), respectively. Nine patients were fully repaired within the 6- to 18-month time frame after shunting, while 1 patient awaited definitive surgery for pulmonary hypoplasia. In the primary-repair group, in which the increases the LVEDVI and McGoon ratio were comparatively higher, 1 patient died of hypoxemia. CONCLUSION: A LVEDVI 20 mL/m2 may be an indication for primary repair in patients with tetralogy of Fallot. With values <20 mL/m2, staged procedures (i.e., shunt with a polytetrafluoroethylene interposition graft) can promote left ventricle development and allow safe transition to the final repair.