Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare form of congenital heart disease with a high risk of perinatal mortality, particularly when diagnosed before birth. We aimed to assess whether global left and right ventricular function in fetal TOF/APV, using the Tei index, correlate with outcome. We reviewed the fetal echocardiogram and clinical outcome of eight fetuses diagnosed with TOF/APV. Of the eight cases, four developed cardiovascular compromise, leading to intrauterine death in two fetuses and neonatal demise in two fetuses, and four fetuses survived the neonatal period. The right ventricular (RV) Tei index was significantly greater and the left ventricular (LV) Tei index tended to be greater in nonsurvivors compared with survivors with TOF/APV (RV Tei, 0.90 +/- 0.17 versus 0.30 +/- 0.28, p < 0.05; LV Tei, 0.97 +/- 0.42 versus 0.54 +/- 0.21). The global LV and RV function can be affected in TOF/APV. Furthermore, more severe pulmonary insufficiency and worse biventricular function as assessed by Tei index likely contribute to the high perinatal mortality associated with this disease.