[Successful primary correction for tetralogy of Fallot associated with total anomalous pulmonary venous drainage (type Ia)].

The surgical correction was performed successfully in a 3-year-old girl with tetralogy of Fallot associated with total anomalous pulmonary venous drainage (type Ia). The preoperative cardiac catheterization and angiography showed high systolic pulmonary artery pressure (58 mmHg) and small left ventricular volume (54% of normal). The early postoperative course was stormy because of the unstable circulatory state. During the early postoperative period, we estimated the volume of left ventricle by 2 D echocardiography. A significant increase of the volume was observed on the 8th postoperative day, when her circulatory state became stable. The cardiac catheterization before discharge revealed Pp/Ps of 0.38 and normal LV volume. We conclude that primary repair should be done for this combined cardiac anomaly, even if small left ventricle and pulmonary hypertension is present.