Seven consecutive patients with total anomalous pulmonary venous drainage were operated at the Medical College of Ohio at Toledo and long-term follow-up is reported on their rhythm status. The patients were generally under 3 months of age at operation. All patients are alive at the present time. The median length of follow-up is 4 years. Five of the 7 patients demonstrated ectopic pacemaker activity and 2 exhibited episodes of nodal rhythm as well. In general, these were not apparent on standard screening electrocardiography. There was no correlation between the presence or absence of this arrhythmia and the type of repair. It is concluded that arrhythmias may be present in these patients, but that they are unrelated to the type of surgery performed. The ectopic atrial focus may be of no clinical significance but there is more cause for concern in those patients with nodal rhythm.