The development of transvenous ventricular pacing leads with proximal electrodes capable of atrial sensing and the recent availability of smaller generators has created the opportunity to treat children with complete AV block and normal sinus node function with a transvenous single lead VDD pacing system. Studies in adults have demonstrated this system to be efficacious with low complication rates. Transvenous single lead VDD pacemakers were implanted in ten children, aged 5-15 years, between December 1993 and April 1996, in our institution. The indications were complete AV block with severe bradycardia in 5 patients, second-degree or complete AV block following congenital heart surgery in 3, complete AV block with long QT syndrome in 1, and second-degree AV block and syncope in 1. There were no complications related to the procedure in any case. P and R wave amplitudes were measured and thresholds were determined intraoperatively on all patients. Amplitudes and thresholds were remeasured on seven patients with a mean follow-up of 17 months; Holter monitors were performed on seven patients with mean follow-up of 16 months. P and R wave amplitudes were generally diminished at follow-up compared to initial values but remained within an acceptable range for all patients. Four patients required reprogramming after pacemaker insertion, 1 received an atrial lead for dual chamber pacing, 1 required reposition for lead dislodgment, and 1 patient required a new lead for an inadequate ventricular pacing threshold. No patient had evidence of failure to sense or capture as evaluated by Holter monitoring at last follow-up. Single lead VDD pacing systems can be successfully used in properly selected children with high degree or complete AV block with normal sinus node function.