We present a rare case of incidentally diagnosed Twiddler’s syndrome in a child 7 years after implantation of a dual-chamber pacemaker system with epicardial leads. During revision, an insulation defect of the ventricular lead was evident, despite unremarkable prior pacemaker lead testing. The lead was repaired and a new generator was suture-fixated to prevent re-occurrence of generator manipulation.

Transvenous placement of pacemaker leads is the recommended method for permanent pacemaking in children. We evaluated 11 children with transvenous ventricular pacemaker leads by digital subtraction angiography in order to determine the incidence of venous obstruction. The ages at the implantation ranged from 4.5 to 11.2 years with a mean of 8.05±2.17 and a median of 7.9 years. The interval from implantation to the time of procedure ranged from 12 to 64 months, with a mean of 37.4±19.2 and a median of 45 months. Complete occlusion of the subclavian vein was demonstrated in two patients. We also showed significant stenosis of the venous system in five further patients. Of the 7 patients, 5 had an extensive venous collateral circulation. We did not observe obstruction of the superior caval vein, or superior caval venous syndrome. Although we did not demonstrate this syndrome, we propose future regular evaluation of all our children undergoing transvenous pacemaking. This point may be important when second or third leads are implanted.