DDD pacemaker implantation after Fontan-type operations

Bradyarrhythmias developing after Fontan-type operations impair the function of the univentricular heart causing fatigue, headaches, ascites, and protein-losing enteropathy (PLE). Transvenous inaccessibility, requiring epicardial implantation, accounts for the reluctance to implant a pacemaker (PM). Between 1997 and 2000, 24 patients (mean age 9.5 years, range 6 months to 19 years) with Fontan-type operations received DDD pacing systems with atrial steroid-eluting stitch-on electrodes (mean capture threshold 1.9 V/0.5 ms, range 0.4-3.5 V) and ventricular screw-in electrodes (mean capture threshold 1.7 V/0.5 ms, range 0.1-3 V). The systems were implanted at the time of conversion from atrio- to cavopulmonary connections in 5 patients, at the time of a total cavopulmonary Fontan operation in 6, and 1-50 months thereafter (mean = 18) in 13 patients. A right ventricular anatomy was present in 13 (54%) of 24 of PM recipients, versus 35% of the overall population. After a mean follow-up of 3.5 years, the PM were functioning in DDD mode in 23 of the 24 patients. Length of hospital stay in the ten patients who underwent repeat sternotomy was 5 days, without procedure related complications. In three children a repeat sternotomy was avoided by implanting the atrial electrodes during the Fontan operation. All patients improved clinically, including resolution of PLE in four patients. Bradyarrhythmias may lead to significant morbidity after Fontan-type operations. Electrophysiological evaluation is advised at follow-up. The indication for implantation of a DDD pacemaker system should be liberal. Placing atria] electrodes during the Fontan operation, especially in the presence of a right ventricular anatomy, avoids repeat sternotomy.