Permanent pacing in patients with congenital atrio-ventricular block--experience of 33 cases

The authors review permanent pacing in patients with congenital atrioventricular block (CAVB) and present their experience in permanent pacing in this pathology.

In a population of 4,355 patients submitted to implantation of permanent pacing between January 1980 and January 1998, 33 (0.75%) had CAVB. The mean age of the patient population with CAVB was 16.7 years (aged from eleven days to 35 years); 33% were below 10 years of age; 16 patients were male. The majority of the patients had symptoms of brain hypoperfusion; two patients had concomitant malignant ventricular tachyarrythmias (one of these with Torsade de Pointes due to congenital long QT syndrome). Transvenous (endocardial) pacing was used in 32 patients (two with previous epicardial pacing and exit block) and epicardial pacing in one. The mode of stimulation used was VVI in three patients, DDD in eight patients, VVIR in 14 patients, DDDR in four patients and VDD in four. Smaller pulse generators were used in children of lower weight. In recent years single lead VDD systems have been preferred whenever technically possible. Vascular access was the left cephalic vein in 17 patients; the left subclavian vein in 14 patients and the right jugular vein in one patient. During a mean follow-up of 6.9 years, two patients with ventricular stimulation systems developed "Pacemaker Syndrome" and required a change of mode of stimulation. Lead fracture and posterior cutaneous necroses were observed in two other patients, who were accordingly submitted to surgical revision. It was deemed necessary, one year later, to increase the lead loop in a child with a permanent pacemaker implanted at eleven days of age. No other complications occurred with the other patients; replacement of the pulse generators was performed in an elective manner.

CAVB is a rare indication for the implantation of a permanent pacemaker. In children, in the majority of cases, endocardial stimulation is possible in spite of the obvious technical difficulties due to low weight. Sequential, more physiological, stimulation systems should be preferred. However, VVIR stimulation systems of smaller dimensions can be the first choice of mode of stimulation in smaller children, mainly due to anatomical and technical limitations.