Congenital complete atrioventricular block can be concomitant with congenital heart diseases or maternal connective tissue disorders like systemic lupus erythematosus and Sjögren's syndrome. Such patients may require implantation of a permanent pacemaker due to ventricular dysfunction. While many methods of pacemaker implantation have been tested, one that is optimal for low birth weight infants remains to be determined. We present a preterm infant with maternal Sjögren's syndrome with congenital heart block and describe the technique for implantation of an intrathoracic dual-chamber pacemaker.
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