Long QT syndromes encompass the most prevalent group of ion channelopathies. Long QT syndromes are predominantly familial and predispose the affected individual to ventricular arrhythmias and sudden death. Permanent pacemaker insertion for long QT syndrome is discouraged apart from younger patients exhibiting 2:1 atrioventricular block. However, permanent pacemaker insertion is a relatively common procedure in neonates with atrioventricular block, and dual-chamber permanent pacemaker insertion in low birth weight infants is challenging. We describe the management of long QT syndrome − type 2 − presenting in an extremely preterm neonate including epicardial, dual-chamber permanent pacemaker insertion.
