This article describes two infants with prolonged QT interval and intermittent second-degree atrioventricular block. An asymptomatic 14-month-old child with persistent 2:1 atrioventricular conduction since birth underwent electrophysiology study including measurements with a contact monophasic action potential catheter. During 2:1 conduction, atrioventricular block occurred distal to the site of the His-bundle recording. Monophasic action potential duration was closely related to prior RR intervals. Single premature atrial or ventricular depolarizations during 1:1 conduction followed by a pause, lead to monophasic action potential prolongation and subsequent 2:1 atrioventricular conduction, which was perpetuated by the resulting long RR intervals. Paired premature ventricular contractions or short bursts of ventricular pacing elicited monophasic action potential shortening and subsequent 1:1 atrioventricular conduction that was perpetuated by the resulting short RR intervals. A second infant presented at birth with a prolonged QT interval, ventricular tachycardia, and episodes of second-degree atrioventricular block with persistent 2:1 atrioventricular conduction. The atrioventricular block was repeatedly elicited by single premature ventricular contractions and terminated by ventricular couplets. We conclude that the atrioventricular block in both patients is functional in nature and results from the interrelationships between ventricular rate, action potential duration, and His-Purkinje system refractoriness.