Fetal presentation of congenital long QT syndrome

Thirty-two-week prenatal ultrasound revealed a fetal heart rate of 100 bpm with decreased variability on the heart rate tracing. Echocardiogram documented normal anatomy and sinus bradycardia. Newborn electrocardiogram revealed sinus rhythm at 100 bpm with a QTc of 0.657. Follow-up electrocardiogram revealed a QTc interval of 0.568, 2:1 atrioventricular block with a ventricular rate of 60 bpm, and ventricular ectopy. The infant received a pacemaker and beta-blocker therapy. Long QT syndrome should be in the differential diagnosis of the fetus with bradycardia and decreased heart rate variability in the absence of distress. Early diagnosis allowed for preventative care in the infant and identification of family members at risk.