The coexistence of significant congenital cardiac disease with accesso
ry pathways is not uncommon, and presents a number of issues for clini
cal management. These issues include the propensity of such patients t
o have poorly tolerated tachycardia; the difficulties in choosing appr
opriate medical management given potential problems with preexisting s
inus node disease, poor contractility, and the possibility of proarrhy
thmia; the need to seriously consider ablation prior to, or at the tim
e of, intracardiac repair of congenital cardiac defects; and finally,
the difficulties that complex anatomy may pose to the interventional e
lectrophysiologist. Definitive management requires expertise both in e
lectrophysiology and in pediatric cardiology. Success can usually be a
chieved with a careful, anatomically precise approach, combined with i
magination and persistence. Because of the difficulties with medical m
anagement, catheter ablation may be the most attractive modality for c
ontrol of accessory pathway tachycardia in this patient population.