UCSF

Improvements in surgical and medical treatment have led to improved survival of infants with congenital heart disease. Coincident with this improved survival has been an increase in the number of patients with congenital heart disease and arrhythmia. Arrhythmias in this population can be life threatening but are more commonly life altering, creating considerable patient distress--both from the symptoms of the arrhythmia and from fear of paroxysmal events. Arrhythmia in the setting of congenital heart disease can result from hemodynamic compromise and can in turn result in more hemodynamic compromise, creating a cycle of clinical deterioration. Aggressive treatment of arrhythmia and aggressive evaluation for surgically correctable hemodynamic burdens is therefore warranted. Treatment options for arrhythmia in patients with congenital heart disease include pharmacologic therapy, catheter intervention, implantable device therapy, and surgical intervention. Pharmacologic therapy is currently the primary mode of treatment of arrhythmia in this setting; however, data from large trials of patients with ischemic or dilated cardiomyopathy suggest that many antiarrhythmic agents may increase overall mortality rates in certain patient groups. In addition, pharmacologic therapy is associated with significant short- and long-term side effects, relatively low success rates, and problems with compliance. For these reasons and because of recent advances in catheter and device therapy, nonpharmacologic therapy should be considered in lieu of medication, whenever possible.