Management of arrhythmias in patients with tetralogy of Fallot

Patients with tetralogy of Fallot are subject to arrhythmic sequelae that substantially impact morbidity and mortality. This review focuses on recent advances in our understanding of the prevalence and types of arrhythmias encountered. Diagnostic and prognostic tools are considered and therapeutic options discussed.

Multicenter studies have characterized the arrhythmia burden, assessed the impact of implantable cardioverter-defibrillators, and generated a risk score for primary prevention. Left ventricular hemodynamics are increasingly recognized as important contributors to risk for sudden death. Arrhythmia circuits have been characterized, and the impact of pulmonary valve replacement on sudden death has been further questioned. Recent studies cast doubt on the value of right ventricular pacing alone for cardiac resynchronization and provide a rationale for biventricular pacing.

Supraventricular arrhythmias exceed ventricular arrhythmias in prevalence, as atrial fibrillation increases with the aging population. Sudden death is the leading cause of late mortality, although therapeutic advances may alter this profile. Combinations of factors should be considered in risk stratification schemes to select appropriate implantable cardioverter-defibrillator candidates. The role of concomitant intraoperative ablation during pulmonary valve replacement surgery remains to be defined. Cardiac resynchronization therapy, particularly biventricular pacing, offers promise, but requires careful study before widespread implementation.