We determined the prevalence of sustained atrial tachyarrhythmia (AT) in ad
ults late after repair of tetralogy of Fallot (ToF) and examined its impact
on subsequent heart failure, reoperation, and mortality. Ventricular arrhy
thmias are associated with increased morbidity and mortality in patients wi
th repair of ToF. The clinical impact of AT in this population has not been
established. A retrospective cohort study of 242 patients with repaired To
F identified 29 patients (prevalence of 12%) with sustained episodes of AT.
Patients with repaired ToF but without sustained arrhythmia (n = 213) cons
tituted a comparison group. Baseline characteristics and clinical outcomes
in the 2 groups were compared. An echocardiographic analysis compared 15 pa
tients with AT and 15 matched for age at operation and timing of echocardio
graphy. The development of AT was associated with substantial morbidity inc
luding congestive heart failure, reoperation, subsequent ventricular tachyc
ardia, stroke, and death (combined events, 20 of 29 patients [69%]). The ra
te of combined events (congestive heart failure, stroke, and deaths) in the
213 "arrhythmia-free" patients was 30% (64 of 213 patients). Event-free su
rvival after repair was 18 +/- 2 years for the AT group and 28 +/- 1 years
for the arrhythmia-free group (p <0.001). Patients with AT were older at su
rgical repair (25 +/- 16 vs 10 +/- 9 years, p = 0.001), and at most recent
assessment were aged 48 +/- 12 vs 32 +/- 10 years (9 = 0.001), The AT group
had a higher mean right atrial volume and proportion of significant pulmon
ary regurgitation than matched controls. The development of AT in the adult
late after ToF repair identifies patients at risk and is associated with o
lder age at repair, a higher frequency of hemodynamic abnormalities, and in
creased morbidity, (C) 2001 by Excerpta Medica, Inc.