Sustained ventricular tachycardia in adult patients late after repair of tetralogy of Fallot.

OBJECTIVES: We sought to determine the features associated with sustained monoform ventricular tachycardia (VT) in adult patients late after repair of tetralogy of Fallot (TOF) and to review their management. BACKGROUND: Patients with repair of TOF are at risk for sudden death. Risk factors for ventricular arrhythmia have been identified from patients with ventricular ectopic beats because of the low prevalence of sustained VT. METHODS: From a retrospective chart review of patients assessed between January 1990 and December 1994, 18 adult patients with VT were identified and compared with 192 with repaired TOF free of sustained arrhythmia. RESULTS: There was no significant difference in age at repair, age at follow-up or operative history. Patients with VT had frequent ventricular ectopic beats (6 of 9 vs. 21 of 101), low cardiac index ([mean +/- SD] 2.4 +/- 0.4 vs. 3.0 +/- 0.8) and more structural abnormalities of the right ventricle (outflow tract aneurysms and pulmonary or tricuspid regurgitation) than control patients. Electrophysiologic map-guided operation was performed in 10 of 14 patients who required reoperation. VT has reoccurred in three of these patients. Four patients did not undergo operation (three received amiodarone; one underwent defibrillator implantation). Two patients with VT also had severe heart failure and died. CONCLUSIONS: Most patients with VT late after repair of TOF have outflow tract aneurysms or pulmonary regurgitation, or both. These patients have a greater frequency of ventricular ectopic beats than arrhythmia-free patients after repair of TOF. A combined approach of correcting significant structural abnormalities (pulmonary valve replacement or right ventricular aneurysmectomy, or both) with intraoperative electrophysiologic-guided ablation may reduce the potential risk of deterioration in ventricular function and enable arrhythmia management to be optimized.

Sustained atrial arrhythmias in adults late after repair of tetralogy of fallot.

We determined the prevalence of sustained atrial tachyarrhythmia (AT) in adults late after repair of tetralogy of Fallot (ToF) and examined its impact on subsequent heart failure, reoperation, and mortality. Ventricular arrhythmias are associated with increased morbidity and mortality in patients with repair of ToF. The clinical impact of AT in this population has not been established. A retrospective cohort study of 242 patients with repaired ToF identified 29 patients (prevalence of 12%) with sustained episodes of AT. Patients with repaired ToF but without sustained arrhythmia (n = 213) constituted a comparison group. Baseline characteristics and clinical outcomes in the 2 groups were compared. An echocardiographic analysis compared 15 patients with AT and 15 matched for age at operation and timing of echocardiography. The development of AT was associated with substantial morbidity including congestive heart failure, reoperation, subsequent ventricular tachycardia, stroke, and death (combined events, 20 of 29 patients [69%]). The rate of combined events (congestive heart failure, stroke, and deaths) in the 213 "arrhythmia-free" patients was 30% (64 of 213 patients). Event-free survival 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 surgical repair (25 +/- 16 vs 10 +/- 9 years, p = 0.001), and at most recent assessment were aged 48 +/- 12 vs 32 +/- 10 years (p = 0.001). The AT group had a higher mean right atrial volume and proportion of significant pulmonary regurgitation than matched controls. The development of AT in the adult late after ToF repair identifies patients at risk and is associated with older age at repair, a higher frequency of hemodynamic abnormalities, and increased morbidity.