A first population-based long-term outcome study in adults with repaired tetralogy of Fallot in Malta

Abstract

Objectives: To determine overall and reintervention-free survival for repaired Maltese tetralogy of Fallot patients and to investigate the potential impact of gender, age at repair, genetic syndromes, previous palliation, and type of repair on these outcomes.

Design: All 130 tetralogy of Fallot patients born before the end of 1997 included in the local database were extracted. Surgical repair type, age at repair and operative survival were analyzed among the 103/130 repaired patients. Kaplan–Meier survival analyses were performed on the 75 repair survivors with complete follow-up data (mean follow-up 26.37 ± 9.27 (range 9.95–51.21) years).

Results: Patients born after 1985 were operated at a younger age (median 1.28 years) compared with patients born before 1985 (median 9.64 years) (P < .001). Transannular patch repair was the commonest operation among patients born after 1985 (43.90%), while repair without transannular patch use prevailed among those born before 1985 (66.13%). 90.24% of patients born after 1985 survived reparative surgery compared with 70.37% of those born before 1985. Of the 75 repair survivors, 7 (9.33%) died of cardiac causes and 22 (29.33%) needed reintervention during follow-up. Overall estimated mean survival was 45.56 years (95% CI 41.67–49.24) with estimated survival rate of 77.5% at 40 years from repair. Estimated mean reintervention-free survival was 37.71 years (95% CI 33.75–41.66) with estimated reintervention-free survival rate of 59.2% at 40 years. Patients with genetic syndromes had significantly lower overall survival after repair. Transannular patch repair was associated with significantly lower reintervention-free survival (median 32.37 years (95% CI 12.75–51.99)) compared with repair without transannular patch [median 44.21 years (95% CI 43.06–45.35); P = .03].

Conclusions: Although survival after tetralogy of Fallot repair in contemporary patients is very good, cardiac death can occur at any stage and structural reintervention is common. Regular follow-up with imaging and rhythm monitoring remains of utmost importance in all patients.