Long-term outcome of adult survivors of tetralogy of Fallot

Abstract

Objective: To examine long-term outcomes after tetralogy of Fallot (TOF) repair. Methods: Single-centre retrospective study of 293 patients aged ≥16 years with previous TOF repair at a median age of 2.6 years (IQR:1.1–5.1 years). Surgical approach was trans-atrial (51% (149/293)), right ventriculotomy (34% (101/293)) and unknown (15%, 43/293). Results: After a median adult centre follow-up of 9 years (IQR:3.9–15.5 years, max 40), survival was excellent (n = 284, 97%). Age-adjusted survival was 99%, 95%, and 83% at 20, 40, and 60 years of age, respectively. Over half required ≥1 pulmonary valve (PV) intervention (166/293, 57%). Arrhythmia endpoints occurred in 24% (70/293) including atrial arrhythmia in 12% (34/293) and ventricular arrhythmia in 6% (18/293). Freedom from PV intervention and arrhythmia reduced with time (85% and 95% at 20, 46% and 82% at 40, and 16% and 31% at 60 years of age, respectively). The vast majority have excellent functional status at latest follow-up (NYHA Class I/II: 99.5%, 202/203). Independent risk factors for lower RVEF at latest cardiac magnetic resonance imaging (CMR) were male sex (p = 0.01), PV intervention (p = 0.02), increased QRS duration on ECG on latest follow-up (p < 0.0001), shorter exercise stress test duration (p < 0.0001), and decreased LVEF (p < 0.0001). RVEF was an independent risk factor for CMR-derived LVEF even after adjustment for follow-up age, sex, and RVEDVI (coefficient:0.4, p < 0.0001, 95% CI:0.2–0.5). Conclusions: Long-term adult survival after TOF repair is excellent. A significant proportion of patients require a PV intervention or experience an arrhythmia by the sixth decade of life. Lifelong follow-up for complications is essential.

Keywords

• Arrhythmia
• Mortality
• Outcomes
• Pulmonary valve replacement
• Tetralogy of fallot